Nagasaka Satoshi, Kina Satsuki, Arimoto Yoshihito, Yokote Fumi, Uchida Tsuyoshi, Matsubara Hirochika
General Thoracic Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.
Second Department of Surgery, University of Yamanashi Hospital, 1110, Shimokato, Chuo-shi, Yamanashi, 409-3898, Japan.
Surg Case Rep. 2017 Dec;3(1):47. doi: 10.1186/s40792-017-0321-3. Epub 2017 Mar 21.
Extralobar sequestrations constitute a rare form of congenital pulmonary airway malformations that are difficult to diagnose. Here, we report a rare case of a localized extralobar sequestration in the right superior portion of the mediastinum accompanied by congenital cystic adenomatoid malformation.A 19-year-old man presented with a right upper mediastinal mass that was detected using chest radiography, had a history of left spontaneous pneumothorax, and had undergone a bullectomy 4 years previously.The initial diagnosis included a mature teratoma and a bronchogenic cyst in the mediastinum; however, the presence of a cystic mass in the right upper lobe of the lung prompted further examination. A preoperative diagnosis of extralobular sequestration was finally determined using contrast-enhanced computed tomography. The aberrant artery was connected to the brachiocephalic artery, and its drainage vein was connected to the right pulmonary artery, uniquely behind the pericardium. Despite the unique location, right mediastinal extralobular sequestration with a congenital cystic adenomatoid malformation in the right upper lobe was confirmed pathologically. Examination of contrast-enhanced chest computed tomography (CT) and three-dimensional computed tomography images enabled a correct diagnosis. It is very important for surgeons to correctly diagnose and identify an aberrant artery and drainage vein to prevent uncontrolled hemorrhage.
肺叶外型隔离症是一种罕见的先天性肺气道畸形，难以诊断。在此，我们报告一例罕见的位于纵隔右上部分的局限性肺叶外型隔离症，伴有先天性囊性腺瘤样畸形。一名19岁男性因胸部X线检查发现右上纵隔肿块就诊，有左侧自发性气胸病史，4年前曾行肺大疱切除术。初步诊断包括纵隔成熟畸胎瘤和支气管源性囊肿；然而，右肺上叶囊性肿块的存在促使进一步检查。最终通过增强计算机断层扫描确定术前诊断为肺叶外型隔离症。异常动脉与头臂动脉相连，其引流静脉与右肺动脉相连，位置独特，位于心包后方。尽管位置独特，但经病理证实为右纵隔肺叶外型隔离症伴右上叶先天性囊性腺瘤样畸形。增强胸部计算机断层扫描（CT）和三维计算机断层扫描图像检查有助于正确诊断。对于外科医生来说，正确诊断并识别异常动脉和引流静脉以防止术中大出血非常重要。
