Pediatric Surgery Update Volume 38, 2012

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6/29/12 PEDIATRIC SURGERY UPDATE Volume 38, 2012

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PEDIATRIC SURGERY UPDATE

VOLUME 38, 2012

PSU Volume 38 No 01 JANUARY 2012

Dopamine-producing Pheochromocytoma

Pheochromocytomas are neuroendocrine tumors that produce, metabolize and secrete catecholamines such as

norepinephrine and epinephrine. In very rare occasions hormonally active pheochromocytomas can produce and secrete

only dopamine as the only catecholamine. Predominance of dopamine in this tumors and lack of production of other

catecholamines is due to deficiency in tumor cells of dopamine-B-hydroxylase, the enzyme that converts dopamine into

norepinephrine. Dopamine-producing pheochromocytomas are usually found as extraadrenal paragangliomas. Clinically

patients harboring these tumors are normotensive. Most patients are diagnosed after identifying the adrenal or

extraadrenal position of the tumor during incidental imaging or as the result of symptoms caused by the space-occupying

tumor. Due to lack of hormonally associated symptoms these tumors can grow into large s ize. Tumors that produce

dopamine can be identified by high plasma or urine concentrations of dopamine, or high plasma levels of free

methoxytyramine. The incidence of malignancy is higher in this type of pheochromocytoma. Nausea, vomiting, flushing

and orthostatic hypotension are a few of the symptoms exhibited by these patients . MRI is the imaging of choice to localize

these tumors. Management consists of resection of the tumor. Persistent elevation of blood pressure after tumor

res ection have been documented. PET-Scan is more specific than MIBG in localizing res idual disease.

References:

1- Minamiguchi N, Inui E, Nukui M: [A case of dopamine-secreting pheochromocytoma]. Hinyokika Kiyo. 45(12):831-3, 19992- Yasunari K, Kohno M, Minami M, Kano H, Ohhira M, Nakamura K, Yoshikawa J: A dopamine-secreting pheochromocyt oma. J CardiovascPharmacol. 36 Suppl 2:S75-7, 20003- Awada SH, Grisham A, Woods SE: Large dopamine-secreting pheochromocyt oma: case report . South Med J. 96(9):914-7, 20034- Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai EW, Adams KT, Pacak K: Biochemical and clinical manifestations ofdopamine-producing paragangliomas: utility of plasma methoxyt yramine. J Clin Endocrinol Metab. 90(4 ):2068-75, 20 055- Tam V, Ng KF, Fung LM, Wong YY, Chan MH, Lam CW, Tam S, Lam CW: The importance of the interpretation of urinecatecholamines is essential fo r t he diagnosis and management of patient with dopamine-secreting paraganglioma. Ann Clin Biochem. 42(Pt1):73-7, 20056- Foo SH, Chan SP, Ananda V, Rajasingam V: Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features andmanagement. Singapore Med J. 51(5):e89-93, 20107- Dubois LA, Gray DK: Dopamine-secreting pheochromocyt omas: in search of a syndrome. Wo rld J Surg. 29(7 ):909-13, 200 5

Laparoscopy for Ventriculoperitoneal Shunts

Ventriculoperitoneal (VP) shunt is the standard treatment for hydrocephalus in children and adults. Malfunctioning VP

shunts causing increase intracranial pressure needs surgical revision. Such malfunctioning can occur due to

obstruction from peritoneal adhesion, multiple infections, ascites and pseudocysts formation, or mechanical causes such

as catheter fracture, disconnection, migration and misplacement. VP shunt failure is common with 25-40% occurring

within the first year, and 50% by the second year. Laparoscopy has an important role in initial VP s hunt placement and

later revisions. Laparoscopy reduce the trauma to the abdominal wall decreasing adhesion formation and optimizing

visualization during placement. With VP shunt failure laparoscopy can be both diagnostic and therapeutic. While

visualizing the entire abdominal cavity the causes of failure can be identified, lysis of adhesions can be performed,

retrieval of disconnected shunt can be accomplished, flow of CSF fluid can be observed and proper placement of the

catheter can be obtained The technique is safe with a very low morbidity rate. The use of laparoscopy ass is ted VP shunt

revision is advocated for patients with multiple previous s hunt revisions, prior abdominal surgery, previous

intraperitoneal infections, broken devices, obesity or CSF pseudocysts.

References:

1- Khosrovi H, Kaufman HH, Hrabovsky E, Bloomfield SM, Prabhu V, el-Kadi HA: Laparoscopic-assisted distal ventriculoperitoneal shunt


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placement . Surg Neurol. 49(2):127-34, 19982- Jackson CC, Chwals WJ, Frim DM: A single-incision laparoscopic t echnique for retr ieval and replacement of disconnectedventriculoperitoneal shunt tubing found in the peritoneum.Pediatr Neurosurg. 36(4):175-7, 20023- Kirshtein B, Benifla M, Roy-Shapira A, Merkin V, Melamed I, Cohen Z, Cohen A: Laparoscopically guided distal vent riculoperiton ealshunt placement. Surg Laparosc Endosc Percutan Tech. 14(5):276-8, 20044- Bani A, Hassler WE: Laparoscopy-guided insertion of perit oneal cathet ers in vent riculoperiton ealshunt procedures: analysis of 39 children. Pediatr Neurosurg. 42(3):156-8, 20065- Yu S, Bensard DD, Partr ick DA, Pett y JK, Karrer FM, Hendrickson RJ: Laparoscopic guidance or revision of ventr iculoperiton eal shunts inchildren.JSLS. 10(1):122-5, 20066- Johnson BW, Pimpalwar A: Laparoscopic-assisted placement of vent riculo-peritoneal shunt tips in children with multiple prev ious open

abdominal vent riculo-peritoneal shunt surgeries. Eur J Pediatr Surg. 19(2):79-82 , 20097- Martin K, Baird R, Farmer JP, et al: The use of laparoscopy in ventriculoperitoneal shunt revisions. J Pediatr Surg. 46(11): 2146-2150,2011

Magnet-assisted Laparoscopic Surgery

Magnets have been introduced in the armamentarium of laparoscopic surgery to recapture the triangulation that is

affordable by conventional laparoscopy while decreasing the number and size of the abdominal incis ions utilized.

