Displacement of lymphatic vessels by an abnormal vein.
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This paper reviews for the first time the normal and abnormal appearances of lymphatic channels of the skin using ultrasound. After a review of anatomy and histology, the authors present the current imaging modalities available for lymph vessel imaging. The ultrasound examination is presented with a description of the author's technique as well as the technical requirements of the ultrasound unit (12 MHz linear probe with a resolution of 400 microns). They present the ultrasound appearance of normal lymphatic channels and their relationships to the dermis, hypodermis and lymph nodes, and at last the ultrasound appearance of abnormal lymphatic pathways
A patient who has expectorated bronchial casts for 23 years is reported. The casts contained mucus, fibrin, and lymphocytes, and the resected middle lobe showed dilated lymphatics with thickened walls at the hilum and lymphoid aggregates in the terminal bronchioles.
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IgA heavy chain disease (alpha chain disease) was detected in a 46 year old South American (Colombian) of mixed Spanish and Indian (Mestizo) descent. The clinical course was characterized by severe malabsorption, initially thought to be a variant of tropical sprue. Jejunal mucosal biopsy revealed marked villous atrophy with heavy infiltration of the lamina propria by a single monotonous cell type which, by light microscopy, had the appearance of either a large, abnormal plasma cell or a small histiocyte. Electron microscopy of the biopsy specimen demonstrated the presence of abnormal plasma cells, lymphatic distention and abnormalities of surface epithelial cells. At autopsy a 6 by 3 cm mass was found in the right para-aortic area along with many firm 2 cm mesenteric and para-aortic lymph nodes. Histologic examination revealed the presence of a monotonous cell type similar to that found in the jejunal mucosal biopsy specimen.
The authors present an unusual case of massive enlargement of the right lower extremity that was treated as lymphedema secondary to filarial infection. A multiple-method approach beginning with scintigraphy led to the diagnosis of massive hemangioma with probable associated lymphatic proliferation. Abnormal local proliferation of vascular and lymphatic tissue is a developmental anomaly that is difficult to diagnose or characterize, even with histopathologic data. Individual imaging methods may also yield nonspecific findings. Blood-pool scintigraphy, therefore, has a definitive role to play in assessing such complex lesions.
Lymphatic malformations are due to an abnormality in lymphatic development only rarely present in adults. Their prognosis is better than those of infants. We report 6 cases of lymphatic malformations of head and neck in adults reviewed from 1990 to 2001. The treatment was surgical in 5 cases. Surgical excision is the treatment of choice and reccurences are rare.
Although the clinical features of lymphedema are often distinctive, it is essential to confirm the diagnosis with an objective test. Isotope lymphography is simple and 95% accurate for defining deficient lymph clearance. It is particularly useful for separating venous from lymphatic edema. Definition of the precise abnormality--peripheral lymphatic obliteration, proximal lymph node obstruction, or valvular incompetence--can only be made with lymphangiography. The mainstay of treatment is the reduction of edema by regular elevation and massage and external compression with elastic stockings. Pneumatic leggings are also helpful. Gross edema caused by peripheral obliteration may be reduced surgically by simple excision (Homans' operation) or complete excision and skin grafting (Charles' operation). Reflux through incompetent vessels may be prevented by vessel ligation. Obstruction by the iliac lymph nodes may be bypassed with an enteromesenteric pedicle.
FOXC2 mutations cause the lymphatic/ocular disorder Lymphedema-Distichiasis (LD), and Foxc2 haploinsufficient mice mimic this disorder. To determine if FOXC2 overexpression might also cause lymphatic and/or ocular abnormalities, we performed dynamic lymphatic imaging (Evans blue dye), ocular tissue examination, and metabolic profiles in mice: transgenic for FOXC2 with an adipocyte (aP2) promoter (aP2-FOXC2 Tg), heterozygous for targeted disruption of Foxc2 (Foxc2+/-), or compound heterozygous and transgenic (Foxc2+/-, Tg) compared to wild-type controls (WT). Foxc2+/-; aP2-FOXC2 Tg; and Foxc2+/-, Tg, exhibited LD's distinctive hyperplastic lymphatic phenotype characterized by increased number of lymphatic channels and lymph nodes as well as retrograde lymph reflux. Foxc2+/-, and Foxc2+/-, Tg but not aP2-FOXC2 Tg or WT showed an abnormal ocular phenotype. Previously described alterations in brown/ white fat distribution and lean phenotype in aP2-FOXC2 transgenics were confirmed. AP2-FOXC2 Tg immunohistochemistry disclosed aberrant FOXC2 expression in ectopic sites, especially embryonic heart. Lymphatic system links with fat metabolism are discussed.
