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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Klippel-Trenaunay (KT) syndrome is a rare, sporadic, congenital vascular disease of unknown etiology. We describe pulmonary findings in an 18-year-old male patient followed up since birth with the KT syndrome. The patient developed pleural and pericardial serous effusions that led to an open lung biopsy. Previous pulmonary findings have been limited to thromboembolic phenomena and pulmonary vein varicosities. On the other hand, reports of lymphatic hyperplasia, aplasia, and hypoplasia in KT have been limited to the extremities. For the first time, we describe lymphatic involvement of the lung in KT. The plexiform hyperplasia of the lymphatic channels with smooth muscle hyperplasia leading to lymphatic obstruction, pleural and pericardial effusions are new findings. The lymphatic nature of the plexiform channels was confirmed by immunohistochemistry. Von Willebrand factor and QD-END/10 monoclonal antibodies either did not react or reacted poorly with lymphatic endothelium, features used to distinguish lymphatic and venous endothelium. Ultrastructurally, the absence of basement membrane continuity further substantiated the lymphatic nature of the channels. From our findings, the lymphatic abnormality in the syndrome appears to be more generalized than previously thought. This entity should be distinguished from lymphangioleiomyomatosis to which it bears a superficial morphologic appearance.
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PMID:Pulmonary abnormalities in Klippel-Trenaunay syndrome. A histologic, ultrastructural, and immunocytochemical study. 132 65

The value of the vascular examination cannot be over-estimated. Symptoms of vascular disease present in the foot and lower extremity may actually be manifestations of severe life-threatening disease. Symptoms, their location, and the frequency and quality of the patient's pain often provide valuable clues for the clinician's diagnosis. Central nervous system symptoms, ocular disturbances, cardiac symptoms, impotence, or constitutional disturbances may all indicate systemic arterial disease. Risk factors for this disease include smoking, hypertension, hyperlipidemia, genetic predisposition, diabetes, emotional stress, and physical inactivity. Those factors attributable to hypercoagulability and venous disease are birth control pill use, estrogen chemotherapy, obesity, prolonged immobilization, paralysis, previous thrombotic episodes, venous stasis disease, and varicose veins. An accurate bilateral assessment of blood pressure, pulses, and capillary perfusion is of critical importance. Careful inspection of the extremity for trophic changes, skin color, texture, temperature, edema, ulceration, atrophy, or paresis, will provide clues of vasculopathy. A relatively accurate assessment of circulatory status may be obtained without the use of exotic instruments. Simple tests such as the elevation and dependency tests, capillary bed return test, venous filling time test, along with blood pressure, pulse, and possibly oscillometry data are valuable in arterial evaluation. Such venous tests as inspection, percussion, Homan's sign, Trendelenburg, and Perthes' tourniquet are useful in the determination of the presence of venous disease. Fortunately, over the past few years tremendous advances have been made in the technology of the vascular laboratory. If symptoms are discovered during the vascular history and physical examination, the complete noninvasive study will provide impressive data to quantitate and specifically establish the diagnosis.
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PMID:The vascular history and physical examination. 173 54

Three patients with intermittent exophthalmos were studied by computed tomography (CT) and colour-coded Doppler sonography (CCDS). By CT the possible diagnosis of an orbital varix was established. CCDS, however, with the dynamic evaluation and realtime direct imaging of flow and the possibility of examination in different positions facilitated the diagnosis of this orbital vascular disorder without the need for any contrast material. This technique may prove to be a useful adjunct to computed tomography for the evaluation of suspected vascular lesions of the orbit. Surgery confirmed the diagnosis in all patients.
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PMID:[Varicosis orbitalis. Its demonstration by color-coded Doppler sonography and computed tomography]. 191 37

Congenital dysplastic angiopathy is a syndrome consisting of vascular angiomata, congenital varicosities, and trophic changes of the soft tissue and the skeleton. Frequently referred to as Klippel-Trenaunay or Klippel-Trenaunay-Weber syndrome, it rarely affects the female genitalia. An 18-year-old woman underwent evaluation and treatment for Klippel-Trenaunay syndrome with vulvar involvement. Preoperative evaluation included consultation with pediatric surgeons, gynecologic surgeons, and an interventional radiologist as well as individual and family psychological counseling. Attention to intraoperative detail resulted in minimal operative blood loss and preservation of normal anatomy. A postoperative hematoma was treated aggressively with surgical evacuation and drainage. Six-month follow-up revealed functionally and cosmetically normal vulvar anatomy.
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PMID:Vulvar congenital dysplastic angiopathy. 215 31

A 24-year-old patient suffered from acute intestinal haemorrhage three times within 10 years. After the last bleeding coloscopy demonstrated mesenterial varices in the terminal ileum and in the colon between the caecum and the left flexure. There were no signs of portal hypertension or obstruction of the superior mesenteric vein either by sonography or by angiography. Arteriovenous malformations of the vascular ectasia type were identified as other potential causes of haemorrhage when selective digital subtraction angiography of the superior mesenteric artery was performed segmentally in the proximal jejunum. Since the bleeding stopped spontaneously, no curative therapeutic consequences resulted from the combined vascular disorder. The patient was advised to submit to treatment immediately if bleeding recurred, to enable an angiography to be performed in the acute stage.
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PMID:[Vascular anomalies as the cause of recurrent intestinal hemorrhages]. 232 64

