Editor,—Primary intestinal lymphatic obstruction with protein losing enteropathy (PLE) may be associated with other lymphatic drainage abnormalities outside the gastrointestinal tract. The pathogenesis, classification, management, and prognosis of these conditions continues to be discussed.1 2 We present a patient with PLE whose disorder has been followed up for nearly 20 years.

The patient, was aged 13 months when he originally presented to the Paediatric University Hospital Centre in Rijeka, Croatia with watery diarrhoea and generalised oedema. The laboratory features, including a low serum total protein and albumin, were consistent with PLE. He was treated with a high protein and low fat diet supplemented with medium chain triglycerides.

After initial improvement …