成人双脐尿管未闭1例

脐尿管是脐与膀胱间疏松结缔组织内的一条纤维索,由胚胎期尿囊管退化而成.脐尿管未闭畸型为泌尿系少见的先天性疾病,多数为儿童发病,国内报道发病率约为1/30万[1],以男性多见.本文报道成人双脐尿管未闭1例,并结合文献对成人双脐尿管未闭的诊断、治疗作一介绍.     

先天性脐尿管瘘11例治疗体会

目的:探讨先天性脐尿管瘘的治疗方法。方法:回顾性分析11例采用脐尿管切除术先天性脐尿管瘘患者的临床资料。结果:所有患者均痊愈出院,脐下伤口无感染,手术时间约30~50 min。结论:先天性脐尿管瘘均需手术治疗,手术应以保留脐部彻底切除脐尿管为宜。     

超声检查对脐尿管病变的诊断价值

脐尿管来源的先天性畸形以脐尿管瘘、脐尿管囊肿、脐尿管窦相对多见，而脐尿管畸形所致膀胱憩室、后天性病变继发恶变较为少见。本文对我院1996-2004年收治的16例脐尿管来源性病变进行临床体征、超声表现、病理结果综合分析，效果满意，现报告如下。     

脐尿管未闭24例分析

脐尿管未闭合为先天性畸形之一，临床少见。近年来，我院收治24例，治疗效果满意，报告如下。     

14例脐尿管瘘治疗体会

脐尿管瘘为少见的泌尿系统先天性畸形,1984～1999年我院共收治14例,现就临床诊治体会报道如下.     

成人脐尿管囊肿并脐部瘘管形成1例报告

<正>脐尿管囊肿是少见的先天性畸形,大部分是由于胚胎时期的尿囊管残余在发育过程中未能自行纤维化闭塞所引起的先天性疾病。以男性居多,临床上易误诊。现将我院近期收治的1例脐尿管囊肿并感染的典型病例介绍如下。     

脐尿管病2例误诊教训

脐尿管疾病是泌尿系少见的先天性畸形，我院自1984～2002年共收治2例，均误诊，现报道如下。     

先天性脐尿管脓肿的诊治体会——附1例报告

[背景 ]提高对先天性脐尿管脓肿的认识和诊治水平 .[病例报告 ]根据典型的病史、B型超声波和CT、穿刺抽液及囊内注入造影剂检查等辅助检查方法诊断先天性脐尿管脓肿 1例 ,并进行相应的治疗 .术中诊断及病理检查证实为先天性脐尿管脓肿 .患者术后 12d痊愈出院 ,随访 3个月 ,无复发和癌变 .[讨论 ]穿刺抽液及囊内注入造影剂检查、B型超声波、CT等辅助检查是有效的诊断方法 .手术应尽可能切除包括脐部到膀胱顶部的整个脐尿管 ,以免复发和癌变     

一例病程14年慢性脐尿管瘘患者的护理

脐尿管瘘为一种罕见的先天性膀胱发育畸形中脐尿管畸形之一[1].今年我院收治1例病程14年慢性脐尿管未闭患者,经手术治疗痊愈出院.本文主要报道了在该患者的治疗和护理工作中的体会与经验.     

小儿先天性脐尿管疾病的治疗及护理

目的:总结小儿先天性脐尿管疾病的临床特点,探讨其外科治疗及护理技术.方法:回顾性分析22例小儿先天性脐尿管疾病住院患儿的临床资料.结果:22例患儿均行手术治疗,通过围术期治疗及护理,恢复良好,均痊愈出院.结论:小儿先天性脐尿管疾病无论何种类型,均适合外科手术治疗.围术期治疗及护理是保证患儿预后的重要因素.     

Ventricular tachycardia an atypical presentation of Ebstein’s disease

Ebstein’s anomaly (EA) is a rare congenital malformation, characterized by an apical displacement and dysplasia of the septal leaflet of the tricuspid valve with a right ventricular atrialization compromising its function. This malformation includes electrical conduction abnormalities, and very rarely ventricular arrhythmias. We report the case of a 22-year-old male, presenting with dizziness and palpitations. The electrocardiogram showed a sustained monomorphic ventricular tachycardia (SMVT). The presentation of the disease with ventricular tachycardia associated with hemodynamic instability is extremely rare. We placed a surgical implantable cardiac defibrillator when the diagnosis of EA, SMVT, and right ventricular systolic impairment was confirmed.Ebstein’s anomaly (EA) is a rare congenital malformation, characterized by an apical displacement and dysplasia of the septal leaflet of the tricuspid valve with a right ventricular atrialization compromising its function.1 This malformation includes electrical conduction abnormalities and supraventricular arrhythmias. The cases of EA and ventricular tachycardia reported in the literature are few. The objective in presenting this particular case is to review the EA and the management of ventricular tachyarrhythmias.     

