56例小儿重复肾临床诊疗分析

目的探讨小儿重复肾的诊断与治疗方法,以进一步规范其诊疗流程。方法对遵义医学院附属医院近10年收治的小儿重复肾畸形56例的临床资料进行回顾性分析。对其临床表现、分型、诊断及治疗方法进行总结分析。结果 56例中重复肾重复输尿管无积水且IVU显影正常者11例,保守随访观察。重复肾发育不良、重复肾积水伴重复输尿管积水并输尿管异位开口、IVU不显影和SPECT检测GFR(肾小球滤过率)无功能35例,予以重复肾重复输尿管探查切除。重复肾功能尚存、重复输尿管扩张(直径大于4. 0 cm)、积水3例,予以重复肾与下位肾肾盂吻合成形术治疗;重复肾功能尚存、重复输尿管扩张(直径小于4. 0 cm)、积水伴输尿管末端囊肿5例(不伴膀胱输尿管反流),予以输尿管末端囊肿戳穿术治疗;不伴输尿管末端囊肿2例,予以重复输尿管膀胱再植术治疗。术后随访6个月至7年,除1例因合并骶脊膜膨出术致神经源性膀胱表现为尿失禁外,其余术前存在泌尿系感染的病例感染症状均消失。术前不存在泌尿系感染的病例术后亦未出现泌尿系新发感染,行输尿管囊肿戳破术及膀胱输尿管再植的病例术后未出现膀胱输尿管反流。结论小儿重复肾畸形临床表现多样,B超、IVU、SPECT、VCUG及泌尿系MRU检查是诊断的主要方法,膀胱镜输尿管插管逆行造影有一定的补充性诊断价值。治疗则应依据重复肾功能、重复肾重复输尿管有无积水以及是否合并重复输尿管异位开口,采用个体化治疗方案。     

输尿管侧侧捷径吻合在重复肾输尿管返流中的应用

患儿，女，2岁，因胎儿期B超检查示双肾积水伴生后反复泌尿系感染住院。体格检查：神志清楚，心肺腹未见异常，正常女性外阴。影像学检查：B超示双侧肾积水、双侧输尿管积水，双侧重复肾伴左输尿管囊肿。CT及MRI也支持B超诊断。排泄性膀胱尿道造影示双侧膀胱输尿管返流，右侧显著，右肾区可见“垂朵征”。实验室检查未见异常，肾功能正常。诊断：双侧重复肾输尿管畸形，双下肾输尿管返流Ⅲ级，左输尿管囊肿。     

马蹄肾伴双侧不完全重复肾盂输尿管畸形一例并文献回顾

目的回顾性分析1例马蹄肾合并双侧不完全重复肾盂输尿管畸形患儿的临床资料,并通过文献复习探讨该病的诊治方法,提高临床上对此类疾病的认识。方法重庆医科大学儿童附属医院泌尿外科收治1例马蹄肾合并双侧不完全重复肾盂输尿管畸形患儿,女,5岁5个月,因发现腹部包块入院,门诊彩色多普勒超声及CT检查均提示马蹄肾,右肾囊肿性疾病可能,入院后行逆行输尿管膀胱插管造影提示右肾上极可见囊状造影剂显影,并与右肾盂相连,左侧上下肾盏显影。我们采取开放手术,详细解剖,手术成功。结果患儿术后恢复好,无并发症,术后1个月复查彩色多普勒超声提示右肾囊性结构较前缩小,现仍处于密切随访中。通过对Pubmed , Embase, Scopus, CNKI、万方、维普中文文献库进行了系统的检索,至今仅有1例英文案例详细报道。结论马蹄肾伴双侧肾盂输尿管重复畸形十分罕见。马蹄肾伴双侧肾盂输尿管重复畸形的患儿若无症状一般不需要治疗,定期复查彩色多普勒超声、肾功能。影像学检查对其有辅助诊断功能。手术疗效可靠。     

