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1/119. Abdominal lymphatic dysplasia and 22q11 microdeletion.

    We report the case of a child with 22q11 microdeletion who presented with abdominal lymphatic dysplasia resulting in exsudative enteropathy. This primitive and localized lymphatic malformation is consistent with the vascular theory in the velocardiofacial syndrome. ( info)

2/119. Bilateral groin adenolymphoceles: an unusual presentation of chylous reflux.

    We report an unusual presentation of a young man with bilateral groin lymph nodal adenolymphoceles and right leg lymphedema as a manifestation of intestinal lymphangiectasia. Chylous reflux was supported by conventional and isotopic lymphography as well as by a total lipid test showing delayed triglyceride absorption 24 hours after ingestion of 60 gm of butter. After excision of groin masses in conjunction with dietary control (short-medium chain triglycerides), manual massage, pneumatic compression, and long-term wearing of a low stretch elastic garments he remains well. ( info)

3/119. Unusual vascular dysplasia presenting as an intra-abdominal catastrophe.

    A case of severe, generalised vascular dysplasia presenting as an intra-abdominal catastrophe is reported. Associated clinical features included cutis marmorata telangiectatica congenita, congenital glaucoma, hypertension, and focal fits. The case has been reported in view of its rarity and nature of presentation. ( info)

4/119. Lymphoscintigraphic manifestations of Hennekam syndrome--a case report.

    Hennekam syndrome is a rare, recently described genetic disorder in which facial anomalies and mental retardation accompany congenital lymphedema and intestinal lymphangiectasia. Several other somatic abnormalities have variously been described, as have milder degrees of lymphatic dysfunction. The authors herein describe a case of Hennekam syndrome in which the diagnostic difficulties were partially overcome by the judicious use of radionuclide scintigraphy to verify the lymphedematous component of the patient's presentation. ( info)

5/119. Two brothers with Hennekam syndrome and cerebral abnormalities.

    We report two brothers with mental retardation, lymphoedema of the limbs and facial anomalies. Hennekam et al. (Am J Med Genet 34:593-600; 1989) described four patients with identical signs and intestinal lymphangiectasia. To confirm the diagnosis of Hennekam syndrome we undertook a duodenal biopsy from the older brother which revealed intestinal lymphangiectasia. So far only one patient with Hennekam syndrome and cerebral abnormalities has been described. This patient presented with pachygyria in the parietal area. Cerebral MRI in our two cases revealed small subcortical hyperintensities in both patients and a large cystic lesion in the younger patient probably representing an old media infarction. ( info)

6/119. Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet.

    We report on a patient with protein-losing enteropathy due to primitive intestinal lymphangiectasia with an early reversal of clinical and biochemical signs and a stable late reversal of pathologic signs after treatment with a hypolipidic diet enriched with medium-chain triacylglycerols. ( info)

7/119. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.

    As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development. ( info)

8/119. Computed tomography after lymphangiography in the diagnosis of intestinal lymphangiectasia with protein-losing enteropathy in Noonan's syndrome.

    Noonan's syndrome is a rare congenital disorder that may be associated with abnormalities in the lymphatic drainage. In this case of a 21-year-old man CT after bipedal lymphangiography confirmed the diagnosis of intestinal lymphangiectasy causing protein-losing enteropathy in Noonan's syndrome by showing contrast-enhanced abnormal lymphatic vessels in the mesentery and the intestinal wall. Because of the benefit of diet in case of intestinal involvement, we recommend a thorough documentation of the lymphatic drainage with lymphangiography followed by CT, if clinical signs of lymphatic dysplasia, such as pleural effusions, lymphedema, or hypoproteinemia are present. ( info)

9/119. A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.

    Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels. ( info)

10/119. Isolated fetal ascites caused by primary lymphangiectasia: a case report.

    We present a case of isolated fetal ascites diagnosed by ultrasonography at 29 weeks' gestation. cordocentesis revealed a normal karyotype and negative viral titers. Postnatally, the diagnosis of primary lymphangiectasia was made by intestinal biopsy. To our knowledge this is the first description of prenatal manifestation of primary lymphangiectasia. ( info)
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