Rare case of congenital transsphenoidal meningoencephalocele causing respiratory problems

INTRODUCTION: Congenital meningoencephalocele is a rare and severe malformation. AIMS: The authors describe the case of congenital, basal transsphenoidal meningoencephalocele associated with other neurological malformations, which projected between the margins of palatoschisis producing respiratory problems at neonatal age. METHOD: Presented with detailed photo-documentation. RESULTS: Cranial meningoencephalocele reconstruction with an approach of bifrontal craniotomy was performed. The base of skull was closed multilaminarly with auto-graft gained from parietal bone and Lyodura. The closure of nasopharyngeal sac was performed in second sitting. Postoperatively the progression of hydrocephalus was so extensive, that implantation of ventriculo-peritoneal shunt was unavoidable. Later on closure of tracheotomy performed for continued air passage support was done. The authors report the course of disease and the surgeries performed. CONCLUSIONS: Auto-graft from skull bone for closure of large basal-cranial defect proved to be ideal. Prognosis depends on other malformations. Successful treatment needs co-operation of different specialties.