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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 size. 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 resection have been documented. PET-Scan is more specific than MIBG in localizing residual disease.
Re fe re nce s: 1- Minamiguchi N, Inui E, Nukui M: [A case of dopamine-secreting pheochromocytoma]. Hinyokika Kiyo. 45(12):831-3, 1999 2- Yasunari K, Kohno M, Minami M, Kano H, Ohhira M, Nakamura K, Yoshikawa J: A dopamine-secreting pheochromocytoma. J Cardiovasc Pharmacol. 36 Suppl 2:S75-7, 2000 3- Awada SH, Grisham A, Woods SE: Large dopamine-secreting pheochromocytoma: case report. South Med J. 96(9):914-7, 2003 4- Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai EW, Adams KT , Pacak K: Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 90(4):2068-75, 2005 5- T am V, Ng KF, Fung LM, Wong YY, Chan MH, Lam CW, T am S, Lam CW: T he importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma. Ann Clin Biochem. 42(Pt 1):73-7, 2005 6- Foo SH, Chan SP, Ananda V, Rajasingam V: Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management. Singapore Med J. 51(5):e89-93, 2010 7- Dubois LA, Gray DK: Dopamine-secreting pheochromocytomas: in search of a syndrome. World J Surg. 29(7):909-13, 2005

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 shunt 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 assisted VP shunt revision is advocated for patients with multiple previous shunt revisions, prior abdominal surgery, previous intraperitoneal infections, broken devices, obesity or CSF pseudocysts.
Re fe re nce s: 1- Khosrovi H, Kaufman HH, Hrabovsky E, Bloomfield SM, Prabhu V, el-Kadi HA: Laparoscopic-assisted distal ventriculoperitoneal shunt 1/12



placement. Surg Neurol. 49(2):127-34, 1998 2- Jackson CC, Chwals WJ, Frim DM: A single-incision laparoscopic technique for retrieval and replacement of disconnected ventriculoperitoneal shunt tubing found in the peritoneum.Pediatr Neurosurg. 36(4):175-7, 2002 3- Kirshtein B, Benifla M, Roy-Shapira A, Merkin V, Melamed I, Cohen Z, Cohen A: Laparoscopically guided distal ventriculoperitoneal shunt placement. Surg Laparosc Endosc Percutan T ech. 14(5):276-8, 2004 4- Bani A, Hassler WE: Laparoscopy-guided insertion of peritoneal catheters in ventriculoperitoneal shunt procedures: analysis of 39 children. Pediatr Neurosurg. 42(3):156-8, 2006 5- Yu S, Bensard DD, Partrick DA, Petty JK, Karrer FM, Hendrickson RJ: Laparoscopic guidance or revision of ventriculoperitoneal shunts in children.JSLS. 10(1):122-5, 2006 6- Johnson BW, Pimpalwar A: Laparoscopic-assisted placement of ventriculo-peritoneal shunt tips in children with multiple previous open abdominal ventriculo-peritoneal shunt surgeries. Eur J Pediatr Surg. 19(2):79-82, 2009 7- Martin K, Baird R, Farmer JP, et al: T he 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 incisions 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, stomach, lung tissue, gallbladder providing traction and facilitating exposure. Magnetassisted laparoscopy is safe and effective means of reducing the number and size of abdominal incisions while improving exposure, triangulation, and the ergonomics of the procedure.
Re fe re nce s: 1- Rothenberg SS, Shipman K, Yoder S: Experience with modified single-port laparoscopic procedures in children. J Laparoendosc Adv Surg T ech A. 19(5):695-8, 2009 2- 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, 2011 3- de Armas IA, Garcia I, Pimpalwar A: Laparoscopic single port surgery in children using T riport: our early experience. Pediatr Surg Int. 27(9):985-9, 2011 4- Dutta S: Early experience with single incision laparoscopic surgery: eliminating the scar from abdominal operations. J Pediatr Surg. 44(9):1741-5, 2009 5- Padilla BE, Dominguez G, Millan C, Martinez-Ferro M: T he use of magnets with single-site umbilical laparoscopic surgery. Semin Pediatr Surg. 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 2/12



