Surgical Treatment of Giant Cervicomediastinal Lymphatic Malformations

Andrey Ryabov ¹, Oleg Pikin ¹, Vladimir Trunov ², Dmitry Vursol ¹, Oleg Alexandrov ¹

¹ P. A. HERZEN MOSCOW ONCOLOGY RESEARCH INSTITUTE, Moscow, Russia; ² Morozovskaya Children Hospital, Moscow, Russia;

Date, time and location: 2018.05.25 15:30, Congress Hall, 2F–C

Abstract

Objective. Lymphatic malformation is a rare disorder, that affects pathologic lymphatic cysts and channels. Mediastinal extension is extremely rare, only isolated cases are described in scientific literature. Being asymptomatic, most of them are found in adulthood accidentally.

Material and methods. We report 3 cases of cervicomediastinal lymphatic malformation, in 1 adult of 42 years and 2 children of 4 years and 1 year 7 month respectively. Two cases were asymptomatic, in one patient respiratory compromise was observed due to mediastinal shift. All patients underwent computed tomography, which revealed marginated cystic malformation with extensive involvement of the neck and mediastinal portion. In all cases cytological and biochemical examination of cystic content was performed. In one case the mediastinal portion was chylous, lymphography revealed connection of the cyst with thoracic duct. Two of three cases had local recurrence after previous incomplete surgical resection.

Results. All patients underwent surgical removal, in two cases by L-shaped sternothoracotomy, in one case thoracoscopic mobilization of mediastinal component was performed with additional cervical incision. Patient with communication between thoracic duct and cyst underwent preventive thoracoscopic thoracic duct ligation. Lymphatic malformation was proven by histologic examination in all cases. One patient required thoracoscopic thoracic duct ligation due to chylothorax in postoperative course. No signs of recurrence observed in 2 year follow up.

Conclusions. The mainstay in treatment of large macrocystic lymphatic malformations is surgical removal. Incomplete excision highly predispose to local recurrence. Preoperative examination of cystic content is essential, if chylous is proved, lymphography is indicated to rule out possible communication with thoracic duct. L-shaped sternothoracotomy is surgical approach of choice in massive cervicomediastinal lesions.