Repair of a large primary subtemporal encephalocele in a 3-year-old child: case report

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Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance that they project through the floor of the middle fossa they are secondary. In this case report the authors report on the management of a giant PE extending through the floor of the middle fossa.

An 8-month-old boy presented to the authors’ service with a large PE projecting into his neck through a missing left middle fossa floor; the lesion was causing significant meta-, dys-, and hypoplasia of the structures of the anterolateral neck on that side. Surgical goals for this patient included the following: 1) removal of potentially epileptogenic and dysfunctional tissue; 2) preservation of cranial nerves; 3) prevention of cognitive decline or iatrogenic deficit; 4) prevention of CSF leak; 5) reconstruction of skull base; 6) prevention of airway and swallowing compromise; and 7) cosmesis. After a multidisciplinary evaluation with ENT, plastic surgery, and neurology, an operation was performed using a preauricular infratemporal approach when the patient was 3 years old. Gliotic tissue was resected and amygdala, hippocampus, and middle cerebral artery were preserved.

The immediate results of the operation showed good immediate outcome. Seizure freedom and neurodevelopment outcomes remain to be seen at follow-up.

ABBREVIATIONS ECoG = electrocorticography; PE = primary encephalocele.
Article Information

Contributor Notes

Correspondence Ian Mutchnick: Norton Neuroscience Institute/Norton Children’s Hospital, Norton Healthcare, Louisville, KY. ian.mutchnick@nortonhealthcare.org.INCLUDE WHEN CITING Published online October 18, 2019; DOI: 10.3171/2019.8.PEDS19266.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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