Murovic J A, Posnick J C, Drake J M, Humphreys R P, Hoffman H J, Hendricks E B
Department of Neurosurgery, Mount Sinai Medical Center, New York, N.Y.
Pediatr Neurosurg. 1993 May-Jun;19(3):151-5. doi: 10.1159/000120720.
A retrospective evaluation was carried out to define the incidence of hydrocephalus and associated factors in 44 patients with Apert syndrome treated at The Hospital for Sick Children in Toronto over a 22-year period. Forty-three of these patients underwent cranioorbital decompressive procedures within 1 year of birth. Fifteen of 25 (60%) patients who had either a computed tomography scan or pneumoencephalogram had ventriculomegaly, and 3 of the 25 (12%) had associated brain anomalies. Ten of the 44 (23%) patients had cerebrospinal fluid (CSF) shunts placed, 7 lumboperitoneal and 3 ventriculoperitoneal. Six of the shunts were placed early after cranioorbital procedures (CSF leaks in 5 cases and a subgaleal fluid collection in 1 case). The average IQ of 15 patients evaluated by the Wechsler Intelligence Scale was 72.5, indicative of significant intellectual impairment. There was no correlation between IQ and ventricular size. Although hydrocephalus characterized by progressive ventricular dilatation is uncommon in Apert syndrome, postoperative problems related to impaired CSF circulation are common and may indicate an underlying CSF absorptive deficit.
进行了一项回顾性评估，以确定22年间在多伦多病童医院接受治疗的44例Apert综合征患者中脑积水的发生率及相关因素。其中43例患者在出生后1年内接受了颅眶减压手术。在25例进行了计算机断层扫描或气脑造影的患者中，15例（60%）有脑室扩大，25例中有3例（12%）伴有脑异常。44例患者中有10例（23%）进行了脑脊液（CSF）分流术，7例为腰大池-腹腔分流，3例为脑室-腹腔分流。6例分流术在颅眶手术后早期进行（5例脑脊液漏，1例帽状腱膜下积液）。通过韦氏智力量表评估的15例患者的平均智商为72.5，表明存在明显的智力障碍。智商与脑室大小之间无相关性。虽然以进行性脑室扩张为特征的脑积水在Apert综合征中并不常见，但与脑脊液循环受损相关的术后问题很常见，可能提示存在潜在的脑脊液吸收缺陷。
