VOLUME 39, 2012

PSU Volume 39 No 01 JULY 2012

Intrathoracic Kidney

Congenital intrathoracic kidney is a very rare ectopic anomaly of the kidney. Most ectopic kidneys are found in the pelvic or lower lumbar region. Delayed closure of the pleuroperitoneal membrane as it occurs in diaphragmatic hernia permits excessive cranial migration of the kidney. Other postulates that delayed ingrowth of the ureter into the metanephros results in prolongation of the ascending process involving a long ureter and prolonged migration. In either case the kidney assumes a thoracic position. Intrathoracic kidneys do not display dysplastic parenchymal architecture and the kidneys are usually functional. Thoracic kidneys can be classified as thoracic renal ectopia with closed diaphragm, eventration of the diaphragm, diaphragmatic hernia (congenital diaphragmatic defects or acquired hernia such as Bochdalek hernia), and traumatic rupture of the diaphragm with renal ectopia. Conditions that affect the normal kidney such as ureteric stones, hydronephrosis, and renal cell carcinoma has also been reported to occur in intrathoracic kidneys later in life. Intrathoracic kidney can be found as a unique anomaly or associated with a diaphragmatic hernia defect, respiratory symptoms and bowel herniation within the thorax. Diagnosis is confirmed with CT or MR imaging. When presenting as a unique anomaly not associated to a bowel herniation or respiratory symptoms they can be managed conservatively with regular follow-up since normal growth and development of the affected kidney occurs. Surgical treatment has been reserved for children with associated bowel herniation or respiratory compromise. In such situation repair of the diaphragmatic defect and nephropexy is in order.

1- Ho HF, Liaw SB, Chang PY: Delayed manifestation of congenital diaphragmatic hernia with intrathoracic kidney: report of one case. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 38(1):61-4, 199
2- Oon PC, Shen HN, Yang PC: Intrathoracic kidney. J Formos Med Assoc. 104(2):120-2, 2005
3- Panda B, Rosenberg V, Cornfeld D, Stiller R: Prenatal diagnosis of ectopic intrathoracic kidney in a fetus with a left diaphragmatic hernia. J Clin Ultrasound. 37(1):47-9, 2009
4- Rouanne M, Le Mandat A, Dorgeret S, Philippe-Chomette P, El Ghoneimi A: A rare case of ectopic intrathoracic kidney in a 1-year-old child. Urology. 76(1):57-9, 2010
5- Fiaschetti V, Velari L, Gaspari E, Mastrangeli R, Simonetti G: Adult intra-thoracic kidney: a case report of bochdalek hernia. Case Report Med. 2010. pii: 975168, 2010
6- Murphy JJ, Altit G, Zerhouni S: The intrathoracic kidney: should we fix it? J Pediatr Surg 47: 970-973, 2012

Port Inversion

Ports connected to central venous catheter are necessary for a variety of reasons such as management of children who require chemotherapy, prolonged antibiotic administration, frequent blood sampling, parenteral nutrition and transfusion of blood and blood products. Totally implantable access ports improve the quality of life and have the advantage of not requiring an external dressing.  Ports are inserted as totally implantable devices underneath the subcutaneous tissue infraclavicularly most commonly or under the pectoralis fascia. The most common complications of port application and maintenance  include occlusion, vein thrombosis, port pocket infection, catheter related sepsis, device rotation or dislodgement, catheter migration, skin necrosis, pocket hematoma and exposed port. Port inversion occurs when the port completes a 90-degree rotation due to mobility and the caretaker cannot cannulate the port diaphragm. A simple lateral chest film will reveal the problem of port inversion. Port inversion is seen more commonly after subcutaneous placement than subpectoral fascia placement. Port implantation in the subpectoral fascia pocket has been found to have a lower rate of skin complications than the subcutaneous pocket implantation site.  Whether to fix the port with sutures to avoid rotation or inversion is a matter of debate, since suturing will need more extensive dissection during removal. Port inversion will need revision.

1- Dillon PA, Foglia RP: Complications associated with an implantable vascular access device. J Pediatr Surg. 41(9):1582-7, 2006
2- Charles HW, Miguel T, Kovacs S, Gohari A, Arampulikan J, McCann JW: Chest port placement with use of the single-incision insertion technique. J Vasc Interv Radiol. 20(11):1464-9, 2009
3- McNulty NJ, Perrich KD, Silas AM, Linville RM, Forauer AR: Implantable subcutaneous venous access devices: is port fixation necessary? A review of 534 cases. Cardiovasc Intervent Radiol. 33(4):751-5, 2012
4- Rouzrokh M, Shamsian BS, KhaleghNejad Tabari A, Mahmoodi M, Kouranlo J, Manafzadeh G, Arzanian MT, Fallah F, Anoush M, Abdollah Gorji F: Totally implantable subpectoral vs. subcutaneous port systems in children with malignant diseases. Arch Iran Med. 12(4):389-94, 2009

Idiopathic Megaduodenum

Idiopathic megaduodenum is a rare congenital condition defined as a massive dilatation of the duodenum without evidence of mechanical extraluminal or intraluminal obstruction. The etiology is unknown. It may be caused by neuromuscular disorder or abnormal gut collagen and interticium. Duodenal dilatation and stasis may be caused by decreased peristalsis and accumulation of motilin in the duodenal cavity. Children with idiopathic megaduodenum (ID) present with recurrent abdominal distension, nausea vomiting, diarrhea and malnutrition. Diagnosis is obtained with upper GI series. Management is surgical and should achieve drainage of the duodenum, reduce the capacity of the duodenum and cut off the forceful feeding of the duodenum. This can be accomplished more physiologically with tapering duodenoplasty and side-to-side gastrojejunostomy, duodenojejunostomy or Roux-en-Y duodenojejunostomy. A familiar variety of this condition has been described in the literature. Also, children with chronic intestinal pseudoobstruction can present with idiopathic megaduodenum.  

