Role of Partial Splenectomy in Gaucher's Disease in Resource Challenged Nations.

Abstract

Gaucher's disease (GD) is a rare inherited metabolic disorder caused by the defective activity of glucocerebrosidase. Enzyme replacement therapy (ERT) and substrate reduction therapy are the treatment of choice. Total splenectomy has a role when the child develops complications of massive splenomegaly. There are only a few case series of partial splenectomy in GD in the pediatric age group. To study the role, technical feasibility, and challenges of partial splenectomy in children with GD with hypersplenism. Retrospective review of children of GD who had partial splenectomy between February 2016 and April 2018. Demographics, clinical findings, laboratory, operative details, transfusion requirements, and perioperative, immediate, and late complications were retrieved. Clinical courses after discharge were obtained from follow-up data. Eight children with GD underwent partial splenectomy between 2016 and 2018. The median age at surgery was 3 years and 6 months (range -2 years to 8 years). Five children underwent partial splenectomy successfully, of which one child required postoperative ventilatory support for 48 h owing to lung atelectasis. Three children underwent completion splenectomy due to bleed from the cut surface of the splenic remnant. One of the children who underwent completion splenectomy expired on the postoperative day 5 due to refractory shock with multi-organ dysfunction. Partial splenectomy has a definite role in selected children who present with massive splenomegaly with mechanical effects and/or hypersplenism while awaiting ERT.