eMedicine Specialties > Pediatrics: Surgery > General Surgery

Management of Spina Bifida, Hydrocephalus and Shunts: Workup

Author: Lynne C Kramer, MD, Fellow in Developmental Pediatrics, Department of Pediatrics, Madigan Army Medical Center
Coauthor(s): Kenneth Azarow, MD, Program Director, Pediatric Surgery, Children's Hospital and University of Nebraska Medical Center; Professor, Department of Surgery, Uniformed Services University of the Health Sciences; Brett A Schlifka, DO, Consulting Staff, Department of Neurosurgery, Madigan Army Medical Center; Spyros Sgouros, MD, FRCS(SN)(Glasg), Senior Lecturer, Department of Neurosurgery, Division of Neuroscience, Section of Pediatrics, University of Birmingham, England
Contributor Information and Disclosures

Updated: Nov 19, 2009

Workup

Imaging Studies

  • Ultrasonography
    • Currently, most fetuses undergo in utero ultrasonographic scanning.
    • Ultrasonography allows for good identification of any ventricular dilatation that indicates active hydrocephalus. In such cases, or in patients who present with progressive head enlargement to pediatricians or family doctors, ultrasonography is usually performed first because it is widely available and does not expose the child to ionizing radiation.
    • In babies with open fontanelles and large heads, ultrasonography reveals the enlarged ventricular system and any mass lesions or hemorrhage. However, the anatomic detail produced by ultrasonography remains poor and serves only as a guide to further investigations.
  • CT scanning 
    • Once hydrocephalus is suspected, either clinically or based on ultrasonography findings, the diagnosis must be confirmed with a more detailed investigation.
    • In most parts of the world, CT scanning remains the most widely available neuroimaging investigation. Because image acquisition is rapid, the study does not require sedation of the child and, in most cases, is helpful for obtaining an accurate diagnosis.
    • CT scanning provides a good image of the dilated ventricular system and any obstructive lesions, such as brain tumors, or associated abnormalities, such as arachnoid cysts. Anatomic detail is generally good, and, in the vast majority of cases, proceeding to treatment based on CT scan findings is regarded as safe. However, if endoscopic treatment is considered, MRI should be performed.
  • MRI 
    • For the last 2 decades, MRI has played an increasing role in the management of hydrocephalus in children. Unfortunately, it still is not as widely available as CT scanning.
    • The need for sedation (or even general anesthesia) to obtain good images is a major consideration, especially in very young infants, because acquisition takes several minutes or more and any movement severely deteriorates picture quality. This is particularly problematic in children with associated congenital conditions that cause poor respiratory drive.
    • MRI shows structures of the brain with superior anatomic detail and assists the surgeon in choosing the best treatment. MRI is especially necessary in conjunction with endoscopic treatment, in which the surgeon must verify the presence of aqueductal stenosis (see Media file 2-4) or any intracranial cysts that may be fenestrated, and in determining the relationship of important anatomic structures (eg, the floor of the third ventricle to the basilar artery) in children with aqueductal stenosis.

    • Axial T1-weighted MRI scan of an 8-week-old girl ...

      Axial T1-weighted MRI scan of an 8-week-old girl who presented with enlarging head circumference. Considerable ventricular dilatation is shown on the lateral and third ventricles. Periventricular lucency is observed around the frontal horns, indicating raised intraventricular pressure.

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      Axial T1-weighted MRI scan of an 8-week-old girl ...

      Axial T1-weighted MRI scan of an 8-week-old girl who presented with enlarging head circumference. Considerable ventricular dilatation is shown on the lateral and third ventricles. Periventricular lucency is observed around the frontal horns, indicating raised intraventricular pressure.


    • Sagittal T1-weighted MRI scan of the infant in Me...

      Sagittal T1-weighted MRI scan of the infant in Media file 2. The third and lateral ventricles are dilated, whereas the fourth ventricle is of normal size. Aqueductal stenosis is shown. The appearance is typical of this condition.

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      Sagittal T1-weighted MRI scan of the infant in Me...

      Sagittal T1-weighted MRI scan of the infant in Media file 2. The third and lateral ventricles are dilated, whereas the fourth ventricle is of normal size. Aqueductal stenosis is shown. The appearance is typical of this condition.


    • Phase-contrast MRI scan of the same child in Medi...

      Phase-contrast MRI scan of the same child in Media file 2-3, obtained 3 months after endoscopic third ventriculostomy. A large signal void is shown in the prepontine region, corresponding to the flow through the stoma in the floor of the third ventricle, indicating that the ventriculostomy is functioning well.

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      Phase-contrast MRI scan of the same child in Medi...

