Klippel-Trenaunay syndrome: frequency of cerebral and cerebellar hemihypertrophy on MRI

We examined 11 patients, clinically and radiographically diagnosed as having the Klippel-Trenaunay syndrome (KTS) by MRI. There were four females and seven males, aged 3–51 years (mean 21 years). Two had clear asymmetry of the cerebral and cerebellar hemispheres. The thickness of the grey matter was normal, without sulcation abnormalities, but the thickness of the white matter was increased; the size of the ipsilateral ventricle was normal. These patients had hypertrophy of the leg and a cutaneous haemangioma on the same side as the brain abnormality. No patient had an intracranial vascular malformation, unilateral megalencephaly, cerebral atrophy or hydrocephalus. The prevalence of cerebral hemihypertrophy in our series of patients with KTS was thus 18 %. Received: 15 March 1999/Accepted: 30 August 1999     

静脉顺行造影在静脉畸形骨肥大综合征的应用

目的：探讨静脉顺行造影在静脉畸形骨肥大综合症（KTS）的应用价值。方法：对9例下肢静脉畸形骨肥大症患者行顺性静脉造影检查,其中有4例于术后复查顺性静脉造影,以评价静脉畸形的部位、狭窄的范围与程度。9例患者均行B超检查。结果：静脉造影显示本组9例患者中,1例胫腓干静脉狭窄,1例胫腓干静脉狭窄,1例胫腓干静脉狭窄并Guo静脉狭窄,4例静脉狭窄。4例行手术治疗的患者,于术后复查下肢静脉造影,显示浅静脉曲张较术前减少,深静脉通畅度改善。结论：静脉造影提供了明确的静脉畸形范围,从而对临床治疗提供可靠的依据;并有利于术前、术后对比,以评价疗效。     

Klippel-Trenaunay综合征的影像学表现

目的:探讨Klippel-Trenaunay(KT)综合征的影像学表现以及临床意义。方法:总结10例KT综合征患者的顺行静脉造影、多谱勒彩超、双下肢平片以及6例患者患肢动脉造影的表现。结果:顺行静脉造影和多谱勒彩超显示单纯异常扩张浅静脉8例(80%),深静脉异常2例(20%);动脉造影显示动脉二、三级分支增多,软组织内见染色影;平片显示患肢骨骼增长。结论:影像学检查对KTS综合征的诊断以及临床治疗方案的选择具有重要意义。     

Peripheral MR Angiography of Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disease of unknown etiology that affects one or more limbs. It is characterized clinically by three physical findings (the so-called triad): port-wine stain hemangioma, hypertrophy of the bony and/or soft tissue, and varicose veins. A review of the medical literature in 1999 revealed about 1,000 case studies. We present here the case of a patient with clinical diagnosis of KTS studied using peripheral magnetic resonance angiography.     

Tissue distribution of copper-labeled 3-ethoxy-2-oxobutyraldehyde bis (thiosemicarbazone) (Cu-64 KTS) in mice and rats: concise communication

The antitumor activity of, 3-ethoxy-2-oxobutyraldehyde bis (thiosemicarbazone) (KTS), is related to the presence of copper(II) ion. We have studied the tissue distribution of Cu-64-labeled KTS in rats and mice carrying transplanted tumors to evaluate whether the uptake of the radioactivity in the tumor is adequate to warrant further investigation of the tracer as a tumor-seeking agent in patients. Four groups of three of four animals each were studied: (a) mice with fibrosarcoma; (b) mice with mammary adenocarcinoma; (c) rats with fibrosarcoma; and (d) rats with squamous cell carcinoma of the lung. The animals were killed at intervals of 0.25, 1, 4, 24, and 48 hr after i.v. injection of 1.6 X 10(-3) M Cu.KTS containing 3 to 18 muCi Cu-64. Blood, tumor, and six to ten additional tissues were counted for radioactivity. The mouse fibrosarcoma concentrated Cu-64, reaching 15% of the administered dose/g at 48 hr after injection. This suggests that for tumor scanning, the 61.7-hr Cu-67 might be more suitable as a label for KTS than the 12.7-hr Cu-64.     

