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.
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.
OBJECTIVE: To introduce a surgical approach for reconstruction of nail folds in congenital complete syndactyly release. METHODS: A narrow flap and a broad flap were raised on the common distal phalanx to cover the denuded nail-edge in 30 fingers of 15 cases whose webs were separated. RESULTS: All of the flaps were successfully transferred and survived. The reconstructed nail folds had satisfied figure in 21 out of 30 fingers. The nail folds in the other 9 fingers, covered by a broad flap in 2 fingers and by a narrow flap in 7 fingers, were a little smaller than normal. All of the 30 fingers had normal fullness of pulp and no twisty nails. CONCLUSION: The reconstruction of nail folds by double pulp flap can be performed with a one-stage technique, and the outcome is satisfactory, which make it as a good surgical approach to reconstruct nail folds in congenital complete syndactyly release.
目的:评价先天性心脏病（CHD）不同病变类型在64层螺旋CT（64-MDCT）三维重建图像上的表现及显示效果。方法:回顾性分析36例CHD患者的64-MDCT资料,分别在轴位、冠状、矢状位及容积再现（VR）重组图像上观察病变,统计分析不同重组图像显示总体病变及房、室间隔缺损效果有无差异。结果:36例患者共有病变59处,轴位、冠状及矢状位图像在发现病变（59处,100.0%；57处,96.6%；53处,89.8%）及室间隔缺损显示（显示效果评分:41、38及39分）方面无明显差异（P＞0.05）,但轴位图像显示房间隔缺损明显优于冠、矢状位图像（显示效果评分:19、13及12分）（P＜0.05）。VR图像与轴位及冠、矢状位图像显示心外大血管病变效果无差异P＞0.05）。不同类型病变在轴位,冠、矢状位及VR图像上表现特征不尽相同,而不同重组图像有各自优势显示的病变类型。结论:64-DCT三维重建图像能够很好地显示先心病各种类型病变,了解不同重建图像上病变表现特征及显示效果有利于做出准确、全面的诊断。
Objective To investigate the operative characteristic and results of the modified Fontan procedure, and improve the application of Fontan procedure in the clinic. Methods From Sep. 1992 to June 2006, 77 cases (aged 2.5 years to 20.0 years) with a wide range of complicated congenital heart diseases underwent the modified Fontan procedure.Right atriumpulmonary artery connection were performed in 21 cases, intraatrium fenestrated baffle or conduit total cavopulmonary connection were performed in 28 cases, extracardiac conduit total cavopulmonary connection were performed in 24 cases, atrium wall lateral tunnel total cavopulmonary anastomosis were performed in 2 cases, extracardiac pericardialtube total cavopulmonary anastomosis was performed in 1 case, and extracardiac direct total cavopulmonary connection was performed in 1 case. Results In early postoperative period, there were 1 case of successful reoperation and 5 death, the cause of death were heart failure (3 cases), arrhythmia (1 case) and cerebral hemorrhage (1 case). The early survival rate was 93.5%(72/77), with 92.0% free from failure. The eject fractions of left ventricle (LVEF) after operation were increased than those before operation （68.5%±4.0% vs.62.0%±4.5%,P=0.032） and left ventricular enddiastolic diameter (LVEDD) were decreased than those before operation （52.5±7.8mm vs.62.5±11.0mm, P=0.013）. A total of 63 cases (87.5%) were followed up from 1 to 15 years after operation. In late postoperative period, there were 4 cases of death, and 1 of successful re-operation. Late survival rate was 88.3%, with 86.0% free from failure. Conclusion In the treatment for complicated congenital heart diseases, the modified Fontan procedure could result in good early and longterm outcome, and the choices for surgical procedure are various.