PURPOSE: The capability of the interstitial magnetic resonance lymphography (MR-LG) using a widely available extracellular contrast agent, gadopentetate dimeglumine, for the visualization of normal and interrupted lymphatic drainage was tested in dog legs. MATERIALS AND METHODS: With a 7-day interval, 1 mL and 2 mL undiluted gadopentetate dimeglumine were injected intradermally into the dorsal foot of both hind legs of 12 dogs. T1-weighted 3-dimensional (3D) spoiled gradient-echo (repetition time/echo time = 6.7/1.6 ms) and maximum intensity projection (MIP) images covering the legs and pelvic region were acquired at 1.5 T for 10 minutes after 30 seconds gentle massage at the injection sites. These dogs also underwent the MR-LG with 2 mL of this contrast agent 7 days after surgical ligation of the popliteal lymphatic vessels. RESULTS: In the normal dogs, the interstitial MR-LG quickly and consistently visualized the direct connection of the lymphatic vessels and lymph nodes draining from the injection sites up to the inguinal region, without any late adverse effects. The enhancement effect was significantly greater with a 2-mL dose than with a 1-mL dose. After lymphatic obstruction, the MR-LG clearly revealed the anatomic compromises of the lymphatics, such as poor enhancement of the affected lymphatic vessels and lymph nodes, collateral lymphatic vessels, abnormal soft-tissue enhancement, and delayed lymphatic migration of the contrast agent. The topographic 3D images provided a comprehensive anatomy of these normal and compromised lymphatic drainage. CONCLUSION: Although gadopentetate dimeglumine is not lymphotropic, the interstitial MR-LG using this T1-contrast agent appears to have potential for quick and sufficient mapping of the lymphatic drainage from the injection sites and for the characterization of interrupted lymph flow.
OBJECTIVE: To determine whether an immunologic abnormality exists in patients with lymphatic malformation (LM). DESIGN: Retrospective case series. SETTING: Tertiary care pediatric hospital. PATIENTS: Twenty-one consecutive patients (11 male and 10 female) undergoing LM treatment. INTERVENTIONS: Clinical data (ie, age, clinical LM stage, radiographic appearance, and histologic findings) were correlated with complete blood cell count and detailed lymphocyte differential. Complete blood cell counts and lymphocyte subsets were measured in 21 and 18 patients, respectively. RESULTS: The average age at the time of testing was 67 months (range, 1-231 months). The patients were categorized according to LM stage, including 4 (19%) with stage 1, 4 (19%) with stage 2, 4 (19%) with stage 3, 7 (33%) with stage 4, and 2 (10%) with stage 5 disease. Radiographic LM appearance was macrocystic in 6 patients (29%), mixed macrocystic and microcystic in 8 (38%), and microcystic in 7 (33%). Complete blood cell count data demonstrated lymphocytopenia in 6 patients (29%). The results of the lymphocyte subset tests showed concomitant T-, B-, and natural killer (NK)-cell deficiency in 6 (33%) of 18 patients. All 6 patients with T-cell lymphocytopenia had normal neutrophil and platelet counts. Spearman rank and chi(2) analyses showed that LM stage 4 or 5 and microcystic LM were significantly associated with lymphocytopenia (P = .002 and P = .008, respectively). Histologic analysis did not demonstrate increased lymphocytes in any LM specimens. CONCLUSION: We found T, B, and NK lymphocytopenia in patients with large bilateral or microcystic LM. Although the relationship between lymphocytopenia and infection was not addressed in this study, the recognition of lymphocytopenia in patients with LM may have important clinical and prognostic implications.
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.
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UVB irradiation of the skin induces erythema, epidermal hyperplasia, vascular hyperpermeability, and edema formation. Previous studies have revealed that the cutaneous blood vasculature plays a critical role in the mediation of photodamage. In contrast, the role of lymphatic vessels, which play an essential role in the maintenance of tissue fluid balance, in the response to UVB irradiation has remained unknown. We report here that both acute and chronic UVB irradiation of murine skin results in prominent enlargement of lymphatic vessels. Surprisingly, these enlarged lymphatic vessels were functionally impaired and hyperpermeable, as detected by intravital lymphangiography. The expression levels of vascular endothelial growth factor (VEGF)-A but not of the known lymphangiogenesis factors VEGF-C or VEGF-D, were enhanced in UVB-irradiated epidermis. Targeted overexpression of VEGF-A in the epidermis of transgenic mice led to increased enlargement and leakage of lymphatic vessels after acute UVB irradiation, whereas systemic blockade of VEGF-A signaling largely prevented lymphatic vessel abnormalities and photodamage induced by UVB. Together, these findings identify lymphatic vessels as novel targets for UVB-induced cutaneous photodamage and suggest that VEGF-A mediates impairment of lymphatic vessel function, thereby contributing to the adverse effects of UVB irradiation on the skin.
