Correction of blepharoptosis in oculopharyngeal muscular dystrophy.
Oculopharyngeal muscular dystrophy is a hereditary, autosomal dominant, slowly progressive disorder with onset that occurs during middle age. Major symptoms are ptosis and dysphagia resulting primarily from selectively involved levator palpebrae and the pharyngeal muscles. Progressive, usually symmetrical blepharoptosis, with or without dysphagia, appears during middle age. Muscular weakness in the limbs can be noted in some patients. The guidelines for surgery in myopathic ptosis are conservative in view of the increased risk of postoperative corneal complications. However, orbicularis function remains intact in oculopharyngeal muscular dystrophy; therefore, corrective surgery is performed in most patients. This report describes four cases of ptosis correction in patients with oculopharyngeal muscular dystrophy in one family. The frontalis action was very poor to qualify for frontalis transfer; therefore, the authors performed moderate to large levator resection in all patients. The follow-up results 5 years postoperatively are promising to date and all the patients are satisfied with the results.
Kang
Dong Hee
DH
Department of Plastic & Reconstructive Surgery, Korea University Anam Medical Center, Seoul, Korea.
Koo
Sang Hwan
SH
Ahn
Duck Sun
DS
Park
Seung Ha
SH
Yoon
Eul Sik
ES
D000328:Adult; D001763:Blepharoptosis / Q000150:complications / Q000235:genetics / Q000601:surgery*; D006801:Humans; D008297:Male; D039141:Muscular Dystrophy, Oculopharyngeal / Q000150:complications* / Q000235:genetics; D010375:Pedigree