Multicystic kidney dysplasia

The clinical course of 48 children (27 boys and 21 girls) with multicystic kidney dysplasia was analysed retrospectively. The patients were seen and treated at the Children's Hospital of Medical School Hannover between 1976 to 1989. There was no familial occurrence of the disease, yet in eight families various other renal diseases could be observed. In 20 patients the diagnosis of multicystic renal dysplasia was presumed by prenatal sonographic examination, in the other patients the diagnosis was established at the age between 1 day to 12 years. The first manifestations were palpable abdominal mass (n = 16), urinary tract infection (n = 4), casually because of a sonographic evaluation for other reasons (n = 4) and vomiting (n = 2). Associated malformations were found in 18 patients: cardiac malformations (n = 6), dysplasia of the other kidney (n = 5), ureter obstruction of the other kidney (n = 3), horseshoe kidney (n = 1) and others (n = 3). There was no hypertension and serum creatinine levels were normal in those children, who did not suffer from associated malformations of the other kidney. The multicystic kidney was removed operatively in 42 patients at the age of 3 days to 9.5 years (median 4 weeks). The prognosis of multicystic kidney dysplasia depends on associated renal and other malformations.     

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.     

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     

Multicystic dysplasia in one-half of a horseshoe kidney with megaureter and lower ureteric atresia

During evaluation of chronic abdominal pain, a 9-year-old male was found to have a horseshoe kidney (HSK) with multicystic dysplasia (MCD) of the left-sided component. Attached to the MCD was a very large, tortuous ureter occupying almost the whole left side of the abdomen. This ureter on dissection was found to end blindly adjacent to the bladder. MCD of one-half of a HSK is an unusual lesion. Its association with a large megaureter with juxtavesical atresia is a unique event. In HSKs, controversy exists about the need to remove a small dysplastic segment. If this segment is associated with a large ureter, as in our case, removal is mandatory in order to avoid pain and infection.     

Bilaterally obstructed ureteropelvic junction of the upper moieties in a complete duplex collecting system

Maternal ultrasound is a routine examination in prenatal evaluation. The number of fetal abnormalities detected has been increasing, and includes fetal hydronephrosis which is secondary to ureteropelvic junction obstruction in 80% of cases. We report a case of a 1-year-old female infant with hydronephrosis prenatally detected by ultrasound. After close postnatal follow-up and diagnosis, she eventually underwent a definitive reconstructive procedure. This is the first reported case of a bilateral ureteropelvic junction obstruction of the upper moieties of a duplex collecting system.     

Multicystic renal dysplasia. Spontaneous remission of a hypertrophic form

A case of spontaneous regression of the tumor type of multicystic dysplastic kidney (MDK) is reported. This case is consistent with recent data on the natural history of MDK. The classical therapeutic approach which involves surgery is discussed in the context of this case and of others with a similar course.     

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.     

Multicystic kidney disease in a segment of horseshoe kidney: an uncommon combination of malformations

Multicystic dysplasia is the most common type of renal cystic disease, and it is one of the most common causes of an abdominal mass in infants. The contralateral system is also frequently abnormal. The most commonly associated anomalies are contralateral ureteropelvic junction obstruction and contralateral vesicoureteral reflux. In a few cases, multicystic dysplasia involves a horseshoe kidney or one pole of a duplex kidney. We present a female patient diagnosed with this condition at 9 years old, when she was being studied due to recurrent abdominal pain. The renal function was normal.     

Laparoscopic heminephroureterectomy for duplex kidney anomalies in the pediatric population

ObjectiveDespite increasing acceptance of laparoscopy in pediatric urology, few published data are available on laparoscopic heminephroureterectomy in patients with duplex kidney anomalies. In the present study, we report our own experiences with this technique.Patients and methodsFrom August 2003 to January 2006, five laparoscopic heminephroureterectomies were performed in four girls with a mean age of 41 (9–67) months. In all cases, a non-functioning upper pole with an obstructive (n = 4) or refluxing (n = 1) megaureter was found. The upper pole was resected laparoscopically en bloc with the megaureter using three to four trocars.ResultsMean follow-up was 42.4 (±7.9) months. All procedures were performed successfully without conversion to open surgery with a mean operative time of 190 (170–210) min. Blood loss was minimal and no intraoperative complications occurred. Despite chronic inflammation in the resected specimens, the patients showed no clinical signs of infection postoperatively. The average length of hospital stay was 5.6 (4–7) days. All patients were followed using duplex sonography.ConclusionsThese data demonstrate that, even in infants, laparoscopic heminephroureterectomy is feasible and associated with minimal morbidity, a better cosmetic result and a shorter hospital stay compared to open surgery. The main disadvantage of the laparoscopic approach is a longer operative time. Laparoscopic heminephroureterectomy is a technically demanding procedure and should be performed only in specialized centers.     