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 syndrome presenting in childhood. Br J Surg. 83(2):235-40, 1996 2- 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, 2009 3- Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC: Mid-aortic syndrome: a case report and review of the literature. Int J Cardiol. 123(3):348-52, 2008 4- Chocrn de Benzaquen S, Munoz Lopez M, Madrid Aris AD, Castellote Alonso A, Enriquez G, Nieto Rey JL: [Midaortic syndrome: case studies from a paediatric nephrology department]. An Pediatr (Barc). 75(1):33-9, 2011 5- Bleacher J, Turner ME, Quivers E, Schwartz MZ: Renal autotransplantation for renovascular hypertension caused by midaortic syndrome. J Pediatr Surg. 32(2):248-50, 1997 6- 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 of an 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 cyst in a young child. J Pediatr Surg. 40(11):e51-3, 2005 2- Stringer MD, Jones MO, Woodley H, Wyatt J: Ciliated hepatic foregut cyst. J Pediatr Surg. 41(6):1180-3, 2006 3- Betalli P, Gobbi D, Talenti E, Alaggio R, Gamba P, Zanon GF: Ciliated hepatic foregut cyst: from antenatal diagnosis to surgery.Pediatr Radiol. 38(2):230-2, 2008 4- Sharma S, Dean AG, Corn A, Kohli V, Wright HI, Sebastian A, Jabbour N: Ciliated hepatic foregut cyst: an increasingly diagnosed condition.Hepatobiliary Pancreat Dis Int. 7(6):581-9, 2008 5- Goodman MD, Mak GZ, Reynolds JP, Tevar AD, Pritts TA: Laparoscopic excision of a ciliated hepatic foregut cyst.JSLS. 13(1):96-100, 2009 6- Zaydfudim V, Rosen MJ, Gillis LA, Correa H, Lovvorn HN 3rd, Pinson CW, Kelly BS Jr: Ciliated hepatic foregut cysts in children. 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 postnatal Ciliated Hepatic Foregut Cysts in infants. J Pediatr Surg. 45(3):E9-14, 2010

ALTE 3/12



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 death syndrome 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, 1987 2- McGovern MC, Smith MB: Causes of apparent life threatening events in infants: a systematic review. Arch Dis Child. 89(11):1043-8, 2004 3- Dewolfe CC: Apparent life-threatening event: a review. Pediatr Clin North Am. 52(4):1127-46, 2005 4- Esani N, Hodgman JE, Ehsani N, Hoppenbrouwers T: Apparent life-threatening events and sudden infant death syndrome: comparison of risk factors. J Pediatr. 152(3):365-70, 2008 5- Valusek PA, St Peter SD, Tsao K, et al. The use of fundoplication for prevention of apparent life-threatening events. J Pediatr Surg 42:1022-1024, 2007 6- Tirosh E, Ariov-Antebi N, Cohen A: Autonomic function, gastroesophageal reflux in apparent life threatening event. Clin Auton 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, and the 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: 4/12



1- Knechtle SJ, Filston HC: Anomalous biliary ducts associated with duodenal atresia. J Pediatr Surg. 25(12):1266-9, 1990 2- Panuel M, Bourliere-Najean B, Delarue A, Viard L, Faure F, Devred P: [Duodenal atresia with bifid termination of the common bile duct]. Arch Fr Pediatr. 49(4):365-7, 1992 3- Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y: Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia. J Hepatobiliary Pancreat Surg. 6(1):50-4, 1999 4- Tashjian DB, Moriarty KP: Duodenal atresia with an anomalous common bile duct masquerading as a midgut volvulus. J Pediatr Surg. 36(6):956-7, 2001 5- Mali V, Wagener S, Sharif K, Millar AJ: Foregut atresias and bile duct anomalies: rare, infrequent or common? Pediatr Surg 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 of distal 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 recessive 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, 2001 2- Ruemmele FM, Schmitz J, Goulet O: Microvillous inclusion disease (microvillous atrophy). Orphanet J Rare Dis. 26;1:22, 2006 3- Iancu TC, Mahajnah M, Manov I, Shaoul R: Microvillous inclusion disease: ultrastructural variability. Ultrastruct Pathol. 31(3):173-88, 2007 4- 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, 2011 5- Fuchs J, Fallon EM, Gura KM, Puder M: Use of an omega-3 fatty acid-based emulsion in the treatment of parenteral nutrition-induced cholestasis 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 5/12



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, 2009 2- Park J, Puapong DP, Wu BM, Atkinson JB, Dunn JC: Enterogenesis by mechanical lengthening: morphology and function of the lengthened small intestine. J Pediatr Surg. 39(12):1823-7, 2004 3- Chang PC, Mendoza J, Park J, Lam MM, Wu B, Atkinson JB, Dunn JC: Sustainability of mechanically lengthened bowel in rats. J Pediatr Surg. 41(12):2019-22, 2006 4- Stark R, Zupekan T, Bondada S, Dunn JC: Restoration of mechanically lengthened jejunum into intestinal continuity in rats. J Pediatr Surg. 46(12):2321-6, 2011 5- Stark R, Panduranga M, Carman G, Dunn JC: Development of an endoluminal intestinal lengthening capsule. J Pediatr Surg. 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 are lost 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 are placed 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, et al: An evaluation of abdominal staging procedures performed in pediatric patients with advanced Hodgkin's disease: a report from the Childrens Cancer Study Group.J Pediatr Surg. 27(9):1175-80. 1992 2- Hloury Y, Guiberteau V, Sagot P, Plattner V, Baron M, Rogez JM: Laparoscopy in adnexal pathology in the child: a study of 28 cases. Eur J Pediatr Surg. 3(2):75-8, 1993 3- Williams RS, Littell RD, Mendenhall NP: Laparoscopic oophoropexy and ovarian function in the treatment of Hodgkin disease. Cancer. 86(10):2138-42, 1999 4- Meirow D, Nugent D: The effects of radiotherapy and chemotherapy on female reproduction. Hum Reprod Update. 7(6):535-43, 2001 5- Terenziani M, Piva L, Meazza C, Gandola L, Cefalo G, Merola M: Oophoropexy: a relevant role in preservation of ovarian function after pelvic irradiation. Fertil Steril. 91(3):935.e15-6, 2009 6/12