Specialized magnetic grasper are inserted into the peritoneal cavity through the port cannula and attached to

intraabdominal organs. These magnets are controlled by another external magnet placed on top of the abdominal wall. The

magnet grasper moves to provide further traction on an organ without the additional need of another port. With the

magnet you can retract the liver, s tomach, lung tissue, gallbladder providing traction and facilitating exposure. Magnet-

ass isted laparoscopy is safe and effective means of reducing the number and size of abdominal incis ions while improving

exposure, triangulation, and the ergonomics of the procedure.

References:

1- Rothenberg SS, Shipman K, Yoder S: Experience with modified single-port laparoscopic procedures in children. J Laparoendosc Adv SurgTech A. 19(5):695-8, 20092- Garey CL, Laituri CA, Ostlie DJ, Snyder CL, Andrews WS, Holcomb GW 3rd, St Peter SD: Single-incision laparoscopic surgery in children:initial single-center experience. J Pediatr Surg. 46(5):904-7, 20113- de Armas IA, Garcia I, P impalwar A: Laparoscopic single port surgery in children using Triport : our early experience. P ediatr Surg Int.27(9):985-9, 20114- Dutt a S: Early experience with single incision laparoscopic surgery: eliminating the scar from abdominal operations. J Pediatr Surg.

44(9):1741-5, 20095- Padilla BE, Dominguez G, Millan C, Martinez-Ferro M: The use of magnets with single-site umbilical laparoscopic surgery. Semin PediatrSurg. 20(4):224-31, 2011

PSU Vulome 38 NO 02 FEBRUARY 2012

Middle Aortic Syndrome

Middle aortic syndrome (MAS) is a rare condition involving diffuse narrowing of the distal thoracic

or abdominal aorta involving its visceral and renal branches. The etiology of MAS is unknown and

occurs in young patients. MAS have been associated with neurofibromatosis, Alagille's and

William syndrome. The mesenteric vascular stenosis is clinically silent, while the renal stenosis is

responsible for the clinical picture of hypertension in these children. Absent femoral pulses and an

abdominal bruit are also present. Bilateral involvement is common. Ultrasonography is the primary

screening technique. Biplanar arteriography is the standard imaging to establish the diagnosis and

involvement of the disease. Arteriography shows a smooth segmental narrowing of the abdominal

aorta with concomitant stenosis at the origins of the renal arteries. Primary treatment goals are

preservation of functional renal tissue and amelioration of the renal hypertension. Therapeutic

approaches included medical management, percutaneous transluminal angioplasty and/or surgical

intervention. Repair of the narrowing requires prosthetic material for bypass or patch


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reconstruction. Techniques used to revascularize the kidney included thoracoabdominal to

infrarenal aortic bypass with renal artery reimplantation, splenorenal bypass, gastroduodenal to

renal bypass, aortorenal bypass and autotransplantation.

References:

1- Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB, Taylor PR: Mid-aortic s yndrome presenting inchildhood. Br J Surg. 83(2):235-40, 19962- Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, Dillon MJ,

Tullus K: Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol. 24(11):2225-32, 20093- Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC: Mid-aortic syndrome: a case report and review of the literature. Int JCardiol. 123(3):348-52, 20084- Chocrn de Benzaquen S, Munoz Lopez M, Madrid Aris AD, Castellote Alonso A, Enriquez G, Nieto Rey JL: [Mid-aortic syndrome: case s tudies from a paediatric nephrology department]. An Pediatr (Barc). 75(1):33-9, 20115- Bleacher J, Turner ME, Quivers E, Schwartz MZ: Renal autotransplantation for renovascular hypertension caused bymidaortic syndrome. J Pediatr Surg. 32(2):248-50, 19976- Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE: Idiopathic mid-aortic syndrome in children. Pediatr

Nephrol. 23(7):1135-42, 2008

Ciliated Hepatic Foregut Cyst

Ciliated Hepatic Foregut Cyst (CHFC) is a rare cystic benign disease of the liver increasingly

diagnosed. Most patients with CHFC are asymptomatic at the time of diagnosis. The most

common symptom is right upper quadrant pain. In almost all cases the cyst is located within the left

liver segment IV or in close proximity. Average size of the cyst is 3.6 cm with most cyst unilocular,

avascular and lying beneath the anterior surface of the liver. Cyst content is viscous or mucinous.

It is the presence of shared histological features in the form of pseudostratified ciliated columnar

epithelium that leads to label these cysts of foregut origin. The origin of CHFC is detachment and

migration of foregut with subsequent entrapment in the liver. Infantile presentation is in the form ofan abdominal mass due to the larger size. In infants the cyst may have bile duct communication and

can develop squamous metaplasia, reason why excision and ligation of the biliary communication

should be preferred to internal drainage. CT will show a complex hypodense cystic mass without

enhancement. FNA cytology will show the ciliated pseudostratified columnar cells and establish the

diagnosis. The presence of a tough fibrous outer layer in the cyst makes it amenable to

mobilization and enucleation without spillage. This can be accomplished by laparoscopy or open

surgery.