Multicystic dysplastic kidney: Impact of imaging modality selection on the initial management and prognosis

ObjectiveTo evaluate the impact of imaging modalities on the evaluation and prognosis of children with multicystic dysplastic kidney (MCDK) disease.Patients and methodsA retrospective analysis of all children with MCDK diagnosed from 2004 until 2012 was performed. The study included 63 patients for whom all postnatal imaging modalities were available: renal bladder ultrasound (RBUS), dimercaptosuccinic acid scan (DMSA) and voiding cystourethrogram (VCUG). Cases with major congenital abnormalities or incomplete data were excluded. Abnormalities in the contralateral kidney and the fate of MCDK were also addressed.ResultsAt diagnosis, the average age was four-and-a-half months. The majority of cases were detected antenatally (87%). Postnatal RBUS and DMSA scans established the diagnosis of MCDK in 92% and 98% of patients, respectively. DMSA showed photopenic areas in the contralateral kidneys in 10% of patients; all of them had hydronephrosis and were confirmed to have vesicoureteral reflux (VUR). Contralateral VUR was detected in 16 patients; 63% of them had hydronephrosis. After a mean follow-up of three-and-a-half years, involution occurred in 62% of patients and the involution rate was inversely proportional to the initial size.ConclusionsThe classical appearance of MCDK on RBUS was sufficient to establish the diagnosis in most patients. DMSA scan was more accurate in confirming the diagnosis and evaluating the contralateral kidney. Selective screening for VUR in patients with contralateral hydronephrotic kidney should be considered.     

Uterus duplex with hematometrocolpos and ipsilateral agenesis of the kidney

BACKGROUND: Malformations of the mullerian-duct-system occur with an incidence of about 0.001-0.005% of the population, and they are frequently associated with unilateral agenesis of the kidney. CASE: A 12-year-old girl presented to our emergency department with cyclic abdominal pain which increased with each of her menses. Ultrasound investigation showed a uterus duplex with obstructed hemivagina and hematometrocolpos as well as an ipsilateral absence of the kidney. Additionally, a cystic expansion in the small pelvis was detected which required further investigations (MRT and MR-angiography) to establish the diagnosis of a hematosalpinx. Excision of the vaginal septum resulted in drainage of the hemato-metrocolpos and the hematosalpinx. CONCLUSION: Unilateral hematometrocolpos should be considered as an important differential diagnosis in female adolescents with relapsing lower abdominal pain, even if normal menstruation occurs.     

Surgical significance of the duplex kidney with bifid ureter.

Between 1974 and 1990 in 16 children (5 boys and 11 girls) with a duplex kidney and bifid ureter surgery was indicated. The mean age of the 15 already operated patients was 7 years (0.3-12 years). 14 had uretero-ureteric reflux, 1 had a stenosis of the ureteric bifurcation, and 1 had a lower pole pelvi-ureteric junction obstruction. An interpyelic anastomosis was performed in 14 and a ureteroneocystostomy "en bloc" in 1. The follow-up was 3-10 years with a mean of 5 years. All 15 patients had normal uroradiological findings, and out of the 9 patients with longer follow-up (more than 3 years) 7 had a significantly diminished infection rate.     

Multicystic dysplastic kidney disease: update and information for parents at the time of prenatal diagnosis]

Multicystic kidney disease (MCKD) is the most common form of Congenital Abnormality of Kidney and Urinary Tract (CAKUT). This anomaly of renal development is characterized by unilateral enlarged cystic formations and fibrous dysplastic parenchyma. The long-term prognosis is usually good; however because of reduced nephron mass, an early prevention of cardiovascular risk and nephrotoxicity is recommended. A lifelong follow-up of blood pressure, serum creatinine and microalbuminuria seems logical as well as in other patients with a single kidney. MCKD is usually diagnosed during pregnancy so that parents often question about long-term prognosis and follow-up. Therefore, we propose an information sheet for parents.     

The role of minimal surgery with renal preservation in abnormal complete duplex systems

Among 36 children, mostly less than 1 year of age, referred for a complete duplex-system anomaly, 18 were diagnosed before birth by obstetrical ultrasonography where a diagnosis of dilated upper urinary tract was the most frequent report; in the remaining cases urinary tract infection was the main presenting symptom. Five children had bilateral anomalies. Principal diagnoses associated with the complete duplex system were: 18 ureteroceles (UC) (11 extravesical [EUC] with bladder-neck or urethral extension, 7 intravesical [IUC]), 5 ectopic ureters without UC and 28 lower-polar vesicoureteral reflux (VUR) associated in 6 with upper polar VUR. The initial assessment was based on a voiding cystogram and radionuclide scan. Renal-polar function was severely impaired when major ureteral ectopia or severe primary reflux was present. Primary surgery was performed in 8 patients, demolitive in 4 (3 upper-polar nephrectomies, 1 nephrectomy) and reconstructive in 4 (duplex en-bloc reimplantation); staged management with minimal endoscopic incision was undertaken in 15 UCs (9 EUCs and 6 IUCs). Expectant management was elected in all cases of mild primary, single, or double VUR not associated with UC and was followed by spontaneous reduction in one-half of the cases. Secondary VUR complicated endoscopic incision in 2 of 6 IUCs and 5 of 9 EUCs; a certain degree of functional improvement after decompression could be observed in all IUCs versus only 2 EUCs. Twenty-one patients requested secondary surgery; 17 needed an open intravesical procedure for ureteral reimplantation, combined in 8 with UC excision and bladder-floor reconstruction and in 5 with upper-pole nephrectomy. A nephrectomy was required in 4 cases. All primary or secondary demolitive procedures involved 9 of 11 EUCs extravesical and 2 of 5 ectopic ureters. EUCs and ureteral ectopia were associated with severe renal-polar damage, and function was rarely affected by primary decompressive procedures even in prenatally detected, uninfected cases. Endoscopic incision of EUCs was frequently followed by secondary VUR, which made secondary intravesical operations more complex. For these reasons, primary elective resection of a dysplastic upper pole is preferable in most cases to temporary decompressive measures. Conservative surgery is always indicated in IUC, which may benefit from endoscopic decompression. Isolated VUR involving the lower pole of a completely duplicated system may respond to expectant management in a significant number of cases. Accepted: 5 January 1999     

