Atlas of the Newborn [Vol 1] - A. Rudolph (BC Decker, 1997) WW

Rudolph

NewbornAtlas of the

V O L U M E 1

Neonatal and Perinatal Medicine

V O L U M E 1


NewbornAtlas of the

Arnold J. Rudolph, M.D.(Deceased)

Professor of PediatricsBaylor Medical College

Houston, Texas

V O L U M E 1


Arnold J. Rudolph, M.D. (Deceased)

Professor of Pediatrics Baylor Medical College

Houston, Texas1997

B.C. Decker Inc. Hamilton • London

NewbornAtlas of the

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ForewordSir William Osler stated, “There is no more

difficult task in medicine than the art ofobservation.” The late Arnold Jack Rudolphwas an internationally renowned neonatolo-gist, a teacher’s teacher, and, above all, onewho constantly reminded us about how muchcould be learned by simply observing, in hiscase, the newborn infant.

This color atlas of neonatology represents adistillation of more than 50 years of observingnormal and abnormal newborn infants. TheAtlas begins with a section on the placenta,its membranes, and the umbilical cord. JackRudolph delighted in giving a lecture entitled“Don’t Make Mirth of the Afterbirth,” inwhich he captivated audiences by showingthem how much you could learn about thenewborn infant from simply observing theplacenta, its membranes, and the umbilicalcord.

In a few more than 60 photomicrographs,we learn to read the placenta and gain insightinto such disorders as intrauterine growthretardation, omphalitis, cytomegalic inclu-sion disease, congenital syphilis, and congen-ital neuroblastoma. Congenital abnormalitiesof every organ system are depicted along withthe appearance of newborn infants who havebeen subjected in utero to a variety of differ-ent drugs, toxins, or chemicals. We also learnto appreciate the manifestations of birth trau-ma and abnormalities caused by abnormalintrauterine positioning.

More than 250 photographs are used toillustrate the field of neonatal dermatology.The collection of photographs used in thissection is superior to that which I have seenin any other textbook or atlas of neonatologyor dermatology; this section alone makes thisreference a required addition to the library ofany clinician interested in the care of infantsand children. Photographs of the Kasabach-Merritt syndrome (cavernous hemangiomawith thrombocytopenia), Klippel-Trénaunaysyndrome, Turner’s syndrome, Waardenburg’ssyndrome, neurocutaneous melanosis, mas-tocytosis (urticaria pigmentosa), and incon-

tinentia pigmenti (Bloch-Sulzberger syn-drome) are among the best that I have seen.

Cutaneous manifestations are associatedwith many perinatal infections. The variedmanifestations of staphylococcal infection ofthe newborn are depicted vividly in photomi-crographs of furunculosis, pyoderma, bullousimpetigo, abscesses, parotitis, dacryocystitis,inastitis, cellulitis, omphalitis, and funisitis.Streptococcal cellulitis, Haemophilus influen-zae cellulitis, and cutaneous manifestations oflisteriosis all are depicted. There are numer-ous photomicrographs of congenital syphilis,showing the typical peripheral desquamativerash on the palms and soles, as well as otherpotential skin manifestations of congenitalsyphilis which may produce either vesicular,bullous, or ulcerative lesions. The variousradiologic manifestations of congenitalsyphilis, including pneumonia alba, ascites,growth arrest lines, Wegner’s sign, periostitis,and syphilitic osteochondritis, are depicted.Periostitis of the clavicle (Higouménaki’ssign) is shown in a photograph that alsodepicts periostitis of the ribs. A beautiful pho-tomicrograph of Wimberger’s sign also hasbeen included; this sign, which may appear inan infant with congenital syphilis, revealsradiolucency due to erosion of the medialaspect of the proximal tibial metaphysis.

The Atlas also includes a beautiful set ofphotographs which delineate the ophthalmo-logic examination of the newborn. Lesionswhich may result from trauma, infection, orcongenital abnormalities are included. Thereare numerous photographs of the ocular man-ifestations of a variety of systemic diseases,such as Tay-Sachs disease, tuberous sclerosis,tyrosinase deficiency, and many more.Photographs of disturbances of each of thevarious organ systems, or disorders affectingsuch organ systems, also are included alongwith numerous photographs of different formsof dwarfism, nonchromosomal syndromes andassociations, and chromosomal disorders. Inshort, this Atlas is the complete visualtextbook of neonatology and will provide any

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physician, nurse, or student with a distillationof 50 years of neonatal experience as viewedthrough the eyes of a master clinician.

Arnold Jack Rudolph was born in 1918,grew up in South Africa, and graduated fromthe Witwatersrand Medical School in 1940.Following residency training in pediatrics atthe Transvaal Memorial Hospital forChildren, he entered private pediatric prac-tice in Johannesburg, South Africa. Afteralmost a decade, he left South Africa andmoved to Boston, where he served as a SeniorAssistant Resident in Medicine at theChildren’s Medical Center in Boston,Massachusetts, and subsequently pursued fel-lowship training in neonatology at the sameinstitution and at the Boston Lying-InHospital, Children’s Medical Center andHarvard Medical School under Dr. ClementA. Smith.

In 1961, Dr. Rudolph came to BaylorCollege of Medicine in Houston, Texas, theschool at which he spent the remainder of hiscareer. He was a master teacher, who receivedthe outstanding teacher award from pediatricmedical students on so many occasions thathe was elected to the Outstanding FacultyHall of Fame in 1982. Dr. Rudolph alsoreceived numerous awards over the years fromthe pediatric house staffs for his superb teach-ing skills.

He was the Director of the NewbornSection in the Department of Pediatrics atBaylor College of Medicine for many years,until he voluntarily relinquished that posi-tion in 1986 for reasons related to his health.

Nevertheless, Jack Rudolph continued towork extraordinarily long hours in the care ofthe newborn infant, and was at the bedsideteaching both students and house staff, aswell as his colleagues, on a daily basis untiljust a few months before his death in July1995.

Although Dr. Rudolph was the author orco-author of more than 100 published papersthat appeared in the peer-reviewed medicalliterature, his most lasting contribution toneonatology and to pediatrics is in the legacyof the numerous medical students, house staff,fellows, and other colleagues whom he taughtincessantly about how much one could learnfrom simply observing the newborn infant.This Atlas is a tour de force; it is a spectacularteaching tool that has been developed, collat-ed, and presented by one of the finest clinicalneonatologists in the history of medicine. It isan intensely personal volume that, as Dr.Rudolph himself states, “is not intended torival standard neonatology texts,” but ratherto supplement them. This statement revealsDr. Rudolph’s innate modesty, since with theexception of some discussion on pathogenesisand treatment, it surpasses most neonatologytexts in the wealth of clinical informationthat one can derive from viewing and imbib-ing its contents. We owe Dr. Rudolph andthose who aided him in this work a debt ofgratitude for making available to the medicalcommunity an unparalleled visual referenceon the normal and abnormal newborn infant.

Ralph D. Feigin, M.D.June 13, 1996

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PrefaceI first became attracted to the idea of pro-

ducing a color atlas of neonatology manyyears ago. However, the impetus to synthesizemy experience and compile this current col-lection was inspired by the frequent requestsfrom medical students, pediatric house staff,nurses and others to provide them with acolor atlas of the clinical material provided inmy “slide shows.” For the past few decades Ihave used the medium of color slides andradiographs as a teaching tool. In these week-ly “slide shows” the normal and abnormal, aswords never can, are illustrated.

“I cannot define an elephant but I know onewhen I see one.”1

The collection of material used has beenadded to constantly with the support of thepediatric house staff who inform me to “bringyour camera” whenever they see an unusualclinical finding or syndrome in the nurseries.

A thorough routine neonatal examinationis the inalienable right of every infant. Mostnewborn babies are healthy and only a rela-tively small number may require special care.It is important to have the ability to distin-guish normal variations and minor findingsfrom the subtle early signs of problems. Thetheme that recurs most often is that carefulclinical assessment, in the traditional sense, isthe prerequisite and the essential foundationfor understanding the disorders of the new-born. It requires familiarity with the widerange of normal, as well as dermatologic, car-diac, pulmonary, gastrointestinal, genitouri-nary, neurologic, and musculoskeletal disor-ders, genetics and syndromes. A backgroundin general pediatrics and a working knowl-edge of obstetrics are essential. The generallayout of the atlas is based on the above.Diseases are assigned to each section on thebasis of the most frequent and obvious pre-senting sign. It seems probable that the find-ings depicted will change significantly in thedecades to come. In this way duplication has

been kept to a minimum. Additional spacehas been devoted to those areas of neonatalpathology (e.g., examination of the placenta,multiple births and iatrogenesis) which poseparticular problems or cause clinical concern.

Obviously, because of limitations of space,it is impossible to be comprehensive andinclude every rare disorder or syndrome. Ihave tried to select both typical findings andvariations in normal infants and those foundin uncommon conditions. Some relevantconditions where individual variations needto be demonstrated are shown in more thanone case.

As the present volume is essentially one ofmy personal experience, it is not intended torival standard neonatology texts, but is pre-sented as a supplement to them. It seemslogical that references should be to standardtexts or reviews where discussion on patho-genesis, treatment, and references to originalworks may be found.

Helen Mintz Hittner, M.D., has been kindenough to contribute the outstanding sectionon neonatal ophthalmology.

I have done my best to make the necessaryacknowledgements to the various sources forthe clinical material. If I have inadvertentlyomitted any of those, I apologize. My mostsincere appreciation and thanks to DonnaHamburg, M.D., Kru Ferry, M.D., MichaelGomez, M.D., Virginia Schneider, PA, andJeff Murray, M.D., who have spentinnumerable hours in organizing and cullingthe material from my large collection. Wewish to thank Abraham M. Rudolph, M.D.,for his assistance in reviewing the material.We also wish to thank the following peoplefor their photo contributions to this work:Cirilo Sotelo-Avila, Stan Connor, AvoryFanaroff, Milton Finegold, Brian Kershan,Tom Klima, Susan Landers, Gerardo Cabera-Meza, Ken Moise, Don Singer, EdwardSingleton.

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It is hoped that this atlas will provideneonatologists, pediatricians, family physi-cians, medical students and nurses with abasis for recognizing a broad spectrum of nor-mal variations and clinical problems as wellas provide them with an overall perspectiveof neonatology, a field in which there contin-ues to be a rapid acceleration of knowledge

and technology. One must bear in mind thecaveat that pictures cannot supplant clinicalexperience in mastering the skill of visualrecall.

1. Senile dementia of Alzheimer’s type — normal aging ordisease? (Editorial) Lancet 1989; i:476-477.

Arnold J. Rudolph, M.D.

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CONTENTS

Volume INeonatal and Perinatal Medicine

1. The Placenta, its Membranes, and the Umbilical Cord 1

2. Multiple Births 23

3. Effects of Maternal Medication 47

4. Birth Trauma 57

5. Deformations and Disruptions 81

6. Fetal Growth and Assessment of Gestational Age 117

7. Iatrogenesis 125

Index 154

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Although several texts provide extensive written descriptions of disorders o the newborninfant, the senses of touch, hearing and, especially, sight create the most lasting impressions.Over a period of almost five decades, my brother Jack Rudolph diligently recorded in pictorialform his vast experiences in physical examination of the newborn. The Atlas of the Newbornreflects a selection from the thousands of color slides in his collection, and truly represents"the art of medicine" as applied to neonatology. A number of unusual or rare conditions areincluded in this atlas. I consider this fully justified because, if one has not seen or heard of acondition, one cannot diagnose it.

In this, the first in a five-volume series, three main topics are covered. Although it is com-mon practice to discard the afterbirth, or placenta and its membranes, careful examination ofthis fetal organ often provides insight into conditions affecting the newborn. Thus, it mayreveal evidence of intrauterine infection, which may be transmitted to the neonate; of hem-orrhage, which may cause asphyxia; or of vascular or developmental anomalies, which mayresult in intrauterine growth retardation. Many of these placental abnormalities are illustrat-ed in this volume.

Physical forces acting during fetal development, during delivery, or after birth may beresponsible for a variety of anomalies in the newborn. The influences of uterine constraint,of fetal position, and of amniotic bands are demonstrated magnificently, with resulting anom-alies being related to specific fetal postures. Conditions associated with birth trauma, includ-ing fractures, nerve disruptions and other disturbances, are clearly depicted. Many examplesof complications resulting from treatment of the newborn, or iatrogenic problems such as vas-cular complications of umbilical arterial catheterization, are shown graphically.

A major section demonstrates many of the physical anomalies resulting from fetal exposureto various chemicals, such as occurs through maternal drug abuse or administration of phar-macologic agents to the mother during embryonic or fetal development.

This volume will be enormously valuable to obstetricians and neonatologists, as well as tomidwives and nurses involved in the delivery and care of the newborn.

Abraham M. Rudolph, M.D.

Introduction

Chapter 1The Placenta, Its Membranes, and theUmbilical CordThe human placenta is a highly sophisticated organ of interface between mother and fetus, oftenreferred to as the “gate-keeper to the fetus.” Careful examination of the placenta, its membranes,and the umbilical cord can prove to be a valuable aid in the diagnosis and treatment of theneonate. Gross examination of the placenta takes five minutes, and more sophisticated examina-tion should be considered when there is poor pregnancy outcome, recognizable malformations orabnormalities, multiple gestation, extremes of amniotic fluid volume, severe intrauterine growthretardation, short umbilical cord (< 32 cm), and profound acidemia. The maternal surface of theplacenta (decidual plate) is soft, spongy and dark red; and the fetal surface (chorionic plate) isshiny and steel blue to gray. The placenta, membranes, and umbilical cord weigh approximately400 to 600 g at birth. The ratio of fetal to placental size increases with gestation, being less thanor equal to 1:1 at prior to three months, 4:1 at four to six months, and 6:1 at term. Abnormalitiesin structure can result in an inefficient transport of oxygen and nutrients to the developing baby.Despite this importance, it is one of the least understood and investigated human organs.

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Figure 1.1. A succenturiate (accessory)lobe is common and has no effect on thefetus. This occurs in about 3 to 5% of deliv-eries. Its importance arises from the factthat it may be retained within the uterusand cause postpartum bleeding. (Sotelo-Avila, C.)

Figure 1.2. Another example of a succenturiatelobe. Note that this is very small and the diag-nosis can easily be missed if the placenta is notexamined carefully. (Singer, D.)

Figure 1.3. Fetal surface of a bipartiteor bilobed placenta (placenta du-plex). The two parts of the placentaare of nearly equal size and this occursin about 1% of deliveries. Note thatthe lobes are separated by membranes.The umbilical cord may insert intoone or other lobe, or may insertbetween the two.

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2 Neonatal and Perinatal Medicine

Figure 1.4. A close-up of thesame placenta. The risk to theinfant is that the vessels crossingthe membranes may rupture,resulting in massive blood loss. It issuggested that this condition arisesas a result of superficial implanta-tion of the ovum.

Figure 1.5. Another example ofa placenta duplex showing thematernal surface.

Figure 1.6. In a circumvallate (circummarginate)placenta the fetal surface may be reduced if decidualtissue has made its way between the amnion andchorion. This appears as a yellow, peripheral,hyalinized fold circumscribing the edge of the chori-onic plate. This type of placenta has been reportedto be a cause of antepartum bleeding and prematurelabor.

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The Placenta, Its Membranes, and the Umbilical Cord 3

Figure 1.7. This is an example of placenta mem-branacea (placenta diffusa). These placentas are rare.The ovum implants too deeply, the villae of thechorion fail to regress, and the placental tissuedevelops over the entire surface of the chorion. Theplacenta is very thin and is associated with poor fetalgrowth and antepartum hemorrhage. There may beprevia type bleeding.

Figure 1.8. Transillumination of the same placentashows the thinness of this type of placenta and thatislets of placental tissue are present throughout themembranes. Pregnancy rarely goes to term and fetaldeath is common. If pregnancy continues to term,placenta accreta may occur. In this condition, there isfailure of separation of the placenta during the thirdstage of labor and there may be severe postpartumhemorrhage.

Figure 1.9. An annular (“girdle” or ring-shaped) is arare form of placenta which resembles a segment of ahollow cylinder. Sometimes a complex ring of placen-tal tissue is seen. More commonly a portion of the ringundergoes atrophy resulting in a placenta which isapproximately horseshoe-shaped. This type of pla-centa is probably a variant of placenta membranacea. Its clinical significance is uncertain but it appears to be associated with a high incidence of both ante-and postpartum bleeding. The fetus is often small forgestational age. (Connor, S.)

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Figure 1.10. This otherwise normal placenta showsthe presence of an intrauterine contraceptive device,indicating that it did not prevent pregnancy.

Figure 1.11. In premature separation of the placenta(abruptio placentae) there may be massive bleeding ofmaternal origin. Note the massive blood loss on theleft of the maternal surface of the placenta. In thesecases, there may be severe fetal asphyxia or death. Theinfant in this case had blood in stool (melena neo-natorum) at birth. This was shown to be ingestedmaternal blood by the Apt test.