Genitourinary involvement in pediatric patients with Klippel-Trenaunay Syndrome

BackgroundKlippel-Trenaunay Syndrome (KTS) is a genetic vascular malformation disorder which induces a variety of phenotypic expression in patients which differ in terms of severity/location. While previous studies have documented genitourinary (GU) complications in adult KTS patients, documentation of the scope and incidence of GU involvement in the pediatric population with imaging findings is currently limited. This study represents the largest KTS genitourinary review to date.ObjectiveTo assess the incidence, scope, clinical findings and imaging characteristics of GU pathology in pediatric KTS patients.Materials/MethodsUsing a retrospective data analysis design, the charts and imaging studies of pediatric KTS patients were reviewed. All patients received care at a specialized vascular clinic within a multicenter tertiary care system. Variables studied included age, age at KTS diagnosis, gender, urologic involvement, and age of urologic complication.Results58 patients were identified. 33 were male and 25 were female. 10 patients had GU findings. Three of these patients had multifocal GU involvement (greater than 1 finding). Urologic manifestations were diverse with 9 distinct diagnoses involving 6 unique organs. Renal, vesical and scrotal pathologies were most common. Hematuria was the most common presenting symptom in 30% (3/10). Previously unreported findings (labial swelling, renal lymphatic cysts) were identified. The average age of KTS diagnosis was 4.9 years. The average age of documented GU complication and involvement was 7.6 years.ConclusionSignificant GU complications due to KTS can occur in the pediatric population. Early clinical and imaging characterization of these conditions is important for management, family education and early intervention strategies.     

MR imaging of muscle and tender points in fibromyalgia

Fibromyalgia is a syndrome manifested by chronic, diffuse muscu-loskeletal aching and soreness, palpable muscle tender points, and other symptoms. Standardized clinical diagnostic criteria have recently been developed. Skeletal muscle has been postulated as the end organ in this disease. Biochemical, histologic, electromyographic, and conventional radiographic studies have demonstrated no definitive abnormality. This study sought to establish whether magnetic resonance (MR) imaging could demonstrate any abnormality in these patients. Eighteen patients were entered in the study, 14 of whom were able to complete their examinations. T1 -weighted, T2-weighted, gradient-echo, and STIR (short-tau inversion-recovery) sequences were performed in all patients, with selected patients examined with T1weighted, gadopentetate dimeglu-mine-enhanced sequences. The trapezius and suboccipital regions were imaged in patients who, clinically, had active fibro-myalgia. No abnormalities could be detected. The authors conclude that the conventional MR imaging used in this study was unable to depict any primary skeletal muscle abnormality in fibromyalgia.     

The Klippel-Trenaunay syndrome: clinical and radiological aspects.

Klippel-Trenaunay syndrome (KTS) is characterized by port-wine hemangiomas, deep venous system abnormalities, superficial varicosities, and bony and soft-tissue hypertrophy. When associated with an arteriovenous fistula, it has been termed Klippel-Trenaunay-Parkes-Weber syndrome. It is imperative that both the radiologist and surgeon be aware of this entity, as incomplete evaluation and inappropriate surgery may be devastating. Radiological workup includes phlebography, angiography, and conventional radiography of the involved extremities. Surgery should be performed only to relieve deep venous obstruction (if present) or to correct inequality in the lengths of legs. Removal of superficial varicosities is contraindicated because it will worsen existing symptoms. Five cases of KTS are presented and the literature reviewed.     

Evaluation of Klippel-Trenaunay syndrome with radionuclide total body angiography. A case report.

A 21-year-old Italian woman with extensive Klippel-Trenaunay syndrome (KTS) developed recurrent pulmonary embolism in spite of the insertion of a Greenfield filter in the inferior vena cava. Clinical and radiologic diagnostic tests failed to demonstrate the pathway of the emboli. Radionuclide venography and intravenous radionuclide total body arteriography were performed. These radionuclide procedures helped to evaluate the extent of KTS and to detect the route of the emboli. This is the first case of KTS studied with intravenous total body arteriography reported in the literature.     

小儿癫痫的CT表现及其应用价值分析

目的：分析螺旋CT扫描在小儿癫痫诊断中的应用价值。方法：120例小儿癫痫患者于发作间期分别行螺旋CT及EEG检查，并对其CT表现及EEG异常率进行对比分析研究。结果：本组120例小儿癫痫患者中CT表现异常者80例，阳性率为67％。其中脑萎缩28例，炎性病变14例，外部性脑积水11例，先天性疾病7例，颅内肿瘤6例，脑内缺血灶5例，外伤（包括产伤）3例，新生儿缺血缺氧性脑病2例，蛛网膜囊肿2例，颅内钙化2例。EEG异常者107例，异常率89．1％。80例CT异常患儿EEG异常率为100％。结论：CT检查在小儿癫痫病因查找及病变定位中有较高的应用价值，Cr与EEG二者的结合可提高小儿癫痫的诊断准确率及定位率。     

