Primary lymphangiomyomatosis is a benign tumour of lymphatic channels and lymph nodes, clinically manifested by chylous ascites. This disease is usually progressive and unresponsive to surgery, chemotherapy or irradiation. A case of a 36-year-old lady with chylous ascites due to underlying primary lymphangiomyomatosis is reported.
Three cases of neonatal chylous ascites (CCA) were managed in the neonatal unit, University Hospital, Kuala Lumpur, over the past 9 years. Fetal ascites and polyhydramnios were the sole abnormalities detected in all three babies by antenatal ultrasonography. They were born at 36 weeks' gestation and their birth weights ranged from 3.0 kg to 3.8 kg. All three infants had abdominal distension at birth. Milky ascitic fluid was obtained after starting enteral feedings. Analysis of the ascitic fluid revealed a raised white blood cell count (predominantly lymphocytic) and triglycerides (1.4 - 3.8 mmol/l), cholesterol (1.6 - 2.8 mmol/l), and protein levels (25 - 41 g/l). Conservative management with skimmed milk and medium-chain triglycerides in one infant and Pregestimil in another was instituted. these two infants with CCA were clinically normal when reviewed at 19 months and 3.5 years of age. The third infant had a gut malrotation and associated pyloric septum; he died from complications of a laparotomy. The literature on this rare condition is reviewed.
Chylous ascites specifically after anterior resection for rectal cancer has not been documented in the literature thus far. All previously reported postoperative chylous ascites developed in other types of malignancies and were diagnosed within a few days of surgery. However, the patient we report had symptoms 2 years after surgery. Intraoperatively, chylous ascites was found with multiple lymph node metastasis in the small bowel mesentery and retroperitoneal region. The rarity of chylous ascites after anterior resection in rectal carcinoma is documented and discussed.
A 41-year-old Malay housewife presented with recurrent chylous ascites and progressive cachexia over 17 years. A diagnosis of lymphangioleiomyomatosis (LAM) was established by laparotomy where biopsy of the liver, peritoneum and adhesions from previous surgery showed smooth muscle proliferation in the blood vessels and lymphatics. Clinically and radiologically, there was no evidence of pulmonary involvement. She was treated with dietary fat restriction and medium-chain triglycerides. This is the first case of LAM reported in Malaysia.
This is a report of 3 Chinese children with intestinal lymphangiectasia in Malaysia. Two children responded to a low fat diet and medium-chain triglyceride supplement. The third child has recurrent chylous ascites. None of the children has recurrent infections despite low CD4+ cells and low levels of IgG and IgA. Intestinal lymphangiectasia is a rare congenital disorder of the mesenteric lymphatic that leads to the obstruction of the lymphatics of the intestine and protein losing enteropathy. Restriction of dietary fat intake will usually result in remission. Recurrent chylous ascites is a problem and management can be difficult.