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:

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