Giant occipital encephalocele in a neonate undergoing successful neurosurgical intervention

A female neonate antenatally diagnosed with occipital encephalocele (OE) at 7 months of gestation was born by lower segment cesarean section at 38 weeks, weighing 3000 g. A large cystic swelling was seen arising from the posterior part of the head measuring 20 × 15 × 20 cm with a rim of transparent tissue, with no evidence of bleed or rupture [Figure 1]. She was hypotonic with a depressed sensorium. Magnetic resonance imaging showed a large OE through a 1.6 cm defect [Figure 2], requiring emergency surgical repair [Figures 3 and 4]. She also required a ventriculoperitoneal shunt secondary to post-surgical communicating hydrocephalus and was successfully discharged.

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Figure 1:

Large occipital encephalocele with an intact rim of transparent tissue covering it.

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Figure 2:

Magnetic resonance imaging brain showing a large occipital encephalocele through a 1.6 cm defect.

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Figure 3:

Intraoperative image following removal of dysplastic neural tissue.

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Figure 4:

Post-operative image with dura mater and skin closed in layers.

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The incidence of encephaloceles is approximately 1–4 cases per 10,000 live births, with OE being the most common.^[1] The surgical prognosis depends on the timing of presentation and associated complications such as hydrocephalus and rupture of the sac. Delay leads to increasing difficulty during surgical restoration with hemodynamic instability during the same.^[2]