6- Han SS, Kim YH, Lee SH, Kim GJ, Kim HJ, Kim JW, Park NH, Song YS, Kang SB: Underuse of ovarian transposition in reproductive-aged cancer patients treated by primary or adjuvant pelvic irradiation. J Obstet Gynaecol Res. 37(7):825-9, 2011 7- 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 gland associated. 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, 2009 2- Poiana C, Carsote M, Chirita C, Terzea D, Paun S, Beuran M: Giant adrenal cyst: case study. J Med Life. 3(3):308-13, 2010 3- Guazzoni G, Montorsi F, Rigatti P, Lanzi R, Pontiroli AE, Silvestre P, Breda G: Laparoscopic unroofing of adrenal cysts. Eur Urol. 31(4):499-502, 1997 4- Pradeep PV, Mishra AK, Aggarwal V, Bhargav PR, Gupta SK, Agarwal A: Adrenal cysts: an institutional experience. World J Surg. 30(10):1817-20, 2006 5- Lal TG, Kaulback KR, Bombonati A, Palazzo JP, Jeffrey RB, Weigel RJ: Surgical management of adrenal cysts. Am Surg. 69(9):812-4, 2003 6- Castillo OA, Litvak JP, Kerkebe M, Urena RD: Laparoscopic management of symptomatic and large adrenal cysts. J Urol. 173(3):915-7, 2005

Measuring IAP
The abdominal compartment syndrome (ACS) is a clinical syndrome caused by persistency elevated 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 7/12



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 of different volumes and temperatures of saline on the bladder pressure measurement in critically ill patients. Crit Care. 11(4):R82, 2007 2- Gudmundsson FF, Viste A, Gislason H, Svanes K: Comparison of different methods for measuring intra-abdominal pressure. Intensive Care Med. 28(4):509-14, 2002 3- Zengerink I, McBeth PB, Zygun DA, Ranson K, Ball CG, Laupland KB, Widder S, Kirkpatrick AW: Validation and experience with a simple continuous intra-abdominal pressure measurement technique in a multidisciplinary medical/surgical critical care unit. J Trauma. 64(5):1159-64, 2008 4- Balogh Z, Jones F, D'Amours S, Parr M, Sugrue M: Continuous intra-abdominal pressure measurement technique. Am J Surg. 188(6):679-84, 2004 5- Suominen PK, Pakarinen MP, Rautiainen P, Mattila I, Sairanen H: Comparison of direct and intravesical measurement of intraabdominal pressure in children. J Pediatr Surg. 41(8):1381-5, 2006 6- Ejike JC, Bahjri K, Mathur M: What is the normal intra-abdominal pressure in critically ill children and how should we measure it? Crit Care Med. 36(7):2157-62, 2008 7- Davis PJ, Koottayi S, Taylor A, Butt WW: Comparison of indirect methods of measuring intra-abdominal pressure in children. Intensive 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, mortality and 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: Peripheral intravenous catheter complications in critically ill children: a prospective study. Pediatrics. 89(6 Pt 2):1145-50, 1992 2- 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, 2001 4- Tong R: Preventing extravasation injuries in neonates. Paediatr Nurs. 19(8):22-5, 2007 5- Rose RE, Felix R, Crawford-Sykes A, Venugopal R, Wharfe G, Arscott G: Extravasation injuries. West Indian Med J. 57(1):40-7, 2008 6- Amjad I, Murphy T, Nylander-Housholder L, Ranft A: A new approach to management of intravenous infiltration in 8/12



pediatric patients: pathophysiology, classification, 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. Hrthle cells 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 expression 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, 1998 2- Hoos A, Stojadinovic A, Singh B, Dudas ME, Leung DH, Shaha AR, Shah JP, Brennan MF, Cordon-Cardo C, Ghossein R: Clinical significance of molecular expression profiles of Hrthle cell tumors of the thyroid gland analyzed via tissue microarrays. Am J Pathol. 160(1):175-83, 2002 3- Stojadinovic A, Hoos A, Ghossein 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, 2002 4- Maximo V, Soares P, Lima J, Cameselle-Teijeiro J, Sobrinho-Simaes M: Mitochondrial DNA somatic mutations (point mutations and large deletions) and mitochondrial DNA variants in human thyroid pathology: a study with emphasis on Hrthle cell tumors. Am J Pathol. 160(5):1857-65, 2002 5- 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, 2006 6- 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 potential in 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 9/12



