Diagnosis and management of congenital vascular rings: a 22-year experience.

Between 1968 and 1990, we operatively treated 39 patients (19 boys, 20 girls) with congenital aortic arch anomalies. Median age was 7 months (range, 1.5 months to 23 years). Thirty-seven patients (95%) had respiratory symptoms. Barium swallow was diagnostic in 95%. Right arch with aberrant left subclavian artery and double aortic arch were the most common types (11 each). Treatment of an aortic diverticulum was documented in 19 patients; the aortic diverticulum was excised (9), managed by aortopexy (7), or left in situ (3). Postoperative recovery was rapid, with a median intensive care unit stay of 2 days, time to oral feeding of 1 day, and postoperative time to discharge of 7 days. Two deaths occurred: 1 infant had undergone emergent operation for control of hemorrhage from an aortotracheal fistula due to tracheostomy tube erosion, and the other had multiple associated congenital heart defects. Postoperative complications included bleeding (1), pneumonia (5), and chylothorax (4). One boy had persistent severe symptoms due to an untreated aortic diverticulum and underwent subsequent excision of the aortic diverticulum with complete relief of symptoms. Median length of follow-up was 12.5 months, with at least 97% of survivors completely or nearly completely free of symptoms from the vascular ring. These results suggest that early repair of congenital aortic vascular rings, including fixating or excising an associated serious aortic diverticulum, is safe and effective and allows for normal tracheal growth.     

先天性胆管囊肿的外科治疗（附131例分析）

本文报告先天性胆管囊肿１３１例，其中Ⅰ型８９例，Ⅱ型２例，Ⅲ型４例，ＩＶ．型２５例，Ｖ型１１例。１１９例施行手术治疗。作者认为：对先天性胆管囊肿的手术治疗，首次手术时应尽可能切除肝外胆管囊肿，有困难时可大部切除，行高位大口胆肠吻合。对局限于肝段或一侧肝叶的胆管囊肿，可行肝段或一侧肝叶切除。对左右肝叶均有胆管囊肿者，可切除病变较重的肝叶或部分肝组织，解除主要肝管的狭窄与扩张，行肝管空肠吻合术。     

先天性血管环的外科治疗

目的 讨论先天性血管环的诊断和手术治疗及影响预后的因素.方法 回顾性分析2010年1月至2013年12月收治的42例先天性血管环患儿(占同期手术患儿的1.04％)的病例资料.其中男性26例,女性16例;手术年龄24d至6岁,平均10.7个月.包括肺动脉吊带26例,双主动脉弓10例,右位主动脉弓合并迷走左锁骨下动脉3例,肺动脉吊带合并右位主动脉弓并迷走左锁骨下动脉2例,肺动脉吊带合并左位主动脉弓并迷走右锁骨下动脉1例.38例患儿合并其他的心血管畸形.36例患儿行螺旋CT检查气道重建,23例患儿行纤维支气管镜检查,除2例气道未见异常外其余患儿均存在不同部位不同程度的气管狭窄或软化.所有患儿均行手术矫治先天性血管环,根据情况同期行心内畸形矫治手术;1例同期行气管狭窄切除并Slide成形术,1例在术后行气管内支架植入术.结果 3例患儿院内死亡(7.1％),死亡原因均为术后反复发生气管内肉芽肿形成;其余39例均顺利出院.存活患儿总体术后呼吸机辅助时间14 h(22 h)[M(QR)],心脏ICU停留时间5d(8 d),住院时间19 d(9 d).肺动脉吊带患儿呼吸机辅助时间、心脏ICU术后停留时间均长于双主动脉弓患儿[23 h(123 h)比9 h(9 h),7 d(13 d)比4 d(2 d)],但差异无统计学意义(P＞0.05);住院时间明显长于双主动脉弓患儿[23 d(9 d)比16 d(6 d)],差异有统计学意义(x2=10.157,P=0.006).结论 先天性血管环早期手术治疗是安全、有效的,近期效果良好.合并的气管软化和狭窄的程度和范围是影响患儿预后的关键.     

Surgical management of congenital pulmonary valve dysplasia.

