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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.
PURPOSE: Primary chylous disorders (PCDs) are rare. Rupture of dilated lymph vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or genitalia. Chyle may reflux through incompetent lymphatics, causing lymphedema. To assess the efficacy of surgical treatment, we reviewed our experience. METHODS: The clinical data of 35 patients with PCDs treated between January 1, 1976, and August 31, 2000, were reviewed retrospectively. RESULTS: Fifteen men and 20 women (mean age, 29 years; range, 1 day-81 years) presented with PCDs. Sixteen (46%) patients had chylous ascites, and 19 (54%) had chylothorax (20 patients), and of these, 10 (29%) had both. In 16 patients, reflux of chyle into the pelvic or lower limb lymphatics caused lymphedema (14, 88%) or lymphatic leak through cutaneous fistulae (11, 69%). Presenting symptoms included lower-limb edema (19, 54%), dyspnea (17, 49%), scrotal or labial edema (15, 43%), or abdominal distention (13, 37%). Primary lymphangiectasia presented alone in 23 patients (66%), and it was associated with clinical syndromes or additional pathologic findings in 12 (yellow nail syndrome in 4, lymphangiomyomatosis in 3, unknown in 3, Prasad syndrome (hypogammaglobulinemia, lymphadenopathy, and pulmonary insufficiency) in 1, and thoracic duct cyst in 1). Twenty-one (60%) patients underwent 26 surgical procedures. Preoperative imaging included computed tomography scan in 15 patients, magnetic resonance imaging in 3, lymphoscintigraphy in 12, and lymphangiography in 14. Fifteen patients underwent 18 procedures for chylous ascites or pelvic reflux. Ten (56%) procedures were resection of retroperitoneal/mesenteric lymphatics with or without sclerotherapy of lymphatics, 4 (22%) were lymphovenous anastomoses or grafts, 3 (17%) were peritoneovenous shunts, and 1 (6%) patient had a hysterectomy. Six patients underwent eight procedures for chylothorax, including thoracotomy with decortication and pleurodesis (4 procedures), thoracoscopic decortication (1 patient), ligation of thoracic duct (2 procedures), and resection of thoracic duct cyst (1 patient). Postoperative mean follow-up was 54 months (range, 0.3-276). Early complications included wound infections in 3 patients, elevated liver enzymes in 1, and peritoneovenous shunt occlusion with innominate vein occlusion in 1. All patients improved initially, but four (19%) had recurrence of symptoms at a mean of 25 months (range, 1-43). Three patients had postoperative lymphoscintigraphy confirming improved lymphatic transport and diminished reflux. One patient died 12 years postoperatively, from causes unrelated to PCD. CONCLUSIONS: More than half of the patients with PCDs require surgical treatment, and surgery should be considered in patients with significant symptoms of PCD. Lymphangiography is recommended to determine anatomy and the site of the lymphatic leak, especially if lymphovenous grafting is planned. All patients had initial benefit postoperatively and two thirds of patients demonstrated durable clinical improvement after surgical treatment.
Intestinal lymphangiectasia (IL) usually presents with either non-specific general or gastro-intestinal symptoms. As IL may mimic other gastro-intestinal disorders, the diagnosis is often delayed. Intestinal lymphangiectasia was diagnosed in three children who were originally treated as cases of coeliac disease. Two were sisters who had been placed on a gluten-free diet, for 3 years in one and 10 years in the other, with no favourable response. The third patient had been tried on various formulae and underwent many investigations for failure to thrive, oedema, abdominal distension and recurrent chest infections. The diagnosis of IL was based on clinical history, physical examination and radiological and histological findings. The three patients were commenced on a medium-chain triglyceride-based diet and vitamins, with satisfactory results.
