Endoscopic Management of Giant Pediatric Craniopharyngioma

PROGRAM DESCRIPTION: Pediatric craniopharyngiomas are sellar-suprasellar epithelial tumors with devastating effects on childhood development. It has the potential to cause obstructive hydrocephalus which can lead to developmental delay, visual changes, endocrinopathies, and motor skill regression. A 13-year-old girl presented with panhypopituitarism and intermittent headaches. CT and MRI scans demonstrated a massive 5 cm craniopharyngioma involving the suprasellar, prepontine, and internal acoustic meatal cisterns resulting in significant hydrocephalus. A complete endoscopic endonasal excision was accomplished. The key portions of this approach included bilateral total sphenoethmoidectomies, nasoseptal flap harvest, and a posterior septectomy. Then, the sphenoid rostrum, tuberculum sellae, and planum sphenoidale were also removed. Key portions of intradural tumor resection included using the working corridor between the optic chiasm and pituitary gland to perform intracapsular debulking. This allowed egress of the cyst wall, which was removed until a clear view of the basilar artery, third ventricles, and foramen of Monro was appreciated. Duragen inlay and nasoseptal flap onlay reconstructed the skull base. The patient is currently 1 year from surgery with no evidence of disease.

This Masters of Surgery video presentation will discuss the approach selection, salient operative technique, the reconstructive ladder, and the need for lumbar drain for pediatric skull base surgery. This presentation is intended for pediatric otolaryngologists and rhinologists. It assumes a complex knowledge of sinonasal and neuroanatomy.