Choledochal Cyst and Right Congenital Diaphragmatic Hernia: When to Intervene?

Patients with congenital diaphragmatic hernia (CDH) can present with other congenital anomalies, but an associated choledochal cyst (CC) has rarely been described. The simultaneous diagnosis of both anomalies complicates patient management. There is no consensus on the ideal timing for CC excision. Unrepaired CC is associated with risks of developing biliary sludge, choledocholithiasis, and cholangitis. After a CDH repair with mesh, secondary bacterial translocation caused by a delayed CC repair could lead to mesh superinfection. Conversely, early CC surgical management could cause mesh displacement and lead to CDH recurrence, requiring reintervention. We present the rare case of a CC occurring in a neonate with a prenatally diagnosed right CDH. One month after an uncomplicated CDH repair with mesh, while the patient was still hospitalized for pulmonary hypertension, she developed progressive cholestasis and acholic stools. Investigations revealed a nonpreviously suspected CC. Conservative treatment was attempted, but CC perforation with secondary biliary peritonitis occurred. Open CC excision with a Roux-en-Y hepaticojejunostomy was therefore performed on day of life (DOL) 41. Having suffered no short-term surgical complications, the patient was discharged on DOL 83 because of prolonged ventilatory support due to pulmonary hypertension. Now 12 months after surgery, she is doing well with normal liver function tests and imaging studies. In summary, CC should be considered in the differential diagnosis of progressive cholestasis in patients with CDH. Surgical repair of a symptomatic CC should not be delayed even in the presence of mesh given the risks of CC complications.