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Hydrocephalus Workup

  • Author: Stephen L Nelson, Jr, MD, PhD, FAAP; Chief Editor: Jasvinder Chawla, MD, MBA  more...
 
Updated: Apr 13, 2016
 

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.

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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.[13] Guidelines for imaging studies in suspected NPH have been established.[14]

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 >98 th 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.[15] 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.[2]

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.[3]

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Other Tests

See the list below:

  • After shunt insertion, confirm correct positioning of installed hardware with a plain radiograph.
  • EEG if seizure occurs
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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.[16]

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.

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Histologic Findings

Histologic findings include the following:

  • 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.
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Contributor Information and Disclosures
Author

Stephen L Nelson, Jr, MD, PhD, FAAP Section Head of Pediatric Neurology, Associate Professor of Pediatrics, Neurology, and Psychiatry, Tulane University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAAP is a member of the following medical societies: Academic Pediatric Association, American Academy of Neurology, American Academy of Pediatrics, American Medical Association, Association of Military Surgeons of the US, Child Neurology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jasvinder Chawla, MD, MBA Chief of Neurology, Hines Veterans Affairs Hospital; Professor of Neurology, Loyola University Medical Center

Jasvinder Chawla, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

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

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

Disclosure: Nothing to disclose.

Acknowledgements

Alberto J Espay, MD, MSc Associate Professor, Director of Clinical Research, Gardner Family Center for Parkinson's Disease and Movement Disorders, University of Cincinnati College of Medicine

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

Disclosure: Abbott Consulting fee Consulting; Chelsea therapeutics Consulting fee Consulting; Novartis Honoraria Speaking and teaching; TEVA Consulting fee Consulting; NIH Grant/research funds K23 Career Development Award; Eli Lilly Consulting fee Consulting; Great Lakes Neurotechnologies Other; Michael J Fox Foundation Grant/research funds Other; Lippincott Williams & Wilkins Royalty Book; American Academy of Neurology Honoraria Speaking and teaching

Eugenia-Daniela Hord, MD Instructor, Departments of Anesthesia and Neurology, Massachusetts General Hospital Pain Center, Harvard Medical School

Eugenia-Daniela Hord, MD is a member of the following medical societies: American Academy of Neurology and American Pain Society

Disclosure: Nothing to disclose.

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

  2. 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. 2007 Jan. 60(1):124-9; discussion 129-30. [Medline].

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

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

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

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

  7. 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. 2009 Apr 30. [Medline].

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

  9. Vinchon M, Rekate HL, Kulkarni AV. Pediatric hydrocephalus outcomes: a review. Fluids Barriers CNS. 2012 Aug 27. 9(1):18. [Medline].

  10. DeBenedictis CN, Rubin SE, Kodsi SR. Esotropia in Children with Ventricular-Peritoneal Shunts. Strabismus. 2015 Sep. 23 (3):117-20. [Medline].

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

  12. 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. 2009 May 8. [Medline].

  13. 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. 2008 Jan. 4(1):50-5. [Medline].

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

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

  16. 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. 2002 Apr. 72(4):503-6. [Medline].

  17. Lee JH, Back DB, Park DH, Cha YH, Kang SH, Suh JK. Increased Vascular Endothelial Growth Factor in the Ventricular Cerebrospinal Fluid as a Predictive Marker for Subsequent Ventriculoperitoneal Shunt Infection : A Comparison Study among Hydrocephalic Patients. J Korean Neurosurg Soc. 2012 Jun. 51(6):328-33. [Medline]. [Full Text].

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

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

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

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

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

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

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

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

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

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

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

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

  30. 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. 2004 May. 100(5):855-66. [Medline].

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

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

  33. 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. 1996 Dec. 23(12):2104-6. [Medline].

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

  35. Vogel TW, Bahuleyan B, Robinson S, Cohen AR. The role of endoscopic third ventriculostomy in the treatment of hydrocephalus. J Neurosurg Pediatr. 2013 Jul. 12(1):54-61. [Medline].

  36. 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].

 
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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).
 
 
 
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