Nephrosonography in the evaluation of renal failure and masses in infants.

The diagnostic accuracy of nephrosonography is compared with conventional radiologic techniques and surgical findings in 13 infants aged 1 day to 14 months, who were in renal failure or had abdominal masses. Ten of the 13 infants presented with marked renal failure and in half of them neither kidneys nor collecting systems were visualized on the first intravenous pyelogram. Normal renal architecture was demonstrated by nephrosonography in three, hypoplastic kidneys in three, hydronephrosis in three, and a combination of hydronephrosis and contralateral multicystic kidney in one. In the three infants without renal failure, only one kidney was demonstrated by IVP; The cystic contralateral kidney in each of them was demonstrated by nephrosonography. In all cases the diagnosis was confirmed by conventional radiologic techniques when renal function had improved or by surgical exploration. This technique appears to be a useful adjunct to conventional radiography in the differential diagnosis of the infant with abnormalities of the urinary tract. Nephrosonography is of especial value when a kidney is not visualized by IVP, and it may appropriately aid in therapeutic decisions regarding the use of invasive procedures in small critically ill infants.     

Observations on the use of Doppler ultrasound in multicystic dysplastic kidney

Pulsed Doppler ultrasound was used to assess the renal artery in patients with multicystic kidney. In 7 out of 7 patients there was marked abnormality of the wave-form. Doppler ultrasound may be a useful complementary diagnostic method in patients suspected of having a multicystic dysplastic kidney.     

Multicystic kidney dysplasia: a prospective study on the natural history of the affected and the contralateral kidney

In a 6-year period, 41 young infants with multicystic kidney dysplasia were seen in our department. In 30 cases, the diagnosis had already been suspected by prenatal ultrasonography. A prospective protocol was proposed to the parents which comprised ultrasound evaluation every 3 months until the age of 24 months and renal function assessment at the age of 18 months. In 33 patients, the study was completed as scheduled. At the start of the study, the maximal diameter of the multicystic kidney was above the mean length of normal kidneys in all cases where precise measurement was possible. Within 24 months, 7 of the dysplastic kidneys disappeared, 20 regressed in size, 1 remained unchanged and only 5 increased in size. Between the age of 0 to 3 months, renal length of the contralateral kidney was within the normal range in 19 infants and above +2SD in 14 cases. At the age of 18 to 24 months, renal length was, with few exceptions, between 0 and +4SD. Inulin clearance was normal in all 33 individuals with a median value of 112 ml/min per 1.73 m². Conclusion As a rule, multicystic kidneys shrink in the first 2 years of life. In most cases the contralateral kidney maintains a normal renal function as a consequence of progressive compensatory hypertrophy. Received: 19 November 1997 / Accepted in revised form: 31 January 1998     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

Single-system ectopic ureters associated with renal dysplasia

The purpose of this study was to inquire into the clinical features and methods for the diagnosis and therapy of single-system ectopic ureters associated with renal dysplasia. Intravenous urography (IVP), diuretic B-mode ultrasound (B-US), and, in four cases, computerized tomography (CT) were performed in twelve female patients with renal dysplasia-seven on the left and five on the right-and the clinical records were retrospectively summarized and analyzed. The main presentation was droplet urinary incontinence. IVP and B-US showed that in all in whom the affected kidneys did not function, the contralateral kidneys had undergone compensatory hypertrophy. Only in four cases were the dysplastic renal tissues displayed in the pelvic cavity on B-US. In eight cases, the affected kidneys were not found. Of the four patients who had CT scanning, the dysplastic kidneys were explored in the pelvis in two cases. All patients underwent ureteronephrectomy, and the urinary incontinence disappeared after operation. Single-system ectopic ureters associated with renal dysplasia mainly affect female children, and most present with droplet urinary incontinence; the affected kidney may not be displayed on ultrasonography and IVP, but the contralateral kidney may display compensatory hypertrophy on IVP and can easily be misdiagnosed as a solitary kidney. The curative effect of ureteronephrectomy is definite.     

