PDF(Pubmed)
Abstract：
A 27-year-old female with a past medical history of neurofibromatosis type 1 (NF1) presented with obstructive hydrocephalus due to a thalamic tumor. The neurosurgery team attempted an operative intervention twice, but both times, the patient experienced a hypertensive emergency and unstable supraventricular tachycardia upon induction of anesthesia. After the second failed surgery, a pheochromocytoma was suspected and the workup demonstrated a left pheochromocytoma. Pheochromocytomas in patients with NF1 are known to be more dangerous and labile, requiring in-depth discussion and preparation by personnel in endocrinology, anesthesia, neurosurgery, and minimally invasive surgery. Once the patient was stable and deemed fit for surgery, a robotic adrenalectomy followed by ventriculoperitoneal shunt placement began. After induction of anesthesia, the patient went into hypertensive emergency again. However, the anesthesia team was prepared and quickly resolved this with medical therapy. Minimally invasive surgeons had the patient\'s live vitals displayed on their robotic monitors to increase their awareness of patient hemodynamics. This provided live feedback on the surgeons\' effect as they removed the pheochromocytoma. Surgeons also performed vein clamping to preemptively see the effects of adrenalectomy. When vein clamping demonstrated safety to proceed, adrenalectomy was completed without complication. This case not only highlights the rare pathology of a woman with NF1 with pheochromocytoma, but it also demonstrates the importance of preparedness and communication among a multidisciplinary team in complex cases to ensure a successful outcome. Novel techniques were also used in performing a robotic-assisted adrenalectomy that can aid other adrenal surgeons.
摘要：
一名27岁的女性，既往有1型神经纤维瘤病（NF1）病史，因丘脑肿瘤而出现阻塞性脑积水。神经外科团队两次尝试手术干预，但两次,患者在麻醉诱导后出现高血压急症和不稳定型室上性心动过速。第二次手术失败后,怀疑有嗜铬细胞瘤，检查显示有左嗜铬细胞瘤。已知NF1患者的嗜铬细胞瘤更危险和不稳定，需要内分泌科人员的深入讨论和准备，麻醉,神经外科,和微创手术。一旦患者稳定并认为适合手术，开始进行机器人肾上腺切除术，然后进行脑室-腹腔分流术。麻醉诱导后,病人再次进入高血压急症。然而,麻醉小组做好了准备，并通过药物治疗迅速解决了这个问题。微创外科医生在他们的机器人监视器上显示患者的生命体征，以提高他们对患者血流动力学的认识。这为外科医生切除嗜铬细胞瘤时的效果提供了实时反馈。外科医生还进行了静脉钳夹以预先观察肾上腺切除术的效果。当静脉夹紧证明安全进行时，肾上腺切除术完成无并发症。这种情况不仅突出了NF1伴嗜铬细胞瘤的女性的罕见病理，但它也表明了在复杂情况下，多学科团队之间做好准备和沟通的重要性，以确保成功的结果。新技术也用于执行机器人辅助肾上腺切除术，可以帮助其他肾上腺外科医生。
