Extended Endonasal Transtuberculum Approach for Management of Supradiaphragmatic Craniopharyngioma in Pediatrics, Assessment of Clinical Outcome, Resection Rate, Postoperative Complications with Intermediate-Term Follow-up: A Single-Center Study

Abstract

Objective: The aim of this study is to assess the efficacy and safety of extended endonasal transtuberculum approach (EETA) for management of supradiaphragmatic craniopharyngioma in pediatrics (age <18 years). Methods: A retrospective review to all pediatric patients underwent EETA for management of supradiaphragmatic craniopharyngioma at our institute from 2018 to 2023 was performed. Demographics, clinical presentation, previous interventions or radiation, preoperative tumor characteristics, and sinus pneumatization were studied. Repair technique, the extent of resection, length of hospital stays, clinical outcome including visual and endocrinological function, weight gain, and postoperative complications were assessed. Follow-up duration, postoperative adjuvant therapy, and tumor recurrence/progression were recorded. Results: We performed this approach in 16 patients, 11 (69%) males and 5 (31%) females, mean age of patients at the time of surgery was 11.9 years (range: 7–16 years). All patients had Visual affection before surgery, 14 (86%) had partial or panhypopituitarism, four (25%) had preoperative diabetes insipidus (DI), nine (56%) presented by headache, ten (62%) patients had hydrocephalus before surgery, and we performed V-P shunt to all of them, four (25%) patients had previous transcranial surgery, two (13%) had previous Ommaya reservoir inserted, two (13%) performed previous transsphenoidal surgery, and three (19%) had previous radiotherapy. The average maximum tumor diameter was 4.2 cm (range: 2.7–6.1 cm), 13 (81%) of them had mixed cystic and solid components, two were purely solid and one case was purely cystic, the tumor had hypothalamic involvement by variable degrees in 13 (81%) cases and extending to 3rd ventricle in 10 (62%) cases. Presellar sphenoid sinus pneumatization was present in six (38%) cases versus 10 (62%) cases with sellar pneumatization. We achieved gross total resection (GTR) of the tumor in 8 (50%) patients, near total resection (>95%) in 5 (31%) and subtotal resection (<95%) in 3 (19%) patients, location of the residual was attached to hypothalamus in 4 (50%) cases, to infundibulum in two (25%) cases, to optic nerve chiasm in two (25%) cases, stalk was preserved in 6 (37%) cases. Visual improvement achieved in 10 (63%) cases and was stable in 4 (25%) cases. Two cases had postoperative visual deterioration. Eight (50%) cases had more postoperative hormonal deficit, five (31%) patients had new onset obesity. No cases had postoperative CSF leak after surgery; however, two (13%) have symptomatic progressive pneumocephalus that required reoperation to explore the repair and was successfully managed, three patients had shunt malfunction, two cases had meningitis and required long-term hospital stay, one of them had postoperative medical complication and died 32 days after surgery. The mean follow-up duration time was 28.5 months (range: 12–65 months), two patients had tumor recurrence/progression, both received radiation therapy and one inserted an Ommaya reservoir. Conclusion: EETA is a very versatile approach for management of supradiaphragmatic craniopharyngioma in pediatrics even in young children with presellar sphenoid sinus, and large tumors with solid components, direct inspection of the hypothalamus spares hypothalamic injury; however, the rate of postoperative endocrine dysfunction is high and the learning curve for the approach is steep. Publication History Article published online: 07 February 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany