Kayawake Hidenao, Motoyama Hideki, Date Hiroshi
Department of Thoracic Surgery, Kyoto University, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan.
Gen Thorac Cardiovasc Surg. 2020 Jan;68(1):74-76. doi: 10.1007/s11748-019-01098-3. Epub 2019 Mar 4.
Scimitar syndrome is a rare congenital anomaly in which the right lung is hypoplastic and a scimitar vein is draining into the inferior vena cava. It is known to be sometimes complicated with pulmonary sequestration. However, a right single pulmonary vein draining normally into the left atrium is rare. Here, we report a case of variant scimitar syndrome complicated by intralobar pulmonary sequestration containing an adenocarcinoma. In this case, the aberrant arteries from the abdominal aorta were ligated and the pulmonary sequestration containing adenocarcinoma was completely removed through wedge resection. Twenty months after the surgery, the patient was alive without cancer recurrence. An elaborate preoperative plan is important for safety in cases with vascular malformation.
弯刀综合征是一种罕见的先天性异常，其特征为右肺发育不全且弯刀状静脉引流至下腔静脉。已知该综合征有时会并发肺隔离症。然而，一条正常引流至左心房的右单支肺静脉较为罕见。在此，我们报告一例变异型弯刀综合征并发叶内型肺隔离症并伴有腺癌的病例。在该病例中，结扎了来自腹主动脉的异常动脉，并通过楔形切除术将含有腺癌的肺隔离症完全切除。术后20个月，患者存活且无癌症复发。对于伴有血管畸形的病例，精心的术前规划对手术安全很重要。
