31/58. Identification and successful treatment of congenital microfistulas with the aid of directional Doppler.Hitherto unidentifiable and therefore untreatable congenital microfistulas were detected with the aid of continuous-wave directional Doppler and a fine-beam pencil probe. The microfistulas formed part of the Klippel-Trenaunay (K-T) syndrome. The management of one case is reported in detail and in three others salient features are touched upon. Careful clinical and radiological examination failed to demonstrate any arteriovenous microfistulas. When Doppler ultrasound scanning was carried out, two discrete fistulas were discovered. Their extent and direction were mapped out accurately. Incisions were made directly over the markings displaying a pulsating capillary tuft of vessels. Further dissection exposed a feeding arteriole which was less than 1 mm. in diameter. Excision of the vascular malformations resulted in the cure of the patient. In another patient with the K-T syndrome in whom a cutaneous hemangioma involved the whole lower limb, in spite of a thorough and systematic search with a Doppler, no microfistulas could be demonstrated. It is suggested that all patients suffering from the K-T syndrome should be examined by Doppler ultrasound in the hope that microfistulas which elude radiodiagnostic techniques might be detected and treated surgically.- - - - - - - - - - ranking = 1keywords = limb (Clic here for more details about this article) |
32/58. Case report 671: Arteriovenous malformation of the right lower extremity with associated intraosseous hemangiomatosis.Since extensive involvement of the lower extremity with arteriovenous malformations occurs often combined with the potential risk of hemorrhage, transcatheter embolization has become an attractive alternative to surgical resection [7, 9]. We describe an unusual case of extensive intraosseous hemangiomatosis, with a concomitant arteriovenous malformation that presented in a 35-year-old woman as synovitis in a knee. MR images demonstrated that the lesion was far more extensive than originally suggested and directed the subsequent biopsy and embolization. The differential diagnosis was discussed and the subject of intraosseous hemangiomatosis with associated AVM was considered in some detail.- - - - - - - - - - ranking = 52.084687855069keywords = extremity (Clic here for more details about this article) |
33/58. Unusual retinal manifestation in a combination of Sturge-Weber and Klipplel-Trenaunay syndrome--a case report.An unusual retinal manifestation of arteriovenous communications is reported in a case showing a combination of Sturge-Weber and Kippel-Trenaunay syndromes. The arteriovenous communications are thought to be similar to those seen in the limbs in Parkes-Weber syndrome. This retinal finding indicates that the three syndromes are intricately related to each other and may be an incomplete manifestation of a single pathophysiologic entity which may be named neurocutaneous angiomatosis.- - - - - - - - - - ranking = 1keywords = limb (Clic here for more details about this article) |
34/58. Anterolateral transthoracic transvertebral resection of an intramedullary spinal arteriovenous malformation. Case report.The case is reported of a 16-year-old girl with an anterior thoracic spinal cord arteriovenous malformation (AVM) who presented with subarachnoid hemorrhage and sudden change in lower-extremity strength. Spinal angiography revealed a Type II (glomus) intramedullary AVM at the T7-8 level fed by multiple branches of the anterior spinal artery. The AVM was successfully resected using an anterolateral transthoracic approach. The details of this approach and its use for surgery of anterior thoracic spine lesions are described.- - - - - - - - - - ranking = 10.416937571014keywords = extremity (Clic here for more details about this article) |
35/58. Peripheral arteriovenous malformation: diagnosis and localization by intraarterial injection of technetium-99m-MAA.radionuclide angiography with technetium-99m-labeled macroaggregates of albumin (99mTc-MAA), was successful in a single patient with a lower limb arteriovenous (AV) malformation, not only in diagnosis and quantitation of AV shunting, but also in localizing the site of shunting. This information proved useful to the angiographer, permitting a carefully tailored examination of the area of interest. This technique may hold promise as a preliminary examination in patients with limb AV malformations prior to angiography.- - - - - - - - - - ranking = 2keywords = limb (Clic here for more details about this article) |
36/58. klippel-trenaunay-weber syndrome variant.We describe a patient with soft-tissue and bony hypertrophy of all four limbs. The upper limbs were erythematous, with associated finger deformity. The soft-tissue irregular hypertrophy and engorgement subsided when the limbs were elevated. Retinal examination showed large, dilated, tortuous veins, with no dye leakage on fluorescein angiography. We feel that this case represents an unusual variant of the klippel-trenaunay-weber syndrome.- - - - - - - - - - ranking = 3keywords = limb (Clic here for more details about this article) |
