The rationale:
Encephalocoele is a herniation of the brain formed during embryonic development, because of the incomplete closure of Neural Tube. It is a rare skull defect and most cases are located in the occipital bone. Frontal encephalocoeles are very rare and they may involve the ethmoid bone, nasal bones and/or the orbits. Surgical repair is complex and usually requires a multidisciplinary approach.
Patient concerns:
We present a case of a 6-month-old baby girl with a congenital frontoethmoidal encephalocoele.
Diagnosis:
The diagnosis was made by computed tomography scan that showed a defect on the frontoethmoidal zone.
Treatment:
Cranial approach was employed using standard bicoronal access. The malformation was removed and the defect was repaired by using an autologous parietal bone graft, without any complication in the follow-up.
Outcomes and take-away lessons:
The goal of the surgery is to reconstruct the normal anatomy, to achieve a good cosmetic repair, and to avoid a cerebrospinal fluid leak. A description of the case and the surgical technique is presented with a review of literature.