影像学指标在股髋关节撞击综合征诊断中的价值

目的：本研究前瞻性的分析股髋关节撞击综合征（ Femoroacetabular impingement syn－drome , FAI）的影像学表现,探寻FAI诊断及分型的相关影像学指标,并与手术结果相对照,以期有助于术前对手术方案制定。方法对20例拟诊的FAI患者进行CT检查。观察指标如下：从股骨头异常突起、股骨颈a角、髋臼缘软骨增生硬化。结果20例患者中,共有20个髋关节诊断为FAI,其中左侧9例,右侧11例。股骨颈a角的范围为31．3～69．1,股骨颈a角的平均值为43．55°。股骨头异常突起位于股骨头颈连接处前部或前上部,共11例,占55％,其股骨颈a角的平均值为52．69°;髋臼边缘软骨增生6例,占30％,其股骨颈a角的平均值为39．8°;股骨头颈连接处前部或前上部骨质异常突起与髋臼边缘软骨增生并存3例,占15％,其股骨颈a角的平均值为38．9°。结论 CT诊断指标即股骨头异常突起、a角、髋臼缘软骨增生硬化有助于FAI的诊断、分型及术前病情程度评估,并指导术式选择。     

Protrusio acetabuli in Marfan's syndrome

Marfan's syndrome is an autosomal dominant disorder of connective tissue, commonly involving the cardiovascular, ocular, and skeletal systems. Revised criteria for the clinical diagnosis of Marfan's syndrome regard skeletal involvement as a major criterion if at least four of eight typical skeletal manifestations are present, one of which is protrusio acetabuli. Using Kulman's method to determine the presence of protrusio, we analysed the pelvic X-rays of 15 patients with Marfan's syndrome and 15 controls. Protrusio was present in 47% (7/15) of Marfan patients, compared with 7% (1/15) of controls (P = 0.035). Using the revised criteria, the presence of protrusio would have affected the final diagnosis of Marfan's syndrome in only one patient out of 15. Therefore, we recommend that a pelvic X-ray is reserved for those cases in which the presence of protrusio will alter the final diagnosis. With regard to the radiological assessment of protrusio, in our opinion this can be performed simply and reliably using the position of the acetabular line alone.     

Protrusio acetabuli. An update on the primary and secondary acetabular protrusion

Acetabular protrusion refers to intrapelvic displacement of the medial wall of the acetabulum and is defined as inward movement of the acetabular line so that the distance between this line and the laterally located ilioischial line is 3 mm or more in adult men and 6 mm or more in adult women. As discussed in this article, acetabular protrusion may be found in many bone disorders such as degenerative joint disease, Paget's disease, rheumatoid arthritis, ankylosing spondylitis, osteomalacia, Marfan's disease and as an effect of irradiation. Protrusio acetabuli appearing in absence of any recognizable cause is termed primary acetabular protrusion or Otto pelvis. Primary acetabular protrusion usually affects both hips in young to middle aged women with a history of diminished abduction, rotation and hip pain since puberty. Radiographically one notes a bilateral axial migration of the femoral head without joint space loss and with moderate degenerative changes.     

升主动脉瘤的外科治疗分析

目的总结升主动脉瘤的外科治疗经验。方法23例主动脉根部病变患者(年龄22~55岁,平均31.8岁),其中Mafan’s综合征12例,Stanford A型主动脉夹层8例,主动脉瓣狭窄伴升主动脉扩张3例。手术类型:Bentall手术18例,Wheat手术3例,David手术1例,全弓置换加象鼻手术1例。结果全组手术死亡1例,术后随访12例平均41个月,无并发症。结论Bentall手术是治疗升主动脉瘤的主要术式,主动脉瓣叶正常,瓣膜轻度关闭不全,瓣环无明显扩张,David手术可以减少换瓣后服用抗凝药物所致的出血、血栓等并发症。     

Non-marfan idiopathic medionecrosis (cystic medial necrosis) presenting with multiple visceral artery aneurysms and diffuse connective tissue fragility: Two brothers

Two brothers with multiple visceral artery aneurysms or dilatations and diffuse connective tissue fragility who did not have clinical features of Marfan syndrome are reported. One presented with retroperitoneal hemorrhage during angiography, and idiopathic medionecrosis was proved by resection of the aneurysms. These cases belong to the heterogeneous group of Marfan syndrome. The angiographical features (multiple dilation of visceral arteries) suggests fragility of connective tissue and is predictive of hazards during and after a catheterization and operation.     

