A 37-year-old woman diagnosed with LAM in 2003 as a result of spontaneous right haemopneumothorax requiring surgery with evacuation of the haemothorax and resection of bullous dystrophy. He was followed up as an outpatient without incident until 2009 when he presented with chylous ascites and a large retroperitoneal cystic lymphangioma was detected on abdominal computed tomography (CT) scan.
In February 2011, he was admitted for exertional dyspnoea and extensive right pleural effusion. The pleural fluid showed characteristics of chylothorax: pH 7.43; triglycerides 1,216 mg/dl; cholesterol 73 mg/dl, leukocytes 2,700 cells/μl (mononuclear 92%), protein 4.5 g/dl, LDH 142 U/l. The patient had a weight of 57 kg, height 169 cm, BMI: 19.2 and had normal blood protein and lymphocyte values.
Initially, conservative treatment with evacuating thoracentesis, fat-free diet and oral nutritional supplements (ONS) rich in medium-chain triglycerides (MCT) was performed. After one week, the patient presented respiratory worsening and a right thoracic drainage tube was placed, obtaining 2,000-4,000 ml/day of pleural fluid. Given the amount of pleural fluid, parenteral nutrition (PN) was started and treatment with octeotride was started at increasing doses up to 100 μg/8 h, which was discontinued a few days later due to digestive intolerance and severe water and electrolyte disturbance.
Despite digestive rest, pleural effusion increased and became bilateral, requiring bilateral thoracic drainage. Three pleurodesis with talc were performed, which were partially effective and did not allow removal of the drainage.
Despite treatment, the patient's nutritional status deteriorated, with a weight loss of 5 kg, hypoalbuminaemia of 1.8 g/dL and lymphopenia of 700 cells/μl.
During treatment with PN, she developed a catheter-associated infection that required withdrawal of the central line and suspension of the catheter. The fat-free oral diet was progressively reintroduced with MCT-rich ODS. The debit of both thoracic drains decreased progressively and after two and a half months of hospitalisation the patient was discharged from hospital with both thoracic drainage tubes and bowel movements every 3 days with a debit of 500 ml/day, with an albumin of 2 g/dl and lymphocytes of 700 cells/μl.
Subsequent evolution was good and the pleural drains were removed 4 and 6 months after discharge, with a progressive improvement in the patient's nutritional status. At present, there is still minimal bilateral pleural effusion, predominantly on the right, which has not changed in the last 4 months, the patient has no dyspnoea at rest although she requires home oxygen therapy and has a BMI of 18.7, with normal nutritional parameters.