VOLUME 40, 2013

PSU Volume 40 No 01 JANUARY 2013

Von Willebrand's Disease

Von Willebrand's disease (vWD) is the most common inherited bleeding disorder characterized by quantitative or qualitative defects in Von Willebrand factor with a prevalence of 1% in the world population. Von Willebrand factor (vWF) is a large multimeric glycoprotein contained in plasma, platelets and endothelial cells that causes platelet adhesion and aggregation following microvascular injury, and in addition is a carrier for coagulation factor VIII. Patients with vWD manifest dual hemostatic defect characterized by prolonged bleeding time and low plasma level of factor VIII. Deficiency in vWF results in mucocutaneous bleeding, including epistaxis, menorrhagia, and excessive bleeding after trauma or surgery. Classification of vWD is based on the combined results of multiple laboratory tests related to vWF amount and activity as well as the relative amounts of large vWF multimers as determined by gel electrophoresis. vWD is classified as Type 1, seen in 70-80% of all cases, when there is a modest and variable reduction in vWF; Type 2 (20%) if there is qualitative abnormalities in vWF and factor VIII levels; and Type 3 (1-3%) when the patient present near complete absence of vWF. Management of type 1 consists of administration of desmopressin and antifibrinolytics which increases vWF and factor VIII levels. Plasma
derived vWF and factor VIII concentrate is utilized to manage type 2 and 3 patients undergoing major surgery and in patients who are unresponsive or desmopressin is contraindicated. VWF:ristocetin cofactor activity may be useful in evaluating the response to vWD treatment in patients who require replacement therapy.

1- Schneppenheim R: The pathophysiology of von Willebrand disease: therapeutic implications. Thromb Res. 128 Suppl 1:S3-7, 2011
2- O'Brien SH: Common management issues in pediatric patients with mild bleeding disorders. Semin Thromb Hemost. 38(7):720-6, 2012
3- Gill JC, Shapiro A, Valentino LA, Bernstein J, Friedman C, Nichols WL, Manco-Johnson M: von Willebrand factor/factor VIII concentrate (Humate-P) for management of elective surgery in adults and children with von Willebrand disease. Haemophilia. 17(6):895-905, 2011
4- Thompson AR, Gill JC, Ewenstein BM, Mueller-Velten G, Schwartz BA; Humate-P Study Group: Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII/VWF concentrate (Humate-P). Haemophilia. 10(1):42-51, 2004
5- Federici AB, Mannucci PM: Advances in the genetics and treatment of von Willebrand disease. Curr Opin Pediatr. 14(1):23-33, 2002
6- Rodriguez KD, Sun GH, Pike F, Mandel EM, Casselbrant ML, Chi DH: Post-tonsillectomy bleeding in children with von Willebrand disease: a single-institution experience. Otolaryngol Head Neck Surg. 142(5):715-21, 2010
7- Mannucci PM, Chediak J, Hanna W, et al: Treatment of von Willebrand disease with high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study. Blood. 99: 450-456, 2002


With increase availability of ultrasound guidance use of regional anesthesia has increased. Local anesthetic systemic toxicity (LAST) is a complication associated with regional anesthesia difficult to manage and potentially fatal. LAST affect either or both the cardiovascular and central nervous systems depending on the free plasma concentration of the local anesthetic used. This is most commonly the result of intravascular injection causing high blood concentration of the anesthetic. CNS excitation (agitation, auditory change and metallic taste) progresses to seizures or CNS depression (drowsiness, coma, and respiratory arrest). This is followed by CVS excitation (tachycardia, ventricular arrhythmia, and hypertension) then depression (bradycardia, conduction block, asystole, and cardiac depression). Anesthetic cardiotoxicity primarily arises from a blockade of sodium channels.  Aspiration prior to injection and use of intravascular marker such as adrenaline can reduce the incidence of accidental intravascular injection. Bupivacaine, levobupivacaine, and ropivacaine are longer acting and more toxic drugs with bupivacaine being the most cardiotoxic. Plasma concentration depends on amount injected and site of injection. Highest plasma concentration occurs with intercostal, epidural and brachial blocks. Combining anesthetic of rapid onset with longer duration anesthetics can be dangerous as individual safe doses with multiple drugs are unknown. Successful resuscitation of LAST, especially with cardiac collapse, consists of lipid emulsion (intralipid rescue). Proposed mechanisms of action include the intralipid acting as a "lipid sink" extracting the lipophilic local anesthetic from plasma and tissues, interference with sodium channel binding and reversal of the anesthetic induced inhibition of myocardial fatty acid oxidation restoring myocardial ATP supply. Adequate monitoring throughout the procedure is essential to detect early signs of toxicity.

1- Ciechanewicz S, Patil V: Lipid Emulsion for Local Anesthetic Systemic Toxicity. Anesthesiology Research and Practice. Anesthesiol Res Pract. 2012:131784, 2012
2- Kosh MC, Miller AD, Michels JE: Intravenous lipid emulsion for treatment of local anesthetic toxicity. Therapeutics and Clinical Risk Management. 6: 449-451, 2012
3- Dillane D, Finucane BT: Local anesthetic systemic toxicity. Can J Anaesth. 57(4):368-80, 2010
4- Burch MS, McAllister RK, Meyer TA: Treatment of local-anesthetic toxicity with lipid emulsion therapy. Am J Health Syst Pharm. 15;68(2):125-9, 2011
5- Mercado P, Weinberg GL: Local anesthetic systemic toxicity: prevention and treatment. Anesthesiol Clin. 29(2):233-42, 2011
6- Lannqvist PA: Toxicity of local anesthetic drugs: a pediatric perspective. Paediatr Anaesth. 22(1):39-43, 2012
7- Wolfe JW, Butterworth JF: Local anesthetic systemic toxicity: update on mechanisms and treatment. Curr Opin Anaesthesiol. 24(5):561-6, 2011
8- Weinberg GL: Lipid emulsion infusion: Resuscitation for local anesthetic and other drugs overdose. Anesthesiology 117:180-87, 2012


