Subject(s)
Pregnancy Complications, Cardiovascular/diagnosis , Pregnancy Complications, Cardiovascular/therapy , Venous Thrombosis/diagnosis , Venous Thrombosis/therapy , Algorithms , Anticoagulants/adverse effects , Anticoagulants/therapeutic use , Exercise Therapy , Female , Humans , Platelet Aggregation Inhibitors/therapeutic use , Pregnancy , Pregnancy Complications, Cardiovascular/etiology , Risk Factors , Stockings, Compression , Thrombectomy , Thrombolytic Therapy , Venous Thrombosis/etiology , Vitamin K/antagonists & inhibitorsABSTRACT
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.
Subject(s)
Arteriovenous Malformations/diagnosis , Femoral Vein/abnormalities , Neurofibromatosis 1/diagnosis , Phlebography , Ultrasonography, Doppler, Color , Varicose Veins/diagnosis , Arteriovenous Malformations/genetics , Calcinosis/diagnosis , Calcinosis/genetics , Constriction, Pathologic/diagnosis , Female , Fibromuscular Dysplasia/diagnosis , Humans , Middle Aged , Muscle, Smooth, Vascular/pathology , Neurofibromatosis 1/genetics , Varicose Veins/geneticsABSTRACT
BACKGROUND: A chronic exertional compartment syndrome has only been observed in athletes and soldiers. In the vast majority, the disease affects the anterior compartment and the fibular muscle group, and only rarely the lateral and dorsal muscle compartments. Muscle tissue necrosis does not occur. In the course of venous diseases with a severe chronic venous stasis syndrome, a chronic venous compartment syndrome develops that differs considerably from the familiar functional syndrome. The predominant symptom is an uncurable cuff ulceration on the lower leg. PATIENTS AND METHODS: From 1993 to 1996 a total of 16 patients with a chronic fascial compression syndrome underwent surgery on 18 extremities. The crural fascia was resected and a mesh graft was applied. RESULTS: In the group of ten controls with healthy veins the average pressure in the deep compartment was 13.6 mmHg (range 9-17 mmHg) lying down and 29.9 mmHg (range 15-42 mmHg) standing up. In 14 patients with chronic fascial compression syndrome, the average pressure was higher, measuring 21.1 mmHg (range 8-47 mmHg) lying down and 62.5 mmHg (range 33-87) standing up. After surgery, the pressure dropped to 15.5 mmHg (range 5-24 mmHg) lying down and 34.5 mmHg (range 10-58 mmHg) standing up, but did not fall as low as the average values recorded in the control group or in the patient's healthy leg. The results from the standing up position were statistically significant (p = 0.003). Computed tomography showed major changes in the muscles indicating muscle atrophy and fatty degeneration. The crural fascia seemed to be incorporated in the scars of the subcutaneous tissue in large areas. After crural fasciectomy and healing of the ulceration, the tissue structure of the muscles recovered. CONCLUSIONS: In chronic fascial compression syndrome, the trellis arrangement of the collagen fibres becomes disordered. This results in a loss of flexibility during muscle contraction. Every step causes an increase of intracompartmental pressure and microstructural injury. The consequence is resection of the crural fascia.
