Pediatric splenectomy for hematologic disorders: two-decade experience and prophylactic cholecystectomy outcomes

While splenectomy remains a cornerstone treatment for certain hematologic diseases, controversy persists regarding the optimal timing and indications for prophylactic cholecystectomy. This study evaluates long-term outcomes from a large single-center series.

We retrospectively analyzed 87 patients (48 male, 39 female) with hematologic disorders who underwent splenectomy between 2003 and 2023. Primary outcomes included improvement in hematologic parameters, resolution of bilirubinemia, prevalence of cholelithiasis, and complication rates. Subgroup analyses examined disease-specific outcomes and age-stratified results.

Hereditary spherocytosis was the predominant diagnosis (70.1%), followed by thalassemia (11.5%) and immune thrombocytopenia (ITP) (10.3%). The mean age at splenectomy was 9.6 years (range 3-17), with a mean interval from diagnosis to surgery of 4.3 years. Spleen size significantly differed across diseases, being largest in rare hematologic disorders (178 mm) and smallest in ITP (97.56 mm). Cholelithiasis was present in 65.6% of hereditary spherocytosis cases and 50% of thalassemia cases but was absent in ITP. Simultaneous cholecystectomy was performed in 55 patients (63.2%), including 10 prophylactic procedures. Postoperatively, hemoglobin levels increased by factors of 1.24, 1.13, and 1.25 in hereditary spherocytosis, thalassemia, and ITP, respectively. Platelet counts increased by factors of 2.24, 2.8, and 6.5, respectively. Bilirubin levels decreased significantly in all groups. Notably, preoperative total bilirubin was significantly higher (p = 0.016) in patients selected for prophylactic cholecystectomy (mean 4.17 mg/dL) compared to those without cholecystectomy (mean 1.92 mg/dL).

Splenectomy offers durable improvements in hematologic parameters across various hematologic diseases, with minimal complications. Prophylactic cholecystectomy appears justified in patients with marked hyperbilirubinemia, even without cholelithiasis, particularly in hereditary spherocytosis. The absence of post-splenectomy sepsis in this series supports the continued role of total splenectomy when appropriate vaccination protocols and antibiotic prophylaxis are implemented.