Spina Bifida Hydrocephalus and Shunts Clinical Presentation

Updated: May 16, 2023
  • Author: Spyros Sgouros, MD, FRCS(Glasg), FRCS(SN); Chief Editor: Robert K Minkes, MD, PhD, MS  more...
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History and Physical Examination

Infants with hydrocephalus develop an enlarging head with bulging fontanelle, enlarged scalp veins, macrocrania, suture diastasis, and positive Macewen (ie, cracked pot) sign. If the hydrocephalus is not treated, these infants develop sunset eyes, recurrent vomiting, and, later, respiratory arrest. Persistent leakage of cerebrospinal fluid (CSF) from the repaired spinal wound almost invariably indicates active hydrocephalus, even if the ventricular size is only modestly enlarged and the anterior fontanelle is not bulging.



The particular concern in children with myelomeningocele is the presence of hindbrain hernia in the context of the Arnold-Chiari II malformation, which can cause early clinical symptoms of bulbar palsy due to compression of the brainstem and can remain unnoticed by inexperienced observers or be confused with symptoms of shunt malfunction or untreated hydrocephalus.

Manifestations of brainstem dysfunction caused by hindbrain hernia and aggravated by ventricular dilatation include the following:

  • Poor feeding
  • Recurrent vomiting
  • Poor sucking
  • Generally subdued behavior with poor crying
  • High-pitched cry or stridor caused by vocal cord paralysis - A predictor of poor outcome
  • Episodes of apnea
  • Extremity weakness in older children
  • Recurrent aspiration - Often manifesting as recurrent pneumonia

Approximately 20% of children with myelomeningocele who also have an Arnold-Chiari II malformation develop brainstem symptoms. Myelomeningocele is also a contributor to mortality and morbidity in the first two decades of life.

Older children with closed fontanelles develop clinical signs of intracranial hypertension without progressive head enlargement. They develop headaches, blurred vision, decline in intellectual performance, and gradual drowsiness, which, if left untreated, lead to coma and death due to respiratory arrest.