VATS therapy of chylothorax caused by leiomyomatosis complicated with tuberous sclerosis complex

Abstract

Lymphangioleiomyomatosis with tuberous sclerosis complex is a rare disease. One of the most frequent complications of lymphangioleiomyomatosis is pleural effusion (chylothorax) wich can be treated with the use of VATS. Authors report a case of pulmonary lymphangioleiomyomatosis in a 56-year-old female patient with tuberous sclerosis complex with an 8-week history of recurrent chylothorax, dyspnea and debilitating weakness. By CT scan a flat tissue proliferation was seen in the site of the thoracic duct and it was supposed to be the reason for the pleural effusion. A VATS resection of this laesion and ligation of the thoracic duct was performed successfully. Chylothorax is often associated with pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis combined with tuberous sclerosis complex is extremely rare. In case of chylothorax VATS treatment is successful and may be the first choice.

Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review

“…Figure 1 presents a flowchart showing the complete breakdown of the steps used in the identification of appropriate studies [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,…”

“…Therefore, some authors considered it the standard treatment for LAM [5]. Other treatment modalities have been decortication/pleurectomy (11 patients), chest tube (32 patients), ligation of the thoracic duct (20 patients), and medroxyprogesterone (23 patients) with favorable responses in 9 (81.8%) patients [10,13,45,57,63], 8 (25%) patients [30,33,57,63,87], 12 (60%) patients [10,12,15,24,27,57,70,73,93,96,99], and 13 (56.5%) patients [33,40,42,45,48,49,57,63,66,74], respectively. However, these, possibly very optimistic, results should be interpreted with caution, since in the majority of these studies they were associated with various treatments, and sometimes it is difficult to know which treatment led to the favorable outcome.…”