|摘要||目的 结合1例蝶筛型脑膜脑膨出伴脑积水合并唇腭裂、左眼球发育不良、胼胝体缺如病例,复习相关文献分析其形成原因并探讨其治疗方法 .方法 患儿,女,4岁.上腭底部有反复膨出肿物并进行性增大4年伴尿量增多2年入院.查体发现患儿有唇腭裂、左眼球发育不良、鼻中隔软骨缺损及语言发育障碍,口腔内可见硬腭顶部有膨出肿物,质软,哭闹时增大.CT及MRI显示脑积水,胼胝体缺如,第三脑室、垂体、下丘脑、部分视路经蝶筛部骨缺损疝入口腔.完善检查后行Ⅰ期脑室-腹腔分流,Ⅱ期行经前颅窝底硬膜外入路脑膜脑膨出复位修补术.结果 手术后MRI显示疝出的下丘脑、垂体、视路基本恢复到正常解剖位置,患儿语言功能及尿崩症明显好转.结论 此例患儿疾病产生可能与妊娠早期组织诱导缺陷及神经外胚层与表皮外胚层分离障碍相关,对此类患者Ⅰ期行脑室-腹腔分流减轻脑组织压迫,Ⅱ期行颅底脑膜脑膨出修补以恢复颅底正常解剖结构是行之有效的方法 .
ObjectiveWe report a case of sphenoethmoidal encephalocele combined with hydrocephalus, labial fissure, palatine fissure, left optic organ hypoplasia and agenesis of the corpus callosum and discuss its mechanism, clnical fcature and therapy. Method The patient was a 4 - year - old girl. She was noted to have a palpable, plusatile mass under the palate for 4 years and hydrouria for 2 years. Inspection revealed that crying could increase its volume and she also had labial fissure, palatine fissure, left eyeball hypoplasia, median nasal fissure and mental deficiency. CT and MRI scan showed hydrocephalus, agenesis of corpus callosum, and the third ventricle, pituitary, hypothalamus and visual pathway herniated to oral cavity from the defect of the sphenethmoid bone. V - P shunt was done firstly and 3 months later, the second operation was done to reconstruct the skull base and watertight closure of the dural defect. Results Hypothalamus, pituitary and visual pathway were repositioned after surgery, the language developement and diabetes insipidus improved obviously.Conclusion The concurrence of sphenoethmoidal cephalocele with cerebral and craniofacial malformation may be associated with defective tissular induction with malformation early in embryogenesis and disturbance in the separation of neural and surface ectoderm during the final part of neural tube formation. V - P shunt and then skull base reconstruction may be a useful method for this kind of disease.|