Prenatally diagnosed choledochal cysts: observation or early surgery?

The incidence of subhepatic cysts later confirmed as choledochal cyst has changed with the use of prenatal ultrasonography. A new group has emerged: the neonatal patient with an antenatal diagnosis. Optimal timing for cyst excision depends on variables such as gestational age, weight, associated conditions, biochemical liver alterations, development of complications, and the sonographic surveillance of size. The author's experience (1992) with an antenatally diagnosed choledochal cyst prompted this literature review. The present case showed an abnormal choledochopancreatic ductal junction, high amylase content, and a linear pattern of growth over time (2 mm/wk). Management consisted of cyst excision and bilio-enteric reconstruction. Fourteen cases (including the present one) have been reported in the world literature. All were females. Seventy-two percent of the ultrasound examinations were performed for dating purposes. Subhepatic cysts were identified at a mean gestational age of 26.9 weeks (range, 15 to 37 weeks). Excision and bilio-enteric reconstruction were performed at a mean age of 45 days (range, 9 hours to 6 months). Clinically, 50% of the babies were anicteric, 43% were jaundiced, and 7% had a palpable mass. Evidence of cyst growth was present in 56% of cases, and 60% had liver fibrosis that reverted to normal. The indications for surgery were jaundice (43%), cyst growth (21%), delayed HIDA excretion (7%), and elective (29%). Recommendations for managing the asymptomatic, anicteric neonate are discussed, entailing a rational approach based on growth potential, biochemical liver alterations, and the development of obstructive jaundice.