新生儿先天性畸形与孕妇乙型肝炎病毒感染关系的研究

为探讨孕妇乙型肝炎病毒(HBV)感染和某些因素对先天性畸形的致病作用,本文进行了96对新生儿先天畸形的配对(1:1)病例对照研究,报道先天畸形的频率和构成,单因素和多因素的条件Logistic回归分析,表明孕妇HBsAg阳性等6个因素对先天畸形呈阴性结果,而与接触农药和孕周的因素有明显联系,值得进一步探讨。     

小儿先天性髋关节脱位近年来应用术式的演变及其评估

本文介绍本院近４年来小儿先天性髋脱位治疗应用术式的演变。由２０多年来一直喜欢用骨盆截骨术演变为近１年来用得最多的是造盖术。近４年共手术４６３例，５６５髋。实践证明：比起骨盆截骨术，造盖术显得更简便，侵袭小，不需二次手术拔除内固定针。因在我国大龄髋脱位偏多，造盖术更适合我国国情。经过一些改进，可以克服造盖折断和吸收问题。     

先天性输精管缺如患者生育问题的研究

自1988年至1991年共收治25例先天性输精管缺如患者,在20例行手术诊治术中15例用自体睾丸精索鞘膜制成人工精池囊,5例又在囊内放置用微涤纶制成的异质管。术中抽吸10例附睾内精子进行快速活化,其中5例活化成功,行人工授精,结果2例怀孕,1例已生育一女孩。认为该症是有生育可能的,但需要解决3个问题:(1)采用显微外科技术把附睾内部活着的精子取出一定数量;(2)选配好快速活化剂使精子快速活化成功;(3)用患者的精浆培养自己的精子。建议对梗阻性无精子症在用手术疏通精路的同时可以采用快速活化精子技术行人工授精,以提高生育的效果。     

深圳市公务员先天性白内障现状及发病因素探讨

目的　通过普查深圳市公务员先天性白内障的现状 ,分析影响先天性白内障患病因素 ,探讨有效的防治措施。　方法　在 2 0 0 3年参加深圳市保健中心健康人群体检 ,2 0岁以上的约 3万名公务员中 ,按 3 :1比例随机抽取93 40人调查。按照先天性白内障诊断及分类标准 ,分析年龄、性别、职业的相关性 ,并进行显著性检验。　结果　深圳市公务员先天性白内障总发病率为 0 86% (80 /93 40 ) ,其中男性为 0 87% (4 0 /5 0 40 ) ,女性为 0 84% (3 6/4 3 0 0 ) ,男性高于女性 (P <0 0 5 ) ,职业无明显差异 (P <0 0 5 )与家族史有关。　结论　先天性白内障病发展缓慢 ,发现困难 ,应作为常规体检项目 ,减少遗传病儿出生。     

Management of congenital tracheal stenosis

Objectives: Congenital tracheal stenosis is a rare disease. Various methods for treatment exist but there is still much debate as to the appropriate surgical procedure. We present our surgical experiences of patch tracheoplasty and slide tracheoplasty as viable methods for the treatment of congenital tracheal stenosis. Methods: From 1994 to 2002, 13 patients were diagnosed with congenital tracheal stenosis. Eight patients (7 symptomatic and 1 asymptomatic) had their stenosis corrected, three by means of pericardial patch tracheoplasty, four by slide tracheoplasty, and one by resection and anastomosis. Concomitant operations were performed on six patients to treat congenital cardiovascular disease. Five patients showing no significant symptoms did not undergo tracheal surgery and received only cardiac procedures. A retrospective review of the hospital course, complications, and long-term results was conducted. Results: Among the patch tracheoplasty group, every patient suffered from granulation tissue formation. One patient died of respiratory acidosis and one was hospitalized due to recurrent granulation tissue, which required frequent bronchoscopy. The third patient from this group is free of all symptoms. Among the slide tracheoplasty group, one patient died of anastomosis disruption. The three remaining patients are alive and well. The one patient who received resection and anastomosis is alive without symptoms. Conclusions: Surgical repair of long-segment congenital tracheal stenosis exhibited high mortality and morbidity rates. Every patient that underwent pericardial patch tracheoplasty suffered from troublesome granulation tissue. As slide tracheoplasty provided relatively good results in the short and mid-term follow-up periods, it seems to be a preferred method for the treatment of long-segment congenital tracheal stenosis.     