先天性肠旋转不良合并畸形回顾性分析

目的:总结先天性肠旋转不良常见合并畸形,降低二次手术风险及死亡率。方法:回顾性分析近二十年间本院收治的136例先天性肠旋转不良患儿临床资料,调查其常见合并畸形,分析近十年收治的患儿与前十年相比二次手术率及死亡率的变化。结果:136例患儿有33例合并有一种或多种畸形,以合并十二指肠膜状狭窄最多见;和前十年相比二次手术率和死亡率下降,二次手术原因发生改变。结论:肠旋转不良常合并其他消化道畸形,类型复杂;中肠扭转、小肠坏死和合并其他畸形是主要死因;我们可通过早期诊断、及时手术、仔细探查并处理合并的消化道畸形来降低本病的二次手术率和病死率。     

先天性静脉畸形骨肥大综合征ICD-1O编码

先天性静脉畸形骨肥大综合征为一种复杂且罕见的先天性肢体发育畸形性疾病,该疾病以其血管畸形、骨肥大和软组织增生三联征为主要表现.本文通过其发病机理和临床表现,分析病例内容,探讨该疾病的ICD-10编码.按照国际疾病的分类和查找方法,甄选和变通主导词进行查询.分别以综合征、异常、畸形为主导词进行编码的查找,未获得准确编码;以音译词克利佩尔-特脑纳-韦伯综合征为主导词,得到其编码Q87.2,核对卷一,确认编码.通过对疾病编码的讨论分析,总结了罕见疾病的编码思路.编码人员除了坚持学习和巩固编码原则外,还应深入研究疾病诊断的内涵,通过查阅文献寻找切实依据,并与临床医师互相交流,才能编码科学、准确.     

“1”纵形切口肛门成形术治疗中高位先天性直肠肛门畸形的临床体会

目的　探讨"1"纵形切口肛门成形术治疗中高位先天性直肠肛门畸形的方法及其手术效果。方法 采用"1"纵形切口肛门成形术治疗中高位先天性直肠肛门畸形11例。总结其临床资料。结果　11例患者肛门功能恢复好,远期疗效佳,无大便失禁或肛门狭窄。结论 "1"纵形切口肛门成形术治疗中高位先天性直肠肛门畸形是一种全新的手术方式,具有成形肛门外观美观,为椭圆形,可减少括约肌损伤,降低术后并发症等优点,有着很好的应用前景,适合于各型直肠肛门畸形。     

MSCT对小儿复杂先天性心脏病的诊断价值

目的探讨多层螺旋CT(Multislice Spiral Computed Tomography,MSCT)对小儿复杂先天性心脏病的诊断价值。方法选取2014年到2015年在我院行手术确诊的小儿复杂先天性心脏病患者27例,所有患者术前1个月均经多层螺旋CT和心脏超声(Ultrasound,US)检查,比较两种检查方法对患者心脏畸形确诊率的差异。结果 27例患者共有心脏部分畸形41处,MSCT的确诊率为(39处,95.12%)与US确诊率(40处,97.56%)的差异无统计学意义(χ2=0.346,P=0.556);患者共有大血管部分畸形35处,MSCT确诊率(34处,97.14%)高于US确诊率(27处,77.14%),且差异具有统计学意义(χ2=4.590,P=0.032);患者共有心脏和大血管连接部分畸形35处,MSCT确诊率(34处,97.14%)高于US确诊率(25处,71.42%),且差异具有统计学意义(χ2=8.737,P=0.003);所有患者共有心脏畸形111处,MSCT确诊率(107处,96.40%)高于US确诊率(92处,82.88%),且差异具有统计学意义(χ2=10.913,P=0.001)。结论多层螺旋CT对小儿复杂先天性心脏病心脏畸形的诊断准确率高于心脏超声检查。     

