Ocular and clinical manifestations of Mobius' syndrome

Ocular and clinical manifestations of Mobius' syndrome

Author Cronemberger, Mônica Fialho Google Scholar
Moreira, José Belmiro de Castro Google Scholar
Brunoni, Décio Google Scholar
Mendonça, Tomás Scalamandre Google Scholar
Alvarenga, Eliézia Helena de Lima Google Scholar
Rizzo, Ana Maria Pfeifer Pereira Google Scholar
Diogo, Sandra Maria Martins Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Purpose: To assess ocular and otorhinolaryngologic manifestations and intellectual ability in patients with Mobius' syndrome.Methods: Patients with Mobius' syndrome underwent prospective ophthalmic, genetic-clinical, and otorhinolaryngologic examinations as well as psychological evaluation.Results: Sixteen patients with Mobius' syndrome between the ages of 8 months and 10.6 years underwent ocular examination. Esotropia was present in 12 (75%) patients and V-pattern in 8 (50%). Limited abduction was present in 30 (93.8%) eyes, and limited adduction was present in 21 (65.6%) eyes. The most frequent refractive error was compound hyperopic astigmatism (13 [40.6%] eyes). Eleven (68.8%) patients had lagophthalmos and 12 (75%) patients had bilateral epicanthus. Unilateral amblyopia was present in 2 (12.5%) patients. Clubfoot was the most common lower limb defect (7 [43.8%] patients). Cranial nerve impairments included paralysis of 7th nerve in all patients, paralysis of the 12th nerve in 13 patients, and paralysis of the 9th and 10th nerves in 3 patients. Evaluation of intellectual ability showed that 4 (25%) patients had normal intelligence. The mothers of 3 (18.8%) patients used misoprostol during the first trimester of pregnancy.Conclusion: Prominent ophthalmic features of Mobius' syndrome in this series were esotropia, V-pattern, abduction limitation, and compound hyperopic astigmatism. Intellectual assessment showed some degree of mental retardation in 75% of patients. Mobius' syndrome is associated with prenatal exposure to misoprostol.
Language English
Date 2001-05-01
Published in Journal Of Pediatric Ophthalmology & Strabismus. Thorofare: Slack Inc, v. 38, n. 3, p. 156-162, 2001.
ISSN 0191-3913 (Sherpa/Romeo, impact factor)
Publisher Slack Inc
Extent 156-162
Origin http://dx.doi.org/10.3928/0191-3913-20010501-09
Access rights Closed access
Type Article
Web of Science ID WOS:000168860200007
URI http://repositorio.unifesp.br/11600/44552

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