Congenital Median Perisellar Transsphenoidal Encephaloceles in Children: Surgical Treatment with an Extracranial Transoral Transnasal Approach

Objective: Among developmental defects, median perisellar transsphenoidal encephaloceles (CTE) are rare. The protrusion of the dural sac through a sphenoid bone defect into the extracranial space, combined with the stretching of neural structures within it, exposes both to high risk of CSF leak and meningitis due to dural tear, and multiple dysfunctions, such as endocrine, visual, and respiratory impairments. The natural history will therefore lead to severe maldevelopment and, eventually, death. Surgical treatment with repair of the bone defect and repositioning of the encephalocele into the anterior cranial fossa allows change to this evolution but is associated with a high risk of complications, mortality, and recurrences.

Design: We analyzed the surgical treatment offered to children with congenital median perisellar transsphenoidal encephalocele. The evaluation was focused on pitfalls, complications, and long-term results.

Patients: An extracranial transoral or transnasal surgical approach was the treatment chosen for six patients admitted over 16 years. In one case, parents refused surgery and the patient was followed up as an outpatient. Mean age at surgery was nine years.

Results: Preoperative symptoms remained stable or improved in all the patients after surgery but worsened in the untreated patient. No mortality was recorded. The main complication was palatal dehiscence, which happened in two patients and required further surgical treatment.

Conclusions: The extracranial approach provides all around exposure of the sac, reducing the risks of pulling and tearing of the dura and its contents. Long-term clinical results are satisfactory.