Ciliated hepatic foregut cyst in children and adolescents: a systematic review of clinical presentation, diagnosis, management, and outcomes.
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Ciliated hepatic foregut cyst (CHFC) is a rare congenital cystic liver lesion described predominantly in adults, in whom malignant transformation to squamous cell carcinoma has been reported. Pediatric experience is scattered across case reports and small series, and no systematic synthesis has been published. We conducted a systematic review per PRISMA 2020. PubMed, Scopus, and Embase (Elsevier) were searched on 3 August 2026 without language or date restrictions, supplemented by citation searching and grey-literature screening (JMIR Preprints, bioRxiv, medRxiv). We included case reports and case series of patients aged ≤ 19 years with histopathologically confirmed CHFC. Screening (Rayyan) and extraction were performed in independent duplicate. Risk of bias was assessed with the Joanna Briggs Institute Critical Appraisal Checklists. Findings were synthesised descriptively per SWiM; no meta-analysis was performed. Sixteen studies contributed 23 unique pediatric patients (female-to-male ratio 1.3:1; median age 4.0 years, range 5 months-19 years). Antenatal detection occurred in 5 (21.7%) of 23. Symptomatic presentation was reported in 14 (60.9%) of 23. Median cyst size was 6.0 cm (range 0.6-19.3), and 4 patients (17.4%) had giant cysts (≥ 10 cm). Segment IV was involved in 11 of 19 patients with location reported (57.9%). Biliary communication was documented in 5 of 14 evaluable cases (35.7%; 5/23 overall); 4 of these patients 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) and no malignant transformation (0/22 evaluable) were reported. Postoperative complications occurred in 2 (8.7%) of 23. No recurrence (0/11 evaluable) or mortality (0/23) was reported over a median 19-month follow-up (reported for 8 patients). Pediatric CHFC appears to present more often with symptomatic, larger and biliary-communicating lesions than the classical adult phenotype. Complete excision was associated with favorable reported short-term outcomes, but the evidence is of very low certainty, and limited follow-up and substantial non-reporting preclude conclusions about long-term recurrence or malignant transformation.