Deeg K H
Monatsschr Kinderheilkd. 1984 Nov;132(11):854-60.
22 children with myelomeningocele had serial examination by gray scale ultrasonography through the open fontanelle as an acoustic window. 18 children (82%) developed a progressive hydrocephalus and were eventually shunted. In 18 children the hydrocephalus was caused by an Arnold-Chiari-II-malformation with the sonografic signs of a caudal displacement of the dysplastic cerebellum, brainstem, 4th ventricle and cisterna magna. The forth ventricle was elongated and flattened in all babies with Arnold-Chiari-syndrome. The cisterna magna was not visible in any child. The Arnold-Chiari-II-malformation caused an occlusive hydrocephalus which was characterized by the dilatation of the occipital horns (91%) and frontal horns (86%) of the side ventricles, whereas the temporal horns were normal in size (86%). The dysplastic 3. ventricle was displaced caudally and ventrally and showed a prominent suprapineal recess in 41%. Associated malformations of the brain were a large massa intermedia (55%), a prominent plexus choriodeus (50%) and agenesia (36%) or fenestration (9%) of the septum pellucidum. Gray scale ultrasonography is the best method not only for early diagnosis of Arnold-Chiari-malformation and resulting hydrocephalus in infants but also for further controls after shunt implantation, especially for early detection of shunt complications and shunt insufficiency.
22名患有脊髓脊膜膨出的儿童通过前囟作为声窗进行了灰阶超声连续检查。18名儿童(82%)出现进行性脑积水,最终接受了分流手术。在18名儿童中,脑积水由Arnold-Chiari-II畸形引起,超声检查显示发育不良的小脑、脑干、第四脑室和枕大池尾端移位。所有患Arnold-Chiari综合征的婴儿第四脑室均拉长变平。所有儿童均未见到枕大池。Arnold-Chiari-II畸形导致梗阻性脑积水,其特征为侧脑室枕角(91%)和额角(86%)扩张,而颞角大小正常(86%)。发育不良的第三脑室尾端和腹侧移位,41%可见松果体上隐窝明显。脑部相关畸形包括大脑中间块大(55%)、脉络丛明显(50%)以及透明隔缺如(36%)或透明隔开窗(9%)。灰阶超声不仅是早期诊断婴儿Arnold-Chiari畸形及所致脑积水的最佳方法,也是分流植入术后进一步监测的最佳方法,尤其有助于早期发现分流并发症和分流功能不全。