彩色多普勒超声对于儿童先天性巨输尿管症的诊断价值

目的 探讨彩色多普勒超声对于儿童先天性巨输尿管症的诊断价值.方法 对2005年1月至2010年10月间经我院手术证实的80例先天性巨输尿管症患儿的超声影像结果进行统计学分析.所有患儿在术前均行彩色多普勒超声检查.结果 经手术证实80例患儿101侧输尿管被确诊为巨输尿管（其中21例患儿为双侧巨输尿管）,左侧43例;右侧16例;双侧为21例,有巨输尿管的同侧肾脏多伴有不同程度积水.101侧巨输尿管中95侧由超声首先确诊;80例患儿术前同时行超声及IVP的患者为45例,共有54侧巨输尿管（其中9例为双侧巨输尿管）,超声检出率93.6%（50例/54侧）,IVP检出率81.5%（44例/54侧）,两者对于巨输尿管的检出率差异有统计学意义（P〈0.05）.结论 对于儿童先天性巨输尿管症的诊断,超声检出率更高,便捷无创,有很高的诊断价值.     

Alagille's syndrome associated with cystic renal disease

Although renal abnormalities have been described in children with Alagille's syndrome, cystic kidney disease has not often been documented, and then usually only at necropsy. Three children with Alagille's syndrome are described, in two of whom a unilateral multicystic dysplastic kidney was detected by prenatal ultrasound; in the other, a solitary cortical cyst was found later in childhood. All have normal renal function, growth, and liver synthetic function but continue to have clinical and biochemical signs of cholestasis. These cases show that unilateral cystic kidney disease with or without renal dysplasia may be associated with Alagille's syndrome, that the clinical course is not necessarily unfavourable, and that Alagille's syndrome should be included in the differential diagnosis of cystic kidney disorders associated with cholestatic liver disease. Patients with Alagille's syndrome should be evaluated by renal ultrasound.     

微创小切口离断式肾盂输尿管成形术231例

目的回顾性分析微创小切口离断式肾盂输尿管成形术治疗先天性肾盂输尿管连接部狭窄的可行性及手术效果。方法2009年12月至2013年6月,我们共收治先天性’肾盂输尿管连接部狭窄268例,其中231例采用微创小切口离断式。肾盂输尿管成形术。231例中,男152例,女79例;左侧156例,右侧58例,双侧17例。年龄6d至5岁11个月。诊断依靠超声、MR及ECT检查,部分病例选用静脉肾盂造影或cT检查。Grignon分级：Ⅲ级12例,Ⅳ级85例,V级134例。患儿均行Anderson—Hynes肾盂成形、肾盂输尿管吻合术。术后前半年每6～8周行超声和尿液检查,后半年每2～3个月复查1次,术后1年行静脉肾盂造影（we）和（或）核素肾图（ECT）检查。结果231例中,188例选用1．5～2cm切口顺利完成手术,27例延长切口至2—2．5cm,6例切口2．5～3cm,10例切口3—4cm。231例中,超声检查肾积水明显减轻者211例,积水无加重者19例,1例吻合口不通畅予再次手术。术后1年行IVP和（或）ECT检查,肾功能明显改善。结论对于婴幼儿及部分正常体重的学龄前儿童,微创小切口离断式肾盂输尿管成形术技术上是可行的,可以获得良好的手术效果。     

Fatal hypertensive encephalopathy in a child with association of multicystic kidney and renal artery stenosis

At the age of 5 years, a boy with known multicystic dysplastic kidney disease showed signs of arterial hypertension with progress to fatal hypertensive encephalopathy. Arterial hypertension was refractory to antihypertensive therapy and the child lost consciousness. Computed tomography of the brain revealed multiple cerebral infarctions. Doppler ultrasound showed an elevation of blood flow in the main artery of the functioning kidney consistent with stenosis as a cause of hypertension. CONCLUSION: Arterial hypertension is a known complication of kidney disease. Multicystic dysplastic kidney and renal artery stenosis is a potentially fatal association. Careful evaluation and monitoring, with special emphasis on blood pressure, should be performed in children with multicystic dysplastic kidney disease.     

Alagille's syndrome associated with cystic renal disease.

