Williams syndrome is a congenital multisystem disease. Cardiovascular abnormality caused by elastin deficiency is the main cause of morbidity and mortality in Williams syndrome patients. Recent studies have found that 80% of Williams syndrome patients have cardiovascular abnormalities, most of which are arterial stenosis, especially the aortic valve stenosis and pulmonary artery stenosis. Operation is the main method to treat the stenosis of the artery, and the results of the operation on the aortic valve stenosis in most centers are good, but the effect of transcatheter intervention is still not obvious, pulmonary artery reconstruction has a good effect on the treatment of peripheral pulmonary artery stenosis. Advances in genetic diagnosis, surgical techniques and treatment regimens are expected to significantly improve cardiovascular outcomes in these patients. This article reviews the latest research progress of Williams syndrome combined with cardiovascular disease.
摘要: 目的: 探讨小儿先天性心脏病（CHD）部分性房室间隔缺损（PAVSD）的外科治疗方法，以期提高疗效。方法: 对1999年6月至2009年8月收治27例PAVSD临床资料进行分析，男16例，女11例，年龄1.3~14岁,平均6.08岁。术前均经彩色多普勒超声心动图（Echo）和部分心导管检查确诊。手术全部在中低温体外循环（CPB）下行根治术。结果: 无手术死亡。22例获1个月~10年随访，1例术后3个月因重度二尖瓣返流（MR）,心力衰竭死亡，1例Ⅱ°房室传导阻滞（AVB）6个月后自行恢复，2例仍有轻度二尖瓣关闭不全; 余生活、学习正常，心功能Ⅰ级。结论: 一旦确诊应尽早手术治疗。修补原发孔缺损，注意避免损伤传导束，二尖瓣裂修复完善是手术成功和减少并发症的关键。Abstract: Objective: To discuss the surgical treatment of congenital heart disease as partial atrioventricular septal defect（PAVSD）to improve the therapeutic effect. Methods: From June1999 to December2008, the clinical data of 27 children suffering from PAVSD were analyzd. Male：16,female: 11. The ages ranged from 1.3 to 14 years, the average age is 6.08±3.73 years.All the children were final diagnosised by color Doppler echocardiogram and right catheterization.All underwent the radical correction under moderate hypothermic cardiopulmonary bypass. Results: There was no operative mortality. 25 cases were followed up for 6 months to 10 years, 1 case died of heart failure secondary to severe mitral regurgitation 3 months after the operation, 1 case had Ⅱdegree atrioventricular block , recovered 6 months later. 2 cases still had mild mitral regurgitation. Other cases lived and studied normally, their heart function is first class. Conclusion: Surgical treatment should be taken as soon as the diagnosis is confirmed. Shallow suturing repairing primum atrial defect and complete repairing mitral valve cleft are the key points of success and avoiding complications.
摘要:目的:探讨盆腔脓肿的临床表现,超声诊断结果及手术探查情况以提高诊疗水平。方法:对2000年1月~2006年12月我科收治的25 例手术治疗的盆腔脓肿病例进行回顾性分析。结果：其临床表现主要为腹痛、发热、腹部或盆腔包块等。超声声像显示:盆腔内不规则囊性或多房性输卵管卵巢肿块,囊内不均质光点,伴有或不伴有盆腔积液。手术探查表现为:盆腔粘连,一侧或双侧输卵管脓肿或输卵管卵巢脓肿。结论:盆腔脓肿的临床表现不典型,术前易出现误诊和漏诊;随着病情的变化,结合临床表现、 阳性体征的发现及超声诊断可以提高其检测率。Abstract: Objective: To explore the clinical symptoms, ultrasonography diagnosis results and surgical treatment results of pelvic abscess, thus to raise the diagnosing standard and treatment level from Jan.2000 to Dec. 2006. Methods: Carries on the review analysis to 25 example feminine pelvic abscess patient’s clinical diagnosis material. Results: The principal clinical symptoms of pelvic abscess are abdominal pain and fever,tissue mass is palpated in the abdomen or pelvic cavity and is detected. Sonographic appearance of the pelvis demonstrated irregular, uniloculated or multiseptated cystic tuboovarian mass with nonhomogenic internal echoes, with or without pelvic fluid collection. Surgical results showed some adhesions in pelvis, uniorbilateral pyosalpinx or tuboovarian complex abscess. Conclusion: The pelvic abscess displays not typically,,there is a comparatively high rate of misdiagnosis and missed diagnosis before the surgical treatment.The rate of missed diagnosis is by mistake high.Unifies the clinical symptoms,the masculine symptom, the auxiliary inspection and the guidance of ultrasound,may raise the diagnosis rate of accuracy.