Figure 1.12. Another example of abruptio placentae.A large abruptio placentae may result in poor growthof the infant and fetal blood loss.

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Figure 1.13. Fetal surface of a placenta with a largechorangioma (hemangioma of the placenta). Theseinfants may present with severe nonimmune hydropsfetalis. The majority of cases of hydrops fetalis are nowdue to nonimmune causes.

Figure 1.14. Maternal surface of the same placenta.Note the placental enlargement due to the choran-gioma and edema. If the placenta is not examined, thiscause of nonimmune hydrops fetalis may be missed.

Figure 1.15. A calcified, small placenta. This infanthad severe intrauterine growth retardation at term as aresult of poor fetal nutrition.

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Figure 1.16. In velamentous insertion of the cord theumbilical vessels traverse the fetal membranes unsup-ported by either the umbilical cord or by placental tis-sue. If tearing of these unsupported vessels occursbefore or during delivery, it can result in massive fetalblood loss.

Figure 1.17. Another example of velamentous inser-tion of the cord. Note the vessels traversing themembranes before inserting into the fetal surface of theplacenta. The vessels, lying in loose unsupportedtissue, may easily stretch and tear, especially if theycross the cervical os and result in vasa previa withmassive blood loss. (Sotelo-Avila, C.)

Figure 1.18. An example of velamentous insertion ofthe cord. Note the large vessels exposed in the mem-branes before they insert into the placental tissue, asection of which is shown at the top. Velamentousinsertion of the cord occurs in 0.5 to 1% of singletonbirths, in 7% of twin births, and in 30 to 40% of tripletbirths. (Sotelo-Avila, C.)

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Figure 1.19. Transillumination of the placenta shownin Figure 1.18. (Sotelo-Avila, C.)

Figure 1.20. Fetus born in a caul. Notethat the membranes completely surroundthe fetus and that the umbilical cord(nuchal cord) encircles the neck twice.A cord around the neck once occurs inabout 20%, and twice in about 2% ofpregnancies. Whether the cord causesany problems depends on its tightnessaround the neck. (Klima, T.)

Figure 1.21. Note the petechiae of the face and head and thesubconjunctival hemorrhages in this infant who had a longcord around the neck. The normal umbilical cord is 40 to 60cm long. Long cords (>70 cm) are more apt to be loopedaround the neck or an extremity of the fetus or to have trueknots. Extremely short cords (<30 cm) may lead to abruptioplacentae, inversion of the uterus, and intrafunicular hemor-rhage (bleeding within the umbilical cord).

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Figure 1.22. This cord was extremely longand wrapped around the left wrist and sev-eral times around the neck resulting inintrauterine death and birth of a stillborninfant. A long umbilical cord has usuallybeen stretched by the movement of anextremely active fetus.

Figure 1.23. Intrauterine death as a result of a longumbilical cord which wrapped around the neck and thenthe left leg. As the infant moved in utero, he strangledhimself.

Figure 1.24. Along umbilicalcord can encirclean extremity andleave recognizablegrooves (furrows)with or withoutskin ulceration.These are notassociated with apoor outcome. Inthis infant, thecord encircled theright knee verytightly and inter-fered with the cir-culation distally.

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Figure 1.25. The severe umbilical cord constrictionproximally resulted in intrauterine death of this fetus.(Finegold, M.)

Figure 1.26. A hematoma of the umbilical cord(intrafunicular hemorrhage) resulted from a shortumbilical cord. Short umbilical cords are associatedwith extreme intrauterine immobility, such as in thefetal akinesia syndrome.

Figure 1.27. A true knot in the umbilical cord of thisfetus resulted in intrauterine death. The incidence oftrue knots in the umbilical cord is 0.1 to 1%, and isstrongly associated with long cords and other markersof vigorous fetal activity. It is associated with about10% of stillbirths. The knots must be very tight toobstruct blood flow. At the site of a long standing knot,such as in this fetus, there is a loss of Wharton’s jellyand a constriction of umbilical vessels. Wharton’s jellyprobably prevents umbilical cord blood vessel com-pression by diffusing the pressure exerted by knots. Thejelly is also slippery and this makes it difficult to main-tain a knot.

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Figure 1.28. An example of two true knots in theumbilical cord in an infant who was normal at birth.

Figure 1.29. Close up of the true knot to the right inFigure 1.28. Note that the Wharton’s jelly is normal and that there is no constriction of the cord. This is,therefore, a recent knot. A previously loose knot may be suddenly tightened as the infant descends duringdelivery.

Figure 1.30. This umbilical cord transection duringamniocentesis occurred several years ago, before ultra-sound was used to determine the position of the amnio-centesis needle. There was considerable blood loss.

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Figure 1.31. Another example of amniocentesisthrough a term placenta. The fetus developed tachy-cardia but was normal at birth. (Moise, K.)

Figure 1.32. Hematoma from transection of theumbilical cord during amniocentesis. There wasmarked blood loss and fetal demise.

Figure 1.33. The diagnosis of single umbilical artery ismade by examining a section through the surface ofthe umbilical cord. This anomaly is present in 0.7 to1.0% of single placentas and in 3 to 7.0% of multiplebirth placentas. The incidence is low in black infants,but is increased in infants with associated congenitalmalformations. Further investigation is recommendedif a single umbilical artery is associated with one othermajor anomaly.

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Figure 1.34. A histologic section of an umbilical cordwith a single umbilical artery. Note that the thick-walled vessel is the artery and the thin-walled vessel isthe vein. It may be associated with abnormal cordlength, velamentous cord insertion, or circumvallateplacenta. The finding of other congenital malformationsis not specific for any one organ system. Cardiac, renal,gastrointestinal, and skeletal malformities have beendescribed. There is an increased incidence of a singleumbilical artery in trisomy 13 and 18.

Figure 1.35. Note the markedly enlarged,edematous umbilical cord due to excessWharton’s jelly in an infant of a diabeticmother. These cords are very friable and teareasily.

Figure 1.36. A throm-bosed vessel in anumbilical cord withlittle Wharton’s jelly.Trauma to the cord is more common whenthere is a lack ofWharton’s jelly. Thelack of Wharton’s jellyis seen more commonlyin postmature infants.A thrombosed vessel inthe umbilical cord maycompromise fetal well-being.

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Figure 1.37. This thin, narrowumbilical cord with total lack ofWharton’s jelly was present atbirth in an infant with postmatu-rity and oligohydramnios. Cordcompression is probably morefrequent with a narrow cord,perhaps because the Wharton’sjelly does not “cushion” the cord.

Figure 1.38. A drying umbili-cal cord 4 days after birth. Notethat as the cord dries, theWharton’s jelly disappearsrapidly.

Figure 1.39. This infant has a large cystin the umbilical cord. The chemicalcomposition of the fluid in this cyst wasthat of serum rather than urine. Thesecysts are thought to arise from the allan-tois.

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Figure 1.40. Another example ofan umbilical cord cyst which isthought to arise from the allantois.These cysts are of no clinical signif-icance.

Figure 1.41. Omphalitis andfunisitis in an umbilical cord.Omphalitis is an acute inflamma-tion of the skin surrounding theumbilicus. Funisitis is an acuteinflammation of the umbilicalcord itself. It results from bacteriaor mycoplasma in the amnioticfluid attracting fetal neutrophilsto migrate out of the umbilicalcord vessels. It can be associatedwith necrosis and calciumdeposits within the cord.

Figure 1.42. Histologic sectionof an umbilical cord showingfunisitis. Note the markedinflammatory reaction surround-ing an artery in the cord itself.(Sotelo-Avila, C.)

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Figure 1.43. Histologic section ofan umbilical cord showing markedinflammatory reaction and bacterialcolonies (intensely stained blueareas) surrounding a vein in thecord itself.

Figure 1.44. Histologic sec-tion of the umbilical cord in aninfant with congenital listerio-sis. Note the gram positiveorganisms on the surface of theumbilical cord.

Figure 1.45. Histologic sectionof funisitis occurring as a resultof a catheter in situ in an umbil-ical vessel.

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Figure 1.46. The external surfaceof the umbilical cord shows cheesywhite areas similar to thrushwhich suggest the diagnosis ofcongenital candidiasis. (Sotelo-Avila, C.)

Figure 1.47. A histologic sectionof the umbilical cord of the sameinfant showing funisitis with thehyphae of Candida which arestained pink. (Sotelo-Avila, C.)

Figure 1.48. External surface of anumbilical cord with yellowish brownareas on the external surface whichsuggest the diagnosis of congenitalcandidiasis. (Fanaroff, A.)

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Figure 1.49. Fetal surface of aplacenta showing chorioamnioni-tis. Normally, the fetal surface of aplacenta is shiny and clear with aprominent vascular pattern. Inchorioamnionitis the fetal surfaceappears dull and opaque with anobscure vascular pattern. Theamniotic fluid is cloudy, and theplacenta, membranes, and amni-otic fluid may have a foul odor.Aspiration in this infected milieuby the fetus may result in neona-tal pneumonia, sepsis and/orneonatal meningitis.

Figure 1.50. Histologic sec-tion of the placenta showingchorioamnionitis. Factorsassociated with increased riskof placental infection includepremature and prolonged rup-ture of the membranes,prolonged labor, placentaprevia, and multiple births.Bacterial infections are morecommon than viral and fungalinfections.

Figure 1.51. Histologic sectionof a placenta with cyto-megalovirus infection. Note thetypical “owl’s eye” inclusions inthe villae. Viral agents reach thefetus by hematogenous dissemi-nation and traverse the placentalvillae. Since viral lesions tend tobe microscopic, gross examina-tion of the placenta is not help-ful. (Finegold, M.)

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Figure 1.52. Histologic section ofa placenta with congenital syphilis.Note the numerous spirochetes.(Finegold, M.)

Figure 1.53. The presence of meconium staining of theamniotic fluid occurs with fetal distress and postmaturi-ty. Meconium staining of the placenta may occur with-in 1 hour. In cases of chorioamnionitis, the fetal surfacemay be green; in listeriosis, there may be brown orchocolate staining of the amniotic fluid with similarstaining of the placenta. (Finegold, M.)

Figure 1.54. Infant with alarge “garment” nevus (bathingtrunk nevus). Note the markedbreakdown and ulceration ofthe skin.

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Figure 1.55. Gross section of placenta show-ing multiple nevi in the same infant shownin Figure 1.54. This emphasizes the impor-tance of examining the placenta in infantsborn with such abnormalities. (Sotelo-Avila, C.)

Figure 1.56. Histologic section ofmelanoma cells from the placentaof the same infant shown in Figure1.54 and 1.55. Similarly lesionscan be seen in the placenta ofinfants with congenital neuroblas-toma. (Sotelo-Avila, C.)

Figure 1.57. Squamous metaplasiaof the amnion. In this placenta withsquamous metaplasia, the amnion isstripped from the membranes. Notethe concentric appearance of thesenodules and their frequent umbilica-tion. The amnion is squamous andthese areas of metaplasia form withmaturity. Note the tiny nodules ofkeratin irregularly present on thefetal surface.

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Figure 1.58. Histologic section ofthe amnion showing the squamousmetaplasia. Squamous metaplasiadiffers from amnion nodosum inthat the lesions cannot be separatedreadily, whereas the nodules inamnion nodosum can be picked offof the underlying amnion leaving asemi-transparent, saucer-shaped de-pression with somewhat raggededges.

Figure 1.59. Typical amnionnodosum in one twin. This twin wasan acardiac monster having no uri-nary tract. The amnion is rough andshows numerous fine granules whichare whitish, opaque, and uniform insize. Frequently the nodules are moreirregular in size and slightly yellowishbrown. It is unusual to see this degreeof amnion nodosum. The other twinwas normal.

Figure 1.60. Another example ofamnion nodosum in a twin placenta.Note the normal umbilical cord andplacenta on the right and the smallumbilical cord with a thrombosed ves-sel on the left. This was a twin-twintransfusion syndrome and the twin onthe left became a fetus papyraceus.The amnion nodosum lesions arewhitish, opaque, and uniform in size.

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Figure 1.61. Histologic section of a pla-centa and membranes showing amnionnodosum on the left and chorioamnioni-tis on the right in an infant with renaldysgenesis and hypoplastic lungs.

Figure 1.62. High-power histo-logic section of amnion nodosumwhich consists of lanugo, vernixcaseosa, and squamous epithelialcells. With marked oligohydram-nios, the fetal skin rubs against thefetal surface of the placenta andproduces these lesions.

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Chapter 2Multiple BirthsMultiple gestation occurs frequently in pregnancy. The most common is twinning, whichoccurs in about one in every 80 pregnancies. There are two types of twins: monozygous or“identical” and dizygous or “fraternal.” The monozygous twin rate is 1 in every 200 pregnan-cies. It results from a single ovulation with subsequent splitting of the developing egg withinthe first 14 days. There is no familial tendency. Dizygous twinning results from doubleovulation and fertilization and is probably determined by higher gonadotropin secretion rates.The rate of dizygous twinning is variable and is influenced by heredity (transmitted autoso-mally but expressed only in the mother), race (as high as 1 in 23 births in some West Africanraces; as low as 1 in 300 births in Mongolian races), maternal age (increased frequency withincreasing age), and drugs (incidence of twinning is 6.6% with the use of Clomid®).Examination of the placenta of multiple births is important because of the two- to three-foldhigher incidence of structural defects in monozygous twins and increased potential for suc-cessful future transplant between monozygous twins. Monochorionic placentas are invariablyfused and about 75% of the infants are identical twins. Dichorionic placentas may be fused orseparate. Infants with a dichorionic fused placenta may be identical (monozygous) or fraternal(dizygous). The membrane which separates the two fetal cavities is the key to the evaluationof twin placenta. Typically, this runs across the middle of the fused placenta. Inmonochorionic twins, with one chorion covering two amnia, the dividing membrane consistsof two translucent amnionic layers which can be pulled with ease from the placental surface.In 1% of monozygotic twinning, the placenta will be monochorionic-monoamnionic. Inconjoined twinning, the placenta is also monochorionic-monoamnionic. In dichorionictwins, with two amnia, the dividing membrane consists of four layers, two chorionic and twoamnionic; it is opaque, thicker, and does not separate from the placental surface without tearing.

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Figure 2.1. Theseinfants are normal,identical triplets bornat 32 weeks gestation.They initially hadmild respiratory dis-tress, but improvedrapidly. (Cabera-Meza, G.)

Figure 2.2. The fetal surfaces of the placentas offraternal triplets.

Figure 2.3. A triplet placenta showing a vela-mentous insertion of the cord in one of thetriplets. Note that the placentas are fused andthus the pregnancy could be monozygotic.

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Figure 2.4. The fetal sur-faces of two completelyseparated twin placentas.These are always dizygotic.

Figure 2.5. This low-powerhistologic section demonstratesthat an amnion can be identi-fied on each side of two choria.Thus, this is an example of adichorionic-diamnionic pla-centa. This type of dividingmembranes is typically seen inall dizygotic twinning and inabout 20 to 25% of monozy-gotic twinning.

Figure 2.6. The fetal surface of thisfused, twin placenta shows the divid-ing membranes. In this placenta, his-tologic examination showed thatthere were two amnia and a singlechorion (diamnionic-monochorion-ic). These twins would thus bemonozygotic.

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Multiple Births 25

Figure 2.7. Histology of the mem-branes of the same placenta shows thedouble amnion on the outer surfaceswith a single chorion (diamnionic-monochorionic).

Figure 2.8. On the fetal surface ofthis fused, twin placenta, the dividingmembrane is very thin and clear, sug-gesting that this is a monozygoticpregnancy. This was confirmed by his-tologic examination showing a singlechorion. (Finegold, M.)

Figure 2.9. The histologicappearance of the dividing mem-branes in twin placentas are com-pared in this figure. On the left,note the diamnionic-monochori-onic twin placenta, and on theright, note the diamnionic-dichorionic twin placenta.

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Figure 2.10. Note the two umbilicalcords inserting into the fetal surfaceof the placenta without the presenceof dividing membranes. This is typicalof a monoamnionic-monochorionictwin placenta, which occurs in 1% oftwin pregnancies.

Figure 2.11. The fetal surface of amonoamnionic-monochorionic twinplacenta, showing the insertion oftwo separate umbilical cords. (Sotelo-Avila, C.)

Figure 2.12. Close-up of theabove placenta shows anastomosisof the vessels between the twoumbilical cords. This results in atwin (feto-fetal) transfusion syn-drome. (Sotelo-Avila, C.)

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Multiple Births 27

Figure 2.13. The fetal surface of a monoamnionic-monochorionic twin placenta showing the entan-glement of the two umbilical cords resulting in fetalanoxia and fetal distress with meconium passage.Note the meconium-stained appearance of the fetalsurface of the placenta. Entanglement of the cordsis a complication which may occur because of a lackof a dividing membrane in monoamnionic-mono-chorionic twins. (Karishan, B.)

Figure 2.14. Another exam-ple of entangled cords in amonoamnionic-monochorion-ic twin placenta.

