Shigeru Ueno1, Akihiro Fujino2, Yasuhide Morikawa3, Tadashi Iwanaka4, Yoshiaki Kinoshita5, Michio Ozeki6, Shunsuke Nosaka7, Kentaro Matsuoka8, Noriaki Usui9. 1. Department of Pediatric Surgery, Tokai University School of Medicine, 143, Shimokasuya, Isehara City, Kanagawa, Japan. ps_uenos@is.icc.u-tokai.ac.jp. 2. Department of Pediatric Surgery, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, Japan. 3. Department of Pediatric Surgery, International University of Health and Welfare, 537-3, Iguchi, Nasushiobara City, Tochigi, Japan. 4. Department of Pediatric Surgery, The University of Tokyo Faculty of Medicine, 7-3-1, Hongo, Bunkyo-ku, Tokyo, Japan. 5. Department of Pediatric Surgery, Kyushu University School of Medicine, 3-1-1, Maidashi, Higashi-ku, Fukuoka City, Japan. 6. Department of Pediatrics, Gifu University Hospital, 1-1, Yanagido, Gifu City, Japan. 7. Department of Radiology, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, Japan. 8. Department of Pathology, Dokkyo Medical University Koshigaya Hospital, 2-1-50, Minami-Koshigaya, Koshigaya City, Saitama, Japan. 9. Department of Pediatric Surgery, Osaka Women's and Children's Hospital, 840, Murodocho, Izumi City, Osaka, Japan.
Abstract
PURPOSE: 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. METHODS: Eighty-seven registered cases with mediastinal involvement were analyzed with a review of the literature. RESULTS: 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. CONCLUSIONS: 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.
PURPOSE: 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. METHODS: Eighty-seven registered cases with mediastinal involvement were analyzed with a review of the literature. RESULTS: 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. CONCLUSIONS: 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.