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Cirugía Plástica Ibero-Latinoamericana
On-line version ISSN 1989-2055Print version ISSN 0376-7892
Abstract
ARRIETA-JOFFE, Pablo; PEREZ-DOSAL, Marcia; ORTIZ-DE-ZARATE, Gabriela and CARDENAS-MEJIA, Alexander. Clinical, cytogenetic, molecular and image study of patients with Mobius syndrome at the General Hospital “Dr. Manuel Gea González”, Mexico city. Cir. plást. iberolatinoam. [online]. 2017, vol.43, n.4, pp.395-400. ISSN 1989-2055. https://dx.doi.org/10.4321/s0376-78922017000500010.
Background and Objective:
Möbius syndrome is a rare congenital disease characterized by facial paralysis associated with an absence of abduction of the eyes for abnormalities in VI and VII cranial nerves. The pathogenesis has different hypothesis that include genetic, vascular and teratogenic causes. There are few reports in the literature and especially in Latin America to describe the clinical and genetic characteristics of these patients.
The current study is the result of a multidisciplinary team developed in our center to describe the wide spectrum of the disease and offer the best treatment options to each of our patients.
Methods:
We analyzed 115 patients with the diagnosis of Möbius syndrome in its 3 presentations. All patients underwent a complete clinical examination by a multidisciplinary team formed by orthopedist, ophthalmologist, otolaryngologist, orthodontist, neurologist, plastic surgeon, pediatrician and geneticist. They underwent CTG banded karyotype to identify structural chromosome abnormalities.
Results:
Fifty two patients (45%) patients were male and 63 (55%) female. Clinical manifestations were found with unilateral or bilateral facial paralysis with VI nerve involvement in 100% of patients, associated with strabismus in 62.6%, 46.1% clubfoot, simple syndactyly 15.7%, 17.4% cleft palate, micrognathia 17.4%, Poland syndrome 9.6%, among others. Cytogenetic analysis showed normal karyotype in 114 patients and a reciprocal translocation between chromosome 4 and 10 in 1 patient. Sixteen cases of reported intake of misoprostol during the first trimester.
Conclusions:
As far as we know, this study is the largest global cohort reported in a single hospital of patients with Möbius syndrome. Variability of the clinical presentation justifies the management of these patients is a multidisciplinary team. This study opens the door for new studies that allow us to understand the pathophysiology of this disease and its response to different treatments.
Keywords : Moebius syndrome; Möbius syndrome; Facial nerve; Congenital facial palsy; Abducens nerve.
