Perinatal management of congenital anomalies of the urinary tract detected in utero

The routine use of maternal ultrasonography has led to frequent discovery of fetal anatomical abnormalities of the urinary tract. Herein we report on 35 cases with congenital urogenital anomalies which were found during the last 5 years by the prenatal ultrasonography and referred to our clinic either for treatment or consultation. 1. Findings of prenatal ultrasonography There were 12 cases of hydronephrosis, 7 of multicystic dysplastic kidney, 4 of megaureter, 3 of posterior urethral valve, 2 of ureterocele, 2 of vesicoureteral reflux and 5 other cases. Fetal anomalies were diagnosed during routine maternal examination, as intrauterine growth retardation, oligohydramnios, or the recurrent risk secondary to genetic risk factor or previous abnormal pregnancies. Prenatal diagnosis was made as early as 20 weeks of gestation in a case of hypoplastic urethra with large bladder diverticula. In about 30% of cases, abnormalities were found before 30 weeks of gestation. Oligohydramnios was evident in 5 cases. Prenatal diagnosis was correct in about 60% of cases. In the remainder the diagnosis was either incomplete or incorrect. Errors in the diagnosis resulted from difficulties in the differentiation of dilated ureter, intestinal dilation or intraperitoneal cystic masses. 2. Management in newborn periods Of the patients, 12 were treated urologically in newborn periods. Although patients with distended bladder and dilatation of upper tracts first underwent therapeutic placement of the urethral catheter, upper tract diversion with nephrostomy or tubeless ureterocutaneostomy was required in 2 because of continued elevation of serum creatinine levels. In 2 neonates nephrectomy was indicated for unilateral multicystic kidney, because the large cystic mass might compress the intestine.(ABSTRACT TRUNCATED AT 250 WORDS)     

The value of ultrasonography as a screening procedure of the neonatal urinary tract: A survey of 1021 infants

In 1021 children, aged several hours to 21 days, sonographic investigations of kidneys and bladders were performed. 1001 children without any urinary tract abnormality were divided into 7 body weight-dependent groups. The kidney volume was correlated to somatometric data including body weight and body length, breadth of thorax, and head circumference. Regression line for the dependence of renal volume on body weight was calculated. The remaining 20 children with urinary tract abnormalities were described separately. During ultrasonic screening examination, bladder or renal disorders found in those 20 children were as follows: mild hydronephrosis in 6 cases, and severe hydronephrosis in 4 cases; of the latter 1 case was caused by ectopic ureterocele, 1 by simple ureterocele, 1 by obstruction of the distal ureter (primary megaureter) and 1 by vesicoureteral bilateral reflux. In 2 cases unilateral multicystic kidneys were found, 2 infants had an ultrasound pattern with hyperechoic medulla, in 4 cases unilateral non-obstructed renal duplication was suspected, and in 2 cases only a single kidney was found.     

Contralateral Vesicoureteral Reflux in Children With a Multicystic Kidney

Multicystic kidneys are commonly diagnosed today due to the widespread use of prenatal ultrasound. Children with a multicystic kidney are at increased risk of contralateral renal abnormalities. We performed a voiding cystourethrogram on 65 children with a newly diagnosed multicystic kidney to determine the incidence of contralateral vesicoureteral reflux. Ten children (15 percent) with a multicystic kidney had contralateral vesicoureteral reflux, including 8 of 37 boys (22 percent) and 2 of 28 girls (7 percent). Contralateral reflux occurred in significantly more white (22 percent) than nonwhite (4 percent) patients (p less than 0.001). Reflux was grade I in 2 children, II in 2, III in 2, IV in 1 and V in 3. All children were placed on antimicrobial prophylaxis.During a mean followup of 3.1 years grades I and II reflux resolved. Grade III reflux resolved in 1 child and remained stable in 1. Grade IV reflux was downgraded to III in 1 child on prophylaxis. One child with grade V reflux was stable on prophylaxis while the remaining 2 patients underwent ureteroneocystostomy. No child had a urinary tract infection. A significant proportion of white children with a multicystic kidney have contralateral vesicoureteral reflux and initial imaging should include a voiding cystourethrogram.     

