Association of misoprostol, moebius syndrome and congenital central alveolar hypoventilation: case report

Abstract

-We report a case showing the association of Moebius syndrome, the use of misoprostol during pregnancy and the development of central congenital alveolar hypoventilation. Pathophysiological aspects of these three diseases are discussed and also the unfavorable prognosis of this association. Moebius syndrome is clinically characterized by congenital nonprogressive facial diplegia and restricted lateral eyes movements. It is usually bilateral and can be followed by other cranial nerves palsy (third, fifth, ninth and tenth). It is usually associated with craniofacial malformation (micrognathia, bifid uvula, cleft palate, epicanthus, external ear) and other malformations (talipes equinovarus, syndactily) 1,2 . The etiology of this syndrome was speculated to be a congenital absence of the motor nuclei of the abducens and facial nerves, but genetical and environmental factors can also be involved. Exposure to infections, alcohol, cocaine, thalidomide or misoprostol were also related in association with Moebius syndrome. It was found in most cases that this syndrome can be the result of infarction of brainstem nuclei during fetal life [3][4][5] . It was also showed in some cases evidences of vascular malformations as capillary hemangiomas in mesencephalic and pontine segments 2 .Congenital central alveolar hypoventilation syndrome (CCAHS) is generally recognized in the first days of life. Symptoms, among others, may include cyanosis, apnea, and sudden respiratory failure. Central hypoventilation is characterized by: hypoventilation during sleep, no or minimal ventilatory and arousal responses to hypercarbia and hypoxia during sleep, nonresponsiveness to respiratory stimulants, unawareness of hypoxia, adequate baseline ventilation during wakefulness, absent or negligible response to hypoxia and hypercarbia while awake 7 . A polysomnography is needed

Möbius sequence in children exposed in utero to misoprostol: Neuropathological study of three cases

“…They were most evidence in the area of the cranial nerve nuclei V, VI, and VII, as predicted by the clinical findings in MS. Also, diffuse gliosis was present throughout the tegmentum of the brainstem, mainly at the pontine level, involving reticular formation subnuclei. Kinney et al (1989) and Cortez and Kinney (1996) have previously discussed central apnea in three newborns with tegmental necrosis and olivary atrophy; Igarashi et al (1997) and Nunes et al (1999) have also suggested that the central dysfunction presented by their patients with MS would be the result of lesions in the proximity of respiratory nuclei.…”

“…Gonzalez et al (1993Gonzalez et al ( , 1998 reported two large series of Brazilian children presenting with MS and/or other congenital abnormalities who had been exposed in utero to misoprostol. Several researchers have also reported patients with similar abnormalities who had been exposed to that drug during the first trimester of pregnancy (Pastuszak et al 1998;Nunes et al, 1999;Genest et al, 1999;Coelho et al, 2000;Orioli and Castilla, 2000).…”

Síndrome de Moebius: relato de caso

“…Micrognatia e aplasia do peitoral também são observadas em associação à SM, constituíndo a denominada síndrome de Polland 2 . Já foram descritas outras anormalidades, como defeitos das extremidades, dos dentes 3 , cardíacos 4 e disfunção respiratória central [5][6][7] , dentre outras menos comuns.A origem da SM permanece especulativa mas, certamente, é influenciada por fatores genéticos e ambientais. Isquemia fetal transitória é a teoria mais aceita para explicar a síndrome 8,9 .…”

“…Micrognatia e aplasia do peitoral também são observadas em associação à SM, constituíndo a denominada síndrome de Polland 2 . Já foram descritas outras anormalidades, como defeitos das extremidades, dos dentes 3 , cardíacos 4 e disfunção respiratória central [5][6][7] , dentre outras menos comuns.…”

“…Acreditamos que a popularização do uso do misoprostol como abortivo, em nosso meio, possa ser responsável pelo au-mento do número de casos da SM observado nos anos recentes. Estudos realizados em nosso país 5 aborto espontâneo. Tinha três irmãos saudáveis um deles, com história de convulsão febril.…”