1/49. 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 = vessel (Clic here for more details about this article) |
2/49. Juvenile rheumatoid arthritis and lymphoedema: lymphangiographic aspects.We report a 5 1/2-year-old boy with juvenile rheumatoid arthritis (JRA) and lower-limb lymphoedema. US, MRI and lymphangiography were performed. Based on the lymphangiographic study, we propose a pathogenesis based on obstruction of normal superficial lymphatic vessels in the affected limb. This is discussed with other pathogenetic factors proposed in the 16 previously reported cases of lymphoedema complicating JRA.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/49. Congenital balloon digits in two neonates caused by constriction rings.Balloon digits were found in two neonates with congenital constriction ring syndrome. The affected digits were the right long finger and right great toe. They were surgically treated at the age of 10 and 9 days, respectively. Morphologic improvement was dramatic after surgery. In cases with extensive enlargement, severe cyanosis, redness, and no subsidence of edema within several days after birth, early operative treatment may be necessary to maintain digit viability and prevent autoamputation due to circulatory embarrassment. It can also be helpful to prevent fibrosis of the subcutaneous tissue. Pathologic examination revealed marked proliferation of fibrous tissue and lymphatic vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/49. Vulvar hypertrophy with lymphedema. A mimicker of aggressive angiomyxoma.We report the case of a 43-year-old quadriplegic woman with bilateral vulvar enlargement. The clinical impression was labial hypertrophy, but the microscopic features mimicked aggressive angiomyxoma because of the location, hypocellular proliferation of fibroblastic cells in an edematous-myxoid stroma, and vessels with perivascular collagen deposition, which simulated the thick-walled vessels of aggressive angiomyxoma. Since the lesion lacked true thick-walled vessels and contained ectatic tortuous lymphatics, the pathologic interpretation was lymphedema. This vulvar lesion should be recognized to prevent the misdiagnosis of aggressive angiomyxoma.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
5/49. lymphangioma circumscriptum of the vulva.lymphangioma circumscriptum is a benign disease of the lymph ducts and an unusual pathologic process that rarely affects the vulva. The etiology of this lesion is not clear, but obstruction of the lymph vessels has been suggested as a possible cause in some cases. We report the case of a 44-year-old woman with lesions similar to lymphangioma circumscriptum of the vulva and chronic idiopathic lymphedema of the lower right limb. Because there was no obvious cause, we propose that the lymphangioma was caused by the lymphedema.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/49. Severe lower limbs lymphedema of late onset revealing polysplenia syndrome--a case report.Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/49. Congenital lymphedema presenting with increased nuchal translucency at 13 weeks of gestation.Congenital lymphedema is an autosomal dominant condition characterized by chronic tissue swelling caused by deficient lymphatic drainage due to hypoplastic/aplastic lymphatic vessels and usually affecting the lower limbs. The locus of the gene has been identified in the long arm of chromosome 15. We report one case of congenital lymphedema presenting with increased nuchal translucency at 13 weeks of gestation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/49. prenatal diagnosis of Milroy's primary congenital lymphedema.Milroy's primary congenital lymphedema (PCL) (hereditary lymphedema type I, Milroy disease) is present at birth, and mostly affects the dorsal aspects of feet. It is mostly a life-long condition but does not affect longevity. Complications are rare except for chronic discomfort and warmness of affected areas. PCL is an autosomal dominant disease with incomplete penetrance due to a mutation in the gene locus encoding for VEGFR3 with resultant dysgenesis of microlymphatic vessels. We report on two fetuses where ultrasonographic examination at 15 weeks of gestation showed significant edema of the dorsal aspects of both feet with no evidence of other major malformations. Whereas in one fetus the edema resolved completely, it persisted in the second fetus and proved after birth to be of lymphedematous nature. To the best of our knowledge, this is the first report of early prenatal diagnosis of primary congenital lymphedema via fetal ultrasonographic examination and of spontaneous resolution of lymphedema during fetal life.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/49. lymphedema-lymphangiectasia-mental retardation (Hennekam) syndrome: a review.The Hennekam syndrome is an infrequently reported heritable entity characterized by lymphedema, lymphangiectasia, and developmental delay. Here we add an additional 8 patients, and compare their findings to the 16 cases from the literature. The lymphedema is usually congenital, can be markedly asymmetrical, and, often, gradually progressive. Complications such as erysipelas are common. The lymphangiectasias are present in the intestines, but have also been found in the pleura, pericardium, thyroid gland, and kidney. Several patients have demonstrated congenital cardiac and blood vessel anomalies, pointing to a disturbance of angiogenesis in at least some of the patients. Facial features are variable, and are chiefly characterized, in a typical patient, by a flat face, flat and broad nasal bridge, and hypertelorism. Facial features are thought to mirror the extent of intrauterine facial lymphedema, or may be caused by lymphatic obstruction that affects the early migration of neural crest tissue. Other anomalies have included glaucoma, dental anomalies, hearing loss, and renal anomalies. The psychomotor development varies widely, even within a single family, from almost normal development to severe mental retardation. Convulsions are common. The existence of 10 familial cases, equal sex ratio, increased parental consanguinity rate (4/20 families), and absence of vertical transmission are consistent with an autosomal recessive pattern of inheritance. It seems likely that most (but not all) manifestations of the entity can be explained as sequences of impaired prenatal and postnatal lymphatic flow, suggesting that the causative gene(s) should have a major function in lymphangiogenesis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/49. Post-therapeutic lymphedema: scintigraphy before and after autologous lymph vessel transplantation: 8 years of long-term follow-up.The function of lymphatic vessel grafts was proved in 12 patients with lymphedema of the upper extremity by visual and semiquantitative evaluation of a preoperative baseline study and scintigraphic follow-up data for a period of 8 years after microsurgical treatment. The transplantation site was an upper extremity. In 11 of 12 patients, lymphatic function improved after autologous lymphatic vessel transplantation compared with preoperative findings. This could be verified by a statistically significant decrease of the transport index ( < 0.01), clear demonstration of lymph nodes, and a less diffuse distribution pattern of the Tc-99m-labeled nanocolloids. In three patients, the vessel graft could be detected by scintigraphy. lymphoscintigraphy combined with semiquantitative evaluation of lymphatic transport kinetics has been shown to be an easy and reliable method to assess lymphatic function before and after autologous lymph vessel transplantation.- - - - - - - - - - ranking = 8keywords = vessel (Clic here for more details about this article) |
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