Pediatric Surgery Update Volume 31, 2008

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6/29/12 PEDIATRIC SURGERY UPDATE Volume 31, 2008

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PEDIATRIC SURGERY UPDATE

VOLUME 31, 2008

PSU Volume 31 No 01 JULY 2008Prophylactic Antibiotics

Antibiotics have an important role in preventing wound infections during emergent and elective

surgery. The most critical factors in prevention of postoperative wound infections are sound

judgment and proper technique of the surgeon and surgical team. Antibiotics prophylaxis in surgery

is governed by such factors as: surgical wound classification (most important), host immune

system function and immune competence, host nutritional status, type and prolongation of the

surgical procedure, hospital vs. community emerging difficult to manage strains, and emergency

vs. elective procedure According to wound type, clean procedures (hernias, excisions, biopsy) need

no use of prophylactic antibiotics. Clean contaminated procedures (surgery entering airway, bowel,

breast, urinary or bile lumen) should receive one preoperative dose of broad spectrum antibiotic

followed by 24 hours postoperative prophylaxis. Contaminated and dirty surgical procedures

(empyema, non-prepared bowel perforation, perforated appendicitis, infected urinary tract) should

receive preoperative double antibiotic therapy continued according to individual laboratory and

clinical condition in the postoperative period. Special considerations such immune, asplenic and

nutritional compromised hosts should receive antibiotic prophylaxis. Prolonged procedures beyond

three hours should receive an intraoperative dose. Long-term hospitalized children submitted to

surgery should receive high graded antibiotic therapy due to colonization with antibiotic resistantstrains.

References:

1- ACS Surgery: Principles and Practice, Chapter 1: Basic Surgical and Perioperative Cons iderations , pags 9 and 13, 20062- Lee Nichols R: Preventing Surgical Site Infections : A Surgeons Perspective. Emerging Infectious Diseases, 7(2): 220,

20013- Ichikawa S, Ishihara M, Okazaki T, et al: Prospective study of antibiotic protocol for managing surgical site infections in

children. Journal of Pediatr Surgery 42: 1002-1007, 2007

Playground Injuries

Children sustaining injuries from playground equipment constitute a common cause of visiting the

emergency room of Children hospitals. Injuries are usually minor, but sometimes serious head or

extremity injury or even death can occur. Injuries consist of fractures (most common),

contusion/abrasions, laceration, hematomas, strains/sprains and brain injury. In order of frequency

monkey bars are responsible from the majority of cases, followed very closely by swings and

slides. Geographically, they occur with almost the same frequency at school, recreation/sporting

facilities and home. Body area injured consist of the upper extremity (most common), followed by

head/neck, lower extremity and trunk. Most of the children are managed and released the same

day. Monkey bars injuries are usually fractures, while swings at school cause most brain injuries

(mechanism: a young child moves behind a moving swing). Upper extremity fractures due to

climbing account for the majority of hospitalizations. Older children sustained more injuries on


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climbing apparatus, where younger children sustained more injuries on slides. Removing and

replacing unsafe equipment is an effective strategy for preventing playground injuries.

References:

1 Lillis KA, Jaffe DM: Playground injuries in children. Pediatr Emerg Care. 13(2):149 53, 19972 Macarthur C, Hu X, Wess on DE, Parkin PC: Risk factors for severe injuries as sociated with falls from playground

equipment. Accid Anal Prev. 32(3):377 82, 20003 Phelan KJ, Khoury J, Kalkwarf HJ, Lanphear BP: Trends and patterns of playground injuries in United States children and

adolescents. Ambul Pediatr. 1(4):227 33, 20014 Howard AW, MacArthur C, Willan A, Rothman L, Moses McKeag A, MacPherson AK: The effect of safer play

equipment on playground injury rates among school children. CMAJ. 172(11):1443 6, 20055 Loder RT: The demographics of playground equipment injuries in children. J Pediatr Surg. 43(4):691 9, 2008

Internal Hernias

Internal hernias are a rare cause of bowel obstruction (5%) in children and adults. These

mesenteric defects are most commonly acquired or congenital in nature. Acquired internal herniasoccur postoperatively resulting from incomplete closure of surgically created mesenteric defects.

Congenital mesenteric defects represent 10% of all internal hernias. Pathogenesis of these

congenital defects includes regression of the dorsal mesentery, enlargement of a hypovascular

area and compression of mesentery by the colon. Most congenital mesenteric defects that lead to

herniation occur in the small bowel mesentery, are 2 to 3 cm wide and trap a loop of ileum.

Herniation of small bowel through the falciform ligament and Winslows foramen has also been

reported. Clinically, children shows sign of intermittent small bowel obstruction, nausea, vomiting,

abdominal pain and distension which can follow into bowel incarceration or strangulation.

Preoperative diagnosis is difficult and a high index of suspicion is needed to order appropiatestudies for diagnosis such as upper bowel contrast studies or CT-Scan. Misdiagnosis results in

delayed exploration which leads to bowel necrosis and death. Surgical exploration is the only

means of definitive diagnosis.