1- McClenahan JE, Fisher B: Idiopathic megaduodenum; report of a case. Am J Dig Dis. 15(12):414-6, 1848
2- Eaves ER, Schmidt GT: Chronic idiopathic megaduodenum in a family. Aust N Z J Med. 15(1):1-6, 1985
3- Cheng SC, Sanderson CR, Way NJ, Yoong PM: Familial idiopathic megaduodenum. Aust N Z J Surg. 57(11):879-82, 1987
4- Svartsja B, Sulonen H, Leino R: [Megaduodenum as a first sign of chronic idiopathic intestinal
pseudo-obstruction]. Duodecim. 106(7):578-81, 1990
5- Nichol PF, Stoddard E, Lund DP, Starling JR: Tapering duodenoplasty and Roux-en-Y duodenojejunostomy in the management of adult megaduodenum. Surgery. 135(2):222-4, 2004
6- Zhang XW, Abudoureyimu A, Zhang TC, Zhao JR, Fu LB, Lin F, Qiu XH, Chen YJ: Tapering duodenoplasty and gastrojejunostomy in the management of idiopathic megaduodenum in children. J Pediatr Surg. 47(5):1038-42, 2012

PSU Volume 39 No 02 AUGUST 2012

Propranolol for Hemangiomas

The most common benign tumor in infancy is a hemangioma. Most infantile hemangiomas appear during the first few weeks of life, proliferate during the first two years of life and involute during childhood. Involution is characterized by changing from a cellular into a vascular nature with progressive deposition of perivascular fibrofatty tissue, abundant mast cells and less endothelial cell proliferation. Management of hemangiomas has included steroids, laser, cryosurgery, interferon, vincristine and resection. Recently, propranolol, a pure selective beta-adrenergic antagonist has been found to hasten involution of infantile hemangiomas. The suggested dose of management of propranolol is 2 mg/kg/day in three divided dose. Children receiving this kind of therapy should have a full cardiologic evaluation pretreatment. The possible mechanism of action for the effect on involution of hemangiomas by propranolol includes vasoconstriction (inhibition of vasodilation mediated by adrenaline), inhibition of angiogenesis (reduced expression of VEGF),  induction of apoptosis of endothelial cells and inhibiting nitric oxide production. Propranolol is efficient and highly tolerated. The first noticeable effect is change in color of the hemangioma followed by softening of the lesion and finally regression of  size. Optimal length of treatment should cover the proliferative phase and last until maximal improvement has been achieved. The excellent outcome of this therapy has prompted its recommendation as first line therapy of hemangiomas. Infants with renal or hepatic dysfunction, cardiovascular disease, asthma, diabetes or glaucoma should not be managed with propranolol. Side effects of therapy to monitor include bradycardia, hypotension and hypoglycemia.

1- Hogeling M, Adams S, Wargon O: A randomized controlled trial of propranolol for infantile hemangiomas. Pediatrics. 128(2):e259-66, 2011
2- Dai Y, Hou F, Buckmiller L, Fan CY, Saad A, Suen J, Richter GT: Decreased eNOS protein expression in involuting and propranolol-treated hemangiomas. Arch Otolaryngol Head Neck Surg. 138(2):177-82, 2012
3- Talaat AA, Elbasiouny MS, Elgendy DS, Elwakil TF: Propranolol treatment of infantile hemangioma: clinical and radiologic evaluations. J Pediatr Surg. 47(4):707-14, 2012
4- Hsu TC, Wang JD, Chen CH, Chang TK, Wang TM, Chou CM, Lin HK: Treatment with propranolol for infantile hemangioma in 13 Taiwanese newborns and  young infants. Pediatr Neonatol. 53(2):125-32, 2012
5- Chim H, Armijo BS, Miller E, Gliniak C, Serret MA, Gosain AK: Propranolol Induces Regression of Hemangioma Cells Through HIF-1α-Mediated Inhibition of VEGF-A. Ann Surg. 2012 May 10
6- Chung SH, Park DH, Jung HL, Shim JW, Kim DS, Shim JY, Park MS, Koo HH: Successful and safe treatment of hemangioma with oral propranolol in a single institution. Korean J Pediatr. 55(5):164-70, 2012

Vinyl Glove Ingestion

Toddlers, children with pica and some mentally retarded children introduce objects into their mouth constantly. They are the group with the higher incidence of ingested foreign body. Fortunately most ingested foreign body passes through the gastrointestinal tract without causing problems. Most common foreign body site of impaction is the distal ileum, followed by pylorus, duodenal loop, rectosigmoid and anus in that order. Vinyl glove ingestion has been reported to occur in handicap children causing bowel obstruction, perforation and fistulization. Due to the inability to communicate the diagnosis is usually late in mentally retarded patients. Vinyl glove ingestion produces a bezoar in the stomach or distal ileum. The problem is that these soft pliable gloves become hard and irregular and develop sharp cutting edges after ingestion. The glove hardens creating a barrier to the movement of food or succus entericus. This produces a bowel obstruction. Management consists of surgical removal by either open or laparoscopic technique. Endoscopic removal is not recommended. Vinyl gloves should be removed from the immediate proximity of mentally retarded patients or patients with pica. Institution and families caring for mentally retarded children should monitor the accessibility of vinyl glove or find a safer substitute.

