[Steinert myotonic dystrophy and blepharoptosis surgery: 9 case reports].
Steinert myopathic dystrophy is a generalized, hereditary disease with bone, muscular, heart and ocular involvement.


METHODS
This is a retrospective study of nine patients with significant blepharoptosis due to Steinert disease, who were treated at the Adolphe de Rothschild Ophthalmology Foundation over a period of 5 years. Ptosis was symmetric and major in all cases with poor levator excursion. Severity criteria were an absence of the Bell phenomenon and diminished orbicularis tone. A frontalis suspension was performed in eight cases with intentional undercorrection. The outcome was favorable in all cases, 2 with a slight overcorrection underwent a second operation


CONCLUSIONS
conclusion: Surgical treatment of ptosis in Steinert disease is difficult because of a risk of lagophthalmic, keratopathy due to the severity of the disease, an absence of the Bell phenomenon and ophthalmoplegia. This surgery must be undertaken with caution, most often using a frontalis suspension. Undercorrection must be systematic, with the single goal of freeing the pupil in the primary position.
Karim
A
A
Service d'Ophtalmologie et de Chirurgie Oculo-plastique, Fondation Ophtalmologique Adolphe de Rothschild, Paris.
Schapiro
D
D
Morax
S
S
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001763:Blepharoptosis / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications*; D012189:Retrospective Studies; D012737:Sex Factors; D013997:Time Factors