重复肾输尿管畸形的诊断与治疗

目的 探讨重复肾输尿管畸形的诊断与治疗。方法 16例重复肾输尿管畸形患儿，单侧13例、双侧3例，其中13例伴一侧输尿管异位开口；通过静脉肾盂造影(IVU)、B超、膀胱镜检查确诊；15例行手术治疗，1例保守治疗。结果 经治疗均痊愈出院，术后随访1～8年疗效满意。结论IVU、B超诊断有重要意义，治疗应根据重复肾、输尿管病情及其并发症而定。     

单纯上或下患肾部输尿管膀胱外再植治疗完全性重复肾畸形

目的 评价采用单纯上或下患肾部单根输尿管膀胱外再植治疗完全性重复肾畸形的疗效.方法 从2009年12月1日起至2014年12月31日间,我科采用单纯上或下患肾部单根输尿管膀胱外再植治疗完全性重复肾畸形患儿27例,均为单侧病变,女19例,男8例,年龄2个月至8岁.所有患儿经影像学检查明确诊断为完全性重复肾畸形且均为单根输尿管病变.其中上输尿管异位开口者9例;重复上肾上输尿扩张积水伴上输尿管末端囊肿者10例;重复下肾部输尿管单纯反流者8例.临床症状主要表现为正常排尿间歇性滴尿或发热性尿路感染.所有患儿术后随访观察11个月至6年,随访项目包括泌尿系统超声、同位素和膀胱造影等.结果 所有患儿手术过程顺利,平均手术时间63 min,术后平均住院时间为5d,术后所有患儿临床症状均消失.术后有1例患儿因血尿检查发现再植输尿管开口处结石形成,予以行膀胱镜下钬激光碎石术.术后6个月复查超声示患肾部积水均有好转,同位素示患肾部功能改善或稳定,正常肾部功能未见异常改变.7例患儿术后复查排泄性膀胱尿道造影检查未见膀胱输尿管反流等发生.结论 经腹股沟皮纹小切口单纯上或下患肾部单根输尿管膀胱外再植术治疗完全性重复肾畸形是一种安全、有效的方法,值得临床推广.     

核素利尿肾动态显像在儿童肾重复畸形中的临床应用

为评价99Tc^m-EC利尿肾动态显像在儿童肾重复畸形中的临床价值，对23例肾生复畸形住院患儿99Tc^m-EC利尿肾动态显像（RDI），分析其影像特征和主要功能指标的变化；设计可反映重复上半肾功能的指标（D/N），并以之与临床病理分型比较。结果显示：肾重复畸形在RDI连续叠加影像中有4种表现；上肾稀疏、上肾缺损、肾影增长、肾盂分离；RDI的灵敏度、特异性、准确性分别为73.3%、93.2%、81.4%；RDI对合并输尿管囊肿检出率较好，并可检出部分输尿管扩张积水；利尿肾图显示尿路梗阻类型均为非机械性梗阻。临床病理分型为发育不良型，其上肾D/N值均小于30%，发育型均大于70%，积水型为30%-70%。对术后随访的病例，RDI在双肾形态和功能转归方面有肯定的判断。说明99Tc^m-EC肾动态显像对肾重复畸形有较好的诊断价值可帮助判断重复上半肾功能，为临床床前选择手术方式提供重要依据可作为术后随访复查的主要检查手段。     