References:

1- Kim S, White FV, McAlister W, Shepherd R, Mychaliska G: Ciliated hepatic foregut cys t in a young child. J Pediatr Surg.40(11):e51-3, 20052- Stringer MD, Jones MO, Woodley H, Wyatt J: Ciliated hepatic foregut cys t. J Pediatr Surg. 41(6):1180-3, 20063- Betalli P, Gobbi D, Talenti E, Alaggio R, Gamba P, Zanon GF: Ciliated hepatic foregut cys t: from antenatal diagnosis tosurgery.Pediatr Radiol. 38(2):230-2, 20084- Sharma S, Dean AG, Corn A, Kohli V, Wright HI, Sebastian A, Jabbour N: Ciliated hepatic foregut cys t: an increas inglydiagnosed condition.Hepatobiliary Pancreat Dis Int. 7(6):581-9, 20085- Goodman MD, Mak GZ, Reynolds JP, Tevar AD, Pritts TA: Laparoscopic excision of a ciliated hepatic foregut cys t.JSLS.13(1):96-100, 20096- Zaydfudim V, Rosen MJ, Gillis LA, Correa H, Lovvorn HN 3rd, Pinson CW, Kelly BS Jr: Ciliated hepatic foregut cys ts inchildren. Pediatr Surg Int. 26(7):753-7, 2010

7- Guerin F, Hadhri R, Fabre M, Pariente D, Fouquet V, Martelli H, Gauthier F, Branchereau S: Prenatal and pos tnatal CiliatedHepatic Foregut Cysts in infants . J Pediatr Surg. 45(3):E9-14, 2010

ALTE


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An apparent life-threatening event (ALTE) is defined as an episode that is frightening to the

caretaker and is characterized by some combination of apnea (which can be central or occasionally

obstructive), color change (usually cyanotic or pallid but occasionally erythematous or plethoric),

marked change in muscle tone (usually limpness), choking or gagging. Although the natural history

of ALTE is most often benign, there is a risk for subsequent morbidity and mortality. The most

common causes of ALTE are gastroesophageal reflux, seizure activity and lower respiratory tract

infections, specially respiratory syncytial virus infection. Other causes include otolaryngology

conditions, inborn errors in metabolism and cardiac diseases. The incidence of sudden infant deathsyndrome is reported to be higher after ALTE episodes. Tow mechanisms explain why reflux can

result in ALTE: chemoreflex in the larynx prompted by acid fluid and stimulation of the distal

esophagus by the gastric content. Children with ALTE and gastroesophageal reflux, other cardiac,

respiratory and neurological causes appropriately excluded should be considered candidates for

fundoplication since medical management carries a high risk of recurrent ALTE. After

fundoplication the incidence of ALTE is significantly reduced.

References:

1- National Institutes of Health Consensus Development Conference on Infantile Apnea and Home Monitoring. Pediatrics

79:292- 299, 19872- McGovern MC, Smith MB: Causes of apparent life threatening events in infants: a systematic review. Arch Dis Child.89(11):1043-8, 20043- Dewolfe CC: Apparent life-threatening event: a review. Pediatr Clin North Am. 52(4):1127-46, 20054- Esani N, Hodgman JE, Ehsani N, Hoppenbrouwers T: Apparent life-threatening events and s udden infant deathsyndrome: comparison of risk factors. J Pediatr. 152(3):365-70, 20085- Valusek PA, St Peter SD, Tsao K, et al. The use of fundoplication for prevention of apparent life-threatening events . JPediatr Surg 42:1022-1024, 20076- Tirosh E, Ariov-Antebi N, Cohen A: Autonomic function, gastroesophageal reflux in apparent life threatening event. ClinAuton Res. 20(3):161-6, 2010

PSU Volume 38 No 03 MARCH 2012

Bile Duct Bifurcation

Duplication, or more appropriately phrase bifurcation of the bile ducts is an anomaly associated to

duodenal atresia. This anomaly can lead to find gas in the distal bowel of a duodenal atresia. The

bifurcated bile duct communicates between the proximal and distal atetric segments bypassing gas

between them. A bifid biliary system inserts at blind upper and lower pouches of the duodenum, andthe common bile duct inserts in a Y fashion. The incidence of duodenal atresia with an anomalous

bifurcated bile duct conduit is higher than is thought and occurs more frequently than that

associated with duodenal stenosis. Down syndrome is highly associated with cases of an anomalous

bifurcated bile duct conduit. Contrast studies are generally not performed in the typical clinical and

radiographic evaluation of duodenal atresia; however, an upper gastrointestinal series can

demonstrate the anomalous bile duct bifurcation or duplication. The presence of such anomaly can

predispose the patient to cholestasis and cholangitis due to duodeno-biliary reflux. The

management of duodenal atresia consists of duodenoduodenostomy. Care should be taken at

surgery to avoid obstruction or injury to the anomalous bifurcated bile duct, and operative

cholangiography may be useful to document continued bile duct patency following repair of the

atresia.

References:


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1- Knechtle SJ, Filston HC: Anomalous biliary ducts associated with duodenal atresia. J Pediatr Surg. 25(12):1266-9, 19902- Panuel M, Bourliere-Najean B, Delarue A, Viard L, Faure F, Devred P: [Duodenal atresia with bifid termination of thecommon bile duct]. Arch Fr Pediatr. 49(4):365-7, 19923- Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y: Embryogenesis of pancreaticobiliary maljunction inferred fromdevelopment of duodenal atresia. J Hepatobiliary Pancreat Surg. 6(1):50-4, 19994- Tashjian DB, Moriarty KP: Duodenal atresia with an anomalous common bile duct masquerading as a midgut volvulus. JPediatr Surg. 36(6):956-7, 20015- Mali V, Wagener S, Sharif K, Millar AJ: Foregut atresias and bile duct anomalies: rare, infrequent or common? PediatrSurg Int. 23(9):889-95, 2007

6- Komuro H, Ono K, Hoshino N, Urita Y, Gotoh C, Fujishiro J, Shinkai T, Ikebukuro K: Bile duct duplication as a cause ofdistal bowel gas in neonatal duodenal obstruction. J Pediatr Surg. 46(12):2301-4, 2011

