Lymphatic Malformation Presenting as Recurrent Pleural Effusion and Ascites

Abstract

Background: Chylothorax is defined as the accumulation of chyle in the pleural space and is characterized by elevated triglyceride content in pleural fluid >110 mg/dl. Chylous ascites is the accumulation of chyle in peritoneal fluid characterized by triglyceride levels >200 mg/dL. Causes can be traumatic or nontraumatic; portal or nonportal; and congenital or acquired (inflammatory, postoperative, malignant, or infectious). Lymph duct abnormalities are a rare cause of concurrent occurrence of chylothorax with chylous ascites. Clinical Description: We report a 4½-year-old girl who initially presented a fever and was diagnosed to have right-sided pleural effusion. Since no other focus was identified, she was treated as a case of tubercular pleural effusion, following which her symptoms settled transiently. She thereafter had a recurrence of her symptoms in association with ascites and was managed as a case of relapse of tuberculosis (TB) with category 2 anti-tubercular therapy. Since her symptoms did not resolve, she was referred to our hospital for further management. Management: Clinical reasoning based on history and examination coupled with investigations ruled out cardiac, renal, hepatic, malabsorption, or nutritional pathologies of recurrent effusions. TB was ruled out. A lymphatic malformation was suspected. Therapeutic cum diagnostic paracentesis was done in a fed state, which indicated a chylous nature by the milky appearance and suggestive cytology and biochemistry. Lymphoscintigraphy confirmed the presence of a lymphatic duct abnormality. The child was managed with diet modifications, following which she improved within a week. Conclusions: The approach to recurrent effusions without any focus should be logical and sequential, as described above, to exclude the aforementioned common conditions. If workup for TB is repeatedly negative other less likely causes should be considered.