Pulmonary valvular stenosis secondary to congenital valve dysplasia differs markedly from the classic variety of pulmonary stenosis. The reported mortality of patients treated by standard commissurotomy is 38 to 66%. The clinical features and operative management of 14 patients with dysplastic pulmonary valves are reviewed. Three groups of patients were studied. Group 1 consisted of 5 patients treated by commissurotomy. Group 2 comprised 3 patients treated by partial excision of the valve. In neither group were there operative deaths, but 5 of the 8 patients developed recurrent stenosis; 3 required reoperation. In 1975, because of the high incidence of recurrent stenosis, total valvectomy was begun. Ten patients (Group 3) have undergone valvectomy with 1 death. Nine patients were doing well at 3 to 15 months of follow-up. Based on the reported mortality and present findings, total excision of the valve is recommended for relief of stenosis in pulmonary valve dysplasia.     

Surgical management of congenital coronary artery fistulas.

Congenital fistulas are the most common of the coronary arterial malformations and with the widespread use of selective coronary arteriography are being recognized with increasing frequency. Twenty-eight patients with congenital coronary fistulas have been evaluated at the Duke University Medical Center between 1960 and 1981. An additional 258 patients have previously been reported in the literature, making a total of 286 available for review. The right coronary artery is most commonly involved, and the fistulous communication is most often to the right ventricle, right atrium or pulmonary artery. Slightly more than half of the patients with coronary fistulas are symptomatic at the time the diagnosis is made. Surgical correction is strongly recommended to prevent the development of congestive heart failure, angina, subacute bacterial endocarditis, myocardial infarction, and pulmonary hypertension, as well as coronary aneurysm formation, with subsequent rupture or embolization. There were no operative or late deaths in the patients who underwent operations. Moreover, there have been no recurrent fistulas during a mean follow-up period of ten years. The risks of operative correction appear to be considerably less than the potential for development of serious and potentially fatal complications, even in asymptomatic patients.     

Surgical management of congenital upper-eyelid eversion

We describe a case of congenital eversion of the upper eyelids in which early surgery was performed to prevent occlusion amblyopia. The surgery, described here for the first time for this condition, consisted of using multiple suture placements to anatomically reposition the eyelids.     

Surgical management of congenital tracheal stenosis

Congenital tracheal stenosis (CTS) is a rare but life-threatening disorder, particularly in neonates and infants. The stenotic lesions are commonly composed of complete rings of cartilage varying in length, location, and severity. A definitive diagnosis of CTS may be delayed because of the rarity of this disorder and thus its unfamiliarity among physicians, its variable onset time, its various clinical symptoms, and the diversity of associated clinical conditions due to the cardiovascular disorders that may accompany it. More than half of the patients who manifest clinical symptoms during early infancy show a long-segment stenosis. Long-segment CTS is problematic and challenging to manage. Over the last two decades several surgical techniques for long-segment CTS have been devised and developed, but no definitively advantageous surgical procedure has been established because of insufficient experience and the lack of large-scale studies. Although rib cartilage tracheoplasty and pericardial patch tracheoplasty have provided limited early to midterm success for infants with long-segment CTS, these procedures are associated with early and late complications, including granulation tissue formation, patch collapse, and restenosis necessitating reintervention. By contrast, slide tracheoplasty has given rise to better surgical outcomes. This procedure enables reconstruction of the stenotic trachea using native tracheal walls with preserved blood supply. The trachea is foreshortened by only one-half of the original stenosis, resulting in reduced tension on the anastomosis. Consequently, the technique has several advantages, including less formation of granulation tissue, satisfactory subsequent growth, and infrequent reoperation for restenosis. Slide tracheoplasty is currently recognized as the preferred technique for long-segment CTS. __________ This review was submitted at the invitation of the editorial committee.     

Surgical management of congenital cervical kyphosis

Congenital cervical kyphosis is a rare clinical condition. The purpose of this study was to review the surgical management and outcomes of 12 consecutive cases of congenital cervical kyphosis management by the same surgical team. The authors retrospectively analyzed the records of 12 patients (5 men and 7 women) with an average age of 18.4 years (range, 15-31 years) who underwent surgery for congenital cervical kyphosis at the authors' institution between 2001 and 2005. All patients had congenital cervical kyphosis; those with secondary kyphosis deformity due to causes such as infection, tumors, and surgery were excluded. The indications for surgery were signs of spinal cord compression with progression of clinical symptoms such as decreased muscle strength and paresthesia. All patients had radiographic evidence of cervical kyphosis. Six patients underwent anterior decompression, autogenous bone grafting, and instrumentation, and the other 6 patients underwent combined anterior-posterior surgery. All surgeries were performed successfully with no complications. Bone graft fusion occurred in 11 patients. In 1 patient who underwent anterior surgery, the bone graft was partly absorbed, and pseudarthrosis was noted at 3 years postoperatively. Mean Japan Orthopaedic Association cervical myelopathy score and mean Cobb angle were significantly improved at 1 week and 1 year postoperatively compared with preoperative values.Anterior and combined anterior-posterior surgical approaches are useful for the correction of congenital cervical kyphosis. Bone graft fusion is also critical for maintaining the surgical correction. Choice of surgical methods depends on the patient's clinical condition.     