Figure 2.15. The fetal surface ofthis monoamnionic-monochorion-ic placenta in conjoined twinsshows the insertion of the twoumbilical cords, but note thatthese fuse and present as a singlefused cord in thoracopagus twins.(Sotelo-Avila, C.)

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Figure 2.16. The appearance of the umbilicalcords in a twin pregnancy. Note the small sizeof the umbilical cord in the infant who wasgrowth-retarded as compared to the normalsize of the umbilical cord in the normal twin.

Figure 2.17. These infants born as stillbirthsat 30 weeks gestation are an example of twin(feto-fetal) transfusion syndrome. In this syn-drome, vascular anastomoses permit the trans-fer of arterial blood under high pressure fromthe twin on the right (donor) to the lowpressure venous system of the other twin(recipient). The donor twin is thus kept hypo-volemic, dehydrated, malnourished, or even inshock. His organs are small and his amnioticfluid is decreased. The recipient twin becomeshypervolemic, edematous, and plethoric (poly-cythemic). His organs are large, he may havecongestive failure and his amniotic fluid isincreased.

Figure 2.18. These monozygotic twins at birth repre-sent another example of twin transfusion syndrome.The birth weight of the twin on the left was 3000 gwith a central hematocrit of 86%. The birth weight ofthe twin on the right was 2230 g with a central hema-tocrit of 27%. Twin transfusion syndrome occurs onlyin monochorionic twins. It occurs in 15 to 30% ofmonochorionic pregnancies and is defined in terms ofa difference of greater than 250 g birth weight and/or20% difference in the central hematocrit between thetwins. Twins in this syndrome usually do not look iden-tical at birth although in fact they are monozygotic.

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Multiple Births 29

Figure 2.19. In these infants with twintransfusion syndrome, the difference inbirth weight was only 180 g and the dif-ference in central hematocrit was 32%.Thus, these infants represent a mildexample of twin transfusion syndrome.

Figure 2.20. The maternal surfaceof a monozygotic twin placenta ininfants who had the twin transfusionsyndrome. Note on the left the con-gested appearance of the portion ofthe placenta supplying the recipienttwin who had a central hematocritof 69%, and note on the right themuch paler appearance of theplacenta supplying the donor twinwho had a central hematocrit of45%. (Singer, D.)

Figure 2.21. The fetal surface ofthe same placenta as in Figure.2.20 showing the congestedappearance on the left of therecipient portion of the twin pla-centa and the pallor on the rightof the donor portion of the twinplacenta. These vascular connec-tions may be artery-to-artery,vein-to-vein, or artery-to-vein.Any form may significantly affectthe fetuses physiologically andclinically.

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Figure 2.22. The fetal surface ofplacentas in twin transfusion syn-drome showing the anastomosesbetween the two fetal circula-tions following the injection ofinfant formula, which is used as acontrast medium. Note that onthe fetal surface of the placentaarteries always cross over veins.The anastomosis between anartery on the left and a vein onthe right is shown at the junctionof the left third and the righttwo-thirds of the figure.

Figure 2.23. This close-up viewclearly shows the artery-to-veinanastomosis.

Figure 2.24. Another exampleof an injection of infant formulafilling the arteries which crossover the veins in this vessel anas-tomosis.

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Multiple Births 31

Figure 2.25. In extreme cases the transfusion syn-drome may cause the death of one twin resulting in afetus papyraceus. In this figure, there is an extrachorialplacenta with a fetus papyraceus attached.

Figure 2.26. Close-up of the extrachorial placentawith the fetus papyraceus attached.

Figure 2.27. Another exampleof the twin transfusion syndrome.Note the maternal surface of theplacenta showing the congestedrecipient placenta on the left andthe pale donor placenta on theright with an attached fetuspapyraceus.

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Figure 2.28. The fetus papyraceus may calcify and result in alithopedion (“stone child”) as shown in this figure.

Figure 2.29. A stillborn maleinfant with a birthweight of 1170 gand length of 34 cm is an exampleof acardius acephalus. This is one ofthe acardiac anomalies whichoccurs as a consequence of abnor-mal umbilical artery-to-artery anas-tomoses between two fetuses in thepresence of a fused placenta. It issometimes referred to as the TRAP(twin-reversed-arterial-perfusion)sequence. (Klima, T.)

Figure 2.30. A close-up of the abdomen of the sameinfant shows an omphalocele; there was also hydrops,pulmonary agenesis, and polyhydramnios. Acardiacanomalies include three groups: acephalus in 60 to 75%of cases, amorphus in 20% of cases, and a well-formed head and body in 10% of cases. (Klima, T.)

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Multiple Births 33

Figure 2.31. The fused placenta ofthe same infant with acardiusacephalus shown in Figure 2.29 and2.30. Note the large cord of the“normal” infant which had threevessels and the small cord of theinfant with acardius acephaluswhich had two vessels. There wereno separating membranes betweenthe two cords. Note the large anas-tomoses between the placentas.(Klima, T.)

Figure 2.32. An acardiusacephalus infant deliveredat 30 weeks gestation. Theupper body and shouldersform a fleshy mass cappedby a tuft of short hairs. The lower body has twowell-formed legs but hasclubbed, bifid feet with twotoes. (Klima, T.)

Figure 2.33. Radiographof the infant with acar-dius acephalus. Note thelack of cranial develop-ment. Two clavicles andscapulae are present. Thevertebral column is nor-mal. The chest is narrowdue to lack of the heartand hypoplastic lungs.The pelvis and lowerextremities are well min-eralized and relativelynormal except for lack ofsome metatarsal bones.

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Figure 2.34. The twin withthe dominant heart of thesame pregnancy often showsadditional anomalies such aslimb reduction defects. Thesehave been attributed to em-bolic disease consequent uponstasis in the acardiac fetus.This twin had some limbanomalies which includedfusion of digits of the left handas seen in this figure.

Figure 2.35. This isthe same infant show-ing anomalies of bothfeet.

Figure 2.36. The placenta ofthe infants described aboveshowing two umbilical cordswith a common insertionabout 5 cm from the marginof the placenta. The largercord was approximately 10mm in diameter and hadthree vessels. The smallercord was approximately 6 mmin diameter and had two ves-sels. Note the congestedappearance of the placentaltissue and the vessels on theleft and the pallor of the pla-cental tissue and the vesselson the right. (Klima, T.)

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Figure 2.37. This is an example ofacardius anceps (hemiacardius). Thecranial portion is partially covered byhair, but no head is formed. There is aglobular structure of the upper partwhich shows a rudimentary face withan oral opening through which a cleftpalate is seen. Four extremities are pre-sent. There is a defect in the anteriorabdominal wall with intestinal loopspresent. (Klima, T.)

Figure 2.38. Close-up of the same infantshown in Figure 2.37. The anterior wallof the abdomen is missing and theintestinal loops are exposed. There is noumbilical cord identified. (Klima, T.)

Figure 2.39. In thoracopagus conjoined twins, note the fusion at the thorax andupper part of the abdomen with a single site of umbilical cord insertion. Theposture is typical for thoracopagus conjoined twins in that the heads are hyper-extended and the backs are relatively straight.

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CONJOINED TWINSConjoined twins are rare, one per 50,000 to 100,000 live births. They result from the failure of the zygote to com-pletely divide. This occurs in approximately 1% of monozygotic twins. The incidence of the types of fusion is asfollows: The most common types are thoracopagus twins with an incidence of one per 70,000 live births (73.4%),pygopagus twins (18.8%), ischiopagus twins (5.9%), and the most rare types are the craniopagus twins withan incidence of one per 2,000,000 to 4,000,000 live births (1.7%). Conjoined twinning occurs only in mono-amnionic-monochorionic pregnancies.

Figure 2.40. Radiograph of the conjoined twins shown inFigure 2.39 illustrates the hyperextended heads and thefusion of the thoraces and upper abdomen. On fetal radi-ograph, the hyperextended fetal heads at the same levelwere considered almost diagnostic of thoracopagus con-joined twins. With the advent of ultrasonography, the diag-nosis should be made more readily.

Figure 2.41. Another set of thoracopagusconjoined twins showing the typical pos-ture of the hyperextended heads.

Figure 2.42. Thoracopagus conjoined twins showing the fusion from theupper thorax to the midabdomen.

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Multiple Births 37

Figure 2.43. The same twins as shown in Figure 2.41showing the fused upper abdomen and the fusedumbilical cord.

Figure 2.44. This fused umbilical cord section fromthe same set of thoracopagus conjoined twins showsthe presence of four vessels, two arteries and two veins.In the fused umbilical cords seen in conjoined twin-ning, there may be from two to seven vessels. (Singer, D.)

Figure 2.45. This fused umbilical cord section show-ing three arteries and three veins is another examplefrom conjoined twins. (Singer, D.)

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Figure 2.47. The same set ofpygopagus conjoined twinsshowing the fused genitalia.

Figure 2.48. Close-up of the fused genitalia in thesame set of pygopagus conjoined twins.

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Multiple Births 39

Figure 2.46. Pygopagus conjoined twins are fused at the buttocks.

Figure 2.49. The twins shownin Figure 2.46 were success-fully separated. This figureshows them prior to dischargefrom the hospital.

Figure 2.50. Radiograph of a set ofomphalopagus twins shows the fusion at theabdominal walls and hence they could be separated.

Figure 2.51. An exam-ple of craniopagus con-joined twins.

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Figure 2.52. Note on the left, the vaginal delivery ofprosopothoracopagus conjoined twins. On the right, notethe twins following delivery. Prosopothoracopagusconjoined twins are twins united in the frontal plane withthe fusion extending from the oral region through thethorax and upper abdomen. Diagnosis was not made pre-natally and the twins died at delivery. Note that there areonly two upper extremities (dibrachus) and four lowerextremities (tetrapus). (Caberra-Meza, G.)

Figure 2.53. Cephalothoracopagusconjoined twins. Note the syncephalus.

Figure 2.54. Dicephalus con-joined twins with dicephalus,three upper extremities (tribra-chus), and two lower extremi-ties (dipus). Note the two sepa-rate heads and the fused chest.

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Multiple Births 41

Figure 2.55. A close-up of the same twins shown inFigure 2.54 demonstrates the third upper extremityextending from the fused shoulders. There were twoseparate neurologic systems, two separate pulmonarysystems, two gastrointestinal systems joining at thejejunum, a single genitourinary system, and conjoinedhearts with complex anomalies.

Figure 2.56. Another example of di-cephalus conjoined twins. Note that thehead on the left of the figure is normal butthat there is anencephaly of the head onthe right of the figure.

Figure 2.57. Radiograph ofthe same conjoined twinsshowing the normal head onthe left of the figure and theanencephalic head on theright of the figure. Note thatthere are fused vertebralcolumns with some separa-tion at the lower thorax andupper abdomen and thatthere is dibrachus.

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Figure 2.58. Asymmetric ischiopagus conjoinedtwins. Note that the twin on the right of the figure,which is attached at the ischia, is an anencephalicparasite with a partial thorax and abdomen and twoupper and two lower extremities. The “normal” twinon the left of the figure had gastroschisis, imperforateanus, and rectovaginal fistula. There were twobladders, two kidneys, and two uteri present.

Figure 2.59. Close-up of the anencephalic parasitein the above asymmetric ischiopagus conjoinedtwins.

Figure 2.60. Radiograph of the asymmetric ischiopagusconjoined twins. Note at the bottom the severelyanecephalic twin attached to the “normal” twin who hasa large gastroschisis. Each twin has four extremities.

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Multiple Births 43

Figure 2.61. Preoperative (above) and postoperative(below) appearance of the asymmetric ischiopagusconjoined twins shown in Figures 2.58 to 2.60.

Figure 2.62. Dipygus twins. Recent work hassuggested that genes are involved in establish-ing the body axes, metameric pattern (seg-mentation genes), and regional specialization(homeotic genes). This raises the possibilitythat many of the abnormalities of facial dupli-cation in man are not a manifestation ofincomplete twinning but may be homeoticmalformations. Limb duplication may also bea result of stimulation of homeobox geneexpression and thus a well-formed pair of armsand/or legs may be seen emerging.

Figure 2.63. Thisinfant has a well-developed lower ex-tremity emerging fromthe chest and hasbeen considered to bean asymmetrical dou-ble malformation –heteroadelphia (anunderdeveloped para-site attached to a well-developed autosite).With the new con-cept, this could be an example of a ho-meotic malformation.Also note the largeomphalocele.

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Figure 2.64. Close-up view of thesame accessory lower extremity asshown in Figure 2.63.

Figure 2.65. Duplication of the right legand foot. The question again arises as towhether this is an asymmetric double mon-ster or a homeotic malformation. (Cabera-Meza, G.)

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Multiple Births 45

Figure 2.67. Another exam-ple of discordant twins. Thetwin on the left is an albinoand the twin on the right isnormal. This is the second setof discordant twins (one albi-no and one normal) born tothis mother.

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Figure 2.66. Discordanttwins. The twin on the left isan example of a severe caudalregression syndrome. Therewas oligohydramnios, renalagenesis, imperforate anus,and lack of external genitalia.The twin on the right is nor-mal. (Cabera-Meza, G.)

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Chapter 3Effects of Maternal MedicationDuring pregnancy, the average fetus is exposed to four physician-prescribed and five self-prescribed drugs. Every drug administered or taken by a pregnant woman presents themother with both risks and benefits. The risks include the drug’s potential as a teratogen oras a cause of toxicity in the fetus. Most human teratogens affect the embryo during a very nar-row period of early development as illustrated by the time (24 to 33 days gestation) during which the fetus is susceptible to limb reduction defects caused by thalidomide. Severalhuman teratogens, such as alcohol, androgens, cocaine, diphenylhydantoin, radiation,tetracycline, valproic acid, and warfarin have serious side effects beyond the period oforganogenesis. These effects may include cell deletion, vascular disruption, necrosis,physiologic decompensation, organ pathology, and intrauterine growth retardation. Drugstaken in the third trimester may not have teratogenic effects, but may be toxic to the fetus.Some examples include indomethacin (causing oligohydramnios), propylthiouracil (causingfetal goiter), and erythromycin (causing cholestatic hepatitis). A detailed history of mater-nal drug use and abuse is essential in evaluating most malformations and diseases in theneonatal period.

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Figure 3.1. This illustration contrasts thecraniofacial features of a healthy child on theright to those of a child with fetal alcoholsyndrome on the left. Note the microcephaly,short palpebral fissure, flat maxillary area,poorly developed philtrum and thin upper lip(Peter Shvartsman, Canadian MedicalAssociation Journal, July 15, 1981 cover).

Figure 3.2. This infant, age 6 weeks, was bornto a mother with severe, chronic alcoholism.There was failure to thrive and hypotonia. Notethe microcephaly (head circumference less thanthe third percentile), short nose, absence ofphiltrum and thin vermilion border of theupper lip. Findings in fetal alcohol syndrome includeintrauterine growth retardation, microcephaly,dysplastic facial features, hypoplasia of the midface, and a hypoplastic philtrum with a thinvermilion border of the upper lip. Later theremay be continued failure to thrive and develop-mental and behavioral disorders.

Figure 3.3. Close-up of the face of the sameinfant shows the short nose, absence of thephiltrum, and thin vermilion border of theupper lip. Many other findings in fetal alcoholsyndrome have been reported, including epican-thic folds, ptosis, hypoplastic maxilla, deep oraccentuated palmar creases, and clinodactyly.

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Figure 3.4. Soon after birth, this infant of anarcotic addict shows hypotonia. Note theconcavity of the inner aspect of the thighs andthe position of the lower extremities. This hasresulted from a postural deformation in whichthe fetus has had its thighs flexed over itsabdomen in utero. Because of the mother’snarcotic habit there was minimal fetal move-ment in utero.

Figure 3.5. Drug withdrawal is a major problem inneonates delivered of narcotic addicted mothers. Thisfigure stresses the fact that one should always check forsigns of drug addiction in the mother. This figure showsneedle tracks at both elbows of a mother.

Figure 3.6. Infants withretinoic acid embryopathy(Accutane™ embryopathy)may have craniofacial, cardio-vascular, and central nervoussystem abnormalities.In this infant note the nar-row sloping forehead, flatdepressed nasal bridge, mildmicrognathia, and microtiawith absence of the externalauditory canal. In additionthere was congenital heartdisease. Affected infants mayhave hydrocephalus, micro-cephaly, or thymic abnor-malities. This mother wastreated with retinoic acidduring the first month ofpregnancy.

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Effects of Maternal Medication 49

Figure 3.7. Close-up of the ears of thesame infant as shown in Figure 3.6 showsthe bilateral microtia with absence ofthe external auditory meatus.

Figure 3.8. In infants with thefetal hydantoin (Dilantin™) syn-drome there is moderate growth retardation, usually prenatal, awide anterior fontanelle andmetopic ridging. In this infant,note the growth retardation, pro-fuse scalp hair, and short neck.Other findings included hypopla-sia of the distal phalangeswith small nails and a digitalthumb.

