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Primary intestinal lymphangiectasia (PIL), first described in 1961, is a rare disease of childhood. Oedema, hypoproteinaemia and diarrhoea are characteristic symptoms. Bioptic demonstration of dilated lymphatic capillary vessels in intestinal villi and increased intestinal protein loss are diagnostic. Two patients successfully treated with a low fat diet, containing medium chain triglycerides (MCT) are reported.
The intestinal lymphangiectasia can be proved enteroscopically, laparoscopically as well as histologically after preceding aimed or unaimed biopsy. In 2,250 fibre-endoscopic examinations of the upper gastrointestinal tract we found 54 times endoscopically and 48 times also histologically a lymphangiectasia. Of these 54 patients 9 had an advanced carcinoma of the stomach. The evoking causes of the lymphangiectasia are discussed. When an intestinal lymphagniectasia is diagnosed it should always be sought for an evoking cause and last not least should be thought of a primary or secondary neoformation in the region of the epigastrium.
INTRODUCTION: Primary intestinal lymphangiectasias are often associated with lymphoedema. OBSERVATION: The diagnosis was performed at 4 months when Maxime presented with lymphoedema, diarrhea, hypoprotidemia and hypolipemia. Duodenum biopsies revealed intestinal lymphangiectasias. An hyperprotidic and low fat diet, medium chain triglyceride-supplemented and an elastic contention allowed a decline of the oedemas. DISCUSSION: We report one case of Waldman's disease. It shows very well the typical circumstances of diagnosis in this disease and the two types of oedema (lymphoedema and hypoprotidic oedema).
HISTORY: A 32-year-old man had ten years previously undergone several laparotomies for recurrent ileus of the small intestine. They revealed severe intestinal oedema and histology showed hyperplasia of the lymphatic system but the aetiology was unclear. After a 10-year interval free of symptoms he presented with marked hypoproteinaemic oedema and exudative enteropathy the cause of which was to be clarified by exploratory laparotomy with excision of lymph nodes and a small section of small intestine. INVESTIGATIONS: Histology revealed intestinal lymphangiectasis with partly hyperplastic lymphoid tissue. Lymphangiography demonstrated several lymph nodes in the region of the aortic bifurcation and renal vessels with a central filling defect. It is thought likely that obstruction to lymphatic flow in this region resulted in oedema of the intestinal wall which caused the recurrent episodes of ileus. DIAGNOSIS: Retrospectively it is assumed that primary intestinal lymphangiectasis was responsible for the initial manifestation of an acute abdomen. For treatment of hypoproteiaemia with human albuminea prot system was implanted. The course was complicated by recurrent inflammation and thrombosis of the port catheter. Because of immune deficiency risk of carcinoma is high in primary intestinal lymphangiectasis. CONCLUSION: Primary intestinal lymphangiectasis, even though a rare condition, should be considered in the differential diagnosis of otherwise unclear acute abdomen.
The authors report the observation of a primary intestinal lymphangiectasy diagnosed on a young girl sent for isolated edema of her inferior members, recently appeared. Clinical examination was normal. Biology found a low protein rate at 33 g/l and a low lymph rate : 183 L/mm3. Hepatic and renal records were normal. Test to marked albumin asserted the exsudative enteropathy with a fecal radio-activity of 3.6% (N 1%). Biopsy of the small intestines set out lymphangiectasies of the intestinal mucosa. Referring to this observation and to literature date, the authors realise a clinical and physiopathological analysis of Waldmann's disease or primary intestinal lymphangiectasy.
A patient with a history of familial intestinal lymphangiectasis and exudative enteropathy was observed during 2 consecutive pregnancies. Normal pregnancy is compatible with the condition and pregnancy need not aggravate the disease.
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A case of high grade malignant lymphoma in a young woman with congenital disseminated lymphangiectasis, exudative enteropathy and immunodeficiency is reported. Lymphoid malignancies occur with a high frequency in the course of this rare disease, possibly as a consequence of immunodeficiency. In this case, a selective defect of the OKT4+ peripheral blood lymphocyte subset has been shown. The relationships between congenital lymphangiectasis, immunodeficiency and malignant lymphoma are discussed.
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