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先天性Magendie孔及Luscka孔闭锁通常称Dandy-Walker氏综合征。笔者曾治疗1例,结合文献对其治疗及预后进行探讨。 患儿 男,6岁。足月顺产,产后无明显颅内感染史,婴幼儿期运动,语言、思维明显落后于同龄儿。入院时语言发音困难只能发单音字,对外界刺激无反应,表现为脑发育不全。检查:对强痛刺激有轻微表情反应,躯干及四肢不发育,四肢肌张力低,腱反射活跃,病理反射阴性。CT示全脑系统包括中脑导水管扩张,小脑蚓部缺如,两侧小脑半球呈球形。全麻枕下入路行后颅窝探查术,见小脑半球间有3×2.5×2.5cm透明囊,囊壁菲薄,顶部
Congenital Magendie holes and Luscka hole closure are often called Dandy-Walker’s syndrome. I have treated 1 case, combined with the literature on its treatment and prognosis. Children male, 6 years old. Full-term follow-up, postpartum no obvious history of intracranial infection, early childhood movement, language, thinking significantly behind their peers. Language dysphonia admission only single-tone, no response to external stimuli, manifested as hypoplasia. Check: Strong pain stimulation with a slight expression reaction, trunk and limbs are not developed, limb muscle tension is low, tendon reflexes active, pathological reflex negative. CT showed the whole brain system, including the dilatation of the midbrain aqueduct, lack of cerebellar vermis, cerebellar hemispheres on both sides of the ball. Anesthesia suboccipital approach after posterior fossa exploration, see the cerebellar hemispheres 3 × 2.5 × 2.5cm transparent capsule, thin wall, the top