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Characteristic endoscopic features of intestinal lymphangiectasia: correlation with histological findings.

Six patients with histologically proven intestinal lymphangiectasia were evaluated for the endoscopic features. White villi and/or spots, previously reported as endoscopic findings, were seen. Furthermore, white nodules and submucosal elevations with or without white mucosa were observed. All patients demonstrated the appearance of submucosal elevations. The four characteristic endoscopic features were correlated with histological findings. It is considered that these endoscopic features are of value for the diagnosis of intestinal lymphangiectasia in patients with protein-losing enteropathy.

Adolescent↗

Cystic renal lymphangiectasia: a distinctive clinicopathologic entity in the pediatric age group.

This report describes the features of unilateral cystic renal lymphangiectasia in a 2-year-old child who presented with hypertension, massive ascites, a left flank mass, and no evidence of familial renal cystic disease. The child became normotensive and is now asymptomatic more than 3 years after surgery. The clinical presentation and diffuse pathologic involvement are similar to findings for the few pediatric patients with cystic lymphangiectasia described in the literature and appear distinct from the more localized form of the disease seen in adults.

Child, Preschool↗

Lymphangiectasia of the uterus.

A 27-year-old woman, gravida 2, para 2, who presented with a 5-year history of dysfunctional uterine bleeding following a terminated pregnancy is described. Hysterosalpingography suggested a loss of muscle tone and a mass. Pathologic examination revealed a prominent vascular system consistent with diffuse lymphangiectasia.

Adult↗

[Renal peripelvic multicystic lymphangiectasia: is echographic diagnosis possible?].

We describe the role of US, in the diagnosis of LMPR and in differentiating LMPR from other renal disease, such as hydronephrosis and parapelvic cysts. In 10 patients mild to moderate hydronephrosis showed at the US, bilateral in 8 cases, was not confirmed at IVP and CT scan evaluation. Instead, compression of the collecting system by multiple cysts arising from the renal sinus was revealed by CT scan in 8 cases and by IVP in 2. At the U.S. the profile of the calices appeared irregular, differing from the features of hydronephrosis; furthermore calices were adjacent each other, separated only by a thin membrane. All patients were asymptomatic. The examination of the cystic liquor and wall, obtained percutaneously or during surgical procedures, showed the lymphatic origin of them. We cannot provide definitive data regarding how to differentiate LMPR from hydronephrosis at U.S.. In asymptomatic patients the U.S. evidence of dilated calices with irregular profile and thin membrane separating each other, can strongly suggest the diagnosis of LMPR.

Diagnosis, Differential↗

Expansion of the phenotype in Hennekam syndrome: a case with new manifestations.

We report on a female with lymphedema, facial anomalies, intestinal lymphangiectasia, and moderate mental retardation consistent with the diagnosis of Hennekam syndrome. In addition, she had a number of other anomalies not previously described in this autosomal recessive disorder, including a congenital heart defect, atretic ear canals, vesicoureteral reflux, and rectal prolapse.

Abnormalities, Multiple↗

Congenital lymphangiectasia and atopy.

Intestinal lymphangiectasia, a disease characterized by excessive intestinal protein loss, asymmetrical peripheral edema, ascites, immunologic deficiencies, lymphocytopenia, hypoalbuminemia, imparied lymphocyte transformation, gastrointestinal symptoms and retarded growth, is the result of abnormal, distorted and obstructed lymph channels, causing rupture of intestinal lacteals from back-flow of lymph, with leakage of nutrient-laden lymph into the lumen of the bowel. A case of congenital intestinal lymphagiectasia is described, with the additional problems of allergic asthma, rhinitis, eczema and lactase deficiency. This patient, an 11-year-old child, was greatly benefited by proper allergy management (elimination diet, hyposensitization) plus restriction of fats and supplementing the diet with medium-chain triglycerides (MCT).

Asthma↗

[Radiologic appearance of primary anomalies of the lymphatic vessel system of the lung. Review and personal results].

Congenital disorders of the lymphatics of the lung are rare. On the basis of a literature review and our own experiences the disorders are divided into four groups and their radiographic findings are described. Pulmonary lymphangiectasia shows not typical signs. As an expression of the underlying pathophysiologic processes CT shows thickening of interlobular septs, interstitial edema and pleural effusions. Neither architectural distraction nor thickening of intralobular septs was seen. Lymphangioleiomyomatosis shows rather typical findings with multiple thin-walled bullae. In contrast to the opinion that architectural distraction is not seen in LAM we sometimes found signs of fibrosis. Disseminated pulmonary lymphangioma is characterised by proliferation of lymph vessels. CT-findings of this disorder have not been described before. CT shows multiple disseminated nodules of up to 2.5 cm accompanied by multiple bullae of similar distribution and size.

Humans↗

Primary congenital pulmonary lymphangiectasia--a case report.

We report on a 22 year-old women with recurrent pleural effusions and shadowing of the right lower lobe, which was refractory to antibiotic treatment. Histologic examination (open lung biopsy) was interpretated as indicating an early stage of lymphangioleiomyomatosis. Because of progression of the pulmonary changes and development of a pericardial effusion in spite of antiestrogen treatment to achieve pharmacological castration, and in view of the atypical findings in high resolution computed tomography (lack of cysts) a second open lung biopsy was performed, confirming the diagnosis of pulmonary lymphangiectasia. The patient was given oral corticosteroids postoperatively and showed almost complete resolution of the pleural and pericardial effusions.

Adult↗

[Disorders and correction of lymph outflow in chronic venous insufficiency of the lower limbs].

An analysis of 176 lymphograms, made in 102 patients with venous pathology of the lower extremities, is presented. In 14 of them varicosity in a decompensation stage, an uncomplicated form, in 32--varicosity in a decompensation stage, a complicated form, in 20--postthrombotic disease of an edematous and algesic form, in 36--an edematous-ulcerous form. The duration of the disease was from 3 to 25 years. It was found that the depth of changes in the lymphatic system was dependent on the rate of decompensation in the venous one.

Adult↗

Lymphoscintigraphy and lymphangiography of lymphangiectasia.

Chronic genital edema secondary to lymphangiectasia and chylous reflux in a 23-yr-old man with Noonan syndrome was investigated by 99mTc sulfur nanocolloid lymphoscintigraphy and bipedal contrast lymphangiography. Lymphoscintigraphy showed a delayed lymphatic flow pattern in the pelvis, abdomen and chest consistent with lymphangiectasia and abnormal lymphatic flow dynamics. Lymphangiography showed dilated and tortuous abnormal lymphatics in the abdomen and pelvis. Ligation of incompetent retroperitoneal lymph vessels and lymphaticovenous anastamosis were performed, resulting in clinical improvement. Lymphangiectasia has been described previously in Noonan syndrome, but it is relatively uncommon below the diaphragm. This case demonstrates the use of lymphoscintigraphy and lymphangiography in providing important physiological and anatomical information before surgical intervention. Careful presurgical planning using such tests also allows the most appropriate operation to be performed.

Adult↗