Encephaloceles are an extension or protrusion of any intracranial matter through a cranial bone defect. The sphenoethmoidal encephalocele is often fatal. For those who survive long enough, expeditious repair is critical. We report a case that was repaired successfully via a combined transcranial and transpalatal approach, and because of successful repair, the patient underwent multiple secondary reconstructions resulting in a 25-year follow-up.
A 3960-g, term male infant from a normal gestation was delivered via cesarean delivery for breech position. Initial examination revealed a 2 × 3-cm gray intraoral nasopharyngeal mass with smooth mucosal covering extruding through a midline palatal cleft. At 4 weeks of life, extradural and intradural exploration of the encephalocele was performed via a bifrontal craniotomy. At 15 months of age, the patient underwent median cleft lip repair. At 6 years of age, hypertelorism was corrected by wedge resection of the frontal and nasal bones and medial mobilization of the orbits. Follow-up was continued until 25 years of age, which revealed excellent maintenance of correction.
Sphenoethmoidal encephalocele is a rare sporadic congenital cranial floor defect associated with typical facial and cerebral anomalies. Encephaloceles extending into the nasopharynx may cause airway obstruction and feeding difficulty and present a potential pathway for central nervous system infection. Repair of the encephalocele should then be performed as soon as possible. Care of patients with nasopharyngeal encephaloceles requires a lifetime of reconstructive surgery. Care of these patients can be rewarding to both families and surgeons.