2025 Proffered Presentations
S383: UTILITY OF ENDOSCOPIC ENDONASAL CYST FENESTRATION AND MARSUPIALIZATION IN THE TREATMENT OF PEDIATRIC CRANIOPHARYNGIOMA
David K Lerner, MD1; Dominick Rich, BA2; Alan D Workman, MD, MTR3; Sanjena Venkatesh2; Sarah Barnett, BA2; Jacob Eide, MD4; Adam J Kundishora, MD5; Jennifer E Douglas, MD2; Michael A Kohanski, MD, PhD2; James N Palmer, MD2; Nithin D Adappa, MD2; Phillip Storm, MD5; 1University of Miami; 2University of Pennsylvania; 3Massachusetts Eye and Ear Infirmary; 4Mayo Clinic; 5Children's Hospital of Philadelphia
Introduction: Craniopharyngiomas are rare anterior skull base tumors typically associated with loss of pituitary function. For young patients who present with craniopharyngiomas and do not have panhypopituitarism, treatment with resection or radiation often results in significant hypopituitarism, hypothalamic obesity, neurocognitive effects, and vascular complications, which can lead to stroke either in the acute phase (surgery) or in a delayed timeframe (radiation). Additionally, there is a risk of secondary malignancy following radiation therapy. Here we report our outcomes utilizing a novel approach of endoscopic cyst fenestration and marsupialization for decompression and pituitary preservation.
Methods: A retrospective review of all pediatric patients undergoing endoscopic craniopharyngioma cyst fenestration and marsupialization between 2014 and 2024 at our institution was performed.
Results: Fourteen patients with an average age at surgery of 6.8 years underwent 19 endoscopic cyst fenestration and marsupializations. All patients had intact pituitary function preoperatively, and pituitary function was preserved after 16 procedures (84.2%) postoperatively. Our technique evolved from cyst marsupialization without nasoseptal flap (3 procedures) to a nasoseptal flap-lined marsupialized cyst cavity without and then with temporary silastic stenting (7 procedures, 9 procedures). A cerebrospinal fluid leak (CSF) was encountered intraoperatively in 10 cases (52.6%) with one post-operative CSF leak (5.3%). The average time from cyst marsupialization to either resection or most recent follow-up across all patients was 16.1 months. Three patients have not required any further intervention with an average follow-up length of 23.4 months. Four patients underwent repeat fenestrations, including one patient who underwent two additional fenestrations with an average time from first fenestration to resection or radiation of 30.3 months.
Conclusions: Endoscopic cyst fenestration and marsupialization is a decompressive option for pediatric patients with cystic craniopharyngioma and intact pituitary function as a hormonal preservation strategy during years of critical development. Our work provides proof of concept that endoscopic cyst marsupialization can be accomplished while preserving pituitary function. Nearly half of the patients experienced durable decompression for more than 30 months before undergoing resection. By extending the time before resection with preserved pituitary function, endoscopic cyst marsupialization may support normal childhood development hormonally or serve as a bridge until a child meets the criteria for a therapeutic trial. As we refine our technique and patient selection, we may be able to further improve long-term preservation of pituitary function.