[Lymph-cysts of the duodenal mucosa ].
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The case of a woman with SLE and protein-losing enteropathy secondary to intestinal lymphangiectasia is described. Corticosteroid therapy improved all clinical symptoms, laboratorial findings and intestinal histological alterations.
A case of a 5 1/2 year old girl with intestinal lymphangiectasia is described. The diagnosis was established by endoscopic biopsies. The value of endoscopy is discussed and, clinical aspects and management difficulties are stressed.
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The development of generalized edema is the consequence of a decreased level of proteins in the plasma, due to a deficient intake or exaggerated loss. The gastrointestinal system may cause this deficiency at three different levels. We report three clinical cases of protein losing enteropathy at different locations, such as: giant hypertrophy of the gastric mucosa, intestinal lymphangiectasia and Crohn's disease. In each case we tried to demonstrate the sites that could mainly be affected in the gastrointestinal system and taking into consideration that these are rare entities, we made a brief description of them.
It is reported on 4 patients with intrahilary renal cysts. After references to etiology and difficulties in the diagnosis the case reports are given. In 3 out of 4 observations the cases in question were histologically dilations of the lymphatic vessels.
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A case of lymphangiomatosis of the cervical spine with progressive instability of the cervical spine despite anterior and posterior fusion is presented and discussed.
The pathogenesis of intestinal lymphangiectasia and mechanism of enteric loss of serum protein has been discussed from the pathologic and roentgenologic viewpoints of the two patients with this disease. Enteric loss of serum protein was attributed to exudation of lymph from ruptured lymphatic vessels, resulting from non-specific multiple ulcers of the small intestine in one patient and malrotation of the gut in another.
Two children with primary intestinal lymphangiectasia (PIL) are presented, both of whom had been prescribed an MCT supplemented diet low in long chain fatty acids. The hypoproteinemic edema improved in one patient, but persisted in the other. Following several years of dietary therapy, both subjects were re-evaluated, and a duodenoscopy and radiography of the digestive tract were performed. The duodenoscopic procedure evidenced two types of lymphangiectatic plaques on the surface of the duodenal mucosa: one form had a diameter of less than 1 mm, while the other exceeded 3 mm. The smaller lesions were seen in the child with the more favorable clinical course, whereas both types were observed in the other patient. Radiological examination disclosed the typical anomalies of intestinal lymphangiectasia only in the subject who had not responded to the dietary regimen. These results suggest that endoscopy affords a more precise assessment of the anatomic injury and could play an important role in formulating an accurate clinical diagnosis.
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We describe an isolated recurrent non-inflammatory tumorous swelling of the supraclavicular fossa in four premenopausal women. Ultrasonography, magnetic resonance imaging and computer tomography of the neck each suggested an inhomogeneous mass consistent with "lymphangioma." In each patient the clinical course and histopathologic findings suggested that the swellings were due to chronic localized lymph stasis with subsequent lymphangiectasia, possibly initiated by intermittent obstruction of the juncture of the thoracic or right lymph duct with the internal jugular vein. Enlargement may have been hormonally triggered by estrogens as each woman was taking oral contraceptive pills at the onset of the disease. To characterize this unique entity, we have termed the disorder benign supraclavicular tumorous lymphangiectasia.