Klippel-Trenaunay综合征介入栓塞治疗探讨

目的评价Klippel-Trenaunay综合征(KTS)介入治疗的疗效。方法23例KTS患者均采用Seldinger技术行经股动脉行患肢动脉DSA,以显示其异常的血管造影表现,继而用微导管超选择进入靶血管并用PVA颗粒加弹簧圈进行栓塞。结果23例KTS患者均成功地进行了PVA加弹簧圈栓塞治疗,随访6～24个月见临床症状均有明显改善,未见严重并发症及复发。结论经导管动脉栓塞是介入栓塞治疗KTS的一种安全、成功率高和疗效肯定的治疗手段。     

256层CT下肢静脉成像评价Klippel-Trenaunay综合征

目的探讨256层CT下肢静脉成像在Klippel-Trenaunay综合征的诊断价值。方法回顾性分析经临床和影像学检查确诊的7例KTS患者的临床及影像学资料,观察其CT下肢静脉成像的影像特征。结果本组7例患者CT下肢静脉成像均显示浅静脉曲张及患肢皮下静脉畸形,其中伴有深静脉病变4例（3例深静脉狭窄,1例深静脉闭塞）。结论多层CT下肢静脉成像是KTS有效和可靠的影像检查方法,对于指导临床治疗具有重要意义。     

The Use of Endovenous Laser Treatment in Toddlers

The use of endovenous laser ablation therapy in children is limited. Klippel-Trenaunay syndrome (KTS) is associated with persistent ectatic anomalous veins within the affected extremity, with increased risk of thromboembolism. The present report describes four toddlers (<20 kg) with KTS of the lower extremity who underwent a total of five endovenous laser procedures for treatment of ectatic anomalous marginal venous system, without complications.     

MSCT血管成像对静脉畸形骨肥大综合征的诊断价值

目的:探讨多层螺旋CT血管成像对静脉畸形骨肥大综合征影像诊断价值。方法:回顾性分析6例经临床证实的静脉畸形骨肥大综合征的CT血管扫描及三维重建影像学资料。结果:6例静脉畸形骨肥大综合征多层螺旋CT均有浅静脉曲张及肢体长短及粗细差异。4例同时伴有深静脉迂曲畸形或狭窄,2例可见多发静脉石。结论:多层螺旋CT血管成像可以为静脉畸形骨肥大综合征的诊断提供比较确切的影像学诊断支持,为临床诊断、鉴别诊断和治疗提供可靠的影像学依据。     

Cost-Effectiveness of Saline-Assisted Hysterosonography and Office Hysteroscopy in the Evaluation of Postmenopausal Bleeding: A Decision Analysis

RATIONALE AND OBJECTIVES: The authors performed this study to evaluate whether hysterosonography (HSG) is a more cost-effective initial diagnostic examination than office hysteroscopy in the evaluation of postmenopausal bleeding (PMB). MATERIALS AND METHODS: A computer model simulated the diagnosis and treatment of PMB in otherwise healthy women. The hypothetical patient who had one episode of PMB precipitating a clinic visit would undergo either HSG or office hysteroscopy as the initial examination. Algorithms were designed such that a finite number of false-negative and false-positive findings would be expected, and clinical decision making would rely on the reported results. Performance characteristics for diagnostic tests and other clinical probabilities were taken from the literature. Costs were based on actual 1997 Medicare reimbursements. The primary clinical outcome considered was the correct diagnosis of any anatomic abnormality that was amenable to definitive treatment. The primary cost outcome considered was the cost per abnormality detected. Sensitivity analysis was performed to examine the effect of varying performance characteristics for diagnostic techniques. RESULTS: HSG and office hysteroscopy correctly depicted 68.1 and 67.6 anatomic abnormalities per 100 patients, respectively. The average cost per abnormality detected was $7,978 with HSG and $8,400 with office hysteroscopy. CONCLUSION: HSG depicted more abnormalities at a lower cost per abnormality, which suggests that it should be the preferred initial diagnostic examination in the setting of PMB.     

