Occipital Encephalocele


Risk

  • Most frequent type of encephalocele in North America and Western Europe

  • Incidence in North America: 1:3000–10,000 live births

  • Worse prognosis compared with frontal encephaloceles

Perioperative Risks

  • Seizures

  • Hydrocephalus

  • Cranial nerve deficits

  • Poor feeding

  • Spasticity

  • Blindness

Worry About

  • Positioning concerns

  • Coexisting congenital anomalies, especially renal and facial

  • Difficult airway

  • Elevated ICP

  • Body temperature changes

  • IV access

  • Blood loss

  • Hemodynamic disturbances

Overview

  • Herniation of brain, meninges, and/or CSF through a skull defect (cranium bifidum) that is usually covered with skin

  • One of the three most common neural tube defects

  • Cranial nerve deficits, poor sucking and feeding, spasticity, blindness, seizures, or developmental delay

  • May be associated with hind-brain anomaly (Chiari III malformation), in which herniating occipital/cerebellar tissues distort the posterior fossa structures

  • Associated conditions include:

    • Hydrocephalus (30–50%)

    • Corpus colossal abnormalities (18%)

    • Cerebral dysgenesis (13%)

    • Seizures

    • Meckel Gruber syndrome

    • Occipital encephalocele

    • Microcephaly

    • Microphthalmia

    • Polycystic kidneys

    • Ambiguous genitalia

    • Polydactyly

    • Cleft lip and palate

    • Other malformations

Etiology

  • Unknown

  • Isolated encephaloceles showing no familial inheritance

  • Possibly a syndrome with an autosomal recessive pattern or inheritance

  • Usually obvious at birth, with many diagnosed prenatally using fetal US or fetal MRI

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