[Intestinal lymphangiectasis in exudative enteropathy].
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Autopsy↗
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[Enteric lymphangiectasis and protein loss].
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Journal Article↗
[2 patients with extensive resection of the small intestine and one patient with intestinal lymphangiectasies treated with MCT fat].
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Adolescent↗
[Chronic peritonitis, intestinal lymphangiectasis and protein-losing enteropathies. (Surgical treatment)].
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Adult↗
[Chronic diarrhea in a 43-year-old patient].
A 43-year old patient came to our clinic with chronic diarrhea. Determination of the faecal alpha 1-antitrypsin-clearance led to the diagnosis of exsudative enteropathy. Blood counts showed pronounced lymphocytopenia. Histopathological findings from intestinal and colorectal biopsies showed diffuse lymphangiectasis. Following exclusion of secondary types, our diagnosis was primary intestinal lymphangiectasis. Additional distinctive morphological and anamnestic features strongly suggested presence of Noonan's syndrome. Characteristic manifestations of Noonan's syndrome include changes in the lymphatic vessels in accordance with primary lymphangiectasis. Frequently, these changes are localized in the lungs. To date, only rare cases of intestinal lymphangiectasia in Noonan's syndrome have been reported. Treatment consisted of a protein-rich diet, with reduced fat content enriched by middle-chain fatty acids, as well as twice-daily injections of 200 micrograms octreotide. With this therapy, the symptoms improved.
Adult↗
Congenital pulmonary lymphangiectasia and other anomalies in a child: provisionally unique syndrome?
Congenital pulmonary lymphangiectasis is a rare anomaly that is causally heterogeneous. It can occur as either an isolated finding or one manifestation of several multiple congenital anomaly syndromes. We describe a child with congenital pulmonary lymphangiectasis and the anomalies who likely has a provisionally unique condition.
Abnormalities, Multiple↗
Cutaneous lymphangiectases after therapy for carcinoma of the cervix--a case with unusual clinical and histological features.
Secondary lymphangioma (lymphangiectasis) has been reported as a consequence of lymphatic damage. No specific histological criteria can be used to differentiate primary lymphangioma circumscriptum from lymphangiectasis. We describe a woman who developed lymphangiectases on both non-lymphoedematous upper thighs after hysterectomy and radiation therapy for carcinoma of the cervix. This case differs from the secondary lymphangiomas reported previously in that the vascular channels appeared in newly formed adventitial dermis without histological changes in the epidermis.
Carcinoma, Squamous Cell↗
Chyloptysis in adults: presentation, recognition, and differential diagnosis.
Chyloptysis is a rare finding, and the accompanying respiratory symptoms are usually nonspecific. The recognition of the chylous nature of the sputum is requisite for proper diagnosis, especially if chyloptysis is not accompanied by chylous pleural effusion. The key to the differential diagnosis of chyloptysis is to consider illnesses that can induce reflux of chyle into the bronchial tree. There are two mechanisms postulated: the first requires the presence of an abnormal communication between the bronchial tree and the lymphatic channels, and the second requires a bronchopleural fistula in the context of a chylous pleural effusion. Chyloptysis in adults should prompt assessment for evidence of lymphatic obstruction from trauma, radiation, and malignancy, and to exclude diseases with known association with chyloptysis, ie, lymphangioleiomyomatosis, yellow nail syndrome, or thoracic lymphangiectasis. A lymphangiogram is recommended to define the abnormality. In the case of lymphangiectasis, patients respond to either dietary modification and/or ligation of the thoracic duct.
Adult↗
[Waldmann disease and pregnancy].
Waldman disease, also called intestinal lymphangiectasis, is a protein-loosing enteropathy caused by anomalous intestinal lymphatic canals. The condition is transmitted by autosomal dominant inheritance. Diet therapy including medium chain triglycerides improves the disease course. The condition occurs in early childhood. We report a case observed in a pregnant woman with intestinal lymphangiectasis. She delivered a full-term infant and experienced no major complications.
Adult↗
The lymphatic pathology of Brugia pahangi in nude (athymic) and thymic mice C3H/HeN.