Figure 3.9. Close-up of the face of the same infant. Note the marked hir-sutism, low hairline, low nasal bridge with a short upturned nose (“pug” nose),and long philtrum.

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Figure 3.10. Hypertrichosis inanother infant with the fetalhydantoin syndrome. Motherwas treated throughout pregnan-cy with hydantoin. The risk offetal hydantoin syndrome ininfants of treated mothers isabout 10%.

Figure 3.11. Gum hypertrophyin an infant with the fetal hydan-toin syndrome. Many other find-ings have been reported in infantswith fetal hydantoin syndrome,including widely spaced nipples,rib anomalies, abnormal palmarcreases, pilonidal sinus, and con-genital heart disease.

Figure 3.12. This infant of an epileptic mother on hydantoindeveloped seizures at the age of 36 hours. He had hypocalcemia with acalcium level of 6.4 mg/dL and a phosphorus level of 11.2 mg/dL. Infetal hydantoin syndrome the digital hypoplasia may be associated withnarrow distal phalanges and hypoplastic nails.

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Figure 3.13. This infant with thefetal hydantoin syndrome presentedwith many of the findings alreadydescribed. There was growth retarda-tion, hypertelorism, small pug nose,anteverted nostrils, long philtrum,and thin vermilion border of theupper lip, and short neck.

Figure 3.14. The same infant shows the characteris-tic changes in the fingers. Note the hypoplasia of thedistal phalanges with hypoplastic or absent nails andthe digital thumbs. There is mild webbing.

Figure 3.15. The same infantwith fetal hydantoin syndromeshows the marked hypoplasia ofthe distal phalanges of the toesand absent or hypoplastic nails.

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Figure 3.16. Postnatal growthdeficiency and microcephaly arepresent in two-thirds of childrenexposed to valproic acid incombination with other anticon-vulsants. It does not occur withmonotherapy with valproic acid.This infant with the fetal vaproate syndrome shows the typicalcraniofacial abnormalities. Note the trigonocephaly with a promi-nent metopic ridge, bifrontalnarrowing, outer orbital ridgedeficiency, midface hypoplasia,epicanthic folds, small shortupturned nose, and long flatphiltrum.

Figure 3.17. A cranial view of the sameinfant shows the trigonocephaly due topremature closure of the metopic suture,bifrontal narrowing, and outer orbital ridgedeficiency.

Figure 3.18. Lateral view of thehead and face of the same infantshows the marked metopic ridge,small flat short nose, micrognathiaand “square” ears.

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Figure 3.19. The sameinfant with fetal valpro-ate syndrome as shown in Figures 3.16 to 3.18,had distal phalangealhypoplasia and taperingof the fingers. Note the abnormal creases on the fingers and palmdue to lack of fetal movement in utero.Other changes reportedin infants with this syn-drome include tracheo-malacia, congenital heartdefects, and urogenitalanomalies.

Figure 3.20. Yellow staining ofthe teeth in a child exposed tomaternal tetracycline in utero.

Figure 3.21. A Wood’s filter showsthe fluorescence of the nails in aninfant exposed to maternal tetracy-cline. If young infants are giventetracycline after birth the stainingof the teeth and nails also occurs.

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Figure 3.22. Drug-induced pseudoher-maphroditism in a female infant who wasvirilized by progestational agents during thefirst trimester of pregnancy. The incidenceof this condition has decreased because,with recognition of this iatrogenic cause ofvirilization of the fetus, there has been adecreased use of incriminating drugs such asprogestational agents or androgens duringthe first trimester. There may be fusion oflabioscrotal folds with formation of a uro-genital sinus and clitoromegaly. (SeeVolume V, chapter 5).

Figure 3.23. The thalidomide syn-drome in twin infants born to a motherwho took thalidomide early in gestation.Maternal ingestion of thalidomidebetween the 25th to 44th day after con-ception may cause malformations. Inthe thalidomide syndrome the limbs areusually asymmetrically involved and themalformations of the extremities are ofall grades of severity (digits are usuallypresent). There may be microph-thalmia, ear deformities, and cardiac,renal and intestinal malformations.

Figure 3.24. Phocomelia inanother infant born to anoth-er mother who took thalido-mide in early gestation. Notethe asymmetric phocomelia.

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Figure 3.25. This infant with the fetal warfarin syndrome (Coumadin™embryopathy) was born to a mother who was being treated with warfarin dur-ing the first trimester of pregnancy. These infants typically are low birth-weight and have facial and skeletal abnormalities. Less commonly they mayhave central nervous system and eye abnormalities. In this baby note the typ-ical facial features of a broad flat face and nasal hypoplasia with a low nasalbridge, a prominent philtrum, and micrognathia.

Figure 3.26. The lateral view of theface strikingly demonstrates themarked nasal hypoplasia resulting ina very flat face. Because of themarked nasal hypoplasia theseinfants often present with upper air-way obstruction.

Figure 3.27. Radiograph of the lower extremities of thesame infant shows the stippling of the epiphyses at the prox-imal femora. Stippling of the epiphyses may occur along thevertebral column and the tarsal bones. The stippling disap-pears in the first few years of life. Coumadin™ embryopathy isphenotypically similar to hereditary chondrodystrophiapunctata and it must thus be distinguished from the differenthereditary forms of Conradi-Hünermann syndrome.

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Chapter 4Birth TraumaBirth trauma refers to those injuries sustained during labor and delivery. Despite skilled and com-petent obstetric care, some may be unavoidable. Factors predisposing infants to injury includemacrosomia, prematurity, cephalopelvic disproportion, dystocia, prolonged labor, and abnormalpresentation. In 1988, birth injuries ranked eight as major causes of neonatal mortality and caused4.6 deaths per 100,000 live births. The clinician who cares for newborn infants must be familiarwith the conditions caused by birth injury. Injuries are known to occur to the soft tissues, head,eyes, ears, vocal cords, neck and shoulder, spine and spinal cord, intra-abdominal organs, extrem-ities, and genitalia. Although many are mild and self limited, others are serious and potentiallylethal.

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Figure 4.1. Severe molding of the head following anoccipitoposterior presentation. The mobile skull bonesand brain deform to comply with pressure in the birthcanal. Note the flattened forehead and long occiput. Itresolves spontaneously and needs no intervention.

Figure 4.2. Persistent occipitoposterior presentationwith marked molding and a caput succedaneum. Acaput succedaneum occurs as a result of the presentingpart pressing against the partly dilated cervix whoseconstricting rim obstructs the return flow of venousflood and lymph from the scalp leading to edema. Thedistribution crosses suture lines (compare with cephal-hematoma) and is usually present at birth.

Figure 4.3. Caput succedaneum with prolonged labor.In a caput succedaneum the tissues involved are thoseencircled by the “girdle of contact” formed by thematernal passages. The location indicates theintrauterine lie of the fetus. In breech delivery theremay be similar edema and bruising of the perineum,buttocks, or genitalia. These are really an equivalent ofcaput succedaneum.

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Figure 4.4. “Blisters” of the skin following prolongedlabor over a caput succedaneum in an infant at the ageof 1 day following prolonged labor. Aspiration of thematerial was sterile.

Figure 4.5. A large caput (chignon) following vacuumextractor delivery. The word “chignon” refers to thelocalized area of scalp edema caused by the suction ofthe cup of the vacuum extractor. Most cases resolvespontaneously but if associated with perinatal asphyxiathere may be necrosis of the chignon leading to ulcer-ation of the scalp.

Figure 4.6. “Caput ring.” In rare cases with persistentstrong contractions and a slowly dilating cervix, necro-sis of the scalp may occur in the area of a caput due topressure ischemia occurring during a prolonged labor.

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Figure 4.7. Cephalhematoma over the right parietalbone. A cephalhematoma is a subperiosteal hemor-rhage occurring as a result of vessel rupture at birth. Itis generally not apparent at birth but is noted in thefirst day or two of life. It should be distinguished froma caput succedaneum. It is most commonly seen overthe parietal bones and more commonly over the rightparietal than the left. A caput succedaneum andcephalhematoma may occur concurrently.

Figure 4.8. Large left parietal cephalhematoma. Notethat the cephalhematoma is limited by suture linesbecause it is a subperiosteal hemorrhage. Cephal-hematoma occurs more commonly after prolongedprimigravida labor or forceps delivery especially in postterm infants where suture fusion makes the skull hardand unyielding.

Figure 4.9. Bilateral cephalhematomas in this infantdemonstrate very clearly the limitation of the cephal-hematoma by suture lines. Complications of cephal-hematomas include anemia, jaundice, infection, andunderlying skull fracture. In general, cephalhematomasresolve spontaneously over a period of weeks tomonths.

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Figure 4.10. Cephalhematoma with linear fracture of the skull.This occurs in 4 to 5% of infants with a cephalhematoma.

Figure 4.11. Calcification in a cephalhematoma giving thelesion an “egg shell” feel occurs as a result of deposition ofcalcium in the organizing blood. Periosteal new bone formsaround the perimeter of the cephalhematoma and this rimof calcification may be noted for several months.

Figure 4.12. Infected cephalhematoma inan infant with Escherichia coli sepsis.Osteomyelitis of the underlying parietalbone was present.

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Figure 4.13. The same infant asin Figure 4.12 with pus beingdrained from the infectedcephalhematoma (pyocephal-hematoma). The pus grew pureE. coli in culture. In general it is recommended that cephal-hematomas not be aspirated, buton rare occasions, if the infantdevelops sepsis due to anyorganism, infection may occurin the cephalhematoma requir-ing drainage.

Figure 4.14. Radiograph of the skull of the sameinfant showing osteomyelitis of the parietal bone.

Figure 4.15. Massive scalp hemorrhage of thenewborn (subgaleal hemorrhage). When thereis rupture of the capillaries in the subaponeu-rotic area, there may be massive scalp hemor-rhage with spread over the entire scalp andmassive blood loss. Disseminated intravascularcoagulopathy may ensue rapidly. This condi-tion may occur as a result of a precipitousdelivery or poor application of a vacuumextractor. Note the massive soft tissue swelling.

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Figure 4.16. Frontal view of thesame infant who had a massive scalphemorrhage with a skull fracture.Note the ecchymoses of the uppereyelids and marked swelling fromthe bridge of the nose extendingover the scalp. This is characteristicof a subgaleal hemorrhage as theaponeurosis of Galen attaches at theupper eyelids and has no attach-ment to the scalp until the nape ofthe neck and the sternocleidomas-toid muscle on the sides, hence themassive hemorrhage in the sub-galeal area of the head.

Figure 4.17. A massive scalp hemorrhagefollowing vacuum extractor delivery. Notethe limitation of bleeding at the nape of theneck and the sternocleidomastoid muscle.The bleeding into the subaponeurotic spacemay result in massive blood loss leading tohypovolemia and shock.

Figure 4.18. Hematoma of the rightcheek and mouth which occurred during adifficult spontaneous delivery.

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Figure 4.19. Trauma from a forceps delivery. Forcepsmarks on the cheek are fairly common. Rarely, a tran-sient facial palsy may be associated with this type oftrauma and occasionally the forceps may actually trau-matize the skin, leading to ulceration.

Figure 4.20. Forceps mark following delivery.The pressure of the forceps blade may result indamage to the underlying tissue and, as in thisinfant, subcutaneous fat necrosis may occur.

Figure 4.21. Fetus born in acaul with a nuchal cord. Cordaround the neck once is presentin about 20% of deliveries and,in about 2% of deliveries, thereis a cord around the neck twice.This common finding generallydoes not cause problems unlessthe cord constricts the necktightly. (Klima, T.)

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Figure 4.22. Facial suffusion due to a nuchalcord.

Figure 4.23. Cord around the neck interfering with cir-culation. Note the petechiae of the face and head and thesubconjunctival hemorrhages. Petechiae are also seen ininfants in whom there is abnormal delay following deliv-ery of the head and neck before the trunk and shouldersare delivered. In subconjunctival hemorrhage a linear orlunar hemorrhage is often seen to the side of the iris.Petechiae and subconjunctival hemorrhages do not havethe same ominous significance as those on the trunk orlimbs and usually fade in the first few days of life.

Figure 4.24. Marked suffusion and bruising of theface as a result of a face presentation. This could beconsidered the equivalent of a caput with the face asthe presenting part. There is deep blue discolorationand swelling of the face and there may be consider-able disfiguration of the face which is of short dura-tion.

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Figure 4.25. Face presentation in a prematureinfant of 34 weeks gestation. Note the very markedecchymotic appearance of the face.

Figure 4.26. Face/brow presentation. Note themarked edema and ecchymoses over the faceand brow, particularly the left eye.

Figure 4.27. A brow presentationwith hyperextension of the head.This is the classic “militaristic atti-tude” with head back and chin out.Opisthotonos is excluded by lack of arching of the back. As this is the baby’s “position-of-comfort” inutero, during the first few days post-natally the infant will be unhappy ifan attempt is made to flex the head.After several days the infant willadopt a normal posture.

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Figure 4.28. In this infant who was a breech presentation note the edema, bruising, and ecchymosis. In breech presentations the perineum,buttocks, and thighs may be severely bruised.

Figure 4.29. This infant is another example of abreech presentation. Note the extended legs andthe equivalent of a caput over the right buttock,which was the presenting part.

Figure 4.30. Bruising ofmale genitalia due to abreech presentation. Notethe marked swelling of thescrotum and penis. In rarecases testicular trauma mayoccur.

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Figure 4.31. Breech presentation in a female infantwith marked bruising and swelling of the genitalia.Note the swollen labia majora and bruised labia minora.

Figure 4.32. Petechiae following a breechdelivery are frequently seen in otherwise nor-mal infants. They are of no consequence andimprove spontaneously in a few days.

Figure 4.33. Typical position-of-comfort of an infant who was a frankbreech presentation. Note the mildgenu recurvatum. This infant kepther legs in extension with the kneesflexed for several days. “Position-of-comfort” deformations are commonin breech presentations and improvein a few days. These infants shouldall be checked for congenital disloca-tion of the hip.

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Figure 4.34. The characteristic moldingof the head of an infant in a breech pre-sentation. The frontal view shows theoccipitofrontal head elongation along witha prominent occipital shelf and the neckappears long.

Figure 4.35. Lateral view of the head of the same infantshows the flattening of the vertex and the prominentoccipital shelf. The plane of flattening is directedupward and forward from the occipital protuberance,which is quite prominent. The characteristic headresults from prolonged pressure of the flexed headagainst the fundus of the uterus in breech presentation.

Figure 4.36. “Hangingneck” contusion in a difficultdelivery of a breech pre-sentation. The infant wasextremely depressed andrequired ventilatory support.

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Figure 4.37. Double footlingbreech presentation. The right legpresented through the cervical osfor 6 hours prior to delivery. Notethe marked ecchymoses and edema.This resolved spontaneously butthe infant developed hyperbiliru-binemia.

Figure 4.38. This mother had a history of adifficult previous abortion. With this preg-nancy she had a spontaneous rupture of theuterus. On laparotomy a large perforationwas noted in the uterus through which theinfant’s lower extremities presented. Notethe compression edema and ecchymoses ofthe left leg and the gangrene of the rightfoot.

Figure 4.39. A close-up view of thegangrene of the right foot in the sameinfant.

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Figure 4.40. Subcapsular hematoma of the liver isgenerally a pathologic finding. It occurs most com-monly with breech delivery especially in prematureinfants. Occasionally, if bleeding persists, the capsuleof the liver ruptures and there is massive hemorrhageinto the peritoneal cavity. This usually occurs on thethird or fourth day of life. The same may occur in thecourse of mismanaged artificial cardiopulmonary resus-citation. Rupture of the spleen is very rare and is morecommon with a transverse lie.

Figure 4.41. Transverse lie with a shoulderpresentation. Note the marked swelling andecchymoses of the right shoulder and upperextremity.

Figure 4.42. Compound presen-tation of the right arm, hand andvertex. Note the depression in theskull.

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Figure 4.43. The same infant with the arm and handplaced in its in utero position. Note the marked edemaof the right forearm and hand compared to that of theleft arm and hand. This occurred as the result of thecompound presentation.

Figure 4.44. In this infant the membranes ruptured 10days prior to delivery which was by cesarean birth forchorioamnionitis. There was difficulty in deliveringthe right arm which presented at the elbow. The handis normal; therefore this was not due to an amnioticband.

Figure 4.45. Intrauterine skull fracture (congenitalmolding of the skull) which occurs in cases of infantswith poor mineralization of the skull where the mother has a prominent sacral promontory and has aprolonged labor or delivers precipitously. It results in a greenstick or depressed fracture of the skull.

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Figure 4.46. Radiograph of the skull of theinfant shown in Figure 4.45 showing adepressed fracture in a poorly mineralizedskull after a spontaneous vertex delivery. Inthese infants the fracture may improve spon-taneously but there is a question as towhether treatment should be passive or sur-gical lifting of the fracture is indicated.

Figure 4.47. Another example of an intrauterine skullfracture (also called congenital molding of the skull).

