Orbicularis myectomy with levator advancement in Schwartz-Jampel syndrome.
OBJECTIVE
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.


CONCLUSIONS
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.
Lucci
L M
LM
Department of Ophthalmology, Federal University of São Paulo, São Paulo, Brazil.
Yen
M T
MT
Anderson
R L
RL
Hwang
I P
IP
Black
R E
RE
D016569:Blepharophimosis / Q000209:etiology / Q000601:surgery*; D001763:Blepharoptosis / Q000209:etiology / Q000601:surgery*; D001764:Blepharospasm / Q000209:etiology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005143:Eyelids / Q000601:surgery*; D005260:Female; D006801:Humans; D009801:Oculomotor Muscles / Q000601:surgery*; D010009:Osteochondrodysplasias / Q000150:complications / Q000601:surgery*