Lundar T, Bakke S J, Nornes H
Department of Neurosurgery, National Hospital, University of Oslo, Norway.
J Neurosurg. 1990 Jul;73(1):138-40. doi: 10.3171/jns.1990.73.1.0138.
A 10-month-old child with achondroplasia with progressive head enlargement, ventriculomegaly, and wide subarachnoid spaces over the hemispheres was referred for evaluation. A steady-state lumbar infusion test revealed increased cerebrospinal fluid (CSF) outflow resistance (14 mm Hg/ml/min), and intra-arterial digital subtraction angiography (DSA) demonstrated bilateral venous outflow obstruction due to stenosis of the jugular foramen. Surgical decompression by opening the right jugular foramen relieved the clinical signs of intracranial hypertension. During the following year, the patient's head enlargement was moderate with relative normalization of size. Repeat DSA demonstrated improved venous runoff on the right side, and a steady-state lumbar infusion test demonstrated reduced CSF outflow resistance (10 mm Hg/ml/min). Venous decompression is causal therapy and may prove to be preferable to shunting in children with hydrocephalus and bilateral stenosis of the jugular foramen.
一名10个月大的软骨发育不全患儿，出现进行性头部增大、脑室扩大以及半球蛛网膜下腔增宽，前来接受评估。稳态腰段灌注试验显示脑脊液（CSF）流出阻力增加（14毫米汞柱/毫升/分钟），动脉内数字减影血管造影（DSA）显示由于颈静脉孔狭窄导致双侧静脉流出道梗阻。通过打开右侧颈静脉孔进行手术减压缓解了颅内高压的临床症状。在接下来的一年里，患者头部增大程度适中，大小相对正常。重复DSA显示右侧静脉回流改善，稳态腰段灌注试验显示脑脊液流出阻力降低（10毫米汞柱/毫升/分钟）。静脉减压是一种病因治疗方法，对于患有脑积水和双侧颈静脉孔狭窄的儿童，可能比分流术更可取。
