Management of Hydrocephalus and Abnormal CSF Circulation in Children

Initial Management at Presentation

Cerebrospinal fluid shunt

Authors

Kemel Ahmed Ghotme, M.D.

James M. Drake, M.D.

Section Editor

Shlomi Constantini, M.D.

Editor in Chief

Rick Abbott, M.D.

CSF shunts are the most common treatment for hydrocephalus, and they are available worldwide. Before CSF shunts became available, almost 50 years ago, hydrocephalus was a lethal or severely disabling condition. Currently, shunts are a very common neurosurgical procedure, especially in pediatric centers, and have improved the survival and quality of life for many hydrocephalic patients around the world. Notwithstanding, shunts frequently are associated with high rates of complications and a need for re-operation. Shunt malfunction is a frequent cause of consultation in emergency service. Opportune diagnosis, and timely, appropriate treatment are crucial for prevention of permanent injury or death in patients with hydrocephalus. The management of hydrocephalus with shunts is discussed in the pages below starting with Preparation for Managing Hydrocephalus with a Shunt in Children.

Third ventriculostomy

Authors

Jothy Kandasamy, M.D.

Conor Mallucci, M.D.

Rick Abbott, M.D.

Section Editor

Shlomi Constantini, M.D.

Editor in Chief

Rick Abbott, M.D.

The use of ETV as a first-line treatment for pediatric and adult hydrocephalus is becoming more prevalent. Nevertheless, uncertainty persists on the best treatment for patients – ETV or shunt. There have been conflicting reports on who the best candidates are, particularly with regard to the effect of age and etiology. ETV is a useful treatment for obstructive hydrocephalus, which can spare lifelong shunt dependency. Consensus exists regarding the high likelihood of an ETV being successful as a primary treatment in cases of aqueductal stenosis. Controversy remains for the role of ETV in treating hydrocephalus in children that results from other specific disease entities discussed in the pages below beginning with Preparation for Managing Hydrocephalus with a Third Ventriculostomies in Children.

Adjunctive Therapies

Medical management

• Decrease CSF formation rate: Acetazolamide can be used to slow the rate of CSF formation in patients who have a subtle mismatch between the rate of CSF formation and CSF resorption. The most common condition for which acetazolamide is used is benign extraaxial CSF collections. It can also be considered for managing transient postoperative inflammatory meningitis with resulting impairment in CSF resorption.
• Open CSF pathways: Steroids can be used to manage obstructive hydrocephalus due to lesions such as tumors for a short period of time. They presumably work by removing peritumoral edema and shrinking the obstructing mass. They may also slow the rate of CSF formation.

Surgical management

• Removal of obstructing lesions: Increasingly, primary resection of obstructing lesions is felt to be the preferred treatment for obstructive hydrocephalus due to lesions such as tumors.

Follow-up

• Scheduled follow-up visits: It should not be assumed that hydrocephalus can be passively managed with visits to treating physicians being required only for the management of treatment failure. Annual or semiannual visits are helpful for screening for subtle signs of failure in treatment (deterioration in cognition as reflected in school or work performance, discomfort along path of shunt catheter, transient, intermittent symptoms suggesting a mild increase in ICP or inflammation when a shunt is present). It is also an opportunity for patient education about the subtle signs that can identify an impending shunt or ETV failure.
• No accepted rule for frequency for follow-up imaging: There are no studies that clearly identify a frequency of scanning that will reveal an impending treatment failure. Consequently, the frequency of follow-up scanning in a patient without signs of treatment failure is determined by the personal preference of the treating physician.