Upper pole pelviureteric junction obstruction

A case of neonatal hydronephrosis affecting the upper moiety of a duplex kidney is presented. In addition to the ectopic insertion of the upper-pole ureter into the urethra, the ureter was obstructed by the lower pole at its pelviureteric junction. Accepted: 26 February 1997     

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.     

Complete pelviureteric junction obstruction following a diuretic renal scan

A 13-day-old neonate with a single functioning, hydronephrotic kidney developed complete pelviureteric junction (PUJ) obstruction and anuria following a diuretic radionuclide renal scan. Urgent pyeloplasty resulted in a favourable outcome. Possible dynamics of the obstruction are discussed. Monitoring urine output after diuretic renal scans, especially in infants with a single functioning kidney and PUJ obstruction, is of paramount importance.     

Non-intubated pyeloplasty for pelviureteric junction obstruction in children

Sixty-one consecutive patients undergoing pyeloplasty (5 bilateral) were reviewed retrospectively; 54 pyeloplasties were non-intubated (NIP) and 12 were intubated. NIPs were managed by an extrarenal wound drain, which was removed after 2–4 days in 44 repairs with minimal or no urinary leakage and after 6–8 days in 10 with significant leakage. Fifty-two were successful after the primary procedure. One patient who developed a urinoma after drain removal required a percutaneous nephrostomy followed by a revision pyeloplasty. A second revision pyeloplasty was necessary for persistent postoperative obstruction. The results of NIP compared favorably with series where intubation was used routinely and were superior to alternative methods of management such as endopyelotomy.     

Pelviureteric junction obstruction as sequelae of Kawasaki disease

We present a 7-year-old boy who was admitted with a history of cough for a week, neck pain with associated swelling for 4 days, fever, and vague periumbilical pain. He was diagnosed with Kawasaki disease, and subsequently developed vasculitis of the ureter and stricture of the ureteric lumen at the level of the pelviureteric junction.     

Multicystic dysplastic kidney detected by fetal sonography: conservative management and follow-up

The most common cystic lesion recognized antenatally is multicystic dysplastic kidney (MCDK). Recently, conservative management without nephrectomy has been advocated. The purpose of this study was to report our experience in the conservative management of unilateral MCDK. Between 1989 and 1997, 20 children with MCDK detected by prenatal ultrasonography (US) were prospectively followed. At birth, US confirmed the prenatal findings in all cases. All patients were submitted to radioisotope scans and a micturating cystogram. Follow-up US examinations were performed annually. Mean age at diagnosis during the prenatal period was 31 weeks of gestation (range 24–38). Median follow-up time was 33 months (range 7–91). Follow-up US was performed in 19 children; 13 (68%) showed partial involution, 4 (21%) complete involution, and 2 (11%) an increase in unit size. The mean age at complete or partial involution of the lesion was 18 months. No children developed hypertension or tumors, and all maintained normal growth. In conclusion, the natural history of MCDK is usually benign, and serial US examinations show that affected kidneys frequently show involution with time. Accepted: 14 November 1999     

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     

Ambulatory blood pressure monitoring in children with unilateral multicystic dysplastic kidney