Figure 4.48. Radiograph of adepressed fracture of the skull follow-ing forceps trauma. A linear skull frac-ture may be seen underlying a cephal-hematoma or may occur from postna-tal trauma such as in an infant fallingfrom the bed to the floor.

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Figure 4.49. This infant had depression on both sidesof its skull from its intrauterine position. Note that thisis not traumatic and requires no treatment.

Figure 4.50. Cervical cord injury. This veryrare complication is invariably associated withbreech delivery. Note the crying infant lyingflat on the bed in the “pithed-frog” positionwith abdominal distention due both to lack ofmuscle tone and to an enlarged bladder.

Figure 4.51. Radiograph of the neckshowing the cervical cord and spinalinjury following breech delivery. Notethe fracture dislocation and separa-tion involving C5 and C6.

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Figure 4.52. Congenital torticollis is usuallynot apparent at birth but within the firstweek a swelling is noted over the sternoclei-domastoid muscle (stenomastoid tumor).This is thought to occur as a result of spasm,hemorrhage or fibrosis. It results in shorten-ing of the sternocleidomastoid muscle andtilting of the head. It is important to recog-nize since it may cause astigmatism.

Figure 4.53. Fracture of the right claviclein an infant at the age of 3 days. There wassoft tissue swelling but not much callus for-mation. The baby may be asymptomaticand the first clinical sign may be a swellingover the clavicle from callus formation orthere may be pseudoparesis of the upperlimb on the affected side.

Figure 4.54. This infant did nothave the fracture of the left claviclediagnosed until the age of 10 daysbut the nurses had noted that theinfant was irritable and restless espe-cially when handled. Examinationrevealed the excessive callus due to afracture of the left clavicle. In anyinfant with a fracture of the clavicleone should also check for injury tothe brachial plexus, phrenic nerve,recurrent laryngeal nerve, and thesympathetic chain.

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Figure 4.55. A radiograph of the chest showing a frac-ture of the right clavicle. The clavicle is the site of themost common fracture during delivery especially ifshoulder dystocia is present. Healing is rapid because ofthe speed with which callus formation occurs and it israre to have any permanent deformity.

Figure 4.56. A radiograph of a fracture of the middlethird of the right humerus following a difficult delivery.This commonly arises with shoulder dystocia and maybe associated with lesions of the brachial plexus due totraction on the shoulder girdle. The upper arm isimmobile, painful and may be swollen.

Figure 4.57. A radiograph of a fracture of the rightfemur occurring as a result of birth injury followingbreech extraction.

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Figure 4.58. A radiograph of a fracture of the leftfemur with marked soft tissue swelling. Note the largeright inguinal hernia.

Figure 4.59. Facial nerve palsy occurred inthis infant as a result of application of forceps.It may also occur following prolonged labor ina mother with a prominent sacral promontory.Note the ptosis and drooping mouth on theright side.

Figure 4.60. This shows the same infant crying. Thefacial palsy becomes readily apparent. This demonstrateshow easily the diagnosis may be missed in a quiet orsleeping infant. There is diminished movement of theaffected side of the face, the eye frequently but not alwaysremains partly open, the nasolabial fold is absent and themouth droops, being drawn over to the healthy sidewhen the infant cries. Note that the forehead is smoothon the affected side. Restoration of normal function anddisappearance of the paresis may be complete in a fewdays or usually within a few weeks. Permanent paralysisis exceptional and suggests a central lesion.

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Figure 4.61. Facial palsy of the left side in an infant. This infantshows the typical findings in that he is unable to close his eye orcontract the lower facial muscles and has loss of the nasolabial foldon the affected side. In traumatic facial paresis frequently only themandibular branch of the facial nerve is affected. With central facialparesis the two lower branches are affected allowing for movementof the forehead. Facial paresis in Möbius syndrome is usually bilat-eral and incomplete and the face is expressionless.

Figure 4.62. Congenital hypoplasia of thedepressor anguli oris muscle (angular depressormuscle). The localized facial weakness inwhich the lower lip on one side fails to bedepressed on crying results in an “asymmetriccrying facies.” The resulting facial asymmetrywhen the child cries may be misinterpreted.There is a weakness of the muscles controllingmovement of the mouth but not those of theupper face. The nasolabial folds are normal andthe affected side will not move when the infantcries. The normal side of the face is assumed tobe abnormal because the lower lip on theintact side appears to be pulled down andeverted. This is a benign condition and is notfacial palsy. It lessens as the child gets older. Itmay be associated with other anomalies, par-ticularly those of the cardiovascular system.

Figure 4.63. Erb’s palsy (upperbrachial plexus injury) occurs as aresult of traction on the brachialplexus (most often the upper nerveroots, C3, C4, and C5). This typeof injury occurs most commonly incases of shoulder dystocia. It pre-sents with the infant lying with theaffected upper extremity adductedand internally rotated, the elbowextended, and the hand partiallyclosed with the palm directed out-wards and posteriorly resulting inthe typical “waiter’s tip” position.The majority of these injuriesresolve spontaneously in 3 to 4weeks.

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Figure 4.64. Bilateral involvement of the upper brachial plexus resulting inthe typical position in both upper extremities. In the rare event of a bilater-al palsy the possibility of damage to the spinal cord has to be considered.Note that infants with Erb’s palsy may lack a Moro response on the affectedside.

Figure 4.65. Anteroposterior and lateral radi-ograph of the chest in an infant with a right Erb’spalsy. Note the ipsilateral paralysis of the rightdiaphragm due to phrenic nerve palsy which mayoccur in association with upper motor brachialplexus trauma. A rare complication associatedwith Erb’s palsy is a Horner’s syndrome on thesame side, due to involvement of the cervical sym-pathetic nerves. If Horner’s syndrome persists, theinfant may develop heterochromia iridis caused byfailure of development of secondary pigmentationin the affected eye.

Figure 4.66. Left radial nerve palsy in a neonate presentsas a typical wrist-drop. The condition probably resultsfrom interference with blood supply to the nerve if thereis abnormal compression or traction during a difficultlabor. With the wrist-drop there is ability to grip with thefingers and voluntary extension of the fingers. A similarappearance may occur in an infant with a postural defor-mity, or a pseudo “wrist-drop” may be seen in a floppy,hypotonic infant hence it is important to check for vol-untary wrist extension.

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Chapter 5Deformations and Disruptions A structural abnormality or anomaly may be a malformation, deformation, or disruption.Deformations and disruptions are the result of mechanical forces affecting normal tissue,while malformations are the result of a primary problem in morphogenesis. A deformation isa physical change in form, shape, or position caused by mechanical forces secondary torestricted intrauterine motion (e.g., clubfoot secondary to constraint of the foot). Most ofthese have an excellent prognosis once the fetus is released from the constraining environ-ment. In some instances, application of an opposite force may be needed to correct the defor-mation. Some deformations are caused by a problem intrinsic to the fetus and are notreversible (clubfoot secondary to neuromuscular disorder). A clear distinction between defor-mation and malformation is important when educating parents on prognosis, managementand recurrence risk. A disruption is a congenital defect resulting from an extrinsic inter-ference with an originally normal developmental process (e.g., abnormality caused by anamniotic band). Disruptions usually result in cell death and are not correctable.

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Figure 5.1. The infant in the following five figures wasreferred to hospital with a diagnosis of multiple con-genital malformations. It should be noted that these“malformations” represent examples of congenital pos-tural deformities. Note the position of the hands, lowerextremities and the feet occurring as a result of thisinfant’s position in utero.

Figure 5.2. A close-up of the postural deformi-ties involving the feet.

Figure 5.3. This figure demonstratesthe congenital postural scoliosis andpseudo “wrist-drop.”

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DEFORMATIONS (Congenital Postural Deformities, “Position-of-Comfort” Anomalies) and DISRUPTIONS (Amniotic band disruption complex–The Early Amnion Rupture Spectrum (TEARS))

Autoamputation of digits, furrows due to amniotic bands, and syndactyly may develop between the affected digits.Amniotic bands may cause major disruptions that lead to constriction or amputation or to craniofacial disruption. Lack of symmetry of the lesions differentiate disruption caused by amniotic bands from genetic causesof craniofacial or limb anomalies.

Figure 5.4. This figure of the same infant shows a skindimple over the left hip. Skin dimples are not uncom-mon in association with deformations (postural defor-mities or “position-of-comfort” deformities).

Figure 5.5. The infant has beenplaced into her in utero position.This demonstrates clearly how theabove changes occurred as a resultof the infant’s “position-of-com-fort” in utero. If placed in a normalposition, infants with deforma-tions will be uncomfortable andwill cry, but will quiet down rapid-ly when allowed to return to their“position-of-comfort.”

Figure 5.6. Abdominal preg-nancy is associated with multi-ple congenital postural deformi-ties as there is no cushion ofamniotic fluid.

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Figure 5.7. Asymmetry of the face and head in aninfant at birth due to a deformation.

Figure 5.8. The same infant demonstrating thatthis occurred as a result of the right upperextremity lying in apposition to the face andhead on the right side in utero.

Figure 5.9. This normal infantpresented at birth with a depres-sion over its left temporal area.This occurred as a result of an inutero positional deformity.

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Figure 5.10. In the same infant as shown inFigure 5.9 the left temporal depression was dueto pressure of the baby’s left foot on the fetalskull in utero.

Figure 5.11. This very common finding of folding ofthe ear lobe occurs as a result of a postural deformity.

Figure 5.12. In thisinfant the same typeof postural deformityis noted demonstrat-ing that this occurs asa result of the shoul-der pressing up againstthe ear lobe in utero.In general, over 90%of congenital posturaldeformities correctspontaneously.

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Figure 5.13. In rare cases marked pressure of theshoulder on the fetal head in utero can result in adepression over the temporal area. Note the ear ispushed forward.

Figure 5.14. The same infant in its “position-of-comfort” shows that the shoulder caused thedepression and abnormal appearance of the ear.

Figure 5.15. This infant with asymmetry of the jaw atbirth was noted to have some abrasions on the neck.Skin abrasions can occur in relation to a posturaldeformity.

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Figure 5.16. The same infant showing its“position-of-comfort” in utero.

Figure 5.17. There is marked asymmetry of the facein this infant. When the face and head are straight-ened, the infant is very uncomfortable and cries.

Figure 5.18. The same infant quiets downimmediately when allowed to go into its“position-of-comfort” in utero. This alsodemonstrates the reason for the asymmetryof the face.

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Figure 5.19. Positional deformity showing asymmetryof the face and jaw.

Figure 5.20. Asymmetry of the face occur-ring as a result of a “position-of-comfort”deformity.

Figure 5.21. The same infant shows that the asymmetryis due to its left foot being placed up against the side ofthe face and jaw. Another example of “position-of-com-fort” deformity.

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Figure 5.22. Malocclusion of the jaw may occur ifthere is a marked and prolonged positional deformity.Infants with malocclusion should be followed as themalocclusion may require treatment at a later date.

Figure 5.23. There is asymmetry of the nostrils in thisinfant. Note the vertical left nostril and horizontalright nostril. This can occur as a result of a posturaldeformity or dislocation of the nasal cartilage.

Figure 5.24. The same infant shows that the asymme-try was associated with a postural deformity due topressure of the right hand on the nose in utero. Thisinvariably corrects spontaneously.

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Figure 5.25. In this infant note the asymmetry of thenostrils and ecchymosis due to a dislocation of the tri-angular cartilage of the nasal septum, which may occurduring delivery, especially if the mother has a promi-nent sacral promontory. When the septum is manuallymoved toward the midline the asymmetry persistsconfirming the dislocation. These infants require anotolaryngology evaluation.

Figure 5.26. Note the subcostal depression on eitherside of the xiphoid in an infant who was a breechpresentation.

Figure 5.27. This figure shows the same infant with both kneesfitting well into the depressions. This is a fairly common exam-ple of a “position-of-comfort” deformity.

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Figure 5.28. Another example of a positional deformityon both sides of the subcostal area resulting from posi-tion in utero in a breech presentation. This should notbe confused with subcostal retraction in this prematureinfant who had respiratory distress.

Figure 5.29. In this infant the chestappears to be narrow compared with the restof the body.

Figure 5.30. The same infant shows the arms lyingalong side the chest wall compressing the thorax.This is a fairly common postural deformity andshould not be confused with a narrow thoraxobserved in cases of dwarfism.

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Figure 5.31. In this infant it appearedthat there was a left wrist-drop and the diagnosis of radial palsy was con-sidered. However, with stimulationthe left hand moved normally and the postural deformity resolved com-pletely in a few days.

Figure 5.32. In breech presentation theremay be a marked concavity of the inneraspect of the thigh. The lower extremities ina frank breech may lie up against the fetalabdomen causing a “position-of-comfort”deformity.

Figure 5.33. The same infant showing its “position-of-comfort.” Asimilar concavity of the inner aspect of the thighs may occur in infantswho have lack of movement in utero.

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Figure 5.34. Radiograph of an otherwise normalinfant with the uncommon finding of bilateral bow-ing of the femora occurring as a result of a “position-of-comfort” deformity. Bowing of one or both tibiaein which they curve gently toward the midline mayhave the soles of the feet face each other. Femoralbowing is rare but is occasionally present in babiesborn after prolonged breech presentation. Con-genital bowing of the forearm and humerus almostnever occur. These tend to improve gradually as withall deformations.

Figure 5.35. Posterior view of an infant showing congenital pos-tural scoliosis which occurred as a result of position in utero.

Figure 5.36. The same infant demon-strating the postural scoliosis. Posturalscoliosis in the newborn is rare. If a truecongenital scoliosis is present it is usuallyassociated with a structural anomaly ofthe vertebral column.

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Figure 5.37. This infant has a fairly common con-genital postural deformity – genu recurvatum. This“position-of-comfort” deformity gives the impressionthat there is a dislocation at the knees. Note the hyper-extensibility at the knees and note that the creases onthe thighs which are normally seen posteriorly areanteriorly placed.

Figure 5.38. The same infant with genu recurvatum in its“position-of-comfort.” When it occurs, genu recurvatum (“backknee”) is almost invariably associated with breech presentationsand the incidence is much more common in females.The majoritycorrect spontaneously; in severe cases posterior splinting may benecessary.

Figure 5.39. Another example of genurecurvatum in an infant with a neural tubedefect. Note the bilateral clubfeet that arealso considered to be postural deformities.Genu recurvatum may occur in neurologicdisorders and in syndromes with general-ized joint laxity and hypermobility, such asEhlers-Danlos syndrome.

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Figure 5.40. This infant has acongenital dislocation of the rightknee. This uncommon findinghas the same appearance as genurecurvatum. Congenital disloca-tions of the knee rarely occur asan isolated condition but may beseen in Larsen’s syndrome, a condition in which there aremultiple joint dislocations. It maybe confirmed by radiography andrequires treatment.

Figure 5.41. Another view of thecongenital dislocation of the rightknee in the same infant.

Figure 5.42. Metatarsus adductus (metatarsusvarus) is a common postural deformity whichrequires no treatment. The forefoot is turnedmedially so that the lateral border of the sole is quiteconvex. The heel is in a neutral position and thefoot can be dorsiflexed normally. If intrauterineconstraint has been prolonged a deep plantar creasewill be seen on the medial side.

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Figure 5.43. The position of thefeet in utero in the same infant asshown in Figure 5.42 demon-strates how the midfoot becomesadducted.

Figure 5.44. Talipes calcaneoval-gus is the most common of the con-genital postural deformities. Thefoot is dorsiflexed on the fibular sideof the ankle and everted with thesole facing anterolaterally. Asimplied by the name, the calcaneusalso is deviated laterally. A deepskin crease is often present in theplane of abnormal flexion and thesubcutaneous tissue is diminishedover the anterolateral region of thefoot.

Figure 5.45. In this infant withtalipes calcaneovalgus, note themarked dorsiflexion of the footwhich is lying against the anteriorpart of the leg. Talipes calcaneo-valgus may occur as a result ofabnormal intrauterine posture ormay be associated with lowermotor neuron defects such as spinabifida. Spontaneous correctionusually occurs. This anomaly ismore common in babies born inthe breech position, especially ifthe knees were flexed in utero.

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Figure 5.46. The same infant showing the flatteningof the dorsum of the foot and marked concavity ofthe lateral side of the ankle joint.

Figure 5.47. An infant with severe talipescalcaneovalgus with marked bowing of the tibiaand fibula. Osseous changes such as these areuncommon. This infant required surgicalcorrection.

Figure 5.48. Radiograph of the same infant showing themarked bowing of the distal ends of the tibia and fibula.

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Figure 5.49. Bilateral club-foot (talipes equinovarus).Talipes equinovarus causesthe foot to be sharply plantarflexed and inverted so thatthe sole is toward the medianplane. Note the “tip toe”position with the soles of thefeet nearly facing each other.The calcaneus is in varusposition and some degree ofmetatarsus adductus isalmost always present. Theskin and subcutaneous tissueover the lateral part of thejoint may be thin and dorsi-flexion is minimal or absent.

