Occipital Encephalocele

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

Seizures

Hydrocephalus

Cranial nerve deficits

Poor feeding

Spasticity

Blindness

Positioning concerns

Coexisting congenital anomalies, especially renal and facial

Difficult airway

Elevated ICP

Body temperature changes

IV access

Blood loss

Hemodynamic disturbances

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

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