心血管造影在复杂和(或)复合先天性心脏病诊断中的应用

目的 探讨心血管造影在先天性心脏病（简称先心病）复杂和（或）复合畸形中的应用价值。方法 分析360例复杂和（或）复合畸形造影所见及其与超声心动图等临床检查的联系。结果 本组360例（包括75例肺动脉闭锁合并室间隔缺损、62例右室双出口、60例法乐四联症、52例单心室、42例大动脉错位、15例三尖闭锁、6例冠状动脉异常、5例完整型肺静脉畸形连接、5例完全型心内膜垫缺损、4例共同动脉干、3例室间完整的肺动脉闭锁、7例其他病例和24例外科术后检查）心血管造影和超声对比，纠正后者误、漏诊分别为34、30例及对合并畸形误诊16例。对复杂和（或）复合畸形中体肺侧支血管、冠状动脉畸形和肺动脉段分支及其异常的检测和诊断优于超声心动图，并可测量肺动、静脉压力及体肺侧支血管压力而优于其他影像学检查方法。结论 对于先心病复杂和（或）复合畸形的疑难病例诊断和鉴别诊断，尤其显示体、肺及冠状动脉分支的全貌及相关病变，以及测量肺动脉和心室压力等，心血管造影（含DSA）仍有重要或不可替代的作用。     

先天性胆总管囊肿23例的术式及疗效分析

目的：探讨先天性胆总管囊肿的术式及疗效。方法：回顾分析手术治疗先天性胆总管囊肿23例，其中Roux-Y囊肿-空肠吻合8例；囊肿切除，Roux-Y肝总管-空肠吻合15例。结果：20例获得随访，Roux-Y囊肿-空肠吻合随访6例，仅2例无症状，另4例反复发作腹痛，发热或黄疸，其中1例再手术，囊肿切除，Roux-Y肝总管-空肠吻合随访14例。仅1例有腹痛，余13例无症状，结论：Roux-Y肝总管-空肠吻合是治疗先天性胆总管囊肿的首选术式。     

先天性髋脱位骨刀模型的研究

目的：在建立先天性髋脱位（CDH）的计算机三维交互模型的基础上，建立三维骨刀模型，模拟截骨。方法：利用螺旋体积CT获得患儿髋关节扫描数据，用B＋树组织按marching cubes方法完成骨刀模型的重建。通过对B＋树的不完全遍历和回溯，定位表面模型间的交点并分割髋关节模型。结果：重建的骨刀模型与CDH三维模型之间，可进行实时交互。初步实现了对骨性三维交互模型任意位置的平面切割、球面切割、拾取和拼合。结论：此建模、切割方法可行有效，可用于计算机模拟骨性手术的研究。     

Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication.

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.     

Double congenital bronchoesophageal fistulae in an adult

We report adult congenital bronchoesophageal fistula with both symptomatic fistula and asymptomatic one. A 56-year-old woman with a history of cough after drinking fluids was diagnosed as bronchoesophageal fistula by upper gastrointestinal series that showed a diverticulum in the middle portion of the esophagus with a fistula between the esophagus and right lower lung. Esophagoscopy revealed an orifice of the fistula located 27 cm from the incisors. Computed tomography showed chronic inflammatory change with bronchiectasis in the S6 segment of the right lung. The patient underwent video assisted thoracic surgery that identified two fistulae without missing a symptomatic one, and both were successfully resected. The fistulae were lined by squamous epithelium and smooth muscle without evidence of malignancy, infection or chronic inflammation that were histologically compatible with congenital fistulae.     

Ocular findings in familial adenomatous polyposis

Background: Retinal pigment epithelium (RPE)lesions are predictive congenital phenotypic markersfor familial adenomatous polyposis (FAP). Thisprospective screening study aims at assessing theincidence and significance of these lesions in FAPpatients and their family members.Methods: Sixty-two members from three familiesincluding five patients with the diagnosis of FAP havebeen ophthalmologically surveyed. All RPE lesions weredocumented with fundus photography and fluoresceinangiography was performed in 13 subjects.Sigmoidoscopy and/or radiological examination wereperformed annually in 9 family members with typicalRPE lesions during 4 years to allow early diagnosis ofFAP.Results: Typical RPE lesions were present infive FAP patients and 15 family members.Telangiectatic dilatations in the retinal peripherywith small dot-like hemorrhages were detected in 6subjects from 3 families These lesions wereparticularly evident on fluorescein angiography.Annual colon analysis showed polyps in 3 out of 9subjects who were positive for RPE lesions.Conclusion: RPE lesions are valuable as aclinical marker in predicting FAP. The co-existingperipheral vascular alterations which have not beenreported before, are probably related to FAP.     

巢式PCR检测先天性心脏病心脏石蜡标本中B19病毒的感染

目的：探讨微小病毒Ｂ１９与先天性心脏病（ＣＨＤ）的相关性及可能致畸机理。方法：采用病例对照研究，病例组为２９例ＣＨＤ尸解心脏组织，对照组为３０例同期非先天性畸形尸解心脏组织，应用巢式ＰＣＲ扩增Ｂ１９－ＤＮＡ，单纯疱疹病毒（ＨＳＶ），兔弓形虫（ＴＯＸ），巨细胞病毒（ＣＭＶ），结果：２９例ＣＨＤＢ１９－ＤＮＡ５例阳性，全瓿对照组为均阴性（Ｐ＝０．０２３７），ＨＳＶ，ＴＯＸ两组中均阴性，ＣＭＶ在两组中均