ObjectiveTo review the research progress of the surgical treatment for lateral malleolus defect.MethodsThe related literature about surgical treatment and effectiveness of lateral malleolus defect at home and abroad was reviewed, summarized, and analysed.ResultsLateral malleolus defects are often caused by severe trauma or wide resection of fibular neoplasms. Although the incidence is not high, the defects are difficult to handle. These bony defects should be reconstructed to prevent an abnormal gait induced by ankle instability and avoid the occurrence of traumatic arthritis. Various repair methods have been developed, including bone transplantation, fibula lengthening, and ankle arthrodesis.ConclusionThere are various surgical methods to repair the defect of lateral malleolus, but each has its own advantages and disadvantages. In order to achieve the best results, the surgeon should choose the appropriate operation according to his own level, the patient’s specific injury, and age.
ObjectiveTo discuss the reoperation methods of urethral stricture after urethroplasty of hypospadias and their effectiveness.MethodsBetween September 2010 and April 2018, 169 patients with urethral stricture after urethroplasty of hypospadias, who underwent ineffective conservative treatments first, were accepted. The age ranged from 1 year and 7 months to 41years with a median age of 5 years and 8 months. The stricture located at the external urethral orifice in 80 cases, internal anastomosis connection in 87 cases, and constructed urethra in 2 cases. The symptoms of urethral stricture occurred at 2 weeks to 52 months after urethroplasty, with a median time of 4.5 months. The patients with external urethral orifice stenosis were treated with urethral meatus augmentation (74 cases) and urethral advancement (6 cases). The patients with internal anastomosis connection stenosis were treated with internal urethrotomy with urethroscopy (10 cases), urethrotomy and one-stage urethroplasty (26 cases), and urethrostomy (51 cases) including 43 cases of two-stage urethroplasty. The patients with constructed urethral stricture were treated with urethrolysis.ResultsOne hundred and fifty-four patients were followed up 6–86 months with an average of 47 months. The stenosis was relieved in 137 cases, and re-stenosis in 12 cases, urethral fistula in 4 cases, all of which were treated successfully. In addition, 1 case with mild urethral diverticulum did not need to be treated.ConclusionIf it is ineffective for the conservative treatment of urethral stricture after urethroplasty of hypospadias, appropriate surgical treatments could be selected according to the location and length of the stricture, local tissue conditions, complications, and so on.
Objective To investigate the safety and efficacy of preoperative ultra-short-course chemotherapy combined with surgical treatment of chest wall tuberculosis. Methods The clinical data of 216 patients with chest wall tuberculosis from January 2013 to June 2016 in our hospital were retrospectively analyzed, including 121 males and 95 females with an average age of 35±15 years (range, 4-74 years). Results All patients were treated with anti-tuberculosis drugs for 17.0±11.3 days preoperatively, including 12.5±5.0 days in simple chest wall tuberculosis and 19.4±12.3 days in combined chest wall tuberculosis. The postoperative recurrence rate of chest wall tuberculosis was 3.7%, which was close to or lower than that of routine preoperative antituberculous therapy in patients with ultra-short-course anti-tuberculosis treatment before surgery. Conclusion Preoperative ultra-short-course chemotherapy combined with surgical treatment for chest wall tuberculosis will not increase the recurrence rate of chest wall tuberculosis, and can effectively shorten the hospital stay. Timely adjustment of anti-tuberculosis chemotherapy based on thorough debridement, postoperative drugs, not the preoperative drugs, is the key to reinforce the surgical outcome.
