Kuppler Patrick, Ditz Claudia, Küchler Jan, Fieseler Katharina, Kehler Uwe, Leppert Jan
Department of Neurosurgery, University Hospital of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
Department of Neuroradiology, University Hospital of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
J Neurosurg Case Lessons. 2025 Mar 3;9(9). doi: 10.3171/CASE24672.
Chronic negative-pressure hydrocephalus (cNegPH) is a rare condition, with only a few cases reported worldwide. Improvement of symptoms correlates with a reduction in ventricular size, which has been well documented in low-pressure hydrocephalus after prolonged subatmospheric cerebrospinal fluid (CSF) drainage and is considered the standard of care in such cases. However, the management of treatment failure and manifestation of cNegPH is still challenging, as well-documented treatment concepts are lacking.
Ventriculomegaly in a 36-year-old posthemorrhage, posttrauma patient with hydrocephalus remained refractory to low-gradient ventriculoperitoneal shunting, neck wrapping, endoscopic third ventriculostomy, and prolonged subatmospheric CSF drainage. Extended clinical care with multiple surgical and diagnostic interventions such as repeated replacements of the external ventricular drainage catheter, ventriculoperitoneal shunt revisions, and multiple cranial computed tomography scans was required in the course of the diagnosis. Complications including shunt malfunction, infection, meningitis, and recurrent clinical deteriorations were observed.
Diagnosing cNegPH can be challenging, and the failure of common treatment approaches has to be accepted after a certain point in time. At such a point, alternative options must be considered to provide outpatient care and reduce the risk of treatment complications. Intermittent shunt reservoir pressing proved to be an adequate long-term treatment option with a favorable outcome at the 6-month follow-up. https://thejns.org/doi/10.3171/CASE24672.
慢性负压性脑积水（cNegPH）是一种罕见病症，全球仅报道过少数病例。症状改善与脑室大小减小相关，这在长时间低于大气压的脑脊液（CSF）引流后的低压性脑积水中已有充分记录，并且在这类病例中被视为标准治疗方法。然而，由于缺乏充分记录的治疗理念，cNegPH治疗失败的管理及表现形式仍然具有挑战性。
一名36岁的出血后、创伤后脑积水患者的脑室扩大，对低梯度脑室腹腔分流术、颈部包裹、内镜下第三脑室造瘘术及长时间低于大气压的CSF引流均无反应。在诊断过程中，需要进行多次手术和诊断干预的广泛临床护理，如反复更换外部脑室引流导管、脑室腹腔分流术修复及多次头颅计算机断层扫描。观察到包括分流器故障、感染、脑膜炎及临床症状反复恶化等并发症。
诊断cNegPH可能具有挑战性，在一定时间点后必须接受常见治疗方法的失败。此时，必须考虑其他选择以提供门诊护理并降低治疗并发症的风险。间歇性按压分流器储液囊被证明是一种合适的长期治疗选择，在6个月的随访中取得了良好的效果。https://thejns.org/doi/10.3171/CASE24672。
