Abstract
This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5–15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) post-cholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)–(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.
Similar content being viewed by others
References
Hadjis NS, Collier NA, Blumgart LH (1985) Malignant masquerade at the hilum of liver. Br J Surg 72:659–661
Standfield NJ, Salisbury JR, Howard JR (1989) Benign non-traumatic inflammatory strictures of the extrahepatic biliary system. Br J Surg 76:849–852
Bowles MJ, Salisbury JR, Howard ER (2001) Localized, benign, non traumatic strictures of the extrahepatic biliary tree in children. Surgery 130:55–59
Verbeek PCM, van Leeuwen DJ, de Wit LT et al (1992) Benign fibrosing disease at the hepatic confluence mimicking Klatskin tumors. Surgery 112:866–871
Corvera CU, Blumgart LH, Darvishian F et al (2005) Clinical and pathological features of proximal biliary strictures maequerading as hilar cholangiocarcinoma. J Am Coll Surg 201:862–869
Sylvester FA, Shuckett B, Cutz E (1998) Management of fibrosing pancreatitis presenting in children with obstructive jaundice. Gut 43:715–720
Deshpande AV, LaHeia ER, Shun A et al (2006) Idiopathic fibrosing pancreatitis in children—a single-center experience. J Pediatr Surg 41:1987–1991
Harb L Naon H (2005) Idiopathic fibrosing pancreatitis in a 3 year old: a case report and a review of literature. J Pediatr Surg 40:1335–1340
Scalbas G, Kirschstein T, Waldemer UHL (2002) Juvenile idiopathic fibrosing pancreatitis. Dig Dis Sci 47:1230–1235
Barkin J, Stollman N, Friedman J et al (1994) Idiopathic fibrosing pancreatitis causing obstructive jaundice in young adults: two case reports and a literature review. Am J Gastroenterol 89:2063–2065
Jarnagin WR, Blumgart LH (2007) Benign biliary strictures. In: Blumgart LH (eds) Surgery of the liver, biliary tract and pancreas. Elsevier, Philadelphia, pp 628–654
Strasberg SM, Hertl M, Soper NJ et al (1995) An analysis of the problem of bile duct injury during cholecystectomy. J Am Coll Surg 180:101–123
Sikora SS, Pottakkat B, Srikanth G et al (2006) Postcholecystectomy benign biliary strictures—long-term results. Dig Surg 23:304–312
Bismuth H (1982) Postoperative strictures of biliary tract. In: Blumgart LH (eds) The biliary tract: clinical surgery international. Churchill Livingstone, Edinburgh, pp 209–218
Lillimoe KD, Melton GB, Cameron JL et al (2000) Postoperative bile duct strictures: management and outcome in the 1990s. Ann Surg 232:432–441
Chapman WC, Halevy A, Blumgart LH (1995) Post cholecystectomy bile duct strictures: management and outcome in 130 patients. Arch Surg 130:597–604
Csendes A, Diaz C, Burdiles P (1992) Indications and results of hepaticojejunostomy in benign strictures of biliary tract. Hepatogastroenterology 39:333–336
Teh SH, Pham TH, Lee A (2006) Pancreatic pseudocyst in children: the impact of management strategies on outcome. J Pediatr Surg 41:1889–1893
Warshaw AL, Rattner DW (1985) Timing of surgical drainage for pancreatic pseudocyst. Ann Surg 202:720–724
Warshaw AL, Rattner DW (1980) Facts and fallacies of common bile duct obstruction by pancreatic pseudocysts. Ann Surg 192:33–37
Skellenger ME, Patterson E, Foley NT (1983) Cholestasis due to compression of the common bile duct by pancreatic pseudocysts. Am J Surg 145:343–348
Nealon WH, Townsend CM, Thompson JC (1989) Preoperative endoscopic retrograde cholangiopancreatography (ERCP) in patients with pancreatic pseudocyst associated with resolving acute and chronic pancreatitis. Ann Surg 209:532–538
Karaguzel G, Senocak ME, Nebil B (1995) Surgical management of the pancreatic pseudocyst in children: a long-term evaluation. J Pediatr Surg 30:777–780
Fidias P, Carey WR, Grossbard ML (1995) Non Hodgkin’s lymphoma presenting with biliary tract obstruction—a discussion of seven patients and a review of literature. Cancer 75:1669–1677
Pietsch JB, Shankar S, Ford C (2001) Obstructive jaundice secondary to lymphoma in childhood. J Pediatr Surg 36:1792–1795
Das K, Fisher A, Wilson DJ et al (2003) Primary non-Hodgkin’s lymphoma of the bile ducts mimicking cholangiocarcinoma. Surgery 134:496–500
Saif MW (2006) Primary pancreatic lymphomas. J Pancreas (online) 7:262–273
Ravindra KV, Stringer MD, Prasad KR (2003) Non Hodgkin’s lymphoma presenting with obstructive jaundice. Br J Surg 90:845–849
Leonidas JC, Guelfguat M, Valderrama E (2003) Langerhans’ cell histiocytosis. Lancet 361:1293–1295
Kilborn TN, Teh J, Goodman TR (2003) Paediatric manifestations of Langerhans Cell Histiocytosis: a review of the clinical and radiological findings. Clin Radiol 58:269–278
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Krishna, R.P., Lal, R., Sikora, S.S. et al. Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature. Pediatr Surg Int 24, 183–190 (2008). https://doi.org/10.1007/s00383-007-2087-3
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-007-2087-3