Anesthetic approach to Niemann–Pick Type C patient for dental treatment

Niemann–Pick disease is an autosomal recessive lipid storage disease, characterized with differentiating levels of hepatosplenomegaly and progressive psychomotor retardation (this disease exhibiting heterogenic symptoms has four subtypes; Type A and B, in which sphingomyelinase values are normal, and Type C and D, in which sphingomyelinase values are close to normal values). Disease emerges with early childhood ataxia and progressive dementia, and the most evident features are early childhood hepatosplenomegaly, vertical supranuclear ophthalmoplegia, ataxia, dysarthria, mental‑motor retardation, and seizures.[1,3] In this report, we present our anesthetic approach to an uncooperative 3‑year‑old female patient with hepatosplenomegaly, mental‑motor retardation, developmental retardation, deglutition, speech deficiency, ataxia, and seizures who admitted to our clinic in collaboration with our pedodontics clinic for preventive dentistry practice.