Achados oftalmológicos em 28 crianças portadoras da seqüência de Möebius

Abstract

INTRODUÇÃOA seqüência de Möebius, definida como uma paresia ou paralisia congênita do sexto e sétimo nervos cranianos, está freqüentemente associada à paralisia de outros nervos cranianos, anomalias craniofaciais e músculo-esqueléticas (1)(2)(3)(4)(5) . Sua etiopatogenia ainda não está perfeitamente esclarecida. A teoria mais aceita sugere que existiria um mecanismo de insuficiência vascular em idade gestacional precoce, desencadeada por uma série de fatores heterogêneos, dentre os quais se destacam anomalias cromossômi-cas (6)(7)(8) , exposição a agentes ambientais como a talidomida (9) , cocaína (10) , benzodiazepnicos (11) , álcool (3) e, em especial, o misoprostol (12)(13)(14)(15)(16) , o que levaria a produção da seqüência de Möebius (5,(17)(18) . Em virtude das alterações multisistêmicas da seqüência de Möebius, observa-se que a conduta apropriada varia de acordo com a gravidade e tipo das malformações presentes. A conduta oftalmológica deve ser dirigida para o estrabismo e para exposição corneana, essa última podendo ocorrer, principalmente ao dormir, devido ao acometimento do VII nervo craniano, devendo-se também tratar as ametropias e a ambliopia, se presente (3) . Freqüentemente os genitores e pacientes com a seqüência de Möebius

Further considerations about the ophthalmic features of the Möbius sequence, with data of 28 cases

“…We have seen many cases in which there is no esotropia in the primary position ( Figure 2A) and even some cases, though rare, with exotropia ( Figure 3), which coincides with the series of other authors (6)(7)(8)(9) . It is difficult to imagine an exotropia or even an orthotropia in the presence of an isolated paralysis of the lateral recti.…”

“…The binocular alignment in primary position and the incidence of abduction and adduction limitations among 28 of our consecutive patients with Möbius sequence and in patients of 5 other randomly selected publications are presented for comments (6)(7)(8)(9)(10) (Tables 1 and 2). …”

Teratogen exposure and congenital ocular abnormalities in Brazilian patients with Möbius sequence

“…The findings of the present study corroborated with previous studies that reported ocular motility defects, palpebral ptosis, aberrant tearing, corneal opacification, refractive errors, and amblyopia as the main ocular findings in misoprostol-exposed patients during pregnancy (7,27,32,33) . The most common eye motility pattern in patients with Möbius sequence is horizontal gaze paresis that is usually associated with marked abduction limitations and variable adduction deficits (32,33) . In the present series, strabismus was observed in 73.2%, and the most frequent form was esotropia (80%).…”

“…Maternal exposure to misoprostol during organogenesis (first trimester) does not induce abortion in most cases, but it is frequently associated with the appearance of congenital anomalies, such as skullcap, cranial nerve and limb defects, arthrogryposis, facial anomalies, and Mobius sequence (6,7,10) . The findings of the present study corroborated with previous studies that reported ocular motility defects, palpebral ptosis, aberrant tearing, corneal opacification, refractive errors, and amblyopia as the main ocular findings in misoprostol-exposed patients during pregnancy (7,27,32,33) . The most common eye motility pattern in patients with Möbius sequence is horizontal gaze paresis that is usually associated with marked abduction limitations and variable adduction deficits (32,33) .…”

“…Prior investigations have reported this same symptom in patients with Möbius sequence in Northeastern Brazil and Italy in 45% and 17.9% of children, respectively (13,22) . Lagophthalmos was also found in more than one-third of the individuals with Mobius sequence who were assessed in the present study, and this was a higher percentage than the percentage described in a previous study (14.3%) (32) . The main limitations of the current study were the sample size and the low prevalence of target outcomes due to Möbius sequence being a rare disorder.…”