Microvillous Inclusion Disease

Microvillous Inclusion Disease (MVID) or microvillous atrophy is a very rare congenital disorder

of the intestinal epithelial cells that present with persistent life-threatening watery diarrhea and is

characterized by morphological enterocyte abnormalities. Onset of diarrhea most often occurs

within the first days of life (early) or in the first two months of life (late). MVID is very rare and

transmitted as an autosomal recess ive trait. Patients can lose up to 30% of body weight. Small

bowel biopsy is diagnostic revealing villous atrophy without significant crypt hyperplasia. Periodic

acid-Schiff positive granules accumulate in the apical cytoplasm of immature intestinal epithelial

cells. Children with MVID are totally dependent on parenteral nutrition. Long-term outcome is

generally poor, due to metabolic decompensation, repeated states of dehydration, infectious and

liver complications related to the parenteral nutrition. Relentlessly the child develops intestinal

failure secondary to the diarrhea. There is no specific treatment for MVID other than hydration

and parenteral nutrition. Isolated intestinal transplantation or combined liversmall bowel

transplantation is a last treatment option when significant liver disease exists. Replacement of the

conventional soybean oilbased lipid emulsions with an omega-3rich product have been found to

rapidly reverse parenteral nutrition-induced cholestasis in patients with MVID.

References:

1- Wilson W, Scott RB, Pinto A, Robertson MA: Intractable diarrhea in a newborn infant: microvillous inclusion disease.Can J Gastroenterol. 15(1):61-4, 20012- Ruemmele FM, Schmitz J, Goulet O: Microvillous inclusion disease (microvillous atrophy). Orphanet J Rare Dis. 26;1:22,20063- Iancu TC, Mahajnah M, Manov I, Shaoul R: Microvillous inclusion disease: ultrastructural variability. Ultrastruct Pathol.31(3):173-88, 20074- Halac U, Lacaille F, Joly F, Hugot JP, Talbotec C, Colomb V, Ruemmele FM, Goulet O: Microvillous inclusion disease:

how to improve the prognosis of a severe congenital enterocyte disorder. J Pediatr Gastroenterol Nutr. 52(4):460-5, 20115- Fuchs J, Fallon EM, Gura KM, Puder M: Use of an omega-3 fatty acid-based emulsion in the treatment of parenteralnutrition-induced cholestas is in patients with microvillous inclusion disease. J Pediatr Surg. 46(12):2376-82, 2011

Endoluminal Intestinal Lengthening

Endoluminal intestinal lengthening is an option still in experimental phase for intestinal lengthening

in cases of short bowel syndrome. Mechanical force is a viable method for increasing intestinal

length while preserving the intestinal function. Distraction enterogenesis or the application of

forces to the small bowel has been shown to increase length through the induction of cellular

proliferation. Some designs previously used for such purposes consist of screws, hydraulic pistons

and remotely controlled ratcheting devices. Experimental studies have demonstrated the feasibility

of using a polymer-coated spring capsule intraluminally placed in a piece of intestine of


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experimental animals for timed deployment of an expanding device for bowel Lengthening. The

restored jejunal segment had an increase in crypt depth and no difference in villus height compared

with normal jejunum. Sucrase activity in the restored segment was not different from that in

normal jejunum. Using these methods the small bowel can be lengthened three to fourfold times its

original length. Mechanical lengthening may be a useful technique to increase intestinal length in

patients with short bowel syndrome.

References:1- Shekherdimian S, Scott A, Chan A, Dunn JC: Intestinal lengthening in rats after massive small intestinal resection.Surgery. 146(2):291-5, 20092- Park J, Puapong DP, Wu BM, Atkinson JB, Dunn JC: Enterogenesis by mechanical lengthening: morphology andfunction of the lengthened small intestine. J Pediatr Surg. 39(12):1823-7, 20043- Chang PC, Mendoza J, Park J, Lam MM, Wu B, Atkinson JB, Dunn JC: Sustainability of mechanically lengthened bowelin rats . J Pediatr Surg. 41(12):2019-22, 20064- Stark R, Zupekan T, Bondada S, Dunn JC: Restoration of mechanically lengthened jejunum into intestinal continuity inrats . J Pediatr Surg. 46(12):2321-6, 20115- Stark R, Panduranga M, Carman G, Dunn JC: Development of an endoluminal intestinal lengthening capsule. J PediatrSurg. 47(1):136-41, 2012

PSU Volume 38 NO 04 APRIL 2012

Ovarian Transposition

Ionizing radiation used in adjuvant management of malignancy can have an adverse effect on

gonadal function at all ages. The ovaries are exposed to significant doses of irradiation when

radiotherapy is used to managed pelvic and abdominal malignant diseases such as Hodgkin's

lymphoma, cervical and rectal cancer to children before childbearing age. Half of the follicles arelost when a dose of 4 Gy is used. The risk of premature ovarian failure increases significantly with

increasing doses of abdominal pelvic irradiation. Ovarian transposition, or repositioning the ovaries

out of the irradiation field can preserve ovarian function before pelvic irradiation. Ovarian

transposition can be done laparoscopically prior to pelvic irradiation. The procedure can reduce

damage caused by radiotherapy but does not protect against damage caused by systemic

chemotherapy. The laparoscopic procedure for ovarian transposition is highly efficient, can be done

as outpatient and is associated with few postoperative complications. Proper location to fix the

transposed ovaries depends on the planned irradiation field. For cervical cancer the ovaries are

transposed high and lateral above the pelvic rim, while for pelvic lymph node irradiation they areplaced medially or preferably laterally. Surgical complications reported includes injury to the

ovarian vasculature, fallopian tube infarction and ovarian cyst formation.