Figure 5.50. Bilateral clubfoot. The occur-rence of club feet has been considered to bethe result of a congenital malformation or apostural deformity. If the legs and feet are sub-jected to mechanical stress during the lastweeks in utero, especially if the fetus is in thebreech position, a clubfoot may develop. If theconstraint has been relatively mild or brief, thedeformity is usually flexible in that the footcan be manipulated into normal position. Afixed deformity implies either severe, pro-longed immobilization with contractures ofthe ligaments and capsules of the joints or anintrinsic skeletal anomaly. This type is resis-tant to conservative treatment, and casting orsurgery is the treatment of choice.

Figure 5.51. The same infant with the feetin their “position-of-comfort.” Note the dim-ples at the ankles suggesting that thisoccurred as a result of a postural deformity.Some authors have suggested that the pres-ence of dimples at the ankles in an infantwith clubbing of the feet indicates that it hasoccurred as a result of a postural deformity.

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Figure 5.52. Con-genital curly toes(overlapping toes) area common finding in newborn infants.There is often a fami-ly history of the samefinding in parents orsiblings. Treatment isnot necessary.

Figure 5.53. Another infant with congenitalcurly toes. The abnormality becomes less obvi-ous as the infant grows.

Figure 5.54. Twins with asymmetricalheads occurring as a result of an in uteropositional deformity. Both infants werevertex presentations. Note how theheads “fit” together.

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Figure 5.55. The same infants asshown in Figure 5.54 with theirheads together (in utero position).As the infants grew the asymmetryimproved.

Figure 5.56. Thisinfant with arthrogry-posis demonstrates thesevere congenital jointcontractures. Theseoccur as a result of lackof intrauterine move-ment and may be asso-ciated with muscle orneurologic pathology.Such severe contrac-tures may be accompa-nied (or produced) byjoint webbing (e.g.,multiple pterygiumsyndrome or poplitealpterygium syndrome).

Figure 5.57. Frontal view of anotherinfant with arthrogryposis. Extremeintrauterine compression over anextended period of time can producesuch severe distortion in multiple jointsthat supporting ligaments become con-tracted and opposing tendons stretch.The muscles acting across these jointsmay atrophy, creating a picture indis-tinguishable from the intrinsic forms ofarthrogryposis multiplex congenita.

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Figure 5.58. Posterior view of the arthro-gryposis in the same infant as in Figure 5.57.

Figure 5.59. This infant is an example of arthro-gryposis with joint contractures involving thelower extremities only. The upper extremitieswere normal. This may occur in infants withneural tube defects, caudal regression syndromeand muscle disease.

Figure 5.60. An infant with the cau-dal regression syndrome resulting inarthrogryposis. Note the markedunderdevelopment of the hips andthe webbing at the joints. There isalso talipes calcaneovalgus.

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Figure 5.61. Lateral view of thesame infant shows the markedunderdevelopment of the hips, thearthrogryposis, webbing, and thedimples at the joints due to lack ofmovement. Note the talipescalcaneovalgus.

Figure 5.62. An infantwith an amyoplasia con-genita disruptive sequence.In this condition theelbows are usually inextension with the wristsand hands flexed.

Figure 5.63. The same infant showing a close-up of the right wristand hand. Note the dimples at the contracture site. In this condi-tion there may be cord wrapping of the limb and amniotic bands.

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Figure 5.64. This figure showsthe left hand of the same infant.Note the lack of development ofthe finger and hand creases. Lackof development of the creasesindicates lack of fetal movementof the fingers occurring before the10th to 12th week of gestation.

Figure 5.65. There is an intrauter-ine constriction band at the rightwrist in this infant. Shallow con-stricting rings can encircle a limb atany level and destroy subcutaneoustissue sometimes producing distaledema. Amniotic bands may resultin constriction or amputation (par-tial or complete) of extremities andare thought to occur as a result of anactive fetus pushing an extremitythrough and tearing the amnion.This represents an example of TheEarly Amnion Rupture Spectrum(TEARS).

Figure 5.66. The congenital anomaly of the fourth finger of the lefthand in this infant is due to an amniotic band. Also note the collodi-on appearance of the skin of this postmature infant. The hands andfeet (and especially the digits) are the most common sites of damagefrom amniotic bands. If there is mild constriction, distal edema is pro-duced; with more severe compression there can be disruption of tissuedown to the periosteum with eventual loss of the distal avascular por-tion – a congenital amputation. The findings in such infants are bizzareand very variable.

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Figure 5.67. An infant with amputation of thesecond, third and fourth fingers occurring as aresult of intrauterine constriction bands. At birth,the amputation site will be scarred and sometimesbands of fibrous tissue still will be attached to theinjured area. The damage is usually asymmetricwith digits affected at different levels and, oc-casionally, several digits will be bound together at the tips by scar tissue producing a form ofpseudosyndactyly.

Figure 5.68. The amniotic band syndrome inthis infant resulted in amputated fingers,intrauterine constriction bands and fusion of theamputated digits.

Figure 5.69. Another ex-ample of the amniotic bandsyndrome in which theamputated digits, raw areasand strand of amnion on theright hand can be seen.

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Figure 5.70. This figure of the same infantas shown in Figure 5.69 shows the involve-ment of the left hand as a result of theamniotic band syndrome.

Figure 5.71. Amniotic bands involving the fingers of aninfant. Note the amputated fourth finger and the con-striction involving the base of the third finger resulting ingangrene.

Figure 5.72. An intrauter-ine constriction band at theright wrist resulted inmarked obstruction of circu-lation to the hand. Notethat at the site of constric-tion a band has beenremoved, but because of thedistal involvement of thecirculation, the conditionworsened and the infanteventually required amputa-tion.

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Figure 5.73. In this infant there is adisruption of the right hand due to con-striction bands resulting in amputationsof the second, third, and fourth fingers.Note the congenital absence of thethumb in the left hand, which was nota disruption but occurred as a result of atrue congenital malformation.

Figure 5.74. There is a minimalamniotic band at the distal partof the right foot of this infant. Itshould be noted that this type offinding could easily be missed onexamination.

Figure 5.75. In this same infant shown in Figure 5.74there were severe amniotic bands of the fingers andtoes. Note the strands of amnion.

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Figure 5.76. Intrauterine constriction bands(amniotic bands) may disrupt tissue growth andresult in partial or complete amputation. In thisinfant there was amputation of the toes but asevere constriction band interfered with circula-tion of the left leg. Note the marked distalswelling of the left leg.

Figure 5.77. Radiograph of the same infantshown in Figure 5.76, showing marked bowingand fracture of the tibia and fibula of the left leg asa result of the constriction band. Also note thesevere tissue swelling of the foot.

Figure 5.78. An in-trauterine amputationof the right leg and a constriction band of the left leg werenoted in this infant atbirth. If there is acongenital amputation,consideration shouldbe given to whetherthe amputation is aprimary or secondarylimb reduction defect.This is an example ofa secondary limbreduction defect.

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Figure 5.79. A radiograph of the same infant as shown in Figure5.78 showing the amputation of the right leg. Note the soft tissueconstriction of the distal left leg.

Figure 5.80. Another example of con-genital amputation of the left leg and thetoes of the right foot by amniotic bands.In primary limb reduction defects, theskin at the amputation is smooth andthere is underlying subcutaneous tissue.In secondary limb reduction deformities,the skin shows ulceration or scarring andhas no underlying subcutaneous tissue.Radiography shows the stump of the boneis smooth in primary limb reductiondefects and the stump of the bone isjagged in secondary defects.

Figure 5.81. A radiograph of the same infant shown in Figure 5.80 showing the con-genital amputation of the left leg at the site of the amniotic band.

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Figure 5.82. Congenital amputa-tion of the right hand. This is aprimary limb reduction defect.

Figure 5.83. This infant presented with multiple anomaliesincluding a bilateral cleft lip and palate, partial amputation of theright arm, and a congenital scalp defect.

Figure 5.84. In the same infant shown in Figure 5.83, note the amniotic bandconstriction down to the bone almost resulting in amputation of the rightarm.

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Figure 5.85. The same infant as in Figures 5.83 and5.84 showing the congenital scalp defect in the occip-ital area. Note the strand of amnion attached to thedefect. Figure 5.86. The early amnion rupture spec-

trum (TEARS) in this infant shows thebizarre findings involving the face and head.There is a skin defect of the scalp with anencephalocele and gross malformation of theface. The ADAM complex is an example ofTEARS. The complex includes AmnioticDeformities, Adhesions, and Mutilations.Findings in the ADAM complex includecleft lip, bizarre midfacial clefts, central ner-vous system abnormalities (hydrocephalus,microcephaly, asymmetric encephalocele),gastrointestinal abnormalities (omphalocele,gastroschisis), and ocular abnormalities(coloboma, anophthalmia, corneal opacity).

Figure 5.87. This infant is another example of the earlyamnion rupture spectrum. Note the asymmetric encephalo-cele in addition to the bizarre anomalies of the face and eye.In general, encephaloceles are midline and an asymmetricencephalocele is always suggestive of a disruption.

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Figure 5.88. This infant with TEARS has bizarrefacial clefting, a central nervous system defect, anabdominal wall defect, and limb defects.

Figure 5.89. A close-up of this infant showingthe bizarre facial clefting and a strand of amnionextending from the right eye to the scalp defect.Also note the severe defect of the mouth andpalate. In general, bizarre facial clefting (obliquefacial clefting) is noted in disruptions whereas lat-eral or transverse facial clefts occur in infants withsyndromes (e.g., Goldenhar’s syndrome).

Figure 5.90. The same infantshowing the abdominal walldefect (omphalocele) and strandsof amnion extending from this to the central nervous systemmalformation.

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Figure 5.91. The early amnion rupture spectrum caused a limb/body walldeficiency with spine and central nervous system defects in this infant.Note the hydrocephalus.

Figure 5.92. The limb/body walldeficiency in this infant caused asevere thoracoabdominal wall de-fect. Note the severe scoliosis andkyphosis and the short umbilicalcord with the placenta attached tothe abdominal viscera.

Figure 5.93. A close-up showingthe right lung and the evisceratedabdominal organs with theplacenta attached.

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Figure 5.94. Another viewshowing the short umbilicalcord.

Figure 5.95. In this infant withdisruption, there is severe scolio-sis and the placenta was attachedto the right leg. The striking dif-ference between this infant andthe other examples of disruptionis that this infant also had con-genital malformations.

Figure 5.96. In the same infant,note that the disruption involvedthe lower extremities and the righthand but the infant also had animperforate anus, lumbar spinedefect and dextrocardia, plus apatent ductus arteriosus. Con-genital anomalies include malfor-mations, disruptions, and defor-mations. This figure should alertone to the fact that any combina-tion of these can occur.

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Figure 5.97. A radiograph of the sameinfant as in Figures 5.95 and 5.96 showsthe dextrocardia, abnormalities of theribs, and abnormal segmentation of thethoracolumbar vertebrae. In this infantthe kyphosis and scoliosis was caused byboth the malformation of the spine andthe disruption.

Figure 5.98. The typical reduc-tion sequence of limb reductionanomalies and scalp and skindefects are noted in this infantwith Adams-Oliver syndrome.

Figure 5.99. Close-up of theskin defect of the abdominal wallin the same infant.

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Figure 5.100. Close-up of the scalpdefect in the same infant as in Figures5.98 to 5.99.

Figure 5.101. The early amniotic rup-ture spectrum in an infant with a cervicalmeningomyelocele and an omphalocele.Note the amniotic band extending acrossthe right shoulder. There was an amnioticband 1 cm proximal to the left ankle.

Figure 5.102. A close-up of the cervical me-ningomyelocele in the same infant. Note the am-niotic band extending across the right shoulder.

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Figure 5.103. A close-up of theamniotic tissue band over the rightshoulder in the same infant asshown in Figures 5.101 and 5.102.

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Chapter 6Fetal Growth and Assessment ofGestational AgeThe normal term infant has completed a gestation of > 37 weeks and has a birth weight > 2500 g.Accurate dating of pregnancy is important in evaluating the abnormally grown infant at birth.There are two different populations of low birthweight infants, those who are 1) born prematurein gestation (i.e, at < 37 weeks); or 2) small for gestational age (SGA). Infants are SGA for oneof two reasons: 1) a normal intrauterine environment but abnormal development due to fetalfactors (i.e., chromosomal abnormalities); or 2) an abnormal uterine environment (i.e., maternaltoxemia, heart disease, etc.) leading to abnormal growth.

Many criteria have been used to estimate gestational age. Obstetrical factors employed to deter-mine age include date of last menstrual period, auscultation of fetal heart tones (audible byDoppler at 10 weeks, and stethoscope at 20 weeks), quickening (first maternal perception of fetalmovement usually occurring at 18 to 20 weeks), and fundal height (used to estimate fetal size dur-ing the first trimester ). Ultrasonography with first trimester measurements of crown-rump lengthaccurately estimates gestation ± 3 days. Up to 30 weeks gestation, measurements of the fetalbiparietal diameter, femur length, and abdominal circumference are a reliable index of fetal size(body weight) and gestational age, but afterwards may be quite variable. Physical examinationmay also be used in assessing gestational age. Gestational age only indirectly measures maturity,and other measurements are needed to determine organ-specific maturity (e.g., amniotic fluid lac-tase/sucrase ratio > 2 for fetal lung maturity).

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Figure 6.1A & B. Manyscoring systems have beendevised to assess by examthe gestational age ofinfants at birth. The orig-inals are shown here infigures A & B, publishedby Amiel-Tison. TheDubowitz examination isone of the most completesystems, combining thegeneral physical examina-tion performed in the firsthours of life with the neu-rologic evaluation carriedout when the infant is atleast 24 hours old. TheBallard Method abbrevi-ates the Dubowitz scoringsystem and uses only 12physical and neurologiccriteria. Statistically,however, the Ballard is asaccurate in assessing ges-tation age (± 2 weeks) asmore thorough examina-tions and is most useful inthe busy clinical setting.

1.Posture

1.Heel to ear maneuver

3.Popliteal angle

4.Dorsiflexion angle of foot

5.‘Scarf’ sign

6.Return to flexion of forearm

6 months 28 weeks

61–2 months 30 weeks

7 months 32 weeks


8 months 36 weeks


9 months 40 weeks

HypertonicFlexion of the four limbs

Stronger flexion

Beginning of flexion of thigh at hip

Completely hypotonic

Frog-like attitude

Very hypertonic

’Scarf‘ sign complete with no resistance

Upper limbs very hypotonic lying in extension

Flexion of forearms begins to appear, but very weak

Strong ‘return to flexion.’ Flexion tone inhibited if forearm maintained 30 seconds in extension

Strong ‘return to flexion.’ Forearm returns very promptly to flexion after being extended for 30 seconds

’Scarf‘ sign more limited Elbow slightly passes midline Elbow almost reaches midline

100°110°150° 100° 90° 80°

40–50° 40–50°

40°

Premature reached 40wk

Full term

Passive tone. Increase of tone with maturity illustrated by means of six clinical tests. (From Amiel-Tison, C.: Arch. Dis. Child.,43:89,1968.)

Gestational age

Lower extremity

Brief support

Transitory straightening

Excellent straightening of legs when upright

Good straightening of trunk when upright

Head maintained for a few seconds

Head maintained for a few seconds

Good straightening but not maintained

Maintained in axis for more than a few seconds

Maintained in axis for more than a few seconds

Brisk movement head passes in the axis of trunk

[face view] Head rolls on the shoulder

[profile view] Brisk movement head passes in the axis of trunk

No movement of the head.

Head begins to lift but falls down

Trunk –+ –

Neck flexors

Neck extensors

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Active tone. Increase of tone with maturity illustrated by means of four tests of righting reactions. (From Amiel-Tison, C.: In Gluck, L. (ed.), Modern Perinatal Medicine, Chicago, Year book Medical Publishers, 1974, p. 347.)

Figure 6.4. The premature infant has a large head inrelation to body size and the eyes are protruding due todisproportion between the size of the eyeballs and theorbital cavity. The skin is red to pink, shiny due toedema, transparent with highly visible arterioles andvenules, and is covered by lanugo. Subcutaneous tissueis poorly developed. Scalp hair is sparse and straight.Vernix is not formed and no nipples or areolae areseen. Note that the infant lies flat on the bed, in a frog-leg position with shoulders, elbows, and knees alltouching the mattress. The head is to one side or other,not in line with the trunk.

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Fetal Growth and Assessment of Gestational Age 119

Figure 6.2. A prena-tal ultrasound exami-nation showing a viewof a foot. Foot lengthon prenatal ultra-sound has been shownto correlate well withgestational age.

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Figure 6.3. Graphic rep-resentation of the correlationbetween fetal foot length andgestational age. (Mercer etal. Scatter plots of ultrasonicfeetus. gestational age. AM JObstet Gynecol 1987;156:350 Mosby Year Book,used with permission)

Figure 6.6. When a fetus is undergrown due tointrauterine dysfunction, the sequence beginswith loss of fat and muscle mass as noted in thisgrowth-retarded infant. This is followed by lossof mass of less essential organs (liver, thymus,spleen, adrenals), loss of mass of more essentialorgans (heart), and finally loss of brain mass. Ifhead circumference is compromised, it indicatesthat malnutrition must have been very severe,and it carries a poor prognosis.

