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Romano M., Russo S., Notaro M., Cotticelli L., Romano A.
Dipartimento di Oftalmologia, Scuola di Medicina, II Università di Napoli, Napoli, Italia
AIM: Myotonic dystrophy (Steinert's disease) is the most common hereditary disease of the neuromuscular system in adults. Ocular abnormalities described in patients with Steinert’s disease include cataract, ptosis, exposure keratitis, pigmentary abnormalities, abnormal electroretinograms, and weakness of the orbicularis oculi muscles.
METHODS:A 64-year-old man patient with Steinert's disease came to our observation. A complete ophthalmologic examination was performed.
RESULTS: Ocular examination showed severe bilateral ptosis, bilateral subcapsular posterior (scp) cataract, BVCA 20
200 in both eyes, IOP 11 mmHg in both eyes, no fuction of frontalis muscle, weakness of orbicularis oculi muscles, poor Bell’s phenomenon and the absence of jaw-winking were detected. The results of surgical treatment are reported.
CONCLUSIONS: In the described case, because of absence of frontalis muscle function, frontalis suspension technique, traditionally performed, didn’t represent an efficient solution. So we thought it right to perform Whitnall’s sling procedure, a simple, rapid, reversible procedure without typical complications of frontalis suspension.