Acute low-pressure hydrocephalus: a case series and systematic review of 195 patients

View More View Less
  • 1 Department of Surgery, University of Alberta, Edmonton;
  • 2 Department of Clinical Neurosciences, University of Calgary; and
  • 3 Department of Radiology, University of Calgary, Alberta, Canada
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00
Print or Print + Online

OBJECTIVE

Acute low-pressure hydrocephalus (ALPH) is characterized by clinical manifestations of an apparent raised intracranial pressure (ICP) and ventriculomegaly despite measured ICP that is below the expected range (i.e., typically ≤ 5 cm H2O). ALPH is often refractory to standard hydrocephalus intervention protocols and the ICP paradox commonly leads to delayed diagnosis. The aim of this study was to characterize ALPH and develop an algorithm to facilitate diagnosis and management for patients with ALPH.

METHODS

EMBASE, MEDLINE, and Google Scholar databases were searched for ALPH cases from its first description in 1994 until 2019. Cases that met inclusion criteria were pooled with cases managed at the authors’ institution. Patient characteristics, presenting signs/symptoms, precipitating factors, temporizing interventions, definitive treatment, and patient outcomes were recorded.

RESULTS

There were 195 patients identified, with 42 local and 153 from the literature review (53 pediatric patients and 142 adults). Decreased level of consciousness was the predominant clinical sign. The most common etiologies of hydrocephalus were neoplasm and hemorrhage. While the majority of ALPH occurred spontaneously, 39% of pediatric patients had previously undergone a lumbar puncture. Prior to ALPH diagnosis, 92% of pediatric and 39% of adult patients had a ventricular shunt in situ. The most common temporizing intervention was subatmospheric CSF drainage. The majority of patients underwent a shunt insertion/revision or endoscopic third ventriculostomy as definitive ALPH treatment. Although the mortality rate was 11%, 83% of pediatric and 49% of adult patients returned to their pre-ALPH neurological functional status after definitive treatment. Outcomes were related to both the severity of the underlying neurosurgical disease causing the hydrocephalus and the efficacy of ALPH treatment.

CONCLUSIONS

ALPH is an underrecognized variant phenotype of hydrocephalus that is associated with multiple etiologies and can be challenging to treat as it frequently does not initially respond to standard strategies of CSF shunting. With early recognition, ALPH can be effectively managed. A management algorithm is provided as a guide for this purpose.

ABBREVIATIONS ALPH = acute low-pressure hydrocephalus; ETV = endoscopic third ventriculostomy; EVD = external ventricular drain; ICP = intracranial pressure; iNPH = idiopathic normal pressure hydrocephalus; SAS = subarachnoid space.

Supplementary Materials

    • pdf Supplementary Tables 1 and 2 (PDF 506 KB)

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

Contributor Notes

Correspondence Mark G. Hamilton: University of Calgary, AB, Canada. mghamilton.hydro@gmail.com.

INCLUDE WHEN CITING Published online July 31, 2020; DOI: 10.3171/2020.4.JNS20476.

M.B.K. and A.M.I. contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • 1

    Rekate HL. A contemporary definition and classification of hydrocephalus. Semin Pediatr Neurol. 2009;16(1):915.

  • 2

    Kahle KT, Kulkarni AV, Limbrick DD Jr, Warf BC. Hydrocephalus in children. Lancet. 2016;387(10020):788799.

  • 3

    Leinonen V, Vanninen R, Rauramaa T. Cerebrospinal fluid circulation and hydrocephalus. Handb Clin Neurol. 2017;145:3950.

  • 4

    Cowan JA, McGirt MJ, Woodworth G, The syndrome of hydrocephalus in young and middle-aged adults (SHYMA). Neurol Res. 2005;27(5):540547.

    • Search Google Scholar
    • Export Citation
  • 5

    Ono K, Hatada J, Yamada M. Long-standing overt ventriculomegaly in adults (LOVA) needing ventriculo-peritoneal shunt with double programmable pressure valves. Article in Japanese. No Shinkei Geka. 2012;40(1):3742.

    • Search Google Scholar
    • Export Citation
  • 6

    Mori E, Ishikawa M, Kato T, Guidelines for management of idiopathic normal pressure hydrocephalus: second edition. Neurol Med Chir (Tokyo). 2012;52(11):775809.

    • Search Google Scholar
    • Export Citation
  • 7

    Relkin N, Marmarou A, Klinge P, Diagnosing idiopathic normal-pressure hydrocephalus. Neurosurgery. 2005;57(3)(suppl):S4S16 , ii–v.

  • 8

    Bräutigam K, Vakis A, Tsitsipanis C. Pathogenesis of idiopathic normal pressure hydrocephalus: a review of knowledge. J Clin Neurosci. 2019;61:1013.

    • Search Google Scholar
    • Export Citation
  • 9

    Hamilton MG, Price AV. Syndrome of inappropriately low-pressure acute hydrocephalus (SILPAH). Acta Neurochir Suppl (Wien). 2012;113:155159.

    • Search Google Scholar
    • Export Citation
  • 10

    Pang D, Altschuler E. Low-pressure hydrocephalic state and viscoelastic alterations in the brain. Neurosurgery. 1994;35(4):643656.

