Gaucher’s disease is a monogenic disorder, enzymatic genetic deficiency of ß-glucocereboside, leading to acumulation of the non-degraded metabolic substrate, in the macrophages lysosomes = Gaucher cells (1).
There are 3 types, bone pathology being encountered in type 1 (Nonneuronopathic form) along with: hepatosplenomegaly, hematological and pulmonary pathology.
Avascular necrosis is frequently encountered with bone damage in multiple sites such as femoral head, femoral condyle or humeral head. Osteoporosis affects in a severe mode, bone strength and the ability to sustain an implant. We present a complex case of a 50 years old male with Gaucher’s disease, and avascular necrosis of femoral head and lateral distal condyle.
Avascular necrosis in Gaucher’s disease
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