Wolman disease


Patient with known Wolman disease. The appearance of enlarged, densely calcified adrenal glands with preserved normal shape is nearly pathognomonic. Other common causes of adrenal calcification in young children include neuroblastoma and prior adrenal hemorrhage, but the former is located in a mass rather than outlining the shape of a normal gland, and the latter could outline a gland that is of normal or reduced size.

Survival in this disease classically does not exceed the first few years of life, but enzyme replacement therapy and bone marrow transplant have increased survival and reduced morbidity in recent years.