A. Emergency Management. If patient demonstrates signs of increased intracranial pressure on presentation, perform a complete assessment expeditiously, along with urgent neuroimaging.
B. Neurosurgery Consultation. If acute signs of increased intracra-nial pressure are noted, arrange neurosurgical consultation. Emergent placement of ventriculostomy or ventriculoperitoneal shunt may be indicated.
C. Evaluate for Underlying Etiologies. If suggested by history, physical exam, or laboratory evaluation.
D. Follow-up. Patients with macrocephaly and hydrocephalus need frequent follow-up. Patients are seen regularly for signs of increased intracranial pressure (before shunting is required or for shunt failure) in addition to developmental and cognitive follow-up.
VI. Problem Case Diagnosis. The 6-month-old infant was found on examination to have macrocephaly and mild gross motor delay. His prenatal history was significant for ventriculomegaly, noted during the third trimester. Family and past medical histories were unremarkable, as was physical exam (no neurocutaneous stigmata or organomegaly). Neurologic exam was significant only for macrocephaly and mild gross motor delay. Head circumference was above the 98th percentile for a 6-month-old male infant, which is 50th percentile for a 2-year-old child. Nonemergent MRI scan of the brain demonstrated hydrocephalus, cystic dilation of the fourth ventricle, and cerebellar hypoplasia: the Dandy-Walker malformation. No other intracranial abnormalities were noted. This case is not a medical emergency because the macrocephaly is not associated with any symptoms at this time; however, patient will require frequent follow-up.
VII. Teaching Pearl: Question. Does the presentation of Dandy-Walker malformation vary depending on age of diagnosis?
VIII. Teaching Pearl: Answer. The disorder presents with the triad of hydrocephalus, cystic dilation of the fourth ventricle, and cerebellar hypoplasia (complete or partial agenesis of the cerebellar vermis). Hydrocephalus can develop in utero or during early childhood (typically, first year of life). In older children, the disorder can present with signs of increased intracranial pressure (lethargy, headache, and vomiting) or cerebellar dysfunction (ataxia). Other CNS anomalies may be present, including agenesis of the corpus callosum, heterotopias, congenital tumors, and aqueductal stenosis.
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