Combined lymphatic malformations in children: a modern look at the problem

Abstract

Objective. To improve the informativeness of the diagnosis and the treatment outcomes of combined LMs in children.
Methods. The study included 14 inpatient children with combined LMs aged from 3 month to 15 years within the time period from December 2010 to March 2017. The follow-up period was from 6 to 76 month. The diagnostic methods were US, MRI, CT angiography.
Results. Among 126 patients with LM, combined forms were diagnosed in 14 (11.11%) children, in particular, Gorham-Stout disease (n=1), generalized lymphatic anomaly (n=3), central conducting lymphatic anomaly (n=3), CLOVES syndrome (n=7). In 14 patients diagnosed 27 complications related to lymphatic loss, coagulopathy and sepsis. It was performed from 1 to 6 surgical interventions, on average 2.78±1.47 per patient. The type of performed operations: thoracocentesis (n=4), puncture and drainage of the pericardium (n=4), mediastinotomy, drainage of mediastinum (n=1), thoracotomy, removal of LM (n=3), laparoscopic removal of LM (n=1), laparoscopic splenectomy (n=1), laparocentesis (n=3), pleurodesis (n=2), debulking procedures (n=20). Two patients died, mortality is 14.29%.
Conclusions. Combined LMs are characterized by progressive course and high risk of complications. The method of radical treatment of combined LMs is unknown.