A previously well 3-month-old girl presented with diarrhea, progressive pale stools, weight loss, and jaundice. Preliminary investigations showed obstructive cholestatic jaundice with increased γ-glutamyl transferase activity, conjugated bilirubin, and bile salts. A magnetic resonance cholangiography showed dilation of the main bile duct with a sudden narrowing in the distal segment and no signs of biliary stones and extrinsic compression (Fig. 1).
Intraoperative cholangiography (Fig. 2) confirmed the presence of a distal choledochal stricture without signs of choledochal cysts. A duodenotomy was performed with transpapillary dilatation of the stenotic segment. Subsequently a cholecystectomy was carried out and a T tube was positioned via the remnant of the cystic duct to drain the bile and to stent the dilated segment. No sludge or stones were obtained after the CBD dilatation. After 1 month, T tube was removed without complications, and the results of follow-up ultrasound studies showed no dilation of the biliary tract. Liver blood test quickly normalized and remained stable after T tube removal. At last follow-up visit, 9 months after surgery, total bilirubin was 0.51 mg/dL, conjugate bilirubin was 0.03 mg/dL, and γ-glutamyl transferase activity was 8 U/L.
In our patient, biliary atresia, choledochal cyst, and extrinsic compression were excluded by radiologic studies. Inflammatory choledochal stricture has been described in infants (1–3), but it seems unlikely in our patient given the precocious age at presentation and the site of stricture. Isolated congenital distal choledochal stricture is the most probable diagnosis (4). Our case is one of the few reported and highlights the need for a conservative approach in such a rare and not well-known disease.
1. Krishna RP, Lal R, Sikora SS, et al. Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature. Pediatr Surg Int
2. Standﬁeld NJ, Salisbury JR, Howard JR. Benign non-traumatic inﬂammatory strictures of the extrahepatic biliary system. Br J Surg
3. Bowles MJ, Salisbury JR, Howard ER. Localized, benign, non traumatic strictures of the extrahepatic biliary tree in children. Surgery
4. Vázquez Rueda F, Paredes Esteban RM, Escassi Gil A, et al. Isolated congenital stenoses of the extrahepatic bile ducts. Cir Pediatr