Tailor-made orbitocranioplasty for a sphenorbital encephalocele presenting as pulsatile exophthalmos. Case report

Basal encephaloceles account for only 1.5% of all encephaloceles. Within this group, the sphenorbital type is quite rare but causes herniation of the brain into the orbit, leading to pulsatile exophthalmos and disturbance of visual acuity. The authors present a case of successful orbitocranioplasty for a sphenorbital encephalocele in which they used a skull model in preparation for the operation. A plaster skull model was made from 3D computed tomography (CT) data obtained in a 6-year-old girl with a sphenorbital encephalocele to determine the appropriate size and shape of an implant for repair of the malformation. A hydroxyapatite ceramic implant with high affinity to autologous bone was designed to cover the defect and simultaneously to enlarge the volume of the orbit as well as allow rigid fixation. Postoperative 3D CT scanning demonstrated good placement of the implant, and the patient's pulsatile exophthalmos diminished immediately. The patient's postoperative condition has been good, and she has exhibited no neurological deficit or deformity of the skull around the cranioplasty 30 months after the operation. A skull model using 3D CT data is useful for determining the most appropriate strategy for reconstruction. Hydroxyapatite ceramic implants have high affinity to autologous bone and thus are suitable for children. In cases such as the present one, however, the patient should be observed carefully to ensure that there is not an imbalance between the implant and the still growing autologous skull.