37/58. Differentiation and treatment of hemangiomas and arteriovenous malformations.confusion exists about the differentiation of hemangiomas and other congenital vascular anomalies although they are the most common type of birth defect. During the past 10 years, these lesions have been reclassified on a pathophysiological basis which assists in defining their prognosis and treatment. Hemangiomas are broken down into three categories, those which affect the afferent capillary bed and may involute, efferent venous or lymphatic lesions which grow with the patient, and progressively growing vascular hamartomas which affect both venous and arterial limbs of the circulation and may threaten with limb loss or congestive heart failure. The afferent-efferent lesions require a multidisciplinary approach with close follow-up for their successful management.- - - - - - - - - - ranking = 2keywords = limb (Clic here for more details about this article) |
38/58. Nuclear magnetic resonance imaging: its diagnostic value in patients with congenital vascular malformations of the limbs.Congenital vascular malformations (CVMs) of the limb include simple and cavernous hemangiomas, microarteriovenous and macroarteriovenous fistulas, venous angiomas, and mixed CVMs. In differentiating these lesions, Doppler waveform analysis, labeled microsphere studies, arteriography, closed-space phlebography, and contrast-enhanced CT scans have all been advocated, but each has significant limitations. This article evaluates the ability of magnetic resonance imaging (MRI) in characterizing CVMs. Limb CVMs were evaluated by MRI in eight patients: four in the upper and four in the lower extremity. Before MRI, seven of the patients had arteriography, five had phlebography, and five had Doppler waveform analysis. MRI showed a highly cellular network with little arteriovenous flow in five patients. In four of these, arteriography and phlebography confirmed the presence of a predominantly venous or microfistulous anomaly. In the other three patients, MRI demonstrated high-flow arterial and venous channels and were confirmed by arteriography to have macrofistulous arteriovenous malformations. In all eight patients, MRI revealed the anatomic location and the longitudinal and transverse extent of the vascular malformation as well as their relationships with contiguous muscle groups, bones, and vessels. We conclude that CVMs of the limbs can be characterized accurately with MRI, with the anatomic extent, degree of cellularity, and flow characteristics readily gauged. Because MRI provides the same basic information supplied by angiography and the noninvasive laboratory and assesses anatomic extent and cellularity, it serves well as the primary diagnostic test for suspected CVMs, particularly in infants and children in whom competitive tests pose additional limitations.- - - - - - - - - - ranking = 16.416937571014keywords = extremity, limb (Clic here for more details about this article) |
39/58. Congenital arteriovenous fistulas in the chest wall.The clinical manifestations of large congenital, haemodynamically significant arteriovenous fistulas in the chest wall, originating from the subclavian area, are described in three cases and the results of surgical treatment are reported. Symptoms related to arteriovenous shunting were present in all three, with precordial pain and/or cardiac dysrhythmia, dyspnoea and intermittent painful ischaemia of the upper extremity. Altogether 19 operations were performed. The symptoms disappeared when fistulas were excised, but returned when fistula recurred. Lasting healing (9 years) was achieved in only one patient, after extensive ligation of all fistulas in the chest wall and sceletation of the subclavian artery from its proximal to its distal extent.- - - - - - - - - - ranking = 10.416937571014keywords = extremity (Clic here for more details about this article) |
40/58. tuberous sclerosis associated with partial gigantism in a child.The authors report a case of a six-year-old boy with seizures, mental retardation and some classic features of tuberous sclerosis, i.e., cutaneous hypomelanotic macules, poliosis, cyst-like lesions of the right upper limb and cerebral calcifications. From birth he showed partial gigantism of the first two fingers of the right hand associated with an arteriovenous malformation of the ulnar artery and agenesis of the radial artery. The EEG and CT scan studies are also reported. The occurrence of two dysgenetic disorders in one patient as a manifestation of disturbed embryogenesis at a very early stage of development is discussed.- - - - - - - - - - ranking = 1keywords = limb (Clic here for more details about this article) |
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