A 7-year-old boy underwent combined en bloc liver–small bowel transplantation (LSbTx) for intestinal failure secondary to extensive Hirschsprung disease. On day 43 after transplant, elevated γGT (glutamyl transpeptidase), bilirubin, alkaline phosphatase, and transaminase levels were recorded. Liver biopsy specimen revealed a moderate liver graft rejection that responded well to methylprednisolone bolus. However, cholestasis persisted and increased. Infectious causes, as well as a hepatic or intestinal rejection, were excluded. Liver biopsy at that time showed ductular proliferation. Cholangio-magnetic resonance imaging and percutaneous cholangiogram (Fig. 1A,B) revealed the origin of this rare complication after combined LSbTx.
1. What is this rare complication?
A. Duplication of the choledochus
B. Choledochal cyst
D. Bile leakage
After surgery, cholestasis increased, accompanied by fever and elevated C-reactive protein level and erythrocyte sedimentation rate. A small bowel follow-through (Fig. 2) clearly revealed the postoperative difficulty.
2. What was the complication after surgery?
E. Cholangitis secondary to an enterobiliary reflux
F. Cholangitis secondary to a foreign body
G. Cholangitis secondary to a congenital malformation of the transplant
1. Answer C and D: Choledocholithiasis with subsequent bile leakage
2. Answer E: Cholangitis secondary to an enterobiliary reflux
Combined LSbTx in this child was complicated by the occurrence of choledocholithiasis (arrow) and subsequent bile leakage, clearly seen in Figure 1. This is an extremely rare complication of combined LSbTx because the normal biliary anatomy remains intact during surgery (1). As an explanation, necrosis of the bile epithelium secondary to the surgical washing during organ preparation or sludge are proposed (1,2). A biliary enteric anastomosis (Roux-en-Y loop) was created using the transplanted small bowel. However, this procedure was immediately complicated by episodes of recurrent cholangitis caused by a major enterobiliary reflux, visible on the small bowel follow-through. The anatomy is unusual on that plane because the barium column fills at the same time the patient's own duodenal C-loop and the duodenal C of the intestinal transplant. After reconfiguration of the anastomosis with lengthening of the Roux-en-Y loop, the patient experienced no additional infectious episodes of the biliary tract. At no time did hepatic rejection complicate the course of this patient.
1. Sudan DL, Iyer KR, Deroover A, et al. A new technique for combined liver/small intestinal transplantation. Transplantation 2001; 72:1846–8.
2. Goulet O, Lacaille F, Jan D, et al. Intestinal transplantation: indications, results and strategy. Curr Opin Clin Nutr Metab Care 2000; 3:329–38.