Presentation of Atretic Encephaloceles in Children

Symptoms

  • Scalp-covered midline lesion: The symptom that brings most children with atretic encephalocele to consultation is the presence of the lesion itself, which is usually detected at birth (28). Atretic encephaloceles occur almost exclusively in two main locations of the head’s midline, the parietal and the occipital regions (16, 23, 33). There is only one report of a laterally placed lesion and another of a fronto-ethmoidal atretic encephalocele (2, 29).
  • Pain: Some lesions may produce spontaneous local pain (16). In addition, some patients refer pain on palpation, after straining, or with activities of daily life (washing or combing their hair).
  • Other organ involvement when syndromic: Extracranial abnormalities (eye, heart, muscle, etc.) may be found in instances of atretic encephaloceles pertaining to a syndrome (8, 16).

Patterns of evolution

  • Unusual to grow: Most atretic encephaloceles do not grow over time.
  • Possible regression: Some atretic encephaloceles may undergo regression, and the nodular lesions may become smaller and firmer, while cystic encephaloceles may become flat or scarred.

Intervention

Stabilization

  • General condition usually stable: Patients with atretic encephaloceles are usually stable. Most cases do not endanger the patients’ life. Concerns may arise in the presence of hydrocephalus or associated malformations, in which instances patients must be evaluated individually for special needs.

Preparation for definitive intervention, nonemergent

  • Preanesthetic consultation: Consultation with the anesthetist should be performed, especially in cases with extracranial problems.
  • Possible significant blood loss: Given the vicinity of the sagittal sinus, availability of preoperative cross-matched blood is advisable.

Preparation for definitive intervention, emergent

  • Emergent intervention highly improbable: Theoretically, a lesion leaking CSF or bleeding could necessitate emergent intervention. Measures are similar to those described above. Rarely, a child might need placement of an EVD for management of acute hydrocephalus.

Admission Orders

  • Admission for surgery: Children presenting with atretic encephaloceles need no admission for diagnostic evaluation. They usually can be evaluated on an outpatient basis.
  • Routine admission orders: At the author’s institution, routine blood tests and cross-matching are customarily performed the evening before operation. The duration of fasting time is established according to the child’s age.