VATS therapy of chylothorax caused by leiomyomatosis complicated with tuberous sclerosis complex
A 56-year-old non-smoker female patient was admitted with an 8-week-history of recurrent chylothorax, dyspnea and debilitating weakness. Meanwhile she had thoracocentesis several times. There were no valuable cell elements found during cytology; the diagnosis of chylothorax was devised. Since age 5 she had documented TSC with alterations of her limbs, liver, kidney and some minor degree of mental retardation. At age 26 she underwent a left nephrectomy; the histological features - renal angioleiomyomatosis - confirmed the diagnosis of TSC. She had gouty complaints for 7 years, too.\nOn admission the only physical findings were the absence of breath sounds and dullness during percussion on the right chest. Laboratory tests revealed hemoglobin level of 107 g/L. The chest X-ray showed complete opacification of the right hemithorax. The CT-scan showed thin-walled cysts through both lungs surrounded by normal lung parenchyma []. Most cysts were round in shape, measured from 3 to 22 mm in diameter. There was an alteration -30 mm in largest diameter-, seamed to grasp to the wall of the thoracic duct []. We thought this was the reason for the chylothorax, so chose diagnostic VATS in first sit.\nGeneral anaesthesia with selective ventilation was performed in a left lateral position. During VATS 3 litres of chylus was removed from the chest cavity. After that the thoracic duct was checked, there was no leakage found, but 10 cm above the diaphragm there was a flat alteration on the chest wall, which captured the wall of the thoracic duct too. Intraoperatively butter was given through a nasogastric tube and after 20 minutes chylus appeared near the place of the flat alteration on the thoracic duct. This was supposed to be the reason of the lymphatic leakage, so resection of this alteration and ligature of the thoracic duct was decided []. After the resection ligation of the duct was performed with stitches made with intra-corporal knotting technique. The leakage stopped. Chest tube drainage was performed in the postoperative course. Oral, but low fat content feeding was started on the first postoperative day.\nThe chest tube was removed and the patient was discharged home 10 days later.\nThe final histology showed HMB45 and CD31 positivity, which squares with the diagnosis of LAM [].\nAfter one year she was free of complaints and chest X-ray showed normal conditions.