Unilateral congenital fibrosis of the extraocular muscles with lid retraction: surgical treatment with a silicon plate on the orbital floor.

Fibrosis of the extraocular muscles can be an acquired or congenital disorder (CFEOM). The congenital disorder(1) is a complex strabismus with congenital restrictive ophthalmoplegia with or without ptosis. The surgery is challenging because the eye muscles are replaced by fibrous tissue or fibrous bands and in most cases the results are not satisfactory. We present the first case report of unilateral CFEOM with palpebral adherence and hypotropia, which was managed with our technique of a silicon plate implant on the orbital floor. The purpose of the implantation of the silicon plate in the orbital floor is to improve the hypotropia caused by CFEOM.

Clinical features associated with an I126M alpha2-chimaerin mutation in a family with autosomal-dominant Duane retraction syndrome.

PURPOSE: We describe the clinical phenotype of a Mexican family segregating Duane syndrome as an autosomal-dominant trait linked to chromosome 2q31 (DURS2) and previously reported to harbor a heterozygous alpha2-chimaerin missense mutation. METHODS: A 5-generation Mexican family was analyzed. Ten affected subjects were available for clinical examination. Participating subjects were tested for visual acuity, ocular alignment by prism cover testing, ocular ductions and versions, and globe retraction. In children, alignment was measured with the Krimsky test in cardinal positions of gaze. RESULTS: Ten cases were included, 6 female and 4 male subjects. Five cases presented with bilateral and 5 with unilateral Duane syndrome. The right side was the most commonly affected side on unilateral cases. Five cases exhibited exotropia, 4 esotropia, and 1 hypotropia. Seven patients had severe limitation of abduction and two had moderate limitation. Four patients had mild adduction limitation and 4 had moderate limitation. No additional anomalies such as fourth (trochlear) nerve palsy, blepharoptosis, or dense amblyopia, which have been reported in previous families with Duane syndrome, were observed. All 3 cases that exhibited vertical dysfunction had upgaze limitation. One instance of nonpenetrance was recorded. CONCLUSIONS: Considerable intrafamilial clinical variability was observed in this Duane syndrome pedigree that carried a alpha2-chimaerin mutation. The presence of bilateral involvement and associated vertical movements, which commonly are observed in this and other DURS2 families, could suggest the occurrence of CHN1 mutations as the source of the disease in isolated or familial DURS cases.

Resolución temprana con tratamiento médico en el estrabismo parético en pacientes con diabetes mellitus / Early resolution with medical treatment in the paretic strabismus in patients with diabetes mellitus

Objetivo: reconocer el mejor tratamiento para la pronto recuperación del nervio parético. Material y método: es un ensayo en comunidad, prospectivo y experimental. Se evaluaron 4 grupos: grupo 1: con tóxina botulínic, grupo 2: con carbamazepina oral, grupo 3: con complejo B oral y grupo 4: control metabólico. A todos los pacientes se les realizó exploración oftalmológica completa y estrabológica con prueba de ducción forzad, puntos de Worth y prismas, así como tiras reactivas para la determinación de glucosa en sangre cada 2 semanas y hemoglobina glucosilada al inicio y final del tratamiento, para su control. Resultados: del total de 80 pacientes estudiados (39 por ciento femeninos y 61 por ciento masculinos) con edad promedio de 60 años; el grupo 1 presentó un tiempo promedio de resolución más rápido (12.2 semanas), en comparación a los otros grupos. Conclusión: la resolución del cuadro parético es más rápida en los grupos tratados con toxina butolínica y carbamazepina (12 semanas), y más tardada en el grupo con complejo B (14 semanas).

Purpose: to recognize the best treatment for recovery of paretic craneal nerve. Material and methods: prospective, experimental and community trial, divided in four groups: group 1: with botulinum toxin, group 2: with oral carbamazepine, group 2: with oral carbamazepine, group 3: with oral vitamin B and group 4: with metabolic control. All pacients were evaluated with complete opthalmological and strabological examination with Worth, prism and force duction text; metabolic control was ascertained with glucosilade hemoglobine and a reactive blood test every two weeks. Results: A total of 80 patients were included (39 percent female and 61 percent male) with a mean age a 60 years old; group 1 showed the shortest mean time of resolution of 12.2 weeks compared to others groups. Conclusions: paretic resolution is faster with botulinum toxin and carbamazepine (12 weeks), and slower with vitamin B (14 weeks).

Síndrome de Duane tipo II / Duane's Syndrome tipe II

Se presenta el caso clínico de un síndrome de Duane Tipo II de acuerdo a la clasificación de Huber. Se discuten las características clínicas de acuerdo con otras clasificaciones.

Diagnóstico en Miastenia Gravis / Myasthemia gravis diagnosis

La miastenia gravis (MG) es uno de los desórdenes neuromusculares con involucro oftalmológico inicial más frecuente, por lo que el oftalmólogo debe conocer esta patología. Se debe sospechar de esta entidad en los casos en que se presenta ptosis y estrabismo con limitación en las ducciones; se deben realizar pruebas clínicas y de laboratorio para el diagnóstico de certeza. Se mencionan las pruebas clínicas, serológicas y electromiográficas diagnósticas más importantes, las ateraciones genéticas asociadas a este padecimiento y, brevemente, el tratamiento