Results of the Ross operation in a pediatric population

Objective: To analyse the results of the mid-term clinical and echocardiographic follow-up of the pediatric Ross operation. Methods: Echo-Doppler follow-up of 53 consecutive pediatric Ross procedures performed between 1994 and 2003. Median age was 9.7 years at time of operation (2 weeks–17.7 years). Six patients were younger than 3 months. Median age at follow-up was 15.6 years. Aortic valve/left ventricular outflow tract (LVOT) anomalies were congenital in 49 (92%). Seventy percent had previous surgery or balloon valvuloplasty. Root replacement was used in all. Thirteen patients (25%) had LVOT enlargement. Mean cross-clamp time was 113 (69–189) minutes. Results: Early mortality occurred in 3 patients after emergency surgery following balloon failure (n=1) and extended Ross following interrupted arch/VSD repair (n=2). Late mortality was due to LV fibroelastosis in 2 patients and complicated pulmonary artery stenting in another. RVOT reoperations were required because of late homograft obstruction in 2 patients and because of pulmonary artery stenosis in another. Five patients (9.4%) were reoperated for pulmonary autograft dilatation (n=3) and for leaflet fibrosis or perforation (n=2). Autografts were repaired in two patients, while a mechanical valve was inserted in 3 cases. At 9 years the actuarial survival and event free survival were 89 and 74%, respectively. At last follow-up 90% of autograft diameters indexed to body surface area was above the 90th percentile of normal aortic root diameters. LVOT and RVOT gradients were low and autograft insufficiency was trivial to mild in 84% and mild to moderate in 16%. Autograft stenosis was not noticed. Conclusions: The pediatric Ross procedure remains an important tool but autograft dilatation also occurs in the pediatric population. The significance of this finding has yet to be determined.     

Significance of beaten copper appearance on skull radiographs in children with isolated sagittal synostosis

Aims and Objectives The significance of beaten copper appearance (BCA) on skull radiographs in children following surgery for isolated sagittal craniosynostosis has not been studied. This study was designed to look for any correlation between BCA and symptoms suggestive of intracranial hypertension in this group of patients. Materials and Methods Forty-eight consecutive children, who were operated for isolated sagittal synostosis from1987 to 2000 and had postoperative skull radiographs, were included. Patients were divided into: (a) BCA group (n = 20), consisting of children who had beaten copper appearance on skull radiographs at last follow up, and (b) Non-BCA group (n = 28), consisting of children who did not have this finding. Records were reviewed to look for symptoms suggestive of intracranial hypertension, such as headache, head banging, and irritability. Results Median age at surgery was 4.8 months for BCA group and 4 months for the non-BCA group. Follow up ranged from 4 to 156 months with a mean of 36.2 months. Total of 28.6% (n = 6) of the children with follow up radiographs done at ≤18 months of age had BCA. The incidence of BCA increased to 83.3% in children with skull radiographs performed after 48 months of age. In 18 (90%) children, the BCA was ‘diffuse’ with 5 (25%) children having the maximum possible score of 8. In the BCA group, 45% (n = 9) had symptoms compared to 10.7% (n = 3) in the control group (p = 0.0068). Conclusions This study suggests a significant number of children with BCA on radiographs develop symptoms suggestive of raised ICP following surgical treatment in infancy and prolonged follow up may be warranted in this group of patients.     

Teratoma tongue: Case report and review of literature

Teratomas are true neoplasms composed of tissues from all three germinal layers and may exhibit variable levels of maturity. They have an unknown origin and eccentric microscopic appearance. Teratomas arising from the oral cavity are rare in the newborn; only 22 cases have been reported in the literature. We describe a female neonate with an oral teratoma originating from the tongue that was successfully treated with surgery.     

矢状缝早闭(舟状头)全颅成形术

目的探讨治疗矢状缝早闭(舟状头)畸形的手术方法。方法采用David“‖”形颅缝重建法(6例)、旋转骨瓣截骨法(2例)、梅花颅骨瓣头颅盖成型法(3例)等术式对舟状头进行矫治。结果11例患儿均治愈,外形满意。结论1~3个月舟状头宜采用简单的David“‖”形颅缝重建法进行治疗,3个月以上的患儿可选用浮动颅骨瓣头颅成型术、梅花瓣法颅骨瓣头颅盖成型术等方法进行治疗。