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J Neurol Surg A Cent Eur Neurosurg 2024; 85(05): 492-500
DOI: 10.1055/s-0044-1785671
Review Article

Low-/Negative-Pressure Hydrocephalus: To Understand the Formation Mechanism from the Perspective of Clinicians

Guangzhao Li
1   Department of Neurosurgery, The First People's Hospital of Hefei, Hefei, Anhui, Peoples' Republic of China
,
Bin Lin
1   Department of Neurosurgery, The First People's Hospital of Hefei, Hefei, Anhui, Peoples' Republic of China
,
Fei Yang
1   Department of Neurosurgery, The First People's Hospital of Hefei, Hefei, Anhui, Peoples' Republic of China
› Author Affiliations Funding None.
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Abstract

Low-/negative-pressure hydrocephalus (LPH/NePH) is uncommon in clinical practice, and doctors are unfamiliar with it. LPH/NePH is frequently caused by other central nervous system diseases, and patients are frequently misdiagnosed with other types of hydrocephalus, resulting in delayed treatment. LPH/NePH therapy evolved to therapeutic measures based on “external ventricular drainage below atmospheric pressure” as the number of patients with LPH/NePH described in the literature has increased. However, the mechanism of LPH/NePH formation is unknown. Thus, understanding the process of LPH/NePH development is the most important step in improving diagnosis and treatment capability. Based on case reports of LPH/NePH, we reviewed theories of transcortical pressure difference, excessive cerebral venous drainage, brain viscoelastic changes, and porous elastic sponges.

Keywords

low-pressure hydrocephalus - negative-pressure hydrocephalus - cerebral viscoelasticity change theory - excessive drainage of cerebral veins theory - porous elastic sponge theory


Publication History

Received: 30 August 2023

Accepted: 17 January 2024

Article published online:
30 April 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
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  • References

