Congenital bronchobiliary fistula: management of the involved hepatic segment.

We present three children with congenital bronchobiliary fistula (CBBF). The first infant underwent resection of the fistula at age 3 weeks and was reported as a success. She returned 7 years later with bilious expectoration for which a second thoracotomy was performed. One year thereafter a bile collection in the right chest required open drainage. Subsequently, the left hepatic lobe was resected. The second child was initially diagnosed as suffering from gastroesophageal reflux. He underwent a fundoplication and a gastrostomy at age 9 months. Persistence of bilious sputum led to the diagnosis of CBBF at age 23 months. The fistula was excised through a right thoracotomy. The distal end was injected demonstrating an arborization involving the entire left hepatic lobe. Since there was no communication with the remaining biliary tree or the intestine, a left hepatectomy was performed. The third child was reported in a communication focusing on the diagnosis of CBBF by hepatobiliary scintigraphy. Her history is almost identical to the second patient. However, instead of a hepatectomy, she underwent a Roux-en-Y drainage of the fistula. The three children had uneventful recoveries and are well. We believe that an abnormal biliary tract should be studied by contrast injection. If no decompressing communication with the normal biliary ducts or the duodenum can be demonstrated, the involved hepatic segment should be resected or internally drained. Children who underwent simple fistula excision must have close long-term follow-up.