Although renal abnormalities have been described in children with Alagille''s syndrome, cystic kidney disease has not often been documented, and then usually only at necropsy. Three children with Alagille''s syndrome are described, in two of whom a unilateral multicystic dysplastic kidney was detected by prenatal ultrasound; in the other, a solitary cortical cyst was found later in childhood. All have normal renal function, growth, and liver synthetic function but continue to have clinical and biochemical signs of cholestasis. These cases show that unilateral cystic kidney disease with or without renal dysplasia may be associated with Alagille''s syndrome, that the clinical course is not necessarily unfavourable, and that Alagille''s syndrome should be included in the differential diagnosis of cystic kidney disorders associated with cholestatic liver disease. Patients with Alagille''s syndrome should be evaluated by renal ultrasound.     

Urinary tract masses

Disorders of the urinary tract account for the majority of abdominal masses in infants. The most common causes of a flank mass in a neonate are a hydronephrotic kidney followed by a multicystic kidney. An increasing number of disorders resulting in hydronephrosis are diagnosed by prenatal ultrasound scan, and their natural history continues to be elucidated. This review will examine the common anatomic and functional causes of fetal and neonatal hydronephrosis as well as the diagnosis and treatment of solid and cystic urinary tract masses.     

Neonatal abdominal cystic masses: spontaneous regression demonstrated with ultrasound

Abdominal cyst was diagnosed in 14 babies by ultrasound examination (9 multicystic dysplastic kidneys, 4 ovarian cysts, 1 bowel duplication). The cysts were followed in 6 patients by ultrasound and marked reduction of multicystic dysplastic kidneys was observed in 2 patients, and 1 ovarian cyst fully disappeared. Their experience in agreement with the literature data suggest that conservative management with sonographic reevaluation is an acceptable alternative to surgical therapy in uncomplicated cases.     

Evaluation of a three-year (1988-1990) prenatal screening of malformative uropathies in the department of Puy-de-D?me]

During a 3-year period, 93 prenatal diagnoses of kidney or urinary tract abnormalities were carried out in the French district of Puy-de-D?me. Sixty-nine mothers were resident in this area giving an incidence of 2.8 out of 1000 births. The pregnancy was interrupted in 10 cases, there were 2 stillbirths and three infants died within two months of life. The most frequent abnormalities were: hydronephrosis (48% of cases), megaureter with or without ureter duplication (19%) and multicystic dysplasia (16%). The prenatal diagnosis was confirmed after birth in 82% of cases. Of the 56 infants with obstructive uropathies, 17 underwent a pyeloplasty within three months of life, 32 had conservative treatment, of whom 4 were operated on afterwards, and seven could not be traced. Of the seven infants who had normal ultrasound scan at birth, three had abnormal scan during the follow-up one of whom was operated on. Nephrectomy was not performed in any of the 11 cases of multicystic dysplasia: one patient was lost to follow up, three had stable lesions and in seven cases, the size of the cysts decreased.     

The management of renal conditions in the perinatal period

Most urogenital abnormalities are now diagnosed antenatally on high resolution ultrasound scans. This has enabled recognition of those that are not compatible with survival and these are managed with termination of pregnancy. Renal anomalies that require surgical intervention continue to pose challenges. Conditions such as multicystic dysplastic kidney can be easily recognised and managed based on the experience gained with long-term studies of its natural history. Polycystic kidney on the other hand while not posing a diagnostic problem remains beyond the reach of therapeutic intervention and postnatal supportive measures are the only available means of dealing with this entity at present. The major difficulty is with the management of antenatally diagnosed pelvicalyceal dilatation. The goal of intervention is to preserve renal function when dilatation is the consequence of obstruction. Unfortunately, by the time ultrasound evidence of significant obstruction is apparent renal damage is already established. Fetal intervention should be considered in those cases where severe oligohydramnios is associated with hydronephrosis, especially in the presence of a solitary kidney or in bilateral disease. Postnatally, all neonates with renal tract dilatation should be managed according to a protocol which mandates serial measurements of renal pelvis diameter and correlates this with data from radionuclide scans. This will enable recognition of kidneys that are at risk of losing function while at the same time avoiding unnecessary surgical intervention in those which remain dilated but are functionally stable.     

Multicystic dysplastic kidney: our experience in non-surgical management

Introduction  

Although multicystic dysplastic kidney (MDK) is a common renal anomaly, the management of this condition remains controversial. The purpose of this study was to focus on its regression by ultrasound (US) scan for MDK managed conservatively.     

