先天性静脉畸形组织肥大综合征的诊治体会

先天性静脉畸形组织肥大综合征 (ｋｌｉｐｐｅｌ ｔｒｅｎａｕｎａｙｓｙｎ ｄｒｏｍｅ ,ＫＴＳ)是一种较少见的先天性肢体静脉疾病 ,随着对该病认识的增加 ,近年来确诊率明显增高。我们从 1988年至1997年底共诊治ＫＴＳ 2 9例 ,计 3 1条肢体 ,现就本病的诊断和处理等问题探讨如下。临床资料一、一般情况 :本组 2 9例中 ,男 13例 ,女 16例 ,年龄 12～40岁 ,平均 (2 3 .3 3± 6.7)岁。共累及下肢 3 1条 ,无累及上肢者。二、临床表现 :肢体沉重酸胀感 2 2例 ,肿胀 10例 ,有出血史 5例 ,疼痛 6例 ,浅表性静脉炎 5例。肢体或躯干部毛细血管瘤 2 …     

静脉畸形骨肥大综合征的手术治疗

我们报道６２例（６５条患肢）手术治疗的经验。指出静脉造影是可靠的诊断方法；深静脉主干有病变者，必需按病情的不同，采用适当的手术方法，才能取得满意的效果；畸形的外侧静脉和病变的交通静脉，都是血液倒流的通道，均应予以结扎。     

中西医结合诊治先天性静脉畸形骨肥大综合征

静脉畸形骨肥大综合征(klippel-trenaunay syndrome,KTS)是一种先天性静脉畸形病变,长期以来缺乏完善的诊断技术及有效的治疗方法.2000年1月-2003年7月我们采用中西医结合疗法诊治24例,报道如下.     

先天性静脉畸形骨肥大综合征临床病理研究

１９８７年１１月至１９９４年１月，我们共对１７例先天性静脉畸形骨肥大综合征（ＫＴＳ）进行了研究，旨在探讨临床表现与病理变化之间的关系，为临床医师选择有效的治疗方法提供参考依据。对象和方法一、一般资料１７例中，男７例，女１０例，年龄６岁～２３岁，病变全部位于下肢，左侧６例，右侧１１例。二、方法（１）详细询问病史及全面有重点的体格检查；（２）患肢动脉造影或彩色多普勒超声检查，合并广泛性或活动性动静脉瘘患者不在本研究内；（３）深静脉造影，了解深静脉病变部位及性质；（４）除２例深静脉正常保守治疗外，余１…     

先天性静脉畸形骨肥大综合征的诊断与治疗

目的　探讨先天性静脉畸形骨肥大综合征的诊断依据及治疗方法 ,提高治疗效果。　方法　对 5例患者诊断与治疗的观察、分析、总结影像片及特征性体征 ,并结合文献资料 ,不断完善对此病的认识。　结果　 5例患者近期症状均改善 ,患肢葡萄酒色红斑变淡 ,病灶明显减少 ,患肢周径缩小 ,溃疡愈合。随访时间 1～ 44年 ,1例失访。1例股浅动脉结扎、病灶切除术 44年后再复发 ,2例手术治疗者分别随访 2年、4年 ,血管瘤样病变无复发 ,患肢无继续增生 ,酸胀感明显减轻。弹力绷带保守治疗的患儿随访仅 1年 ,有待进一步观察。　结论　先天性静脉畸形骨肥大综合征一旦确诊后 ,应尽早手术 ,时机宜选择在青春发育期前 ,能获得满意治疗效果     

