Endoscopic Endonasal Management of Pediatric Congenital Transsphenoidal Encephalocele: A Case Report

Abstract

Congenital transsphenoidal encephaloceles are rare and typically observed only in very young children. The surgical management of these patients remains challenging. Here we describe the case of a 15-month-old female child with a large trans-sphenoidal encephalocele that was managed purely with the endonasal endoscopic approach (EEA). The child was born with a large nasal mass that caused respiratory distress and feeding difficulty, and she required a tracheostomy and G-tube placement. Imaging studies showed a large left middle cranial fossa arachnoid cyst and a midline naso-oropharyngeal encephalocele extending from the sella through the defect in the sphenoid bone. A cystoperitoneal shunt was inserted for the arachnoid cyst at the age of three months. The patient developed bacterial meningitis twice, one month after shunt placement, and at the age of 14 months. Due to her recurrent infections, we decided to proceed with surgical repair. We felt that a transcranial approach will be limited by the location of the anterior cerebral arteries. Therefore, the patient underwent EEA with insertion of a lumbar drain (LD) to repair the encephalocele sac and bony defect. Cerebrospinal fluid (CSF) drainage from the LD continued at a rate of approximately 10 mL/hour. On postoperative day 5, a CSF leak was noted that required a repeat EEA. Further dissection of the encephalocele sac was performed up to the layer of the dural ring. The dural ring was exposed anteriorly and laterally on both sides. We then proceeded with the multilayered reconstruction of the defect. The patient recovered after the surgery with no signs of a CSF leak and was discharged home. At the four month follow-up, she had no further respiratory distress and was decannulated. To our knowledge, EEA for the repair of trans-sphenoidal encephalocele has been performed in only four children ≤18 months, and a postoperative CSF leak occurred in one of them. The EEA is a safe and feasible alternative to transcranial and transpalatal approaches.