颅内静脉异常回流致脑积水一例及文献复习

目的 探讨先天性颅内静脉回流异常所致脑积水的临床特点和治疗方法.方法 对1例先天性颅内静脉回流异常所致脑积水患者,经CT、MRI、MRV及DSA检查明确诊断后,行脑室-腹腔体外可调压分流术治疗,对其病史结合文献进行回顾分析.结果 先天性颅内静脉回流异常导致的脑积水临床上非常罕见,患儿常有头颈部皮肤毛细血管扩张,对高颅压耐受较好,回流静脉受损可致颅内压调节失代偿,DSA检查能够明确颅内静脉回流的异常路径,体外可调压脑室-腹腔分流可随时根据患儿反应调节分流压力,分流压力一般需维持在较高水平,以保证静脉回流.结论 出生后有头颈部毛细血管扩张者需考虑先天性颅内静脉回流异常,DSA检查有助于明确诊断,体外可调压脑室-腹腔分流可安全有效地控制先天性颅内静脉回流异常所致脑积水引起的头颅进行性增大.

Hydrocephalus associated with congenital intracranial anomalous venous return: a case report and review of the literature

Objective To study the clinical features and management of the hydrocephalus associated with congenital intracranial anomalous venous return. Methods Combined with review of the literatures, the history and clinical data of a patient suffered from hydrocephalus associated with congenital intracranial anomalous venous return, whose diagnosis was confirmed by CT, MRI, MRV scan and DSA and programmable ventriculoperitoneal shunting was given, were analysed. Results The hydrocephalus associated with congenital intracranial anomalous venous return was rare. Commonly, the patient had a portwine-colored nevus of the head and neck at birth, a better tolerance to intracranial hypertention. Damage to the venous return may induce the decompensation of intracranial pressure regulation. DSA can make sure of the anomalous venous return. Programmable ventriculoperitoneal shunting can control the shunting pressure according to the patient's reaction at any time. A little higher shunting pressure was often necessary in order to keep the venous return. Conclusions Congenital intracranial anomalous venous return should be considered to those who had a port-wine-colored nevus of the head and neck at birth, DSA is helpful to confirm the diagnosis, programmable ventriculoperitoneal shunting can control the rapid increase in head circumference due to the hydrocephalus associated with congenital intracranial anomalous venous return safely and effectively.