BANNAYAN-RILEY-RUVALCABA SYNDROME PDF

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder characterized by hamartomatous intestinal polyposis, lipomas, macrocephaly and. SUMMARY: Bannayan-Riley-Ruvalcaba syndrome is a congenital disorder characterized by macrocephaly, intestinal polyposis, lipomas, and. Bannayan-Riley-Ruvalcaba syndrome, Authors: Jean-Loup Huret. Published in: Atlas Genet Cytogenet Oncol Haematol.

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Bannayan-Riley-Ruvalcaba syndrome BRRS is a rare, autosomal dominant condition characterized by macrocephaly, benign hamartomatous tumors, pigmented penile macules, lipomas, hemangiomas, and cognitive deficits. We report the cases of a father and son with clinical features of BRRS with airway obstruction secondary to pharyngeal papillomas.

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The father developed refractory papillary lymphoid hyperplasia of the oropharynx and hypopharynx and bannayan-rileey-ruvalcaba been tracheotomy dependent for more than 10 years, whereas the son’s obstructive sleep apnea resolved after adenotonsillectomy. To our knowledge, this is the first report of BRRS tumors causing airway obstruction that required surgical intervention.

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Recognition of this condition by the otolaryngologist—head and neck surgeon and referral to a geneticist is important bannayxn-riley-ruvalcaba allow diagnosis and facilitate aggressive cancer surveillance.

Arch Otolaryngol Head Neck Surg. Sharma, MD ; Elizabeth M.

Bannayan–Riley–Ruvalcaba syndrome – Wikipedia

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Airway Obstruction Caused by PTEN Hamartoma (Bannayan-Riley-Ruvalcaba) Syndrome

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