  • 1 Badran A, Shepard MJ, Ksendzovsky A. et al. Hemangioblastomatosis-associated negative-pressure hydrocephalus managed with improvised shunt. J Clin Neurosci 2018; 58: 226-228
    CrossrefPubMedSearch in Google Scholar
  • 2 Michael AP, Elkouzi A, Elble RJ. Pearls & oy-sters: low-pressure hydrocephalus and inadequate shunting. Neurology 2017; 88 (17) e174-e177
    CrossrefPubMedSearch in Google Scholar
  • 3 Funnell JP, D'Antona L, Craven CL, Thorne L, Watkins LD, Toma AK. Ultra-low-pressure hydrocephalic state in NPH: benefits of therapeutic siphoning with adjustable antigravity valves. Acta Neurochir (Wien) 2020; 162 (12) 2967-2974
    CrossrefPubMedSearch in Google Scholar
  • 4 Rekate HL. Low or negative pressure hydrocephalus demystified. World Neurosurg 2019; 128: 287-288
    CrossrefPubMedSearch in Google Scholar
  • 5 Keough MB, Isaacs AM, Urbaneja G, Dronyk J, Lapointe AP, Hamilton MG. Acute low-pressure hydrocephalus: a case series and systematic review of 195 patients. J Neurosurg 2020; 135 (01) 300-308
    CrossrefPubMedSearch in Google Scholar
  • 6 Cheng Z, Wang W, Han Y. et al. Low pressure hydrocephalus: clinical manifestations, radiological characteristics, and treatment. Br J Neurosurg 2017; 31 (04) 410-414
    CrossrefPubMedSearch in Google Scholar
  • 7 Wu X, Zang D, Wu X, Sun Y, Yu J, Hu J. Diagnosis and management for secondary low- or negative-pressure hydrocephalus and a new hydrocephalus classification based on ventricular pressure. World Neurosurg 2019; 124: e510-e516
    CrossrefPubMedSearch in Google Scholar
  • 8 Hunn BH, Mujic A, Sher I, Dubey AK, Peters-Willke J, Hunn AW. Successful treatment of negative pressure hydrocephalus using timely titrated external ventricular drainage: a case series. Clin Neurol Neurosurg 2014; 116: 67-71
    CrossrefPubMedSearch in Google Scholar
  • 9 Strand A, Balise S, Leung LJ, Durham S. Low-pressure hydrocephalus: a case report and review of the literature. World Neurosurg 2018; 109: e131-e135
    CrossrefPubMedSearch in Google Scholar
  • 10 Filippidis AS, Kalani MY, Nakaji P, Rekate HL. Negative-pressure and low-pressure hydrocephalus: the role of cerebrospinal fluid leaks resulting from surgical approaches to the cranial base. J Neurosurg 2011; 115 (05) 1031-1037
    CrossrefPubMedSearch in Google Scholar
  • 11 Casado Pellejero J, Moles Herbera J, Vázquez Sufuentes S, Orduna Martínez J, Rivero Celada D, Fustero de Miguel D. Acute negative-pressure hydrocephalus: Management algorithm and value of early endoscopic ventriculostomy. Neurocirugia (Astur Engl Ed) 2022; 33 (01) 1-8
    PubMedSearch in Google Scholar
  • 12 Smalley ZS, Venable GT, Einhaus S, Klimo Jr P. Low-pressure hydrocephalus in children: a case series and review of the literature. Neurosurgery 2017; 80 (03) 439-447
    CrossrefPubMedSearch in Google Scholar
  • 13 Vilela MD, Pedrosa HA, Mesquita BF, Horiguchi CLF. Low-pressure hydrocephalus and shunt malfunction following a lumbar puncture in an adult reversed by an epidural blood patch. World Neurosurg 2020; 143: 56-61
    CrossrefPubMedSearch in Google Scholar
  • 14 Pandey S, Jin Y, Gao L, Zhou CC, Cui DM. Negative-pressure hydrocephalus: a case report on successful treatment under intracranial pressure monitoring with bilateral ventriculoperitoneal shunts. World Neurosurg 2017; 99: 812.e7-812.e12
    CrossrefPubMedSearch in Google Scholar
  • 15 Vassilyadi M, Farmer JP, Montes JL. Negative-pressure hydrocephalus. J Neurosurg 1995; 83 (03) 486-490
    CrossrefPubMedSearch in Google Scholar
  • 16 Rekate HL, Nadkarni TD, Wallace D. The importance of the cortical subarachnoid space in understanding hydrocephalus. J Neurosurg Pediatr 2008; 2 (01) 1-11
    CrossrefPubMedSearch in Google Scholar