      Phase-contrast MRI scan of the same child in Media file 2-3, obtained 3 months after endoscopic third ventriculostomy. A large signal void is shown in the prepontine region, corresponding to the flow through the stoma in the floor of the third ventricle, indicating that the ventriculostomy is functioning well.

    • The use of phase-contrast cardiac-gated sequences can provide information on CSF flow through the aqueduct, and ventriculostomy can provide information on patency of the stoma.
    • Children born with open myelomeningocele have a very high incidence (close to 100%) of hindbrain hernia (see Media file 6).

    • Sagittal T1-weighted MRI scan of the same patient...

      Sagittal T1-weighted MRI scan of the same patient in Media file 5. Significant hindbrain hernia and low-lying fourth ventricle are shown in the context of the Arnold-Chiari II syndrome.

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      Sagittal T1-weighted MRI scan of the same patient...

      Sagittal T1-weighted MRI scan of the same patient in Media file 5. Significant hindbrain hernia and low-lying fourth ventricle are shown in the context of the Arnold-Chiari II syndrome.

    • Marked hydrocephalus is present in as many as 15-20% of these patients, and a further 20% have moderate ventriculomegaly at birth. A large proportion of patients (as many as 90%) eventually develop clinical hydrocephalus. The lateral ventricles have a characteristic appearance in almost all patients with spina bifida (see Media file 5); the occipital horns are more dilated than the frontal horns and the long axis of the lateral ventricles tend to be parallel.

    • Axial T1-weighted MRI scan of a 15-year-old girl ...

      Axial T1-weighted MRI scan of a 15-year-old girl who was born with thoracic myelomeningocele, hydrocephalus, and Arnold-Chiari II syndrome. She was treated with a ventriculoperitoneal shunt. The ventricular system has a characteristic shape, with small frontal and large occipital horns, which are typical in patients with spina bifida. The shunt tube is shown in the right parietal region.

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      Axial T1-weighted MRI scan of a 15-year-old girl ...

      Axial T1-weighted MRI scan of a 15-year-old girl who was born with thoracic myelomeningocele, hydrocephalus, and Arnold-Chiari II syndrome. She was treated with a ventriculoperitoneal shunt. The ventricular system has a characteristic shape, with small frontal and large occipital horns, which are typical in patients with spina bifida. The shunt tube is shown in the right parietal region.

    • A contributing factor may be the partial or complete absence of the falx and absence of the septum pellucidum in a very large proportion of these patients. MRI produces a good image of the hindbrain hernia, the small posterior fossa, and the midbrain deformity with kinking of the aqueduct.

More on Management of Spina Bifida, Hydrocephalus and Shunts

Overview: Management of Spina Bifida, Hydrocephalus and Shunts
Workup: Management of Spina Bifida, Hydrocephalus and Shunts
Treatment: Management of Spina Bifida, Hydrocephalus and Shunts
Follow-up: Management of Spina Bifida, Hydrocephalus and Shunts
Multimedia: Management of Spina Bifida, Hydrocephalus and Shunts
References
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References

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Further Reading

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Keywords

hydrocephalus, infantile hydrocephalus, hydrocephaly, ventriculomegaly, aqueductal stenosis, intraventricular hemorrhage, spina bifida aperta, spina bifida occulta, hydrocele spinalis, schistorrhachis, myelomeningocele, Arnold-Chiari II malformation, Dandy-Walker syndrome  

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Contributor Information and Disclosures

Author

Lynne C Kramer, MD, Fellow in Developmental Pediatrics, Department of Pediatrics, Madigan Army Medical Center
Lynne C Kramer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Kenneth Azarow, MD, Program Director, Pediatric Surgery, Children's Hospital and University of Nebraska Medical Center; Professor, Department of Surgery, Uniformed Services University of the Health Sciences
Kenneth Azarow, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

Brett A Schlifka, DO, Consulting Staff, Department of Neurosurgery, Madigan Army Medical Center
Brett A Schlifka, DO is a member of the following medical societies: American College of Osteopathic Surgeons, American Osteopathic Association, and Philadelphia County Medical Society
Disclosure: Nothing to disclose.

Spyros Sgouros, MD, FRCS(SN)(Glasg), Senior Lecturer, Department of Neurosurgery, Division of Neuroscience, Section of Pediatrics, University of Birmingham, England
Spyros Sgouros, MD, FRCS(SN)(Glasg) is a member of the following medical societies: British Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert Kelly, MD, Chairman, Department of Surgery, Departments of Surgery and Pediatrics, Children's Hospital of the King's Daughters; Associate Professor, Eastern Virginia Medical School
Robert Kelly, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society of Abdominal Surgeons, Medical Society of Virginia, Norfolk Academy of Medicine, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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