References:

1- Hays DM, Fryer CJ, Pringle KC, Collins RD, Hutchinson RJ, O'Neill JA, Constine LS, Heller RM, Davis PC, Nachman J, etal: An evaluation of abdominal staging procedures performed in pediatric patients with advanced Hodgkin's d isease: areport from the Childrens Cancer Study Group.J Pediatr Surg. 27(9):1175-80. 19922- Hloury Y, Guiberteau V, Sagot P, Plattner V, Baron M, Rogez JM: Laparoscopy in adnexal pathology in the child: astudy of 28 cases. Eur J Pediatr Surg. 3(2):75-8, 19933- Williams RS, Littell RD, Mendenhall NP: Laparoscopic oophoropexy and ovarian function in the treatment of Hodgkin

diseas e. Cancer. 86(10):2138-42, 19994- Meirow D, Nugent D: The effects of radiotherapy and chemotherapy on female reproduction. Hum Reprod Update.7(6):535-43, 20015- Terenziani M, Piva L, Meazza C, Gandola L, Cefalo G, Merola M: Oophoropexy: a relevant role in preservation of ovarianfunction after pelvic irradiation. Fertil Steril. 91(3):935.e15-6, 2009


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6- Han SS, Kim YH, Lee SH, Kim GJ, Kim HJ, Kim JW, Park NH, Song YS, Kang SB: Underuse of ovarian transposition inreproductive-aged cancer patients treated by primary or adjuvant pelvic irradiation. J Obstet Gynaecol Res. 37(7):825-9, 20117- Morris SN, Ryley D: Fertility preservation: nonsurgical and surgical options . Semin Reprod Med. 29(2):147-54, 2011

Adrenal Cysts

Adrenal cysts are rare, usually found incidentally during autopsy series. Adrenal cysts are usually

asymptomatic; mostly an occasional discovery during ultrasound or CT done for other reason.

Acute abdominal or flank pain may be presented in some cases. From the histological point of view

adrenal cysts are vascular or endothelial, hemorrhagic or pseudocyst and epithelial-lined or "true"

adrenal cysts. Less than 10% of adrenal cysts are malignant. The most common types are

epithelial and pseudocysts. Intra-cystic hemorrhage spontaneously or post-traumatic may be

present. Due to the asymptomatic nature of the cyst they can attain large sizes usually in the range

of 10 cm when diagnosed. Preoperative CT-guided aspiration for cytology and biopsy are useful

technique to establish a diagnosis. Resection of the cyst should be performed for cysts larger than

3 cm, symptomatic, suspicion of malignancy, hormonally active or rapidly enlarging. Either

laparoscopic or open are standard methods to remove the cyst along with the adrenal glandassociated. Laparoscopic adrenalectomy or cyst unroofing is a safe and effective treatment for

benign adrenal cysts with the advantages of a shorter hospital stay, less blood loss and enhanced

cosmesis.

References:

1- El-Hefnawy AS, El Garba M, Osman Y, Eraky I, El Mekresh M, Ibrahim el-H: Surgical management of adrenal cysts:single-institution experience. BJU Int. 104(6):847-50, 20092- Poiana C, Carsote M, Chirita C, Terzea D, Paun S, Beuran M: Giant adrenal cyst: case study. J Med Life. 3(3):308-13, 20103- Guazzoni G, Montorsi F, Rigatti P, Lanzi R, Pontiroli AE, Silves tre P, Breda G: Laparoscopic unroofing of adrenal cysts .

Eur Urol. 31(4):499-502, 19974- Pradeep PV, Mishra AK, Aggarwal V, Bhargav PR, Gupta SK, Agarwal A: Adrenal cysts: an institutional experience.World J Surg. 30(10):1817-20, 20065- Lal TG, Kaulback KR, Bombonati A, Palazzo JP, Jeffrey RB, Weigel RJ: Surgical management of adrenal cysts. Am Surg.69(9):812-4, 20036- Castillo OA, Litvak JP, Kerkebe M, Urena RD: Laparoscopic management of symptomatic and large adrenal cys ts. J Urol.173(3):915-7, 2005

Measuring IAP

The abdominal compartment syndrome (ACS) is a clinical syndrome caused by persistencyelevated intraabdominal pressure (IAP) leading to decreased venous return and cardiac output,

increase intracranial pressure, impaired ventilation, and kidney and bowel end-organ damage. The

most common etiologies of ACS in children are isolated head trauma, abdominal trauma, surgery

for abdominal wall defect, bowel ischemia/necrosis and meningococcemia. The most accurate

method for measurement of IAP is directly via an intraperitoneal catheter. Due to invasiveness,

the indirect method of measuring IAP using the intravesical method has prevailed as the gold

standard. The bladder technique requires that the bladder be infused with a certain amount of

saline, to ensure that there is a conductive fluid column between the bladder and the transducer.

The method uses a Foley catheter inserted into the bladder while the bladder is filled with saline.The closest correlation with intraabdominal pressure occurs when a volume of 1 ml/kg of weight is

utilized in children. The higher the bladder filling volume, the higher the overestimation of IAP.

Optimal patient position for IAP measurement is supine, taken at end-expiration with the

transducer calibrated to the level of the mid-axillary line. Infusion of saline at room temperature


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causes higher bladder pressure due to contraction of the detrusor bladder muscle. Mean IAP in

critically ill children is 7 +/- 3 mm Hg. Above 12 mm Hg IAP is elevated.

