Orbicularis myectomy with levator advancement in Schwartz-Jampel syndrome.

PURPOSE: Schwartz-Jampel syndrome is a disorder of continuous myotonia causing blepharospasm, acquired ptosis, and blepharophimosis. We report the management of the associated eyelid anomalies with orbicularis oculi myectomy, levator aponeurosis resection, and lateral canthopexy. METHODS: Interventional case reports. Two patients with Schwartz-Jampel syndrome presented with blepharospasm, acquired ptosis, and blepharophimosis. Orbicularis myectomy, levator aponeurosis resection, and lateral canthopexy were performed to relieve the blepharospasm and to correct the ptosis and blepharophimosis. RESULTS: Significant functional and cosmetic improvements were achieved by increasing the palpebral fissure height and length while greatly decreasing the blepharospasm of the patients. No recurrence of the blepharospasm or eyelid anomalies has been noted after 1 and 15 years of follow-up, respectively. CONCLUSION: The blepharospasm and eyelid alterations caused by Schwartz-Jampel syndrome should be treated to provide functional and cosmetic improvements. Our technique of myectomy, levator resection, and lateral canthopexy provides an excellent, long-lasting result.

Levator aponeurosis surgery in Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome is a rare disorder with prominent palpebral fissure changes. These changes are complex and have been described as ptosis, blepharophimosis, and blepharospasm. Two new cases of the Schwartz-Jampel syndrome and the results of elevating the upper eyelid margin with levator aponeurosis surgery are presented, along with a review of the literature.