Hydrocephalus Prognosis

By Jonas Wilson, Ing. Med.

A disruption in the flow, formation, or absorption of cerebrospinal fluid (CSF) may lead to hydrocephalus. It is a very dangerous condition with life-threatening consequences if left untreated. The disturbance in the hydrodynamics of CSF may be acute, subacute, or chronic, occurring over days, weeks, and months to years, respectively. The disorder has a bimodal distribution in its peak incidence, peaking first in infancy and usually associated with a congenital malformation. Its second peak incidence occurs after the sixth decade of life.

The signs and symptoms that accompany hydrocephalus vary with the patient’s age. In addition to this, the causative factor of the hydrocephalus and the rapidity of the buildup of fluid inside the brain as well as the duration are key determinants to the clinical features seen. Infants present with symptoms such as poor feeding, vomiting, irritability, and reduced activity. Toddlers, older children, and adults show decreased mental capacity and may experience headaches, neck pain, vision disturbances, drowsiness, and unstable balance.

Diagnosis may be made with radiographic imaging techniques such as ultrasound in children less than 12 months old and MRI or CT scans in everyone else. The preferred therapeutic option is surgical placement of shunts to re-channel CSF or removal of blockages that can be removed. Alternatively, choroid plexus cauterization combined with third ventriculostomy may also be performed to reduce the amount of CSF that is produced by the choroid plexus.

Outcomes of Surgery

The mortality rate for hydrocephalus and associated therapy ranges from 0 to 3%. This rate is highly dependent on the duration of follow-up care. The shunt event-free survival is approximately 70% at 12 months and is nearly half that at 10 years, post-operatively. Shunts that are placed to channel CSF to other parts of the body may fail due to malfunction or infection. Infections occur in less than 10% of all surgeries.

Malfunctions that may be seen include over- and under-drainage as well as obstruction and hidden shunt failure. The occurrence of malfunction is noted as being duration-dependent and may generally be regarded as actuarial survival. Malfunctions can be managed with or without further surgery. For instance, over-drainage can be resolved with simple valve adjustment. Infections leading to shunt failure may be classified as incisional, deep-incisional and organ-space infections. Infection is ascertained when there is a diagnosis based on clinical findings and/ or culture that lead to further operation or death.

Long-Term Outlook

The triad of early detection, prompt treatment, and taking the necessary steps to avoid surgical site infections is the cornerstone to ensuring a great long-term outlook. It particularly helps in reducing the chances of developing clinically significant neurological damage. Failure to catch hydrocephalus on time and treat it accordingly may lead to long-term neurological deficits that require multidisciplinary medical teams to assist patients with developmental and lasting cognitive impairment. Neurological damage that may have occurred prior to treatment is unfortunately irreversible and can have a significant impact on functional social outcomes such as social integration, schooling, and marriage.

Reviewed by Susha Cheriyedath, MSc

Further Reading

Last Updated: Oct 10, 2016

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