Aplasia cutis congenita of the scalp with large underlying skull defect: a case report

Localised agenesis of the scalp is the most frequent patern in aplasia cutis congenita (ACC), a congenital absence of the skin and occasionally of deeper layers. Several clinical groups are characterised by the location and pattern of skin defects, associated malformations and the mode of inheritance. Death occurs in 20% of cases, secondary to the associated anomalies, to infections or to haemorrhage from ulceration of the sagittal sinus when there is also a defect of the underlying skull. In this latter case, we close the defect by two rotational scalp flaps (Orticochea technique) at birth. A three-dimensional CT study is useful for showing the extent of the skull defect and the deformity of the craniofacial complex and the changes in the bone after treatment.     

小儿先天性巨结肠42例手术疗效分析

手术治疗小儿先天性巨结肠４２例，其中Ｄｕｈａｍｅｌ术９例，Ｓｗｅｎｓｏｎ术１１例，Ｓｏａｖｅ术６例，Ｒｅｈｂｅｉｎ术１０例，直肠后壁肌切除４例，直肠肛管纵切心形斜吻合术２例。术后污粪、便秘、小肠结肠炎及肛门狭窄发生率２１％。认为：手术疗效是与术前充分准备，选择适宜术式，术中精细操作，术后加强护理，精心喂养等直接相关     

Revascularization of a thrombosed aortopulmonary shunt with the use of the AngioJet thrombectomy system.

We report the use of the AngioJet F140 rheolytic catheter to recannalize an acutely thrombosed aortopulmonary shunt in a 21-year-old female with palliated, complex congenital heart disease. After extracting the thrombus that filled the entire length of the shunt, three stents were placed at sites where the lumen was compromised by distortion or thrombus. Unobstructed flow was restored to the left pulmonary artery that persisted at 1-year follow-up.     

Congenital esophageal atresia successfully treated by early ligation of a tracheoesophageal fistula and delayed repair of the esophagus in a premature infant: Report of a case

We report herein the case of a premature infant with esophageal atresia (EA) and a tracheoesophageal fistula (TEF) associated with cardiac anomalies who was successfully treated by an early ligation of the TEF following gastrostomy, and delayed repair of the esophagus. A 1212-g male was born prematurely at 31 gestational weeks, at which time he was diagnosed as having EA with TEF and patent ductus arteriosus (PDA), ventricular septal defect (VSD), and atrial septal defect (ASD). A gastrostomy was initially performed but following extubation he gradually became tachypneic. A chest roentogenogram revealed atelectasis and ground-glass appearance, and reintubation was required. Ligation of the TEF was performed 53h after his birth. Following the improvement of his respiratory condition through ventilatory support and the intratracheal administration of pulmonary surfactant, he underwent repair of the esophagus on the 6th day of life. Postoperatively, he suffered from heart failure, but was treated with peritoneal dialysis and pharmacological closure of the PDA. Weaning the infant from the ventilator proved difficult, but it was finally achieved when he had reached a weight of 2268g at 3 months of age by enteral feeding. Our experience of this case demonstrates that early ligation of TEF should be performed for a premature infant with EA and TEF before respiratory distress syndrome (RDS) has developed. If a gastrostomy is required to prevent gastric distention, it should be followed by simultaneous or immediate ligation of the TEF.     

先天性心脏病患儿术后多脏器功能障碍的预后分析

目的 探讨小儿先天性心脏病(先心病)术后多脏器功能障碍(MODS)预后情况及其影响因素,为对此类患儿进行针对性护理提供依据.方法 收集先心病术后并发MODS 77例患儿的临床资料.结果 11例放弃治疗出院,66例中44例救治存活,22例死亡.出现时间最早、累及最多的脏器为心脏;病死率最高的为累及中枢神经系统的患儿(57.69%),其次是累及血液系统的患儿(55.56%);患儿的病死率与累及脏器的数量呈显著正相关(P＜0.01).死亡患儿手术体外循环时间和主动脉阻断时间显著长于存活患儿(均P＜0.05),术中意外及术后心肺复苏发生率显著高于存活患儿(均P＜0.05).结论 先心病术后患儿应加强心功能监护,特别是体外循环时间＞120 min,主动脉阻断时间＞60 min及术中发生过意外情况、术后采取过心肺复苏术的患儿;尽早采取有利措施避免其他脏器功能受损是提高患儿存活率的关键.     