  • 17 Diaz-Romero Paz R, Avendaño Altimira P, Coloma Valverde G, Balhen Martin C. A rare case of negative-pressure hydrocephalus: a plausible explanation and the role of transmantle theory. World Neurosurg 2019; 125: 6-9
    CrossrefPubMedSearch in Google Scholar
  • 18 Suzuki T, Kaku S, Karagiozov K, Murayama Y. Negative-pressure hydrocephalus in the course of a complex postoperative intracranial pressure disturbance: illustrative case. J Neurosurg Case Lessons 2021; 1 (01) CASE206
    CrossrefPubMedSearch in Google Scholar
  • 19 Das S, Montemurro N, Ashfaq M. et al. Resolution of papilledema following ventriculoperitoneal shunt or endoscopic third ventriculostomy for obstructive hydrocephalus: a pilot study. Medicina (Kaunas) 2022; 58 (02) 281
    CrossrefPubMedSearch in Google Scholar
  • 20 Uche EO, Okorie C, Iloabachie I, Amuta DS, Uche NJ. Endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) in non-communicating hydrocephalus (NCH): comparison of outcome profiles in Nigerian children. Childs Nerv Syst 2018; 34 (09) 1683-1689
    CrossrefPubMedSearch in Google Scholar
  • 21 Montemurro N, Indaimo A, Di Carlo DT, Benedetto N, Perrini P. Surgical treatment of long-standing overt ventriculomegaly in adults (LOVA): a comparative case series between ventriculoperitoneal shunt (VPS) and endoscopic third ventriculostomy (ETV). Int J Environ Res Public Health 2022; 19 (04) 1926
    CrossrefPubMedSearch in Google Scholar
  • 22 Ozeki T, Kubota A, Murai Y, Morita A. Suspected low-pressure hydrocephalus caused by spinal drainage after subarachnoid hemorrhage. J Nippon Med Sch 2022; 89 (02) 238-243
    CrossrefPubMedSearch in Google Scholar
  • 23 Hamilton MG, Price AV. Syndrome of inappropriately low-pressure acute hydrocephalus (SILPAH). Acta Neurochir Suppl (Wien) 2012; 113: 155-159
    CrossrefPubMedSearch in Google Scholar
  • 24 Barami K, Sood S, Ham SD, Canady AI. Postural changes in intracranial pressure in chronically shunted patients. Pediatr Neurosurg 2000; 33 (02) 64-69
    CrossrefPubMedSearch in Google Scholar
  • 25 Portnoy HD, Branch C, Castro ME. The relationship of intracranial venous pressure to hydrocephalus. Childs Nerv Syst 1994; 10 (01) 29-35
    CrossrefPubMedSearch in Google Scholar
  • 26 Gisolf J, van Lieshout JJ, van Heusden K, Pott F, Stok WJ, Karemaker JM. Human cerebral venous outflow pathway depends on posture and central venous pressure. J Physiol 2004; 560 (Pt 1): 317-327
    CrossrefPubMedSearch in Google Scholar
  • 27 Alperin N, Hushek SG, Lee SH, Sivaramakrishnan A, Lichtor T. MRI study of cerebral blood flow and CSF flow dynamics in an upright posture: the effect of posture on the intracranial compliance and pressure. Acta Neurochir Suppl (Wien) 2005; 95: 177-181
    CrossrefPubMedSearch in Google Scholar
  • 28 Barami K. Cerebral venous overdrainage: an under-recognized complication of cerebrospinal fluid diversion. Neurosurg Focus 2016; 41 (03) E9
    CrossrefPubMedSearch in Google Scholar
  • 29 Valdueza JM, von Münster T, Hoffman O, Schreiber S, Einhäupl KM. Postural dependency of the cerebral venous outflow. Lancet 2000; 355 (9199): 200-201
    CrossrefPubMedSearch in Google Scholar
  • 30 Lesniak MS, Clatterbuck RE, Rigamonti D, Williams MA. Low pressure hydrocephalus and ventriculomegaly: hysteresis, non-linear dynamics, and the benefits of CSF diversion. Br J Neurosurg 2002; 16 (06) 555-561
    CrossrefPubMedSearch in Google Scholar
  • 31 Franzini A, Messina G, Nazzi V. et al. Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and a novel patch method in a series of 28 consecutive patients. J Neurosurg 2010; 112 (02) 300-306
    CrossrefPubMedSearch in Google Scholar
