Síndrome de Moebius y ortotropía en posición primaria: ¿es infrecuente esta asociación? (Colombia) / Moebius syndrome and orthotropia in primary position: is this association uncommon? (Colombia) / Síndrome de Moebius e ortotropia em posição primária: é uma associação incomum? (Colômbia)

El síndrome de Moebius es una enfermedad congénita poco común que se caracteriza por el compromiso unilateral o bilateral del VI y VII par craneal, lo que compromete los músculos que controlan la oculomotricidad, produciendo una parálisis en la abducción del globo ocular y los músculos involucrados en la expresión facial. Su presentación clínica y grados de severidad son variables, puede presentar compromiso simétrico o asimétrico. Adicionalmente, gran parte de los casos se relacionan con trastornos del lenguaje, anomalías musculoesqueléticas y orofaciales. En el presente artículo se presenta el caso de una paciente femenina de 3 años producto de un embarazo trigemelar con diagnóstico clínico de síndrome de Moebius al nacer, confirmado por neuroimagen en la que se evidencia la ausencia bilateral del nervio facial en ángulos pontocerebelosos, adicionalmente con un defecto completo en los movimientos oculares de abducción y aducción lo que impide el estrabismo convergente común en estos pacientes.

Moebius syndrome is a rare congenital disease characterized by unilateral or bilateral involvement of the VI and VII cranial nerves, which compromises the muscles that control ocular motricity with paralysis in the abduction of the eyeball and the muscles involved in the facial expression. Its clinical presentation and degrees of severity are variable, and it can be symmetric or asymmetric. Additionally, most of the cases are related to language disorders, musculoskeletal and orofacial anomalies. This paper presents the case of a 3-year-old female patient, product of a trigemellar pregnancy with a clinical diagnosis of Moebius syndrome at birth, confirmed by neuroimaging, which shows the bilateral absence of the facial nerve in point-lateral angles. Additionally she has a complete defect in abduction and adduction eye movements, which prevents the common convergent strabismus in these patients.

A síndrome de Moebius é uma doença congênita rara caracterizada pelo envolvimento unilateral ou bilateral dos nervos cranianos VI e VII, que compromete os músculos que controlam a oculomotricidade com paralisia na abdução do globo ocular e dos músculos envolvidos na expressão facial. Sua apresentação clínica e graus de gravidade são variáveis, podendo ser um comprometimento simétrico ou assimétrico. Além disso, a maioria dos casos está relacionada a distúrbios de linguagem, anomalias musculoesqueléticas e orofaciais. Este paper apresenta o caso de uma paciente de 3 anos de idade, fruto de uma gravidez trigêmea com diagnóstico clínico de Síndrome de Moebius ao nascimento, confirmado por neuroimagem em que é evidente a ausência bilateral do nervo facial nos ângulos ponto-cerebelares. Além disso, ela tem um defeito completo nos movimentos oculares de abdução e adução, o que impede o estrabismo convergente comum nesses pacientes.

Parálisis del nervio motor ocular externo luego de anestesia neuroaxial combinada epidural-espinal / Paralysis of the external oculomotor nerve after combined epidural-spinal neuroaxial anesthesia

Introducción: El abordaje del espacio subaracnoideo fue descrito por Quincke en el 1891. En la actualidad es práctica común para la realización de la anestesia neuroaxial subaracnoidea en las pacientes obstétricas. Las complicaciones descritas, asociadas a esto, son varias. Dentro de estas, la parálisis del nervio abducens o VI par no es frecuente y en ocasiones, no está relacionada a la punción ya que se produce días después del evento. Objetivo: Revisar la información relacionada con la complicación de parálisis del VI par. Presentación del caso: Paciente de 33 años de edad, femenina, de profesión médico, con antecedentes personales de migraña, historia de anestesia neuroaxial epidural sin complicaciones, que para la realización de una cesárea de segmento arciforme y salpinguectomia parcial bilateral, recibió una anestesia combinada peridural-espinal. El transoperatorio transcurre con estabilidad hemodinámica, hizo cefalea al tercer día del posoperatorio, que la atribuyó al antecedente de migraña y fue tratada sin evaluación por anestesiología con dipirona. A los 10 días de operada hace desviación de la mirada y diplopia, se diagnostica parálisis del VI par. Fue tratada por Neurología y se plantean varios diagnósticos diferenciales. Los estudios imagenológicos resultan negativos, se trató con vitaminas y se produjo remisión a las 6 semanas. Conclusiones: El diagnóstico de esta complicación es necesario ya que puede pasar inadvertida la relación con la anestesia y, por tanto, ser mal conducido su tratamiento(AU)

Introduction: The approach to the subarachnoid space was described by Quincke in 1891. It is now a common practice to perform subarachnoid neuroaxial anesthesia in obstetric patients. The complications described, associated with this, are several. Within these, the paralysis of the abducens nerve or sixth pair is not frequent and sometimes is not related to the puncture, since it occurs days after the event. Objective: To review the information related to the complication of paralysis of the sixth pair. Case presentation: A 33-year-old female patient, a physician, with a personal history of migraine, a history of epidural neuroaxial anesthesia without complications, who underwent combined epidural-spinal anesthesia for performing a cranial segment cesarean section and bilateral partial salpingectomy. The transoperative period runs with hemodynamic stability. There was headache three days after surgery, which was attributed to the migraine history and the patient was treated, without evaluation by anesthesiology, with dipyrone. At 10 days after surgery, the eyes are diverted and diplopia is manifested, paralysis of the sixth pair is diagnosed. She was treated by neurology and several differential diagnoses were proposed. Imaging studies are negative. She was treated with vitamins and remission occurred at six weeks. Conclusions: The diagnosis of this complication is necessary, since the relationship with anesthesia may go unnoticed and, therefore, its treatment may be poorly conducted(AU)

A Case of Herpes Zoster with Abducens Palsy

Only a few reports have focused on ocular motor paralysis in herpes zoster ophthalmicus. We report a case of ocular motor paralysis resulting from herpes zoster. The patient, an 80-yr-old woman, presented with grouped vesicles, papules, and crusting in the left temporal area and scalp, with diplopia, impaired gaze, and severe pain. Her cerebrospinal fluid analysis was positive for varicellar zoster virus IgM. Magnetic resonance imaging was performed to rule out other diseases causing diplopia; there were no specific findings other than old infarctions in the pons and basal ganglia. Therefore, she was diagnosed of abducens nerve palsy caused by herpes zoster ophthalmicus. After 5 days of systemic antiviral therapy, the skin lesions improved markedly, and the paralysis was cleared 7 weeks later without extra treatment.

Presentations of cranial nerve involvement in two patients with Herpes zoster ophthalmicus.

Two cases of Herpes zoster ophthalmicus complicated by motor nerve palsies are being reported. The investigations ruled out other diseases which can affect ocular motor nerves, e.g., diabetes, hypertension, syphilis and malignancy. The cases are being reported because of the rare presentations of Herpes zoster ophthalmicus like isolated internal ophthalmoplegia and VI nerve palsy in Case-1 and absence of iritis with third nerve involvement in Case-2. The probable etiology for occurrence of these uncommon phenomena has been postulated.