Abstract :
Gaucher’s disease, a lysosomal storage disorder, is caused by an inherited deficiency of beta glucocerebrosidase enzyme leading to the accumulation of glucocerebroside in the reticuloendothelial system. Patients with this disease often manifest coagulation abnormalities and multi-organ complications. These factors present a challenge to the anaesthesiologist, in deciding the type as well as the conduct of anaesthesia. We aim to report the anaesthetic management in a 3-years old child with Gaucher’s disease posted for splenectomy.
Keyword :
Gaucher, Thrombocytopenia, Anaemia, Splenomegaly.