Citation, DOI, disclosures and article data
Citation:
Niknejad M, Bell D, Sharma R, et al. Bannayan-Zonana syndrome. Reference article, Radiopaedia.org (Accessed on 18 Apr 2024) https://doi.org/10.53347/rID-22041
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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1. Gujrati M, Thomas C, Zelby A et-al. Bannayan-Zonana syndrome: a rare autosomal dominant syndrome with multiple lipomas and hemangiomas: a case report and review of literature. Surg Neurol. 1998;50 (2): 164-8. Pubmed citation
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2. Miles JH, Zonana J, McFarlane J et-al. Macrocephaly with hamartomas: Bannayan-Zonana syndrome. Am. J. Med. Genet. 1984;19 (2): 225-34. Am. J. Med. Genet. (abstract) - doi:10.1002/ajmg.1320190204 - Pubmed citation
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3. Moretti-Ferreira D, Koiffmann CP, Souza DH et-al. Macrocephaly, multiple lipomas, and hemangiomata (Bannayan-Zonana syndrome): genetic heterogeneity or autosomal dominant locus with at least two different allelic forms?. Am. J. Med. Genet. 1989;34 (4): 548-51. Am. J. Med. Genet. (abstract) - doi:10.1002/ajmg.1320340419 - Pubmed citation
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4. Hayashi Y, Ohi R, Tomita Y et-al. Bannayan-Zonana syndrome associated with lipomas, hemangiomas, and lymphangiomas. J. Pediatr. Surg. 1992;27 (6): 722-3. Pubmed citation
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