山西某地区近年先天畸形发生情况及相关因素分析

目的：：了解长治地区近年来先天性畸形的发生情况,探讨畸形发生的相关因素。方法：对8967例新生儿中151例先天畸形进行分析。结果：本次统计先天畸形发生率为168.40/万;畸形发生较高的依次为：神经管畸形、多并指(趾),唇、腭裂。未补充叶酸畸形发生率高于叶酸补充者(P <0.01),≥35岁产妇先天畸形儿发生率高于<35岁者(P <0.01);农村先天畸形发生率高于城市(P <0.01)。结论：先天畸形的发生除与普遍认知的叶酸缺乏相关外,还和父亲生活习惯、医疗条件、卵子及宫内环境、婚源等因素有关。因此,应重视评估父亲对后代的影响,防范有可能诱发畸形的其它因素,以避免先天畸形的发生。     

先天性心脏病术后肠梗阻并发急性胰腺炎1例

术后肠梗阻并发急性胰腺炎在临床上非常少见,肠梗阻和胰腺炎均为临床常见急腹症,两者互为关联,一般急性胰腺炎导致肠梗阻临床较为多见,而急性重症肠梗阻导致急性胰腺炎发作临床报道甚少,笔者联合阜外心脏中心专家就本例心脏术后肠梗阻致急性胰腺炎做相关报道。     

Trans-omental hernia as a rare cause of small bowel obstruction

Internal hernia is a very rare case of small bowel obstruction (incidence: 0.2-09%) with a significant mortality rate of about 45%. It has few different forms including congenital or acquired and persistent or intermittent. According to its anatomic location, internal hernia has multiple different types. Trans-omental type is the rarest one that only accounts for 1-4% of internal hernia cases. It can be discovered incidentally but can also cause small bowel strangulation and obstruction with very high mortality rate. Based on presentation, immediate diagnosis and urgent surgical intervention are crucial to save the patient's life. Only very seldom cases o f trans-omentalhernia were reported in the literature that always had acute presentation. We report a very rare case of trans-omental hernia that started with slow presentation over decades with vague abdominal symptoms but then developed acute small bowel obstruction that necessitated immediate surgery.     

逆行胰胆管造影诊断胰胆系罕见、少见疾病的价值

目的：明确国人胰胆系罕见、少见疾病与其他胰胆疾病的关系,提高胰胆系疾病的检出率。方法：分析1984年5月－2001年1月间成功行逆行胰胆管造影1061例,其中先天性胆管囊性扩张症、胰胆管合流异常、乳头旁憩室等胰胆系罕少疾病160例。结果：胰胆系罕少疾病占15％（160／1061）, 单病种的检出占0．09％－6．22％。胰胆系罕少疾病中约60％合并其他胰胆疾病。结论：胰胆系罕少疾病同时合并其他胰胆疾病的发病率高,应引起临床重视。     

Anatomical development of the normal urachus during the fetal period

OBJECTIVE: To evaluate the development of the normal urachus during the fetal period. METHODS: The study was carried out on 149 human fetuses (male 87, female 62) aged between 9-40 weeks, obtained with families' consent from Isparta Maternity and Children's Hospital, Isparta, Turkey, between 1997 and 2002. The study was carried out in the Department of Anatomy, Faculty of Medicine, Suleyman Demirel University, Isparta, Turkey during 2004. The type of the urachus was assessed and was classified. The length, antero-posterior depth, and the transverse width of the urachus were measured. Finally, the relationship between the urachus and umbilical arteries was evaluated during the fetal period, whether they ran parallel to each other. RESULTS: Two types of urachus were observed: the inverted funnel (84%) and tubular (16%). The length, depth, and width of the urachus increased with gestational ages in all cases. A 2% variation was observed in the neighborhood between urachus and umbilical artery. CONCLUSION: Comparing our results with previous studies, the development of the urachus can be different in neonates and children. The mean urachus length was 14.8 -/+ 7.7mm during this period. The inverted funnel type is the most common type in our study. Hence, there can be some differences in the relationship between the urachus and umbilical arteries. The data obtained in the present study can be used as base knowledge related to the development of the urachus, and for evaluating the urachus in utero pediatric urology, radiology, pediatric surgery, and fetopathology.