Arteriovenous malformation is a rare vascular disease of the orbit that has to be differentiated from arteriovenous fistulas (e.g., of the carotid sinus), arterial aneurysms, the varices and the angiomas. It develops in the presence of an underlying constitutional maldifferentiation of the vascular bed but usually only becomes manifest in early adulthood. Typical clinical signs are exophthalmos, often with pulsation and thrill; episcleral and conjunctival vascular congestion; secondary glaucoma; and, to varying degrees in the late stage, restricted movement with double images as well as functional losses. The therapeutic measures otherwise routinely applied for such vascular deformities - e.g., surgical excision or, even better, embolization - have to be handled with ease in the orbit, since these vascular convolutions are also supplied in all cases by the ophthalmic artery. Two typical cases are presented to illustrate the clinical picture, diagnosis, differential diagnosis, and treatment possibilities.
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PMID:[Arteriovenous malformation of the orbit]. 271 30

A quantitative ultrastructural study has been made of the innervation of 461 arterioles in 114 skeletal muscle biopsies of patients with or without neuromuscular disease excluding diabetes and autonomic neuropathy. In 18 controls the number of nerves and Schwann cells around each vessel was related to the size of the vessel, whether the vessel was within a muscle fascicle or between muscle fascicles. The innervation of arterioles increased with increased diastolic blood pressure. There was no statistically significant change in innervation with increased systolic blood pressure or with age, from 4 to 85 years. In 96 cases of neuromuscular disease and especially in motor neurone disease, axonal varicosities in cross section tended to be larger, more often contained no vesicles or only a few and had altered satellite cell cover depending on the location of the arteriole. Whilst the numerical density of Schwann cells did not change with disease, fewer varicosities were identified within Schwann cells in motor neurone disease, metabolic myopathy and neuropathy and myopathy due to toxins or vascular disease. Preterminal axons in nerve fascicles adjacent to arterioles were lost in polymyositis and muscle disease due to toxins or vascular disease. In polymyositis, metabolic myopathy and motor neurone disease there was some evidence of compensatory nerve sprouting, either in the nerve fascicles or in the adventitia of the arterioles. These structural changes may be related to the changes in blood flow or vascular reactivity described by others in motor neurone disease, polymyositis and metabolic myopathy. It is concluded that the ultrastructure of the vascular innervation of human skeletal muscle is similar to that in other mammals and is changed more with increased diastolic blood pressure and neuromuscular disease than with age.
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PMID:Vascular innervation in human skeletal muscle with and without neuromuscular disease. A quantitative ultrastructural study with references to the effects of age and different blood pressure. 318 43

Cutis marmorata telangiectatica congenita (CMTC) is characterized by a persistent vascular mottling of the skin, usually on the limbs. Four cases (2 males and 2 females) followed up for varying lengths of time are reported. In the first patient, whose vascular lesions were mild, careful re-examination after 10 years revealed some scars. In the second patient the lesions were located on the right side of the body, notably on the leg; 6 years later atrophic scars and a brownish plaque were visible on that leg. In the third patient the lesions occupied almost the entire body, although one side was more affected than the other and hypotrophic; the child's face was asymmetrical because of hypoplasia of the jaw and curvature of the nose; after 1 year the patient's general condition was satisfactory and the skin lesions were less evident but still present. In the fourth patient hypoplasia of a lumbar vertebra was discovered; the skin lesions were similar to those observed in the third patient. Examination of the deep vessels, performed whenever possible, did not show any abnormality, but the possibility of future vascular defects, such as varicosities, must be considered. While some authors emphasize the functional character of the disease, in our opinion the high frequency of multiple associated congenital abnormalities makes CMTC not only a vascular disorder but also a syndrome including other neuroectodermal and mesodermal defects. Therefore, any patient presenting with the cutaneous changes typical of CMTC should be examined with this in mind.
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PMID:[Cutis marmorata telangiectatica congenita. 4 new cases and review of the literature]. 332 46

Out of 334 patients operated on for PHPT in the years 1956-79, 34 (10.2%) had died before the end of the year 1980. In the sex and age matched control material of 334 patients operated on at the same time for varicose veins, appendicitis or haemorrhoids the mortality was 21 (6.3%). The difference in mortality between these groups was statistically significant (p less than 0.05). The mean age at death of the PHPT patients was 65 years and that of the control patients 67 years. The PHPT patients who died differed from the whole PHPT material in higher mean age at operation (61 years v. 53 years), higher preoperative serum calcium values (3.31 mmol/l v. 3.08 mmol/l), more frequently elevated serum creatine preoperatively (44% v. 17%) and higher mean weight of the removed adenomas (3300 mg v. 2000 mg). The PHPT patients who died had also more often the severe form of PHPT: 55% of the patients with hypercalcaemic crises and 24% of the patients with cystic bone changes had died, whereas the respective percentage was 4% in the renal stone group. Four of the PHPT patients had died of uraemia, and, in addition, four patients had progressive renal damage. 18 PHPT patients had died of cardiac disease, four patients of a cerebrovascular attack and one patient of other vascular disease. There were eight cardiovascular deaths in the control group (p less than 0.01). There were no differences in other causes of death, such as malignant tumours, between the groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Causes of death in patients previously operated on for primary hyperparathyroidism. 401 16

Congenital dysplastic angiopathy (Klippel-Trenaunay-Weber Syndrome) consists of angiomata and congenital varicosities with trophic changes of soft tissues and various parts of the skeletal system. Involvement of female genitalia is rare and has been reported to occur in only four patients. The authors treated a patient with a lymphangioma of the vulva associated with congenital dysplastic angiopathy.
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PMID:Congenital dysplastic angiopathy (Klippel-Trenaunay-Weber syndrome) with vulvar involvement. A case report. 632 79


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