Renal artery stenosis in patients with aortic dissection: increased prevalence

The authors retrospectively analyzed the prevalence of renal artery stenosis in 63 consecutive patients with aortic dissection who underwent thoracic and abdominal aortography. Ten patients (16%) had renal artery stenosis, five with atherosclerosis and five with fibromuscular lesions. Risk factors for aortic dissection were Marfan disease in nine patients, bicuspid aortic valve in one, and hypertension in 54 (including seven patients with Marfan syndrome). If the patients with Marfan syndrome and the patient with the bicuspid aortic valve are excluded, renal artery stenosis was present in 10 of 53 patients (19%) when the cause of dissection was presumably hypertension. This finding suggests that renovascular hypertension is a greater risk factor for aortic dissection than is essential hypertension. The success of angiotensin converting enzyme inhibitors and percutaneous transluminal renal angioplasty (PTRA) in controlling renovascular hypertension has been proved. In this series, emergent PTRA successfully controlled the hypertension in one patient with a type B dissection, resulting in an excellent clinical outcome. Angiography should be routinely performed on patients with aortic dissections to evaluate for renal artery stenosis.     

Evaluation of three different measurement methods for dural ectasia in Marfan syndrome

AIM: Dural ectasia is a major diagnostic criterion for Marfan syndrome using the Ghent nosology. Our aim was to evaluate the efficacy of three different radiological methods previously proposed for the assessment of dural sac diameter in Marfan syndrome. METHODS: Marfan syndrome was diagnosed in our study using the Ghent criteria, disregarding dural ectasia as a criterion. Three proposed radiological methods were applied to measure dural sac diameter, examined for 41 patients (18 patients with and 23 without Marfan syndrome) by computed tomography or magnetic resonance imaging. RESULTS: Using Oosterhof's method, 94% of the patients with and 44% of the patients without Marfan syndrome fulfilled the criteria of dural ectasia. According to Villeirs, dural ectasia was diagnosed in 18% of the patients with and in none of the patients without Marfan syndrome. With Ahn's method, dural ectasia was found in 72% of the patients with and in 44% of the patients without Marfan syndrome. In only two patients with Marfan syndrome was dural ectasia diagnosed by all three methods. CONCLUSION: Our results reveal overt discrepancy between the three methods of assessing dural ectasia. Considering the key role played by dural ectasia in reinforcing the diagnosis of Marfan syndrome according to the Ghent nosology, a standardized and reliable method should be sought.     

Idiopathic hyperphosphatasia

Idiopathic hyperphosphatasia is a rare autosomal recessive bone disorder, characterized by excessive bone resorption and bone formation. The radiographic appearances include widening of the diaphyses, vertebral osteoporosis, acetabular protrusion, and thickening of the skull vault. There is considerable variability in phenotype, with some cases diagnosed in infancy and others in later childhood. Most cases appear to arise from inactivating mutations in the gene encoding osteoprotegerin, a product of osteoblasts that is critically involved in osteoclastogenesis. Treatment with inhibitors of bone resorption (calcitonin or bisphosphonates) is successful in ameliorating some aspects of the disorder.     

Patterns of femoral head migration in osteoarthritis of the hip: a reappraisal with CT and pathologic correlation

Although medial, superior, and axial patterns of migration of the femoral head in osteoarthritis of the hip have been well described, it is not clear what anatomic and biomechanical factors determine the direction of migration. The authors studied 22 patients with bilateral (11 patients) or unilateral (11 patients) osteoarthritis by means of conventional radiography and computed tomography (CT) to define any relationships between migration in the coronal plane and that in the transverse plane and to determine whether femoral anteversion, acetabular anteversion, femoral neck-shaft angle, or acetabular inclination were related to particular migration patterns. Anterior migration was evident in 14 of the 19 hips with a superior migration pattern, whereas posterior migration was present in five of the seven hips with a medial migration pattern. In the remainder of cases, no migration in the transverse plane was present. Femoral anteversion as determined with CT, femoral neck-shaft angle, angle of acetabular inclination, and acetabular anteversion angle in this relatively small sample were all found to be within normal limits and appeared to have no influence on the occurrence of a specific pattern of femoral head migration.     