Ross procedure in an infant weighing 4.5 kg

A 6 month-old male infant (weight: 4.5 kg) with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft (Ross procedure). The right ventricular outflow tract (RVOT) was reconstructed with a polytetrafluoroethylene (PTFE)-valved equine pericardial conduit. At the age of 5, re-RVOT reconstruction with an equine pericardial patch bearing a PTFE monocusp was required because of severe pulmonary stenosis resistant to 2 attempts of percutaneous transluminal pulmonary valvotomy. Currently, at the age of 8, the degree of aortic regurgitation is trivial and the pulmonary autograft is free of functional deterioration despite somatic growth.     

Pemberton手术为主多种辅助手段治疗大龄儿童先天性髋脱位

目的　应用Pemberton手术为主多种辅助手段治疗大龄儿童先天性髋脱位 ,从而降低术后髋关节脱位、僵硬和股骨头坏死发生率。方法　自 1989年 8月 - 1999年 8月 ,用Pemberton手术为主多种辅助手段治疗大龄儿童 (7～ 14岁 )先天性髋脱位 4 6例 (5 8髋 ) ,随访 2～ 9年 ,平均 5年 3个月。辅助手段包括 :①髂腰肌及内收肌切断 ,股骨髁上骨牵引 ;②股骨粗隆下旋转加短缩截骨 ;③单髋人字石膏制动 ;④髋关节早活动 ,晚负重 ;⑤术后被动活动髋关节加主动功能锻炼。结果　髋关节完全复位率10 0 % ,髋关节屈曲小于 90°者 4髋 ,占 6 9% ,按Salter提出的股骨头坏死诊断标准 4 6例 (5 8髋 )中 ,股骨头坏死 6髋 ,占 10 3% ,明显降低了大龄儿童先天性髋脱位术后髋关节僵硬、股骨头坏死率。结论　Pemberton手术为主多种辅助手段治疗大龄儿童先天性髋脱位疗效确切 ,优良率高。     

Percutaneous transcatheter occlusion of coronary artery fistulas using detachable balloons

Three pediatric patients underwent successful transcatheter coronary artery fistula occlusion using the Debrun system. This latex balloon system offers several advantages over other occlusion systems. First, the balloon delivery and release is controlled. Second, “test occlusions” can be performed that allow simultaneous balloon inflation, coronary cineangiography, and electrocardiographic monitoring. Third, because the balloons are flow-directed, they are easily positioned in properly chosen locations. Finally, the balloons can be constructed to suit the size of the fistula. In this study, two patients received only one balloon; in the other patient two balloons were placed in the same fistula. All fistulas drained into either the right atrium or ventricle and were successfully occluded. After a follow-up period of up to 3 years, no local or systemic reactions to the balloons were recognized. We conclude that detachable balloon occlusion of coronary artery fistulas is a safe, effective alternative to surgical ligation in selected pediatric patients.     

先天性肾上腺皮质增生伴睾丸间质细胞腺瘤性增生一例报告并文献复习

目的提高对先灭性肾上腺皮质增生伴睾丸间质细胞腺瘤性增生的认识。方法总结1例先天性肾上隙皮质增生伴睾丸间质细胞腺瘤性增生患者资料。患者，男，25岁。以双侧睾丸结节就诊。查体见双侧睾丸、附睾结节感，右睾丸增大，表面凹凸不平，质地硬。实验审检查睾酮（T）18．7nmol／L、17α-羟化酶（17α-（OH）P）〉20．0ng／ml、快速ACTH兴奋试验阳性、中剂量地塞米松抑制试验阳性。B超提示双睾丸弥漫性病变合并部分占位，示双侧肾上腺皮质增生。睾丸活枪结果为间质细胞增生结节，免疫组化α-inhibin（＋）。结果结合病史、实验室、影像学及病理学检查诊断为先大性肾上腺皮质增生伴睾丸间质细胞腺瘤性增生。口服氢化可的松替代治疗，40mg／d，2周后30mg／d维持治疗，8个月后超声检查双侧睾丸结节消退。随访2年，肿物无复发。结论双侧睾丸肿块应结合病史和内分泌检查除外先天性肾上腺皮质增生伴睾凡间质细胞腺瘤性增生，经口服皮质激素替代治疗，激素敏感型睾丸间质细胞腺瘤性增生可以消退。