Hideki Ogiwara1, Nobuhito Morota. 1. Division of Neurosurgery, National Center for Child Health and Development, Tokyo, Japan. hideki_o@d5.dion.ne.jp
Abstract
OBJECT: Transsphenoidal encephalocele (TSEC) is a rare developmental anomaly with herniation of neural elements including pituitary gland or optic apparatus, through a defect of sphenoid bone. To prevent obstructive dyspnea, endocrine dysfunction, or visual disturbance, surgical correction is usually performed. However, the optimal operative approach has not been determined yet. The authors present their surgical experiences with transpalatal, transcranial, or combined approach. METHODS: The authors retrospectively analyzed surgical results in patients with TSEC. The charts were reviewed for demographic data, clinical presentation, surgical therapy, and clinical outcomes. RESULTS: Seven consecutive patients with TSEC were surgically treated between March 2004 and February 2012. The mean age was 23.9 months (range 2-69 months). Four patients with TSEC who had a bone defect within the sphenoid bone were successfully treated using a transpalatal approach. Two patients with an extensive sphenoethmoidal bone defect that was treated either by a transpalatal or transcranial approach experienced several relapses with a prolapse of the encephalocele. One patient with an extensive sphenoethmoidal bone defect was successfully treated by combined transpalatal and transcranial approach without relapse for 3.6 years. CONCLUSIONS: The combined transpalatal and transcranial approach is useful for TSEC with an extensive sphenoethmoidal bone defect to dissect the sac and expose the bone defect circumferentially. If dissection is not sufficient, relapse with a prolapse of the encephalocele can ensue. The transpalatal approach is sufficient for TSEC in which a bone defect is limited within the sphenoid bone.
OBJECT: Transsphenoidal encephalocele (TSEC) is a rare developmental anomaly with herniation of neural elements including pituitary gland or optic apparatus, through a defect of sphenoid bone. To prevent obstructive dyspnea, endocrine dysfunction, or visual disturbance, surgical correction is usually performed. However, the optimal operative approach has not been determined yet. The authors present their surgical experiences with transpalatal, transcranial, or combined approach. METHODS: The authors retrospectively analyzed surgical results in patients with TSEC. The charts were reviewed for demographic data, clinical presentation, surgical therapy, and clinical outcomes. RESULTS: Seven consecutive patients with TSEC were surgically treated between March 2004 and February 2012. The mean age was 23.9 months (range 2-69 months). Four patients with TSEC who had a bone defect within the sphenoid bone were successfully treated using a transpalatal approach. Two patients with an extensive sphenoethmoidal bone defect that was treated either by a transpalatal or transcranial approach experienced several relapses with a prolapse of the encephalocele. One patient with an extensive sphenoethmoidal bone defect was successfully treated by combined transpalatal and transcranial approach without relapse for 3.6 years. CONCLUSIONS: The combined transpalatal and transcranial approach is useful for TSEC with an extensive sphenoethmoidal bone defect to dissect the sac and expose the bone defect circumferentially. If dissection is not sufficient, relapse with a prolapse of the encephalocele can ensue. The transpalatal approach is sufficient for TSEC in which a bone defect is limited within the sphenoid bone.