Reliability of ultrasound in the prenatal diagnosis of urinary tract abnormalities

To investigate the ability of ultrasonography to detect urinary tract abnormalities prenatally, we reviewed the records of 26 pregnancies diagnosed by prenatal ultrasound to have urinary tract anomalies. We compared the prenatal diagnoses with postnatal renal and urinary tract pathology. This comparison showed different degrees of agreement for different prenatal diagnoses (2 individuals had more than one diagnosis): 4 of 8 for multicystic kidneys, 1 of 1 for polycystic kidney disease, 1 of 2 for renal agenesis, 6 of 7 for ureteropelvic junction obstruction, 1 of 3 for posterior urethral valves, 4 of 5 for no pathology noted, and 2 of 2 for other abnormalities. Prenatal diagnosis and postantal findings were in agreement in 68% of cases. Varying levels of diagnostic reliability should be considered when managing pregnancies complicated by fetal urinary tract abnormalities and subsequent postnatal evaluation and diagnosis. Further efforts are needed to improve on the techniques and reliability of prenatal diagnosis of urinary tract abnormalities.     

Ureterocystoplasty: an alternative reconstructive procedure to enterocystoplasty in suitable cases

PURPOSE: The objective of the report is to present the results of ureterocystoplasty in 6 children with megaureters and low-capacity, high-pressure bladders. METHODS: Of the 6 patients, 2 had valve bladders, 1 had Hinmann's syndrome, 1 had neuropathic bladder, and the remaining 2 with ureterocutaneostomy were mainly diverted because of refluxing megaureters. Nephrectomy was performed in both of the boys with posterior urethral valve because of vesicoureteral reflux dysplasia (VURD) syndrome, and the ipsilateral ureter was used for the augmentation. In 2 patients with ureterocutaneostomy and in 1 with Hinmann's syndrome, a transureteroureterostomy was carried out, and the distal part of the ureter was used to perform augmentation. The patient with neuropathic bladder had a nonfunctioning crossed ectopic left kidney with an associated ipsilateral, refluxing megaureter, and the ureter was used for augmentation after the nephrectomy. RESULTS: All of the patients are continent, and 4 patients who are neurologically normal void spontaneously without requiring clean intermittent catheterization (CIC). The average increase in bladder capacity is 263% (range, 190% to 340%). CONCLUSIONS: Ureterocystoplasty is the bladder augmentation of choice for patients with a nonfunctioning kidney with an associated ipsilateral, refluxing megaureter and for patients with kidneys both in good function and megaureters suitable for a transureteroureterostomy.     

Outcome of neonates born with unilateral multicystic dysplastic kidneys

AIMS: To review our experience of neonates with unilateral multicystic dysplastic kidneys (MCDKs) and to plan how to manage this anomaly. PATIENTS AND METHODS: Forty-eight neonates (30 boys, 18 girls) with unilateral MCDK were referred to our institute between August 1991 and February 1999. Urological evaluation was performed by USG, VCUG and radionuclide study. Follow-up period was 15 month to 106 months (Ave. 54 months). RESULTS: Forty-five of 48 (93.8%) MCDKs were found prenatally. No surgical procedure was performed in utero. Seven (14.6%) had low grade vesicoureteral reflux (ipsilateral 5, contralateral 2). Dilation of contralateral upper urinaly tract was detected in 16 (33.3%) neonates and diuretic renography revealed 4PUJ obstruction and 2 mid-ureteral stenosis. Although 29 of 48 (60.4%) MCDKs were large, no neonate showed mass effect which caused vomiting or dyspnea. All MCDKs except two, which removed because of ipsilateral ureterocele or ectopic ureter, were followed conservatively. Two boys had nephrectomy when they became 5-year-old on their parent's request. Surgical correction of contralateral urinary tract anomaly, 3 pyeloplasty and 2 end-to-end ureteral anastomosis, was performed. All neonates but one with contralateral hypodysplastic kidney had good renal function. Neither hypertension nor malignant tumor had occurred. CONCLUSION: These findings suggest that neonates with unilateral MCDKs can be treated conservatively only if they have no contralateral serious anomaly. Surgical intervention is not necessary for unilateral MCDKs before and after birth but socially and/or economically it depends on patients' request.     

