Azais M, Echenne B
Service de Neuropédiatrie, Centre Gui de Chauliac, Montpellier.
Ann Pediatr (Paris). 1992 Nov;39(9):550-8.
Benign enlargement of the subarachnoid spaces was diagnosed in 41 infants on the basis of ultrasound and/or CT scan findings. 10 MHz transfontanellar ultrasonography is without doubt the most reliable investigation in this condition (skull-to-cortex distance greater than 5 mm). Patients with extracerebral collections due to a clearly identifiable pathologic process (e.g., prematurity, IUGR, neonatal distress, malnutrition) were excluded from the study. Macrocrania developed rapidly in 72% of patients, either as the single manifestation (30%) or with delayed motor development and hypotonia (30%). Other clinical patterns included evidence of intracranial hypertension (15%) and hypotonia without macrocrania (20%). The two main findings of this study were the high rate of familial forms and the severity of early hemorrhagic complications, i.e., spontaneous subdural hematoma (5/41 cases), with permanent neurologic impairment in some instances (2/5 cases). These complications call into question the benignity of this syndrome whose long-term outcome, particularly in terms of cognitive function, is as yet unknown.
根据超声和/或CT扫描结果，41例婴儿被诊断为蛛网膜下腔良性扩大。10兆赫经囟门超声检查无疑是诊断这种情况（颅骨至皮质距离大于5毫米）最可靠的检查方法。因明确可识别的病理过程（如早产、宫内生长受限、新生儿窘迫、营养不良）导致脑外积液的患者被排除在研究之外。72%的患者出现巨头症，要么作为单一表现（30%），要么伴有运动发育迟缓及肌张力减退（30%）。其他临床模式包括颅内高压证据（15%）和无巨头症的肌张力减退（20%）。本研究的两个主要发现是家族性形式的高发生率以及早期出血性并发症的严重性，即自发性硬膜下血肿（5/41例），在某些情况下会导致永久性神经功能损害（2/5例）。这些并发症让人质疑该综合征的良性性质，其长期预后，尤其是在认知功能方面，目前尚不清楚。