References:

1 Ozen? A, Ozdemir A, Coskun T: Internal hernia in adults. Int Surg. 83(2):167 70, 19982 Moran JM, Salas J, Sanjun S, Amaya JL, Rinc?n P, Serrano A, Tallo EM: Paramesocolic hernias: consequences of

delayed diagnos is. Report of three new cas es. J Pediatr Surg. 39(1):112 6, 20043 Agresta F, Michelet I, Candiotto E, Bedin N: Incarcerated internal hernia of the small intes tine through a breach of the

broad ligament: two cases and a literature review. JSLS. 11(2):255 7, 20074 Mboyo A, Goura E, Massicot R, Flurin V, Legrand B, Repetto Germaine M, Caron Bataille S, Ndi? J: An exceptional caus e

of intestinal obs truction in a 2 year old boy: strangulated hernia of the ileum through Winslow's foramen. J Pediatr Surg.

43(1):e1 3, 20085- Gingalewski C, Lalikos J: An unusual cause of small bowel obstruction: herniation through a defect in the falciform

ligament. J Pediatr Surg. 43(2):398 400, 20086 Page MT, Ricca RL, Resnick AS, Puder M, Fishman SJ: Newborn and toddler intestinal obstruction owing to congenital

mesenteric defects. J Pediatr Surg 43(4): 755 758, 2008

PSU VOL 31 NO 02 AUGUST 2008

Visceral Myopathy


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Bowel dysmotility leading to severe intractable constipation in children is a very serious unsolved

condition in pediatric surgery. Constipation needs initial rectal biopsy to determine if the child has

ganglion cells or not present. As histopathological studies increase their diagnostic acuity we have

a more relevant understanding of the physiology of altered motility in the small and large intestine.

One of those components are the interstitial cell of Cajal (ICC) of the bowel. ICC are known to be

essential regulators of gastrointestinal motility, they are called the pacemaker cells of the smooth

muscle of the gastrointestinal tract. Studies have revealed reduced numbers or the absence of ICC

in small intestine and colon that do not exhibit normal peristaltic activity (chronic idiopathicconstipation). In patients with slow-transit constipation, the number of ICC is significantly

decreased in all layers except the outer longitudinal muscle layer, while the myenteric plexus

shows moderate hypoganglionosis. Persistent dysmotility problems after pull-through operation in

aganglionosis may be due to altered distribution and impaired function of ICC. Delay in the

development of ICC in the gastrointestinal tract may be a cause of intestinal pseudoobstruction in

the newborn. Such conditions as pyloric stenosis, Hirschsprung's disease, hypoganglionosis,

intestinal neuronal dysplasia, internal anal sphincter achalasia, megacystis microcolon intestinal

hypoperistalsis syndrome have been reported to be associated with loss or deficiency of ICC.

References:1- Huizinga JD: Neural injury, repair, and adaptation in the GI tract. IV. Pathophys iology of GI motility related to interstitial

cells of Cajal. Am J Physiol. 275(3 Pt 1):G381-6, 1998

2- Wedel T, Spiegler J, Soellner S, Roblick UJ, Schiedeck TH, Bruch HP, Krammer HJ:Enteric nerves and interstitial cells of Cajal are altered in patients with slow-transit cons tipation and megacolon.

Gastroenterology. 123(5):1459-67, 20023- Rolle U, Piotrowska AP, Nemeth L, Puri P: Altered distribution of inters titial cells of Cajal in Hirschsprung disease. Arch

Pathol Lab Med. 126(8):928-33, 20024- Rolle U, Yoneda A, Solari V, Nemeth L, Puri P.: Abnormalities of C-Kit-positive cellular network in isolated

hypoganglionosis. J Pediatr Surg. 37(5):709-14, 20025- Kenny SE, Vanderwinden JM, Rintala RJ, Connell MG, Lloyd DA, Vanderhaegen JJ, De Laet MH: Delayed maturation of

the interstitial cells of Cajal: a new diagnos is for transient neonatal pseudoobs truction. Report of two cases . J Pediatr Surg.

33(1):94-8, 19986- Rolle U, Piaseczna-Piotrowska A, Puri P: Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of

childhood. Pediatr Surg Int. 23(12):1139-52, 2007

Endoscopic Injuries

The volume of gastrointestinal endoscopies done to children yearly has increased considerably

over the last ten years. More children undergo diagnostic and therapeutic upper, lower and ERCP

endoscopies. Most therapeutic endoscopic procedures are done on an ambulatory basis. The

incidence of complications is near 0.06 to 0.5% depending on the procedure. Perforation of the

colon during colonoscopy is the most serious endoscopic related injury in children due to the need

of operative intervention from bacterial seeding and peritonitis. Upper endoscopy and ERCP

entails complications such as mucosal tear, bleeding and bowel perforation. Most cases are

amenable to nonoperative therapy. Bleeding complications might need transfusion, cauterization,

endoscopic hemoclip or angiographic embolization. Perforation of the duodenum may need prompt

surgical repair as they cause rapid chemical and bacterial peritonitis. Esophageal perforations are

amenable to observation. Needless to say endoscopic procedures in children are very safe with a

low incidence of complications. The more complicated the procedure, the higher the incidence of

complications. Surgeons are always verb-called to assist such complications. Patients who survive


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the initial complications have excellent long-term outcomes.

