Sun Hao-Rui, Song Hai-Bo, Zhang Yan-Zi, Lin Ke, Liu Jin
Department of Anesthesiology, West China Hospital, Sichuan University, No. 37 Guo Xue Ave., Chengdu, 610041, Sichuan, People's Republic of China.
Can J Anaesth. 2014 May;61(5):446-51. doi: 10.1007/s12630-014-0130-0. Epub 2014 Mar 1.
Intravenous leiomyomatosis is a rare disorder characterized by benign smooth-muscle tumours, termed leiomyomas, which originate from uterine leiomyomas or pelvic veins. Tumours may extend into the right-sided heart chambers, termed intracardiac leiomyomatosis (ICLM), and may be potentially life-threatening due to mechanical interference with cardiac structures or pulmonary arteries. While surgical excision is the optimal therapy, incomplete retrieval of a tumour or fatal retroperitoneal hemorrhage may occur. We present a case where intraoperative transesophageal ultrasound (TEU) guided complete removal of an intracardiac leiomyoma in a single-stage surgery solely through the right atrium without vein injury.
A 46-yr-old female patient presented with a two-week history of exertional dyspnea, palpitations, and syncope. Preoperative imaging modalities revealed a continuous solid mass extending from the inferior vena cava (IVC) into the right atrium, and the patient subsequently underwent open heart surgery for tumour removal and definitive diagnosis. A systematic intraoperative TEU examination performed before resection showed that the serpentine tumour was free from any attachment to the IVC and the heart. Furthermore, the diameter of the intracardiac end of the tumour was wider than that of the IVC. Given these findings, the surgeons carefully drew the cord-like tumour out of the right atrium under close TEU monitoring without vein injury. Post-extraction TEU examination showed complete removal of the tumour. Microscopic examination of the specimen confirmed the diagnosis of intravenous leiomyomatosis.
For cases with ICLM, intraoperative TEU plays a significant role in helping to plan the surgical approach, monitor the movement of the tumour and the IVC during the extraction, and assess the completeness of tumour resection.
静脉内平滑肌瘤病是一种罕见疾病，其特征为良性平滑肌肿瘤，即平滑肌瘤，起源于子宫平滑肌瘤或盆腔静脉。肿瘤可延伸至右侧心腔，即心内平滑肌瘤病（ICLM），由于对心脏结构或肺动脉的机械干扰，可能具有潜在生命危险。虽然手术切除是最佳治疗方法，但可能会出现肿瘤切除不完全或致命的腹膜后出血。我们报告一例病例，术中经食管超声（TEU）引导下，仅通过右心房在单阶段手术中完整切除心内平滑肌瘤，且未损伤静脉。
一名46岁女性患者，有两周劳力性呼吸困难、心悸和晕厥病史。术前影像学检查显示一个连续的实性肿块从下腔静脉（IVC）延伸至右心房，患者随后接受心脏直视手术以切除肿瘤并明确诊断。切除前进行的系统性术中TEU检查显示，蜿蜒的肿瘤与IVC和心脏无任何附着。此外，肿瘤心内端的直径比IVC宽。鉴于这些发现，外科医生在密切的TEU监测下小心地将条索状肿瘤从右心房中抽出，未损伤静脉。抽出后TEU检查显示肿瘤已完整切除。对标本的显微镜检查证实为静脉内平滑肌瘤病。
对于ICLM病例，术中TEU在帮助规划手术入路、在抽出过程中监测肿瘤和IVC的移动以及评估肿瘤切除的完整性方面发挥着重要作用。
