Bannayan-Riley-Ruvalcaba syndrome
Bannayan-Zonana syndrome, also known as Bannayan-Riley-Ruvalcaba syndrome, is a rare hamartomatous disorder.
Epidemiology
Male predominance is reported .
Clinical presentation
Bannayan-Zonana syndrome is characterized by:
- macrocephaly
- multiple lipomas
- hemangiomas
Other findings that make it possible to delineate a recognisable syndrome include:
- down-slanting palpebral fissures
- high palate
- joint hyperextensibility
- pectus excavatum
- strabismus (squint)
- amblyopia (lazy eye)
- prolonged drooling
Pathology
Inheritance is by autosomal dominant transmission with a few reported sporadic cases.
See also
