Purpose: This paper reports an unusual case of a transsphenoidal encephalocele and discusses our experience with a minimally invasive management. To the best of our knowledge, we present the first case of a combined endoscopic transnasal and transoral approach to a transsphenoidal encephalocele in an infant.
Methods: A 17-day-old boy, who was referred for further assessment of upper airway obstruction, presented with respiratory distress and feeding difficulties. Bronchoscopy and imaging revealed a transsphenoidal encephalocele. At the age of 48 days, he underwent a combined endoscopic transnasal and transoral excision of the nasal component of the encephalocele. This approach, with the aid of neuronavigation, allows good demarcation of the extra-cranial neck of the transsphenoidal encephalocele. We were able to cauterize and carefully dissect the sac prior to excision. The defect of the neck was clearly visualized, and Valsalva manoeuvre was performed to exclude any CSF leak. As the defect was small, it was allowed to heal by secondary intention.
Results: The patient's recovery was uneventful, and he tolerated full feeds orally on day 2. Postoperative imaging demonstrated no evidence of recurrence of the nasal encephalocele. Endoscopic follow-up showed good healing of the mucosa and no cerebrospinal fluid leak.
Conclusions: The surgical management of transsphenoidal encephalocele in neonates and infants is challenging. We describe a safe technique with low morbidity in managing such a condition. The combined endoscopic transnasal and transoral approach with neuronavigation is a minimally invasive, safe and feasible alternative, even for children below 1 year of age.