Hypoganglionosis is a very rare entity of intestinal innervation disorder surrounded by a controversial aura of existence. No genetic basis or mutation has been associated with this condition. Hypoganglionosis is reported in 0.3 to 6% of rectal biopsies and mostly diagnosed in the preschool child. Hypoganglionosis is defined as 40% reduction in the number of nerve cells in the bowel wall. Most common presenting symptoms  consist of intestinal obstruction, severe chronic constipation, ileus and enterocolitis. A full-thickness bowel specimen is required for the diagnosis of hypoganglionosis with findings of sparse and small myenteric ganglia, absent or low acetylcholinesterase activity, hypertrophy of muscularis mucosa, mucosa and circular muscle. Interticial cell of Cajal have been reported to be decreased in hypoganglionosis.  Hypoganglionosis is managed similarly to Hirschsprung's disease with resection of the affected bowel and some form of subsequent pull-through procedure. Complications associated with management of hypoganglionosis consist of enterocolitis, chronic constipation, overflow encopresis and need of redo pull-through due to residual disease. Mortality is associated with enterocolitis and short bowel complications.

1- Kubota A, Yamauchi K, Yonekura T, Kosumi T, Oyanagi H, Mushiake S, Nakayama M, Imura K, Okada A: Clinicopathologic relationship of hypoganglionosis. J Pediatr Surg. 36(6):898-900, 2001
2- Rolle U, Yoneda A, Solari V, Nemeth L, Puri P: Abnormalities of C-Kit-positive cellular network in isolated hypoganglionosis. J Pediatr Surg. 37(5):709-14, 2002
3- Zhang HY, Feng JX, Huang L, Wang G, Wei MF, Weng YZ: Diagnosis and surgical treatment of isolated hypoganglionosis. World J Pediatr. 4(4):295-300, 2008
4- Dingemann J, Puri P: Isolated hypoganglionosis: systematic review of a rare intestinal innervation
defect. Pediatr Surg Int. 26(11):1111-5, 2010
5- Watanabe Y, Takasu H, Sumida W: A preliminary report on the significance of excessively long segment congenital hypoganglionosis management during early infancy. J Pediatr Surg. 46(8):1572-7, 2011
6- Puri P, Gosemann JH: Variants of Hirschsprung disease. Semin Pediatr Surg. 21(4):310-8, 2012

PSU Volume 40 No 02 FEBRUARY 2013

Closed Gastroschisis

Gastroschisis is a congenital abdominal wall defect occurring toward the right of the insertion of the umbilical vessels. Bowel and viscera protrude through a full-thickness small defect. Contact of the exposed bowel to the amniotic fluid produces serositis with edema and foreshortening of the bowel. The diagnosis is made prenatally using ultrasound. Most babies are born from young mothers. Closed gastroschisis refers to a small group of gastroschisis (6%) where there is spontaneous antenatal closure of the abdominal ring around the prolapsed intestine. Abdominal ring closure in gastroschisis produces several sequelae such as complete midgut infarction, intestinal resorption, small right-sided mummified midgut remnant, or simple luminal occlusion without vascular impairment. In most cases there is some remnant of the extraabdominal bowel identified to the right of the umbilicus, either a shrunken nonviable mass or and obvious gangrenous bowel of normal length. Needless to say the baby ends with dilated proximal bowel due to the created intestinal atresia and short bowel from the ischemic event. Survival in closed gastroschisis depends on the length of proximal remnant bowel left within the abdominal cavity. Close antenatal monitoring may prevent severe bowel loss in some cases using bowel dilatation as an index of suspicion and performing prenatal birth in such situations. Mortality of closed gastroschisis can approach 75% due to short bowel complications such as sepsis and liver failure.

1- Davenport M, Haugen S, Greenough A, Nicolaides K: Closed gastroschisis: Antenatal and postnatal features. J Pediatr Surg. 36(12):1834-7, 2001
2- Basaran UN, Inan M, Gacer F, Yardim T, Pul M: Prenatally closed gastroschisis with midgut atresia. Pediatr Surg Int. 18(5-6):550-2, 2002
3- Winter LW, Giuseppetti M, Breuer CK: A case report of midgut atresia and spontaneous closure of gastroschisis. Pediatr Surg Int. 21(5):415-6, 2005
4- Vogler SA, Fenton SJ, Scaife ER, et al: Closed gastroschisis: total parenteral nutrition-free survival with aggressive attempts at bowel preservation and intestinal adaptation. J Pediatr Surg. 43: 1006, 1010, 2008
5- Houben C, Davenport M, Ade-Ajayi N, Flack N, Patel S: Closing gastroschisis: diagnosis, management, and outcomes. J Pediatr Surg. 44(2):343-7, 2009
6- Kassa AM, Lilja HE: Predictors of postnatal outcome in neonates with gastroschisis.  J Pediatr Surg. 46(11):2108-14, 2011

