Outcome of Therapies for Atretic Encephaloceles in Children

Outcome After Surgery

  • >50% normal development: More than 50% of patients with atretic encephalocele attain normal neurological and psychomotor development.
  • Occipital better than parietal in some series: In the series of Yokota et al., occipital atretic encephaloceles carried a better prognosis than parietal lesions The author and colleagues found the opposite, namely that children with occipital atretic encephaloceles had a worse outcome than those with parietal lesions (16, 33). However, in the author’s series, patients with Walker-Warburg syndrome accounted for 5 of the 9 cases of occipital atretic encephaloceles (16, 17).
  • Associated CNS anomalies affect prognosis: The author concludes that the prognosis for atretic encephaloceles does not depend on the size or location of the lesions, but, on the contrary, depends solely on the associated intracranial anomalies, designated “occult brain anomalies,” that can occur with these lesions. The atretic encephalocele merely represents the “tip of the iceberg.”