Clinical outcome of fetal uropathy. I. Predictive value of prenatal echography positive for obstructive uropathy

Clinical followup was performed in 73 neonates with a prenatal echographic suspicion of uropathy. Of 42 patients with a prenatal suspicion of unilateral hydronephrosis only 15 had pathological obstruction and 2 had multicystic dysplastic kidneys. Among 10 infants with a prenatal suspicion of bilateral hydronephrosis only 1 had true bilateral obstruction and 2 had unilateral obstruction. In 2 patients hydroureteronephrosis seen on prenatal echography was due to massive bilateral vesicoureteral reflux. In this group there was also a multicystic dysplastic kidney and 1 patient with bilateral cystic dysplasia. There was a prenatal suspicion of cystic disease in 8 infants. Postnatally, diagnosis was multicystic dysplastic kidney in 2 patients and a simple renal cyst in 4. The remaining 2 neonates had obstructive uropathy. Finally, of 13 neonates with a prenatal suspicion of anatomical-echo-structural abnormalities a definitive abnormality could be established in only 8. The predictive value of prenatal echography positive for obstructive uropathy was 34.6%.     

小儿输尿管膨出症的治疗

目的探讨各种方法治疗输尿管膨出症的疗效,寻找影响预后的有关因素。方法 31例输尿管膨出症,男4例,女27例;左侧15例,右侧12例,双侧4例。其中单一输尿管膨出2例,重肾并输尿管膨出29例。VCU检查18例,3例发现中—重度反流,31例均行超声和IVU及CT检查。结果术后随访0.5～3 a,2例单一输尿管膨出症行膨出切除输尿管膀胱再植术,29例重肾中,4例经膀胱行输尿管膨出切除输尿管膀胱再植术。3例中—重度反流,2例行输尿管膨出切除加上半肾切除术,1例行上半肾切除术,术后因反流持续存在,需再次手术;其余22例均行上半肾切除术,术后2例因严重尿路感染行输尿管残端切除术。结论输尿管膨出的治疗应根据输尿管膨出的类型、肾功能、有无反流决定手术方式,对于大多数重肾,单纯上半肾切除预后良好,若术前VCU检查有中—重度返流,应行完全重建术。     

Unilateral massive vesicoureteral reflux associated with congenital mechanical obstruction of lower urinary tract

We reviewed 9 children with unilateral massive (III and IV, Dwoskin-Perlmutter) vesicoureteral reflux associated with congenital mechanical obstruction of the lower urinary tract. Four children with bilateral reflux with bilateral difference of severity more than 2 grades were also included. Obstruction was attributed to posterior urethral valve in 5, anterior urethral stricture in 3 and mega-urethra in 1. The chief complaints were febrile episodes and urinary infection, followed by voiding difficulty, bed wetting and/or urinary incontinence and voiding through anus. All 4 children with troubles other than posterior urethral valve had either anorectal anomaly or H-type rectourethral fistula without imperforate anus. Of the 9 children 8 had massive reflux on the left side. The majority of these left ureters with massive reflux were associated with ipsilateral severe renal hypofunction on scintigraphy. After prompt resolution of obstruction, the majority of these massive reflux did not disappear. Decompression appeared to salvage none of such kidneys. The orifice positions of such ureters were far cranio-lateral and the resected kidneys showed dysplasia or severe hypoplasia. The prognosis was generally excellent except one child with massive reflux on the right side who progressed to an end-stage renal disease. The embryogenesis of this "VURD syndrome" was discussed in its relation to the "bud theory" of renal hypo-dysplasia. It was showed that this syndrome was observable in obstructions other than the posterior urethral valve. Massive reflux appeared to serve as "pop-off" mechanism to buffer high pressure and to lead to preservation of better renal function. Reflux in children with congenital mechanical obstruction may be the result of 2 different mechanisms.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical treatment of vesicoureteral reflux in children

There is no universally applicable method for the treatment of vesicoureteral reflux. In choosing the conservative measures or surgical technique, aetiology, the presence of other congenital or acquired urogenital changes, and the consequences of vesicoureteral reflux should be taken into consideration. During the past 10 years anti-reflux operations have been performed in 66 children; neoimplantation in 24, Boari's plastic operation in 33 and Gregoir's operations in 5 cases. The results of operation have been more favourable in bladder neck sclerosis, congenital anomalies of the orifice and megaureter than in the cases of inflammation. As far as the different operations are concerned, Boari's plastic operation has proved to produce the best results.     

