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Lymphoscintigraphic demonstration of intestinal lymphangiectasia.

The authors report a case of intestinal lymphangiectasia that was clearly demonstrated during abdominal lymphoscintigraphy using Tc-99m Sb colloid. Although lymphoscintigraphy has been used in many different clinical situations, this is, to our knowledge, the first scintigraphic description of this disease. Clinical and pathologic presentations of intestinal lymphangiectasia are briefly reviewed. Some possible approaches to the confirmation of the presence of a protein-losing gastroenteropathy are mentioned.

Adult↗

Scintigraphic diagnosis of protein losing enteropathy using Tc-99m dextran.

The authors performed abdominal scintigraphy using intravenously administered Tc-99m dextran in a patient with protein losing enteropathy. The study revealed abnormal leakage of the radiotracer in the left lumber area that moved over time in a pattern suggestive of small intestinal transit. Besides being a nonprotein and having long stay in intravascular compartment, the macromolecule may have many advantages over Tc-99m human serum albumin, the current radiotracer of choice for imaging intestinal protein loss.

Adult↗

Intestinal lymphangiectasia: value of Tc-99m dextran lymphoscintigraphy.

Intestinal lymphangiectasia is a common cause of protein-losing enteropathy characterized by diarrhea, generalized edema, enteric protein loss, hypoproteinemia, and lymphopenia. Diagnosis is based on demonstration of enteric protein loss and characteristic small bowel mucosal histology. Various imaging modalities including barium studies, computed tomography, and lymphangiography have had limited clinical use. The authors report a case of intestinal lymphangiectasia in which Tc-99m dextran lymphoscintigraphy played a significant role in the patient management.

Adult↗

Clinicopathologic study of conjunctivochalasis.

OBJECTIVE: To clarify the structural features of conjunctivochalasis histopathologically. METHODS: A biopsy of redundant conjunctiva from the same anatomic location of 44 +/- 7.5 years (mean +/- SD, 50.87 years), underwent conjunctivoplasty (termed tear meniscus reconstruction). RESULTS: In all 44 cases, histologic examination disclosed normal conjunctival epithelium and negligible inflammation and lymphocyte infiltration; 39 patients manifested microscopic lymphangiectasia. Elastica van Gieson staining demonstrated elastic fiber fragmentation and sparsely assembled collagen fibers in all 44 cases. There was no discernible difference between specimens from patients with and without complications including tear-deficient dry eye, meibomian gland dysfunction, and clinically observable lymphangiectasia and/or pinguecula. CONCLUSION: Based on our histopathologic findings, we hypothesize that mechanical forces between the lower lid and conjunctiva gradually interfered with lymphatic flow. Chronic, prolonged mechanical obstruction of lymphatic flow may result in lymphatic dilation and eventually give rise to clinical conjunctivochalasis.

Adolescent↗

The CT halo sign: a new finding in intestinal lymphangiectasia.

A "halo sign" has been described in patients with Crohn disease, ulcerative colitis, radiation enteritis, ischemic colitis, and pseudomembranous colitis. This sign is characterized by an inner ring of low CT attenuation surrounded by a higher attenuation outer ring. We present a patient with primary intestinal lymphangiectasia in whom CT demonstrated a halo sign correlated with mucosal biopsy.

Adult↗

Cross-sectional imaging findings in congenital intestinal lymphangiectasia.

A 37-year-old woman presented with an 18 month history of recurrent severe abdominal pain, recurrent diarrhea since her second year of life, and mild peripheral edema. CT and more clearly MRI revealed signs of small bowel and mesenterial edema with partial tubular appearance, which correlated well with the histopathologic findings shown in duodenal biopsy. This is the first report of MR findings in congenital intestinal lymphangiectasia with correlation with other imaging modalities.

Abdominal Pain↗

Multiple abdominal telangiectases and lymphangiectases. A limited form of Osler-Weber-Rendu disease?

We describe a 23-year-old man with protein-losing enteropathy, iron deficiency anemia, and recurrent gastrointestinal bleeding. The patient eventually developed disseminated intravascular coagulation, portal vein thrombosis, and extensive small bowel infarction. The autopsy showed multiple telangiectases in the intestines, mesentery, liver, gallbladder, renal pelves, and diaphragm. In addition, lymphangiectases were found in the retroperitoneal space, intestines, and liver. These lesions appeared to have been the cause of the gastrointestinal bleeding and the protein-losing enteropathy. The case most likely represents Osler-Weber-Rendu disease without the usual manifestations in the skin and oral-nasal cavities.

Adult↗

Failure of blind small bowel biopsy in the diagnosis of intestinal lymphangiectasia.

A case of primary intestinal lymphangiectasia is presented in which multiple blind peroral jejunal biopsies were unable to document any abnormality, despite strongly suggestive clinical history and radiographic findings. Endoscopically directed biopsy was necessary to document the characteristic pathologic lesion. This report documents the importance of endoscopy in the diagnosis of intestinal lymphangiectasia when clinical history is suggestive of intestinal lymphangiectasia but standard small bowel biopsy fails to show any abnormality.

Biopsy↗

Congenital pulmonary lymphangiectasia: CT and pathologic findings.

Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood. The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy. The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.

Adult↗

Different patterns of lymphoscintigraphic findings in patients with intestinal lymphangiectasia.

Technetium-99m antimony sulfide colloid lymphoscintigraphy conveniently demonstrates intestinal leakage of lymph in patients with intestinal lymphangiectasia. However, we found no intestinal radioactivity in some patients. We evaluated lymphoscintigraphic findings and compared them with clinical data. Technetium-99m antimony colloid lymphoscintigraphy was performed in 12 patients (age, 8.9+/-6.4 years; male:female=8:4) with histologically proven intestinal lymphangiectasia. After subcutaneous injection of 103.6 MBq of technetium-99m antimony colloid into the webs of both feet, sequential abdominal images were obtained up to 24 h post-injection. Four patients underwent technetium-99m methylene diphosphonate bone scintigraphy. Patients were divided into two groups according to the presence or absence of intestinal radioactivity. Five showed intestinal activity (Group 1), but seven did not (Group 2). No Group 1 patient had a history of ascites, while all Group 2 patients had ascites as the initial manifestation. Serum total protein and albumin levels were significantly lower in Group 1 patients than in Group 2 patients. In three Group 1 patients, technetium-99m methylene diphosphonate bone scintigraphy revealed intestinal radioactivity, while in one Group 2 patient this was not found. We observed two types of lymphoscintigraphic pattern in patients with intestinal lymphangiectasia. To clarify the exact pathophysiology, further study is required.

Adolescent↗