Galviz Tabares Brandon, Lopez Carlos E B, Lara A Vladimir, Melo Uribe Mario A, Oviedo Cañón Martín A, Ovalle Saulo H P, Fernandez H Arango
Department of Oral and Maxillofacial Surgery, Universidad El Bosque, Bogota, Colombia.
Universidad El Bosque, Oral and Maxillofacial Resident.
J Craniofac Surg. 2025 Sep 11. doi: 10.1097/SCS.0000000000011919.
Juvenile nasopharyngeal angiofibroma (JNA) is a rare, highly vascular benign tumor that predominantly affects adolescent males. Despite its benign histology, JNA may exhibit aggressive behavior, often invading adjacent anatomic regions, such as the skull base, orbit, and intracranial space, which complicates surgical management due to the risk of severe hemorrhage. The authors report the case of a 13-year-old male with recurrent JNA (Andrews IVB, Chandler IV, Radkowski IIIB) presenting with left-eye amaurosis and a 2-year history of tracheostomy. Imaging confirmed extensive tumor involvement. Preoperative embolization was performed, followed by surgical resection through a Le Fort I osteotomy combined with deep hypothermic circulatory arrest (DHCA) at 19°C for 35 minutes. Extracorporeal circulation was established through femoral arterial and venous cannulation, with controlled rewarming after tumor removal. The surgery was carried out by a multidisciplinary team including maxillofacial, neurosurgical, and cardiovascular specialists. Intraoperative cell salvage minimized the need for blood transfusion. Postoperative care in the intensive care unit was uneventful, and a secondary intervention confirmed complete hemostasis. This case illustrates the potential of combining Le Fort I osteotomy with DHCA to achieve safe and effective resection of extensive JNA, offering excellent hemostatic control and favorable clinical outcomes. Multidisciplinary collaboration and careful perioperative planning are essential in managing such complex cases.
青少年鼻咽血管纤维瘤(JNA)是一种罕见的、血管丰富的良性肿瘤,主要影响青春期男性。尽管其组织学表现为良性,但JNA可能表现出侵袭性,常侵犯相邻解剖区域,如颅底、眼眶和颅内间隙,由于严重出血风险,这使得手术治疗变得复杂。作者报告了一例13岁男性复发性JNA(Andrews IVB、Chandler IV、Radkowski IIIB)患者,表现为左眼失明和有2年气管切开史。影像学检查证实肿瘤广泛累及。术前进行了栓塞治疗,随后通过Le Fort I截骨术联合19°C深低温循环停搏(DHCA)35分钟进行手术切除。通过股动静脉插管建立体外循环,肿瘤切除后进行控制性复温。手术由包括颌面、神经外科和心血管专家在内的多学科团队进行。术中细胞回收最大限度地减少了输血需求。重症监护病房的术后护理顺利,二次干预证实完全止血。该病例说明了将Le Fort I截骨术与DHCA相结合以实现广泛JNA安全有效切除的潜力,提供了出色的止血控制和良好的临床结果。多学科协作和精心的围手术期规划对于处理此类复杂病例至关重要。