Fücsek Mihály, Szarvas István, Kelemen Agnes, Pohárnok László, Horváth Imre
Petz Aladár Megyei Oktató Kórház, Csecsemó- és Gyermekgyógyászati Központ, Gyór.
Orv Hetil. 2004 Feb 29;145(9):483-9.
Congenital meningoencephalocele is a rare and severe malformation.
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.
Presented with detailed photo-documentation.
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.
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.
先天性脑膜脑膨出是一种罕见且严重的畸形。
作者描述了一例先天性经蝶窦基底脑膜脑膨出合并其他神经畸形的病例，该脑膜脑膨出在腭裂边缘之间突出，在新生儿期导致呼吸问题。
提供详细的照片记录。
采用双额开颅术进行颅脑膜脑膨出重建。用取自顶骨和人工硬脑膜的自体移植物多层封闭颅底。在第二次手术中封闭鼻咽囊。术后脑积水进展广泛，以至于不可避免地进行了脑室 - 腹腔分流术植入。后来为持续的气道支持而进行的气管切开术被关闭。作者报告了疾病过程和所进行的手术。
颅骨自体移植物用于封闭大型颅底缺损被证明是理想的。预后取决于其他畸形。成功的治疗需要不同专科的合作。
