[Vascular manifestation of von Recklinghausen neurofibromatosis: case report of venous dysplasia].
We present the case report of a patient with neurofibromatosis and regional dysmorphism in the superficial femoral vein. Colour coded duplex sonography revealed an incidental finding: thickening of a short portion of the vein wall with calcification. This unusual finding strongly suggests a causal relationship. Pathological processes in the arteries have often been described in patients with von Recklinghausen's disease (neurofibromatosis). Dysplastic and hyperplastic reactions of the intramural nerve tissues and the smooth muscle elements in the vascular wall have been observed. This leads to the formation of aneurysms and arteriovenous fistulas and to stenosing processes in the peripheral, visceral and cerebral arteries. However, involvement of the veins in type 1 von Recklinghausen's neurofibromatosis has only been reported in old histopathological studies of medium-sized and small vessels.
Präve
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Venenzentrum Frankfurt am Main. Fevarp@aol.com
Hach-Wunderle
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V
Hach
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D001165:Arteriovenous Malformations / Q000175:diagnosis* / Q000235:genetics; D002114:Calcinosis / Q000175:diagnosis / Q000235:genetics; D003251:Constriction, Pathologic / Q000175:diagnosis; D005260:Female; D005268:Femoral Vein / Q000002:abnormalities*; D005352:Fibromuscular Dysplasia / Q000175:diagnosis; D006801:Humans; D008875:Middle Aged; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000235:genetics; D010690:Phlebography; D018615:Ultrasonography, Doppler, Color; D014648:Varicose Veins / Q000175:diagnosis* / Q000235:genetics