Akademik Veri Yönetim Sistemi
JOURNAL OF CRANIOFACIAL SURGERY, cilt.17, sa.4, ss.656-660, 2006 (SCI-Expanded)
Schwartz-Jampel syndrome (SJS) is a rare congenital disorder of continuous myotonia, causing visual and eyelid problems such as blepharospasm, acquired ptosis, and blepharophimosis. We report the management of blepharospasm in two sisters with Schwartz-Jampel syndrome with application of botilinum toxin type A (BTX-A), as an alternative to the surgical treatments, such as orbicularis oculi myectomy, levator aponeurosis resection, and lateral canthopexy as described in the literature. Three consequent doses of commercially available BTX-A (Botox(R)-Allergan, Inc. 100 u/via) were injected to orbicularis oculi muscle in two sisters with Schwartz-Jampel syndrome. At the least, the authors waited for a six-month interval to prevent development of sensitivity to BTX-A in patients. No significant improvement was observed after the injection of first dose of BTX-A, with the total dose of 25 units of BTX-A to each orbicularis oculi muscle of the eyes. The muscle tone weakened after the second dose, which was the twice as such of the first applied total dose (50 units of BTX-A to each orbicularis oculi muscle of the eyes) and significant functional and cosmetic improvements were achieved after injection of the third dose, in which the total applied dose was the same as the one applied in second dose. The blepharospasm and eyelid alterations caused by Schwartz-Jampel syndrome should be treated to provide functional and cosmetic improvements. Application of BTX-A to orbicularis oculi muscle for the treatment of blepharospasm could be considered as an alternative to levator resection, and lateral canthopexy in Schwartz-Jampel syndrome.