Cystic dysplasia of the testis associated with multicystic dysplastic kidney

Cystic dysplasia of the testis (CDT) is a rare congenital defect characterized by the formation of multiple irregular cystic spaces in the mediastinum testis. Co-existent genitourinary lesions have commonly been associated with this lesion and have included absence of the ipsilateral kidney, duplication anomalies, and cryptorchidism. We describe the first case in which multicystic dysplastic kidney (MCDK) is associated with CDT.     

Multicystic dysplastic kidney disease presenting with a single large cyst in a fetus-anatomical basis and radiological aspects

Multicystic dysplastic kidney (MCDK) is a congenital maldevelopment in which the renal cortex is characteristically replaced by numerous cysts of multiple sizes. MCDK presenting as a single predominant large cyst in morphology is less common. We report on the prenatal imaging findings and perinatal management of a fetus with MCDK unusually presenting as a single predominant large cyst, erroneously interpreted as a severe fetal hydronephrosis. Details of the perinatal history, radiological evaluation, morphological characteristic, and clinical aspect of this case are presented. We also discuss a few studies addressing the sensitivity of magnetic resonance urography for the prenatal diagnosis of MCDK.     

Colour-coded duplex sonography in the diagnostic assessment of vascular complications after kidney transplantation in children

Background. Vascular complications are a major cause of dysfunction or transplant loss in children. Arterial or venous occlusion, transplant renal artery stenosis (TRAS) and some arteriovenous (AV) fistula require rapid detection and prompt intervention. The present study was performed to determine the accuracy of colour Doppler sonography (CDS) in the early and late phase after renal transplantation and to correlate the results with angiographic and intraoperative findings. Objective. CDS is the preferred imaging modality with a high diagnostic accuracy for follow-up of renal transplantation in children. The indication for angiography should be established on the basis of the CDS diagnosis. Materials and methods. In 87 children (mean age 10.9 years, range 2–17), 423 CDS examinations were performed after renal transplantation. Angiography was performed in 17 cases; surgery was necessary in 16 patients. Results. CDS correctly identified 8/8 arterial or venous occlusions and 7/7 TRAS. The only false positive diagnosis of TRAS was due to misinterpretation of an iliac artery stenosis. Six AV fistulae were diagnosed by CDS. The overall positive predictive value of CDS was 94 % in this study. Conclusion. CDS is a noninvasive, non-radiation producing imaging modality with a high diagnostic accuracy. It is the method of choice in the assessment of vascular complications after renal transplantation in children. Received: 15 January 1997 Accepted: 3 July 1997     

Bladder surgery as first-line treatment of complete duplex system complicated with ureterocele

ObjectivesWe retrospectively analyzed our experience to determine the optimum management of complete duplex system complicated with ureterocele.Materials and methodsBetween 1994 and 2003, we reviewed 15 patients treated with bladder surgery for complete duplex system complicated with ureterocele. The associated anomalies were seven vesicoureteral reflux (VUR) of the lower pole ureter and one of both poles. All patients had ureterocele (11 intravesical, 4 extravesical) and a functioning upper moiety. Initial treatment was transurethral incision of ureterocele (TUI) (14) or common sheath reimplantation (1). The median follow-up was 30 (13–48) months.ResultsThere were no requirements for secondary procedures, with a significant improvement or conservation of ipsilateral renal function and no reflux, in 10 patients (67%). Five patients (33%) with ureterocele (1 intravesical, 4 extravesical) who initially underwent TUI required additional operative management due to moderate to severe VUR, recurrent urinary tract infection or decreased function of upper moiety. The secondary operation performed was lower pole nephrectomy with ureteral reimplantation (1), ureteroureterostomy with ureteral reimplantation (2) or common sheath reimplantation (2). One patient who underwent common sheath reimplantation as secondary operation needed a nephrectomy due to breakthrough febrile urinary tract infection and decreased renal function.ConclusionTUI is recommended as the first-line treatment of complete duplex system with intravesical ureterocele and well-conserved renal function.