References:

1- Chiumello D, Tallarini F, Chierichetti M, Polli F, Li Bassi G, Motta G, Azzari S, Carsenzola C, Gattinoni L: The effect ofdifferent volumes and temperatures of saline on the bladder pressure measurement in critically ill patients. Crit Care.11(4):R82, 20072- Gudmundsson FF, Viste A, Gislason H, Svanes K: Comparison of different methods for measuring intra-abdominal

pressure. Intensive Care Med. 28(4):509-14, 20023- Zengerink I, McBeth PB, Zygun DA, Ranson K, Ball CG, Laupland KB, Widder S, Kirkpatrick AW: Validation andexperience with a simple continuous intra-abdominal pressure measurement technique in a multidisciplinarymedical/surgical critical care unit. J Trauma. 64(5):1159-64, 20084- Balogh Z, Jones F, D'Amours S, Parr M, Sugrue M: Continuous intra-abdominal pressure measurement technique. Am JSurg. 188(6):679-84, 20045- Suominen PK, Pakarinen MP, Rautiainen P, Mattila I, Sairanen H: Comparison of direct and intravesical measurement ofintraabdominal pressure in children. J Pediatr Surg. 41(8):1381-5, 20066- Ejike JC, Bahjri K, Mathur M: What is the normal intra-abdominal press ure in critically ill children and how should wemeasure it? Crit Care Med. 36(7):2157-62, 20087- Davis PJ, Koottayi S, Taylor A, Butt WW: Comparison of indirect methods of measuring intra-abdominal pressure inchildren. Intens ive Care Med. 31(3):471-5, 2005

PSU Volume 38 NO 05 MAY 2012

Extravasation Injury

Extravasation injury (EI) during intravenous therapy is a significant source of morbidity, mortalityand liability in children and adults. The spectrum of injury goes from mild irritation without

inflammation to tissue necrosis depending if the agent extravasated is a vesicant, irritant or flare

producing. Vesicants can produce tissue necrosis by absorption from local tissue and DNA binding.

They should be administered by central vein route. Patients at increased risk of EI include the very

young or old with impaired venous circulation and lymphatic drainage, and the critically ill child.

Device related factors include metal needles, large gauge catheters, poorly secured IV cannulas,

IV placed in antecubital fossa, dorsum of hand or near joint area, and catheter dysfunction by

separation, breakage or dislodgement. Patients should be informed of risk of EI and encourage to

notify the nurse of any change in sensation, swelling, leakage, pain, machine flow malfunction or

burning. Management consists of immediate discontinuance of infusion, aspirate residual

extravasated fluid and removal of the peripheral catheter. Affected extremity should be elevated.

Avoidance of the extremity for future cannulation or blood pressure recording. Prompt consultation

with the wound nurse should follow. Serial photographs are useful to monitor wound progress.

Conservative local care is given and plastic and physical therapy are consulted as needed.

References:

1- Garland JS, Dunne WM Jr, Havens P, Hintermeyer M, Bozzette MA, Wincek J, Bromberger T, Seavers M: Peripheralintravenous catheter complications in critically ill children: a prospect ive study. Pediatrics. 89(6 Pt 2):1145-50, 19922- Gault DT: Extravasation injuries. Br J Plast Surg. 46(2):91-6, 1993

3- Kumar RJ, Pegg SP, Kimble RM: Management of extravasation injuries. ANZ J Surg. 71(5):285-9, 20014- Tong R: Preventing extravasation injuries in neonates. Paediatr Nurs . 19(8):22-5, 20075- Rose RE, Felix R, Crawford-Sykes A, Venugopal R, Wharfe G, Arscott G: Extravasation injuries. West Indian Med J.57(1):40-7, 20086- Amjad I, Murphy T, Nylander-Housholder L, Ranft A: A new approach to management of intravenous infiltration in


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pediatric patients: pathophysiology, class ification, and treatment. J Infus Nurs. 34(4):242-9, 2011

Hrthle Cell Neoplasms

Oncocytic or Hrthle cell adenoma and carcinoma represent less than 5% of all thyroid neoplasms

in children and adults. The distinction between Hrthle cell adenoma and carcinoma is

histologically determined by the presence of vascular or capsular invasion in the latter. Hrthlecells are large polygonal eosinophilic cells with pleomorphic hyperchromatic nuclei and fine

granular cytoplasm containing an abundance of mitochondria commonly associated with Hashimoto

thyroiditis, nodular goiter and well-differentiated thyroid cancer. Hrthle cell tumors contain

increased number of mitochondria with structural abnormalities resembling patients with

mitochondrial disease or myopathy. Adenomas are more common than carcinomas. Hrthle

carcinoma is more aggressive, produces more metastasis, has a lower survival rate and low uptake

for radioiodine. Hrthle adenomas can be managed with hemithyroidectomy, while the carcinoma

variety will need total thyroidectomy. Since most Hrthle cell neoplasms secrete thyroglobulin it

can be used to detect recurrent disease. Hrthle cell tumor size is predictive of malignancy with

adenomas on the average smaller than carcinomas (2 cm vs. 4 cm). Hrthle carcinoma has a

prognosis that is reliably predicted by degree of invasion, tumor size, extrathyroidal disease

extension, and initial nodal or distant metastasis. Molecular express ion of high Ki-67 phenotype

proliferative index correlates with recurrence and tumor-related mortality among Hrthle cell

tumors.

References:

1- Chen H, Nicol TL, Zeiger MA, Dooley WC, Ladenson PW, Cooper DS, Ringel M, Parkerson S, Allo M, Udelsman R:Hrthle cell neoplasms of the thyroid: are there factors predictive of malignancy? Ann Surg. 227(4):542-6, 19982- Hoos A, Stojadinovic A, Singh B, Dudas ME, Leung DH, Shaha AR, Shah JP, Brennan MF, Cordon-Cardo C, Ghos sein R:Clinical significance of molecular expression profiles of Hrthle cell tumors of the thyroid gland analyzed via tissuemicroarrays. Am J Pathol. 160(1):175-83, 20023- Stojadinovic A, Hoos A, Ghos sein RA, Urist MJ, Leung DH, Spiro RH, Shah JP, Brennan MF, Singh B, Shaha AR:Hrthle cell carcinoma: a 60-year experience. Ann Surg Oncol. 9(2):197-203, 20024- Maximo V, Soares P, Lima J, Cameselle-Teijeiro J, Sobrinho-Simaes M: Mitochondrial DNA somatic mutations (pointmutations and large deletions) and mitochondrial DNA variants in human thyroid pathology: a s tudy with emphasis onHrthle cell tumors. Am J Pathol. 160(5):1857-65, 20025- Besic N, Hocevar M, Zgajnar J, Petric R, Pilko G: Aggressiveness of therapy and prognosis of patients with Hrthle cell

papillary thyroid carcinoma. Thyroid. 16(1):67-72, 20066- Bremer AA, Feldman BJ, Iezza G, Clark OH, Rosenthal SM: Report of a Hrthle cell neoplasm in a peripubertal girl.Thyroid. 17(2):175-8, 2007

