Anaesthetic Management of a case of Heriditary Spherocytosis for Splenectomy and Cholecystectomy

Abstract

Objectives:Hereditary spherocytosis is a heterogeneous group of disorder that results in the formation of abnormal red blood cells with fragile cell walls causing anaemia, jaundice, splenomegaly and ultimately gall stone formation. Most children have mild disease do not require splenectomy. Splenectomy is reserved for those with severe disease or who develop symptomatic gall stone. Individuals with symptomatic gallstones usually have a cholecystectomy and at the same time splenectomy if indicated.

Case Report: A 20 years old female diagnosed as hereditary spherocytosis since age of one year. After 19 years she was diagnosed as splenomegaly with cholelithiasis. After proper investigations and vaccination patient was posted for surgery splenectomy and cholecystectomy at the same time which is challenging from the anaesthetic point of view because the sickling oriented anaesthetic approach. Commonly recommended perioperatoive management includes preemptive erythrocyte transfusion, aggressive hydration and avoidance of hypoxia, aplastic crisis, hypothermia and acidosis.

Conclusion: Patients with Hereditary spherocytosis, as they are more prone to develop infection, were meticulously controlled through out the perioperative period. Removing the spleen does not cure the disease, but it does allow the red blood cells to live longer so that a child no longer became anaemic during periods of stress or infection. It is very important that all these patients should receive all of the normal childhood immunizations and a few special immunizations (pneumococcal and meningococcal) to prevent infection.

Key Words: Hereditary Spherocytosis, Splenectomy, cholecystectomy, aplastic crisis.

Journal of BSA, Vol. 18, No. 1 & 2, 2005 p.50-53