Suprasellar and Recurrent Pediatric Craniopharyngiomas: Expanding Indications for the Extended Endoscopic Transsphenoidal Approach

Background The expanded endonasal endoscopic transsphenoidal approach (EEA) has become increasingly utilized for craniopharyngioma surgery in the pediatric population, but questions still persist regarding its utility in younger children, recurrent and radiated tumors, and those masses primarily in the suprasellar region. The narrow corridor, incomplete pneumatization, and fear of hypothalamic injury have traditionally relegated this approach to older children with mostly cystic craniopharyngiomas centered in the sella.

Objective We present a series of consecutive pediatric patients in whom EEA was utilized to remove craniopharyngiomas from patients with varied ages, regardless of the location of the tumor, or previous treatments or surgeries to ascertain if traditional concerns about limitations of this approach are worth re-evaluating.

Methods Eleven consecutive pediatric (≥18 years) patients had surgery at the New York Presbyterian Hospital, Weill Cornell Medical College from 2007 to 2016 using EEA. Location, consistency, size, radiographic hypothalamic invasion, and skull base measurements were measured and parameters such as extent of resection, visual function, endocrinological function, weight gain, and return to school were assessed.

Results The average age at the time of surgery was 7.9 years (range: 4−17 years) and the tumor sizes ranged from 1.3 to 41.7 cm³. Five cases were purely suprasellar, five had solid components, four were reoperations, and 5 had a conchal sphenoid aeration. Nevertheless, GTR was achieved in 45% of the patients and 50% of the 10 in whom it was the goal of surgery, without any correlation with the location, consistency of the tumor, or the age of the patient. STR was performed intentionally in one to avoid hypothalamic injury. Anterior pituitary dysfunction occurred in 81.8%, and 63.3% had diabetes insipidus. Two patients (18%) had a > 9% increase in BMI. Visual function was stable or improved in 73%. All children were back to an academic environment, with 10 of them in the grade appropriate for age. There was one cerebrospinal fluid leak, one loss of vision in a single eye, and one abscess.

Conclusion EEA is suitable for removing pediatric craniopharyngiomas even in young children with suprasellar tumors, conchal sphenoid sinus, recurrent tumors, and tumors with solid components. The extent of resection is dictated by intrinsic hypothalamic tumor invasiveness rather than the approach. EEA affords the ability to directly inspect the hypothalamus to determine invasion, which may help spare hypothalamic injury. Irrespective of approach, the rates of postoperative endocrinopathy remain high and the learning curve for the approach to a relatively infrequent tumor is steep.