7- Sippel RS, Elaraj DM, Khanafshar E, Zarnegar R, Kebebew E, Duh QY, Clark OH: Tumor size predicts malignant potentialin Hrthle cell neoplasms of the thyroid. World J Surg. 32(5):702-7, 2008

Cloacogenic Polyps

Inflammatory cloacogenic polyps are very rarely found in the pediatric age. They arise from the

transitional zone of the anal canal, but can extend proximally toward the sigmoid colon.

Characterized histologically by marked hyperplasia of the muscularis mucosa with extension of

smooth muscle and fibrous stroma into the lamina propria. The typical presentation of the patient is

difficulty with defecation and passage of mucous and blood per rectum. The polyps can prolapse;this is due to the malfunction of the internal anal sphincter; and the smooth muscle that covers the

rectum. During endoscopy they can appear polypoid with flat base. The polyps vary in size from 3-4

cm in diameter, and have a sessile appearance. Inflammatory cloacogenic polyp is related to


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solitary rectal ulcer syndrome and is most likely due to prolapse of the anorectal transition zone.

Cloacogenic polyps are not a neoplasm arising from a preexistent normal transitional epithelium

but a nonspecific regenerative process. Management consists of endoscopic removal of the polyps.

Those unable to be removed endoscopically or endorectal will need sigmoidectomy with low

anterior resection.

References:

1- Ciriza de Los Rios C, Tomas Moro E, Garcia Duran F, et al: [Inflammatory cloacogenic polyps : a rare caus e of rectalbleeding]. Gastroenterol Hepatol. 30(8):461-4, 20072- Calva-Rodriguez R, Gonzalez-Palafox MA, Rivera-Dominguez ME, et al: [Inflammatory cloacogenic polyp]. RevGastroenterol Mex. 72(4):371-5, 20073- Washington K, Rourk MH Jr, McDonagh D, Oldham KT: Inflammatory cloacogenic polyp in a child: part of the spectrumof solitary rectal ulcer syndrome. Pediatr Pathol. 13(4):409-14, 19934- Bass J, Soucy P, Walton M, Nizalik E: Inflammatory cloacogenic polyps in children. J Pediatr Surg. 30(4):585-8, 19955- Poon KK, Mills S, Booth IW, Murphy MS: Inflammatory cloacogenic polyp: an unrecognized cause of hematochezia andtenesmus in childhood. J Pediatr. 130(2):327-9, 19976- Siafakas C, Vottler TP, Andersen JM: Rectal prolapse in pediatrics. Clin Pediatr (Phila). 38(2):63-72, 1999

PSU Volume 38 NO 06 JUNE 2012

Pulmonary Metastasectomy

The most common thoracic pediatric malignancy is pulmonary metastasis secondary to solid

tumors. Survival has been improved with the use of pulmonary metastasectomy. By examining

tumor types individually it is seen that certain histologies (adrenocortical carcinoma, alveolar soft

part sarcoma, osteosarcoma) mandate surgical metastasectomy for patient survival. Other

pediatric tumors (Wilms tumor, Ewing's sarcoma) are radiation sensitive and application ofmetastasectomy is controversial, though a possible benefit for Ewing's cases has been found

recently when combined with radiotherapy. In Wilms, pulmonary metastasectomy is indicated if

complete remission can be achieved to avoid lung irradiation. In the case of neuroblastoma,

differentiated thyroid cancer, rhabdomyosarcoma, metastasectomy is seldom performed except in

highly unusual situations. During metastasectomy wedge resection is more commonly performed

than anatomic resection in order to minimized the volume of resected lung tissue. Significant longer

survival is observed for patient after complete resection, with five or fewer metastatic nodules,

unilateral disease, and disease-free interval of more than two years. Performing metastasectomy at

least three months after detection might significantly improve the prognosis. Other factorsassociated with dismal prognosis are poor chemonecrosis and central distribution instead of

peripheral location of the metastasis. The open thoracotomy approach is preferred since the

pulmonary nodule must be manually palpated for an optimal resection.

References:

1- Kayton ML: Pulmonary metas tasectomy in pediatric patients . Thorac Surg Clin.16(2):167-83, 20062- Tronc F, Conter C, Marec-Berard P, Bossard N, Remontet L, Orsini A, Gamondes JP, Louis D: Prognostic factors andlong-term results of pulmonary metastasectomy for pediatric histologies.Eur J Cardiothorac Surg. 34(6):1240-6, 20083- Tanaka Y, Maniwa Y, Nishio W, Yoshimura M, Okita Y: The optimal timing to resect pulmonary metastas is. Eur JCardiothorac Surg. 33(6):1135-8, 2008

4- Rasalkar DD, Chu WC, Lee V, Paunipagar BK, Cheng FW, Li CK: Pulmonary metas tases in children with os teosarcoma:characteristics and impact on patient s urvival.Pediatr Radiol. 41(2):227-36, 20115- Letourneau PA, Shackett B, Xiao L, Trent J, Tsao KJ, Lally K, Hayes-Jordan A: Resection of pulmonary metastases in

pediatric patients with Ewing sarcoma improves s urvival.J Pediatr Surg. 46(2):332-5, 20116- Warmann SW, Furtwngler R, Blumens tock G, Armeanu S, Nourkami N, Leus chner I, Schenk JP, Graf N, Fuchs J: Tumor


