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        west china medical publishers
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        find Keyword "先天性" 519 results
        • Open heart operation on neonates with critical congenital heart disease 推薦 CAJ下載 PDF

          Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.

          Release date:2016-08-30 06:27 Export PDF Favorites Scan
        • ANATOMICAL FEATURES OF CONGENITAL CHORDEE WITHOUT HYPOSPADIAS IN CHILDREN AND IMPLICATION FOR DIAGNOSIS AND TREATMENT

          Objective To investigate the anatomical features of congenital chordee without hypospadias in children and to discuss the diagnosis and treatment.Methods From August 1984 to December 2004, 94 children with chordee withouthypospadias treated in the West China Hospital of Sichuan University were classified and analyzed for anatomical alterations. Their ages ranged from 18 months to 13 years (mean 6.9 years). Ninety-four patients were divided into four groups. With intraoperation artificial erection, the patients with penis straightened after degloving were classified as type Ⅰ patients (skin-tethering), those with peins straightened after fibrotic tissue in Buck’s fascia released as type Ⅱ patients (dysgenetic fascia), those with normal urethra and orthoplasty failed after degloving and removing fibrotic tissue as type Ⅲ patients (corporal disproportion), and those with dysgenetic urethra tethering the corpora cavernosa as type Ⅳ patients (short urethra).Results In type Ⅰ (n=31, 32.9%) patients, the ventral skin and dartos fascia were contracted while Buck’s fascia and the urethra was normal, in some (7 cases) scrotal skin extended to the ventral portion of penis (webbed penis). In type Ⅱ (n=45, 47.9%), contracture of Buck’s fascia was evident and the thickening fibrotic tissue constituted the chief obstacle to orthoplasty, though in some skin was shortened. In type Ⅲ (n=6), the dorsal and ventral sides of the corpora cavernosa were disproportionated, and the morphologically normal urethra tightly adhered to the ventral aspect of corpora cavernosa. In some cases ventral skin and fascia were contracted, but orthoplasty could notbe achieved through releasing these layers. In type Ⅳ (n=12, 12.8%), the distal urethra was paper-thin and lacking corpus spongiosum, or dense fibrotic bandswere found to be deep to the urethra. The urethra tethered the corpora cavernosaand formed a bow-to-string relation. The overlying skin and fascia were contracted in varying degrees while none had significance in straightening the penis. After operation, the length of penis increased to 6.9 cm from 5.2 cm on average and the chordee was corrected to 1.6° from 42.6° before operation on average. The patients were followed up 1 months to 15 years. The results were satisfactory.Chordee remained in 2 cases, fistula and urethral stricture occurred in 2 casesrespectively, fistula in association with urethral stricture and diverticulum in 1 case; the operation was given again and the results was satisfactory. Conclusion Patients with chordee without hypospadias may be divided into four types depending on which layer of the ventral penis constitutes thechief contribution to chordee. A systematic approach with repeated artificial erection tests is needed in determining the classification and surgical correction. 

          Release date:2016-09-01 09:25 Export PDF Favorites Scan
        • 經胸微創膜部室間隔缺損封堵術圍術期炎癥因子的變化

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        • 上腔靜脈右肺動脈分流術治療三尖瓣閉鎖

          目的 總結上腔靜脈右肺動脈分流術治療三尖瓣閉鎖(TA)的臨床經驗。 方法 2004年10月至2008年4月,采用上腔靜脈右肺動脈分流術治療TA 10例,男8例,女2例 ;年齡2~13歲,平均年齡5.6歲;體重10.0~33.5 kg,平均體重16.4 kg。TA合并大動脈錯位(右位心)2例,房室間隔缺損3例,永存左上腔靜脈1例。均在常溫非體外循環下手術,切斷上腔靜脈,近端縫合,遠端與右肺動脈做端側吻合;1例合并永存左上腔靜脈患者行雙側上腔靜脈肺動脈吻合術。 結果 全組無死亡患者,術后末梢血氧飽和度由74%±9%上升至92%±5%。所有患者紫紺明顯減輕,順利出院。隨訪10例,分別隨訪6~36個月,血氧飽和度86%±3%,活動能力明顯改善。 結論 上腔靜脈右肺動脈分流術治療TA效果滿意,是比較理想的術式。