静脉畸形骨肥大综合征38例的诊断与治疗

我院于 1988年 3月至 2 0 0 2年 6月共收治静脉畸形骨肥大综合征 (klippel trenaunaysyndrome ,KTS) 38例 ,现报告如下。临床资料1.一般资料 :本组 38例 ,男 32例 ,女 6例 ,平均年龄 6～6 0岁 ,所有病变位于下肢 ,左侧 2 1例 ,右侧 17例。临床表现 :血管瘤或血管痣 18例 ,其中 8例于出生时就存在 ,10例在 1～ 2岁时才出现 ;骨肥大及软组织增生 2 5例 ,小腿周长平均较健侧粗 2 0cm以上 ,最粗可达 5 6cm。 5例患肢较健侧增长 1～ 3 0cm。其中 3例曾行截骨术 ;浅静脉曲张 38例 ,13例曾行单纯浅静脉剥脱 ;其他 :患肢肿胀者 2 8例 ,足靴区色…     

静脉畸形骨肥大综合征的泌尿生殖器后遗症

以前关于静脉畸形骨肥大综合征的生殖系疾患,我们的回顾性文献总是利用来自不同专业的具体文章来评定其发病率和生殖系疾患的后遗症.我们认为,这导致了关于静脉畸形骨肥大综合征的泌尿系统并发症的错误结论.     

头颈部海绵状静脉畸形肿胀切除法体会

海绵状静脉畸形好发于头颈部，其瘤体血管丰富。直接手术出血多，术野不清晰，常影响完整切除，甚至有时出血不易控制，手术风险大，自1999年3月以来，我们应用肿胀技术，共切除头颈部海绵状静脉畸形24例，效果较好。     

An experience of arthroplasty in Klippel–Trenaunay Syndrome

We wish to discuss the peri-operative problems, difficulties and management in a patient with Klippel–Trenaunay (KT) syndrome undergoing total hip arthroplasty. Although Klippel–Trenaunay is a well-described entity in the realm of haematology, we are unable to find any previous report that could view this syndrome from an arthroplasty point of view from our review of literature and MEDLINE search.     

Klippel Trenaunay syndrome

Klippel-Trenaunay syndrome (KTS) is a congenital vascular disorder of unknown cause, characterized by port wine stain (capillary malformations), venous malformation and limb hypertrophy. We present a case of this rare syndrome in a young girl.     

布加综合征的介入治疗

目的：总结１０６例布加综合征介入疗法治疗的经验。病人：１９８６年至１９９８年我们共以介入疗法治疗布加综合征１０６例，其中男７０例，年龄６￣６５岁，平均３４岁。病变种类包括下腔静脉完全阻塞４０例；下腔静脉局限性狭窄３６例；下腔静脉膜性阻塞带孔者２６例；单纯肝静脉膜注阻塞３例；下腔静脉广泛血栓形成者１例；１０６例中１４例为下腔静脉和肝静脉联合病变。方法：下腔静脉破膜扩张或单纯扩张５７例；下腔静脉破膜加     

Experience of Supraclavicular Exploration and Decompression for Treatment of Thoracic Outlet Syndrome

The purpose of this study was to assess the symptomatic outcome of patients with thoracic outlet syndrome who underwent decompression of the thoracic outlet. In our unit we prefer the supraclavicular approach, performing anterior scalenectomy with excision of fibrous bands or cervical ribs if present. Operative details were gained by theater logbook and case note review. Over a 6-year period, 31 patients (37 limbs) underwent thoracic outlet decompression. Of the 37 affected limbs, the indications for surgery were a combination of both neurological and vascular symptoms in 24 patients (65%), neurological symptoms in 24 (65%), and 4 patients (11%) had vascular symptoms alone. All patients were assessed for postoperative outcome either at out-patient clinics or by personal contact. From the results of this study we concluded that supraclavicular scalenectomy and cervical rib excision with selective first rib excision is a safe and effective procedure for most patients with thoracic outlet syndrome.     

