Ueno Shigeru, Fujino Akihiro, Morikawa Yasuhide, Iwanaka Tadashi, Kinoshita Yoshiaki, Ozeki Michio, Nosaka Shunsuke, Matsuoka Kentaro, Usui Noriaki
Department of Pediatric Surgery, Tokai University School of Medicine, 143, Shimokasuya, Isehara City, Kanagawa, Japan.
Department of Pediatric Surgery, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, Japan.
Surg Today. 2018 Jul;48(7):716-725. doi: 10.1007/s00595-018-1640-0. Epub 2018 Feb 26.
Clinical guidelines on lymphatic malformation (LM) influencing the airway have been crafted in the Research Project for Intractable Diseases. We herein report an analysis of a nationwide survey of mediastinal LM and the therapeutic recommendations.
Eighty-seven registered cases with mediastinal involvement were analyzed with a review of the literature.
Mediastinal LM was located more often in the upper and anterior mediastinum and was found without any accompanying symptoms in 56/87 cases. Tracheostomy was required in 23/87 cases, mostly < 2 years of age (87%). All patients who needed tracheostomy had a lesion in contact with the airway, while only 55% of those without tracheostomy had contact. Tracheostomy tended to be placed more when the longer segment of the airway was in contact with the LM. Multimodal treatments were performed in 29 patients, but the lesions remained in most cases, and chylothorax, hemorrhaging, nerve palsy, and infections were noted as complications.
In patients with mediastinal LM, tracheostomy may be necessary, especially when the lesion is extensive and contacts the airway. Extirpation of the mediastinal LM may be the only therapeutic option, but in cases with few or no symptoms, non-surgical treatment should be considered in light of potential postoperative complications.
难治性疾病研究项目制定了关于影响气道的淋巴管畸形（LM）的临床指南。我们在此报告一项对全国范围内纵隔LM调查的分析及治疗建议。
对87例登记的纵隔受累病例进行分析，并复习相关文献。
纵隔LM更常位于上纵隔和前纵隔，87例中有56例无任何伴随症状。87例中有23例需要气管切开术，大多数患者年龄小于2岁（87%）。所有需要气管切开术的患者均有病变与气道接触，而无需气管切开术的患者中只有55%有接触。当气道较长节段与LM接触时，气管切开术的实施倾向增加。29例患者接受了多模式治疗，但大多数病例病变仍存在，乳糜胸、出血、神经麻痹和感染被记录为并发症。
对于纵隔LM患者，可能需要气管切开术，尤其是当病变广泛且与气道接触时。纵隔LM的切除可能是唯一的治疗选择，但在症状较少或无症状的情况下，鉴于潜在的术后并发症，应考虑非手术治疗。
