Mental Retardation in the Bannayan Syndrome
- 1 May 1982
- journal article
- case report
- Published by American Academy of Pediatrics (AAP) in Pediatrics
- Vol. 69 (5) , 642-644
- https://doi.org/10.1542/peds.69.5.642
Abstract
Bannayan syndrome, first reported in 1971, is characterized by macrocephaly and multiple softtissue and visceral hamartomas.1 Two reports have documented autosomal dominant inheritance and an altered rate of linear growth.2,3 An intracranial tumor occurred in one patient.3 We report a third family with Bannayan syndrome with a significant additional feature, mental retardation. CASE REPORT The proband, a 1710½-year-old white male adolescent, was noted shortly after a normal birth to have a large head, diagnosed as "hydrocephalus." No specific intervention was initiated. Skin tumors developed within the first year, and a 5x6x7-cm lipoma (confirmed by histologic examination) was removed from the right axilla at 2 years of age.Keywords
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