1/29. Autonomic regulation of lymphatic flow in the lower extremity demonstrated on lymphoscintigraphy in patients with reflex sympathetic dystrophy.PURPOSE: nuclear medicine techniques were used to show that the peripheral lymphatics are under autonomic control in much the same way as the blood vessels that supply the same anatomic region. methods: Three patients with complex regional pain syndrome type 1 (reflex sympathetic dystrophy) involving a lower extremity were evaluated using three-phase bone scintigraphy and peripheral lymphoscintigraphy. Each patient was treated with ipsilateral chemical lumbar sympathectomy, and lymphoscintigraphy was repeated within several days of the procedure. RESULTS: All three patients had evidence of decreased flow (compared with the contralateral extremity) to normal flow after ipsilateral sympathectomy. Bone scintigraphy, before and after sympathectomy, was difficult to interpret because of the effects of altered weight bearing. Two patients who had unilateral peripheral edema showed marked improvement after sympathectomy and increased lymphatic flow. CONCLUSIONS: Peripheral lymphatic function is controlled by the autonomic nervous system. In reflex sympathetic dystrophy, peripheral edema may be caused by an increased sympathetic stimulus to the lymphatics. Further study of this phenomenon may show that nuclear medicine studies, such as bone scintigraphy and lymphoscintigraphy, can be used to distinguish patients who will benefit from sympathectomy from those who will not, thereby obviating invasive testing and unnecessary invasive treatment.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/29. Acquired immune deficiency syndrome-related hyperkeratotic Kaposi's sarcoma with severe lymphoedema: report of five cases.Kaposi's sarcoma (KS) is the most frequent neoplasm in acquired immune deficiency syndrome (AIDS) patients. Whereas typical cases present as erythematous, plaque or nodular lesions, hyperkeratotic variants of AIDS-associated KS are rare. We describe five patients with AIDS-associated KS with hyperkeratosis and lymphoedema as prominent features. We also speculate on its pathogenesis.- - - - - - - - - - ranking = 2.0044820440608keywords = neoplasm (Clic here for more details about this article) |
3/29. Primary cutaneous B-cell lymphoma of the leg in a chronic lymphedematous extremity.This is a case report of a woman who had chronic lymphedema on one leg and who developed a primary cutaneous large B-cell lymphoma of the leg at that site. She received radiotherapy and did not show any systemic involvement thereafter. Other neoplasms may appear in a clinical setting of chronic lymphedema, namely, lymphangiosarcoma (Stewart-Treves), melanoma, and metastatic carcinoma. There are four other reports in the English literature of cutaneous lymphoma arising in an extremity with chronic lymphedema.- - - - - - - - - - ranking = 2.0044820440608keywords = neoplasm (Clic here for more details about this article) |
4/29. Massive localized lymphedema: additional locations and association with hypothyroidism.We report the second series of a new entity called "massive localized lymphedema in morbidly obese patients" (MLL), recently described in medical literature. Our 6 cases present additional locations as well as an association with hypothyroidism. Huge masses, of longstanding duration ranging from 9 months to 8 years, afflicted the thigh, popliteal fossa, scrotum, suprapubic and inguinal region, and abdomen of morbidly obese adults. Although clinical impressions were generally of a benign process, including lipoma and recurrent cellulitis, the possibility of a malignant neoplasm could not be eliminated. Poorly defined and non-encapsulated, these skin and subcutaneous lesions were most remarkable for their sheer size, measuring 50.6 cm in mean diameter (range, 38-75 cm) and weighing a mean of 6764.5 g (range, 2,060-12,000 g) The overlying skin exhibited the induration and peau d'orange characteristic of chronic lymphedema. Grossly and histologically, a prominent marbled appearance, rendered by fibrous bands intersecting lobules of adipose tissue, simulated sclerosing well differentiated liposarcoma. However, the absence of atypical stromal cells, atypical adipocytes, and lipoblasts precluded the diagnosis of well differentiated liposarcoma. Instead, reactive features, encompassing lymphatic vascular ectasia, mononuclear cell infiltrates, fibrosis, and edema between the collagen fibers, as well as ischemic changes including infarction and fat necrosis, established the diagnosis of MLL. Although the pathogenesis of MLL may be as simple as obstruction of efferent lymphatic flow by a massive abdominal pannus and/or prior surgery, the presence of hypothyroidism in 2 of our patients suggests an alternative pathogenesis. Recognition of this entity by both clinicians and pathologists should avert a misdiagnosis as a low-grade liposarcoma.- - - - - - - - - - ranking = 2.0044820440608keywords = neoplasm (Clic here for more details about this article) |
