Surgical management in a severe OSA patient diagnosed with Stickler syndrome.

Auris, Nasus, Larynx
So Young JeonSung Wan Kim

Abstract

Stickler syndrome is a genetic disorder of connective tissue. One of the major symptoms associated with this disorder is an oro-facial malformation, which may cause a submucous cleft or a complete cleft of the hard palate. A 32-year-old man diagnosed with Stickler syndrome and a submucosal cleft palate (SMCP) visited our hospital with a chief complaint of excessive daytime sleepiness. The patient was diagnosed with severe obstructive sleep apnea (OSA), and administration of a polysomnography test revealed an apnea-hypopnea index (AHI) of 30.9 events/hour (h). Auto-titrating continuous positive airway pressure was initiated to control the OSA symptoms and subsequently the patient showed some improvement. However, due to continuous velopharyngeal insufficiency symptoms, intravelar veloplasty was performed. Three months after surgery, the AHI had decreased to 12.4 events/h. Recent studies have described a greater risk for OSA in individuals with cleft palate, than in the general population. The present case demonstrates surgical success in a patient with OSA and SMCP, suggesting that palatal surgery may be considered an optional surgical treatment for OSA patients with SMCP.

References

Dec 1, 1975·Journal of Medical Genetics·J G Hall, H Herrod
Feb 1, 1996·American Journal of Respiratory and Critical Care Medicine
Jun 1, 1996·Plastic and Reconstructive Surgery·A K GosainD L Larson
Oct 29, 2002·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·N V HermannS Bolund
Aug 29, 2003·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Edmund RoseIrmtrud Jonas
Nov 21, 2007·The Laryngoscope·Harlan MuntzJ Fredrik Grimmer
Mar 4, 2009·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·J E MacleanD A Fitzgerald
Jun 9, 2009·Sleep Medicine Reviews·Joanna E MacLeanKaren Waters
Mar 23, 2011·Archives of Otolaryngology--head & Neck Surgery·Jacob G Robison, Todd D Otteson
Oct 8, 2015·Human Mutation·Mouna Barat-HouariIsabelle Touitou
Jan 31, 2018·The Journal of Craniofacial Surgery·Mosaad Abdel-AzizMohamed Fawaz

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