Urinary tract infections in children with posterior urethral valves after kidney transplantation.

The records of 14 boys with posterior urethral valves who had renal failure and subsequently underwent renal transplantation were reviewed to determine the postoperative incidence of urinary tract infection relative to that of 29 male transplant children without valves, who served as controls. There were no significant differences between the posterior urethral valve patients and controls with regard to age, donor source, immunosuppression, followup after transplantation or mean calculated creatinine clearance. Vesicoureteral reflux was found in 1 child with posterior urethral valves and 3 of the children in the control group (p not significant). A total of 15 urinary tract infections occurred in 5 children (36%) with posterior urethral valves, for a rate of 1 per 30 patient-months of followup, and 6 urinary tract infections occurred in 2 controls (7%), for a rate of 1 per 216 patient-months of followup (p < 0.05). However, only 1 of 26 controls (4%) without vesicoureteral reflux had urinary tract infection, for a rate 1 per 1,144 patient-months (p < 0.01). Conversely, the rate of urinary tract infections in controls with vesicoureteral reflux was similar to that of children with posterior urethral valves. Of the 5 children with posterior urethral valves 4 had the initial urinary tract infection within 2 months of transplantation and 10 of 15 episodes occurred within the first 4 months. Antimicrobial prophylaxis did not appear to decrease the rate of infection in children with posterior urethral valves. A history of posterior urethral valves increases the frequency of urinary tract infection after renal transplantation but the usefulness of antimicrobial prophylaxis and the relationship to long-term graft function remain to be determined. Urinary tract infection rarely develops in other transplanted boys without vesicoureteral reflux.     

Use of 99mtechnetium-dimercaptosuccinic acid to study patterns of renal damage associated with prenatally detected vesicoureteral reflux.

Static isotope imaging with 99mtechnetium-dimercaptosuccinic acid was performed at a mean age of 34 days in 32 children (50 kidneys) whose vesicoureteral reflux had been identified as a result of prenatal ultrasound scanning. Three patterns of isotope uptake were observed: 1) noninfected primary vesicoureteral reflux (15 children, 24 kidneys), 2) noninfected secondary (obstructed) vesicoureteral reflux (9 patients, 11 kidneys) and 3) infected primary reflux (8 infants, 15 kidneys). In 20 pattern 1 kidneys (83%) renal morphology and differential isotope were normal. In the 4 kidneys (17%) that showed evidence of impaired function this took the form of global parenchymal loss, that is small kidneys rather than focal scarring. In pattern 2 the combination of fetal vesicoureteral reflux and obstruction was a potent cause of renal damage with total or near total loss of function in 7 of 9 refluxing units associated with posterior urethral valves and in 2 kidneys with secondary ureteropelvic junction obstruction. Appearances of focal scarring were confined in pattern 3 and were found in 4 kidneys (27%). This overall incidence of detectable renal damage was lower than expected. Even when infection occurs, prenatal diagnosis may lessen the risk of scarring by enabling treatment to be instituted promptly. The findings suggest that uncomplicated primary vesicoureteral reflux is a relatively benign insult to the fetal kidney and that reflux nephropathy found in children presenting clinically is the result of infected vesicoureteral reflux in postnatal life. Any comparison of published studies will prove difficult until there is a more standardized approach to imaging technique and patient selection.     

Single vaginal ectopic ureter and renal hypoplasia associated with urogenital sinus and abdominal muscular hypoplasia-a novel subtype of prune-belly syndrome in a female child?

