Non-traumatic extrahepatic biliary perforation: a diagnostic uncertainty and management dilemma

Spontaneous or non-traumatic perforation involving the wall of the extrahepatic or intrahepatic biliary tree is a rare entity leading to difficulty and delay in preoperative diagnosis and resulting in 30% to 50% mortality in spite of adequate surgical therapy and postoperative intensive care. Multiple factors are involved in its pathogenesis in both adults and infants. Patients can present with acute or insidious onset. Diagnostic modalities include ultrasound, abdominal CECT and radionuclide hepatobiliary scan. However, in the presence of clinical features of generalized peritonitis, surgery should not be unduly delayed due to non-availability of these diagnostic tools. A high index of suspicion and an early surgical intervention are the mainstays of therapy. Surgical management remains controversial. The nature and extent of surgery, both by open exploration and laparoscopy, should be tailored according to the patient’s general condition, the severity of the peritonitis, and intraoperative and cholangiographic findings.