1- Selcuk H, Unal H, Korkmaz M, Yilmaz U: Subacutely formed bezoar resulting from accidentally ingested industrial material.J Chin Med Assoc. 72(4):202-3, 2009
2- Burstein I, Steinberg R, Zer M: Small bowel obstruction and covered perforation in childhood caused by bizarre bezoars and foreign bodies. Isr Med Assoc J. 2(2):129-31, 2000
3- Pitiakoudis M, Tsaroucha A, Mimidis K, Constantinidis T, Anagnostoulis S, Stathopoulos G, Simopoulos C: Esophageal and small bowel obstruction by occupational bezoar: report of a case. BMC Gastroenterol. 3:13, 2003
4- Kamal I, Thompson J, Paquette DM: The hazards of vinyl glove ingestion in the mentally retarded patient with pica:  new implications for surgical management. Can J Surg. 42(3):201-4, 1999
5- Stringel G, Parker M, McCoy E: Vinyl glove ingestion in children: a word of caution. J Pediatr Surg. 47(5):996-8, 2012

Gastrointestinal Basidiobolomycosis

Gastrointestinal Basidiobolomycosis (GIB) is a very rare but aggressive fungal infection rarely reported in the literature. The disease is caused by the fungus Basidiobolus Ranarum, an environmental saprophyte that infects skin, subcutaneous tissue and rarely the gastrointestinal tract. GIB presents with nonspecific signs and symptoms such as abdominal pain, mass and fever. Patient comes from tropical and subtropical countries. Radiographic  findings are consistent with either malignancy or inflammation. Patients have elevated WBC with eosinophilia. GI biopsy specimen shows pleomorphic hyphae surrounded by eosinophilic inflammation (Splendore-Hoeppli phenomenon). Complications include bowel perforation, obstructive uropathy, esophageal varices, and duodenobiliary fistula  Definite diagnosis requires culture of the organism. The fungus enters the GI tract through ingestion of contaminated soil, animal feces or food. Management consists of resection of affected inflammatory bowel and debridement of involved tissue followed by more than three months of antifungal therapy with itraconazole. GIB causes significant morbidity and mortality.

1- Al Jarie A, Al-Mohsen I, Al Jumaah S, Al Hazmi M, Al Zamil F, Al Zahrani M, Al Modovar E, Al Dayel F, Al Arishii H, Shehrani D, Martins J, Al Mehaidib A, Rossi  L, Olaiyan I, Le Quesne G, Al-Mazrou A: Pediatric gastrointestinal basidiobolomycosis. Pediatr Infect Dis J. 22(11):1007-14, 2003
2- Nemenqani D, Yaqoob N, Khoja H, Al Saif O, Amra NK, Amr SS: Gastrointestinal basidiobolomycosis: an unusual fungal infection mimicking colon  cancer. Arch Pathol Lab Med. 133(12):1938-42, 2009
3- El-Shabrawi MH, Kamal NM: Gastrointestinal basidiobolomycosis in children: an overlooked emerging infection? J Med Microbiol. 60(Pt 7):871-80, 2011
4- El-Shabrawi MH, Kamal NM, Jouini R, Al-Harbi A, Voigt K, Al-Malki T: Gastrointestinal basidiobolomycosis: an emerging fungal infection causing bowel perforation in a child. J Med Microbiol. 60(Pt 9):1395-402, 2011
5- Vikram HR, Smilack JD, Leighton JA, Crowell MD, De Petris G: Emergence of gastrointestinal basidiobolomycosis in the United States, with a review of worldwide cases. Clin Infect Dis. 54(12):1685-91, 2012
6- Al-Shanafey S, Alrobean F, Hussain IB: Surgical management of gastrointestinal basidiobolomycosis in pediatric patients. J Pediatr Surg. 47(5):949-51, 2012

PSU Volume 39 No 03 SEPTEMBER 2012

Median Arcuate Ligament Syndrome

Median arcuate ligament syndrome (MALS) also known as celiac artery compression is a rare condition that occurs when the fibrous portion of the diaphragmatic crura known as the median arcuate ligament compresses the proximal celiac artery trunk. This compression can also occur with an excessive amount of sympathetic nerves or ganglions. The compression usually occurs during expiration. MALS is a diagnosis of exclusion characterized by weight loss, postprandial abdominal pain, diarrhea, nausea, vomiting and epigastric bruit. Most cases are female. The diagnosis can be established using invasive arteriography or non-invasive with CT angiography, magnetic resonance angiography and Doppler ultrasound studies. CT-angio demonstrates a focal hook appearance narrowing in the proximal celiac artery. Doppler studies shows variations in peak systolic velocity with a marked increase during expiration and functional geometric changes such as celiac trunk deflection. Treatment options for MALS include surgical or laparoscopic division of the median arcuate ligament, celiac ganglion destruction, endovascular stenting or bypass surgery. Advantages of the laparoscopic approach are the imaging magnification, fewer postoperative adhesions, smaller incisions, better cosmesis and shorter hospital stay. Besides relieving the mechanical effect of the arcuate ligament, the interruption of somatic or sympathetic fibers in the course of division may be responsible for the improvement in abdominal pain due to relieve of vasospasm.

1- Alehan D, Dogan OF: Pediatric surgical image. A rare case: celiac artery compression syndrome in an asymptomatic child.  J Pediatr Surg. 39(4):645-7, 2004
2- Gander S, Mulder DJ, Jones S, Ricketts JD, Soboleski DA, Justinich CJ: Recurrent abdominal pain and weight loss in an adolescent: celiac artery compression syndrome. Can J Gastroenterol. 24(2):91-3, 2010
3- Said SM, Zarroug AE, Gloviczki P, Shields RC: Pediatric median arcuate ligament syndrome: first report of familial pattern and  transperitoneal laparoscopic release. J Pediatr Surg. 45(12):e17-20, 2010
4- Aschenbach R, Basche S, Vogl TJ: Compression of the celiac trunk caused by median arcuate ligament in children and adolescent subjects: evaluation with contrast-enhanced MR angiography and comparison with Doppler US evaluation.  J Vasc Interv Radiol. 22(4):556-61, 2011
5- Ozel A, Toksoy G, Ozdogan O, et al: Ultrasonographic diagnosis of median arcuate ligament syndrome: a report of two cases. Medical Ultrasonography 14(2): 154-157, 2012
6- Wani S, Wakde V, Patel R, et al: Laparoscopic release of median arcuate ligament. J Minim Access Surg 8(1): 16-18, 2012

