Hydrocephalus Workup

  • Author: Alberto J Espay, MD; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA   more...
 
Updated: Apr 27, 2010
 

Laboratory Studies

  • No specific blood tests are recommended in the workup for hydrocephalus.
  • Genetic testing and counseling might be recommended when X-linked hydrocephalus is suspected.
  • Evaluate cerebrospinal fluid (CSF) in posthemorrhagic and postmeningitic hydrocephalus for protein concentration and to exclude residual infection.
Next

Imaging Studies

  • CT can assess the size of ventricles and other structures.
  • MRI can evaluate for Chiari malformation or cerebellar or periaqueductal tumors. It affords better imaging of the posterior fossa than CT. MRI can differentiate normal pressure hydrocephalus (NPH) from cerebral atrophy although the distinctions may be challenging. Flow voids in the third ventricle and transependymal fluid exudates are helpful. However, numerous suitable patients have a brain pattern suggestive of atrophy and small vessel ischemic disease that may ultimately be NPH.[8] Guidelines for imaging studies in suspected NPH have been established.[9]
  • CT/MRI criteria for acute hydrocephalus include the following:
    • Size of both temporal horns is greater than 2 mm, clearly visible. In the absence of hydrocephalus, the temporal horns should be barely visible.
    • Ratio of the largest width of the frontal horns to maximal biparietal diameter (ie, Evans ratio) is greater than 30% in hydrocephalus.
    • Transependymal exudate is translated on images as periventricular hypoattenuation (CT) or hyperintensity (MRI T2-weighted and fluid-attenuated inversion recovery [FLAIR] sequences).
    • Ballooning of frontal horns of lateral ventricles and third ventricle (ie, "Mickey mouse" ventricles) may indicate aqueductal obstruction.
    • Upward bowing of the corpus callosum on sagittal MRI suggests acute hydrocephalus.
  • CT/MRI criteria for chronic hydrocephalus include the following:
    • Temporal horns may be less prominent than in acute hydrocephalus.
    • Third ventricle may herniate into the sella turcica.
    • Sella turcica may be eroded.
    • Macrocrania (ie, occipitofrontal circumference >98th percentile) may be present.
    • Corpus callosum may be atrophied (best appreciated on sagittal MRI). In this case, parenchymal atrophy and ex-vacuo (rather than true) hydrocephalus from a neurodegenerative disease should be considered.
  • Ultrasonography through the anterior fontanelle in infants is useful for evaluating subependymal and intraventricular hemorrhage and in following infants for possible development of progressive hydrocephalus.
  • Radionuclide cisternography can be done in NPH to evaluate the prognosis with regard to possible shunting. If a late scan (48-72 h) shows persistence of ventricular activity with a ventricular to total intracranial activity (V/T ratio) greater than 32%, the patient is more likely to benefit from shunting.[10] Because of its poor sensitivity in predicting shunt response when the V/T ration is less than 32%, this test is no longer commonly used.
  • Skull radiographs may depict erosion of sella turcica, or "beaten copper cranium" (called by some authors "beaten silver cranium"). The latter can also be seen in craniosynostosis.
  • MRI cine is an MRI technique to measure CSF stroke volume (SV) in the cerebral aqueduct. Cine phase-contrast MRI measurements of SV in the cerebral aqueduct does not appear to be useful in predicting response to shunting.[11]
  • Diffusion tensor imaging (DTI) is a novel imaging technique that detects differences in fractional anisotropy (FA) and mean diffusivity (MD) of the brain parenchyma surrounding the ventricles. Impairment of FA and MD through DTI allows the recognition of microstructural changes in periventricular white matter region that may be too subtle on conventional MRI.[32]
Previous
Next

Other Tests

  • After shunt insertion, confirm correct positioning of installed hardware with a plain radiograph.
  • EEG if seizure occurs
Previous
Next

Procedures

  • Lumbar puncture (LP) is a valuable test in evaluating NPH, but should be performed only after CT or MRI of the head. Normal LP opening pressure (OP) should be less than 180 mm H2 O (ie, 18 cm H2 O). Patients with initial OP greater than 100 mm H2 O have a higher rate of response to CSF shunting than those with OPs less than 100 mm H2 O. Improvement of symptoms after a single LP in which 40-50 mL of CSF is withdrawn appears to have some predictive value for success of CSF shunting.
  • Continuous CSF drainage through external lumbar drainage (ELD) is a highly accurate test for predicting the outcome after ventricular shunting in NPH, although false negative results are not uncommon.[12]
  • Continuous CSF pressure monitoring can help in predicting a patient's response to CSF shunting in NPH. Some patients with normal OP on LP demonstrate pressure peaks of greater than 270 mm H2 O or recurrent B waves. These patients tend to have higher rates of response to shunting than those who do not have these findings. This procedure also could differentiate NPH from atrophy.
Previous
Next