Figure 6.7. Two systems are usefulclinically to determine gestational ageby examination of the eye. First,examination of the anterior eye forthe presence of the tunica vasculosalentis, apparent generally from 27 to34 weeks. Note in this composite fig-ure the tunica vasculosa lentis GradeIV at 27 to 28 weeks completely cov-ers the anterior surface of the lens andthen gradually decreases to Grade I by33 to 34 weeks when only peripheralremnants of the vessels are visible onthe anterior surface of the lens.

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Figure 6.5. The postmature infant appears long andskinny due to decreased subcutaneous fat stores withadvancing gestation near term. The skull is hardbecause the sutures have started to fuse. Note thewizened facies and alert expression typical of thepost-term baby. These infants tend to be wakefuland have desquamation of the skin. The hands arelike a washerwomen’s, dry and wrinkled, with longfingernails. Meconium staining may be seen on theumbilical cord and fingernails more commonly inthe post-term infants.

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Figure 6.9. Because calcium is preferen-tially deposited in the last few weeksbefore birth, the stiffness of the baby’sear cartilage provides another test ofmaturity. In premature infants, the earcartilage is deficient and the soft pinnadoes not spring back. On release itremains crumpled against the side of thehead. At term the cartilage is more rigidsimilar to the adult ear.

Figure 6.10. The presence of teeth seenby radiography in the jaw is a relativelyaccurate method of assessing gestationalage. In the figure on the top left, incisorsand cuspids have appeared but nomolars, indicating a gestational age ofless than 33 weeks. In the top right isshown the appearance of incisors andfirst deciduous molars consistent with 33to 37 weeks gestation. On the bottom,the second deciduous molar and follicleof first permanent molar are present,indicating a gestation of greater than 37weeks. This method is not useful inassessing infants with anhydroticectodermal dysplasia who have adentia.

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Figure 6.8. The second system used to determine gesta-tional age involves examination of the fundus to assess mac-ular development from 34 weeks to term. This compositefigure shows Stage I – dark red pigmentation appearing, 34to 35 weeks gestation; Stage II – the annular reflex is par-tially evident, 36 weeks gestation; Stage III – the completeannular reflex is present, 37 weeks gestation; Stage IV – thefoveolar pit can be seen, 38 weeks gestation.

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Figure 6.12. The breast analog does notrespond to hormonal stimulation untilnear the 35th week and thus the size ofthe nodule can be correlated with devel-opment. Before 34 weeks the nipple andareola are identifiable, although they areimmature-looking and non-pigmented.Between 34 and 36 weeks the nipplebecomes erectile and a small nubbin ofbreast tissue can be felt under the areola.By 36 to 38 weeks, there is usually a pal-pable nodule and, by term, the nodulehas enlarged to 5 to 10 mm in size.

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Figure 6.11. Hair, particularly on thehead, is a reliable marker of maturity.Premature hair is fuzzy and the hair endstend to clump together. In the term infantthe hairs are distinct, coarse and silky.Lanugo is absent prior to 20 to 22 weeksgestation, but by 30 to 32 weeks becomesdiffuse over the body. By term, lanugo hasmostly disappeared. There are markedracial differences among babies withrespect to lanugo characteristics. Hispanicinfants, in general, have considerablymore body hair persisting to term andblack infants often have less than averagelanugo.

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Figure 6.15. At 30 weeks or less the soles ofthe baby’s feet are smooth. At 34 weeks,creases have started to appear on the anteri-or third of the plantar surface. By term, theentire plantar area is noticeably creased. Inthe post-term infant shown here, the solesshow excessive creasing on the plantar sur-face together with dry peeling skin.

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Figure 6.13. In the preterm maleinfant the testes are undescended andthe scrotum empty, as pictured here. Inthe preterm female infant the clitoris isrelatively large, and the vulva gapesdue to separation of the labia majoraand minora.

Figure 6.14. Looking for epiphyseal centers in longbone radiographs is useful in the assessment of ges-tational age. The lower extremity radiographs showthe proximal tibial epiphyseal center, which gener-ally appears by 36 weeks gestation, being followedby the distal femoral epiphyseal center at 38 weeksgestation. In this patient no epiphyseal centers areseen, consistent with the infant’s gestational age of35 weeks. Conditions that may alter the normalappearance of the centers include hypothyroidism,congenital viral infections, and congenital syphilis.

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Figure 6.17. Meconium staining ofthe umbilical cord of the same post-term infant.

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Figure 6.16. Meconium staining, asnoted in this post-term infant, is apotential sign of fetal distress and ismore common in the post-term infant.Meconium staining of the fingernails, asnoted in this figure, suggests exposure tomeconium of at least 4 to 6 hours, dura-tion. Presence of meconium in theamniotic fluid may be helpful in assess-ing gestational age. In general, the fetusdoes not pass meconium before 34weeks gestation; hence, the presence ofmeconium may either increase the esti-mated gestation of a premature infant orsuggest that other causes of discoloredamniotic fluid (such as Listeria monocy-togenes) be considered.

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Chapter 7IatrogenesisAll interventions, whether intended as therapy or as monitoring, are inherently invasive andtherefore have associated risks. The task of weighing risks and benefits would be simple if bothwere quantitatively known. Estimating the frequency of complications is difficult except for themost frequent and even those vary widely. Complications due to mechanical trauma, accidents,technique errors, or faulty equipment are potentially preventable. Complications due to interac-tion of the intervention with the patient (e.g., infection, thrombosis) may not be preventableuntil new techniques, equipment, or methods are developed. This section provides a catalogue ofcomplications associated with intensive care in the neonate. Clinicians must continue to be vig-ilant and mindful of potential iatrogenic complications so that these problems may be preventedwhen possible and otherwise rapidly recognized and managed.

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Figure 7.1. Ultrasound examination of the abdomen of apregnant mother (gestational age 35 weeks) who was shotwith a BB gun. Note the shadow from the BB pellet locat-ed in the soft tissue above the fetal femur. (Moise, K.)

Figure 7.2. After delivery of the infant,radiography showed the presence of theBB pellet at the distal end of the femur.The infant had a normal course.

Figure 7.3. This infant developed swelling andsome erythema over the frontal area. Radiographyof the skull shows periosteal elevation of thefrontal bone. This resulted from the application ofa fetal scalp electrode.

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Figure 7.4. Skin incisions onthe buttocks of an infant deliv-ered by cesarean birth. It is notuncommon for infants to haveiatrogenic trauma, such as facialabrasions from forceps delivery,and trauma from diagnostic-therapeutic procedures such asamniocentesis. (Landers, S.)

Figure 7.5. Circular lesions onthe back of an infant as a resultof skin burns from a faulty tran-scutaneous pO2 electrode. Withthe advent of pulse oximetry thetranscutaneous electrodes arerarely used nowadays.

Figure 7.6. Purpuric lesions on the chest of a verysmall premature infant following vigorous physio-therapy.

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Iatrogenesis 127

Figure 7.7. Hypopigmentation of the skin of thelower abdomen as a result of scarring from appli-cation of tape in the newborn intensive care unit.Trauma to the skin from adhesive tape is notuncommon, especially in very low birth weightinfants. The hypopigmentation improves overtime. (Landers, S.)

Figure 7.8. This infant developed necrosis and discol-oration of the toes as the result of burns from the heaterin an incubator at the time Gordon-Armstrong incu-bators were in use for management of prematureinfants. In our nursery, several infants developed burnsover a short period of time. It was determined that thephysician examining the infant would place the infanton top of the incubator and the burn would result fromcontact with a metallic instruction guide which wasnormally present on the incubator cover. Once thecause was determined, this problem was eliminated.The reason for including a historic figure is that in careand management of a sick neonate one should alwaysbe on the alert for possible iatrogenic problems.

Figure 7.9. Percutaneous alco-hol absorption with resultanterythema and burns to thebuttocks of a premature infant.During placement of an umbili-cal arterial catheter, alcohol oriodine may track down the sidesof the abdomen and soak theunderlying sheet. Evaporation isrestricted from the skin in con-tact with the underlying sheetand this may result in irritation,erythema, and severe burns,especially in a premature infantwith very sensitive skin.

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Figure 7.10. In this premature infant with a birthweight of 1000 g and severe hyaline membrane dis-ease, it was elected to do medical management ofthe omphalocele. Mercurochrome was applied threetimes a day to the sac of the omphalocele as atopical solution. Treat-ment with organic mercurialantiseptics is controversial, since infants maydevelop potentially toxic levels of mercury becauseof the reduced skin barriers. The infant developedacute mercurial poisoning with the typical findingsof acrodynia. Other preparations, such as silvernitrate, hexachlorophene, etc., have also beenused with success in medical management of anomphalocele. One should always be on the alert forproblems which may arise from excess absorption.

Figure 7.11. Decubitus ulceration ofthe back of the neck of an infant whowas placed in the supine position andwas on muscle relaxants for 8 days whilebeing treated for septic shock. Thisstresses the importance of nursing carein these infants. (Landers, S.)

Figure 7.12. This infant had alumbar puncture performed toexclude possible meningitis. Fourdays later these nodular erythe-matous lesions developed on theback over the sites of the lumbarpuncture attempts. The lesionswere those of subcutaneous fatnecrosis. The possibility of infec-tion should remain in the differ-ential diagnosis.

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Iatrogenesis 129

Figure 7.13. Slightly fluctuant erythematous mid-line back lesion in an infant with a staphylococcalabscess following lumbar puncture.

Figure 7.14. Appearance of the heels of a lowbirth-weight premature infant after repeated“heel-sticks.” This can be avoided by warming ofthe heel and more gentle squeezing of the heelwhen collecting blood. Miscalculation of thewater temperature when warming a heel maycause scalding and a skin burn. (Landers, S.)

Figure 7.15. Radiographof the feet of an infantwith osteomyelitis of thecalcaneus (on the left)following “heel-stick”trauma. The other heel isnormal (on the right).One of the most commonmethods in infants of col-lecting laboratory bloodspecimens for analysis isby “heel-stick.”

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Figure 7.16. Radiograph ofan infant with a spiral fractureof the distal end of the tibia.This fracture is the result ofincreased angulation withforce applied by a techniciancollecting a “heel-stick” bloodspecimen. On the left, notethe fresh fracture. On theright, note the healing fracture3 weeks later. This infant hadcongenital syphilis. On initialradiographs there were growtharrest lines in the proximaltibia and distal femur, but thetibia was otherwise normal.

Figure 7.17. Edema, bullous excoriation anddiscoloration of the foot of an infant followingdislodgment of the needle from an infusion pump.Ulceration due to extravasation of intravenousinfusion fluid may follow subcutaneous leakage ofany hypertonic solution, but those containingcalcium are particularly irritating.

Figure 7.18. Soft tissueinfiltration following con-tinuous infusion with a nee-dle displaced out of thevein. If these areas are large,they may subsequentlyrequire a skin graft.

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Iatrogenesis 131

Figure 7.19. Calcification ofthe scalp veins following intra-venous infusion of sodium bicar-bonate and calcium gluconate.Deep areas of necrosis mayoccur following infiltrationfrom intravenous solutions con-taining calcium.

Figure 7.20. Radiographic series of an infant’s leg with soft tissueand intravascular calcifications. This infant received calcium glu-conate through a “cut-down” in the ankle. There are two patterns ofcalcification: amorphous (“bulky”) and vascular (going up throughthe thigh).

Figure 7.21. Soft tissue swellingand erythema in this infant withStaphylococcus aureus bacteremiaand abscess. Abscess formationcan occur frequently alone or inassociation with bacteremia atold venipuncture sites.

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Figure 7.22. Infant with staphylococcal scalpinfection following scalp vein infusion.

Figure 7.23. This scalp defect occurred as theresult of a staphylococcal infection with anunderlying osteomyelitis of the skull.

Figure 7.24. Transillumination of thescalp of an infant following infiltrationof fluid from an intravenous scalp infu-sion. Extravasation of fluid in the scalpor scalp edema will transilluminate.

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Figure 7.25. Hypopigmentation, alopecia and scarringon the scalp of a former very low birth weight prematureinfant (birth weight 700 g) following multiple scalp veininfiltrations.

Figure 7.26. Scalp infiltration of total par-enteral nutrition fluid and intralipid in aninfant at the age of 7 days. This healed with-out residual hypopigmentation or scarring.

Figure 7.27. Soft tis-sue nonerythematousswelling of thisinfant’s right side ofneck, arm and chestoccurred as the resultof extravascular extra-vasation of centraltotal parenteral nutri-tion. Note the facialpalsy on the left side.

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Figure 7.28. In this radiograph of the neck andchest, note the right-sided soft tissue swelling inthe neck, a widened mediastinal shadow, and alarge pleural effusion. These findings are the resultof perforation of the vein by the central total par-enteral nutrition catheter.

Figure 7.29. Patchy discoloration of the skin of aninfant as the result of a bolus infusion ofProstaglandin E.

Figure 7.30. Abdominalradiograph of an infantwith a gold-tip transpyloricfeeding tube. Note theright upper quadrant vas-cular calcification of theportal vein. This is theresult of sodium bicarbon-ate and calcium gluconateinfusion given togetherthrough an umbilical ven-ous catheter.

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Figure 7.31. Anteroposteriorand lateral radiographic viewsof an umbilical venous catheterplaced high in the heart. It ispossible to sample oxygenatedleft atrial blood when the cath-eter passes across the patentforamen ovale.

Figure 7.32. Anteroposteriorand lateral radiographic viewsof an infant with an umbilicalvenous catheter placed in theleft pulmonary artery. Theprevious figure and this figurestress the importance ofchecking placement of umbil-ical catheters following theirinsertion. When they areplaced in such abnormal posi-tions they should be with-drawn.

Figure 7.33. Chest and abdominal radiograph of an infant afterplacement of an umbilical venous catheter. Note the placement of thecatheter in the liver with air outlining the portal venous system.

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Figure 7.34. Autopsyspecimen of theumbilical vessels withumbilical vein perfo-ration from a catheterduring umbilical veinplacement.

Figure 7.35. Anteroposterior and lateral radi-ographs in an infant showing an umbilicalartery catheter which is placed high in the tho-racic aorta curving on itself.

Figure 7.36. Anteroposterior andlateral radiographs of the same infantshowing repositioning of the umbilicalartery catheter after its initial highaortic placement. These follow-upfilms show the location of the catheterin the superior mesenteric artery. Theinfant developed abdominal distentionand vomiting and had a large segmentof gangrenous bowel which wasremoved.

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Figure 7.37. Anteroposterior andlateral radiographic views of a“free,” broken umbilical arterycatheter lying in the abdominaland thoracic aorta. The catheterbroke at the insertion site duringplanned removal. The remainingcatheter had to be surgicallyremoved.

Figure 7.38. Histologicview showing on the leftthe umbilical artery and onthe right the juxtaposed“false” catheter passage.The possibility of creating afalse passage should alwaysbe considered when thereis difficulty with placementof an umbilical catheter.

Figure 7.39. Discoloration of the greattoe and little toes of the left foot in thisinfant followed umbilical arterycatheter placement. With removal of thecatheter, vasospasm subsided with sub-sequent normal circulation and appear-ance. Umbilical artery catheterizationmay be associated with thromboticcomplications.

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Figure 7.40. On the left,note the discoloration of thetoes in an infant 4 hours afterplacement of an umbilicalarterial catheter. There wasno significant improvementafter catheter removal. Onthe right, note the gangre-nous and atrophied appear-ance of the same foot 15 dayslater.

Figure 7.41. Severe atrophy andgangrene of the left lower extrem-ity in an infant as a result ofumbilical artery catheter place-ment. This can occur as a result ofvasospasm or clot formation in amajor vessel with inadequate col-lateral support in a very low birth-weight infant.

Figure 7.42. Discoloration andnecrosis of the buttocks as a resultof umbilical artery catheter place-ment in an infant at the age of 4days. This occurs from spasm andthrombosis of the internal obtura-tor artery which supplies the mus-cles of the buttock. Intra arterialinjection of alkali may also be fol-lowed by gangrene of the buttock.

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Figure 7.44. The same infant 10 days later showedmarked improvement of the buttock.

Figure 7.43. Discoloration with gangrene of the right buttock following umbilical artery catheterplacement.

Figure 7.45. Gan-grene of the left buttockfollowing umbilicalartery catherization.The umbilical arterycatheter was posi-tioned in the iliacartery radiographical-ly. There is a well-known associationbetween injection ofmedications into theumbilical artery andnecrosis and gangreneof the buttock and sci-atic nerve palsy.

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Figure 7.46. The same infant with unilateral gan-grene of the buttock also had a sciatic nerve palsyas a result of the umbilical catheter being posi-tioned in the iliac artery. Note the “foot drop.”