  • 32 Kim A, Lim D, Piatt J. Low-pressure CSF shunt malfunction without preceding lumbar puncture treated by epidural blood patch: report of 2 cases. Childs Nerv Syst 2021; 37 (03) 973-976
    CrossrefPubMedSearch in Google Scholar
  • 33 Pang D, Altschuler E. Low-pressure hydrocephalic state and viscoelastic alterations in the brain. Neurosurgery 1994; 35 (04) 643-655 , discussion 655–656
    CrossrefPubMedSearch in Google Scholar
  • 34 Nanjappa M, Kolipaka A. Magnetic resonance elastography of the brain. Magn Reson Imaging Clin N Am 2021; 29 (04) 617-630
    CrossrefPubMedSearch in Google Scholar
  • 35 Olivero WC, Biswas A, Wszalek TM, Sutton BP, Johnson CL. Brain stiffness following recovery in a patient with an episode of low-pressure hydrocephalus: case report. Childs Nerv Syst 2021; 37 (08) 2695-2698
    CrossrefPubMedSearch in Google Scholar
  • 36 Olivero WC, Wszalek T, Wang H, Farahvar A, Rieth SM, Johnson CL. Magnetic resonance elastography demonstrating low brain stiffness in a patient with low-pressure hydrocephalus: case report. Pediatr Neurosurg 2016; 51 (05) 257-262
    CrossrefPubMedSearch in Google Scholar
  • 37 Johnson CL, McGarry MD, Gharibans AA. et al. Local mechanical properties of white matter structures in the human brain. Neuroimage 2013; 79: 145-152
    CrossrefPubMedSearch in Google Scholar
  • 38 Johnson CL, Holtrop JL, McGarry MD. et al. 3D multislab, multishot acquisition for fast, whole-brain MR elastography with high signal-to-noise efficiency. Magn Reson Med 2014; 71 (02) 477-485
    CrossrefPubMedSearch in Google Scholar
  • 39 Akins PT, Guppy KH, Axelrod YV, Chakrabarti I, Silverthorn J, Williams AR. The genesis of low pressure hydrocephalus. Neurocrit Care 2011; 15 (03) 461-468
    CrossrefPubMedSearch in Google Scholar
  • 40 McGovern RA, Sheehy JP, McKhann II GM. Low pressure hydrocephalus acutely following sepsis and cardiovascular collapse. Clin Neurol Neurosurg 2013; 115 (10) 2186-2188
    CrossrefPubMedSearch in Google Scholar
  • 41 Weisbrod L, Davidson C, Gard A, Surdell D. Low-pressure hydrocephalus in spontaneous angiogram-negative subarachnoid hemorrhage following COVID-19 infection. Cureus 2021; 13 (07) e16674
    PubMedSearch in Google Scholar
  • 42 Preuss M, Hoffmann KT, Reiss-Zimmermann M. et al. Updated physiology and pathophysiology of CSF circulation: the pulsatile vector theory. Childs Nerv Syst 2013; 29 (10) 1811-1825
    CrossrefPubMedSearch in Google Scholar
  • 43 Preuß M, Evangelou P, Hirsch W. et al. Acute two-compartment low pressure hydrocephalus: a case report. Childs Nerv Syst 2013; 29 (12) 2307-2310
    CrossrefPubMedSearch in Google Scholar
  • 44 Czorlich P, Schweingruber N, Göttsche J, Mader MM, Westphal M. Acute low-pressure hydrocephalus in aneurysmal subarachnoid hemorrhage. Neurosurg Focus 2023; 54 (04) E5
    CrossrefPubMedSearch in Google Scholar
  • 45 Da Mesquita S, Fu Z, Kipnis J. The meningeal lymphatic system: a new player in neurophysiology. Neuron 2018; 100 (02) 375-388
    CrossrefPubMedSearch in Google Scholar
  • 46 Fang Y, Huang L, Wang X. et al. A new perspective on cerebrospinal fluid dynamics after subarachnoid hemorrhage: from normal physiology to pathophysiological changes. J Cereb Blood Flow Metab 2022; 42 (04) 543-558
    CrossrefPubMedSearch in Google Scholar
  • 47 Wang X, Zhang A, Yu Q. et al. Single-cell RNA sequencing and spatial transcriptomics reveal pathogenesis of meningeal lymphatic dysfunction after experimental subarachnoid hemorrhage. Adv Sci (Weinh) 2023; 10 (21) e2301428
    CrossrefPubMedSearch in Google Scholar
  • 48 Chen Y, Galea I, Macdonald RL, Wong GKC, Zhang JH. Rethinking the initial changes in subarachnoid haemorrhage: focusing on real-time metabolism during early brain injury. EBioMedicine 2022; 83: 104223
    CrossrefPubMedSearch in Google Scholar