title: Ocular and clinical manifestations of Möbius' syndrome.
abstract: 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.
authors.affiliation: Department of Ophthalmology, Federal University of São Paulo, Paulista School of Medicine-São Paulo Hospital, Brazil.
authors.forename: M F
authors.initials: MF
authors.lastname: Cronemberger
authors.forename: J B
authors.initials: JB
authors.lastname: de Castro Moreira
authors.forename: D
authors.initials: D
authors.lastname: Brunoni
authors.forename: T S
authors.initials: TS
authors.lastname: Mendonça
authors.forename: E H
authors.initials: EH
authors.lastname: Alvarenga
authors.forename: A M
authors.initials: AM
authors.lastname: Rizzo
authors.forename: S M
authors.initials: SM
authors.lastname: Diogo
mesh_terms: 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