We report a rare case that can be classified as prune-belly syndrome in a female. This patient came to our attention during the 19th week of gestation when massive ascites was found by ultrasound and the mother was transferred to our hospital. After birth, it was demonstrated that the infant had complicated anomalies, including abdominal muscular hypoplasia, urogenital sinus, a single, right-sided vaginal ectopic ureter, and hypoplasia of the right kidney. A sibling had a right hypoplastic kidney and right vesicoureteral reflux. Considering the combination of anomalies and the family history of urinary tract disease, her anomalies could be attributed to a genetic mutation.     

Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome

Purpose

The aim of the study was to assess the presentation, imaging findings, management, and outcome of segmental multicystic dysplastic kidney (MCDK) in children.

Materials and Methods

Six patients with segmental MCDK were diagnosed and observed at our hospital. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, associated urinary tract anomalies, operation, and outcome.

Results

Three patients had abnormal prenatal ultrasound and were diagnosed by further postnatal imaging; they were asymptomatic after birth. The other 3 children presented with a renal mass, recurrent urinary tract infection, or urinary incontinence. Five patients had associated ipsilateral or contralateral urinary tract abnormalities, including vesicoureteral reflux, ureterocele, duplex collecting system, ureteropelvic junction stenosis, and ectopic ureter. Lower tract reconstruction was performed in 3, and open biopsy was done in 1 child. None underwent partial or total nephrectomy, and all had involution of the cysts for a mean of 40 months.

Conclusions

Most of the cases occur in the upper pole of a duplex kidney and often involute spontaneously without significant complication. The clinical presentation and imaging findings depend on the associated anomalies and complications. Although the latter may require surgery to resolve, segmental MCDK itself rarely requires resection.     

The endoscopic transurethral treatment of ureterocele]

The authors describe results of transurethral endoscopic treatment of ureterocele in 61 patients, two techniques of a relevant operative intervention, indications, complications of an early (pyelonephritis) and late (vesicoureteral reflux) postoperative period, measures of their prevention. Advancing impairment of the urine outflow via the upper urinary tracts on ureterocele side necessitates earlier surgery (transurethral dissection or resection of ureterocele). Good 4-year follow-up results (normal function of the kidney, absence of pyelonephritis attacks and vesicoureteral reflux) suggest endoscopic transurethral treatment of ureterocele to be a method of choice the failure of which implies open surgical intervention.     

Mayer Rokitansky Kuster Hauser syndrome with urogenital sinus anomaly

Mayer Rokitansky Kuster Hauser (MRKH) syndrome is a rare disorder, characterized by the congenital absence of uterus and associated renal tract anomalies. The case presented with primary amenorrhea and primary infertility, despite development of normal female secondary sexual characteristics. CT scan revealed absent uterus, a solitary left sided pelvic kidney and a vesicovaginal communication that, on cystoscopy, revealed urogenital sinus anomaly manifesting as a common channel formed due to absent anterior wall of vagina and posterior wall of urethra. The urogenital sinus anomaly in MRKH syndrome has not been reported earlier.     

Vesicoureteral reflux and ureteropelvic junction obstruction in children with horseshoe kidney: treatment and outcome

PURPOSE: Horseshoe kidney is the most common renal fusion anomaly. We determined the treatment and outcome of vesicoureteral reflux and ureteropelvic junction obstruction in children with horseshoe kidney. MATERIALS AND METHODS: We reviewed the medical and radiological records of 52 consecutive children, including 32 boys and 20 girls, in whom horseshoe kidney was diagnosed at 2 children's hospitals during 1990 to 1999. Patient age at diagnosis was 1 day to 12 years (mean 3.9 years). In 2 children with horseshoe kidney neuropathic bladder was secondary to spina bifida and they were excluded from study. The diagnosis was made in all cases by abdominal ultrasound and confirmed by excretory urography or (99m)technetium-dimercaptosuccinic acid scan. Voiding cystourethrography was performed in 40 cases (80%). Patients were followed for 2 to 11 years (mean 4.2). RESULTS: Associated urological anomalies were identified in 26 patients (52%) with horseshoe kidney, including primary vesicoureteral reflux in 13, ureteropelvic junction obstruction in 12 and ectopic ureter in 1. Surgical intervention to correct the anomalies in 15 of the 26 children (58%) involved pyeloplasty in 8, ureteral reimplantation in 2, endoscopic treatment for vesicoureteral reflux in 2, ureterolithotomy in 1, upper pole heminephrectomy in 1 and valve fulguration in 1. No significant complications were observed in surgically treated patients. CONCLUSIONS: More than half of the patients with a clinically symptomatic horseshoe kidney have vesicoureteral reflux or ureteropelvic junction obstruction. Many patients with horseshoe kidney require surgical intervention for associated urological anomalies with good results.     

