Transpalatine Route for the Correction of Transsphenoidal Encephalocele in a Child: A Case Report and Literature Review

Introduction Transsphenoidal encephalocele is a rare neural tube defect characterized by the herniation of meninges and eventually of parts of the brain through a bony defect in the sphenoid bone. The clinical presentation is variable, and surgical treatment is controversial. Case Report This report describes the case of an 8-month-old female child diagnosed with transsphenoidal encephalocele. The child presented with obstruction of the upper airways and was fed via a nasogastric tube but did not present changes in the hypothalamic-pituitary-axis. The patient underwent surgery with the transsphenoidal­transpalatine route, with an excellent outcome and without fistulas or infections. Conclusions Although transsphenoidal encephalocele is a rare congenital anomaly, the transsphenoidal­transpalatine route for the correction of this type of encephalocele is a safe option and produces a favorable outcome in pediatric patients.

Introdução A encefalocele transesfenoidal é um defeito do tubo neural raro, caracterizado por herniação de meninges e eventualmente partes do cérebro através de uma falha óssea no osso esfenoide. A apresentação clínica é variável e o tratamento cirúrgico é controverso. Relato de Caso No presente trabalho descrevemos o caso de uma criança de 8 meses, sexo feminino, com diagnóstico de encefalocele transesfenoidal. Apresentava-se com obstrução de vias aéreas superiores, alimentando-se por sonda nasoenteral e sem alterações do eixo hipotálamo-hipofisário. Tratada com cirurgia via transesfenoidal transpalatal, evoluiu com excelente resultado, sem fístulas e sem infecções. Conclusões Embora a encefalocele transesfonoidal seja uma anomalia congênita rara, a via transpalatina-transesfenoidal para correção deste tipo de encefalocele é uma opção segura e produz um resultado favorável no paciente pediátrico.

Lesiones quísticas de línea media de base de cráneo anterior / Cystic lesions in midline of anterior skull base

Las lesiones quísticas en línea media del segmento posterior de la base de cráneo anterior corresponden mayoritariamente a malformaciones congénitas, cuya ubicación puede ser selar, supraselar o intraesfenoidal. Generalmente asintomáticas, pueden presentar síntomas determinados por su crecimiento y/o el compromiso mecánico sobre las estructuras adyacentes. Su diagnóstico suele realizarse producto de un hallazgo imagenológico o endoscópico, constituyendo la resonancia magnética la mejor alternativa ante una aproximación diagnóstica inicial. Estas lesiones quísticas presentan un comportamiento benigno y no todas requieren un manejo quirúrgico, siendo suficiente en la gran mayoría de ellas, un seguimiento clínico. La necesidad de cirugía deberá decidirse en cada caso y depende principalmente de la sintomatología producida por la compresión de estructuras vecinas e hipertensión endocraneana. El abordaje quirúrgico endoscópico extendido es el método resectivo de elección. Presentamos una serie de casos clínicos, correspondiente a cinco pacientes con lesiones quísticas en línea media de base de cráneo anterior. Tanto la sintomatología como el diagnóstico etiológico tuvieron una presentación diversa en la serie expuesta; independiente de lo cual, todos fueron manejados exitosamente por cirugía endoscópica. Presentamos finalmente, una propuesta de diagnóstico inicial, basado en el estudio imagenológico con resonancia magnética de estas lesiones.

Cystic lesions in the midline of the anterior skull base are rare and mostly congenital malformations. Usually asymptomatic, symptoms can be determined by their growth and/or mechanical compromise on adjacent structures. The diagnosis is usually the result of imaging or endoscopic findings, MRI is the best alternative for the initial diagnostic approach. These cystic lesions are benign xx and not all require a surgical management. Some of them require a watchfull follow up. If they need surgery the expanded endoscopic approach is the method of choice in our hands. We present a case series, correspondent to five patients with cystic lesions in the midline of the skull base. All these were successfully managed by an extended endoscopic approach. Finally, we presented a proposal for initial diagnosis approach, based on the MRI findings of these lesions.

Results of surgical treatment of meningoceles and menigo-encephalocelles in 16 operated cases in Saint- Paul hospital

During 3 years over 400 cases with neurological diseases in infants were operated on at the Dep. of Neurosurgery of Hanoi Saint-Paul hospital. Among them, there were only 16 cases with meningocele and meningo-encephalocele (congenital malformations). These patients were from 2 days to 16 months of age. To obtain good results, the diagnosis and treatment must be made early in the first days of life of the children. Results: among 16 cases: good:15, death:1