小儿重复肾畸形的早期诊断和治疗

目的 探讨小儿重复肾畸形的早期诊断和治疗方法.方法 回顾性分析本院1995年6月-2007年9月60例经手术治疗的小儿重复肾畸形病例的临床资料.60例均行B超检查,确诊55例,误诊为肾上极囊肿5例.60例患儿均行静脉肾盂造影(IVP)检查,均提示重复肾,其中2例上下肾部均有不同程度的肾盂积水和输尿管扩张,进一步行排尿性膀胱尿道造影,诊断下肾部膀胱输尿管返流、下肾部输尿管膀胱交接部梗阻各1例.结果 重复肾畸形患儿60例均行手术治疗,行上肾部切除及其输尿管切除58例,重复肾并上下肾部积水手术2例,其中1例下肾部输尿管末端梗阻致下肾部积水,上肾部输尿管囊肿,行上肾部及其输尿管切除,术后输尿管囊肿萎陷,下肾部输尿管末端梗阻解除,症状消失,术后6个月复查IVP,下肾部肾盏杯口清晰,无肾积水,肾功能良好,疗效满意;另1例为下肾部输尿管返流致下肾部积水,上肾部输尿管囊肿,行输尿管囊肿切除,双输尿管膀胱再植术,术后6个月复查排尿膀胱尿道造影,膀胱输尿管返流消失,排尿正常,无泌尿系感染.结论 术前诊断不明或误诊的原因多系患儿临床症状与其他疾病症状相类似而误诊为其他疾病,应强调对可疑征象的辅助检查.如重复肾畸形诊断明确,应早期手术治疗.     

先天性输尿管开口异位26例

目的探讨小儿先天性输尿管开口异位的诊断和治疗方法。方法回顾性分析本院1995年2月至2008年2月收治的26例先天性输尿管开口异位病例资料。其中18例为单侧重复肾、重复输尿管并输尿管开口异位，1例为双侧重复肾、重复输尿管并单侧输尿管开口异位，5例为先天性肾发育不良并输尿管开口异位，2例为单侧肾异位伴发育不全输尿管开口异位。结果均经手术治疗，其中16例行上肾部输尿管切除术；1例行患肾上肾部及输尿管切除术；2例行输尿管膀胱再植术；5例肾发育不良和2例异位肾并肾发育不全输尿管开口异位患儿行患肾及输尿管切除术。26例均痊愈出院，获6个月至1年的随访，滴尿症状均消失，2例术后半年行膀胱尿道造影未见膀胱输尿管返流，疗效满意。结论先天性输尿管开口异位易误诊或漏诊，B超、静脉肾盂造影、磁共振泌尿系统水造影是诊断的主要手段；诊断明确者应尽早手术治疗。     

重肾双输尿管合并肾盂输尿管交界部梗阻性肾积水的诊治分析

目的 探讨重肾双输尿管合并肾盂输尿管交界部梗阻性肾积水的诊断与处理方法。方法 回顾分析1986～2004年间收治重肾合并肾盂输尿管交界部梗阻肾积水8例的临床资料。男2例，女6例，年龄7个月～10岁，平均4．8岁。病变位于左侧3例，右侧5例；上肾积水2例，下肾积水5例，上下肾积水1例；3例为重肾完全型双输尿管，5例重肾Y型输尿管。结果 3例重肾完全型双输尿管中，上肾积水1例因肾实质薄无功能行上肾切除术，下肾积水伴上肾输尿管膨出症1例行上肾切除下肾离断性肾盂成形术，另1例下肾积水因临床症状轻微，IVP示积水半肾的肾盏变钝不明显，未行手术门诊随诊。5例重肾Y型输尿管中，上肾积水1例行上肾盂与下输尿管吻合，下肾积水3例行上肾输尿管下肾盂吻合 下肾离断性肾盂成形术，1例上下肾均积水行上下肾盂吻合 下肾盂成形术。术后3～6个月复查IVP肾积水明显好转。结论 重肾肾盂输尿管交界部梗阻肾积水发病率很低，术前不容易明确诊断或被误诊。腹部B超、IVP或MRU是有效的辅助检查手段。治疗应根据息肾功能、形态而定，如息肾功能严重受损，行患肾切除，反之，根据积水的部位、输尿管的形态选择手术方式。     

