2025 Proffered Presentations
S046: ANTERIOR SKULL BASE REPAIR OF A GIANT FRONTONASAL ENCEPHALOCELE IN A BABY
Jignesh Tailor, BMBCh, PhD, FRCSC; Emma Cordes; Jonathan Ting; Indiana University School of Medicine
We report the case of a prenatally diagnosed giant frontonasal encephalocele that was repaired on post-natal day 3. The baby was born by c-section at 39+1 weeks gestation with good APGAR scores and breathing spontaneously. A giant skin-covered sac over the glabella was seen measuring approximately 15cm in the cranio-caudal dimension (Fig 1A). The swelling had a healthy covering with a raw area at the apex without any CSF leak. An MRI scan showed a giant CSF-containing sac with encephalocele measuring 9.5cm x 8.2cm x 14.8cm in AP, transverse and craniocaudal dimensions (Fig 1B). Herniation of the inferior frontal gyri was evident, as well as the anterior aspect of the superior sagittal sinus through a 1cm frontal defect at the anterior skull base. CT imaging that the bony defect in the inferior frontal calvarium extending inferiorly along the nasal bridge and nasal dorsum in the midline, measuring1.7cm in width and 1.1cm craniocaudally. The baby was taken to the operating room on day 3. A one-stage repair of the encephalocele was achieved with a multi-disciplinary team involving neurosurgery, ENT and plastics. The bony defect was exposed and defined through the sac, and the herniated tissue was tied off and truncated. A small dural defect was repaired with a buttonhole graft using duragen and tisseal. The nasal bridge was reconstructed with resorbable plates, and the excess skin was excised and reconstructed. The post-operative period was uneventful and the baby was discharged home 3 weeks post-op. At 2 month follow-up, the baby looked well with a good cosmetic outcome and no residual encephalocele on MRI scan (Fig 2A,B). This case illustrates our multi-disciplinary approach to a rare congenital abnormality affecting the anterior skull base in neonates, and highlights our surgical techniques for skull base repair and reconstruction.