          Release date:2016-08-30 05:59 Export PDF Favorites Scan
        • 十字交叉心的外科治療

          目的 總結4例十字交叉心外科治療的臨床經驗,以提高對該病的認識。 方法 2004年3月~2008年1月手術治療4例十字交叉心患者(平均年齡3.2歲;平均體重10.9 kg)。1例采用心內隧道修補術(intravetricular tunnel rapair IVR),1例采用雙向Glenn手術,1例采用雙側雙向Glenn術,1例采用IVR+大動脈轉位術(arterial switch operation, ASO)。 結果 住院死亡1例,為采用IVR者,死于低心排血量綜合征。2例行雙向Glenn手術患者術后隨訪6~12個月,生長發育良好,SpO2 88%左右;1例行IVR+ ASO者,隨訪6個月,心臟較術前略縮小,生長發育良好。結論 十字交叉心病理解剖復雜,必須根據不同的解剖特點選擇不同的手術方法,明確的術前診斷是優化手術方式的先決條件。

          Release date:2016-08-30 06:09 Export PDF Favorites Scan
        • 先天性心臟病心臟不停跳心內直視手術

          摘要 目的 介紹體外循環心臟不停跳下心內直視手術治療先天性心臟病心內畸形的應用體會。 方法 327例先天性心臟病心內畸形行淺低溫體外循環心臟不停跳心內直視手術。常規建立體外循環,置左心房引流管,降溫至32±1℃并維持,僅阻斷上、下腔靜脈,不阻斷升主動脈,維持灌注壓在60mmHg左右,在心臟不停跳下施行心臟畸形矯正術,方法同心臟停搏手術。 結果 死亡7例,死亡率2.1%,其余均痊愈出院,隨訪1~13個月,恢復良好。 結論 心臟不停跳下心內直視手術有較好的心肌保護作用,能減少并發癥的發生,并簡化操作,縮短體外循環時間。

          Release date:2016-08-30 06:33 Export PDF Favorites Scan
        • A METHOD TO AVOID LENGTHENING LOWER LIMBS AFTER TOTAL HIP ARTHROPLASTY IN PATIENTS WITH CONGENITAL SHORT FEMORAL NECK

          【Abstract】 Objective To investigate the method to avoid lengthening lower limbs after total hip arthroplasty in patients with congenital short femoral neck. Methods The clinical data were analyzed retrospectively from 38 patients undergoing unilateral total hip arthroplasty between April 2005 and December 2010. There were 26 males and 12 females, aged 45-78 years (mean, 62.3 years). Among these cases, there were 11 cases of avascular necrosis of the femoral head, 17 cases of hip osteoarthritis, and 10 cases of femoral neck fracture. Before operation, 29 cases had leg length discrepancy; and the shortened length of the legs was 10-24 mm with an average of 14.5 mm by clinical measurement, and was 11-25 mm with an average of 14.7 mm by X-ray film measurement. The Harris score before operation was 44.0 ± 3.6. Results At 1 day after operation, 3 cases had legs lengthening by clinical and X-ray film measurement; limb length difference less than 10 mm was regarded as equal limb length in the other 35 patients (92.1%). All incisions healed by first intention, and no complication of infection or lower limb deep venous thrombosis occurred. In 3 patients who had legs lengthening, 1 patient had abnormal gait and slight limping after increasing heel pad because the lower limb was lengthened by 16 mm, and 2 patients had slight limping. The other patients could walk normally and achieved pain relief of hip. Thirty-six patients were followed up 12-68 months (mean, 43.8 months). The Harris score was 86.7 ± 2.3 after 6 months, showing significant difference (t=3.260, P=0.031) when compared with that before operation. The X-ray films showed no prosthetic loosening or subsidence. Conclusion For patients with congenital short femoral neck during total hip arthroplasty, the surgeons should pay attention to osteotomy plane determination, limb length measurement, and use of the prosthesis with collar to avoid the lengthening lower limbs