A female infant born at 28 weeks' gestation was found to have mild hydrops foetalis. Initial echocardiography showed a structurally normal heart. During the first week of life, episodic atrial tachycardia with 1:1 or 2:1 conduction was seen, requiring therapy with digoxin. The infant remained ventilator dependent, with a persistent, chylous pleural effusion which contained a preponderance of lymphocytes. Congenital pulmonary lymphangiectasia (CPL) was confirmed histologically. Worsening episodes of atrial tachycardia, including episodes of atrial fibrillation, were further investigated and a repeat echocardiogram revealed thickening of the entire right atrial wall. The cardiac findings of a thickened right atrial wall with the histological signs of myocarditis were thought to be the cause of paroxysms of atrial fibrillation, an extremely rare arrhythmia in the neonatal period. To the authors' knowledge there have been no previous reports of CPL in association with the cardiac abnormalities described herein.
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The results of the conventional 5-hour d-xylose absorption test were surveyed in 38 subjects with disease of the jejunal mucosa, giardiasis or bacterial overgrowth, or no small-bowel disorder. The test was in error in 20-40% of cases, depending on the disease category, and the error for the entire group was 30%. We conclude that the test yields little guidance for diagnosis or therapy of clinical problems and is superfluous when a jejunal biopsy can be obtained.
The fine structure of intestinal lymphatics in four patients with Crohn's disease and in two control subjects is described. Although obstructed lacteals are considered to be of major importance in the pathogenesis of regional enteritis, no detailed electron microscopic studies of lymphatic capillaries in this disease could be found. Even though both open and closed intercellular junctions were observed in the normal intestinal lymphatics, only closed junctions were noted in the mucosal and submucosal lymphatic capillaries in patients with regional enteritis. A heavy accumulation of protein rich lymph at the abluminal surface of lymphatic capillaries was consistently seen. None of the control lymphatics showed a similar alteration. The described fine structural changes indicate a decreased permeability of the lymphatic wall. Reduced lymphatic permeability could be a contributing element in the development of submucosal edema, a major microscopic feature of Crohn's disease.
Three immunocompromised patients presented with cellulitis as the primary manifestation of cryptococcal disease. Two were recipients of cadaveric renal transplants who were receiving immunosuppressive drug therapy. The other patient had profound lymphopenia and severe hypoalbuminemia due to intestinal lymphangiectasia. All had failed to respond to empiric therapy for presumed bacterial cellulitis before results of skin biopsy or aspiration were available for the correct diagnosis to be made. With administration of systemic antifungal therapy, two patients survived. Although other forms of cryptococcal involvement of the skin are not rare, cellulitis is seldom considered to be a cutaneous manifestation of the disease. Our cases and a review of the English-language literature indicate that Cryptococcus neoformans must be included in the differential diagnosis of cellulitis in immunocompromised patients and that the presence of cryptococcal cellulitis suggests disseminated cryptococcal disease. Prompt diagnosis and treatment may dramatically reduce mortality.
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Immunohistochemical staining was used to demonstrate basement membrane (BM) laminin and type IV collagen in eight cases of Kaposi's sarcoma (KS). These KS were not associated with AIDS and represented different histological stages of the disease: patch (three cases), plaque (one case), and nodule (four cases). Nine cases of benign angiogenic lesions, five of blood vessel origin, and four of lymphatic vessel origin were also studied. An early event in vascular proliferation at the patch stage of KS was an intersection of dermal collagen bundles and the appearance of granular BM material around this space. With the increase of amount and linear arrangement of BM material, well-defined capillaries were formed. Two types of capillary were found in KS lesions. One showed morphological features of blood capillaries, with a round lumen; thick, continuous BM, and occasional pericytes in the wall. The other included irregularly shaped vessels with thin, often disrupted BMs; thus these capillaries morphologically resembled lymphatic capillaries. BM staining also clearly revealed the vascular nature of the nodular lesions of KS, which were composed of a network of slit-like spaces surrounded by BMs. The solid tumor cell areas were sparse; they were composed of spindle-shaped cells surrounded by thin, interrupted basal laminae. By using antibodies against human laminin and human type IV collagen, it was also possible to demonstrate thin, widely disrupted BMs subendothelially in normal dermal lymphatic capillaries. Typically, the BMs in lymphangiomas and lymphangiectasias were continuous and more clearly defined.
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