References:1- Panieri E, Millar AJ, Rode H, Brown RA, Cywes S: Iatrogenic esophageal perforation in children: patterns of injury,

presentation, management, and outcome. J Pediatr Surg. 31(7):890-5, 19962- Enns R, Eloubeidi MA, Mergener K, Jowell PS, Branch MS, Pappas TM, Baillie J: ERCP-related perforations : risk factors

and management. Endoscopy. 34(4):293-8, 20023- Cobb WS, Heniford BT, Sigmon LB, Hasan R, Simms C, Kercher KW, Matthews BD: Colonoscopic perforations :

incidence, management, and outcomes. Am Surg. 70(9):750-7, 20044- Abadir J, Emil S, Nguyen N: Abdominal foregut perforations in children: a 10-year experience. J Pediatr Surg. 40(12):1903-

7, 20055- Iqbal CW, Askegard-Giesmann JR, Pham TH, Ishitani MB, Moir CR: Pediatric endoscopic injuries: incidence,

management, and outcomes. J Pediatr Surg. 43(5):911-5, 2008

Snowboard Injuries

We don't have snow in the tropics, but yearly thousands of kids suffer from snowboard injury.

Unlike skiing, snowboard is a relatively new sport with a dramatic rise in popularity associated with

serious injuries. Snowboarding males are more commonly affected than females, while skiers have

a longer length of hospital stay. Skiers and snowboarder both sustain in order of higher frequency

head, extremity (skeletal), and intra-abdominal injuries. Two-third occurs at licensed resorts, and

one-third at parks or private property. Head trauma is the leading cause of death among both

groups of sports. Curiously, helmet are required for all international snowboarding competitions,

while is not usually utilized in recreational resorts. Risk of snowboard related injury was highest in

beginners. Mean severity injury score and splenic injuries (snowboard spleen) are more commonly

found in snowboarder than skiers since they do more aerial jumping maneuvers with a higher

incidence of traumatic falls. Snowboarders who wore protective wrist guards were half as likely to

sustain wrist injuries as those who did not wear guards. Elite snowboarders suffer more from knee

than wrist injury.

References:1- Hackam DJ, Kreller M, Pearl RH: Snow-related recreational injuries in children: assess ment of morbidity and management

strategies. J Pediatr Surg. 35(9):1409-10, 20002- Machold W, Kwasny O, G ss ler P, Kolonja A, Reddy B, Bauer E, Lehr S: Risk of injury through snowboarding. J Trauma.

48(6):1109-14, 20003- Idzikowski JR, Janes PC, Abbott PJ: Upper extremity s nowboarding injuries. Ten-year results from the Colorado

snowboard injury s urvey. Am J Sports Med. 28(6):825-32, 20004- Bladin C, McCrory P, Pogorzelski A: Snowboarding injuries : current trends and future directions. Sports Med. 34(2):133-

9, 2004

5- Hagel B: Skiing and snowboarding injuries. Med Sport Sci. 48:74-119, 20056- Hayes JR, Groner JI: The increas ing incidence of snowboard-related trauma. J Pediatr Surg. 43(5):928-30, 2008

PSU Volume 31 No 03 SEPTEMBER 2008Jeune Syndrome

The two most common chest wall deformities in pediatric surgery are pectus excavatum and pectus


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carinatum. Pectus excavatum manifests itself during early childhood, while pectus carinatum is

seen more commonly in early adolescent ages. Jeune syndrome is a rare autosomal recessive

chest wall skeletal dysplasia also known as asphyxiating thoracic dystrophy or insufficiency.

Almost all cases of Jeune syndrome has been described in children. The syndrome is characterized

by respiratory distress, osseous dysplasia, and short stature. Patients generally die during the first

months of life since the thoracic chest wall will not grow creating entrapment of lung and asphyxia.

Besides a small thorax, varying degrees of rhizomelic brachymelia, polydactyly, pelvic

abnormalities, renal anomalies, cystic les ions of the pancreas, retinal anomalies and hepaticfibrosis are also present in the syndrome. Diagnosis can be made prenatally. The life-saving

procedures to expand the chests of infants born with Jeune asphyxiating thoracic dystrophy

provide a static solution incapable of responding to the growth demands of thriving patients. Actual

management consist either of dynamic lateral thoracic expansion with titanium struts or vertical

expandable prosthetic titanium rib thoracoplasty creating additional chest wall that is formed of

autologous tissue.

References:1- Giorgi PL, Gabrielli O, Bonifazi V, Catassi C, Coppa GV: Mild form of Jeune syndrome in two sisters. Am J Med Genet.

35(2):280-2, 19902- Chen CP, Lin SP, Liu FF, Jan SW, Lin SY, Lan CC: Prenatal diagnos is of asphyxiating thoracic dysplasia (Jeune

syndrome). Am J Perinato l. 13(8):495-8, 19963- Kaddoura IL, Obeid MY, Mroueh SM, Nasser AA: Dynamic thoracoplasty for asphyxiating thoracic dys trophy. Ann

Thorac Surg. 72(5):1755-8, 20014- Yerian LM, Brady L, Hart J: Hepatic manifestations of Jeune syndrome (asphyxiating thoracic dystrophy). Semin Liver

