https://pubmed.ncbi.nlm.nih.gov/40389840/ Cholestasis

BMC Pediatr. 2025 May 19;25(1):404.
doi: 10.1186/s12887-025-05760-z.

Biliary obstruction in pediatric hereditary spherocytosis: a clinical review of 16 cases

Xinjie Huang 1, Chunhui Peng 2, Yajun Chen 1, Dongyang Wu 1, Wei Chen 1 3, Zengmeng Wang 1, Kai Wang 1
Affiliations expand
PMID: 40389840
PMCID: PMC12087245
DOI: 10.1186/s12887-025-05760-z

Abstract

Background: Biliary obstruction is a rare complication in patients with hereditary spherocytosis (HS). The clinical course and optimal treatment strategies for HS patients complicated by biliary obstruction remain unclear.
Methods: We conducted a retrospective review of 16 pediatric HS patients complicated by biliary obstruction who were treated at our hospital between January 2018 and October 2024. Based on previously published clinical severity classifications, patients were divided into Group A (non-severe group: trait, mild, and moderate) and Group B (severe group).

Results: The study included 16 patients with a mean age of 9.0 ± 3.2 years, evenly distributed between the two groups (8 patients each). Preoperative routine blood tests showed no significant differences between the groups; however, Group B exhibited higher bilirubin levels and lower liver enzyme levels. Genetic testing was performed in 12 patients, revealing SPTB gene mutations in 7 (58.3%). Conservative management effectively resolved biliary obstruction in 10 patients (62.5%) within 14 days. Invasive interventions, such as endoscopic retrograde cholangiopancreatography (ERCP) or cholecystostomy, were required in 6 patients, with conjugated bilirubin levels normalizing within five days post-procedure. Complications occurred in two patients with prolonged intervals between diagnosis and surgery (> 3 months): one required stent replacement due to blockage after ERCP, and the other developed a gallbladder-skin fistula and coagulation disorder following laparoscopic cholecystostomy. Of the 14 patients who underwent subsequent splenectomy and/or cholecystectomy, 12 recovered without complications. Notably, the 6 patients who underwent splenectomy alone without cholecystectomy did not experience biliary colic during a mean follow-up period of 3.4 years (range: 0.5 - 5.5 years).

Conclusions: Biliary obstruction can complicate HS in pediatric patients regardless of anemia severity, particularly in those with SPTB gene mutations. Conservative management is effective in most cases, while invasive procedures are required for refractory cases. Shortening the interval between diagnosis and subsequent surgery may help prevent complications. Splenectomy alone appears to be a viable option once biliary obstruction is resolved.