Wolman disease

Case contributed by Dr Francis Fortin

Presentation

Rule out pneumonia.

Patient Data

Age: 5 years old
Gender: Male

16 months

X-ray

Enlarged, densely calcified adrenal glands with preserved normal shape. Left PICC and partially-seen gastrostomy.

5 years

X-ray

Enlarged, densely calcified adrenal glands with preserved normal shape. Right jugular port-a-cath. Gastrostomy.

Case Discussion

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.

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