Laparoscopic Excision Choledochal Cyst

The management of choledochal cyst entails complete excision of the cyst and reconstruction using roux-en-y hepaticojejunostomy in most cases or hepaticoduodenostomy. During the last ten years there has been a tendency for the procedure to be performed laparoscopically. Laparoscopic excision of choledochal cysts with reconstruction is feasible and can be performed safely with a low intraoperative complication rate. The laparoscopic technique includes excision of the gallbladder and cyst, followed by a Roux-en-Y anastomosis constructed after exteriorization of the small bowel via an infraumbilical trocar incision or intracorporeally. After repositioning of the bowel, an end-to-side hepaticojejunostomy is carried out laparoscopically. Dissection close to the cyst wall is mandatory to prevent injury to the hepatic vessels, especially the portal vein. Hepaticoduodenostomy is s simple procedure than hepaticojejunostomy, with shorter operating time reducing the risk of postop adhesions, can be performed totally laparoscopically giving superior cosmetic results, but carries a higher incidence of postop cholangitis and bile-reflux gastritis. Laparoscopy presents a longer operation duration than laparotomy and requires more instruments. Less blood loss and chances of transfusion is seen in the laparoscopic group due to the improved accuracy provided by the magnified  view. Cases without severe infection, without common hepatic duct or left/right duct strictures, and those without a cyst deeply embedded in the pancreas can be left undrained. In neonates the laparoscopic procedure curtails further complications of the cyst and reverses the derangement of liver function associated with the choledochal cyst.

1- Li L, Feng W, Jing-Bo F, Qi-Zhi Y, Gang L, Liu-Ming H, Yu L, Jun J, Ping W: Laparoscopic-assisted total cyst excision of choledochal cyst and Roux-en-Y hepatoenterostomy.J Pediatr Surg. 39(11):1663-6, 2004
2- Liu SL, Li L, Hou WY, Zhang J, Huang LM, Li X, Xie HW, Cheng W: Laparoscopic excision of choledochal cyst and Roux-en-Y hepaticojejunostomy in symptomatic neonates. J Pediatr Surg. 44(3):508-11, 2009
3- Nguyen Thanh L, Hien PD, Dung le A, Son TN: Laparoscopic repair for choledochal cyst: lessons learned from 190 cases.  J Pediatr Surg. 45(3):540-4, 2010
4- Liuming H, Hongwu Z, Gang L, Jun J, Wenying H, Wong KK, Miao X, Qizhi Y, Jun Z, Shuli L, Li L: The effect of laparoscopic excision vs open excision in children with choledochal cyst: a midterm follow-up study. J Pediatr Surg. 46(4):662-5, 2011
5- Diao M, Li L, Cheng W: To drain or not to drain in Roux-en-Y hepatojejunostomy for children with choledochal cysts in the laparoscopic era: a prospective randomized study. J Pediatr Surg. 47(8):1485-9, 2012
6- Wang B, Feng Q, Mao JX, Liu L, Wong KK: Early experience with laparoscopic excision of choledochal cyst in 41 children. J Pediatr Surg. 47(12):2175-8. 2012

Radiation Exposure

Children are more radiosensitive than adults. The use of CT-Scans in abdominal pain, trauma and a diverse diagnostic entities has increased significantly during the past twenty years. CT-Scans deliver non-uniform radiation doses across the body. Several studies have reported significant increase cancer risk after radiation exposure in the range received from multiple CT-Scans. Since the bone marrow and the brain tissue are very radiosensitive, leukemia and brain tumors are the most common tumors developing after ionizing radiation. For children with normal life expectancy the lifetime excess risk of any incident cancer for a head CT scan is about one cancer per 1000 head CT scans for young children (<5 years), decreasing to about one cancer per 2000 scans for exposure at age 15 years. For an abdominal or pelvic CT scan the lifetime risks for children are one cancer per 500 scans irrespective of age at exposure. Means to reduce this risky situation include using low-dose abdominal CT-Scans and  alternative diagnostic procedures such as ultrasound and MRI when applicable. Ultrasound has been found to be an excellent tool as initial diagnostic modality in abdominal pain suspicion of appendicitis. There is wide agreement that the benefits of an indicated CT scan far outweigh the risks.

1- Kim K, Kim YH, Kim SY, Kim S, Lee YJ, Kim KP, Lee HS, Ahn S, Kim T, Hwang SS, Song KJ, Kang SB, Kim DW, Park SH, Lee KH: Low-dose abdominal CT for evaluating suspected appendicitis. N Engl J Med. 366(17):1596-605, 2012
2- Pearce MS, Salotti JA, Little MP, McHugh K, Lee C, Kim KP, Howe NL, Ronckers CM,  Rajaraman P, Sir Craft AW, Parker L, Berrington de Gonzalez A: Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet. 380(9840):499-505, 2012
3-Calvert C, Strauss KJ, Mooney DP: Variation in computed tomography radiation dose in community hospitals.  J Pediatr Surg. 47(6):1167-9, 2012
4-Freeman K, Strauchler D, Miller TS: Impact of socioeconomic status on ionizing radiation exposure from medical imaging in children. J Am Coll Radiol. 9(11):799-80, 2012.
5- Thirumoorthi AS, Fefferman NR, Ginsburg HB, Kuenzler KA, Tomita SS: Managing radiation exposure in children-reexamining the role of ultrasound in the diagnosis of appendicitis.J Pediatr Surg. 47(12):2268-72, 2012
6- Brody AS, Frush DP, Huda W, Brent RL: Radiation Risk to Children from Computed Tomography. Pediatrics. 120:677-682, 2007

PSU Volume 40 NO 03 MARCH 2013

Malignant Pancreatic Tumors

Malignant tumors originating primarily in the pancreas occur rarely in children. They mostly arise from the exocrine cells of the pancreas, as most endocrine pancreatic tumors are benign. Malignant pancreatic tumors comprise in order of frequency: solid pseudopapillary tumor, pancreatoblastoma, ductal adenocarcinoma, acinar cell carcinoma and a few malignant endocrine tumors. Pancreatic tumors in children most commonly present with a palpable mass or abdominal pain. Jaundice occurs less frequent than in adult. Primary imaging includes ultrasound, CT-Scan, MRI. This can be followed by surgery or fine-needle image-guided biopsy to establish a histological diagnosis and plan further therapy. This second option of biopsy is used when the child present with distant metastasis or an unresectable primary tumor. The two most common malignant pancreatic tumors are the solid pseudopapillary tumor (SPT) and the pancreatoblastoma. Pancreatoblastoma is a more commonly found in the first decade of life and is a more aggressive tumor, while SPT is more common in females during adolescent years and caries a better overall prognosis. About one third pancreaticoblastoma cases are metastatic at the time of diagnosis. These tumors can be responsive to chemotherapy and radiation. Preoperative chemotherapy can successfully decrease tumor size and allow a more complete surgical resection. Adequate management of malignant pancreatic tumor is complete surgical resection. On multivariate analysis, histologic type is the only factor that significantly predicts survival. Patients with poorly differentiated carcinoma shows the worst survival probability. 