Giant Omphalocele

A giant omphalocele is defined as a defect larger than 10 cm in length that harbors the liver. Prenatally diagnosed giant omphaloceles will need cesarean section as route of birth to avoid fetal liver rupture. Management of giant omphalocele has a high morbidity and mortality due to the defect size, visceroabdominal disproportion, and the associated congenital and genetic malformations. The large size of the defect and small abdominal cavity creates a situation where primary closure is almost impossible unless some sort of stage reduction is tailored. Pulmonary hypoplasia, genetic defects and cardiac malformation are the source of early mortality in these babies. The pendulum of management of giant omphalocele has moved toward a more conservative initial management using topical coverage creams to create granulation tissue and skin on top of the membrane followed by repair of the ventral hernia much later in life when the medical condition of the child permits. Silver sulfadiazine (Silvadene) provides a moist wound healing environment that promotes epithelization and simultaneously minimizes the risk of invasive infection including antifungal coverage. Silver toxicity, though rare can include seizures, peripheral neuropathy, ocular pathology, nephrotic syndrome, raised liver enzymes, leukopenia and algiria. For smaller size defects the use of silo, tissue expanders, biologic mesh, vacuum-assisted closure or component separation technique closure is indicated. Giant omphalocele is associated with deficits in developmental achievements in most of the affected infants ranging from mild to profound delays.

1- Pacilli M, Spitz L, Kiely EM, Curry J, Pierro A: Staged repair of giant omphalocele in the neonatal period. J Pediatr Surg. 40(5):785-8, 2005
2- Kilbride KE, Cooney DR, Custer MD: Vacuum-assisted closure: a new method for treating patients with giant omphalocele.  J Pediatr Surg. 41(1):212-5, 2006
3- Lee SL, Beyer TD, Kim SS, Waldhausen JH, Healey PJ, Sawin RS, Ledbetter DJ: Initial nonoperative management and delayed closure for treatment of giant omphaloceles.J Pediatr Surg. 41(11):1846-9, 2006
4- van Eijck FC, de Blaauw I, Bleichrodt RP, Rieu PN, van der Staak FH, Wijnen MH, Wijnen RM: Closure of giant omphaloceles by the abdominal wall component separation technique in infants.J Pediatr Surg. 43(1):246-50, 2008
5- Danzer E, Gerdes M, D'Agostino JA, Bernbaum J, Siegle J, Hoffman C, Rintoul NE, Liechty KW, Flake AW, Adzick NS, Hedrick HL: Prospective, interdisciplinary follow-up of children with prenatally diagnosed giant omphalocele: short-term neurodevelopmental outcome.  J Pediatr Surg. 45(4):718-23, 2010
6- Ein SH, Langer JC: Delayed management of giant omphalocele using silver sulfadiazine cream: an 18-year experience. J Pediatr Surg. 47(3):494-500, 2012

Congenital Solid Tumors

Congenital solid tumors refer to masses of tissue that have grown in location diverge from the normal pattern of development and do not duplicate any normal structure of the body. Though rare prenatal sonography has increased the rate of diagnosis. Abdominal tumors are the most common prenatally diagnosed fetal tumors. Half of congenital solid tumors  are diagnosed at birth and two-third during the first week of life. The most common congenital tumors are extracranial teratoma, neuroblastoma, soft tissue tumors and brain tumors. The most frequent benign tumor is teratoma, while the most frequent malignant is neuroblastoma. Although easier to detect, cervical and mediastinal tumors have a worse prognosis. Abdominal masses are more difficult to detect but have a  better prognosis. Management should be conservative as possible with surgery playing a major role due to the relative benign behavior of congenital cancer and the potential long term effect of chemotherapy and radiotherapy.

1-Parkes SE, Muir KR, Southern L, Cameron AH, Darbyshire PJ, Stevens MC: Neonatal tumours: a thirty-year population-based study. Med Pediatr Oncol. 22(5):309-17, 1994
2- Sbragia L, Paek BW, Feldstein VA, Farrell JA, Harrison MR, Albanese CT, Farmer DL: Outcome of prenatally diagnosed solid fetal tumors. J Pediatr Surg. 36(8):1244-7, 2001
3- Albert A, Cruz O, Montaner A, Vela A, et al: [Congenital solid tumors. A thirteen-year review].Cir Pediatr. 17(3):133-6, 2004
4- Mahony R, McParland P: Approaches to the management of antenatally diagnosed congenital tumours.Pediatr Radiol. 39(11):1173-8, 2009
5- Lopez Almaraz R, Villafruela Alvarez C, Rodriguez Luis J, Domenech Martinez E: [Neonatal neoplasms: a single-centre experience]. An Pediatr (Barc). 65(6):529-35, 2006
6- Crocoli A, Bagolan P, Boldrini R, et al: Congenital Askin tumor with favorable outcome: case report and review of the literature. J Pediatr Surg 47(7): 1440-44, 2012

PSU Volume 39 NO 04 OCTOBER 2012

Trocar Injury

Injury by a trocar during a laparoscopic or thoracoscopic procedure is a very serious complication in surgery. The two most serious complications most likely to result in death caused during entrance of a trocar are hemorrhage due to vessel injury and infection due to bowel injury. The rate of trocar related complication is less than 3%. The average incidence of trocar-related vascular injury is 0.1%. Major vessel injury is almost invariably operator error. Most vascular, bowel and local hemorrhage injury are caused during the initial trocar insertion. There is some blind force exertion that causes the blunt/sharp injury to the major vessel. The vessels most frequently involved are the aorta, the iliac arteries, the mesenteric vessels, and the vena cava. Force require to insert reusable trocars is twice that for disposable trocars. Shielded trocars might provide a margin of safety. Trocar use requires considerable training, practice, skill, manual dexterity, adequate muscular strength, knowledge of the associated risks, and careful patient selection. In addition to laparoscopist-related issues (trocar insertion technique, patient selection, injury recognition and effective intervention), the lack of standard device designs, a lack of proven-effective fail-safe features, and failure of patients to report symptoms in a timely manner may also contribute to morbidity and mortality. Open (Hasson) entrance or optical access trocars are recommended for patients with prior abdominal surgery, small children, patients with lower abdomen skin cannot be adequately stabilized for safe insertion or Veress needle.