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biology influences the prognosis of nephroblastoma patients with primary pulmonary metas tases: results from SIOP 93-01/GPOH and SIOP 2001/GPOH.Ann Surg. 254(1):155-62, 20117- Letourneau PA, Xiao L, Harting MT, Lally KP, Cox CS Jr, Andrass y RJ, Hayes-Jordan AA: Location of pulmonarymetas tas is in pediatric osteosarcoma is predictive of outcome. J Pediatr Surg. 46(7):1333-7, 2011

Magnet Ingestion

Most foreign body ingestion in infant and children passes through the gastrointestinal tract withoutcausing significant sequelae. Surgical intervention is generally required if an object becomes

lodged in the gastrointestinal tract or if the material has a harmful effect such as the corrosive

effect of batteries. With rare-earth magnets present in many small toys, the situation can be very

different. A single magnet ingestion is innocuous and is expected to pass through the GI tract.

Unfortunately, a misdiagnosis and misconception that a solitary magnet has been ingested when in

fact they are two or more joined together may lead to a delay in diagnosis and subsequent severe

and possibly preventable complications. The ingestion of multiple magnets can cause bowel

obstruction, volvulus, perforation or internal bowel fistula formation owning to pressure necrosis

from magnet attraction. Pressure necrosis and fistula formation can be a gradual process resultingin minimal physical examination findings. In many of the toys the magnets are embedded in plastic

parts that are easily detachable. If in the stomach, the magnet should be removed endoscopically.

If the history, clinical findings and imaging are suggestive of multiple magnetic ingestion early

intervention using laparoscopy or open surgery is indicated to prevent serious life-threatening

complications. It is imperative health authorities give more information to parents and physicians

about the potential risk of small magnetic toys in children.

References:

1- Hernandez Ans elmi E, Gutierrez San Roman C, Barrios Fontoba JE, et al: Intes tinal perforation caused by magnetic toys. J

Pediatr Surg. 42(3):E13-6, 20072- Alzahem AM, Soundappan SS, Jefferies H, Cass DT: Ingested magnets and gastrointestinal complications. J PaediatrChild Health. 43(6):497-8, 20073- Butterworth J, Feltis B: Toy magnet ingestion in children: revising the algorithm. J Pediatr Surg. 42(12):e3-5, 20074- Dutta S, Barzin A: Multiple magnet ingest ion as a source of s evere gas trointestinal complicationsrequiring surgical intervention. Arch Pediatr Adolesc Med. 162(2):123-5, 20085- Naji H, Isacson D, Svensson JF, Wester T: Bowel injuries caused by ingestion of multiple magnets in children: a growinghazard. Pediatr Surg Int. 28(4):367-7, 20126-Salimi A, Kooraki S, Esfahani SA, Mehdizadeh M: Multiple magnet ingest ion: Is there a role for early surgicalintervention? Ann Saudi Med. 32(1):93-6, 2012

HIPEC

Hyperthermic intraperitoneal chemotherapy (HIPEC) is an alternative of management based on

the fact that hyperthermia and chemotherapy have synergistic cytotoxicity for microscopic

carcinomatous disease. In adults it has been applied successfully for extensive peritoneal disease

associated with such tumors as mesothelioma, appendiceal, colonic, gastric and ovarian carcinoma.

During closed-technique HIPEC the skin is temporarily closed and chemotherapy is delivered at

supranormal temperatures. Drugs known to have synergy with hyperthermia include mitomycin C,

doxorubicin and cisplatin. HIPEC has been found to be safe and improve median survival in

children with dermoplastic small round cell tumor after complete surgical excision. Adult and

pediatric patient undergoing HIPEC are a highly selected group who does not have uncontrolled

disease outside the abdominal cavity. HIPEC and cytoreductive surgery is not recommended for

palliative purposes. Other rare instances where HIPEC has been used effectively in children


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include peritoneal metastasis from melanoma, signet cell colonic carcinoma and Wilms's tumor.

Indications for HIPEC include resectability to no visible disease, active disease limited to the

abdomen, no liver metastasis, normal liver and kidney function and disease partially responsive to

neoadjuvant chemotherapy.

References:

1- Reingruber B, Boettcher MI, Klein P, Hohenberger W, Pelz JO: Hyperthermic intraperitoneal chemoperfusion is an optionfor treatment of peritoneal carcinomatosis in children. J Pediatr Surg. 42(9):E17-21, 20072- Hayes -Jordan A, Green H, Fitzgerald N, Xiao L, Anderson P: Novel treatment for des moplas tic small round cell tumor:hyperthermic intraperitoneal perfusion. J Pediatr Surg. 45(5):1000-6, 20103- Msika S, Gruden E, Sarnacki S, Orbach D, Philippe-Chomette P, Castel B, Sabata JM, Flamant Y, Kianmanesh R:Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusionfor desmoplas tic round small cell tumor with peritoneal carcinomatosis in young patients. J Pediatr Surg. 45(8):1617-21, 20104- Owusu-Agyemang P, Arunkumar R, Green H, Hurst D, Landoski K, Hayes-Jordan A: Anesthetic Management and RenalFunction in Pediatric Patients Undergoing Cytoreductive Surgery with Continuous Hyperthermic IntraperitonealChemotherapy (HIPEC) with Cisplatin. Ann Surg Oncol. 2012 Mar 27.5- Hayes -Jordan A, Green H, Ludwig J, Anderson P: Toxicity of hyperthermic intraperitoneal chemotherapy (HIPEC) in

pediatric patients with sarcomatosis/carcinomatosis: Early experience and phase 1 results. Pediatr Blood Cancer. 2012 Apr10. doi: 10.1002/pbc.24160.

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