Dis. 23(2):195-200, 20035- Davis JT, Long FR, Adler BH, Castile RG, Weins tein S: Lateral thoracic expansion for Jeune syndrome: evidence of rib

healing and new bone formation. Ann Thorac Surg. 77(2):445-8, 20046- Waldhausen JH, Redding GJ, Song KM: Vertical expandable prosthetic titanium rib for thoracic insufficiency syndrome: a

new method to treat an old problem. J Pediatr Surg. 42(1):76-80, 2007

Pectus Excavatum

Pectus excavatum (funnel chest) is the most common chest wall deformity in children. The

depression in the sternum in relation to the costal cartilages causes a distressful cosmetic defect

with mild to no changes in pulmonary and cardiac function. Symptoms of lack of endurance,

shortness of breath with exercise , or chest pain are frequent. Indications for surgical treatmentincludes severe, symptomatic deformity; paradoxical respiratory chest wall motion; CT scan with a

pectus index greater than 3.25, cardiac compression/displacement, pulmonary compression,

pulmonary function studies showing restrictive disease, or other cardiac pathology secondary to

compression of the heart. Originally repaired open using the Ratvich technique of costal cartilage

removal, this has fallen into disuse and replaced with a minimally invasive technique called Nuss

procedure where a titanium bar is placed behind the sternum and fixed to each side of the thoracic

wall for two years before removal. Thoracoscopy while placing the bar behind the sternum helps

avoid significant complications. Though the complications reported with the Nuss procedure are

minimal, a few of them are significant such as laceration of the internal mammary artery,

hemopericardium, recurrence of the pectus deformity after bar removal and bar displacement.

There is a 1.5% incidence of postoperative wound infection. Surgical repair of the pectus

excavatum improves cardiovascular function but there is no significant improvement in pulmonary

function. The value of routine testing of pre- and postoperative lung function in patients with pectus


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excavatum is questionable. Good sternal elevation as measured by preoperative and postoperative

CT scans can be achieved with the Nuss procedure regardless of the severity of chest depression

or age. Results are good with excellent cosmetic results. Minimally invasive repair of pectus

excavatum procedure and removal of the pectus bar should only occur in specialized institutions

with wide experience in thoracic surgery.

References:1- Malek MH, Berger DE, Housh TJ, Marelich WD, Coburn JW, Beck TW: Cardiovascular function following surgical repair

of pectus excavatum: a metaanalysis. Chest. 130(2):506-16, 20062- Shin S, Gorets ky MJ, Kelly RE Jr, Gustin T, Nuss D: Infectious complications after the Nuss repair in a series of 863

patients . J Pediatr Surg. 42(1):87-92, 20073- Aronson DC, Bosgraaf RP, Merz EM, van Steenwijk RP, van Aalderen WM, van Baren R: Lung function after the minimal

invas ive pectus excavatum repair (Nuss procedure). World J Surg. 31(7):1518-22, 20074- Vegunta RK, Pacheco PE, Wallace LJ, Pearl RH: Complications associated with the Nuss procedure: continued evolution

of the learning curve. Am J Surg. 195(3):313-6, 20085- Nakagawa Y, Uemura S, Nakaoka T, Yano T, Tanaka N: Evaluation of the Nuss procedure using pre- and postoperative

computed tomographic index. J Pediatr Surg. 43(3):518-21, 20086- Kelly RE Jr.: Pectus excavatum: historical background, clinical picture, preoperative

evaluation and criteria for operation. Semin Pediatr Surg. 17(3):181-93, 2008

7- Nuss D: Minimally invasive surgical repair of pectus excavatum. Semin Pediatr Surg. 17(3):209-17, 2008

Trocar Hernias

With the advent of minimally invasive procedures the trocar (port site) hernia has emerged. A

trocar hernia is an abdominal defect caused by the sheath of the trocar through which omentum or

even small bowel can protrude (incisional hernia). Trocar hernias occur in 3% of all laparoscopic

procedures. Risk factors which increase the development of a trocar hernia are: large trocar (10

mm or larger), trocar design, use of linea alba for port placement, pre-existing fascial defect, pre-

school children and skinny constitution. When small bowel protrudes a Richter's hernia develops.

The usual presentation is of crampy abdominal pain with nausea and vomiting. Treatment is

reduction of the bowel that is incarcerated and then repair of the fascial defect. To avoid trocar

hernias fascial closure of port sites of 10-mm in adults and 5-mm in children should be accomplish

whenever possible. Trocar site hernias in infants are mainly of omental protrusion and occur within

the first postoperative week.

References:1- Holzinger F, Klaiber C: Trocar site hernias. A rare but potentially dangerous complication of laparoscopic surgery.

Chirurg . 73(9):899-904, 20022- Boughey JC, Nottingham JM, Walls AC: Richter's hernia in the laparoscopic era: four case reports and review of the

literature. Surg Laparosc Endosc Percutan Tech. 13(1):55-8, 2003

3- Tonouchi H, Ohmori Y, Kobayashi M, Kusunoki M: Trocar site hernia. Arch Surg. 139(11):1248-56,20044- Mahmoud Uslu HY, Ustuner EH, Sozener U, Ozis SE, Turkcapar AG: Cannula site insertion technique prevents incisional

hernia in laparos copic fundoplication. Surg Laparosc Endos c Percutan Tech. 17(4):267-70, 2007

5- Paya K, Wurm J, Fakhari M, Felder-Puig R, Puig S: Trocar-site hernia as a typical pos toperative complication of minimallyinvasive surgery among preschool children. Surg Endosc. Feb 13,2008

PSU Volume 31 No 04 OCTOBER 2008


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Ethanol Lock

Children needing long-term parenteral nutrition or chemotherapy will do so with the help of a

central venous catheter (CVC). With each episode of thrombosis or catheter-related infection

(CRI) the rate of catheter turnover will increase and fewer veins will be access ible for such

purposes. Most CRI can be managed with systemic antibiotics without removing the catheter. The

use of 70% ethanol lock solution has been described for prevention and management of catheter-

related infections in children and adults. Ethanol lock denatures proteins and is rapidly bactericidaland fungicidal in vitro, exhibits thrombolytic effects eliminating the need to maintain catheter

patency. Ethanol lock has been used to dislodge lipids clots in occluded catheters. Patients who

received prophylactic ethanol lock therapy have a 95% decrease in catheter replacements

required. The use of antibiotic-lock therapy for CRI prevention has the theoretical disadvantage of

increased antibiotic resistance. The ethanol lock volume can be something between 0.5 and 1 ml.