1- Montero M, Tellado MG, Rios J, Mendez R, Somoza I, Pais E, Vela D: Aortic injury during diagnostic pediatric laparoscopy. Surg Endosc. 15(5):519, 2001
2- Schaefer M, Lauper M, Krahenbahl L: Trocar and Veress needle injuries during laparoscopy. Surg Endosc. 15(3):275-80, 2001
3- Thomas MA, Rha KH, Ong AM, Pinto PA, Montgomery RA, Kavoussi LR, Jarrett TW: Optical access trocar injuries in urological laparoscopic surgery. J Urol. 170(1):61-3, 2003
4- Opitz I, Gantert W, Giger U, Kocher T, Krahenbal L: Bleeding remains a major complication during laparoscopic surgery: analysis of the SALTS database. Langenbecks Arch Surg. 390(2):128-33, 2005
5- Yanke BV, Horowitz M: Safety of the Veress needle in pediatric laparoscopy.  J Endourol. 21(7):695-7, 2007
6- Minervini A, Davenport K, Pefanis G, Keeley FX Jr, Timoney AG: Prospective study comparing the bladeless optical access trocar versus Hasson open trocar for the establishment of pneumoperitoneum in laparoscopic renal procedures. Arch Ital Urol Androl. 80(3):95-8, 2008
7- Nakaoka T, Uemura S, Yoshida T, Tanimoto T, Shiokawa C, Harumoto K: Umbilical center insertion method for initial trocar placement in pediatric laparoscopic surgery. Osaka City Med J. 56(2):21-6, 2010

Alagille Syndrome

Alagille syndrome is an autosomal dominant disorder characterized by paucity of intrahepatic bile ducts, typical facies, congenital cardiac defects (pulmonary stenosis), posterior embryotoxon of the eye and butterfly vertebra arch defect. Due to paucity of  bile ducts the baby presents with inefficient bile excretion causing intrahepatic cholestasis, direct hyperbilirubinemia and hypercholesterinemia. The clinical picture is sometimes indistinguishable from biliary atresia needing cholangiogram and liver biopsy for diagnosis. The vast majority of patients present with jaundice and failure to thrive or cardiovascular symptoms before six months of age. Mutation in the JAG-1 gene of chromosome 20p12 is responsible for more than 90% of cases. There is no cure for Alagille syndrome. The most disturbing manifestation includes pruritus and xanthomas. Most children with Alagille syndrome can be managed conservatively with choleretics (ursodeoxycholic acid), nutrition optimization, fat-soluble vitamin supplementation and medication for pruritus (cholestyramine, rifampin, naltrexone). Surgery is aimed at reducing the pruritus consist of partial external biliary diversion creating a conduit between gallbladder and skin using jejunum, performing an ileal exclusion end to side ileocolostomy between proximal ileum and right colon or partial internal biliary diversion between gallbladder and ascending colon with jejunum. These procedures do not improve growth and does not prevent progression of disease toward liver failure. Liver transplant is used for liver failure. Mortality is approximately 10%, with vascular accidents, cardiac disease, and liver disease accounting for most deaths.

1-Piccoli DA, Spinner NB: Alagille syndrome and the Jagged1 gene. Semin Liver Dis. 21(4):525-34, 2001
2- Spinner NB, Hutchinson AL, Krantz ID, Kamath BM: Alagille Syndrome. In: Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-. 2000 May 19.
3- Kamath BM, Loomes KM, Piccoli DA: Medical management of Alagille syndrome.  J Pediatr Gastroenterol Nutr. 50(6):580-6, 2012
4- Subramaniam P, Knisely A, Portmann B, Qureshi SA, Aclimandos WA, Karani JB, Baker AJ: Diagnosis of Alagille syndrome-25 years of experience at King's College Hospital. J Pediatr Gastroenterol Nutr. 52(1):84-9, 2011
5- Vajro P, Ferrante L, Paolella G: Alagille syndrome: an overview. Clin Res Hepatol Gastroenterol. 36(3):275-7, 2012
6- Sheflin-Findling S, Arnon R, Lee S, Chu J, Henderling F, Kerkar N, Iyer K: Partial internal biliary diversion for Alagille syndrome: case report and review of the literature. J Pediatr Surg. 47(7):1453-6, 2012

Transcutaneous Electrical Stimulation

Transcutaneous electrical stimulation (TES) therapy is a noninvasive form of electrical stimulation used in adults to manage painful musculoskeletal conditions and bladder incontinence. In general electrical stimulation therapy can be delivered via the direct route (invasive) with electrodes implantation in the form of sacral nerve stimulation or the indirect route (noninvasive) with pad electrode placement on the skin surface over the affected area and paraspinal region. The battery-operated stimulator makes home treatment a reality. TES increase defecation frequency and reduce soiling and abdominal pain in constipation. This has prompted using TES as therapy for slow transit constipation in children. TES activates sensory nerve fibers in the spinal nerves in the skin, sensory and motor nerve in the spinal nerves, sympathetic and parasympathetic nerves, enteric nerves in the bowel wall or pacemaker cells in the intestine (cells of Cajal), and intestinal muscle cells. Improvement of constipation has been found in more than two-thirds of all patients lasting more than two years in half of them. TES have reduced the number of surgical procedures (appendicostomy, colectomy, colostomy) done for intractable slow transit constipation improving their quality of life.