The nude (congenitally athymic) mouse, C3H/HeN is highly susceptible to infection with Brugia pahangi (Nematoda: Filarioidea). Normal, hairy mice show a strong thymus-dependent resistance and usually terminate the infection in the larval stages. The present study examined chronological histopathologic changes in the lumbar lymph nodes and adjacent lymphatic vessels of both hosts. In thymic mice, lymphangitis and perilymphangitis reached a maximum 14 to 17 days PI, about the time of disappearance of live worms. The infiltrate showed characteristics of both acute and chronic inflammation: eosinophils, neutrophils, eosinophilic precipitates, and sometimes necrotizing lymphangitis, as well as macrophages and plasma cells. The cellular infiltrate in nude mice was weaker and developed more slowly. Inflammatory responses to identifiable dead worms were seen in both types of hosts but appeared more frequently in thymic mice. Although variable in both models, the granulomas of thymic mice generally showed more tendency to cavitation, greater macrophage or epithelioid cell infiltration, more granulocytes, and appeared to be more destructive than the foreign body responses of nude mice. Whereas lymphangiectasis was generally progressive in nude mice, it was arrested before the end of the third week in thymic mice. In thymic mice, at maximum lumbar lymph node size (17 days), there were large areas of lymphocyte hyperplasia and heavy infiltration of plasma cells. Most nodes returned to normal mean size by the end of the second month. Little or no reactivity was seen in athymic mouse nodes. Our results suggest that some lesions of lymphatic filariasis are potentially thymus-independent: lymphatic fibrosis, lymphangiectasis, accumulations of macrophages and giant cells around disintegrating worms, calcification of worms, intralymphatic thrombosis, and moderate vascular infiltrates including eosinophils.
Animals↗
[Structural changes in the ileal mucosa of urinary conduits].
OBJECTIVES: To study the changes found in the terminal ileum mucosa in contact with urine in patients with skin ureteroileostomy following cystectomy due to infiltrant carcinoma of the bladder. MATERIAL AND METHODS: 21 biopsies of gut mucosa were performed in as many patients. Measurements included height of intestinal villi (average values -AV-: 350-300 mu), height of crypts (AV: 70-100 mu), crypt/villus ratio (AV: 0.2), goblet cells/enterocytes ratio, presence of lymphangiectasis and inflammatory infiltrate. Also the existence of lab changes were measured with ionogram and venous gasometry. RESULTS: Mean age of patients was 65.2 years +/- 7.4 SD. Males 66.7%. Time of evolution with urinary by-pass was 59.5 months +/- 53.2 SD. Mean height of villi 178.2 mu +/- 70.2 SD, mean height of crypts 290.9 mu +/- 114.4 SD and mean crypt/villus ratio 4.2 +/- 9.2 SD. Submucous inflammatory infiltrate was mild in 57.1%, moderate in 23.8%, and severe in 19.0%. Only 2 cases had lymphangiectasis images. Goblet cells/enterocytes ratio was 3.3 +/- 1.3 SD. No correlation was seen between time of evolution of urinary by-pass with the various changes in gut mucosa or between degree of mucosal atrophy and existence of metabolic disorders. CONCLUSIONS: Changes in the terminal ileum mucosa in patients with skin ureteroileostomy-like urinary by-pass are characterised by a marked atrophy of intestinal villi with increased crypt length, increased crypt/villus ratio and presence of mild-to-moderate inflammatory infiltrate. There is also an increase of goblet cells in detriment of enterocytes. All these changes are independent from the time of evolution of patients with urinary by-pass.
Aged↗
The incidence and characteristics of neonatal irreversible lung dysplasia.
A 3-year retrospective study of 173 neonates treated with extracorporeal membrane oxygenation in the United Kingdom identified 9 cases of irreversible lung dysplasia, including alveolar capillary dysplasia (n = 5), surfactant protein B deficiency (n = 1), pulmonary hypoplasia (n = 1), pulmonary lymphangiectasis (n = 1), and combined lymphangiectasis and hypoplasia (n = 1).
Bronchopulmonary Dysplasia↗
[Retroperitoneal lymphangiodysplasia with exudative enteropathy. A case of cystic dilated cisterna chyli and splenic cysts].