1- Shorter NA, Glick RD, Klimstra DS, Brennan MF, Laquaglia MP: Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present. J Pediatr Surg. 37(6):887-92, 2002
2- Tapia B, Ahrens W, Kenney B, Touloukian R, Reyes-Magica M: Acinar cell carcinoma versus solid pseudopapillary tumor of the pancreas in children: a comparison of two rare and overlapping entities with review of the literature. Pediatr Dev Pathol. 11(5):384-90, 2008
3- Yu DC, Kozakewich HP, Perez-Atayde AR, Shamberger RC, Weldon CB: Childhood pancreatic tumors: a single institution experience. J Pediatr Surg. 44(12):2267-72, 2009
4- Park M, Koh KN, Kim BE, Im HJ, Kim DY, Seo JJ: Pancreatic neoplasms in childhood and adolescence. J Pediatr Hematol Oncol. 33(4):295-300, 2011
5- Marchegiani G, Crippa S, Malleo G, Partelli S, Capelli P, Pederzoli P, Falconi M: Surgical treatment of pancreatic tumors in childhood and adolescence: uncommon neoplasms with favorable outcome. Pancreatology. 11(4):383-9, 2011
6- Rojas Y, Warneke CL, Dhamne CA, Tsao K, Nuchtern JG, Lally KP, Vasudevan SA, Hayes-Jordan AA, Cass DL, Herzog CE, Hicks MJ, Kim ES, Austin MT: Primary malignant pancreatic neoplasms in children and adolescents: A 20year experience. J Pediatr Surg. 47(12):2199-204, 2012

Rectovestibular Fistula with Normal Anus

Rectovestibular fistula with a normal anus is a rare anorectal malformation affecting females patients. It is also known as anovestibular fistula or H-type rectovestibular fistula. Is more commonly found in Asia. Most cases present with early infant history of fecal discharge through an external opening in the posterior vestibule of the genitalia. A few will develop a perineal vulvar abscess before the fistula becomes visible. Anal stenosis has been associated with this condition. The embryology of this fistula is though  to represent a persisting cloacal canal or an interruption of the dorsal part of the embryonal cloacal membrane by an isolated defect. The fistula uniformly extends from the vestibular fourchette to the anterior wall of the rectum one to 3 cm above the dentate line. Contrast enemas and endoscopic studies are not very useful as diagnostic aid. The diagnosis is best confirmed during an exam under anesthesia passing a small probe from the vestibule to the internal rectal orifice. Management of rectovestibular fistula has included perineal repair, vestibuloanal pull-through, anterior perineal anorectoplasty, fistulectomy and limited posterior sagittal anorectoplasty. To avoid a colostomy bowel preparation and systemic antibiotics are necessary. Most common complications are recurrence and wound deshicence. Fistula recurrence occurs when blood perfusion in the local rectal tissue is poor and separation of the divided ends of the fistula fails. It is important to close the fistula orifice using healthy anterior rectal wall.

1- Tsugawa C, Nishijima E, Muraji T, Satoh S, Kimura K: Surgical repair of rectovestibular fistula with normal anus.J Pediatr Surg. 34(11):1703-5, 1999
2- Yazici M, Etensel B, Gursoy H, Ozklsaclk S: Congenital H-typ anovestibuler fistula. World J Gastroenterol 9(4): 881-882, 2003
3- Banu T, Hannan MJ, Hoque M, Aziz MA, Lakhoo K: Anovestibular fistula with normal anus. J Pediatr Surg. 43:526-529, 2008
4- Li L, Zhang TC, Zhou CB, Pang WB, Chen YJ, Zhang JZ: Rectovestibular fistula with normal anus: a simple resection or an extensive perineal dissection? J Pediatr Surg. 45:519-524, 2010
5- Kim SM, Park YJ, Ahn SM, Oh JT, Han SJ: Infantile vulvar abscess with a normal anus: a suspicious sign of rectovestibular fistula. Yonsei Med J. 51(5):717-21, 2010
6- Lawal TA, Chatoorgoon K, Bischoff A, Pena A, Levitt MA: Management of H-type rectovestibular and rectovaginal fistulas. J Pediatr Surg. 46:1226-1230, 2011

Duodenum Inversum

Duodenum inversum refers to a congenital anomaly where the third portion of the duodenum is located to the right of the second portion or above the duodenal bulb. In other words the third portion instead of continuing leftward toward Treitz, reverses direction and travels in a superior posterior track prior to crossing the midline above the pancreas. Most cases occur in male adults. Duodenum inversum is associated with nonspecific symptoms such as epigastric discomfort, nausea, distension or duodenal obstruction. Other associated conditions include cholelithiasis, pancreatitis and annular pancreas. The condition can mimic mesenteric artery syndrome. Diagnosis is confirmed with UGIS. Management of duodenum inversum without obstruction is conservative (antacids, antispasmodics). With duodenal obstruction surgery may be required. The obstruction is due to fibrotic bands. Lysis can be performed laparoscopically.