1- Clarke MC, Chase JW, Gibb S, Hutson JM, Southwell BR: Improvement of quality of life in children with slow transit constipation after treatment with transcutaneous electrical stimulation. J Pediatr Surg. 44(6):1268-72, 2009
2- Ismail KA, Chase J, Gibb S, Clarke M, Catto-Smith AG, Robertson VJ, Hutson JM, Southwell BR: Daily transabdominal electrical stimulation at home increased defecation in children with slow-transit constipation: a pilot study. J Pediatr Surg. 44(12):2388-92, 2009
3- Yik YI, Clarke MC, Catto-Smith AG, Robertson VJ, Sutcliffe JR, Chase JW, Gibb S, Cain TM, Cook DJ, Tudball CF, Hutson JM, Southwell BR: Slow-transit constipation with concurrent upper gastrointestinal dysmotility and  its response to transcutaneous electrical stimulation. Pediatr Surg Int. 27(7):705-11, 2011
4- Leong LC, Yik YI, Catto-Smith AG, Robertson VJ, Hutson JM, Southwell BR: Long-term effects of transabdominal electrical stimulation in treating children with slow-transit constipation. J Pediatr Surg. 46(12):2309-12, 2011
5- Yik YI, Ismail KA, Hutson JM, Southwell BR: Home transcutaneous electrical stimulation to treat children with slow-transit constipation. J Pediatr Surg. 47(6):1285-90, 2012
6-Yik YI, Leong LC, Hutson JM, Southwell BR: The impact of transcutaneous electrical stimulation therapy on appendicostomy operation rates for children with chronic constipation-a single-institution experience. J Pediatr Surg. 47(7):1421-6, 2012

PSU Volume 39 No 05 NOVEMBER 2012

Slow Transit Constipation

Chronic idiopathic constipation is a distressing problem in children associated with poor appetite, recurrent abdominal pain and irritability. Two types of constipation have been described: slow transit constipation (STC) associated with slow nuclear transit study of the colon, and functional fecal retention (FFR) associated with normal transit but a delay of anorectal release in the presence of ganglion cells. STC children characteristically demonstrate  delayed passage of the first meconium stool beyond 24 hrs of age, symptoms of severe constipation within a year, or treatment-resistant encopresis at 2-3 years, irregular bowel motion, colicky abdominal pain, frequent uncontrollable soiling and softer stools as compared with FFR children who pass hard, infrequent stools. Transverse colon elongation is more common in SCT, whereas sigmoid colon elongation is not more common in FFR.The correlation between STC and deficiency of substance P, an excitatory neuropeptide, in the myenteric axons of the bowel wall implies that this substance is involved in the cause of the dysmotility. Management is different for both conditions. In FFR, transit as far as the rectosigmoid is normal, thus once the rectum is emptied (with enemas or rectal disimpaction)  and behavioral modification is instituted the condition improves. FFR will not improve with colonic resections. With STC long-term management is warranted These children do not benefit from high fiber diet, but can be managed with laxatives and/or enemas. Those with STC that does not improve will need appendicostomy, colostomy or colonic resection. Quality of life is significant lower in children with STC as compared with normal children with both physical and psychosocial functioning score reduced.

1- Wheatley JM, Hutson JM, Chow CW, Oliver M, Hurley MR: Slow-transit constipation in childhood. J Pediatr Surg. 34(5):829-32, 1999
2- Marshall J, Hutson JM, Anticich N, Stanton MP: Antegrade continence enemas in the treatment of slow-transit constipation.  J Pediatr Surg. 36(8):1227-30, 2001
3- Shin YM, Southwell BR, Stanton MP, Hutson JM: Signs and symptoms of slow-transit constipation versus functional retention. J Pediatr Surg. 37(12):1762-5, 2002
4- Cook BJ, Lim E, Cook D, Hughes J, Chow CW, Stanton MP, Bidarkar SS, Southwell BR, Hutson JM: Radionuclear transit to assess sites of delay in large bowel transit in children with chronic idiopathic constipation. J Pediatr Surg. 40(3):478-83, 2005
5- King SK, Sutcliffe JR, Southwell BR, Chait PG, Hutson JM: The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg. 40(12):1935-40, 2005
6- Yik YI, Cook DJ, Veysey DM, Tudball CF, Cain TM, Southwell BR, Hutson JM: How common is colonic elongation in children with slow-transit constipation or anorectal retention?  J Pediatr Surg. 47(7):1414-20, 2012

Intramuscular Hemangioma

The most common type of deep soft-tissue hemangioma occurs within the intramuscular compartment. Most intramuscular hemangiomas (IH) arise within skeletal muscle and occurs within the lower extremity during the first three decades of life. There is a slight female predominance. They are benign lesions histologically classified into capillary, cavernous or mixed types. The most common symptom is pain in the affected muscle region associated with a mass, absent cutaneous changes and neurologic symptoms due to nerve impingement. The most common involved muscle with IH is the quadriceps femoris muscle. Though US and CT-Scans are routinely performed, MRI remains as the imaging of preference for diagnosis (high-signal intensity on T2-weighted images) and extent of local involvement. In MRI the cavernous type is predominantly lobulated and the capillary serpiginous with the mixed type being a combination of both. Angiography can help plan surgical approach for larger lesions and even use preoperative embolization to reduce blood loss. Intramuscular cavernous hemangiomas do not undergo spontaneous regression and may be locally destructive because pressure is exerted on neighboring structures.  Management of IH consists of medical and surgical modality. A trial of propranolol should be tried in small children. Surgery is indicated if there is accelerated growth, intractable pain, functional impairment, local skin necrosis, cosmetic deformity or suspicion of malignancy. To reduce recurrences, surgery consists of wide excision whenever possible avoiding permanent deformity or functional impairment.