Whereas clinical descriptions of grotesque lymphedema and standard light microscopy in human filariasis have elucidated the natural progression of this disease, the link between the nematode and vascular abnormalities including elephantiasis remains poorly understood. Accordingly, we examined the nature and distribution of lymphatic and blood vascular derangements in a variety of tissues and organs from 37 ferrets acutely and chronically infected with Brugia malayi and in 15 patients with Wuchereria bancrofti or Brugia malayi infestation (resected skin, subcutaneous tissue, and lymph nodes) using light and transmission electron microscopy, immunohistochemistry, and in vivo microscopy. In ferrets, eosinophilic abscesses and epithelioid and giant cell granulomas with fragmented worms in various stages of disintegration were found in multiple organs. Blood microvasculopathy consisted of endothelial hyperplasia, focal thickening and stenosis, vessel obliteration with marked perivascular infiltration of lymphocytes, plasma cells, eosinophils, and numerous large macrophages laden with a coarse golden-brown pigment. Endothelial ballooning and swelling, pavementing, denuding, scarring, and sludge formation were seen along with high endothelium in atypical locations. Dilated lymphatics were most prominent near adult worms and showed plump endothelium, thickened walls and valves, thrombus formation, and often perilymphangitis and adjacent tissue fibrosis. In vivo microscopy showed wriggling live adult worms in dilated incompetent sludge-filled groin lymphatics even when microfilaremia and peripheral edema were absent. In human tissues, in addition to "pachyderm" skin changes (keratosis, papillomatosis, acanthosis and collagen deposition), there was blood vessel and lymphatic vasculopathy similar to ferrets (angiocentric inflammation, congestion, vasculitis, thrombosis, thickened vessel walls, dilated lymphatics, lymphangitis, reactive lymph nodal hyperplasia and nodal fibrosis). These changes reflect generalized endothelial damage due to worm products, physical injury to valves and vessel walls from lymphatic-dwelling live worms, and host immune reactivity. Whereas adult worms target the lymphatic apparatus, their offspring and the host immune response primarily affects the blood microvasculature.
This article outlines the classification of vascular anomalies, which include vascular tumors and vascular malformations. We describe the nomenclature, diagnosis, and management of the different types of anomalies. Specific indications for pharmacologic and surgical intervention are discussed.
Oedema of the lower limbs may be associated with a number of factors, most commonly a lymphatic or venous abnormality. Generally effective treatment options are only available with venous disorders. The aim of this study is to show that a combination of duplex Doppler sonography and lymphoscintigraphy in patients with limb oedema will help to determine the aetiology and thus identify patients for whom treatment is appropriate. 32 patients presenting with unexplained oedema involving the lower limb have been studied. Each patient underwent Doppler sonography followed by lymphoscintigraphy. In 16 patients the lymphoscintigraphy was abnormal, showing absent or poorly visualized lymphatics. The Doppler study was abnormal in 17 patients, nine of whom went on to receive definitive surgical treatment. In five patients both studies were abnormal and there were six cases where both investigations were normal. It is concluded that in the great majority of referrals (82%), the combination of lymphoscintigraphy and Doppler ultrasound is a satisfactory means of assessment of patients with unexplained limb oedema.
OBJECTIVE: To determine the effect of inflammatory arthritis on lymphatic function in the upper limb. METHODS: Lymphoscintigraphy was used to measure lymphatic drainage in the upper limbs of 3 groups of patients: Group 1, inflammatory arthritis and edema (N = 10); Group 2, inflammatory arthritis and no edema (N = 18); and Group 3, healthy controls (N = 11). RESULTS: Lymphatic drainage was reduced significantly in Group 1 but was the same in Groups 2 and 3. Multiple regression analysis failed to show any effect of arthritis on lymphatic drainage. CONCLUSION: Inflammatory arthritis alone does not impair lymphatic drainage in the upper limbs. The results suggest that the presence of edema is primarily attributable to an unrelated abnormality influencing lymphatic function.