Alvarez L A, Maytal J, Shinnar S
Pediatrics. 1986 Jun;77(6):901-7.
External hydrocephalus was identified in 63 infants. The 36 infants in whom external hydrocephalus was idiopathic constitute the study population. The group was homogeneous. Although not all were macrocephalic at birth, the head circumference exceeded the 95th percentile in all cases by 1 year of age, and subsequent head growth was parallel to the 95th percentile. The head computed tomographic scan showed a characteristic picture of a prominent interhemispheric fissure, a collection of subarachnoid fluid over the frontal convexities, and prominent basal cisternae. Mild ventriculomegaly was present in ten cases. These abnormalities resolved after 18 to 24 months of age. No infant required therapy for the condition. Development was normal in 32 infants, suspect in three, and clearly abnormal in only one of the 36 infants. However, of the 32 infants with normal development, 14 were found to be delayed in gross motor development and five in language development at a prior time. There was a family history of macrocephaly in 88% of cases. Several families had a mixture of members with external hydrocephalus and benign familial macrocephaly throughout several generations. These findings suggest that idiopathic external hydrocephalus is a relatively benign, self-limited condition that resolves without treatment and is closely related to benign familial macrocephaly.
63名婴儿被诊断为外部性脑积水。其中36名婴儿的外部性脑积水为特发性，构成了研究人群。该组具有同质性。虽然并非所有婴儿出生时头围都大，但到1岁时所有病例的头围均超过第95百分位数，随后的头围增长与第95百分位数平行。头部计算机断层扫描显示出特征性图像，即大脑半球间裂增宽、额部脑凸面蛛网膜下腔积液以及基底池增宽。10例存在轻度脑室扩大。这些异常在18至24个月龄后消失。没有婴儿因该病接受治疗。36名婴儿中，32名发育正常，3名可疑，仅1名明显异常。然而，在32名发育正常的婴儿中，之前发现14名粗大运动发育延迟，5名语言发育延迟。88%的病例有大头症家族史。几个家族在几代人中都有外部性脑积水患者和良性家族性大头症患者的混合情况。这些发现表明，特发性外部性脑积水是一种相对良性的自限性疾病，无需治疗即可缓解，且与良性家族性大头症密切相关。