Computed tomography in acute cholecystitis

It has been our experience that acute cholecystitis can frequently be diagnosed on the basis of computed tomography (CT) alone, without the need for further confirmatory studies. This capability has not been emphasized in the radiologic or surgical literature.Retrospective review of CT scans performed in patients with the initial diagnosis of acute abdomen or sepsis due to abdominal source yielded 29 patients in whom a retrospective CT diagnosis of acute cholecystitis was made when all three of the following findings were present: gallbladder wall thickening (>3 mm), gallbladder distention, and pericholecystic abnormality (either fluid or abnormal fat).Pathologic or surgical follow-up was available in 22 of 29 patients. In 20 of 22 (91%) patients who underwent surgery, there was a pathologic or surgical diagnosis of acute cholecystitis. There were two falsepositive diagnoses: one patient with chronic cholecystitis and one patient with adenocarcinoma of the neck of the gallbladder.A confident diagnosis of acute cholecystitis can be made on CT scan in the appropriate clinical setting when all three of these criteria are met: gallbladder distention, gallbladder wall thickening, and pericholecystic abnormality. If one of these criteria is not met or is equivocal, biliary scintigraphy or ultrasonography may be needed to confirm the diagnosis.     

Nutritional and metabolic bone disease in a zoological population

The radiologic survey technique used at the National Zoological Park has disclosed many cases of nutritional and metabolic bone disease of various types. This communication discusses calcium deficiency states seen in lion cubs associated with a meat diet, the increased need for calcium noted in egg-laying lizards, problems found in New World monkeys, antelopes (due to selenium and/or vitamin E deficiency), hypervitaminosis D, and lead poisoning noted in young primates. In all of these diagnoses, the use of routine radiographic studies has been extremely helpful, first in detecting that a bone or soft tissue abnormality is present, and then in the differential diagnosis of the etiology of this abnormality. As more is learned of the necessary nutriments for exotic species kept in captivity, it is expected that the nutritional disturbances described in this article will be seen with decreasing frequency.     

Klippel-Trenaunay综合征的动脉造影表现和介入治疗

目的 观察K1ippel-TrenaunTdy综合征(KTS)的动脉造影表现并采用新的介入方法对其进行治疗。方法 观察7例KTS患肢动脉DSA的影像表现。采用经导管患肢动脉内注射平阳霉素碘油乳剂(PLE,平阳霉素用量6-12 mg,超乳化碘油4-8 m1)治疗。观察术后疗效、副反应和并发症。结果 动脉造影显示患肢少数动脉2、3级分支增粗,软组织内可见部分斑片状染色伴大小不等血窦显影(5例),引流静脉增粗(4例)和浅静脉迂曲扩张(5例)。病变区碘油沉积良好者6例,1例碘油沉积欠佳。术后随访13-30个月,肢体肥大明显回缩者6例,大腿的周长接近正常肢体,1例病情无明显变化。1例于1年后局部轻度复发。主要术后反应:均有肢体中至重度肿胀和轻度发热,另外有一过性血清转氨酶升高2例和肢端麻木1例。并发症为局限性皮肤坏死1例,经治疗痊愈;另1例出现患侧足拇指背屈无力,治疗无效。结论 患肢动脉造影可以显示KTS部分畸形血管。PLE动脉内灌注治疗KTS引起的患肢肥大具有较好疗效。由于本疗法可能有较严重的副反应和并发症,应慎用。     

磁共振TRICKS技术在评估Klippel-Trenaunay综合征下肢静脉病变中的应用

目的 探讨磁共振时间分辨对比剂动态显像(TRICKS)技术在Klippel-Trenaunay综合征(KTS)下肢静脉病变中的临床应用价值.方法 对本院收治的20例(21条患肢)行磁共振TRICKS技术下肢血管成像和下肢静脉顺行造影的KTS患者的影像资料进行回顾性分析,对图像质量进行分级,应用Kappa检验比较2种检查方法诊断结果的一致性.结果 磁共振TRICKS技术与下肢静脉顺行造影影像质量分级中,下肢深静脉等级优分别占85.71％(18/21)、80.95％(17/21),下肢浅静脉等级优分别占95.24％(20/21)、90.48％(19/21),静脉畸形等级优分别占90.48％(19/21)、85.71％(18/21).21条患肢中应用TRICKS技术显示深静脉正常61.90％(13/21)、发育不良25.57％(7/21)、闭锁4.86％(1/21),浅静脉中大隐静脉曲张25.57％(6/21)、小隐静脉曲张14.29％(3/21),静脉畸形中边缘静脉100％(21/21);下肢静脉顺行造影深静脉正常57.14％(12/21)、发育不良39.10％(8/21),余结果与TRICKS技术一致.2种检查方法结果一致性良好(Kappa值>0.75).结论 磁共振TRICKS技术可精确描述KTS患者下肢静脉解剖结构,图像清晰可靠,可以为KTS的评估及拟定治疗方案提供依据,具有重要的临床应用价值.