Frontoethmoidal encephalocele repair in prematurity: illustrative case

Prenatal care in medically advanced countries dramatically decreases rates of neural tube defects. Yet despite this standardization, rare cases of encephaloceles persist in the United States. Definitive repair is ideally delayed, allowing for physiological maturation and improved tolerance of anesthesia and blood loss.

A male was born at 28 weeks and 3 days' gestation to a nulliparous group B Streptococcus-positive mother who had not received any prenatal care. At the time of birth, the patient was noted to have a large frontoethmoidal encephalocele with subsequent concern for CSF egress through a skin defect, necessitating urgent surgical intervention.

In this illustrative case, the rarity and challenges of anterior encephalocele repair in extreme prematurity are evaluated. The report focuses on the surgical goals of encephalocele repair, including minimization of blood loss, controlled encephalocele excision, durable repair of dural and bony defects, and tension-free skin closure. The authors emphasize an optimal use of cadaveric bone for skull defect reconstruction to limit autograft morbidity. https://thejns.org/doi/10.3171/CASE2614.