1- Saad DF, Shehata BM, Patrick E, Gow KW: Intramuscular hemangioma of the abdominal wall.  J Pediatr Surg. 41(3):601-2, 2006
2-Bella GP, Manivel JC, Thompson RC Jr, Clohisy DR, Cheng EY: Intramuscular hemangioma: recurrence risk related to surgical margins. Clin Orthop Relat Res. 459:186-91, 2007
3- Wu JL, Wu CC, Wang SJ, Chen YJ, Huang GS, Wu SS: Imaging strategies in intramuscular haemangiomas: an analysis of 20 cases. Int Orthop. 31(4):569-75, 2007
4- Melman L, Johnson FE: Intramuscular cavernous hemangioma. Am J Surg. 195(6):816-7, 2008
5- Hashimoto H, Oshika Y, Obara K, Takeshima S, Sato K, Tanaka Y: Intercostal venous hemangioma presenting as a chest wall tumor. Gen Thorac Cardiovasc Surg. 57(4):228-30, 2009
6- Ranero-Juarez AG, Rosales-Galindo VM, Lean-Takahashi AM, Arenas-Guzmn R, Garcia C:  Intramuscular hemangiomas of the extremities: report of six cases. Int J Dermatol. 48(8):875-8, 2009

Branchiogenic Carcinoma

Branchiogenic carcinoma refers to a squamous cell carcinoma that arises from second  branchial cleft cysts. To be labeled as branchiogenic carcinoma strict diagnostic criteria must be met: 1- the location of the tumor in the anatomic region of the branchial cleft cyst or sinus, 2- the histologic appearance of the tumor is consistent with tissue present in a branchial vestige, 3- there is no other evidence of another primary malignancy after exhaustive work-up (including nasopharyngoscopy, laryngoscopy, bronchoscopy and esophagoscopy), and 4- the histologic identification of transition from the normal squamous epithelium of the cyst to carcinoma. The tumor may be papillary within the cyst and will always be accompanied by a significant lymphoid component. Most cases of neck cyst harboring squamous cell carcinoma are metastatic disease from a primary in the head and neck region, and not primary branchiogenic carcinoma. Management of branchiogenic carcinoma includes complete surgical removal with modified radical neck dissection. Postoperative irradiation to the ipsilateral neck is recommended. Chemotherapy is utilized when invasion to surrounding tissue is present.

1- Girgivan MR, Rechdouni AK, Zeger GD: Squamous Cell Carcinoma Arising in a Second Branchial Cleft Cyst. Amer J Clin Oncol 27(1): 96-100, 2004
2- Lin YC, Fang SY, Huang RH: Branchiogenic squamous cell carcinoma: a case report. Oral & Maxillofascial Surg. 33: 209-212, 2004
3- Jereczek-Fossa BA, Casadio C, Jassem J, et al: Branchiogenic carcinoma - conceptual or true clinico-pathological entity? Cancer Treatment Reviews 31: 106-114, 2005
4- Saito T, Sato, Usui H, et al: A Case of Squamous Cell Carcinoma Arising from Branchial Cleft Cyst. Oral Science International 5(2): 135-140, 2008
5- Roche JP, Younes MN, Funkhouser WK, et al: Branchiogenic carcinoma of a first branchial cleft syct. Otolaryng Head & Neck Surg. 143: 167-168, 2010
5- Banikas V, Kyrgidis A, Koloutsos G, et al: Branchial Cyst Carcinoma Revisited: Stem Cells, Dormancy and Malignant Transformation. J of Craniofacial Surg. 22(3): 918-921, 2011

PSU Volume 39 NO 06 DECEMBER 2012

Ovarian Epithelial Tumors

Ovarian epithelial tumors are the second most common ovarian neoplasm in children after germ cell tumors. They arise from the epithelial surface of the ovary representing between 15-20% of all ovarian neoplasms in adolescent females. The histologic subtype of epithelial ovarian tumors in children includes serous and mucinous tumors. Adenocarcinoma of the ovary in children is an extremely rare entity. Epithelial tumors  are further characterized as benign, malignant or of low malignant potential (borderline tumors). Mean age at diagnosis is 13 years. Most children present with history of abdominal pain or an asymptomatic pelvic mass. The most common type of epithelial neoplasm is the benign serous cystadenoma. Borderline epithelial ovarian tumors are defined as epithelial neoplasms of varying level of nuclear atypia, that lacks stromal invasion of the ovary. They are more common in the pediatric age. Preoperative work-up includes imaging (Ultrasound, CT and MRI) and tumor markers (AFP, HCG, LDH, CA-125). Epithelial ovarian tumors frequently cause elevation of the serum level of the CA-125 tumor antigen. During laparotomy strict staging criteria must be met such as collection of ascites/washing, peritoneal surface examination, palpable lymph nodes removal, omentectomy if involved, suspicious contralateral ovarian biopsy and complete resection of the involved ovary intact with sparing of the fallopian tube if not involved (ovarian cystectomy vs. salpingooophorectomy). Fertility preserving surgery is recommended to preserve ovarian function. There is no benefit in removing clinically uninvolved tissue like uterus or contralateral ovary. With mucinous epithelial tumors the appendix should be removed to avoid synchronous lesions.