Whether degradation of silicone catheters occurs with repeated ethanol instillation is unknown, but

it should not be used with polyurethane catheters as it degrades the internal lumen. No severe

clinical side effects of ethanol flush are observed. Mild symptoms that could occur include

tiredness, headaches, dizziness, nausea, and light-headedness.

References:1- Dannenberg C, Bierbach U, Rothe A, Beer J, Krholz D: Ethanol-lock technique in the treatment of bloodstream

infections in pediatric oncology patients with broviac catheter. J Pediatr Hematol Oncol. 25(8):616-21, 20032- Crnich CJ, Halfmann JA, Crone WC, Maki DG: The effects of prolonged ethanol expos ure on the mechanical properties of

polyurethane and silicone catheters used for intravascular access. Infect Control Hosp Epidemiol. 26(8):708-14, 20053- Onland W, Shin CE, Fustar S, Rushing T, Wong WY: Ethanol-lock technique for persistent bacteremia of long-term

intravas cular devices in pediatric patients . Arch Pediatr Adolesc Med. 160(10):1049-53, 2006

4- Opilla MT, Kirby DF, Edmond MB: Use of ethanol lock therapy to reduce the incidence of catheter-related bloodstreaminfections in home parenteral nutrition patients. JPEN J Parenter Enteral Nutr. 31(4):302-5, 20075- Broom J, Woods M, Allworth A, McCarthy J, Faoagali J, Macdonald S, Pithie A: Ethanol lock therapy to treat tunnelled

central venous catheter-associated blood stream infections: results from a prospective trial. Scand J Infect Dis. 40(5):399-

406, 20086- Mouw E, Chessman K, Lesher A, Tagge E: Use of an ethanol lock to prevent catheter-related infections in children with

short bowel syndrome. J Pediatr Surg. 43(6):1025-9, 2008

Splenectomy for Sickle Cell Disease

Sickle cell disease (SCD) is a chronically hemolytic debilitating condition causing recurrent packed

blood cell transfusions in children to manage or prevent complications such as anemia, stroke,

acute chest syndrome, severe pain syndrome or splenic sequestration. The major indication for

splenectomy in children with SCD consists of recurrent splenic sequestrations' episodes and severe

hemolysis due to secondary hypersplenism. Clinically the sequestration is characterized by

hypovolemia, worsening anemia, extreme reticulocytosis and a tender enlarged spleen.

Hypersplenism is characterized by anemia, thrombocytopenia, or neutropenia usually associated

with splenomegaly. Restoration of blood volume and circulating red cell mass forms the basis of

management of sequestration episodes and hypersplenism in SCD children. Splenic sequestrations

occur more often in children less than six years of age. Total laparoscopic splenectomy is

recommended after managing the acute event to prevent further recurrences of splenic

sequestration crisis and reverse hypersplenism. Lap splenectomy, even in functional asplenic

(small spleens) individuals reduce the transfusion requirements (reducing iron overload),


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maintaining stable hematologic parameters and decreasing red cell turnover. The rate of

postoperative acute chest syndrome is smaller with laparoscopic than open splenectomy. The risk

of overwhelming postsplenectomy sepsis crisis has decreased significantly over the years due to

preoperative immunization and use of prophylactic antibiotics.

References:

1- Hendricks-Fergus on VL, Nelson MA: Laparoscopic splenectomy for splenic sequestration crisis. AORN J. 71(4):820-2,2000

2- Overturf GD: Pneumococcal vaccination of children. Semin Pediatr Infect Dis. 13(3):155-64, 20023- Al-Salem AH: Indications and complications of splenectomy for children with sickle cell disease. J Pediatr Surg.

41(11):1909-15, 20064- Rescorla FJ, West KW, Engum SA, Grosfeld JL: Laparoscopic splenic procedures in children: experience in 231 children.

Ann Surg. 246(4):683-7, 20075- Ghantous S, Al Mulhim S, Al Faris N, Abushullaih B, Shalak F, Yazbeck S: Acute chest syndrome after splenectomy in

children with sickle cell disease. J Pediatr Surg. 43(5):861-4, 20086- Haricharan RN, Roberts JM, Morgan TL, Aprahamian CJ, Hardin WD, Hilliard LM, Georgeson KE, Barnhart DC:

Splenectomy reduces packed red cell transfusion requirement in children with sickle cell disease. J Pediatr Surg. 43(6):1052-6, 2008

Pulmonary Arteriovenous Fistula

Congenital pulmonary arteriovenous fistula (PAVF) is a rare vascular malformation where an

abnormal connection develops between an artery and a vein in the lung of a child. The

arteriovenous connection can be tortuous or direct aneurysmal and lacks an intervening capillary

bed. Females are more often affected than males. PAVF can be either large or small, multiple or

diffuse and unilateral or bilateral. The congenital variant of PAVF can be caused by a cavernous

hemangioma, while the acquired form occurs after heart surgery, trauma, actinomycosis,

amyloidosis, hepatic cirrhosis or cystic fibrosis. Symptoms include cyanosis, digital clubbing,

exertional dyspnea, hemoptysis and hemothorax. Most PAVF cases affect the lower lobes.

