We present the case of a 35-year-old female patient. She was referred to our unit after her third hospital admission in the previous 18 months for recurrent chylous ascites. With no previous history of interest, she was admitted for the first time with ascites and mild oedema in the lower limbs for a month and a half, and underwent an exploratory laparotomy on suspicion of ovarian neoplasia. Twelve litres of ascitic fluid with chylous characteristics were evacuated, with no ovarian abnormalities or peritoneal carcinomatosis.
She was discharged with diuretic treatment with spironolactone 100 mg and prednisone 40 mg in descending doses, after 5 months she was readmitted, and 9 litres of chylous fluid were extracted by evacuating paracentesis. On a new admission 6 months later, during which time the symptoms had progressively reappeared, 14 more litres of peritoneal fluid were evacuated.
Throughout his hospital stay, the following complementary tests were performed: blood count, with discrete leukocytosis and normal formula, iron study without alterations; biochemical profile, with hypoproteinaemia and hypoalbuminaemia, hypocalcaemia. Celiac profile, HBsAg, HCV-Ac, tumour markers and Mantoux tests were negative. ASLO, C-reactive protein and serum a-1 antitrypsin levels were normal. Thyroid function study was normal. The serum proteinogram showed decreased values of albumin and gamma globulins.
The ascitic fluid extracted showed exudate characteristics and a chylous appearance. His culture was negative.
Thoracoabdominal CT showed normal thorax, ascites in all peritoneal compartments, oedema in small bowel loops. Abdominal ultrasound showed abundant peritoneal ascitic fluid, with normal liver, portal, great vessels, gall bladder and spleen. Fibroenteroscopy, reaching the cecum, showed from the bulb to the sections of jejunum explored, irregularity in the villous pattern, oedema of folds and numerous punctate lymphangiectasias. Ileoscopy was performed and the terminal ileum showed an extremely irregular mucosa, with whitish, friable plaques on biopsy. The capsuloendoscopy showed diffuse involvement of the small intestine, with punctiform "millet grain" formations and oedematous and congestive folds. Biopsy of the intestinal mucosa was reported as lymphoid follicular hyperplasia, focal lymphangiectasia; and gastric, duodenal and jejunal mucosa biopsy showed no significant alterations.
Treatment was started with diuretics and intravenous albumin, presenting a favourable clinical evolution, being discharged with the diagnosis of primary intestinal lymphangiectasia, with home treatment with Furosemide 40 mg, Spironolactone 100 mg and Prednisone 30 mg, being referred to the Clinical Nutrition and Dietetics Unit for assessment and follow-up.
Examination in the consulting room showed a height of 1.65 m, weight 53.5 kg, BMI 19.3 kg/m2, after evacuation of 14 L of ascitic fluid during her admission; subjectively she was thinner than before the onset of symptoms. Skin pallor, long-lined fingers, discrete symmetrical malleolar oedema. The rest of the physical examination was normal.
No usual gastrointestinal symptoms, except for 1-2 bowel movements per day with a somewhat greasy appearance. She has a varied diet, always avoiding excessively fatty foods due to intolerance. She denies toxic habits. Nuligesta.
Laboratory tests showed serum values of total protein 3.4 g/dL, albumin 2.1 g/dL, calcium 7.3 mg/dL. The rest of the basic biochemistry, lipidogram and haemogram were normal.
We decided to start dietary treatment. A personalised diet of 2,200 kcal in 24 hours and the following distribution of nutrients was established: 52% carbohydrates, 30% lipids, 18% proteins. The intake of fats from food is restricted, and lipids are provided in the form of MCT oil, using 85 ml per day, introduced into the diet progressively to avoid intolerances. The protein intake of the diet is supplemented with 400 ml of hyperprotein formula for enteral nutrition and 20 g of powdered protein module. A vitamin-mineral supplement is also added.
The patient is cooperative and shows strict compliance with the recommended guidelines, with good adaptation and excellent tolerance.
After 11 months of follow-up, she has achieved weight gain, with a weight of 59.3 kg and BMI 21.4. The analytical parameters have improved significantly, with serum values of total protein 5.2 g/L, albumin 3.7 g/L, calcium 8.5 mg/dL. There are no gastrointestinal symptoms. Waist circumference was 78 cm, and no free fluid was observed in the abdomen on abdominal ultrasound. She has not presented new episodes of ascites and has not required hospital admission during this period.