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Bannayan-Zonana syndrome

Last revised by Dr Daniel J Bell on 27 Nov 2019

Bannayan-Zonana syndrome, also known as Bannayan-Riley-Ruvalcaba syndrome, is a rare hamartomatous disorder. 

Male predominance is reported 1.

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 2-4

Inheritance is by autosomal dominant transmission with a few reported sporadic cases.

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