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Aetiology and treatment of chylous ascites.

A series of 45 patients with chylous ascites has been reviewed. The age at presentation ranged from 1 to 80 (median 12) years; 23 patients were aged < or = 15 years. Thirty-five patients had an abnormality of the lymphatics (primary chylous ascites); in the remaining ten, the ascites was secondary to other conditions, principally non-Hodgkin's lymphoma (six patients). Two principal mechanisms of ascites formation were identified using lymphangiography and inspection at laparotomy: leakage from retroperitoneal megalymphatics, usually through a visible lymphoperitoneal fistula (14 patients); and leakage from dilated subserosal lymphatics of the small intestine, invariably associated with leaking lacteals causing protein-losing enteropathy (24 patients). Both sites of leakage were present in a further five patients. In the remaining two patients, chyle was leaking from normal mesenteric lymphatics, in one via a ruptured mesenteric lymph cyst and in the other from the site of a previous lymph node biopsy. Other associated lymphatic abnormalities were present in 36 patients, lymphoedema of the leg being the commonest (26 patients). All patients were initially treated conservatively with dietary manipulation; this was the most satisfactory treatment for those with leaking small bowel lymphatics. Surgery (fistula closure, bowel resection or insertion of a peritoneovenous shunt) was performed in 30 patients. Closure of a retroperitoneal fistula, when present, was the most successful operation, curing seven of the 12 patients so treated.

Adolescent↗

Abnormalities of lymphatic drainage in lower extremities: a lymphoscintigraphic study.

Chronic lower-leg edema in patients with venous disorders was studied by means of lymphoscintigraphy. Lymphatic patterns of flow were evaluated prospectively in 26 patients with technetium 99m antimony trisulfide colloid injected subcutaneously in the interdigital web spaces on the feet. Most patients in this study had postphlebitic syndrome, and all of these patients had abnormal lymphoscintigraphic flow patterns. Nine had evidence of lymphatic obstruction, and one had an enhanced flow pattern. Three patients had veins used for distal arterial bypass, and all these veins showed decreased lymphatic flow. Two patients with Klippel-Trenaunay syndrome (congenital varicose veins associated with limb elongation, a capillary nevus, and an abnormal deep venous system) had obstruction to lymphatic flow, and two others had normal and enhanced patterns. Normal studies were seen in four of five patients who had veins used for coronary artery bypass grafting. The finding of decreased lymphatic flow in patients appears to be the result of the length of time from an episode of deep venous thrombosis, the occurrence and number of episodes of cellulitis and lymphangitis, and mobilization of the vein for use in distal arterial bypass surgery. This study shows that the edema attributed previously to primary venous disorders may have a significant lymphatic component. The degree of lymphatic obstruction can be determined by lymphoscintigraphy with technetium-labeled antimony trisulfide colloid.

Adult↗

Lymphoscintigraphic evaluation in patients after erysipelas.

Erysipelas (cellulitis/lymphangitis) is a superficial cutaneous infection spread by the lymphatic system which may result in permanent injury to the lymphatic vessels. The study evaluated the lymphatic drainage in the lower limbs of 30 patients with at least two episodes of erysipelas by means of lymphoscintigraphy. Twenty-two (73%) were female and 8 (27%) were male with ages ranging from 26 to 77 years (mean 52 years). Lymphoscintigraphy was performed by intradermal administration of 500 microCi (20 Mbq) of 99mTc antimony sulfur-colloid in two interdigital spaces of the feet. Whole body scintigraphy was performed 45 minutes after the administration of the radiopharmaceutical using a computerized gamma camera. Significant lymphatic abnormalities were found in 23 (77%) of these patients. We conclude that most patients with repeated erysipelas have significant and even permanent abnormalities in regional lymphatic drainage. Recurrent erysipelas suggests underlying primary or secondary lymphedema.

Adult↗

Isolated idiopathic chylopericardium.

The case history of a young asymptomatic patient with primary chylous pericardium is reported. On the basis of this case and those reported in the literature, we suggest that diagnosis should be established by echocardiograms, cardiac angiograms, and pericardiocentesis. Lymphangiograms are useful for the detection of the underlying abnormality of the lymph vessels. Pericardiectomy together with thoracic duct ligation are suggested as the preferred treatment. We propose that generalized lymphatic abnormality is the underlying mechanism of this entity. This lymphatic abnormality could be associated with impaired cellular immunity, although no such impairment was found in our patient.