1- Childress MH: Duodenum inversum. J Natl Med Assoc. 71(5):515-6, 1979
2- Long FR, Kramer SS, Markowitz RI, Taylor GE, Liacouras CA: Intestinal malrotation in children: tutorial on radiographic diagnosis in difficult cases. Radiology. 198(3):775-80, 1996
3- Long FR, Mutabagani KH, Caniano DA, Dumont RC: Duodenum inversum mimicking mesenteric artery syndrome. Pediatr Radiol. 29(8):602-4, 1999
4- Azhough R, Bayat A, Hashemzadeh S, Khaki AA, Motayagheni N, Tarzamni MK: The combination of annular pancreas and duodenum inversum presenting with delayed gastric emptying, pain, and feeding intolerance. Am J Gastroenterol. 104(5):1328-9, 2009
5- Kim ME, Fallon SC, Bisset GS, Mazziotti MV, Brandt ML: Duodenum inversum: A report and review of the literature. J Pediatr Surg. 48(1):E47-E49, 2013

PSU Volume 40 No 04 APRIL 2013

Trunk Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children comprising one half of pediatric soft tissue sarcomas and 15% of all pediatric solid tumors. RMS can arise anywhere in the body and survival has been found highly dependent on such variables as tumor site, extent of disease and management. RMS arising in the trunk area is associated with a poor prognosis. The trunk includes chest wall, abdomen and paraspinal region. Trunk RMS presents as a painless, firm mass that increases rapidly in size. The diagnosis is made by incisional or excisional biopsy of the mass. Most patients with trunk RMS have embryonal histology. Adolescent patients with trunk RMS fare worse than younger children. This is due to the high incidence of unfavorable (alveolar) histology in adolescent cases. Factors associated with unfavorable outcomes after managing trunk RMS includes tumor size greater than 5 cm,  advance stage at presentation, alveolar histology, lymph node involvement, and local and distant tumor recurrence. Complete surgical resection including the full-thickness of the abdominal or chest wall when feasible with clear tumor margins is the cornerstone of effective and curative management. The abdominal wall is a site at which tumors can be resected widely with minimal morbidity and the majority of wounds can be closed primarily or with substitution using a biologic mesh. When upfront surgery is not feasible, neoadjuvant chemotherapy followed by delayed surgical resection should be considered. Patient with bulk residual tumor remaining after surgery or metastatic disease requires adjuvant therapy (radiotherapy and chemotherapy).   

1- Beech TR, Moss RL, Anderson JA: What comprises appropriate therapy for children/adolescents with rhabdomyosarcoma arising in the abdominal wall? A report from the Intergroup Rhabdomyosarcoma Study Group. J Pediatr Surg. 34(5): 668-671, 1999
2- Tabrizi P, Letts M: Childhood rhabdomyosarcoma of the trunk and extremities. Am J Orthop (Belle Mead NJ). 28(8):440-6, 1999
3 Chui CH, Billups CA, Pappo AS, Rao BN, Spunt SL: Predictors of outcome in children and adolescents with rhabdomyosarcoma of the trunk--the St Jude Children's Research Hospital experience. J Pediatr Surg. 40(11):1691-5, 2005
4- Hayes-Jordan A, Stoner JA, Anderson JR, Rodeberg D, Weiner G, Meyer WH, Hawkins S, Arndt CA, Paidas C; Children's Oncology Group: The impact of surgical excision in chest wall rhabdomyosarcoma: a report from the Children's Oncology Group. J Pediatr Surg. 43(5):831-6, 2008
5- Raney RB, Anderson JR, Brown KL, Huh WW, Maurer HM, Meyer WH, Parham DM, Rodeberg
DA, Wolden SL, Donaldson SS; Soft-Tissue Sarcoma Committee of the Children's Oncology Group Arcadia California USA. Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984-1997: a report from the Children's Oncology Group. Pediatr Blood Cancer. 55(4):612-6, 2010

Gorham's Disease

Gorham's disease is a very rare lymphatic vascular malformation involving bone and leading to massive osteolysis. The disease is also known as vanishing bone syndrome or Gorham-Stout syndrome. The cause of the disease is not known but there is evidence that massive osteolysis is associated with increased osteoclast activity and increase sensitivity of osteoclast precursors to interleukin 6 which promotes bone resorption. There is no gender or race predilection. Gorham's disease affects more commonly adolescent and young adults. The disease mainly affects the shoulder, pelvis, and skull crossing joint boundaries. Mandible, ribs, scapula, humerus, pelvis and femur can be involved. The child presents with dull pain, swelling of the affected area and pathological fractures. Involvement of pleura and vertebrae is associated with poor prognosis since it leads to chylothorax and nerve root compression. Chylothorax occurs in one-fifth of all cases. Diagnosis of Gorham's disease is difficult. MRI shows evidence of massive osteolysis. The affected bone undergoes resorption replaced by hyper vascular fibrous tissue. Histology shows a non malignant proliferation of dilated thin walled vascular and lymphatic channels with associated loss of bone matrix. Splenic lesions and soft-tissue involvement are common in patients with Gorham's disease. Management of Gorham's disease includes sclerotherapy, immunotherapy, radiotherapy and surgery. Surgery to replace the affected bone with a graft or prothesis has also been used successfully in early and localized disease. Death occurs due to malnutrition, lymphocytopenia or infection.     