Histologic Findings

  • Thinning and stretching of the cortical mantle may be seen as a result of ventricular dilation.
  • In the acute phase, edema of the periventricular white matter is observed. Relatively few neuronal lesions are present. Ventricular ependyma shows cellular flattening and loss of cilia.
  • At a later stage, the edema disappears and is replaced by fibrosis, axonal degeneration, demyelination, focal loss of cerebral cortical neurons, cellular flattening, and further loss of cilia.
Previous
Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Alberto J Espay, MD  Assistant Professor, Department of Neurology, Gardner Family Center for Parkinson's Disease and Movement Disorders, Director of Clinical Research, University of Cincinnati

Alberto J Espay, MD is a member of the following medical societies: American Academy of Neurology and Movement Disorders Society

Disclosure: Boehringer-Ingelheim Consulting fee Board membership; Medtronic Grant/research funds Other; Novartis Honoraria Speaking and teaching; Solvay Consulting fee Board membership; NIH Grant/research funds KL2 Research Scholars mentored career development award

Specialty Editor Board

Anthony M Murro, MD  Professor, Laboratory Director, Department of Neurology, Medical College of Georgia

Anthony M Murro, MD is a member of the following medical societies: American Academy of Neurology and American Epilepsy Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Richard J Caselli, MD  Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale

Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA  Professor of Neurology, University of Central Florida College of Medicine; Director of Cognitive Neurology, Director of Stroke Program, James A Haley Veterans Affairs Hospital

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA is a member of the following medical societies: American Academy of Neurology, American Headache Society, American Heart Association, and American Society of Neuroimaging

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Eugenia-Daniela Hord, MD, to the original writing and development of this article.

References

  1. Rekate HL. A contemporary definition and classification of hydrocephalus. Semin Pediatr Neurol. Mar 2009;16(1):9-15. [Medline].

  2. Woodworth GF, McGirt MJ, Williams MA, Rigamonti D. Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery. May 2009;64(5):919-25; discussion 925-6. [Medline].

  3. Lacy M, Oliveira M, Austria E, Frim MD. Neurocognitive outcome after endoscopic third ventriculocisterostomy in patients with obstructive hydrocephalus. J Int Neuropsychol Soc. May 2009;15(3):394-8. [Medline].

  4. Garne E, Loane M, Addor MC, Boyd PA, Barisic I, Dolk H. Congenital hydrocephalus - prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol. Apr 30 2009;[Medline].

  5. Partington MD. Congenital hydrocephalus. Neurosurg Clin N Am. Oct 2001;12(4):737-42, ix. [Medline].

  6. Chauvet D, Sichez JP, Boch AL. [Early epidural hematoma after CSF shunt for obstructive hydrocephalus]. Neurochirurgie. Jun 2009;55(3):350-3. [Medline].

  7. Oertel JM, Mondorf Y, Baldauf J, Schroeder HW, Gaab MR. Endoscopic third ventriculostomy for obstructive hydrocephalus due to intracranial hemorrhage with intraventricular extension. J Neurosurg. May 8 2009;[Medline].

  8. Espay AJ, Narayan RK, Duker AP, Barrett ET Jr, de Courten-Myers G. Lower-body parkinsonism: reconsidering the threshold for external lumbar drainage. Nat Clin Pract Neurol. Jan 2008;4(1):50-5. [Medline].

  9. [Guideline] Dormont D, Seidenwurm DJ, Davis PC. Dementia and movement disorders. American College of Radiology (ACR). 2007;[Full Text].

  10. Larsson A, Moonen M, Bergh AC, Lindberg S, Wikkelso C. Predictive value of quantitative cisternography in normal pressure hydrocephalus. Acta Neurol Scand. Apr 1990;81(4):327-32. [Medline].

  11. Kahlon B, Annertz M, Stahlberg F, Rehncrona S. Is aqueductal stroke volume, measured with cine phase-contrast magnetic resonance imaging scans useful in predicting outcome of shunt surgery in suspected normal pressure hydrocephalus?. Neurosurgery. Jan 2007;60(1):124-9; discussion 129-30. [Medline].

  12. Walchenbach R, Geiger E, Thomeer RT, Vanneste JA. The value of temporary external lumbar CSF drainage in predicting the outcome of shunting on normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. Apr 2002;72(4):503-6. [Medline].

  13. Hamilton MG. Treatment of hydrocephalus in adults. Semin Pediatr Neurol. Mar 2009;16(1):34-41. [Medline].

  14. Black PML. Hydrocephalus in adults. In: Youmans JR, ed. Neurological Surgery. Philadelphia: WB Saunders Company; 1996:927-44.

  15. Chang CC, Kuwana N, Noji M, Tanabe Y, Koike Y, Ikegami T. Cerebral blood flow in patients with normal pressure hydrocephalus. Nucl Med Commun. Feb 1999;20(2):167-9. [Medline].

  16. Colak A, Albright AL, Pollack IF. Follow-up of children with shunted hydrocephalus. Pediatr Neurosurg. Oct 1997;27(4):208-10. [Medline].

