Endonasal Management of Pediatric Congenital Transsphenoidal Encephaloceles: Nuances of a Modified Reconstruction Technique

Objective: Congenital transsphenoidal encephaloceles are rare malformations and their surgical treatment remains challenging. This paper reports 3 cases of transsphenoidal encephalocele in 8 to 24-month-old infants, who presented mainly with airway obstruction, respiratory distress, and failure to thrive.

Methods: We discuss the surgical management of these lesions via a minimally invasive, endoscopic endonasal approach, as compared with the traditional transcranial and transpalatal approaches. A unique endonasal management algorithm for these lesions is outlined. The lesions were repaired without any resection of the encephalocele sac, and the cranial base defects were reconstructed with titanium mesh plates and vascular nasoseptal flaps.

Results: Reduction of the encephalocele and reconstruction of the skull base was successfully accomplished in all three cases with favorable results.

Conclusions: The described endonasal management algorithm for congenital transsphenoidal encephaloceles is a safe, viable alternative to traditional transcranial and transpalatal approaches, and avoids much of the morbidity associated with these open techniques.