先天性胆管囊肿的外科治疗及疗效分析

目的 探讨先天性胆管囊肿外科治疗手术方式对疗效的影响。方法 在15年间收治先天性胆管囊肿患者120例，在本院首次手术者73例。手术方式分3类5种。I类：单纯囊肿外引流术（7例）；Ⅱ类：囊肿空肠Roux-en-Y吻合术（5例）；Ⅲ类：囊肿切除并肝管空肠Roux-en-Y吻合（32例）或囊肿切除并间置空肠肝管十二指肠吻合术（25例）；不规则手术4例。结果 68例术后随访6个月－5年（中位时间2．年），Ⅰ类手术再手术3例；Ⅱ类手术后因出现反流性胆管炎和胆道结石而再手术2例；Ⅲ类手术类后生活质量达优率为88．7％，无再手术。结论 单纯囊肿外引流只是一种应急措施；囊肿内引流术只有能解决短期的胆汁引流问题，并发症多导致再次手术；囊肿切除并胆道理建术是比较理想的治疗方式。     

Surgical management of congenital lymphedema in infants and children.

Of 67 children and infants with lymphedema, 28 had the congenital type. Congenital lymphedema appears during the first few weeks of life, frequently involves more than one extremity, and enlarges at a slower rate than general body growth. The swelling usually becomes less pronounced with age, and no specific therapy is required in two thirds of the patients. Seven of the 28 children had swelling of the upper extremities and a generalized lymphangiopathy syndrome. Subcutaneous lymphangiectomy was performed on ten of 28 patients who had moderate to severe swelling. Those with hand and arm involvement were particularly benefited; however, operations on the dorsum of the foot produced hypertrophic scars in one third of the cases. The operation is deferred until after age 2 years to permit optimal technical repair and to identify those patients whose conditions will improve spontaneously.     

全身大血管损伤的外科处理

目的探讨全身大血管损伤的早期诊断和修复对挽救生命及维护器官功能的重要性.方法对1991年至2001年期间32例40条全身大血管损伤分析诊断的相关因素及外科处理要点.结果截肢3例,死亡3例,急诊手术26例,随访1～7年,血管修复满意.结论早期诊断和复苏,尽快修复受损血管,提高血管畅通率,能有效挽救肢体和生命,减少术后并发症.     

Surgical management of the congenital neuropathic bladder

We have previously described 3 types of lower urinary tract dysfunction in the congenital neuropathic bladder--contractile, intermediate and acontractile--accounting for 35, 40 and 25% of patients respectively. Subsequent urodynamic and surgical experience has shown that the intermediate type is commoner and the acontractile type less common than was thought. The contractile group accounts for 31% of patients and rarely requires surgery (a "clam" ileocystoplasty). The acontractile group accounts for only 9% but more commonly requires surgical treatment, usually the implantation of an artificial urinary sphincter (AUS). The intermediate group accounts for 60% of patients and usually requires surgery. If there is no significant sphincter weakness incontinence, a "clam" ileocystoplasty alone is performed; if there is sphincter weakness, a "clam and a cuff" procedure is performed with implantation of the remainder of the AUS at a later date, if required.     

Congenital vascular rings: surgical management of 111 cases

Between October 1974 and May 1987, 111 congenital vascular rings were submitted to surgical correction. There were 83 infants (age: 5 days-12 months, median: 7 months; weight: 1.9-8.5 kg, median: 7.1 kg), and 28 children (age: 1-13 years, median: 3.5 years; weight: 7.5-48 kg, median: 26.5 kg). Patients were divided into five groups: (1) double patent aortic arch (44 cases), (2) double aortic arch with atresia in different parts of the left arch (36 cases), (3) right aortic arch with left ligamentum arteriosum (21 cases), (4) left aortic arch with aberrant right subclavian artery and truncus caroticus (8 cases), and (5) pulmonary artery sling (2 cases). We had no intraoperative mortality but in the postoperative period, 2 neonates died of severe bacterial infections of the respiratory tract.