Ocular and clinical manifestations of Möbius' syndrome.
OBJECTIVE
To assess ocular and otorhinolaryngologic manifestations and intellectual ability in patients with Möbius' syndrome.


METHODS
Patients with Möbius' syndrome underwent prospective ophthalmic, genetic-clinical, and otorhinolaryngologic examinations as well as psychological evaluation.


RESULTS
Sixteen patients with Möbius' 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.


CONCLUSIONS
Prominent ophthalmic features of Möbius' 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. Möbius' syndrome is associated with prenatal exposure to misoprostol.
Cronemberger
M F
MF
Department of Ophthalmology, Federal University of São Paulo, Paulista School of Medicine-São Paulo Hospital, Brazil.
de Castro Moreira
J B
JB
Brunoni
D
D
Mendonça
T S
TS
Alvarenga
E H
EH
Rizzo
A M
AM
Diogo
S M
SM
D002648:Child; D002675:Child, Preschool; D003941:Diagnostic Techniques, Ophthalmological; D005128:Eye Diseases / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D007361:Intelligence Tests; D008297:Male; D020331:Mobius Syndrome / Q000150:complications / Q000175:diagnosis*; D010038:Otorhinolaryngologic Diseases / Q000175:diagnosis* / Q000209:etiology; D011446:Prospective Studies; D014792:Visual Acuity