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Pediatría (Asunción)

versão On-line ISSN 1683-9803

Resumo

HERREROS, MB; RODRIGUEZ, S  e  FRANCO, R. Moebius syndrome: a Description of 8 Cases and Relationship to the Use of Misoprostol in the First Trimester of Pregnancy. Pediatr. (Asunción) [online]. 2009, vol.36, n.1, pp. 35-41. ISSN 1683-9803.

Introduction: Moebius syndrome (MS) is caused by paralysis of the sixth and seventh cranial nerves. Various physiopathological causes have been described. It affects men and women in equal numbers. The Moebius sequence is a sign, and the clinical characteristics are not specific, but include a mask-like expression due to bilateral (occasionally unilateral) facial paralysis, micrognathia, and in one third of cases a cleft U shaped palate or bifid uvula. Ptosis, nystagmus, and strabismus may also be present. Unilateral lingual hypoplasia and difficulty in suction or swallowing may also appear. Defects of the extremities such as talipes equinovarus, digital hypoplasia, syndactyly, or more severe reductive defects are observed.  Associated conditions seen include hypodontia, Poland syndrome and on occasion Klippel-Feil syndrome. Cardiac or urinary conditions, hypogenitalism and hypogonadism can also exist. Some patients are more broadly compromised, with the 3d, 4th, 5th, 9th, 10th and 12th cranial nerves affected. Mental retardation, autism, and hypoacusis can also be present. Cases Description: We describe eight (8) cases of Moebius syndrome.  6 patients were male and 2 female. In 3 cases MS and micrognathia were present; 2 cases included right-side facial paralysis and micrognathia; 1 case presented MS, bilateral clubfoot and micrognathia; 1 case presented MS, bilateral clubfoot and ventriculomegaly; and 1 case presented MS, bilateral clubfoot, congenital dislocation of both hips, camptodactyly of the 3d and 4th fingers, and cleft palate. Two cases were associated with the use of misoprostol by the mother during the first trimester of pregnancy. Comments: The etiology of MS is generally sporadic, although there are autosomal dominant and autosomal recessive cases, while families with balanced translocations have also been described. Related events can produce vascular disruption and insufficient supply to the structures irrigated by the primitive subclavian artery. Numerous cases have been described of Moebius syndrome associated with use of misoprostol during the first trimester of pregnancy.

Palavras-chave : Moebius; cranial nerves; paralysis; misoprostol.

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