Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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October 17, 2011
Congenital Infiltrating Lipomatosis
- Congenital infiltrating lipomatosis is a rare condition characterized by diffuse fatty infiltration of the soft tissues. Muscle involvement and bony hyperplasia are common findings.
- It is typically seen in children.
- Psychomotor development is normal. Patients, however, present with craniofacial deformities.
- It is one of the 5 types of benign lipomas. Other benign lipomas include simple encapsulated lipoma; lipoma variants, such as angiomyolipoma; hamartomatous lesions; and benign tumor of brown fat, hibernoma.
- Key Diagnostic Features: Multi-compartmental, trans-spatial infiltrating, fat-containing lesion is seen typically in the region of the face or neck. Bony hyperplasia, when present, can be best assessed by CT studies.
- DDx: Proteus syndrome, Encephalocutaneous lipomatosis, Vascular malformations
- Liposuction, surgical excision. Recurrence is common.