  • 11

    Vassilyadi M, Farmer JP, Montes JL. Negative-pressure hydrocephalus. J Neurosurg. 1995;83(3):486490.

  • 12

    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(5):10311037.

    • Search Google Scholar
    • Export Citation
  • 13

    Moher D, Liberati A, Tetzlaff J, Altman DG. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Int J Surg. 2010;8(5):336341.

    • Search Google Scholar
    • Export Citation
  • 14

    O’Hayon BB, Drake JM, Ossip MG, Frontal and occipital horn ratio: a linear estimate of ventricular size for multiple imaging modalities in pediatric hydrocephalus. Pediatr Neurosurg. 1998;29(5):245249.

    • Search Google Scholar
    • Export Citation
  • 15

    Owler BK, Jacobson EE, Johnston IH. Low pressure hydrocephalus: issues of diagnosis and treatment in five cases. Br J Neurosurg. 2001;15(4):353359.

    • Search Google Scholar
    • Export Citation
  • 16

    Weed LH. Some limitations of the Monro-Kellie hypothesis. Arch Surg. 1929;18(4):10491068.

  • 17

    Olivero WC, Wszalek T, Wang H, Magnetic resonance elastography demonstrating low brain stiffness in a patient with low-pressure hydrocephalus: case report. Pediatr Neurosurg. 2016;51(5):257262.

    • Search Google Scholar
    • Export Citation
  • 18

    Rekate HL, Nadkarni TD, Wallace D. The importance of the cortical subarachnoid space in understanding hydrocephalus. J Neurosurg Pediatr. 2008;2(1):111.

    • Search Google Scholar
    • Export Citation
  • 19

    Dias MS, Li V, Pollina J. Low-pressure shunt ‘malfunction’ following lumbar puncture in children with shunted obstructive hydrocephalus. Pediatr Neurosurg. 1999;30(3):146150.

    • Search Google Scholar
    • Export Citation
  • 20

    Lin JP, Zhang SD, He FF, The status of diagnosis and treatment to intracranial hypotension, including SIH. J Headache Pain. 2017;18(1):4.

    • Search Google Scholar
    • Export Citation
  • 21

    Kalani MY, Turner JD, Nakaji P. Treatment of refractory low-pressure hydrocephalus with an active pumping negative-pressure shunt system. J Clin Neurosci. 2013;20(3):462466.

    • Search Google Scholar
    • Export Citation
  • 22

    Pandey S, Jin Y, Gao L, Negative-pressure hydrocephalus: a case report on successful treatment under intracranial pressure monitoring with bilateral ventriculoperitoneal shunts. World Neurosurg. 2017;99:812.e7812.e12.

    • Search Google Scholar
    • Export Citation
  • 23

    Hatt A, Cheng S, Tan K, MR elastography can be used to measure brain stiffness changes as a result of altered cranial venous drainage during jugular compression. AJNR Am J Neuroradiol. 2015;36(10):19711977.

    • Search Google Scholar
    • Export Citation
  • 24

    Wu X, Zang D, Wu X, Diagnosis and management for secondary low- or negative-pressure hydrocephalus and a new hydrocephalus classification based on ventricular pressure. World Neurosurg. 2019;124:e510e516.

    • Search Google Scholar
    • Export Citation
  • 25

    Chiang VL, Torbey M, Rigamonti D, Williams MA. Ventriculopleural shunt obstruction and positive-pressure ventilation. Case report. J Neurosurg. 2001;95(1):116118.

    • Search Google Scholar
    • Export Citation
  • 26

    Smalley ZS, Venable GT, Einhaus S, Klimo P Jr. Low-pressure hydrocephalus in children: a case series and review of the literature. Neurosurgery. 2017;80(3):439447.

    • Search Google Scholar
    • Export Citation
  • 27

    McGovern RA, Sheehy JP, McKhann GM II. Low pressure hydrocephalus acutely following sepsis and cardiovascular collapse. Clin Neurol Neurosurg. 2013;115(10):21862188.

    • Search Google Scholar
    • Export Citation
  • 28

    Foster KA, Deibert CP, Choi PA, Endoscopic third ventriculostomy as adjunctive therapy in the treatment of low-pressure hydrocephalus in adults. Surg Neurol Int. 2016;7:26.

    • Search Google Scholar
    • Export Citation
  • 29

    Strand A, Balise S, Leung LJ, Durham S. Low-pressure hydrocephalus: a case report and review of the literature. World Neurosurg. 2018;109:e131e135.

    • Search Google Scholar
    • Export Citation
  • 30

    Cheng Z, Wang W, Han Y, Low pressure hydrocephalus: clinical manifestations, radiological characteristics, and treatment. Br J Neurosurg. 2017;31(4):410414.

    • Search Google Scholar
    • Export Citation
  • 31

    Wilson JT, Hareendran A, Grant M, Improving the assessment of outcomes in stroke: use of a structured interview to assign grades on the modified Rankin Scale. Stroke. 2002;33(9):22432246.

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 2021 2021 269
Full Text Views 261 261 32
PDF Downloads 195 195 20
EPUB Downloads 0 0 0