TY - JOUR
T1 - Treatment of mediastinal lymphatic malformation in children
T2 - an analysis of a nationwide survey in Japan
AU - Ueno, Shigeru
AU - Fujino, Akihiro
AU - Morikawa, Yasuhide
AU - Iwanaka, Tadashi
AU - Kinoshita, Yoshiaki
AU - Ozeki, Michio
AU - Nosaka, Shunsuke
AU - Matsuoka, Kentaro
AU - Usui, Noriaki
N1 - Funding Information:
Acknowledgements This study was supported by a grant for the Research Project for Intractable Diseases conducted by Ministry of Health, Labor and Welfare in Japan (Grant No. 26070201, H27-013) received by N.U., and by the grant for Practical Research Project for Rare/Intractable Diseases Japan Agency conducted by Medical Research and Development (AMED) (Grant No. 26310401) received by A.F.
Funding Information:
This study was supported by a grant for the Research Project for Intractable Diseases conducted by Ministry of Health, Labor and Welfare in Japan (Grant No. 26070201, H27-013) received by N.U., and by the grant for Practical Research Project for Rare/Intractable Diseases Japan Agency conducted by Medical Research and Development (AMED) (Grant No. 26310401) received by A.F. The authors declare that they have no conflict of interest.
Publisher Copyright:
© 2018, Springer Nature Singapore Pte Ltd.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2018/7/1
Y1 - 2018/7/1
N2 - 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.
AB - 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.
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U2 - 10.1007/s00595-018-1640-0
DO - 10.1007/s00595-018-1640-0
M3 - Article
AN - SCOPUS:85042555349
VL - 48
SP - 716
EP - 725
JO - Surgery Today
JF - Surgery Today
SN - 0941-1291
IS - 7
ER -