1- Szabo C, Habre W: Gorham syndrome: anaesthetic management. Anaesthesia. 55(2):157-9, 2000
2- Prasanna R, Sankar J, Ramachandran P: Gorhams disease: vanishing bone syndrome. Indian Pediatr. 46(3):255-6, 2009
3- Venkatramani R, Ma NS, Pitukcheewanont P, Malogolowkin MH, Mascarenhas L: Gorham's disease and diffuse lymphangiomatosis in children and adolescents. Pediatr Blood Cancer. 56(4):667-70, 2011
4- Wells K, Gray Hazard FK: Gorham's disease: diagnostic utility of an autopsy for a rare bone disease. J Pediatr Health Care. 25(6):391-8, 2011
5- Kotecha R, Mascarenhas L, Jackson HA, Venkatramani R: Radiological features of Gorham's disease. Clin Radiol. 67(8):782-8, 2012
6- Situma M, Alexander A, Weiselthaler N, Millar A: An aggressive lymphatic malformation (Gorham's Disease) leading to death of a child.  J Pediatr Surg. 48(1):239-42, 2013

Laparoscopic Peritoneal Cannula Placement

Peritoneal dialysis is preferred to hemodialysis to manage chronic renal failure in children because it has a lower incidence of serious complications, is more cost-effective and improves patient nutrition and independence. The peritoneal cannula for such purposes can be place using an open conventional surgical technique, percutaneously or laparoscopically. Laparoscopic placement of peritoneal dialysis catheters or revision allows complete visualization of the peritoneal cavity placing the catheter under direct vision, preferably in the pelvis. Suturing the catheter tip into the pelvis is associated with a low rate of catheter migration. In addition a more complete omentectomy can be performed and lysis of adhesions can be accomplished to increase the peritoneal absorptive surface. Omentectomy is beneficial during placement of peritoneal dialysis catheters to prevent blockage of the catheter. Laparoscopy can allow for the rescue of block catheters in cases of revisions due to malfunction. Others procedures that can be done concomitantly using the laparoscopic technique include repair of inguinal hernias, gastrostomy tube placement, kidney biopsy and cholecystectomy. Peritoneal dialysis is delayed as long as possible to allow for healing of the incision to prevent leakage. Other recognize benefits of the laparoscopic technique include better cosmetic results, less postoperative pain and shorter hospital stay. 

1- Mattioli G, Castagnetti M, Verrina E, Trivelli A, Torre M, Jasonni V, Perfumo F: Laparoscopic-assisted peritoneal dialysis catheter implantation in pediatric patients. Urology. 2007 Jun;69(6):1185-9.
2- Lu CT, Watson DI, Elias TJ, Faull RJ, Clarkson AR, Bannister KM: Laparoscopic placement of peritoneal dialysis catheters: 7 years experience. ANZ J Surg. 2003 Mar;73(3):109-11
3- Carrillo SA, Ghersi MM, Unger SW: Laparoscopic-assisted peritoneal dialysis catheter placement: a microinvasive technique. Surg Endosc. 2007 May;21(5):825-9.
4- Skipper K, Dickerman R, Dunn E: Laparoscopic placement and revision of peritoneal dialysis catheters. JSLS. 1999 Jan-Mar;3(1):63-5
5- Lee M, Donovan JF: Laparoscopic omentectomy for salvage of peritoneal dialysis catheters. J Endourol. 2002 May;16(4):241-4.
6- Stringel G, McBride W, Weiss R: Laparoscopic placement of peritoneal dialysis catheters in children. J Pediatr Surg. 2008 May;43(5):857-60

PSU Volume 40 NO 05 MAY 2013


Hibernoma is a rare benign lipomatous tumor of brown fat origin initially described as a pseudolipoma. The name hibernoma was used to characterize the resemblance of this tumor to the brown fat found in hibernating animals. Brown fat has a thermoregulatory role in neonates and fetus. Hibernoma can develop in sites where brown fat is still present in humans such as interscapular region, axilla, neck, mediastinum, periaortic area, retroperitoneum and peripherally in the limbs. Findings brown fat cells in a lipomatous tumor is diagnostic of hibernoma. Malignant potential has not been demonstrated. Clinically, hibernomas present as a slow growing painless solitary mass. They produce symptoms by virtue of compression of adjacent structures or by torsion if the developed a pedicle. Most hibernoma are found in young adults with an average age of 38 years. Though rare in the pediatric age, hibernomas can affect the thigh, back, chest, neck, breast, abdominal wall and spine of children. The diagnosis of hibernoma by imaging is difficult. CT and MRI are used indistinctly showing a well-defined hypointense mass with septations that enhances with IV contrast. They do not have increased FDG uptake on PET imaging. Malignancy cannot be excluded safely by imaging modalities. Standard of care for managing hybernoma is complete surgical excision. Recurrence is very rare after complete excision of this tumorous mass.

1- Baskurt E, Padgett DM, Matsumoto JA: Multiple hibernomas in a 1-month-old female infant. AJNR Am J Neuroradiol. 25(8):1443-5, 2004
2- Hardes J, Scheil-Bertram S, Hartwig E, Gebert C, Gosheger G, Schulte M: Sonographic findings of hibernoma. A report of two cases. J Clin Ultrasound. 33(6):298-301, 2005
3- Yalniz E, Alicioglu B, Oz Puyan F: Hibernoma: a benign lipomatous tumor mimicking liposarcoma. J BUON. 13(1):127-9, 2008
4- Whitaker JK, Motiwale S: Hibernoma in a 16-year-old boy. Scott Med J. 56(1):59, 2011
5- Papathanassiou ZG, Alberghini M, Taieb S, Errani C, Picci P, Vanel D: Imaging of hibernomas: A retrospective study on twelve cases. Clin Sarcoma Res. 25;1(1):3, 2011
6- Guidry CA, McGahren ED, Rodgers BM, Kane BJ: Pediatric cervicomediastinal hibernoma: a case report. J Pediatr Surg. 48(1):258-61, 2013

