[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.