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 cause of rectal bleeding]. Gastroenterol Hepatol. 30(8):461-4, 2007 2- Calva-Rodriguez R, Gonzalez-Palafox MA, Rivera-Dominguez ME, et al: [Inflammatory cloacogenic polyp]. Rev Gastroenterol Mex. 72(4):371-5, 2007 3- Washington K, Rourk MH Jr, McDonagh D, Oldham KT: Inflammatory cloacogenic polyp in a child: part of the spectrum of solitary rectal ulcer syndrome. Pediatr Pathol. 13(4):409-14, 1993 4- Bass J, Soucy P, Walton M, Nizalik E: Inflammatory cloacogenic polyps in children. J Pediatr Surg. 30(4):585-8, 1995 5- Poon KK, Mills S, Booth IW, Murphy MS: Inflammatory cloacogenic polyp: an unrecognized cause of hematochezia and tenesmus in childhood. J Pediatr. 130(2):327-9, 1997 6- 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 of metastasectomy 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 factors associated 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 metastasectomy in pediatric patients. Thorac Surg Clin.16(2):167-83, 2006 2- Tronc F, Conter C, Marec-Berard P, Bossard N, Remontet L, Orsini A, Gamondes JP, Louis D: Prognostic factors and long-term results of pulmonary metastasectomy for pediatric histologies.Eur J Cardiothorac Surg. 34(6):1240-6, 2008 3- Tanaka Y, Maniwa Y, Nishio W, Yoshimura M, Okita Y: The optimal timing to resect pulmonary metastasis. Eur J Cardiothorac Surg. 33(6):1135-8, 2008 4- Rasalkar DD, Chu WC, Lee V, Paunipagar BK, Cheng FW, Li CK: Pulmonary metastases in children with osteosarcoma: characteristics and impact on patient survival.Pediatr Radiol. 41(2):227-36, 2011 5- 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 survival.J Pediatr Surg. 46(2):332-5, 2011 6- Warmann SW, Furtwngler R, Blumenstock G, Armeanu S, Nourkami N, Leuschner I, Schenk JP, Graf N, Fuchs J: Tumor 10/12



biology influences the prognosis of nephroblastoma patients with primary pulmonary metastases: results from SIOP 9301/GPOH and SIOP 2001/GPOH.Ann Surg. 254(1):155-62, 2011 7- Letourneau PA, Xiao L, Harting MT, Lally KP, Cox CS Jr, Andrassy RJ, Hayes-Jordan AA: Location of pulmonary metastasis 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 without causing 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 resulting in 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 Anselmi E, Gutierrez San Roman C, Barrios Fontoba JE, et al: Intestinal perforation caused by magnetic toys. J Pediatr Surg. 42(3):E13-6, 2007 2- Alzahem AM, Soundappan SS, Jefferies H, Cass DT: Ingested magnets and gastrointestinal complications. J Paediatr Child Health. 43(6):497-8, 2007 3- Butterworth J, Feltis B: Toy magnet ingestion in children: revising the algorithm. J Pediatr Surg. 42(12):e3-5, 2007 4- Dutta S, Barzin A: Multiple magnet ingestion as a source of severe gastrointestinal complications requiring surgical intervention. Arch Pediatr Adolesc Med. 162(2):123-5, 2008 5- Naji H, Isacson D, Svensson JF, Wester T: Bowel injuries caused by ingestion of multiple magnets in children: a growing hazard. Pediatr Surg Int. 28(4):367-7, 2012 6-Salimi A, Kooraki S, Esfahani SA, Mehdizadeh M: Multiple magnet ingestion: Is there a role for early surgical intervention? Ann Saudi Med. 32(1):93-6, 2012

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 11/12



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 option for treatment of peritoneal carcinomatosis in children. J Pediatr Surg. 42(9):E17-21, 2007 2- Hayes-Jordan A, Green H, Fitzgerald N, Xiao L, Anderson P: Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion. J Pediatr Surg. 45(5):1000-6, 2010 3- 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 chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients. J Pediatr Surg. 45(8):1617-21, 2010 4- Owusu-Agyemang P, Arunkumar R, Green H, Hurst D, Landoski K, Hayes-Jordan A: Anesthetic Management and Renal Function in Pediatric Patients Undergoing Cytoreductive Surgery with Continuous Hyperthermic Intraperitoneal Chemotherapy (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 Apr 10. doi: 10.1002/pbc.24160.



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