Salam Smeer, Quintero-Consuegra Miguel, Ryan Megan V, Vargas Enrique, Garcia Catherine, Wong Serena, Rejali Maryam, Babadjouni Robin, Mamelak Adam N
Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, California.
Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California.
J Neurosurg Case Lessons. 2025 May 26;9(21). doi: 10.3171/CASE2554.
Negative-pressure hydrocephalus is characterized by neurological deterioration with ventriculomegaly but negative intracranial pressure (ICP), clinically indistinguishable from acute hydrocephalus with elevated ICP. Subatmospheric CSF drainage, neck wrapping, endoscopic third ventriculostomy (ETV), and shunt placement are treatment methods. Medical therapy remains elusive. The authors describe a patient with refractory negative-pressure hydrocephalus who failed conventional therapies but responded robustly to theophylline.
A 30-year-old man with traumatic brain injury underwent decompressive hemicraniectomy, cranioplasty, and ventriculoperitoneal shunt placement. He was later rehospitalized for acute hydrocephalus and shunt infection. Surgical revision was complicated by intraventricular hemorrhage with worsening hydrocephalus. An external ventricular drain was inserted, demonstrating negative ICP. Treatment for more than 3 months with subzero drainage, two ETVs, and ventriculopleural shunt placement was unsuccessful. Theophylline was initiated and the ventriculomegaly improved drastically on imaging with increased ICP. He developed status epilepticus, managed medically, with resolution within 5 days. He was discharged with return to his neurological baseline.
Theophylline, a methylxanthine derivate used for obstructive pulmonary disease, can raise ICP in intracranial hypotension. The authors present the first successful case of theophylline for the treatment of refractory acute negative-pressure hydrocephalus. Further studies are warranted to explore the safety and efficacy of theophylline for negative-pressure hydrocephalus. https://thejns.org/doi/10.3171/CASE2554.
负压性脑积水的特征是伴有脑室扩大的神经功能恶化，但颅内压（ICP）为负值，临床上与ICP升高的急性脑积水难以区分。低于大气压的脑脊液引流、颈部包扎、内镜下第三脑室造瘘术（ETV）和分流管置入是治疗方法。药物治疗仍然难以捉摸。作者描述了一名难治性负压性脑积水患者，该患者对传统治疗无效，但对茶碱有显著反应。
一名30岁的创伤性脑损伤男性接受了减压性颅骨切除术、颅骨成形术和脑室腹腔分流管置入术。他后来因急性脑积水和分流管感染再次住院。手术翻修因脑室内出血并伴有脑积水加重而复杂化。插入了一根外部脑室引流管，显示ICP为负值。采用零下引流、两次ETV和脑室胸膜分流管置入治疗3个多月均未成功。开始使用茶碱治疗后，影像学显示脑室扩大明显改善，ICP升高。他发生了癫痫持续状态，经药物治疗，5天内得到缓解。他出院时神经功能恢复至基线水平。
茶碱是一种用于阻塞性肺病的甲基黄嘌呤衍生物，可在颅内低压时升高ICP。作者介绍了首例使用茶碱成功治疗难治性急性负压性脑积水的病例。有必要进一步研究茶碱治疗负压性脑积水的安全性和有效性。https://thejns.org/doi/10.3171/CASE2554 。