Adolescent↗

"Occult cutaneous lymphangiectasis": an unusual case of cutaneous lymphangioma.

An unusual case of cutaneous lymphatic abnormality is described and named as "occult cutaneous lymphangietasis". A 26-year-old man had noticed pigmented maculae in the left inguinofemoral region and waist, which had gradually increased in number for as long as he could remember. The peculiar distribution of the eruptions and their transient saccular dilatation due to infection suggested that they were of lymphatic origin. Lymphangiography showed the presence of dilated lymphatics in the left inguinofemoral area and correspondence of the distribution of dermal backflow with the locations of the pigmented maculae. The histology is consistent with a diagnosis of lymphangioma. We could find no other reports of cases of cutaneous lymphangioma featuring pigmented maculae as the sole manifestation, although whether the pigmentation is an original clinical feature of this type of lymphatic abnormality is still an open question.

Adult↗

Abnormal lung lymphatics and respiratory failure.

A 65 year old man presented with respiratory failure, pleural effusions, fine reticulonodular shadowing on a chest radiograph, and severe impairment of carbon monoxide diffusing capacity (transfer factor). Open lung biopsy showed only dilated pleural and subpleural lymphatic channels. Hypoplastic deep pulmonary lymphatics may have led to respiratory failure.

Aged↗

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity.

BACKGROUND: Severe thrombocytopenic coagulopathy may complicate platelet-trapping vascular tumors such as kaposiform hemangioendothelioma and tufted angioma. Low-grade, chronic consumptive coagulopathy may occur with extensive venous and lymphatic malformations. We have also observed patients with rare multifocal, congenital skin and gastrointestinal (GI) tract vascular anomalies of distinctive and remarkably similar appearance, all associated with coagulopathy. We studied the clinical and histopathologic features of 3 patients demonstrating this previously uninvestigated phenomenon. OBSERVATIONS: All 3 patients presented with hundreds of congenital red-brown skin plaques as large as a few centimeters, with similar lesions throughout the GI tract and severe GI tract bleeding. One patient had synovial involvement. All had significant thrombocytopenia, with prothrombin and partial thromboplastin times and fibrinogen levels near the reference range. Corticosteroids and/or interferon alfa treatment resulted in equivocal or no improvement. Skin lesions from all 3 patients were histologically distinctive and similar, including dilated, thin-walled vessels in the dermis and subcutis lined by hobnailed, proliferative endothelial cells (10%-15% immunoreactive for Ki-67), most displaying intraluminal papillary projections. Immunoreaction for the lymphatic marker LYVE-1 was uniformly present. CONCLUSIONS: We propose the term multifocal lymphangioendotheliomatosis with thrombocytopenia to distinguish this newly recognized clinicopathological entity. These congenital lesions, like tufted angioma and kaposiform hemangioendothelioma, show lymphatic differentiation, strengthening the association between abnormal lymphatic endothelium and coagulopathy.

Child↗

Capillary filtration and lymphatic resorption in diabetes. Application to the pharmacodynamic activity of Daflon 500 mg.

Microcirculatory disorders are often a complication of diabetes. They are associated with an increased capillary permeability which can be assessed by the Landis test using 99mTc-labelled albumin. Labelled albumin retention is measured by external detection (residual radioactivity after removal of venous tourniquet). The Fast Fourier Transform of the radioactivity disappearance graph reflects the lymphatic albumin resorption. Thirteen out-patients with diabetes from 1 to 19 years suffering from evident microcirculatory disorders were treated with a micronized flavonoid fraction: Daflon 500 mg (2 tablets per day) during a month. Antihypertensive and/or antidiabetic treatment were continued during the study if they were given before starting. A test was performed before, and at the end of the treatment. In 10 of the 13 patients, the test was carried out a third time 5.5 +/- 1.0 months later, after treatment withdrawal. Results were as follows: 1) labelled albumin retention was abnormal (greater than or equal to 8%) at the start and became normal in the patients and improved in 1 patient. After treatment withdrawal, the results of the third test were again abnormal, in the patients who had been normalized beforehand; 2) the initially abnormal lymphatic resorption became normal in six of the 13 patients at the end of the treatment and became abnormal again in all patients after treatment withdrawal. In a parallel group of 15 patients with abnormal test lymphatic fluctuations and not being treated with a vasculo-protector agent, the initially abnormal test remained abnormal 5 to 48 months later in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Mediastinal adenopathy and cancer of the prostate].