Diagnosis is made with angiography or 3-dimension spiral CT. Main indication for surgery is

hypoxemia and prevention of neurological sequelae due to embolization. Management can entail

segmentectomy, lobectomy, pneumonectomy, or recently the use of embolization with metal coils or

silicon balloons. Excision has good results when the PAVF is single, large and located in one lobe.

References:1- Sheikhzadeh A, Paydar MH, Ghabussi P, Hashemian M, Yazdanyar A, Hakim HS: Pulmonary arteriovenous fistulas. Case

presentations and clinical recognition. Herz. 8(3):179-86, 19832- Batinica S, Gagro A, Bradic I, Marinovic B: Congenital pulmonary arteriovenous fistula: a rare cause of cyanosis in

childhood. Thorac Cardiovasc Surg. 39(2):105-6, 1991

3- Fiane AE, Stake G, Lindberg HL: Congenital pulmonary arteriovenous fistula. Eur J Cardiothorac Surg. 9(3):166-8, 19954- Bernstein HS, Brook MM, Silverman NH, Bristow J: Development of pulmonary arteriovenous fistulae in children after

cavopulmonary shunt. Circulation. 92(9 Suppl):II309-14, 19955- Fraga JC, Favero E, Contelli F, Canani F: Surgical treatment of congenital pulmonary arteriovenous fistula in children. J

Pediatr Surg. 43(7):1365-7, 2008

PSU Volume 31 No 05 NOVEMBER 2008


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Reperfusion Injury

Hypovolemic shock whether hemorrhagic (trauma) or septic is the leading cause of morbidity and

mortality in intensive care units. Reperfusion injury commences after physicians reoxygenate

tissue that has undergo ischemic insults caused by hypoxia. The problem starts in the endothelial

cells bed, since these cells are very sensitive to hypoxia and manifest damage by increasing

cellular volume, loss of cytoskeletal organization, loss of adherence to the basement membrane,

decreases in membrane fluidity and adherence of activated leukocytes. The location of thisendothelial damage occurs at the level of arterioles, capillaries and venules. Specifically this last

(venules) are the most common sites of inflammatory response due to reperfusion causing

leukocyte-endothelial cell adhesion, transendothelial migration, platelet-leukocyte aggregation and

enhanced oxidant production. Leukocytes use binding proteins to attach themselves to the

endothelium and promotes damages liberating oxidative substances. The consumer of oxygen in

the cell is the mitochondria.

There is an inability for the mitochondria to use oxygen during reperfusion leaving the cell in a

cytopathic hypoxia (inability to produce ATP via oxidative phosphorylation). Endothelium is also

damage in reperfusion injury due to oxidative radicals (superoxide and hydrogen peroxide). One ofthe most dramatic examples of reperfusion injury occurs during the development of necrotizing

enterocolitis of babies.

References:1- Langer JC, Sohal SS, Blennerhassett P: Mucosal permeability after subclinical intestinal ischemia-reperfusion injury: an

exploration of possible mechanisms. J Pediatr Surg. 30(4):568-72, 19952- Beuk RJ, oude Egbrink MG, Kurvers HA, Bonke HJ, Tangelder GJ, Heineman E: Ischemia/reperfusion injury in rat

mesenteric venules: red b lood cell velocity and leukocyte rolling. J Pediatr Surg. 31(4):512-5, 1996

3- Chan KL, Hui CW, Chan KW, Fung PC, Wo JY, Tipoe G, Tam PK: Revisiting ischemia and reperfus ion injury as apos sible caus e of necrotizing enterocolitis: Role of nitric oxide and superoxide dismutase. J Pediatr Surg. 37(6):828-34, 20024- Stallion A, Kou TD, Latifi SQ, Miller KA, Dahms BB, Dudgeon DL, Levine AD: Ischemia/reperfusion: a clinically relevant

model of intestinal injury yielding systemic inflammation. J Pediatr Surg. 40(3):470-7, 2005

5- Rushing GD, Britt LD: Reperfusion Injury after Hemorrhage. Ann Surg 247(6): 929-937, 2008

Mesenteric Vascular Occlusion

Mesenteric vascular occlusion causing infarction of the bowel is a very devastating disease rarely

seen in the pediatric age group. Most cases are caused by mesenteric venous thrombosis. In

children, mesenteric vascular thrombosis may occur both in idiopathic form or associated with a

predisposing disease. Predisposing diseases includes thrombotic disorders causing a

hypercoagulable state, cardiac diseases, diabetes mellitus, vasculitis (polyarteritis nodosa),

artificial surfaces, trauma and surgery. Major difficulty is in establishing a prompt diagnosis since

symptoms and signs mimic many disorders and bowel necrosis is already present when surgery is

performed. Abdominal pain, distension, rigidity and tenderness are usually present. Persistent

metabolic acidosis is a warning sign of bowel ischemia. Likewise imaging is nonspecific and can

present with air-fluid levels, pneumatosis intestinalis, portal vein air, and thickened bowel loops.

