Management of an open nasofrontal encephalocele during the first day of life

Abstract

Nasofrontal encephaloceles are extremely rare craniofacial defects that present with herniation of cerebral tissue through the junction of the frontal and nasal bones. They often have a cutaneous covering which allows management to be delayed until early infancy, decreasing the risks of anesthesia and acute blood loss. Further bone development also facilitates cranial remodeling. Encephaloceles with a compromised cutaneous envelope pose an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. This report presents a patient born with an open nasofrontal encephalocele that underwent encephalocele excision, dural repair, and cranial remodeling on the first day of life. Anterior encephaloceles represent a spectrum of uncommon craniofacial dysraphias. Given the rarity of these diseases, it is important to understand their many presentations and the treatment options that exist for each. When the cutaneous covering of the encephalocele is compromised, surgical correction must be undertaken urgently to mitigate the mortality associated with neonatal meningitis. This represents the first report of an open nasofrontal encephalocele managed in the first day of life. A multidisciplinary surgical approach involving the neurosurgical and craniofacial surgical teams is paramount to the treatment and survival of such complicated patients.