1- Morowitz M, Huff D, von Allmen D: Epithelial ovarian tumors in children: a retrospective analysis. J Pediatr Surg. 38(3):331-5, 2003
2- Young JL Jr, Cheng Wu X, Roffers SD, Howe HL, Correa C, Weinstein R: Ovarian cancer in children and young adults in the United States, 1992-1997. Cancer. 2003 May 15;97(10 Suppl):2694-700, 2003
3- Borgfeldt C, et al: Fertility-sparing surgery and outcome in fertile women with ovarian borderline
tumors and epithelial invasive ovarian cancer. Eur J Obstet Gynecol Reprod Biol. 134(1):110-4, 2007
4- Stankovic Z, Djuricic S, Djukic M, Jovanovic D, Vasiljevic M: Epithelial ovarian tumors and CA125 in premenarchal girls. Eur J Gynaecol Oncol. 2006;27(6):597-9, 2006
5- Aggarwal A, Lucco KL, Lacy J, Kives S, Gerstle JT, Allen L: Ovarian epithelial tumors of low malignant potential: a case series of 5 adolescent patients. J Pediatr Surg. ;44(10):2023-7, 2009
6- Song T, Choi CH, Lee YY, Kim TJ, Lee JW, Bae DS, Kim BG: Pediatric borderline ovarian tumors: a retrospective analysis. J Pediatr Surg. 45(10):1955-60, 2010
7- Grapsa D, Kairi-Vassilatou E, Kleanthis C, Dastamani C, Fillipidou A, Kondi-Pafiti A: Epithelial ovarian tumors in adolescents: a retrospective pathologic study and a  critical review of the literature. J Pediatr Adolesc Gynecol. 24(6):386-8, 2011

Ovarian Lymphoma

Primary ovarian non-Hodgkin's lymphomas are unusual and accounts for 0.5% of all non-Hodgkin's lymphomas and 1.5% of all ovarian malignancies. The diffuse, large B-cell  lymphoma is the most common type of primary ovarian non-Hodgkin's lymphoma followed by non-endemic Burkitt's lymphoma. It has been suggested the tumor originates from lymphocytes in the ovaries, surrounding blood vessels at the hilum and related to the corpus luteum. Most patient with ovarian lymphoma present with symptoms of abdominal pain, pelvic mass, ascites and elevation of serum CA-125 antigen. This lymphoma has the fastest doubling time of any tumor and it can be as low as 24 hrs.  Presence of positive staining for leukocyte common antigen (LCA) in the histological specimen distinguishes malignant lymphoma from nonlymphoid neoplasm. The MRI findings include solid mass with low signal intensity on T1 and mildly high signal intensity in T2. CT Scan is utilized for staging the disease in the chest, abdomen and pelvis. Bone marrow biopsy is also mandatory for staging. B-cell lymphoma is positive for CD20 and BCL-6.  Children with localized disease to one ovary are best managed with unilateral surgical resection (salpingo-oophorectomy) followed by systemic chemotherapy. Protocol of chemotherapy used in diffuse large B-cell histology is the standard CHOP regimen. Metastasis to the ovaries from another primary site lymphoma should also be considered and they can be solid, cystic and bilateral. Primary ovarian lymphoma has a good prognosis.

1-  Ambulkar I, Nair R: Primary ovarian lymphoma: report of cases and review of literature. Leuk Lymphoma. 44(5):825-7, 2003
2- Crawshaw J, Sohaib SA, Wotherspoon A, Shepherd JH: Primary non-Hodgkin's lymphoma of the ovaries: imaging findings. Br J Radiol. 80(956):e155-8, 2007
3- Vang R, Medeiros LJ, Warnke RA, Higgins JP, Deavers MT: Ovarian non-Hodgkin's lymphoma: a clinicopathologic study of eight primary cases. Mod Pathol. 14(11):1093-9, 2001
4- Elharroudi T, Ismaili N, Errihani H, Jalil A: Primary lymphoma of the ovary. J Cancer Res Ther. 4(4):195-6, 2008
5- Arnogiannaki N, Grigoriadis C, Zygouris D, Androutsopoulos G, Derdelis G, Terzakis E: Primary ovarian non-Hodgkin's lymphoma. Eur J Gynaecol Oncol. 32(4):441-2, 2011
6- Chakrabarti B, Bhaduri B, Barik S, Gupta D, Chakravorty S: Primary ovarian lymphoma in a child.  J Indian Med Assoc. 109(9):679-80, 2011

Diverticulitis in Children

The most common diverticulum in children causing surgical problems is the Meckel's diverticulum. In very rare occasion the pediatric patient can develop diverticular disease of the colon similar to that occurring in the adult. Such diverticular disease can lead to colonic diverticulitis. Genetic disorders in pediatric patients can lead to weakening of the colonic wall and subsequent development of diverticular disease. Diseases with an early predilection for diverticulitis include cystic fibrosis, Ehlers-Danlos syndrome, Marfan syndrome and William-Beuren syndrome. Some of these syndromes have genetic mutations that alter the collagen or elastin levels within tissue creating diverticula in early age. Children with the above syndromes and symptoms of constipation or relapsing or chronic abdominal pain should undergo colonoscopy or barium study to identify such diverticular disease. Stool softener and high-fiber diet can help in the prevention of early complicated diverticular disease.

1- Benya EC, Nussbaum-Blask AR, Selby DM: Colonic diverticulitis causing partial bowel obstruction in a child with cystic fibrosis. Pediatr Radiol. 27(12):918-9, 1997
2- Shin JH, Son BH, Kim H: Clinically distinguishing between appendicitis and right-sided colonic
diverticulitis at initial presentation. Yonsei Med J. 30;48(3):511-6, 2007
3- Bogue CO, Mann EH: Imaging findings in right-sided diverticulitis in a child. Pediatr Radiol. 38(10):1125-7, 2008
4- Garcia MA, Kling KM, Newbury RO, Huang JS: Complicated diverticular disease in a child with williams syndrome. J Pediatr Gastroenterol Nutr. 48(2):233-6, 2009
5- Santin BJ, Prasad V, Caniano DA: Colonic diverticulitis in adolescents: an index case and associated syndromes. Pediatr Surg Int. 25(10):901-5, 2009
6-Ignacio RC Jr, Klapheke WP, Stephen T, Bond S: Diverticulitis in a child with Williams syndrome: a case report and review of the literature. J Pediatr Surg. 47(9):e33-5, 2012

Journal Club