Pulse Oximeter Probe Injuries

The pulse oximeter is an essential noninvasive tool developed to monitor the arterial hemoglobin saturation of children and adults. It as essential device routinely used in the intensive care units, operating rooms, emergency rooms, recovery rooms and during transfer of patients. The oximeter probe has two components: a light source that uses a light emitting diode reflecting direct red and infrared light into the skin, and a photodetector that measures the absorption of light by hemoglobin. As any device in medicine, injury can result from the continuous use of the oximeter probe. The most common injury is an electrical burn caused by a damage probe or a short circuit. Other injuries include thermal burns due to overheating of the light emitting diode of the probe, distal ischemia with digit gangrene owning to pressure of the probe, dermatologic reaction from the chemicals in the probe, skin necrosis and digital sensory loss. Thinner skin, lower fat content and high water content makes the pediatric population more predisposed to such injury with deeper and more extensive damage. Low cardiac output, poor peripheral circulation, vasopressor therapy, hypotension, hypoxia, hypothermia and arterial cannulation of the affected extremity are considered associated factors implicated in the pathogenesis of pulse oximeter probe induced finger injury. Severely ill patients, as indicated by their need for more aggressive vasopressors, are more likely to develop pulse oximeter induced digital injury. Routine examination and rotation of the probe every 4 to 6 hours as a prevention measure is essential to avoid such injury. Capillary filling time should be checked and noted in the record.

1- Chemello PD, Nelson SR, Wolford LM: Finger injury resulting from pulse oximeter probe during orthognathic surgery. Oral Surg Oral Med Oral Pathol. 69(2):161-3, 1990
2- Kohjiro M, Koga K, Komori M, Fukutome T, Yasaka B, Urakami H: [A case report of a burn produced by the probe of a pulse oximeter]. Masui. 41(12):1991-3, 1992
3- Lin CW, Wang HZ, Hsieh KS: Pulse oximeter-associated toe injuries in a premature neonate: a case report.  Zhonghua Yi Xue Za Zhi (Taipei). 62(12):914-6, 1999
4- Wille J, Braams R, van Haren WH, van der Werken C: Pulse oximeter-induced digital injury: frequency rate and possible causative factors. Crit Care Med. 28(10):3555-7, 2000
5- Punj J, Jaryal A, Mahalingam S, Mukundan C, Pandey R, Darlong V, Chandralekha: Toe gangrene in an infant subsequent to application of adult-type pulse oximeter probe for 10 min. J Anesth. 24(4):630-2, 2010
6- Ceran C, Taner OF, Tekin F, Tezcan S, Tekin O, Civelek B: Management of pulse oximeter probe-induced finger injuries in children: report of two consecutive cases and review of the literature. J Pediatr Surg. 47(11):e27-9, 2012

Pterygium Colli

Pterygium colli or better known as web neck is a physical characteristic of several syndromes including Ulrrich-Turner, Klippel-Feil and Escobar syndrome. Pterygium colli is characterized by bilateral webbing of the neck extending from the mastoid to the acromion. The webbing in the neck is caused by fibrotic bands in the fascia colli superficialis which has showed muscular elements in postmortem specimens. Other cases have an excess of skin. Webbing is the result of large bilateral subcutaneous blebs and cysts particularly of the neck which obliterate throughout the course of fetal development, leading to cutis laxa in the newborn and by further shrinkage and scarring of the subcutaneous tissue to the true webbing of the neck. Correction of this deformity can be accomplished by ellipsoid excision of the skin, excision of the fibrous bands and multiple z-plasty reconstruction of the excess skin. The operative procedure is a purely elective intervention and most of these patients do not have any functional impairment. It is recommended the procedure be done before the child begins school to avoid peer pressure and stigma. The most common complication is hypertrophied scarring, but cosmetic results in general are very good.

1- Doletskia SIa, PolonetskiaVA: [Surgical correction of pterygium colli in children with hereditary pathology]. Khirurgiia (Mosk). (7):37-41, 1988
2- Rossillon D, De Mey A, Lejour M: Pterygium colli: surgical treatment.Br J Plast Surg. 42(2):178-83, 1989
3- Hoffmann J, Cornelius CP, Schwenzer K, Schwenzer N: [Possibilities for correcting the neck profile in pterygium colli]. Mund Kiefer Gesichtschir. Suppl 1:S141-3, 1997
4- Leandris M, Ricbourg B: [Surgical treatment of pterygium colli. A case report and review of the
literature]. Ann Chir Plast Esthet. 42(6):615-22, 1997
5- Posso CM, Wolff GA, Suarez LD: [Pterigium colli: secondary surgical correction of one severe case]. Ann Chir Plast Esthet. 57(3):299-303, 2012
6- Reichenberger MA, Goertz O, Lehnhardt M, Germann G, Ryssel H, Czermak C, Kolbenschlag J: Surgical correction of pterygium colli. J Pediatr Surg. 48(2):464-9, 2013

PSU Volume 40 NO 06 JUNE 2013

Desmosis Coli

Constipation is a very serious condition in children. Medical manageable conditions associated with chronic constipation can be classified into normal transit constipation, slow transit constipation and disorders of defecation with retention in the rectum. The most common, slow transit constipation, can be caused by genetic links, dysfunctional enteric nerves, decrease levels of substance P, reduced numbers of interticial cells of Cajal and disorders of connective tissue synthesis. Desmosis coli is a disturbance of the intramural connective tissue mesh network of the colonic wall which leads to a hypoperistalsis syndrome with chronic constipation in the absence of any anomaly of the vegetative gut innervation. The peristaltic movement of the gut is a function of the alternating contraction and relaxation of circular and longitudinal muscles. This movement is induced by a tendon-like connective-tissue net in the circular and longitudinal muscles, which are both rooted in a connective-tissue plexus layer. This connective tissue layer is not developed in children with desmosis coli. Clinically, the child develops hypoperistalsis or aperistalsis with massive elongation and dilatation of the colon. Hirschsprung's disease can coexist with desmosis coli as a familiar trait. The enteric nervous system is normal or near-normal in the affected areas of desmosis coli. Two major subtypes of desmosis can be distinguished: the rare congenital (primary) aplastic desmosis of childhood, and the more common atrophic (secondary) desmosis of adulthood typically incomplete and associated with a hypoperistaltic syndrome. Seromuscular biopsies are required to prove desmosis in gastrointestinal dysmotility disorders. Management of desmosis coli is controversial.

