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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Copyright American Medical Association. Bannayan-Riley-Ruvalcaba syndrome BRRS is a rare, autosomal dominant condition characterized synddrome 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. The father developed refractory papillary lymphoid hyperplasia of the oropharynx and hypopharynx and has been tracheotomy dependent for more than 10 years, whereas the son’s obstructive sleep apnea resolved bannayan-rileh-ruvalcaba 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 to allow diagnosis and facilitate aggressive cancer surveillance. Arch Otolaryngol Head Neck Surg.

Bannayan–Riley–Ruvalcaba syndrome

Sharma, MD ; Elizabeth M. Petty, MD ; Marci M. Privacy Policy Terms of Use. Sign bannayan-rlley-ruvalcaba to access your subscriptions Sign in to your personal account.

Center For Inherited Colon Cancer | Cleveland Clinic

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Bannayan-Riley-Ruvalcaba syndrome

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