(Lipogranulomatose)is

Autosomal recessive transmission. Lysosomal disorder characterized by a deficiency in ceramidase (accumulation of tissular ceramide). Sometimes neonatal onset. Tissular granulomatous infiltration (subcutaneous, joints). Hoarseness (infiltration of the larynx). Breathing difficulties  (infiltration of the trachea and the lungs) that may require a tracheotomy. Psychomotor retardation, peripheral neuropathy with amyotrophy. Sometimes hepatosplenomegaly.

Anesthetic implications: 

difficult intubation in case of granulomas of the tongue or larynx.

References : 

-        Asada A, Tatekawa S, Terai T, Hayashi M, Hatano M, Ikeshita K, Fujimori M. 
The anesthetic implications of a patient with Farber's lipogranulomatosis. 
Anesthesiology 1994; 80: 206-9.

Updated: May 2019