Figure 7.47. Radiograph of the lower extremitiesin an infant with leg length discrepancy. The softtissue mass and bone growth of the left leg aredecreased in comparison to the right. These post-natal changes were the result of femoral arterythrombosis after umbilical artery catheter place-ment.

Figure 7.48. Placement of anumbilical artery catheter was diffi-cult in this infant. On the firstand second attempts the catheterwent down each leg. On the thirdattempt the catheter was placedin the aorta. It remained in placefor 4 hours when blood was notedin the urine and the legs wereboth noted to have some discol-oration.This figure shows the sta-tus 19 hours after the catheter wasremoved. The infant continued toimprove.

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Figure 7.49. Autopsy specimen demonstratingthrombosis involving the aorta, iliac arteries andrenal arteries following umbilical artery catheteriza-tion at birth. The infant died at the age of 12 days.

Figure 7.50. Autopsy specimenof the aorta with periumbilicalartery catheter thrombosis.

Figure 7.51. Autopsy specimen of the bladder. Notethe engorged hemorrhagic bladder wall which was theresult of an umbilical artery catheter perforation.

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Figure 7.52. Autopsy specimen ofthe heart in an infant who developedvegetations as a result of prolongedumbilical venous catheter place-ment. Note the verrucous vegeta-tions due to staphylococcal endo-carditis.The condition was diagnosedantemortem by echocardiography. Incases of prolonged sepsis in small pre-mature infants, the diagnosis ofendocarditis should always beexcluded.

Figure 7.53. Gangreneresulting in amputation ofthe right arm following aninfusion of 10% dextrosein the right radial artery.

Figure 7.54. This infant developed obstruction tothe circulation of the distal right forearm andhand following attempts at right radial arterycatheterization. Subsequently, a cut-down wasplaced in the right brachial artery, resulting fromthrombosis. Note the white discoloration andatrophy of the hand and distal forearm with gan-grene of the distal fingertips. A sharp demarcationline separated the normal from the abnormal cir-culation.

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Iatrogenesis 143

Figure 7.55. Close-up of the right hand of the sameinfant 2 weeks later shows the atrophy and gangrene ofthe right hand.

Figure 7.56. The same infant, in additionto the thromboses, had embolization fromthe same site which resulted in discolorationand impaired circulation of the right lowerextremity. This improved.

Figure 7.57. Radiograph of the chest of aninfant admitted to the neonatal intensivecare unit. Note the excessive neonatalequipment interfering with evaluation ofthe status of any cardiorespiratory patholo-gy. When doing radiographic evaluation it isextremely important that an attempt bemade to clear all the excess tubes and leadsto obtain as clear a field as possible.

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Figure 7.58. Radiograph of the chest of aninfant with a translucent circular mediasti-nal shadow. If the radiograph is takenthrough the top of the incubator, it may giveartifactual shadows such as this one causedby the hole in an Isolette™ .

Figure 7.59. Radiograph of chest and abdomen showingartifactual shadows from placement of the infant on an airmattress. It is important to be aware of these possible arti-facts when reviewing radiographic films.

Figure 7.60. An infant with respiratory distressdeveloped acute deterioration. It was noted thatthere was poor movement of air into the lungs. Onremoving the nasotracheal tube a blood clot wasfound to be obstructing the tube. With replacementof the tube there was rapid improvement.

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Figure 7.61. Serial chest radi-ographs of an infant with left lungand right upper lobe opacification asthe result of right mainstembronchus intubation (left). Afterrepositioning the endotracheal tubethere was rapid resolution of theatelectasis (right).

Figure 7.62. Lateral view of the chest andabdomen in an infant who has a “swallowed endo-tracheal tube.” Note that the endotracheal tube is acuffed tube which is rarely used at the present time.During resuscitation instead of endotracheal intu-bation the esophagus was inadvertently intubatedand the tube was swallowed. This was successfullyremoved without any complication.

Figure 7.63. Abdominal radi-ographs of an infant who“swallowed a nasal CPAPtube.” The tube was recoveredin the stool 11 days later.(Singleton, E.)

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Figure 7.64. This infant developed increasing res-piratory distress. Radiography of the chest showedabnormal placement of the feeding tube that tracksinto the right thorax. Associated with this areincreased infiltrates, most prominent in the rightlung. The abnormal placement of the feeding tubeoccurred as a result of esophageal perforation.

Figure 7.65. Radiograph of the chest of the sameinfant 24 hours later showing a right-sidedpneumothorax and placement of the nasogastricfeeding tube in the left thorax as the result ofesophageal perforation. It should be noted thatthe feeding tube may pass either to the right or leftside of the chest. Spontaneous perforation of theesophagus (Boerhaave’s syndrome) does occur, butwould be extremely rare in a neonate. Neonatalperforation is usually associated with trauma suchas from a feeding tube.

Figure 7.66. Abdominal radiograph of an infantwith pneumoperitoneum as a result of nasogastrictube perforation of the stomach. Note the midlinelucency, the “football sign,” with visible falciform lig-ament. Perforation of the stomach may occur sponta-neously as a result of a weakness in the wall of thestomach. It is also reported from placement of feedingcatheters. Application of suction, especially if thecatheter tip is up against the stomach wall, can causedamage. (Singleton, E.)

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Figure 7.67. Soft tissue swelling of the neck was crepi-tant to palpation. This infant developed subcutaneousemphysema as a result of resuscitation. The subcuta-neous emphysema absorbs spontaneously.

Figure 7.68. Radiograph of the chest and abdomen inan infant showing massive left pneumothorax andpneumomediastinum with displacement of the heart tothe right side. On the right side some air is notedbetween the diaphragm and the upper border of theliver. This is a mild pneumoperitoneum from trackingdown of air from the pneumomediastinum. This iatro-genic pathology occurred with vigorous bag and maskresuscitation using excessive pressures.

Figure 7.69. Radiograph of the chest in an infantrequiring resuscitation at delivery. Note the pneumo-mediastinum with the lobes of the thymus gland beingvery prominent bilaterally (“butterfly wing” appear-ance) as a result of the air in the pneumomediastinumlifting up the lobes of the thymus.

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Figure 7.70. Radiograph of the chest in an infant withsevere respiratory distress who developed a pneumo-pericardium causing cardiovascular instability.

Figure 7.71. Chest and abdominal radiograph in aninfant with massive air leak. Note the pneumothorax,pneumomediastinum, pneumoperitoneum and subcu-taneous emphysema. This problem occurred as a resultof the use of excessive pressures in ventilating theinfant.

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Figure 7.73. A plain radiograph of the skull inthe same infant with massive air embolism showsair in the ventricular collecting system. Therewas also an associated large intraventricularhemorrhage. Massive air embolism is a rare com-plication of excessive positive pressure resuscita-tion or ventilation.

Figure 7.74. Radio-graph of the chest of an infant with aleft-sided pneumothor-ax. Note the superiorlocation of the im-properly placed chesttube which is lyingoutside the left thorax.

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Figure 7.72. Radiograph in an infant with mas-sive air embolism. Note the presence of air in theheart and in the vascular bed of the liver.

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Figure 7.76. This low birthweight infant (birth weight1440 g) developed a large pneu-mothorax on the right side. Achest tube was placed in theright sixth intercostal space inthe anterior axillary line. Thetube perforated the diaphragmand liver resulting in massivehemorrhage from the liver. Atautopsy the figure on the leftshows the placement of the tubethrough the chest wall withdamage to the diaphragm. Thefigure on the right shows theperforation of the diaphragmwhich then resulted in damageto the liver and hemorrhage.

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Figure 7.75. Radiograph of thechest of the same infant withrapid resolution of the pneu-mothorax after proper placementof the chest tube into the leftthorax.

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Figure 7.78. As the result of prolonged endotra-cheal tube intubation this infant developed ridgingof the hard palate with a subsequent high arch.

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Figure 7.77. Autopsy specimenof the liver from the same infant.Note the laceration of the liverwhich resulted in the hemor-rhage.

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Figure 7.79. Radiographsof the chest in an infantwith (on the left) an appar-ently normal chest soonafter birth. On the right, 2weeks later, note the frac-ture of the right humerusand fractures of the ribs. Theetiology was obscure but wasthought to be iatrogenic.

Figure 7.80. Radiograph of the lower extremities in aninfant with bronchopulmonary dysplasia who developedrickets. Note the poor mineralization of the bones withmetaphyseal flaring and cupping.

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154

Abdomen, 136, 145-149, Abortion, 70Abruptio placentae, 5Acardius acephalus, 33-34Acardius anceps, 36Accessory lobe. See Succenturiate lobeAccutane embryopathy. See Retinoic acid

embryopathyADAM (amniotic deformities, adhesions, and mutilations), 110

Adams-Oliver syndrome, 114Adhesive tape, 128Air embolism, 149-150Alcoholism, 47-48Amniocentesis, 11-12Amnion, 3, 20-22, 25-26Amniotic bands, 102-116Amputation, 103-109Amyoplasia congenita disruptive sequence, 102Anastomosis, 27, 29, 31, 33-34Anencephaly, 42-43Annular, 4Arm(s), 109Arthrogryposis, 100-102Ballard method, 118Bathing trunk nevus. See Garment nevusBilateral cleft lip and palate, 109Bilateral clubfoot, 98Bipartite placenta, 2-3Birth trauma, 57-79Breast, 122 Breech presentation, 67-70, 90-94, 96Bronchopulmonary dysplasia, 152Bruising, 67Burns, 127, 128Buttock(s), 127-128, 139-141Calcification, 6, 61, 132, 135 Candidiasis, 17Caput ring, 59Caput succedaneum, 58-59Caudal regression syndrome, 45, 101Caul, 64Cephalhematoma, 60-62Cephalothoracopagus twins, 41Cervical cord injury, 74Chest,

iatrogenesis and, 135-136, 144-151Chignon, 59Chorangioma, 6Chorioamnionitis, 18-19, 22Chorion, 3, 25-26Circummarginate placenta. See Circumvallate

placentaCircumvallate placenta, 3

Clavicle(s), 75-76Cleft lip and palate, 111

bilateral, 109Clubfoot, 98Congenital amputation, 103-109Congenital curly toes, 99Congenital hypoplasia, 78Congenital molding of skull. See Intrauterine

skull fractureCongenital postural deformities, 81-102Congenital scalp defect, 109-110, 114-115Congenital torticollis, 75Conjoined twins, 36-46Conradi-Hünermann syndrome, 56Coumadin embryopathy. See Fetal warfarin

syndromeCraniopagus twins, 36, 40Curly toes, 99Cutis hyperelastica. See Ehlers-Danlos syndromeCytomegalovirus, 18Decubitus ulceration, 129Deformations, 81-102Diamnionic placenta, 25-26Dicephalus twins, 41-42Dichorionic placenta, 23, 25Dichorionic twins, 23Dilantin, 50Dimples, 83Dipygus twins, 44Discordant twins, 46Disruptions, 102-116Dizygous twins, 23, 25Drugs. See MedicationDubowitz examination, 118Dysplasia, 111

bronchopulmonary, 152Ear(s), 86

lobe, 85Ehlers-Danlos syndrome, 94Encephalocele, 110Endotracheal tube intubation, 151Erb’s palsy, 78-79Escherichia coli, 61-62Face, 65-66Facial palsy, 77-78, 134Facioauriculovertebral spectrum. See Goldenhar’s

syndromeFeet, 123

deformations in, 95-96disruptions in, 106-108iatrogenesis and, 131, 138, 141See also Clubfoot; Heels; Sole(s); Toe(s)

Femur, 76-77Fetal alcohol syndrome, 48

Index

155

Fetal growth, 117-124Fetal hydantoin syndrome, 50-52Fetal valproate syndrome, 53-54Fetal warfarin syndrome, 56Feto-fetal transfusion syndrome. See Twin

transfusion syndromeFetus papyraceus, 32-33Finger(s), 103-106Foot. See Clubfoot; FeetForceps delivery, 64Fractures, 72-77, 131, 152Fraternal triplets, 24Fraternal twins. See Dizygous twinsFunisitis, 15-17Gangrene, 70, 139-141, 143-144Garment nevus, 19Genitalia, 122Genu recurvatum, 94-95Gestational age, 117-124Goldenhar’s syndrome, 111Gum hypertrophy, 51Hair, 122Hand(s) congenital amputation, 109

See also Finger(s)Hanging neck, 69Heels, 130-131Hemiacardius. See Acardius ancepsHeteroadelphia, 44Horner’s syndrome, 79Humerus, 76, 152Hydantoin, 50-52Hydrops fetalis, 6Hypertrichosis, 51Hypertrophy, 51Hypopigmentation, 128, 134

congenital, 78Iatrogenesis, 125-152Identical triplets, 24Identical twins. See Monozygous twinsIncisions, 127Incubators, 128Intrauterine constriction bands. See Amniotic

bandsIntrauterine contraceptive device, 5Intrauterine death, 9-10Intrauterine skull fracture, 72-74Intravenous infusion, 131-133Ischiopagus twins, 36, 43-44Jaw(s), 86, 88-89Knee(s), 94-95Labor, 57-59Larsen’s syndrome, 95Leg(s), 107-108, 141

Lesions, 127Limb(s), 35, 114

See also Arm(s); Leg(s)Lip(s), 109, 111Listeriosis, 16Lithopedion, 33Liver, 71, 151Low-birth-weight infant, 151Lower extremities, 152Lumbar puncture, 129-130Massive air embolism, 149-150Massive scalp hemorrhage, 62-63Maternal medication, 47-56Meconium staining, 19, 124Medication, 47-56Melanoma, 20Metatarsus adductus, 95-96Metatarsus varus. See Metatarsus adductusMonoamnionic placenta, 23, 27-28, 36Monochorionic placenta, 23, 25-26, 27-28, 36Monochorionic twins, 23Monozygous twins, 23, 25-26, 29-30Moro reflex, 79Mouth

See also Lip(s)Multiple births, 23-46

See also TwinsNarcotics addiction, 47, 49Neck, 69Nose, 89-90Nostrils, 89-90Oculoauriculovertebral dysplasia. See Goldenhar’s

syndromeOligohydramnios, 22Omphalitis, 15Omphalocele, 33, 44, 111-112, 114Omphalopagus twins, 40Osteomyelitis, 61-62Overlapping toes. See Congenital curly toesPalate, 109, 111Petechiae, 65, 68Phocomelia, 55Placenta, 1-21

attached to leg, 113bipartite, 2-3calcified, 6hemangioma of, 6with multiple nevi, 20in triplets, 24in twins, 23, 26-35

Placenta diffusa. See Placenta membranaceaPlacenta duplex. See Bipartite placentaPlacenta membranacea, 4

156

Position-of-comfort anomalies, 81-102Postmature infant, 120, 123-124Postural deformities, 81-102Premature infant, 66, 119, 129-130Prosopothoracopagus twins, 41Prostaglandin E, 135Pygopagus twins, 36, 39Respiratory insufficiency, 145-150Retinoic acid embryopathy, 49-50Rib(s), 152Rickets, 152Scalp, 132-134

congenital defects, 109-110, 114-115massive hemorrhage, 62-63

Scarring, 128Scoliosis, 113-114SGA. See Small for gestational ageShoulder, 71Single umbilical artery, 12-13Skin

burns, 127dimples, 83incisions, 127

Skull,intrauterine fracture, 72-74

Small for gestational age (SGA), 117Spinal cord injury, 74Squamous metaplasia, 20-21Stillbirths, 29, 33Stone child. See LithopedionSubgaleal hemorrhage. See Massive scalp

hemorrhageSuccenturiate lobe, 2Syphilis, 19Talipes calcaneovalgus, 96-97, 101-102Talipes equinovarus. See Bilateral clubfootTEARS (the early amnion rupture spectrum), 82,

103, 110-112, 115Teeth, 121Tetracycline, 47, 54Thalidomide syndrome, 47, 55Thoracopagus twins, 28, 36-38

Tibia, 131Toe(s),

amniotic bands, 106, 108and catheterization, 138congenital curly, 99and trisomy 18, 169-170

Torticollis, 75TRAP (twin-reversed-arterial-perfusion), 33Triplets, 24Twins

amnion nodosum in, 21with asymmetrical heads, 99-100conjoined, 36-46dichorionic, 23dizygous, 23, 25monochorionic, 23monozygous, 23, 25-26, 29-30

Twin transfusion syndrome, 27, 29-32Ultrasound, 117, 119, 126Umbilical artery, 12-13Umbilical catheter, 128, 136-143Umbilical cord, 1-21

around neck, 8-9, 64-65cyst in, 14-15fused, 38hematoma of, 10, 12knot in, 10-11long, 8-9meconium staining of, 124short, 10, 113thrombosed vessel in, 13transection of, 11-12in twins, 27-29, 35velamentous insertion of, 7-8, 24

Upper brachial plexus injury. See Erb’s palsyVacuum extractor, 62-63Valproic acid, 47, 53-54Warfarin, 47, 56Wharton’s jelly, 10-11, 13-14Wood’s filter, 54Wrist drop, 79, 82, 92

Atlas of the Newborn [Vol 1] - A. Rudolph (BC Decker, 1997) WW

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