5/29. Massive hemangioma or lymphedema? A case with a diagnostic dilemma.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.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/29. Spread of melanoma after lymphatic drainage: relaunching the debate.Secondary lymphoedema of the leg can result in disruption of lymphatic vessels following lymph node surgery. Evidence supports the use of complex decongestive physiotherapy (CDP) in such cases, despite the possibility of tumour recurrence due to this therapy in cancer patients. We present the case of a 52-year-old woman who developed in-transit metastases and systemic evaluable disease one month after starting CDP for secondary lymphoedema of the leg.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/29. Kaposiform hemangioendothelioma associated with Milroy's disease (primary hereditary lymphedema).Kaposiform infantile hemangioendothelioma (KHE) is a rare recently characterized, locally aggressive, endothelial-derived neoplasm that occurs exclusively in the pediatric age group. Milroy-Nonne disease (primary hereditary lymphedema) is an uncommon congenital entity with familiar history of lower limb edema as typical clinical features. An 8-year-old boy developed a hard painless mass in the right leg 7 years after the diagnosis of congenital primary lymphedema of the right lower extremity. Histopathological analysis of the tumor showed the typical findings of the KHE. To our knowledge this is the first reported case of a KHE engrafting on this infrequent benign lymphatic anomaly.- - - - - - - - - - ranking = 2.0044820440608keywords = neoplasm (Clic here for more details about this article) |
8/29. Successful complex decongestive physiotherapy for lymphedema and lymphocutaneous reflux of the female external genitalia after radiation therapy.Development of secondary lower extremity lymphedema after cervical cancer is common. However, severe lymphedema of the female genitalia and vesicular cutaneous lymphatic reflux without lower limb lymphedema after treatment of cervical cancer is rare. We report successful complex decongestive physiotherapy (CDP) in a 53-year-old female who developed recurrent folliculitis, lymphocutaneous reflux, warty change of the right labium majora, and lymphedema involving the external genitalia after receiving hysterectomy, pelvic lymph node dissection, and radiation. CDP included skin care, manual lymphatic drainage, exercise, and use of a hip spica bandage and panty girdle garment. Oral antibiotics were prescribed to control the skin infection. Lymphatic discharge and folliculitis were markedly improved after a 3-week course of treatment. Traditional conservative treatment with pneumatic compression and massage is ineffective and not suitable in controlling lymphedema of the external genitalia. Use of CDP may help to relieve the symptoms and recurrent infection in patients with this condition.- - - - - - - - - - ranking = 5keywords = complex (Clic here for more details about this article) |
9/29. Lymphstasis in a boy with noonan syndrome: implication for the development of skeletal features.We report on a Japanese boy with noonan syndrome who had short stature, bilateral cryptorchidism, poor pubertal development, mild mental retardation, complex cardiac lesions consisting of hypertrophic cardiomyopathy, mitral valve stenosis and insufficiency, subvalvular aortic stenosis, and single coronary artery, and various dysmorphic features including hypertelorism, epicanthic folds, low set malrotated ears, high arched palate, micrognathia, webbed neck, low posterior hairline, shield chest, pectus excavatum, cubitus valgus, borderline short metatarsals, lymphedema, redundant skin, and nail dysplasia. Because of marked lymphedema in the bilateral lower legs, lymphatic scintigraphy was carried out at 13.3 years of age, indicating extreme lymphstasis in the lower extremities, severe lymphstasis in the forearm, the elbow, and the axillary regions, moderate lymphstasis around the ascending aorta, and mild lymphstasis in the bilateral lungs. The results, in conjunction with those suggested in turner syndrome, imply that lymphatic hypoplasia/dysplasia and resultant distended lymphatics and lymphedema are relevant to the development of not only soft tissue and visceral anomalies but also skeletal anomalies in noonan syndrome.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
10/29. lymphedema of the extremities: prospects of microsurgical treatment.Presented herein is an experience with the treatment of 60 patients with lymphedema of the extremities, who had undergone transplantation of "lymphatic flaps". The treatment success is analyzed in both free microsurgical autotransplantation of tissue complexes (52 patients) containing intact lymphatic structures and in transposition (8 patients). The authors evaluate the potential of radio-nuclide diagnostic techniques for quantification of the effectiveness of surgical treatment. After transplantation and transposition of different types of "lymphatic flaps" good results were obtained in 36 (60%) patients. The prospects of the use of "lymphatic flaps" are assessed from the standpoint of the current concepts related to the surgical treatment of lymphedema of the extremities.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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