不完全性重复肾双输尿管畸形的诊治及文献复习

目的探讨不完全性重复肾双输尿管畸形的诊断与处理方法。方法回顾性分析2004年10月至2008年8月夺院收治的3例Y形输尿管病例的临床资料。男2例,女1例。病变位于左侧2例,右侧1例。结果3例不完全性重复肾双输尿管畸形中,1例为左不完全性重复肾双输尿管畸形,下肾部肾盂和输尿管连接部狭窄,Y形输尿管汇合于近下肾部肾盂和输尿管连接处。行下肾部肾盂与上输尿管端侧吻合术。1例为有不完全性输尿管重复畸形,有膀胱输尿管返流Ⅲ～Ⅳ级,Y形输尿管汇合于输尿管中下段,行有输尿管膀胱再植术。1例为左不完全性重复肾双输尿管畸形,下肾部肾盂和输尿管连接部狭窄,Y形输尿管汇合于输尿管中部,行下肾部离断式肾盂成形术。术后随访6,15个月,症状均明显好转。结论不完全性重复肾双输尿管畸形发病率低,术前不容易明确诊断或被误诊。腹部B超、IVP、DR或CTU是有效的辅助检查手段。治疗应根据患肾功能、形态等多种因素选择手术方式。     

Multicystic dysplasia in the upper component of the complete duplex kidney

Four cases of neonatal multicystic dysplasia involving the upper part of a complete duplex kidney are presented. This malformation, suspected on antenatal ultrasound examination, was confirmed by postnatal IVP and ultrasound. This emphasizes the need to perform an IVP when the diagnosis of multicystic kidney is suggested by ultrasound. Conservative surgery may then be achieved, as in three of the four cases.     

44例小儿重复肾的诊治分析

目的探讨小儿重复肾的诊断方法和手术技巧,以提高其诊治水平。方法回顾性分析2006年9月至2011年10月经作者手术治疗的44例重复。肾患儿临床资料,其中男性10例,女性34例,年龄1个月至13岁,平均年龄2．2岁。均采用B超、MRU、IVU等相结合的方法进行诊断。均行重复肾及所属输尿管切除术。结果44例患儿术中诊断与术前相符,均经手术治疗痊愈出院。42例获随访,随访时间7个月至5年,3例出现输尿管残端综合征,经手术切除输尿管残端后治愈。1例术后出现残肾断面漏尿,经肾周引流管流出,术后6d漏尿停止,B超检查无肾周积液,拔除引流管后康复出院。其余患儿恢复良好。结论B超、IVU、MRU等相结合是诊断小儿重复肾畸形的主要方法,可确诊重复肾;CTU、VCU对诊断有补充作用。手术切除重复肾是治疗重度积水、扩张重复。肾的主要方法,尽量低位切除重复输尿管、完全切除重复肾、保护残留。肾肾盏及所属正常输尿管是手术成功的关键。     

Spectrum of urorectal septum malformation sequence

Urorectal septum malformation sequence (URSMS) is a rare spectrum of malformations involving various organ systems. Here, we present eight cases of URSMS, noted in autopsy, with different degrees of complexity, seven being the complete type and one being the partial type. All cases had gastrointestinal tract malformation in the form of the imperforate anus and indeterminate genitalia. Other gastrointestinal tract anomalies were anal agenesis in two cases, anorectal agenesis in two cases, and malformed lower intestinal tract in four cases. The associated renal abnormality was noted in five cases, which were unilateral renal agenesis, dysplastic kidney, hydronephrosis, horseshoe kidney, and unilateral hypoplastic ectopic kidney. External genital malformation, present in both male and female fetuses, included a knob‐like structure at perineum in female fetuses, genital fold hypoplasia and penile aplasia or hypoplasia in male fetuses. Skeletal abnormalities included two cases of sacral agenesis and one case of lumbosacral dysraphism. Other anomalies included a case with alobar holoprosencephaly, truncus arteriosus with hypoplastic lungs in one case, and three cases with abdominal wall defects. It is our attempt to delineate a spectrum of abnormalities associated with URSMS.     