Selective SMA angiography is the most reliable diagnostic procedure in suspected cases andthrombolysis using urokinase or streptokinase is an alternative therapy. With failed patent arterial

or venous visualization laparotomy is the next step in management.


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References:1- Bognr M, Lb J, Dnes J: Mesenteric vascular occlusion in infants and children: report of two cases and review of the

literature. Acta Paediatr Acad Sci Hung. 17(3):199-206, 1976

2- Meacham PW, Dean RH: Chronic mesenteric ischemia in childhood and adolescence. J Vasc Surg. 2(6):878-85, 19853- Oguzkurt P, Senocak ME, Ciftci AO, Tanyel FC, Bykpamuku N: Mesenteric vascular occlus ion resulting in intes tinal

necrosis in children. J Pediatr Surg. 35(8):1161-4, 20004- Warshauer DM, Lee JK, Mauro MA, White GC 2nd: Superior mesenteric vein thrombosis with radiologically occult

cause: a retrospective study of 43 cases. AJR Am J Roentgenol. 177(4):837-41, 20015- Dahshan A, Donovan K: Isolated superior mesenteric artery thrombosis: a rare cause for recurrent abdominal pain in a

child. J Clin Gastroenterol. 34(5):554-6, 2002

Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is an autoimmune mediated necrotizing vasculitis affecting principally

medium and small sized arteries which become swollen and damaged from attacks by rogue

immune cells. PAN affects principally the skin and kidney, but almost every organ in the body is

involved. In both the glomeruli and blood vessels, endothelial injury and subendothelial fibrindeposition are the earliest detectable ultrastructural changes. Boys and girls seem to be equally

affected, with a peak at the age of ten years. Clinical symptoms vary depending on site of vascular

involvement. Clinically children manifest fever, abdominal pain, vomiting, diarrhoea, weight loss,

joint pains and skin rash. For surgeons, PAN can produce acute mesenteric vascular occlusion from

venous thrombosis causing bowel ischemia. Unfortunately severe gastrointestinal involvement in

PAN is usually fatal despite aggressive therapy. It can also produce acute cholecystitis. Cutaneous

PAN can appear in children and has a benign and chronic course. Skin biopsy will make the

diagnosis. The diagnosis of polyarteritis nodosa is difficult and often delayed. Management

consists of steroids (prednisone) and immunosuppression (cyclophosphamide).

References:1- D'Agat i V, Chander P, Nash M, Mancilla-Jimenez R: Idiopathic microscopic polyarteritis nodosa: ultrastructural

obs ervations on the renal vas cular and glomerular lesions . Am J Kidney Dis. 7(1):95-110, 19862- Gndogdu HZ, Kale G, Tanyel FC, Bykpamuku N, Hisnmez A: Intestinal perforation as an initial presentation of

polyarteritis nodosa in an 8-year-old boy. J Pediatr Surg. 28(4):632-4, 19933- Nez Giralda A, Espejo Ortega M, Ibez Rubio M, Torrelo Fernndez A, Gonzlez, Medierod I, Lpez Robledillo J:

Childhood cutaneous polyarteritis nodosa. An Esp Pediatr. 54(5):506-9, 20014- Crankson SJ, Oda O, Al-Zaben AA, Al Suwairi W, Makanjoula D: Intes tinal ischamemia in a child due to polyarteritis

nodosa: a case report. Trop Gastroenterol. 27(1):41-3, 20065- Kendirli T, Yksel S, Oral M, Unal N, Tulunay M, Dilek US, Yalnkaya F: Fatal polyarteritis nodosa with gastrointes tinal

involvement in a child. Pediatr Emerg Care. 22(12):810-2, 2006

PSU Volume 31 No 06 DECEMBER 2008

Neonatal Appendicitis

Appendicitis can occur in any age group including newborns. Neonatal appendicitis is very rare, hasa high perforation rate, morbidity and mortality. Most described cases occur in males (75%) which

are born prematurely. Some of the reasons babies don't get appendicitis is due to the broad conical

orifice of the appendix, the use of liquid diet, lack of fecalith and reduced lymphatic hyperplasia in


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the periappendiceal area in this age group. The diagnosis is delay in all cases due to the infrequent

nature and only made during the exploratory laparotomy. Several causes of neonatal appendicitis

are appraised. It is suspected is a form of localized necrotizing enterocolitis. This implicates some

form of vascular insufficiency associated with perinatal asphyxia, cardiac anomalies or low flow

states. Obstructive cecal distension associated with Hirschsprung's disease or meconium ileus

(Cystic Fibrosis) causes increased pressure at the base of the appendix leading to perforation.

Appendicitis can also be associated with an incarcerated inguinal hernia (Amyand's hernia). The

babies show abdominal distension, signs of pain through irritability, restlessness, sleep disturbance,fever, vomiting, feeding intolerance and abnormal radiographic findings. Management is usually

exploratory laparotomy with removal of the sick appendix, antibiotherapy and lavage of the

abdominal cavity.

References:1- Stiefel D, Stallmach T, Sacher P: Acute appendicitis in neonates: complication or morbus sui generis? Pediatr Surg Int.