Rare pediatric conditions: Contribution of grey-scale ultrasonography

The authors report some unusual pediatric conditions where grey scale ultrasound was the most useful diagnostic tool. Cases of pericardial cyst, polycystic kidney disease of the adult type, hepatic hamartoma and hepatocarcinoma, gallstones, renal pseudo tumors, calcified inferior vena caval thrombus, acute pancreatitis, multicystic kidney and retroperitoneal lymphangioma are briefly described and compared to the literature. In a patient presenting with hereditary tyrosinemia, ultrasound not only disclosed evidence of hepatocarcinoma but was shown to be the procedure of choice for following the course of the disease.     

Mass screening for early detection of congenital kidney and urinary tract abnormalities in infancy

BACKGROUND: Recent widespread use of ultrasound has led to new efforts at screening for congenital kidney and urinary tract abnormalities. However, a standard screening methodology, criteria defining abnormalities, and follow-up procedures remain to be established. In order to establish screening criteria for these abnormalities, we performed a preliminary study in 800 1-month-old infants using provisional methods and criteria. METHODS: Based on the results of preliminary study, we screened 2700 1-month-old infants in a prospective study using the criteria of renal size (longitudinal diameter or=60 mm, or a difference between sides of >or=10 mm), and of pelvic dilatation (Society for Fetal Urology [SFU] grade 2 or higher) as positive at the first ultrasound screening. We used the SFU grading system instead of anteroposterior pelvic diameter measurements for pelvic dilatation. RESULTS: One hundred and twelve (4.1%) of the 2700 infants had abnormalities at the first ultrasound screening, while 18 (0.67%) had congenital kidney and urinary tract abnormalities on further examination. Use of the SFU grading system enabled us to reduce the false-positive rate at first screening, while maintaining a high diagnostic rate. The abnormalities consisted of ureteropelvic junction obstruction in seven infants, megaureter in two, hypoplastic kidney in four, vesicoureteral reflux in six (three were accompanied by hypoplastic kidneys or multicystic dysplastic kidney), multicystic dysplastic kidney in one, and horseshoe kidney in one. CONCLUSION: These results indicate that our screening methods and criteria are useful variables for detecting congenital kidney and urinary tract abnormalities.     

孤立肾并肾盂输尿管连接部梗阻的手术疗效分析

目的了解孤立肾合并肾盂输尿管连接部梗阻(UPJO)的手术治疗时机对于肾功能的影响。方法回顾性分析2006年1月至2018年1月首都医科大学附属北京儿童医院和北京儿童医院顺义妇儿医院收治的孤立肾合并UPJO 23例患儿临床资料。其中男16例,女7例。首诊年龄为1 d~15岁8个月,平均2岁2个月。全组患儿平均保守治疗时间2年9个月。应用SPSS 20.0软件进行统计学分析。结果23例患儿均接受离断性肾盂成形术(A-H术),手术时年龄10个月~16岁4个月,平均4岁11个月。<1岁者2例,分别为10个月、11个月。术后保留双J管2个月,术后3个月时复查静脉肾盂造影(IVP),显示肾盂积水较前无明显变化或不同程度减轻;3例术前IVP 40 min仍不显影者,术后显影明显改善,在10~20 min时显影。随访1年1个月~10年,平均随访3年9个月,泌尿系统超声提示肾盂扩张程度较术前有不同程度减轻。结论孤立肾并UPJO在严密观察下的密切随访及保守治疗至6个月以后是安全的,首选手术方式为肾盂离断成形术。     

Outcome of antenatally detected cystic dysplastic kidney disease.

Forty four fetuses with multicystic dysplastic kidney (MCDK) disease recognised on antenatal ultrasound were studied prospectively. In nine aborted fetuses and in five who died in the neonatal period the MCDK disease was bilateral or there were associated lethal abnormalities or syndromes. All surviving infants had unilateral disease and in six (20%) there was significant reflux into the normal contralateral kidney. Since 1988 the management of unilateral MCDK disease has been conservative with no child developing sepsis, hypertension, or malignancy. Serial ultrasound examinations suggest that MCDK lesions involute with time and conservative rather than operative management is favoured.