Uncomplicated duplex kidney and DMSA scintigraphy in children with urinary tract infection

Background Renal duplication is the most common malformation of the urinary tract and is frequently seen among children with urinary tract infection (UTI). Objective To evaluate problems in the interpretation of dimercaptosuccinic acid (DMSA) scintigraphy and to establish the range of relative function in uncomplicated unilateral duplication. Materials and methods Retrospective analysis of 303 children less than 2 years of age with first time non-obstructive urinary tract infection investigated by both urography and DMSA scintigraphy. At DMSA scintigraphy, renal lesions and/or relative function below 45% was considered abnormal. Urography was used as reference for the diagnosis of duplication. Results Duplex kidneys were found in 22 of 303 patients (7%). Of the 16 children with unilateral duplication, 10 had bilaterally undamaged kidneys with a range of relative function varying between 51% and 57% in the duplex kidney. In two of the children with unilateral duplication the imaging results were discordant. Conclusion There was risk of underdiagnosis as well as overdiagnosis of renal damage at scintigraphy. Although it is important to be aware of this risk, the rate of misinterpretation was low. A range of 51% to 57% can be used as the limit for normality of the relative function of a unilateral duplex kidney.     

Familial renal abnormalities associated with the oligohydramnios tetrad secondary to renal agenesis and dysgenesis

Abstract Thirty-four families of index cases with the oligohydramnios tetrad secondary to renal agenesis/dysgenesis were screened for renal abnormalities using Real Time ultrasonography. The index cases were separated into two groups. Group 1 consisted of cases of perinatally lethal renal disease and Group 2 of cases of renal dysgenesis secondary to the urethal obstruction malformation. Renal ultrasound screenings of 23 families in Group 1 demonstrated two previously unidentified cases of unilateral renal agenesis in siblings. Screening of 11 families in Group 2 revealed one sibling with a hydronephrotic kidney and one parent with an ectopic pelvic kidney.
There is a recurrence risk of 3.5–5% in families with perinatal lethal renal disease and an increased risk of silent renal anomalies in first degree family members. The recurrence risk is low in families of infants with renal dysgenesis secondary to the urethral obstruction malformation, but immediate family members are at increased risk of structural and functional urinary anomalies. Routine renal ultrasound screening of first degree relatives of infants with lethal renal agenesis and dysgenesis is recommended.     

Familial renal abnormalities associated with the oligohydramnios tetrad secondary to renal agenesis and dysgenesis

Thirty-four families of index cases with the oligohydramnios tetrad secondary to renal agenesis/dysgenesis were screened for renal abnormalities using Real Time ultrasonography. The index cases were separated into two groups. Group 1 consisted of cases of perinatally lethal renal disease and Group 2 of cases of renal dysgenesis secondary to the urethral obstruction malformation. Renal ultrasound screenings of 23 families in Group 1 demonstrated two previously unidentified cases of unilateral renal agenesis in siblings. Screening of 11 families in Group 2 revealed one sibling with a hydronephrotic kidney and one parent with an ectopic pelvic kidney. There is a recurrence risk of 3.5-5% in families with perinatal lethal renal disease and an increased risk of silent renal anomalies in first degree family members. The recurrence risk is low in families of infants with renal dysgenesis secondary to the urethral obstruction malformation, but immediate family members are at increased risk of structural and functional urinary anomalies. Routine renal ultrasound screening of first degree relatives of infants with lethal renal agenesis and dysgenesis is recommended.     

