Resection of a Recurrent Craniopharyngioma in a 4-Year-Old Patient: an Endoscopic Endonasal Transplanum/Transtuberculum Approach with Nasoseptal Flap Reconstruction

Purpose: Craniopharyngiomas are benign lesions that represent 6 to 9% of pediatric brain tumors. Surgical resection is the primary treatment modality, with several approaches described including open and endoscopic approaches. In pediatric patients, the endoscopic approach has been utilized with success; however, a minority of these patients are under 5 years of age. Skull base reconstruction with vascularized nasoseptal flaps are a viable option in the pediatric patient population, although preoperative radiographic measurements should be performed to ensure adequate length and coverage.

Methods: We present the case of a recurrent craniopharyngioma in a 4-year-old patient. The recurrent tumor was resected through an endoscopic endonasal transplanum/transtuberculum approach with nasoseptal flap reconstruction of the skull base defect. Representative imaging and intraoperative photos are presented as well.

Results: This is a 4-year old female with a recurrent adamantinomatous craniopharyngioma treated one year prior to presentation via a right pterional craniotomy. Postoperative MRI revealed progressive recurrence with a large suprasellar cystic component measuring 24.3 mm × 25.3 mm × 19.5 mm. Due to increasing size of the tumor, worsening headaches, and vision loss, surgical resection of the recurrent tumor was planned as a joint case with neurosurgery and otolaryngology. She underwent an endoscopic endonasal transplanum/transtuberculum approach for resection of the suprasellar mass. The skull base was reconstructed with a two-layer button allograft followed by vascularized nasoseptal flap. Gross total resection was achieved. She recovered well postoperatively with no evidence of recurrence on 6 month postoperative imaging.

Conclusion: The present case adds to the literature of endoscopic endonasal approach for gross total resection of a recurrent craniopharyngioma in a pediatric patient. The endoscopic approach for suprasellar tumor resection is valid surgical option, but few of these have been described in patients as young as 4 years of age. This case also represents successful skull base repair with a nasoseptal flap in a pediatric patient. In our case, there is no evidence of recurrence 6 months postoperatively and no evidence of cerebrospinal fluid leak.

Publication History

Article published online:
12 February 2021