ObjectiveTo review research progress of surgical treatment of patellar fractures.MethodsThe domestic and foreign literature about patellar fracture treatment in recent years was extensively consulted, and the advantages, disadvantages, and indications of various surgical treatments were summarized.ResultsThe patella plays an important role in knee flexion and extension activities, and the fracture significantly affects the patient’s quality of life. At present, the surgical methods include open reduction and internal fixation and patella resection. The internal fixation methods include ring/binding patella fixation, tension band wiring and improved technology, tension band wiring combined with other methods, screw fixation (including absorbable screws), steel plate fixation, and patella fixator fixation. Each surgical method has different indications, advantages, and disadvantages. Choosing an appropriate treatment plan plays a crucial role in clinical prognosis.ConclusionThere are many surgical treatments for patellar fractures. In order to improve the effectiveness and reduce postoperative complications, it is necessary to choose the most appropriate treatment strategy for the type of fracture.
Congenital coronary artery anomalies include various congenital anatomic anomalies of coronary artery. Severe congenital coronary artery anomalies usually result in ventricular dysfunction, myocardium ischemia, and even sudden death. Hence, understanding the coronary artery anomalies is critically important for the related surgeons to make reasonable strategies. This consensus is based on current literature and opinions of Chinese experts. And we mainly discuss the anatomy, clinical manifestation, diagnosis and treatment of important congenital coronary artery anomalies in clinics including anomalous aortic origin of a coronary artery, anomalous origin of the coronary artery from the pulmonary artery and coronary fistula.
Objective To review current status of surgical treatment for angular kyphosis in spinal tuberculosis and provide reference for clinical treatment. Methods The literature on the surgical treatment for angular kyphosis of spinal tuberculosis in recent years was extensively reviewed and summarized from the aspects of surgical indications, surgical contraindications, surgical approach, selection of osteotomy, and perioperative management. Results Angular kyphosis of spine is a common complication in patients with spinal tuberculosis. If kyphosis progresses gradually, it is easy to cause neurological damage, deterioration, and delayed paralysis, which requires surgical intervention. At present, surgical approaches for angular kyphosis of the spine include anterior approach, posterior approach, and combined anterior and posterior approaches. Anterior approach can be performed for patients with severe spinal cord compression and small kyphotic Cobb angle. Posterior approach can be used for patients with large kyphotic Cobb angle but not serious neurological impairment. A combined anterior and posterior approaches is an option for spinal canal decompression and orthosis. Osteotomy for kyphotic deformity include Smith-Peterson osteotomy (SPO), pedicle subtraction osteotomy (PSO), vertebral column resection(VCR), vertebral column decancellation (VCD), posterior vertebral column resection (PVCR), deformed complex vertebral osteotomy (DCVO), and Y-shaped osteotomy. SPO and PSO are osteotomy methods with relatively low surgical difficulty and low surgical risks, and can provide 15°-30° angular kyphosis correction effect. VCR or PVCR is a representative method of osteotomy and correction. The kyphosis correction can reach 50° and is suitable for patients with severe angular kyphosis. VCD, DCVO, and Y-shaped osteotomy are emerging surgical techniques in recent years. Compared with VCR, the surgical risks are lower and the treatment effects also improve to varying degrees. Postoperative recovery is also a very important part of the perioperative period and should be taken seriously. Conclusion There is no consensus on the choice of surgical treatment for angular kyphosis in spinal tuberculosis. Osteotomy surgery are invasive, which is a problem that colleagues have always been concerned about. It is best to choose a surgical method with less trauma while ensuring the effectiveness.
Tetralogy of Fallot is the most common cyanotic congenital heart disease. The pathological anatomy changes include ventricular septal defect, right ventricular outflow tract stenosis, aortic stradding and right ventricular hypertrophy. At present, the diagnostic criteria and treatment strategies of this disease are basically unified. However, there are controversies about the timing and method of surgical treatment. Based on the evidence-based information provided in the literature and the opinions of domestic experts of China, we formulate a consensus of Chinese experts to further standardize the surgical treatment of tetralogy of Fallot.