14(1-2):122-3, 19982- Martins JL, Peterlini FL, Martins EC: Neonatal acute appendicitis: a strangulated appendix in an incarcerated inguinal

hernia. Pediatr Surg Int. 17(8):644-5, 20013- Efrati Y, Peer A, Klin B, Lotan G: Neonatal periappendicular abs cess--updated treatment. J Pediatr Surg. 38(2):e5, 20034- Karaman A, Cavusoglu YH, Karaman I, Cakmak O: Seven cases of neonatal appendicitis with a review of the English

language literature of the last century. Pediatr Surg Int. 19(11):707-9, 20035- Managoli S, Chaturvedi P, Vilhekar KY, Gupta D, Ghos h: Perforated acute appendicitis in a term neonate. Indian J Pediatr.

71(4):357-8, 2004

6- Jancelewicz T, Kim G, Miniati D: Neonatal appendicitis: a new look at an old zebra. J Pediatr Surg. 43(10):e1-5, 2008

Spinal Accessory Nerve Injury

The XI cranial nerve called the spinal accessory nerve provides motor innervation to two muscles

in the neck: the sternocleidomastoid and upper portion of trapezius muscle. Injury to the spinal

accessory nerve is usually iatrogenic and occurs most commonly after lymph node or other type of

biopsy in the posterior triangle of the neck in children and adults. The paralysis of the trapezius

disrupts the scapohumeral synchrony manifesting itself clinically as loss of shoulder motion, wing

scapula, pain and a functional deficit. In adults the injury is usually recognized in the early

postoperative period by shoulder pain and active shoulder motion dysfunction, while children have

a later clinical presentation. To avoid damage during surgery use of loupe magnification is needed.

Instead of a transverse incision, which is more pleasing cosmetically, a parallel incision along the

posterior border of the sternocleidomastoid is safer. Because the nerve is adhered to the lymph

node confirmation using nerve stimulator is needed. After the procedure the child should be

examined to determined the integrity of the nerve in scapulothoracic and glenohumeral motion.

With suspicion of nerve injury electrodiagnostic studies should be done. Surgical options after

injury includes neurolysis or neuroma resection with primary microsurgical repair or with nerve

graft reconstruction. Significant recovery is obtained after surgery.

References:1- Okajima S, Tamai K, Fujiwara H, Kobashi H, Hirata M, Kubo T: Surgical treatment for spinal acces sory nerve injury.

Microsurgery. 26(4):273-7, 2006

2- Bostrm D, Dahlin LB: Iatrogenic injury to the access ory nerve. Scand J Plast Reconstr Surg Hand Surg. 41(2):82-7, 2007

3- Lloyd S: Access ory nerve: anatomy and s urgical identification. J Laryngol Otol. 121(12):1118-25, 2007


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4- Kelley MJ, Kane TE, Leggin BG: Spinal access ory nerve palsy: associated signs and symptoms. J Orthop Sports Phys

Ther. 38(2):78-86, 20085- Grossman JA, Ruchelsman DE, Schwarzkopf R: Iatrogenic sp inal accessory nerve injury in children. J Pediatr Surg.

43(9):1732-5, 2008

Posttraumatic Stress Disorder

Posttraumatic stress disorder (PTSD) is a constellation of symptoms associated with re-experience

such as denial, avoidance and arousal after going through a life-threatening event. PTSD can affect

children, parents and family members after a surgical experience. Several studies have

demonstrated that symptoms of post-traumatic stress can be seen in young children undergoing

bone marrow transplantation up to one year after transplant. Burn children with PTSD reported an

impaired overall health related quality of life and limited physical (e.g., more bodily complaints) and

emotional functioning (e.g., more feelings of sadness). High levels of posttraumatic stress disorder

symptoms are common in the recovery period after pediatric orthopaedic trauma, even among

patients with relatively minor injuries. Children admitted to the hospital after injuries are at higher

risk for such symptoms. Parents of children undergoing cardiopulmonary bypass surgery are at

increased risk for intermediate and long-term psychological malfunctioning. Acute symptoms of

PTSD in parents shortly after discharge of their child are a major risk factor for the development

of chronic PTSD. In this era of prenatal ultrasound confirmation of surgical diagnosis is imperative

that pediatric surgeons gather with affected parents and explain before embarking in the surgical

care of their child.

References:

1- Stuber ML, Nader K, Yasuda P, Pynoos RS, Cohen S: Stress responses after pediatric bone marrow transplantation:preliminary results of a prospective longitudinal study. J Am Acad Child Adolesc Psychiatry. 30(6):952-7, 19912- Landolt MA, Buehlmann C, Maag T, Schiestl C: Brief Report: Quality of Life Is Impaired in Pediatric Burn Survivors with

Posttraumatic Stress Disorder. J Pediatr Psychol. Sep 21, 2007.3- Sanders MB, Starr AJ, Frawley WH, McNulty MJ, Niacaris TR: Posttraumatic stress symptoms in children recovering

from minor orthopaedic injury and treatment. J Orthop Trauma. 19(9):623-8, 20054- Helfricht S, Latal B, Fischer JE, Tomaske M, Landolt MA: Surgery-related posttraumatic stress disorder in parents of

children undergoing cardiopulmonary bypass surgery: a prospective cohort study. Pediatr Crit Care Med. 9(2):217-23, 20085- Nagata S, Funakosi S, Amae S, Yoshida S, Ambo H, Kudo A, Yokota A, Ueno T, Matsuoka H, Hayas hi Y: Posttraumatic

stress disorder in mothers of children who have undergone surgery for congenital disease at a pediatric surgery

department. J Pediatr Surg. 43(8):1480-6, 2008

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