Multicystic dysplastic kidney (MCDK) is one of the most common congenital renal anomalies. Arterial hypertension is a potential complication of MCDK. Blood pressure (BP) has so far been measured only casually and the frequency of hypertension has been estimated to be between 0%–8%. Ambulatory blood pressure monitoring (ABPM) provides more precise information on BP than the casual BP measurement. The aim of this study was to investigate the BP profile in children with MCDK using ABPM. A group of 25 children (16 girls), with a mean age of 7.8 years (range 3.8–17.7 years) were investigated. ABPM was performed using the oscillometric SpaceLabs 90207 device. Hypertension was defined as mean systolic and/or diastolic BP during the day and/or in the night exceeding 95th percentile for ABPM. Five (20%) children showed hypertension, two of them had combined daytime and night-time hypertension and three had isolated nocturnal hypertension, although daytime BP was between the 90th–95th percentile in two of them. Children with ultrasonographical and/or laboratory signs of contralateral kidney abnormalities showed a higher incidence of hypertension than those without abnormalities (two of four versus 3 of 21). The mean night-time systolic and diastolic BP of children with MCDK was significantly higher than in healthy children (+0.50 and +0.54 SDS, respectively, P=0.012 and 0.03, respectively). Three of the hypertensive children were already nephrectomised. All five hypertensive children showed ultrasonographical and/or laboratory signs of contralateral kidney abnormalities. Hypertensive children had significantly higher microalbuminuria than normotensive children (6.9 ± 3.2 mg/mmol creatinine versus 1.8 ± 0.7, P=0.03). The nocturnal BP fall (dip) was attenuated in five children, only one of whom was hypertensive. Conclusion Arterial hypertension in children with multicystic dysplastic kidney is seen more often if based on ambulatory blood pressure monitoring than on casual blood pressure recordings. The main risk factor for developing hypertension is contralateral kidney damage. Ambulatory blood pressure monitoring should be performed in children with multicystic dysplastic kidney, especially in those with contralateral kidney abnormalities. Received: 20 July 1999 and in revised form: 27 November 1999, 24 March 2000, 3 May 2000 Accepted: 3 May 2000     

Pelviureteric junction obstruction in duplex system

An unusual case of a PUJ obstruction in the upper moiety of a duplex kidney is presented. PUG obstruction in duplex moieties is commonly seen in the lower moiety ureter and is usually due to extrinsic compression by a crossing vessel. In our case there was a long stenotic segment of the ureter draining a functionally insignificant and infected segment of the kidney. The opposite kidney was hypoplastic and non functional. An upper polar hemi nephrectomy was performed.     

Single center experience in patients with unilateral multicystic dysplastic kidney

ObjectiveTo determine the clinical features, long-term outcomes, and additional urological anomalies of patients treated for multicystic dysplastic kidney (MDK).Materials and methodsPatients with MDK who were followed between January 2004 and October 2012 were reviewed retrospectively. Demographic, clinical, laboratory, and radiological data were evaluated.ResultsA total of 68 patients with MDK were followed for a mean period of 46.8 ± 32.4 months. MDK was detected by antenatal ultrasound in 64 (94.1%) of the patients. Ten patients had (14.7%) additional urological anomalies in contralateral kidney. Vesicoureteral reflux was found in five patients (7.3%). Other urological anomalies were megaureter (two), cortical renal cyst (two), ureteropelvic junction obstruction (one), and renal ectopy (one). Urinary tract infection was detected in 14 (20.5%) patients. Four (5.9%) patients had hypertension. Compensatory hypertrophy was detected in 29 (42.6%) patients. Eight (11.8%) of these 29 patients had glomerular hyperfiltration and three (4.4%) of these eight patients also had proteinuria. Follow-up ultrasound revealed complete involution in 19 (35.8%) patients. Nephrectomy was performed in 15 (22.0%) patients. Indications of nephrectomy were, recurrent urinary infection (four), hypertension (three), ureterocele (two), renal calculi (one), flank pain (one), hematuria (one), persistent large cystic kidney (three), and atypical US findings (two).ConclusionIn patients with MDK, depending on the clinical, laboratory, and radiological findings, appropriate timing of operation by multidisciplinary approach is important for early detection and treatment of any possible complications.     

Multicystic dysplastic kidney disease and hypertension: clinical and pathological correlation

We report on a child with hypertension secondary to MCDK who underwent an elective retroperitoneoscopic nephrectomy and is normotensive on follow up. We looked at the pathological correlation with respect to the hypertension caused by a non-functioning kidney.     

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.     