  17. Czosnyka M, Pickard JD. Monitoring and interpretation of intracranial pressure. J Neurol Neurosurg Psychiatry. Jun 2004;75(6):813-21. [Medline].

  18. Damasceno BP, Carelli EF, Honorato DC, Facure JJ. The predictive value of cerebrospinal fluid tap-test in normal pressure hydrocephalus. Arq Neuropsiquiatr. Jun 1997;55(2):179-85. [Medline].

  19. du Plessis AJ. Posthemorrhagic hydrocephalus and brain injury in the preterm infant: dilemmas in diagnosis and management. Semin Pediatr Neurol. Sep 1998;5(3):161-79. [Medline].

  20. Frim DM, Scott RM, Madsen JR. Surgical management of neonatal hydrocephalus. Neurosurg Clin N Am. Jan 1998;9(1):105-10. [Medline].

  21. Garvey MA, Laureno R. Hydrocephalus: obliterated perimesencephalic cisterns and the danger of sudden death. Can J Neurol Sci. May 1998;25(2):154-8. [Medline].

  22. Goumnerova LC, Frim DM. Treatment of hydrocephalus with third ventriculocisternostomy: outcome and CSF flow patterns. Pediatr Neurosurg. Sep 1997;27(3):149-52. [Medline].

  23. Hoppe-Hirsch E, Laroussinie F, Brunet L, et al. Late outcome of the surgical treatment of hydrocephalus. Childs Nerv Syst. Mar 1998;14(3):97-9. [Medline].

  24. Libenson MH, Kaye EM, Rosman NP, Gilmore HE. Acetazolamide and furosemide for posthemorrhagic hydrocephalus of the newborn. Pediatr Neurol. Mar 1999;20(3):185-91. [Medline].

  25. Mercuri E, Faundez JC, Cowan F, Dubowitz L. Acetazolamide without frusemide in the treatment of post-haemorrhagic hydrocephalus. Acta Paediatr. Dec 1994;83(12):1319-21. [Medline].

  26. Poca MA, Mataro M, Del Mar Matarin M, Arikan F, Junque C, Sahuquillo J. Is the placement of shunts in patients with idiopathic normal-pressure hydrocephalus worth the risk? Results of a study based on continuous monitoring of intracranial pressure. J Neurosurg. May 2004;100(5):855-66. [Medline].

  27. Sainte-Rose C. Hydrocephalus in childhood. In: Youmans JR, ed. Neurological Surgery. Philadelphia: WB Saunders Company; 1996:890-926.

  28. Sansone JM, Iskandar BJ. Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. Nov 2005;103(5 Suppl):388-92. [Medline].

  29. Shbeeb MI, O'Duffy JD, Michet CJ Jr, O'Fallon WM, Matteson EL. Evaluation of glucocorticosteroid injection for the treatment of trochanteric bursitis. J Rheumatol. Dec 1996;23(12):2104-6. [Medline].

  30. Tanaka A, Kimura M, Nakayama Y, Yoshinaga S, Tomonaga M. Cerebral blood flow and autoregulation in normal pressure hydrocephalus. Neurosurgery. Jun 1997;40(6):1161-5; discussion 1165-7. [Medline].

  31. Williams MA, Razumovsky AY, Hanley DF. Comparison of Pcsf monitoring and controlled CSF drainage diagnose normal pressure hydrocephalus. Acta Neurochir Suppl. 1998;71:328-30. [Medline].

  32. Hattingen E, Jurcoane A, Melber J, Blasel S, Zanella FE, Neumann-Haefelin T. Diffusion tensor imaging in patients with adult chronic idiopathic hydrocephalus. Neurosurgery. May 2010;66(5):917-24. [Medline].

 
Previous
Next
 
Noncommunicating obstructive hydrocephalus caused by obstruction of the foramina of Luschka and Magendie. This MRI sagittal image demonstrates dilatation of lateral ventricles with stretching of corpus callosum and dilatation of the fourth ventricle.
Noncommunicating obstructive hydrocephalus caused by obstruction of foramina of Luschka and Magendie. This MRI axial image demonstrates dilatation of the lateral ventricles.
Noncommunicating obstructive hydrocephalus caused by obstruction of foramina of Luschka and Magendie. This MRI axial image demonstrates fourth ventricle dilatation.
Communicating hydrocephalus with surrounding "atrophy" and increased periventricular and deep white matter signal on fluid-attenuated inversion recovery (FLAIR) sequences. Note that apical cuts (lower row) do not show enlargement of the sulci, as is expected in generalized atrophy. Pathological evaluation of this brain demonstrated hydrocephalus with no microvascular pathology corresponding with the signal abnormality (which likely reflects transependymal exudate) and normal brain weight (indicating that the sulci enlargement was due to increased subarachnoid cerebrospinal fluid [CSF] conveying a pseudoatrophic brain pattern).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.