The outcome of renal transplantation in children with posterior urethral valves

The effect of vesical dysfunction on the survival and function of renal transplants was evaluated by a retrospective study in which 18 children with posterior urethral valves and 18 children with vesicoureteral reflux were randomly matched with 36 children used as controls. There was no statistically significant difference in patient survival among the 3 groups. Five years after transplantation 50 per cent of the grafts in children with posterior urethral valves were functioning, while 73 and 75 per cent, respectively, of the grafts were functioning in children with vesicoureteral reflux and in the control group. Renal function during the 5 years was significantly better in children in the control group and in those with vesicoureteral reflux than in children with posterior urethral valves. We believe that the presence of an abnormal bladder may alter graft survival and adversely affect the function of the transplanted kidney.     

Pediatric urological causes of hypertension

PURPOSE: We reviewed the association of hypertension with several common pediatric urological conditions. MATERIALS AND METHODS: We comprehensively reviewed the published literature linking hypertension with urinary tract infection, renal scarring, vesicoureteral reflux, multicystic dysplastic kidney, ureteropelvic junction obstruction and posterior urethral valves. RESULTS: Hypertension is a recognized sequela of several common pediatric urological conditions. It is thought to be a direct consequence of renal damage or scarring often resulting from vesicoureteral reflux and/or febrile urinary tract infection. Multicystic dysplastic kidney has rarely been shown to cause hypertension when examined in large series rather than as isolated case reports. Ureteropelvic junction obstruction and posterior urethral valves have been linked to hypertension, although to our knowledge no series to date has specifically examined this relationship and smaller retrospective reviews seldom describe associated hypertension. CONCLUSIONS: Several pediatric urological conditions are known to cause hypertension. However, methodological flaws in the literature, including a lack of standardized blood pressure followup, highly variable patient populations and poor control of concomitant urological abnormalities, greatly limit the ability to establish a causative link between any 1 specific condition and hypertension. Early diagnosis, the prevention of infection, close clinical followup and early intervention remain the primary means of preventing pediatric urological causes of hypertension.     

Endoscopic treatment of vesicoureteral reflux in children with posterior urethral valves

Purpose

We reviewed the clinical outcome of endoscopic injection therapy in children with vesicoureteral reflux persisting after posterior urethral valve ablation.

Methods

We retrospectively reviewed the charts of 16 patients with posterior urethral valves who have undergone endoscopic injection to correct persistent reflux after successful relief of urethral obstruction. Breakthrough urinary tract infections, persistent high-grade reflux and failed ureteroneocystostomy were the indications of endoscopic antireflux surgery.

Results

Reflux was grade I in 1, grade II in 3, grade III in 11 and grade IV in 4 ureters. Mean age at injection was 6.9?±?3.8?years and the mean interval from initial intervention to injection was 4.3?±?2.4?years. Injected material was dextranomer/hyaluronic acid in the majority (87.5?%) of cases. Reflux was resolved or downgraded in 12 ureters (63.1?%) after a single injection. All failed cases had urodynamically documented bladder dysfunction.

Conclusion

More than half of the patients with vesicoureteral reflux, persisting after initial valve ablation, showed complete resolution or significant downgrading in their reflux grade after endoscopic injection. Given the technical difficulties and potential complications of open surgical reimplantation in valve patients, endoscopic subureteral injection can be considered as an effective alternative to cure persistent vesicoureteral reflux.