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。     

Postnatal outcome and natural history of antenatally-detected hydronephrosis

Routine maternal ultrasonography (US) has revealed a very high incidence of fetal hydronephrosis (HDN), the postnatal outcome and management protocols of which are replete with controversies. Pelviureteric junction (PUJ) obstruction is the commonest postnatal diagnosis, and its management has no consensus to date. This study was carried out to define the postnatal outcome and natural history of fetal HDN and to identify the mode of management to be adopted to the best advantage. All patients were subjected to US 48 h postnatally or at first presentation and isotope renography (DTPA) with nuclear glomerular filtration rate (GFR) was carried out at 3–4 weeks of age or at presentation. Micturating cystourethrography (MCU) and/or direct radionuclide cystography (DRCG) were done in cases with bilateral HDN, nonobstructive HDN, dilated ureter on US, and recurrent urinary tract infection. Patients were then grouped for surgical intervention or conservative follow-up. Investigations were repeated periodically. A total of 56 patients were registered for the study (78 renal units) with a mean follow-up period of 14.6 months; 37 renal units were operated upon or scheduled for surgery and 67 had some organic pathology, of which PUJ obstruction was the commonest. Among patients with PUJ obstruction, indications for surgery included symptoms, a palpable mass, poor or deteriorating function, and a solitary functioning kidney. Patients operated upon showed improvement in function and/or drainage, while those followed conservatively remained stable or improved. Controversy still exists as to the optimal management of PUJ obstruction detected antenatally. While it is the commonest postnatal diagnosis, many other pathologies are now being encountered. In antenatally-diagnosed HDN, there is a good scope for conservative management provided rigid follow-up can be ensured, especially because a good recovery potential exists followed surgery whenever indicated. Accepted: 11 January 2000     

Surgical treatment and outcome of mega-hydronephrosis due to pelviureteric junction stenosis

To study the surgical treatment and outcome of hydronephrosis secondary to pelviureteric junction obstruction (PUJS) that is so massive that the renal pelvis crosses over the vertebral column [mega-hydronephrosis (MH)]. Of the 40 cases of PUJS we treated over the past 5 years, 6 cases had MH secondary to PUJS (MH-PUJS) in 6 renal units and were reviewed. Incidence of MH was 15.0%. All had Anderson–Hynes dismembered pyeloplasty to treat obstructive renal pattern on technetium-99m diethylenetriaminepentaacetic acid (DTPA) scans in five and gait disturbance due to MH in one. The contralateral kidney was normal in five and dysplastic in one. Three had nephrostomy before pyeloplasty. Preoperative DTPA scans showed good renal function in four and poor in two. Plication of the entire dilated, lax renal parenchyma (“nephroplication”) was performed in three to decrease the intra-renal pelvic cavity. Prenatal diagnosis was performed in three cases. Mean age at pyeloplasty was 3.5 years and mean duration of follow-up was 1.5 years. Postoperatively, two of the three who did not have nephroplication had prolonged but not obstructed renal pattern on DTPA scans, and the other case who did not have nephroplication had nephrectomy for non-functioning kidney caused by recurrent pyelonephritis 2 years after pyeloplasty. In the three who had nephroplication, DTPA scans showed acceptable to relatively good passage in all cases. The surgical treatment of MH–PUJS is challenging with no fixed guidelines. However, nephroplication may be useful during pyeloplasty to decrease the size of MH–PUJS.     

Moderate approach to the antenatally diagnosed unilateral ureteropelvic junction obstruction: experience with 93 patients

To evaluate the results of the treatment in patients with antenatally detected unilateral ureteropelvic junction obstruction. Ninety-three patients were analyzed according to the radiologic and scintigraphic findings and mode of the treatment. Fifty-eight patients were treated surgically while 29 patients were followed conservatively. All patients were reevaluated at the first year of the treatment with diuretic renogram (DR). Follow-up procedure was finished at first postoperative year in surgically treated patients, while mean follow-up duration was 14.9±6.9 months for the conservatively treated group. In the operated group, pelvic diameter was greater than 20 mm on ultrasound and overall rate of mean split renal function was 38.65±9.55% on DR and 39.65±12.55% at first postoperative year. In the conservatively treated group, mean split renal function was 46.17±3.42 at the beginning and 47.48±4.00 at first year of follow-up. Sixty-three percent of the patients underwent pyeloplasty while 83% of them were showing obstructive pattern on DR. We believed that surgery should be done in renal units which showed pelvic diameter greater than 20 mm and less than 40% of split renal function, while others might be followed conservatively, with or without an obstructive pattern on DR.