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Hydrocephalus in the child with dysraphism
1Children's Hospital of Buffalo, New York.
Insights
Hydrocephalus affects 85% of myelomeningocele patients, often due to Chiari malformation obstructing cerebrospinal fluid (CSF) flow. Recognizing subtle symptoms is key to timely shunt revision and avoiding risky procedures.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Developmental Biology
Background:
- Hydrocephalus is a common complication in myelomeningocele, affecting up to 85% of patients.
- The primary cause is often obstruction of cerebrospinal fluid (CSF) flow at the posterior fossa and tentorial hiatus.
- This obstruction is frequently linked to posterior fossa crowding associated with Chiari malformation.
Purpose of the Study:
- To elucidate the mechanisms of hydrocephalus in myelomeningocele patients.
- To detail the diverse clinical presentations of hydrocephalus in this population.
- To emphasize the importance of early diagnosis and appropriate management.
Main Methods:
- Review of clinical data and neuroimaging findings in patients with myelomeningocele and hydrocephalus.
- Analysis of the relationship between Chiari malformation, CSF flow dynamics, and hydrocephalus development.
- Correlation of presenting signs and symptoms with diagnostic findings and treatment outcomes.
Main Results:
- Posterior fossa crowding due to Chiari malformation is a significant cause of CSF obstruction and hydrocephalus.
- Clinical manifestations range from overt signs of intracranial hypertension to subtle neurological and behavioral changes.
- Symptoms can mimic other conditions like brainstem compression or spinal cord dysfunction.
Conclusions:
- Prompt identification of hydrocephalus signs and symptoms in myelomeningocele is crucial.
- Appropriate management, primarily shunt revision, can be guided by recognizing these presentations.
- Timely intervention can prevent unnecessary and potentially hazardous procedures.
Abstract:
Hydrocephalus develops in approximately 85% of patients with myelomeningoceles and appears most frequently to result from obstruction to cerebrospinal fluid (CSF) flow within the posterior fossa subarachnoid space and at the tentorial hiatus, due to posterior fossa crowding from the Chiari malformation. The presenting features of hydrocephalus are legion; although signs and symptoms of intracranial hypertension are most common, many patients exhibit more subtle and confusing signs, such as intellectual deterioration or behavioral changes, or those that mimic brainstem compression from the Chiari malformation or spinal cord dysfunction due to tethering or syringomyelia. Prompt recognition of these signs and symptoms will direct appropriate therapy toward shunt revision and will help the clinician avoid potentially unnecessary and more dangerous procedures.