Ten Years’ Experience in Surgical Treatment of Right Middle Lobe Syndrome

Purpose: In this study we present the clinical, radiological, pathological, bronchoscopic and surgical results of 40 patients with diagnosis of middle lobe syndrome who were referred to our thoracic surgery unit for surgical intervention in a 10 years period.Methods: Forty patients with obstructive and non-obstructive causes of middle lobe syndrome referred to our thoracic surgery unit. Clinical data were collected from the patients’ records in a ten years period. This study evaluates diagnostic approaches and surgical treatments in right middle lobe syndrome.Results: We studied 23 females (57.5%) and 17 males (42.5%) with a mean age of 31.7. Clinical findings were cough 95%, sputum 80% and intermittent hemoptysis in 50% of patients. Middle lobe collapse was seen in CT scan of all patients. Bronchiectasis was the most common pathologic finding (55%). Tuberculosis was not rare and was final pathology in 20% of patients. In three patients ruptured hydatid cyst was final finding. Surgery was done without mortality and with only minor complications.Conclusion: Lobectomy of right middle lobe is a good therapeutic option in these patients. Due to high prevalence of tuberculosis and hydatid cyst in Middle Eastern countries these two must be considered as causes of middle lobe syndrome.     

Relief of Iliofemoral Vein Occlusion with the Palma Bypass in a Patient with Klippel Trenaunay Syndrome

Klippel Trenaunay syndrome is usually managed conservatively with surgery reserved for patients with symptomatic but mild cosmetic deformity or persistent venous hypertension despite nonoperative measures. Deep venous reconstruction is necessary in a small group of patients who present with significant chronic venous insufficiency due to hypoplastic, absent, or occluded deep venous outflow. Most often, venous outflow occlusion results from inadequate or surgically removed superficial collateral veins or from complications of endovascular procedures. In this article, we review the English-language literature and the Mayo Clinic experience, and report on a patient with Klippel Trenaunay syndrome who developed symptomatic iliofemoral venous occlusion following iliac vein stenting, which was successfully treated with a crossover saphenofemoral vein (Palma) bypass. Presented at the Fourth Pacific Vascular Symposium on Venous Diseases, Kohala Coast, Hawaii , November 12-16, 2002.     

Treatment of Children with Steroid Refractory Idiopathic Nephrotic Syndrome: The Kuwaiti Experience

The aim of this study is to evaluate histopathological findings induced by N^Ω‐nitro‐L‐arginine methyl ester (L‐NAME) and molsidomine (MOL) on the kidney of bile duct ligated rats. Forty Sprague–Dawley rats, each weighing 125 to 140 g, were included in the study. Extent of histological glomerular injury scores (GIS), arterial injury scores (AIS), and tubulointerstitial injury scores (TIS) in each animal were graded. Alpha‐smooth muscle actin (α‐SMA), tenascin, lectin (Ulex europaeus agglutinin‐1), and vimentin were used to determine extent of the injury. The cholestasis was evidenced by a significant increase in the levels of serum total bilirubin in BDL rats (p < 0.01). Malondialdeyde MDA levels increased by the bile duct ligation (BDL) to 12.10 ± 0.45. This value was significantly higher than the other groups (p < 0.01). Changes in the BDL kidney were marked at 7 days after surgery. GIS were observed to have the highest score, especially at juxtamedullary region in BDL/L‐NAME rats, and AIS were also the highest score in this region. These observations were lower in BDL/MOL rats. There is a correlation between GIS and AIS scores (r = .2, p < .01). TIS revealed that BDL/L‐NAME rats were significantly more damage than rats in the other groups (p < .001). MOL‐treated rats showed considerably fewer lesions in the tubules and interstitium (p < .001). The tubular injuries observed in BDL and BDL/L‐NAME rats were significantly attenuated by MOL treatment. Lectin was more and extensively stained in tubular epithelia of the BDL/L‐NAME group than in the other (p < .05). Expression of tenascin in tubular epithelia was significantly higher in BDL and BDL/L‐NAME as compared with controls (p < .01). Fibrous tissue was only observed in the BDL and BDL/L‐NAME group. These areas were weakly stained with vimentin. α‐SMA staining was more reduced in the L‐NAME‐treated arterioles than in BDL/MOL (p < .05). In conclusion, the analysis of cell injury based on a histological grading system in the model of BDL kidney allows the quantification of the degree of injury.