A 37-year-old woman diagnosed with LAM in 2003 due to spontaneous right hemopneumothorax that required surgery with evacuation of the hemothorax and resection of bullous dystrophy.
Outpatient follow-up was uneventful until 2009 presented chylous ascites and a large retroperitoneal cystic lymphangioma was detected in abdominal computed tomography (CAT).
In February 2011, the patient was admitted due to dyspnea on exertion and extensive right pleural effusion.
Pleural fluid showed characteristics of chylothorax: pH 7.43; triglycerides 1.216 mg/dl; cholesterol 73 mg/dl, leukocytes 2700 cells/μl (mononuclear 92%), proteins 142 g/dl, L
The patient had a weight of 57 kg, height 169 cm, BMI: 19.2 and normal protein and lymphocyte values in blood.
Initially conservative treatment was performed with evacuating emphasizeesis, fat diet and oral nutritional supplements rich in medium chain triglycerides (MCT).
After one week the patient presented respiratory worsening so a right thoracic drainage tube was placed, obtaining 2,000-4,000 ml/day of pleural fluid.
Given the amount of pleural output, parenteral nutrition (PN) was initiated and treatment with increasing doses of octeotride up to 100 μg/8 h was instituted, which was suspended after a few days due to severe digestive intolerance and electrolyte imbalance.
Despite digestive rest, pleural effusion increased bilaterally and required bilateral chest drainage.
Three talc pleurodesis were performed, which were partially effective and did not allow drainage to be removed.
Despite the established treatment the patient presented a deterioration of her nutritional status with loss of 5 kg of weight, hypoalbuminemia of 1.8 g/dl and lymphopenia of 700 cells/μl.
During treatment with PN, the patient developed catheter-associated infection associated with central line removal and its discontinuation.
A progressive reintroduction of the fat-rich oral diet with SNO was performed.
The output of both thoracic drains decreased progressively, and after two and a half months of admission the patient was discharged from the hospital with 700 lymphocytes/day with chest drainage tubes and evacuations every 3 days with a debit of 500μmL/day.
The subsequent evolution was good and pleural drainage could be removed 4 and 6 months after discharge with a progressive improvement in the nutritional status of the patient.
At present, there is still minimal bilateral pleural effusion of right predominance that has not changed in the last 4 months, the patient does not have dyspnea at rest although she needs normal home therapy and has a BMI of 18.7, with nutritional parameters.
