Ciliated hepatic foregut cyst in children and adolescents: a systematic review of clinical presentation, diagnosis, management, and outcomes.

In 30 seconds

This PRISMA 2020 systematic review descriptively synthesised case reports and case series of histopathologically confirmed ciliated hepatic foregut cysts in patients aged 19 years or younger. Sixteen studies included 23 children and adolescents; median age was 4.0 years, and 14 (60.9%) presented with symptoms. Median cyst size was 6.0 cm, and biliary communication was documented in 5 of 14 evaluable cases. No malignant transformation was reported among 22 evaluable patients. Reported short-term outcomes after excision were favorable, but the authors judged the evidence very low certainty.

Key findings

• Symptoms were reported in 14 (60.9%) of 23 patients; median cyst size was 6.0 cm (range 0.6-19.3).
• Biliary communication was documented in 5 of 14 evaluable cases (35.7%; 5/23 overall); 4 underwent Roux-en-Y biliary reconstruction.
• Squamous metaplasia was reported in 4 of 7 evaluable cases (57.1%; 4/23 overall); no dysplasia (0/7 evaluable) or malignant transformation (0/22 evaluable) was reported.
• Postoperative complications occurred in 2 (8.7%) of 23; no recurrence (0/11 evaluable) or mortality (0/23) was reported.

Why it matters

The review characterises pediatric CHFC as often symptomatic and sometimes associated with large cysts or biliary communication, features that may inform diagnostic and operative assessment. Although no malignancy was reported in the evaluable pediatric cases, the small, selectively reported case literature and limited follow-up mean these findings cannot establish long-term risk or outcomes.

What to keep in mind

Evidence came from case reports and small series, with substantial non-reporting and very low certainty. Follow-up was reported for only 8 patients, with a median of 19 months, limiting conclusions about long-term recurrence or malignant transformation.