Lymphangioleiomyomatosis (LAM) combined with tuberous sclerosis complex (TSC) is very rare. Pleural effusion (chylothorax) is one of the most frequent complications of LAM. The therapy is controversial. Fat free diet, total parenteral feeding and/or progesteron therapy are possible conservative treatment options. If conservative treatment is uneffective surgery is indicated. Pleurodesis or ligature of the thoracic duct must be performed by thoracotomy or video-assisted thoracoscopy (VATS). Authors report a successful VATS treatment of pleural effusion in a patient with LAM combined with TSC.
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.
On 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 [Figure 1]. 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 [Figure 2]. We thought this was the reason for the chylothorax, so chose diagnostic VATS in first sit.
General 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 [Figure 3]. 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.
The chest tube was removed and the patient was discharged home 10 days later.
The final histology showed HMB45 and CD31 positivity, which squares with the diagnosis of LAM [Figure 4].
After one year she was free of complaints and chest X-ray showed normal conditions.
Lymphanigioleiomyomatosis (LAM) is generally described as a “rare disease affecting exclusively women of childbearing age”. It is characterized by pulmonary cysts (100% of the patients) at computer tomography and proliferation of abdominal smooth muscle cells at lung biopsy. Pneumothorax, pleural effusion, and chylothorax are common complications of LAM. The most frequent extrapulmonary features are renal angiomyolipomas, abdominal lesions, usually manifest or asymptomatic, small, bilateral tumors of fat attenuation in the renal cortex.
LAM occurs either in sporadic form, or in association with tuberous sclerosis complex (TSC). Both in LAM and TSC proliferation of muscle cells of immature appearence in the pulmonary lymphatics may lead to chylous effusion. Chylothorax is an uncommon but vexing clinical disorder, and may be a life-threatening condition if left untreated.[3–5]
TSC is an autosomal dominant disorder characterized classically by the triad of mental retardation, epilepsy and adenoma sebaceum. The incidence is one per 6-10000 population. The frequency of lung involvement is about 3%.
The optimal treatment of chylothorax is still controversial. Conservative management is usually recommended in first line and consists of total parenteral nutrition or low fat, medium-chain triglyceride supplemented regimens for several weeks. A possible treatment is progesteron therapy. Repeated thoracocentesis or chest tube drainage is often required during conservative treatment. Refractory chylothorax after failure of medical therapy is usually addressed for surgery.
There are several surgical procedures for the treatment of chylothorax like pleurodesis, pleurectomy, right supradiaphragmatic thoracic dust ligation and pleuro-peritoneal shunting. It has been recognized that surgical intervention to ligate the thoracic duct should be performed before complications of chylothorax have manifested. Thoracic duct ligation performed by a minimal invasive approach has not gained wide acceptance yet, though most authors report excellent results of performing surgical procedures by VATS technique.
In conclusion the case of a 56 year-old female patients with chylothorax associated with pulmonary lymphan-gioleiomyomatosis and tuberous sclerosis complex was presented. VATS treatment was safe and effective. VATS is supposed to be the first line surgical treatment for chylothorax.
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Conflict of Interest: None declared.