Symptomatic Recurrent Splenomegaly Following Partial Splenectomy in Patients With Hereditary Spherocytosis

Hereditary spherocytosis (HS) is an inherited hematologic disorder characterized by spherical erythrocytes that are prematurely destroyed in the spleen. Total splenectomy (TS) and partial splenectomy (PS) are surgical interventions used to manage HS, but long-term outcomes, including recurrence of splenomegaly and splenomegaly-related symptoms, remain poorly understood.

This study aims to evaluate the long-term recurrence of splenomegaly and splenomegaly-related symptoms in children with HS who underwent PS versus TS at a single institution between 2008 and 2020.

A retrospective chart review of children with HS who underwent TS or PS was performed. The primary end point was the long-term recurrence of splenomegaly and splenomegaly-related symptoms. Variables collected included age, sex, surgical method (PS vs. TS), postoperative hematologic markers, splenic volume, postoperative complications, length of hospital stay, recurrence of splenomegaly, and need for completion splenectomy. Symptomatic recurrent splenomegaly is defined as splenic volume > 450 mL and the presence of symptoms directly attributable to the spleen and its effects on adjacent organs or hemolysis. Statistical analysis included comparisons of continuous variables using a Kruskal-Wallis test and categorical variables using a Fisher's exact test.

Forty-four patients with HS met the inclusion criteria for the study. Of these, 31 (71%) patients underwent laparoscopic PS and 13 (30%) patients underwent laparoscopic TS. TS was associated with significantly less intraoperative blood loss (p = 0.003), shorter hospital stays (p = 0.01), and greater reduction in hemolysis compared to PS, as evidenced by lower postoperative bilirubin levels and reticulocyte counts (both p < 0.001). Eighteen (58%) PS patients experienced recurrent splenomegaly, and six (19%) PS patients experienced splenomegaly-related symptoms leading to completion splenectomy. Among the six patients who required completion splenectomy, intraoperative hemorrhage requiring conversion to open procedure occurred in half. The median time to need completion splenectomy was 10.5 years (range, 3-15 years).

TS offers more significant long-term hematologic improvements but comes with the loss of immune function and increased risk of overwhelming post-splenectomy infection (OPSI). PS, while preserving some splenic function and theoretically reducing OPSI risk, carries a higher risk of recurrent symptomatic splenomegaly and may require additional surgeries. These findings highlight the importance of long-term follow-up for HS patients who undergo PS and the need to balance the advantages and risks of both surgical approaches.