1- Meier-Ruge WA, Holschneider AM, Scharli: New pathogenetic aspects of gut dysmotility in aplastic and hypoplastic desmosis  of early childhood. Pediatr Surg Int. 17(2-3):140-3, 2001
2- Meier-Ruge WA: Desmosis of the colon: a working hypothesis of primary chronic constipation.
Eur J Pediatr Surg. 8(5):299-303, 1998
3- Marshall DG, Meier-Ruge WA, Chakravarti A, Langer JC: Chronic constipation due to Hirschsprung's disease and desmosis coli in a family. Pediatr Surg Int. 18(2-3):110-4, 2002
4- Habner U, Meier-Ruge W, Halsband H: Four cases of desmosis coli: severe chronic constipation, massive dilatation of the colon, and hypoperistalsis due to of changes in the colonic connective-tissue net. Pediatr Surg Int. 18(2-3):198-203, 2002
5- Meier-Ruge WA, Bruder E: [The morphological characteristics of aplastic and atrophic desmosis of the intestine]. Pathologe. 28(2):149-54, 2007
6- Bruhin-Feichter S, Meier-Ruge W, Martucciello G, Bruder E: Connective tissue in gut development: a key player in motility and in intestinal  desmosis. Eur J Pediatr Surg. 22(6):445-59, 2012

Transfer Burn Center

Burns are a major source of injury in children that must be managed within the confines of specialized centers whenever possible to obtain best results in morbidity, mortality and rehabilitation. Burns are classified as First degree (partial thickness, superficial, red, sometimes painful), Second degree (partial thickness, skin may be red, blistered, swollen, very painful), or Third degree (full-thickness, whitish, charred, translucent, no pin prick sensation in burned area). The American Burn Association has established strict criteria for admission or transfer of infants and children to such regional burn centers. They include: 1- Children with less than 10 years of age with burns compromising more than 10% of total body surface area. 2- Children with age above the 10 years with more than 20% total body surface area burned. 3- Children of all ages with full-thickness burn above the 5% of total body surface area. 4- Children with evidence of inhalation injury. 5- Burn to face, eyes, ears, genitalia or joints. 6- Any burn associated with a major fracture or significant traumatic injury. 7- Third degree burns in any age group. 8- Chemical burns. 9- Electrical burns. 10- Burn injury in patients with preexisting medical disorders that could complicate management, prolong recovery, or affect mortality. 10- Burned children in hospitals without qualified personnel or equipment for the care of children. 11- Burn injury in patients who will require special social, emotional, or rehabilitative intervention.  


Rapunzel Syndrome

Bezoars are concretion of human or vegetable fibers that accumulate in the gastrointestinal tract. The most common type of bezoar is the trichobezoar which is mostly made of hair or hairlike fibers. Trichobezoars most commonly present during adolescent years and during the second decade of life. Most cases of trichobezoars occur in female patients. Eating hair denotes an underlying psychiatry disorder. Most trichobezoars lodge within the body of the stomach. When the trichobezoar extends beyond the pyloric muscle and beyond as a tail is called Rapunzel syndrome. The distal end of the bezoar may be in the jejunum, ileum or the colon Most children with trichobezoars suffer from psychiatry disorders including trichotillomania (pulling out of their own hair) and trichophagia (eating of hair). Hair is retained in the folds of the gastric mucosa, and as it accumulates the peristalsis creates an enmeshed ball. The stomach dilates significantly. Decomposition and fermentation of fats give the bezoar and the child breath a putrid smell. The black color of the bezoar is due to the effect of acid on hair protein denaturalization. Most cases are asymptomatic for a long period of time. Most common presenting signs are abdominal pain, nausea, vomiting, obstruction and peritonitis. Other times children present with anorexia, weight loss, hematemesis or intussusception. Bezoars can cause gastric ulceration, obstructive jaundice, acute pancreatitis, and gastric emphysema. Imaging (UGIS, Ultrasound and CT-Scan) shows the bezoar as a mass or filling defect and are diagnostic. Endoscopic retrieval of the bezoar is rarely a definitive treatment. Management of trichobezoar includes removal by gastrotomy and/or enterotomy and behavioral therapy to avoid recurrence. 

1- Ventura DE, Herbella FA, Schettini ST, Delmonte C: Rapunzel syndrome with a fatal outcome in a neglected child. J Pediatr Surg. 40(10):1665-7, 2005
2- Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, Sharma AK: Rapunzel syndrome reviewed and redefined. Dig Surg. 24(3):157-61, 2007
3- Gonuguntla V, Joshi DD: Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar. Clin Med Res. 7(3):99-102, 2009
4- Crawley AJ, Guillerman RP: Rapunzel syndrome. Pediatr Radiol. 40 Suppl 1:S100, 2010
5- Kohler JE, Millie M, Neuger E. Trichobezoar causing pancreatitis: first reported case of Rapunzel syndrome in a boy in North America. J Pediatr Surg. 47(3):e17-9, 2012
6- Fallon SC, Slater BJ, Larimer EL, Brandt ML, Lopez ME. The surgical management of Rapunzel syndrome: a case series and literature review. J Pediatr Surg. 48(4):830-4, 2013

Journal Club