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Keywords:

  • partial splenic embolization;
  • hereditary spherocytosis;
  • children

Abstract

Objectives:  Although total splenectomy is able to reduce clinical symptoms in patients with hereditary spherocytosis (HS), splenectomized patients are at risk to develop overwhelming bacterial infections and, to a lesser extent, thromboembolic complications. In contrast, partial splenectomy or partial splenic embolization (PSE) may also decrease the rate of hemolytic complications while maintaining residual splenic function. The aim of this study was to investigate the benefit of PSE in children with moderate to severe HS.

Patients and methods:  We performed PSE via retrograde transfemoral access in eight children (four female, four male) with moderate to severe HS at a median age of 8 yr. HS-related complications before PSE included gallstones in six and aplastic crises in four children. One patient was transfusion-dependent.

Results:  No acute side effects were seen during or after PSE. Median hemoglobin increased significantly from levels between 7.5 g/dL and 11.65 g/dL before PSE to levels between 8.4 g/dL and 13.35 g/dL after PSE (P = 0.012). Median splenic sizes before PSE ranged from 9.7 cm/m2 to 19.0 cm/m2 and significantly decreased to values between 4.4 cm/m2 and 15.65 cm/m2 during follow-up (P = 0.012).

Conclusions:  PSE appears to be a safe, effective and feasible treatment option for the management of children with moderate to severe HS.