Marfan syndrome

Marfan syndrome is a heritable disorder of the connective tissue that affects the cardiovascular, skeletal and ocular systems, and often involves the skin, nervous system and lungs. Historically, a person with Marfan syndrome had a poor prognosis due to the cardiovascular effects of this disorder. The life expectancy has improved dramatically over the past 30 years for individuals with Marfan syndrome because of aggressive medical and molecular research and advances in surgical technology. This article discusses the history, genetics, manifestations, diagnosis and treatment of Marfan syndrome.     

Quantitative assessment of dural ectasia as a marker for Marfan syndrome

PURPOSE: To establish normal values for lumbosacral dural sac dimensions with magnetic resonance (MR) imaging and to use these values to assess the sensitivity and specificity of dural ectasia as a marker for Marfan syndrome. MATERIALS AND METHODS: MR imaging was performed to measure dural sac diameter (DSD) from L1 through S1 in 44 adult patients with Marfan syndrome and in 44 matched control subjects. DSD values were corrected for vertebral body size, yielding dural sac ratios (DSRs). The control subjects served to establish the upper limit of normal DSR values at the L1 through S1 levels. RESULTS: Cutoff values for normal DSRs for L1 through S1 were 0.64, 0.55, 0.47, 0.48, 0.48, and 0.57. Significant DSR differences were shown at all levels between patients with Marfan syndrome and control subjects (P <.001 at all levels). At L1 through S1, the sensitivity of dural ectasia as a marker for Marfan syndrome was 45%-77%, and the specificity was 95% or greater. By combining levels L3 and S1, dural ectasia as a marker for Marfan syndrome yielded a sensitivity of 95% (42 of 44 patients) and a specificity of 98% (43 of 44). The presence of dural ectasia excelled, compared with the presence of other Marfan syndrome manifestations in the patient population. CONCLUSION: Abnormal DSR values at L3 or S1 can be used to identify Marfan syndrome with 95% sensitivity and 98% specificity.     

马凡综合征主动脉病变MRI诊断的研究

目的：评价ＭＲＩ对马凡综合征主动脉病变诊断的价值。材料与方法：分析了２０例马凡综合征主动脉病变的ＭＲＩ所见，并与１１例非马凡综合征所致的升主动脉扩张或动脉瘤进行对照研究。结果：主动脉窦和近段升主动脉瘤样扩张是马凡综合征最重要而常见的心血管病变，“瘤体”与正常段或轻度扩张的主动脉分界清楚是颇具特征性的马凡综合征心血管病变，继发于主动脉瓣狭窄和（或）关闭不全的升主动脉扩张大多呈普遍轻度扩张，而主动脉窦大多无明确扩张，其ＭＲＩ所见与马凡综合征升主动脉病变明显不同。结论：ＭＲＩ在马凡综合征主动脉病变的诊断中有优良的价值。     

Digital subtraction angiography of the thoracic aorta

Forty-three patients with acquired and congenital abnormalities of the thoracic aorta were studied using digital subtraction angiography (DSA) after an intravenous bolus injection of 40 ml of contrast material. Abnormalities studied included coarctation, pseudocoarctation, Marfan syndrome, cervical aorta, double aortic arch, aneurysm, dissection, and tumor. Twenty-four patients also had conventional angiography. DSA was accurate in 95% of cases; in the other 5%, involving patients with acute type I dissection, the coronary arteries could not be seen. The authors concluded that in 92% of their patients, DSA could have replaced the standard aortogram.     

Pigmented villonodular synovitis of the hip: review of radiographic features in 58 patients

Clinical and radiographic findings were retrospectively reviewed in a multicentric survey of 58 patients with histologically proven pigmented villonodular synovitis (PVNS) of the hip. The most common clinical features were mechanical pain (47 cases) and limitation of joint motion (47 cases). On plain films, a classic form with large and multiple lucencies was found in 36 cases, followed by an osteoarthritis-like form (9 cases), an arthritis-like form (8 cases), normal radiographic appearance (3 cases), osteonecrosis of the femoral head (one case) and joint destruction with acetabular protrusion (one case). Bilateral involvement of the hips was probable in two patients. In contrast to the knee, the hip showed a high prevalence of bony lesions and joint space narrowing. Although an uncommon disease, PVNS of the hip has to be considered when arthritis is associated with uncharacteristic clinical or radiographic findings.     