Study of the Malformation of Ductal Plate of the Liver in Meckel Syndrome and Review of Other Syndromes Presenting with This Anomaly

Meckel syndrome (MIM 249.000) is an autosomal recessive disorder with a variable spectrum of anomalies. Since the first reports of this syndrome, very broad diagnostic criteria have been proposed, but the process of defining them continues. It is probable that at least two of three manifestations, including cystic kidney dysplasia, occipital encephalocele or other anomaly of the central nervous system, and postaxial polydactyly occur in most cases. Arrest of the development of intrahepatic bile ducts at the stage of the bilaminar plate formation or ductal plate malformation is considered of high diagnostic value in Meckel syndrome, but there is no complete agreement in the literature about its occurrence. The aims of this investigation were to study the prevalence and morphologic patterns of ductal plate malformation of the liver in Meckel syndrome by evaluating the dilatation of primitive biliary structures and the increase in connective tissue of the portal tract. Archival data files from four German centers (Berlin, Freiburg, Heidelberg, Mainz) were reviewed. Liver sections of 30 well-studied fetuses with Meckel syndrome were immunostained with antibodies against cytokeratins (intermediate filaments of the cytoskeleton) and factor VIII (an endothelial cell marker) and were evaluated both qualitatively and quantitatively. Cystic kidney dysplasia, occipital encephalocele, and postaxial polydactyly were found in 100%, 90%, and 83.3% of the fetuses, respectively. Ductal plate malformation of the liver was a constant anomaly in Meckel syndrome, seen as frequently as renal lesions. We observed essentially two kinds of hepatic lesions: 23 cases showed mainly a cystic dilatation of primitive biliary structures with little portal fibrosis, while 7 cases showed mainly rings of interrupted curved lumina around a central fibrovascular axis and pronounced portal fibrosis. In these seven cases an abnormal pattern of the portal vein, with many small and closely spaced branches of the portal vein (the so-called pollard willow pattern), was also seen. With respect to other fetal developmental anomalies, no difference between the two types of lesions was found. We also provide a potentially useful comprehensive review of other genetic syndromes in which ductal plate malformations may occur. Received May 14, 1999; accepted October 11, 1999.     

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??Abstract??Objective??To evaluate the diagnostic value of susceptibility weighted imaging ??SWI?? in cerebral vascular malformation of children. Methods??The imaging data of MRI were retrospectively analyzed in all twenty-seven cases?? which included 16 cases of venous angioma?? 8 cases of cavernous angioma and 3 cases of arterio-venous malformation. The comparison of SWI and conventional MR sequences was obtained. Results??The dilated draining veins and medulla veins were displayed well on SWI sequence. More brain cavernous angioma lesions could be detected on SWI than those on conventional MR sequences??P < 0.05??. Conclusion??SWI can detect lesions of venous angioma?? cavernous angioma and arterio-venous malformation more sensitively than other sequences?? which is a reliable diagnostic technique for cerebral vascular malformation of children. SWI has higher clinical value?? and should be the routine sequence in the MRI examination.     

Rare case of congenital cystic adenomatoid malformation associated with polycystic kidney disease

Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary abnormality that results from aberrant fetal lung development. It about 4-26% of cases it can be associated with other congenital abnormalities. We describe a case of congenital cystic adenomatoid malformation 2 associated with polycystic kidney disease. The association of these two congenital malformations is exceptional. Only four similar cases have been reported in the literature. A 2-year-old girl was referred to the Department of Paediatric Surgery and Oncology Medical University of Lodz with pneumonia and left pneumothorax. For three weeks prior to referral the patient was treated with antibiotics. Chest x-ray revealed hyperinflation of left upper lobe with mediastinal shift to right. Computer tomographic scan of the lung revealed multiple cyst in the left upper lobe, left-site pneumothorax and mediastinal shift to the right. The patient underwent thoracotomy. Intraoperatively, multiple cysts in the left upper lobe were found and left upper lobectomy was performed. Histologic study was compatible with type 2 congenital cystic adenomatoid malformation. Ultrasound examination showed multilocular cysts in both kidneys. The dimensions of the cysts were: MWR4. 54x45x45 mm and 25x21x24 mm on the left and right sides, respectively. Significant increase in cyst size on the left side was observed. Ten months after first hospitalization resection of the cystic lower pole of the left kidney was performed. The presence of even a single renal cyst in a child with CCAM is an indication for further follow up examinations.