Correction of blepharoptosis in oculopharyngeal muscular dystrophy.

Abstract:

: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.

journal_name

Ann Plast Surg

authors

Kang DH,Koo SH,Ahn DS,Park SH,Yoon ES

doi

10.1097/00000637-200210000-00015

subject

Has Abstract

pub_date

2002-10-01 00:00:00

pages

419-23

issue

4

eissn

0148-7043

issn

1536-3708

pii

00000637-200210000-00015

journal_volume

49

pub_type

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