Surgical closure and reconstruction of a large occipital encephalocele without parenchymal excision

Encephalomeningocele is a herniation of the brain and meninges through a congenital bone defect resulting from the failure of normal midline fusion of the cranial neural tube. Various sites are possible; however, among Caucasians more than 70% of encephaloceles are located on the occipital/posterior part of the cranium. Usually they contain degenerative cerebral cortex, which is often excised during surgical closure of the defect. Techniques to preserve the herniated brain tissue have been described, but they focus on increasing the intracranial cavity by ventriculomegaly and retracting the encephalocele into new intracranial space in a second stage, as a result of hydrocephalic treatment.

The authors report a well-documented newborn with a large encephalocystocele of the left occipital lobe. This encephalocele included the left lateral ventricle and a part of the cerebellum, and was covered with allopathic skin. Initially the cystic portion was removed so that a partial reduction of the encephalocystocele was achieved. Six months later, the surgical closure of the defect was performed, with preservation of the occipital and cerebellar parenchyma, by incising the tentorium and retracting the cortex to the newly created infratentorial space. The bony defect was covered with autologous osseous graft harvested from parietal bone and reconstructed.

In the long-term follow-up after 13 years, the child has preserved visual function.