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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 of his symptoms, oedema, and hypoproteinaemia he relapsed at the age of 3 years, when severe oedema of his left leg was also observed. Barium radiography of the stomach and small bowel was normal, but consent for small bowel biopsy was declined. He was treated with frequent transfusions of albumin and diuretics in addition to the dietary regimen. His symptoms improved but his left leg remained swollen.
Subsequently his course was characterised by relapses with watery diarrhoea and generalised oedema, even when he apparently complied with his diet. There was persisting hypoproteinaemia, hypoalbuminaemia, hypoimmunoglobulinaemia, and lymphopenia. At 6 years small bowel radiography showed thickening of mucosal folds and hyperflocculation of barium. A biopsy specimen of the small bowel did not show dilated lymphatics, but a later specimen at 10 years, done at different levels, confirmed dilated lymphatics.
At 14 years, when he was receiving transfusions of albumin weekly, he was admitted to St Thomas’s Hospital, London for reinvestigation. A51Cr labelled albumin study suggested PLE (18% recovery), and small bowel radiography showed an abnormal mucosal pattern throughout the entire small bowel. Lymphangiography showed normal right sided iliac and intra-abdominal lymphatics, a normal thoracic duct, but significant lymphatic obliteration in both legs.
In view of the severe and uncontrollable protein loss, surgical resection of the small bowel was carried out. At laparotomy the small bowel was of normal size, but the mesenteric lymph nodes were hard and there were many dilated lymphatics on the surface of the bowel. Injection of a dye (blue violet) into one of these lymphatics showed the dye running across the surface of the bowel with none of it transversing the mesenteric lymphatics or reaching the mesenteric lymph nodes. The small bowel was 671 cm long. Approximately 305 cm, which was palpably thicker than the rest, was resected and an end to end anastomosis was carried out. When the resected bowel was opened up it had the typical appearance of lymphatic obstruction, a white cobblestone appearance.
After surgery, symptoms of PLE abated, intestinal protein loss was reduced, and his serum proteins and immunoglobulins stabilised to a level just below the normal range. He was last reviewed in January 1996 at the age of 20 years when he was on a normal diet, and growing normally. He remained free of symptoms, and there was no generalised oedema. However, his left leg was grossly swollen (elephantiasis) and also his right leg was swollen.
This patient illustrates that in resistant cases of PLE small bowel resection can control enteric protein loss and the resulting symptoms. However, the prognosis is uncertain, and we need to learn more about the pathogenesis and evolution of the underlying lymphatic abnormality in this group of disorders.3