The authors report a case of metastases from a carcinoma of prostate producing mediastinal adenopathy with compression. The clinical presentation was unusual as this is an unusual area for lymphadenopathy and an abnormal lymphatic pattern was invoked as a possible cause or that the lymphatic channels communicating with these nodes had an abnormal thoracic duct. The aetiological diagnosis was facilitated by the presence of a specific prostatic antigen on the lymph node biopsy which allowed one to use an effective androgen suppression therapy.

Aged↗

Noninvasive evaluation of the swollen extremity: experiences with 190 lymphoscintigraphic examinations.

Lymphoscintigraphy (LS), performed with technetium 99m-labeled antimony trisulfide colloid (Cadema Medical Products, Inc., Middletown, N.Y.), was used as a noninvasive diagnostic examination to evaluate the lymphatic circulation in 190 extremities of 115 patients. Forty-six patients had primary lymphedema, 48 had secondary lymphedema, and 21 patients had other causes of limb swelling. To determine the value of LS in surgical decision making, preoperative and postoperative LS of 16 patients who underwent surgical repair of the lymphatic abnormality were studied separately. Semiquantitative evaluation of the lymphatic drainage and visual interpretation of the image patterns were reliable to differentiate lymphedema from edemas of other origin (sensitivity: 92%, specificity: 100%). Although certain image patterns were characteristic of either primary or secondary lymphedema, LS could not consistently differentiate between the two types. Episodes of cellulitis in lymphedema clearly delayed lymph transport. LS was helpful in patient selection and follow-up after lymphatic surgery, but it did not prove patency of lymphovenous anastomoses. It was diagnostic in the evaluation of lymphangiectasia and was used to document successful surgical treatment of reflux of chyle. LS is safe and reliable and has no side effects. It should replace contrast lymphangiography in the routine evaluation of the swollen extremity.

Adolescent↗

Vulvar lymphangiectasia due to recurrent cellulitis.

We describe a 42-year-old woman in whom extensive lymphangiectasia of the vulva developed after recurrent severe episodes of cellulitis, producing changes rather similar to those seen in lymphangioma circumscriptum. We suspect that an underlying lymphatic abnormality predisposed her to cellulitis, resulting in local lymphatic fibrosis and vulvar lymphangiectasia.

Adult↗

Lymphoscintigraphy in chyluria, chyloperitoneum and chylothorax.

UNLABELLED: Lymphoscintigraphy allows functional assessment of lymphatic transport and depiction of regional lymph nodes, is fast and nontraumatic and has no known side effects. We retrospectively analyzed lymphoscintigraphic studies to determine their efficacy in the investigation of chyluria, chyloperitoneum and chylothorax. METHODS: Twenty-one whole-body lymphoscintigrams using 99mTc-antimony sulfide colloid or dextran were acquired in 18 patients with chyluria, chyloperitoneum and/or chylothorax. The images were reviewed to assess the rate of tracer transport and number, size and distribution of lymph vessels and nodes as well as the presence of collateral, fistula or lymph reflux. RESULTS: Lymphoscintigraphy was normal (5 of 11 patients) or showed lymphatic obstruction (6 of 11 patients) in chyluria associated with filariasis. Lymphatic obstruction was demonstrated in chyloperitoneum and/or chylothorax associated with liver cirrhosis (2 patients), postoperative (1 patient) or congenital (1 patient) lymphatic dysplasia, inferior vena cava obstruction (1 patient) and nephrotic syndrome (1 patient). Enhanced lymph flow was seen in systemic lupus erythematosus (1 patient). Follow-up lymphoscintigrams showed patency of lymphovenous anastomosis (1 patient), improvement (1 patient) or no change (1 patient) in lymphatic drainage after treatment. CONCLUSION: Lymphoscintigraphy can demonstrate abnormal lymphatic drainage in chyluria, chyloperitoneum and chylothorax. It is useful for selecting patients for surgery and assessing the effect of treatment.

Adult↗