In a 16 years old girl we found the syndrome of exsudative enteropathy with intestinal lymphangiectasy which differs from previous reported cases by several malformations of the lymphatic system. Besides changes in the intestinal lymphatics there was a retroperitoneal lymphangiodysplasia, a reduction and hypoplasia of the lymph vessels in the legs and cysts in the spleen. By substitution of albumin followed by a diet with middle chained triglycerids (MCT), serum protein was stablised but a relapse could not be prevented. Surgical treatment was not possible because the changes were too extensive. The value of diagnostic measures for differential diagnosis of the intestinal lymphangiectasy is presented.
Adolescent↗
Endoscopic ultrasonography in the differential diagnosis of giant gastric folds.
Giant gastric folds (or large gastric folds) are found in both benign and malignant diseases, and differential diagnosis with either upper gastrointestinal X-ray or endoscopy is difficult. Sometimes, even endoscopic biopsy cannot establish a definitive diagnosis. Recently, endoscopic ultrasonography (EUS) has been used to study giant gastric folds. We performed EUS in 25 patients with giant gastric folds that had been detected with upper gastrointestinal X-ray or endoscopy. The definitive diagnoses were confirmed by histopathology, other examinations, or long-term follow-up. The final diagnoses of these 25 patients were gastric varices in eight, gastric lymphangiectasis in one, gastritis in four, gastric carcinoma (scirrhous type) in six, and gastric lymphomas in six. All patients with gastric varices had anechoic tortuous varicose veins in the submucosal layer. EUS images of gastric lymphangiectasis were similar to those of gastric varices. EUS revealed regular gastric wall thickening of the second (mucosa) and third (submucosa) layers in all cases of gastritis. The fourth (muscularis propria) layer was intact in the only case of mucosa-associated lymphoid tissue lymphoma (MALToma), but not in the other five cases of gastric lymphoma. The second and third layers of this MALToma were irregular in thickness and heterogenous in echogenicity, different from the characteristic EUS findings in gastritis. The fourth layer was markedly thickened only in malignant conditions. Differentiation of gastric cancer from lymphoma with EUS was difficult because of overlapping EUS findings. In conclusion, EUS is indicated for the differential diagnosis of giant gastric folds. In addition, it avoids the risk associated with biopsy of gastric varices.
Adult↗
Cystic lymph node enlargement of the neck: filariasis as a rare differential diagnosis in MRI.
Cervical lymph node enlargement is a common feature of most inflammatory and neoplastic entities of the head and neck. Filariasis can lead to lymphangiectasis resembling lymph node enlargement; however, this is a rare differential diagnosis in European patients. As ethnic minorities are increasing throughout Europe and personal mobility, e.g. during holidays, is increasing, such rare differential diagnoses have to be taken into consideration. We present the case of an Iraqi patient referred to as for a suspected cystic lymph node mass that was verified histologically by open MRI biopsy and proved to be a cystic manifestation of filariasis.
Diagnosis, Differential↗
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.
Adult↗
[Diagnosis and treatment of chylopericardium (author's transl)].
In a 22-year-old patient without clinical symptoms, a chylous pericardial effusion was found as cause of pericardial enlargement persisting over 7 years. Ultrasonic echocardiography, diagnostic pericardial puncture and the demonstration of chylomicrons enabled the diagnosis to be made. Lymphography showed lymphangiectasis of the thoracic duct. At operation two fistulous connections between pericardium and lymphatic system were found. Surgical ligature of the thoracic duct and the lymphatic fistulae and pericardial fenestration resulted in rapid normalisation of all findings.
Adult↗
Transient intestinal lymphangiectasia.
Intestinal lymphangiectasia may be more protean in clinical manifestations and less rare than earlier suspected. A recent report points out that there are two types of the disorder, one congenital and the other acquired and transitory. A case is reported which fulfills the current clinical, laboratory, radiological and histological criteria for the diagnosis of the disease, and represents the first report in Scandinavia of transient intestinal lymphangiectasis with rapid and complete recovery within a few months after initiation of MCT diet.
Child↗