Mobius Sequence Research Articles

Long-term outcomes of strabismus surgery in Mobius sequence

ABSTRACTPurpose: Mobius sequence is a rare disorder that results from underdevelopment of the sixth and seventh cranial nerves, with subsequent facial weakness and impairment of ocular abduction. Approximately half of the affected patients have esotropia with limitation of extraocular movements. This study retrospectively reviews the long-term outcome of strabismus surgery for such patients.Methods: The long-term follow-up results in five children with Mobius sequence, who were observed after strabismus surgery for congenital esotropia, were analyzed.Results: All patients had a limitation of abduction in both eyes. Preoperative esotropia ranged from 20 to 30 prism diopters (PD) (mean 27 PD). Patient age at surgery ranged from 4.5 to 14 months. Bilateral medial rectus muscle recession was performed in all cases, ranging from 4.0 to 6.5 mm. Inferior transposition of the medial rectus muscles was performed in two patients for treatment of V-pattern, and two patients also had surgery for vertical strabismus. Follow-up ranged from 4 to 19.5 years (mean 11.7 years). The final horizontal alignment was <8 PD in all patients, and all demonstrated some binocularity.Conclusion: Bilateral medial rectus muscle recession is an effective treatment for esotropia associated with Mobius sequence, with good long-term stability.

Congenital Anomalies Associated With Failed Misoprostol Termination Of Pregnancy

Misoprostol exposure in the first trimester of pregnancy has been related to congenital malformations, particularly the mobius sequence and terminal transverse limb defects. An unusual case of a 25-years old primigravida who was exposed to misoprostol at 8-weeks gestation is presented here. Failing to medical termination, the pregnancy continued and a live baby girl was delivered preterm at 35-weeks gestation with multiple anomalies like fronto-nasal bone, scalp defects and protrusion of duramater. Microcephaly and equinovarus deformities were also present. Hence, clinicians must be aware of the teratogenic risk of misoprostol with especial context to its unsuccessful use in the first trimester of pregnancy. For the ongoing pregnancies, patient must also be informed in detail.

Reproductive results associated with misoprostol and other substances utilized for interruption of pregnancy

Sir, In Brazil, the main use of misoprostol is to induce abortion, even though this practice is illegal. There are only two situations in which abortion is not a criminal act: when the pregnant woman’s life is at risk and when pregnancy has resulted from rape or incest. The use of misoprostol as an abortion-inducing substance may lead to a situation in which the pregnancy is not lost [1], thereby generating anxiety regarding the risk to the fetus and the pregnant woman. Case and case-control studies have suggested that the use of misoprostol in unsuccessful abortions may be associated with congenital malformations, for example in children with limb reduction defects and/or Mobius Sequence [2, 3] and anomalies of the central nervous system [4]. However, in one cohort study correlating the use of misoprostol with teratogenic effects, no difference was found in the rates of congenital malformations between 86 cases of exposure to misoprostol during pregnancy and 86 cases without exposure to this drug [5]. In this letter, we report an association observed between the use of misoprostol during the first trimester of pregnancy and congenital malformation of the child or fetal death, from the Brazilian Study of Gestational Diabetes, a cohort of women attending prenatal services at public hospitals in six Brazilian cities, between February 1991 and June 1995. Data relating to the use of misoprostol and other substances with the potential to induce abortion during the pregnancy (obtained during the first phase of the cohort) and data relating to the neonate’s state of health (obtained during the third phase of the cohort) were analyzed. Interviews were held with 5,564 women aged 20 years or over who were between their 21st week and 28th week of pregnancy (Table 1). Information on the use of substances with the potential to induce abortion was obtained from the interviews with the pregnant women. The diagnosis of congenital malformation was made by the attending doctor and entered in the pregnant woman’s records. The present analysis included the pregnant women who were followed through to the third phase of the cohort, thus resulting in a sample of 4,862 women (87.4% of the initial sample). Use of substances with the potential to induce abortion was reported by 707 pregnant women (14.6%); of these, 120 declared that they had used misoprostol during the first trimester of pregnancy. More cases of congenital malformation were found in the group exposed to misoprostol than in the unexposed group (RR=2.39), with threshold significance (95% CI 0.99–5.80) (Table 2). Using a logistic regression model that included the variables of skin color, marital situation and research center, the risk increased slightly (RR=2.61; 95% CI 1.01–6.75). The malformations that occurred in the sample of pregnant women who made use of misoprostol were: syndactyly, twisted foot, meningomyelocele, microcephaly and fingernail defects. A significant difference was also observed between the women with and without exposure to misoprostol, in relation to fetal death (RR=2.63; 95% CI 1.17–5.88 (Table 3). After adjustment using the regression model, the relative risk increased to 3.21 (95% CI 1.34–7.68). The data available in the literature on the effects on the fetus and child mostly come from case reports and case-control studies of limited sample size. Moreover, they do not include adverse events other than the congenital malformations that are important for the outcome of the pregnancy. The prospective populationbased study that included a cohort of 86 cases and 86 externally selected controls did not have sufficient T. da Silva Dal Pizzol (&) University of Passo Fundo, Rua Paissandu, 1973, apto 604, Passo Fundo, Rio Grande do Sul, 99010-102, Brasil E-mail: tatiane@saude.upf.tche.br

Mobius Syndrome and Autistic Spectrum Disorder

The relation between autistic spectrum disorder and Mobius syndrome was studied in 25 patients (18 males and 7 females) included in a multidisciplinary study of Mobius sequence at the Department of Child and Adolescent Psychiatry, Goteborg University, Sweden.

Congenital abnormalities in Brazilian children associated with misoprostol misuse in first trimester of pregnancy

Misoprostol is commonly used to induce abortion in Brazil, and in other countries in South and Central America where abortions are illegal. However, misoprostol is not very effective in inducing abortions, and exposure to the drug in utero can cause abnormalities in the fetus. We aimed to define the common phenotypical effects of exposure to the drug. We studied 42 infants from São Paulo, Brazil, who were exposed to misoprostol during the first 3 months of gestation, and then born with congenital abnormalities. We interviewed each of the infants' mothers to find out about misoprostol exposure and dosage. Each infant was physically examined by a geneticist or a neuropaediatrician. 17 of the infants had equinovarus with cranial-nerve defects. Ten children had equinovarus as part of more extensive arthrogryposis. The most distinctive phenotypes were arthrogryposis confined to the legs (five cases) and terminal transverse-limb defects (nine cases) with or without Mobius sequence. The most common dose of misoprostol taken was 800 microg (range 200-16000 microg). Deformities attributed to vascular disruption were found in these children. We suggest that the uterine contractions induced by misoprostol cause vascular disruption in the fetus, including brain-stem ischaemia. Information on the effects of taking misoprostol during pregnancy should be made more widely available, to dissuade women from misusing the drug.

Prenatal Sonographic Evidence Supporting an in Utero Developmental Etiology of Möbius Sequence

Möbius sequence features masklike facies with sixth and seventh nerve palsy and frequently micrognathia thought to result from a neuromuscular deficiency in early movement of the mandible. Failure to thrive in infancy is commonly due to feeding and aspiration difficulties. We present the first description of prenatal sonographic findings associated with this lesion, which also support an in utero developmental etiology of this rare condition.

The Riemann Hypothesis and Pseudorandom Features of the Mobius Sequence

The Riemann Hypothesis and Pseudorandom Features of the Mobius Sequence