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[Steinert myotonic dystrophy and blepharoptosis surgery: 9 case reports]
1Service d'Ophtalmologie et de Chirurgie Oculo-plastique, Fondation Ophtalmologique Adolphe de Rothschild, Paris.
Journal Francais D'Ophtalmologie
|March 1, 2003
Summary
Surgical correction of blepharoptosis in Steinert myopathic dystrophy requires caution. Frontalis suspension with intentional undercorrection is recommended to avoid complications like lagophthalmos and keratopathy.
Area of Science:
- Ophthalmology
- Genetics
- Neurology
Background:
- Steinert myopathic dystrophy (SMD) is a multisystem genetic disorder.
- Ocular manifestations, particularly severe blepharoptosis, are common in SMD.
- Ptosis in SMD presents unique surgical challenges due to muscle involvement.
Purpose of the Study:
- To evaluate the efficacy and safety of surgical ptosis correction in patients with Steinert myopathic dystrophy.
- To identify optimal surgical techniques and management strategies for blepharoptosis in SMD.
Main Methods:
- Retrospective study of nine patients with SMD-related blepharoptosis.
- Surgical intervention involved frontalis suspension in eight patients.
- Key severity criteria included absence of Bell phenomenon and diminished orbicularis tone.
Main Results:
- Favorable outcomes were achieved in all treated patients.
- Intentional undercorrection was employed in frontalis suspension.
- Two patients required a second operation due to slight overcorrection.
Conclusions:
- Surgical treatment for ptosis in SMD is challenging due to disease severity.
- Risks include lagophthalmos and keratopathy.
- Frontalis suspension with systematic undercorrection is advised to free the pupil.