Anesthetic Challenges in Debulking Surgery of Massive Plexiform Neurofibroma With Neurofibromatosis Type 1: A Case Report of Intraoperative Generalized Involuntary Movements and Severe Blood Loss

1型神经纤维瘤病合并巨大丛状神经纤维瘤减瘤手术麻醉挑战:一例术中全身不自主运动和严重失血病例报告

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Abstract

Neurofibromatosis type 1 is a multisystem genetic disorder commonly associated with plexiform neurofibromas, benign but highly vascular tumors that present significant perioperative challenges. Surgical debulking of plexiform neurofibromas is often associated with difficult anesthetic management due to tumor vascularity, hemorrhage risk, airway considerations, and the need for intraoperative neurophysiological monitoring. We report the case of a 48-year-old man with neurofibromatosis type 1 who underwent debulking of a large right thigh plexiform neurofibroma under general anesthesia without neuromuscular blockade. The intraoperative course was notable for significant blood loss requiring transfusion and the development of transient generalized involuntary movements temporally associated with deep tumor manipulation. Despite these challenges, the procedure was successfully completed. This case highlights the anesthetic complexity of massive plexiform neurofibroma resection and underscores the importance of maintaining a broad differential diagnosis for intraoperative seizure-like phenomena in patients without epilepsy or intracranial pathology. Meticulous planning, multidisciplinary communication, and individualized anesthetic strategies are essential to optimize safety in complex neurofibromatosis type 1-related surgical procedures.

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