Biophysical properties of the normal-sized aorta in patients with Marfan syndrome: evaluation with MR flow mapping

PURPOSE: To investigate the feasibility of magnetic resonance (MR) flow mapping in the assessment of aortic biophysical properties in patients with Marfan syndrome and to detect differences in biophysical properties in the normal-sized aorta distal to the aortic root between these patients and matched control subjects. MATERIALS AND METHODS: Seventy-eight patients with Marfan syndrome with aortic root dilatation and 23 matched control subjects underwent MR flow mapping in four locations in the normal-sized aorta (1, ascending aorta; 2, thoracic descending aorta; 3, descending aorta at the level of the diaphragm; and 4, abdominal descending aorta). Distensibility at each location and flow wave velocity between locations were calculated. RESULTS: Compared with the control subjects, patients with Marfan syndrome had decreased aortic distensibility at three of the four locations (levels 1, 2, and 4; P <.05) and increased flow wave velocity between all locations (P <.05) in the aorta. In patients with Marfan syndrome, flow wave velocity was also significantly increased along the entire aortic tract beyond the aortic root (from level 1 to level 4). CONCLUSION: MR imaging reveals abnormal biophysical properties of the normal-sized aorta in patients with Marfan syndrome. Monitoring of these properties is relevant for evaluating disease progression and treatment options.     

Lumbar spine in Marfan syndrome

Lumbar spine radiographs of 28 patients with Marfan syndrome and a gender and age-matched control group were evaluated for scoliosis and morphologic changes of the L2, L3, and L4 vertebrae. No patient or control subject had any serious low back problems. The Marfan patients showed a high incidence of scoliosis (64%). The incidence of lumbosacral transitional vertebra was also high (18%). The end plates of the vertebral bodies in the Marfan patients were more biconcave than in the control group. In addition, the transverse processes were longer in relation to the vertebral body width in the Marfan group than in the controls. These findings indicate that biconcave vertebral bodies can be added to the list of skeletal manifestations of the Marfan syndrome, and Marfan syndrome to the list of differential diagnoses for biconcave vertebrae (codfish vertebrae).     

Ductus arteriosus aneurysm in Marfan syndrome

We report the angiographic and magnetic resonance imaging findings of an aneurysm of the ductus arteriosus in a woman with Marfan syndrome. This is the first reported ductus aneurysm diagnosed in an adult with Marfan syndrome.     

Case report 582

A patient with Marfan syndrome presented with enlargement of lumbosacral spinal canal with a bulky meningocele, and a fusiform aneurysm of the ascending aorta. In Marfan syndrome, the presence of a meningocele is extremely rare, but 60% of the patients develop cardio-vascular complications (a frequent cause of death). MRI is the imaging technique of choice for the diagnosis of meningocele and aortic aneurysm.     

Marfan syndrome: evaluation with MR imaging versus CT

Twenty-five patients with Marfan syndrome underwent computed tomography (CT) and magnetic resonance (MR) imaging. MR images were interpreted in blinded fashion and then the results were compared with findings from CT scans. MR imaging was found to be equivalent to CT in the depiction of aortic, dural, and hip abnormalities in patients who had not undergone surgery. MR imaging was superior to CT in evaluation of postoperative patients because the artifact produced by Bjork-Shiley or St. Jude valves precludes adequate evaluation of the aortic root on CT scans, while producing only a small inferior field distortion, a "pseudo-ventricular septal defect," on MR images. The absence of radiation exposure is another significant advantage for the relatively young Marfan syndrome population, who require serial studies. MR imaging is the modality of choice for evaluation and follow-up of patients with Marfan syndrome and offers an appropriate means of screening their kindred.     

Carpal ligamentous laxity with bilateral perilunate dislocation in Marfan syndrome

A case of persistent bilateral perilunate dislocation unrelated to trauma in a patient with Marfan syndrome is discussed. This finding is believed to be a manifestation of the generalized ligamentous laxity occurring in this disorder. Radiographs of eight additional Marfan syndrome patients failed to demonstrate similar carpal instability. Because some carpal derangements are dynamic events, stress views or wrist fluoroscopy may be necessary to demonstrate unsuspected carpal instability in Marfan patients.