          Release date:2016-08-31 04:22 Export PDF Favorites Scan
        • EFFECTIVENESS OF PENILE VENTRAL SCROTUM COHESION PLACE WEDGE CUTTING AND IMPROVED Brisson TECHNIQUE FOR CONGENITAL BURIED PENIS

          Objective To investigate the effectiveness of the penile ventral scrotum cohesion place wedge cutting and improved Brisson technique for congenital buried penis. Methods Between March 2010 and June 2012, 68 boys with congenital buried penis were treated by the penile ventral scrotum cohesion place wedge cutting and improved Brisson technique, with a median age of 4 years and 10 months (range, 3 months-13 years). Of 68 cases, 14 were classified as phimosis type, 14 as rope belt type, 20 as moderate type, and 20 as severe type. The body of penis developed well and had no deformity. After operation, complications were observed, and the effectiveness was evaluated by the designed questionnaire. Results Early postoperative complications occurred in 11 cases, including obvious adhesion of the outside wrapping mouth in 4 cases, scrotal skin bloat in 5 cases, and distal foreskin necrosis in 2 cases; long-term complications occurred in 9 cases, including abdominal incision scar formation in 4 cases, wrapping mouth scar stricture in 3 cases, and short penis in 2 cases. Primary healing of incision was obtained in the other boys. Fifty-four cases were followed up 6-12 months (mean, 8 months). According to the designed questionnaire, satisfaction rate with the overall view in parents was 77.78% (42/54); the clinical improvement rate was 85.19% (46/54); exposure of the penis was satisfactory in parents of 50 cases; and the parents had no psychological burden of penis exposure in 46 cases, which were significantly improved when compared with preoperative ones (P ﹤ 0.05). The boys had no psychological burden of penis exposure in 29 cases (53.70%) after operation, showing no significant difference when compared with preoperative one (18 cases, 33.33%) (χ2=1.22, P=0.31). Conclusion Application of the penile ventral scrotum cohesion place wedge cutting and improved Brisson technique can effectively correct congenital buried penis.

          Release date:2016-08-31 04:12 Export PDF Favorites Scan
        • TREATMENT OF HIRSCHSPRUNG’S DISEASE WITH MODIFIED DUHAMEL’S OPERATION

          的討論先天性巨結腸的手術治療。方法回顧106例先天性巨結腸的手術治療方式及療效。結果采用改良Duhamel術式治療先天性巨結腸并發癥少,遠期排便功能良好。結論改良Duhamel術式適于任何年齡和所有類型的巨結腸,療效滿意。

          Release date:2016-08-28 05:10 Export PDF Favorites Scan
        • 同種帶瓣大動脈重建右心室流出道

          摘要目的 應用自行采集和冷凍保存的同種帶瓣大動脈(VHC)完成先天性心臟病右心室流出道的重建,并觀察其療效及存在的問題。方法 用VHC材料治療先天性心臟病95例,90例手術根治,5例行VHC右心室流出道與肺動脈連接姑息手術。結果 院內死亡13例;術后隨訪68例,死亡2例,均為感染。66例長期生存者中25例胸部X線片示VHC有鈣化,多為主動脈材料,僅5例有輕中度壓力階差(35~60mmHg)。結論 VHC可廣泛用于治療復雜先天性心臟病。程序降溫、超低溫保存和兩步化凍是VHC使用質量的保證。VHC的長期通暢比合成管道好,隨植入時間延長鈣化率增加,肺動脈VHC優于主動脈。有肺動脈高壓者宜盡早手術。為了預防VHC植入后感染,應重視、改進其收集和保存的方法。

          Release date:2016-08-30 06:33 Export PDF Favorites Scan
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