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 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.
Twenty five children with congenital biliary dilatation were treated with hepatico-jejuno-duodenostomy following excision of choledochal cysts between 1983 and 1985. The age ranged from two months to eleven years. The last follow-up ranged from 6-9 years (mean 7.5 years). All patients were free of jaundice with normal growth and development and none had peptic ulcer. The results from the last follow-up was better than that of the first one. This procedure was safe, effective and physiologically appealing.
Objective To report the clinical characteristics and treatment analysis of 3 cases of congenital ulnar collateral flexor contracture of the forearm and take a reference for clinic. Methods A total of 3 patients with congenital ulnar collateral flexor contracture of the forearm were admitted between February 2019 and August 2021. Two patients were male and 1 was female, and their ages were 16, 20, and 16 years, respectively. The disease durations were 8, 20, and 15 years, respectively. They all presented with flexion deformity of the proximal and distal interphalangeal joints of the middle, ring, and little fingers in the neutral or extended wrist position, and the deformity worsened in the extended wrist position. The total action motion (TAM) scores of 3 patients were 1 and the gradings were poor. The Carroll’s hand function evaluation scores were 48, 55, and 57, and the grip strength indexes were 72.8, 78.4, and 30.5. Preoperative CT of case 2 showed a bony protrusion of the flexor digitorum profundus tendon at the proximal end of the ulna; and MRI of case 3 showed that the ulnar flexor digitorum profundus presented as a uniform cord. After diagnosis, all patients were treated with operation to release the denatured tendon, and functional exercise was started early after operation. Results The incisions of 3 patients healed by first intention. Three patients were followed up for 12, 35, and 12 months, respectively. The hand function and the movement range of the joints significantly improved, but the grip strength did not significantly improve. At last follow-up, TAM scores were 3, 4, and 4, respectively, among which 2 cases were excellent and 1 case was good. Carroll’s hand function evaluation scores were 95, 90, and 94, and the grip strength indexes were 73.5, 81.3, and 34.2, respectively. ConclusionCongenital ulnar collateral flexor contracture is a rare clinical disease that should be distinguished from ischemic muscle contracture. The location of the contracture should be identified and appropriate surgical timing should be selected for surgical release. Active postoperative rehabilitation and functional exercise can achieve good hand function.
ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
目的 全面介绍先天性囊性腺瘤样畸形(CCAM)其可能的病因，临床、病理特点及诊断手段，循证探讨针对CCAM的治疗方法及预后。 方法 对我院2011年11月收治的1例罕见的CCAM患者的临床资料进行分析，并对相关文献进行复习。 结果 患者数次误诊后最终诊断为CCAM，予手术治疗后痊愈，随访1年无复发。 结论 CCAM是一种少见的、非遗传性的、错构瘤样的肺发育异常，为一种良性的肺部畸形，其特点是局部肺终末呼吸性细支气管过度生长。CCAM多通过产前影像学检查、活组织检查或术后病检诊断。手术为治愈该病的最根本、最重要措施。