Surgical management of complete ureteric duplication abnormalities

In 2 decades (1974–1993), the senior author (S.A.) managed 148 patients with various abnormalities associated with complete ureteric duplication. Included were 72 patients with primary vesicoureteric reflux, 50 with ureteroceles, and 26 with upper-pole ectopic ureters. The majority of the patients were female, and the common clinical presentations included urinary tract infection (UTI), UTI with septicemia, and urinary incontinence. Ten cases were diagnosed after recognition of a renal abnormality on prenatal ultrasonography, an avenue that has provided new challenges, new opportunities, and new dilemmas. This review article is based on the authors' experience together with an analysis of current emphasis on early diagnosis, minimal surgery, and maximum preservation of renal function. Accepted: 15 October 1996     

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.     

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     

Prognosis of antenatally diagnosed oligohydramnios of renal origin

Although clinical outcome data on fetuses with oligohydramnios of renal origin are scarce, prognosis is regarded as poor due to a high risk of renal dysfunction and pulmonary hypoplasia. This review aims to summarize the current knowledge and clinical experience with patients presenting antenatally with renal oligohydramnios. By reviewing the underlying mechanisms, complications, and outcome data, we hope to further improve antenatal counseling and postnatal care. We conclude that prognosis of ROH has changed in recent years. While early data before the introduction of sophisticated neonatal intensive care and renal replacement therapy indicated a poor prognosis, nowadays, a much more optimistic prognosis of children after renal oligohydramnios can be expected from the recent publications in the literature.     

The long-term outcome of antenatal hydronephrosis up to 15 millimetres justifies a noninvasive postnatal follow-up

AIM: To assess the incidence of urinary tract infections (UTIs) and surgery in infants with different grades of antenatal hydronephrosis (ANH) and to evaluate incidence, severity and course of underlying vesicoureteral reflux (VUR). METHODS: Retrospective data of 125 infants with ANH were collected. The patients were divided into two groups according to the anterior-posterior pelvis diameter: group I, 5-14 mm and group II, > or =15 mm. RESULTS: UTIs developed in 4 of 106 infants from group I and 5 of 19 infants from group II. Surgical interventions were performed on 1 of 106 patients of group I and 7 of 19 patients of group II. These differences were statistically significant (p-values 0.004 and <0.001, respectively). In group I, 6 of 106 patients had VUR; none of them required surgical intervention and only two developed a UTI (one of whom also had contralateral ureteropelvic junction obstruction). Five of 19 infants in group II had underlying VUR, four of them with associated anomalies, 1 infant required surgical correction and 4 developed UTIs. CONCLUSION: Infants with ANH up to 15 mm have a low incidence of UTIs and surgery and a low incidence and benign course of underlying VUR. Therefore, noninvasive postnatal follow-up is justified and standard voiding cystourethrography should not be performed, but only in cases of ureteric dilatation.     

Crossed renal ectopia in children

Five cases of crossed ectopic kidneys are presented, four in males and one in female, four fused and one unfused. All were on the right side; in four cases the kidneys were positioned inferiorly and in one superiorly. Urological malformations and accompanying anomalies were observed in most of our cases. Vesicoureteral reflux was demonstrated in the three children in whom cystography was performed. In the presence of anorectal malformations and vertebral anomalies or on diagnosis of crossed ectopic kidneys, early and thourough urological investigation is indicated.     

Renal ultrasound in the neonatal period

Ultrasound was used for imaging the kidneys in 55 neonates. The normal kidney in a neonate is characterized by prominent medulla and fetal lobulation, the main renal vessels are frequently demonstrated. Of 29 infants with normal kidneys by ultrasound, 4 had renal disease (3 acute tubular necrosis, one partial renal artery thrombosis) and one had a pelvic kidney. In 24 infants congenital abnormalities or acquired renal disease was diagnosed. Multicystic dysplastic kidney and hydronephrosis were the most frequently observed abnormalities. Polycystic kidneys at the early stage (both adult type and infantile) appeared as enlarged hyperechoic kidneys. In 2 neonates the kidneys were normal but they had adrenal mass lesions.Based on the paper presented at the 50th Annual Meeting of the American Academy of Pediatrics, Detroit, Michigan, October 1980     

Clinical features of unilateral multicystic renal dysplasia in children

A clinical study of 204 patients with unilateral multicystic renal dysplasia referred to 30 nephrology departments was undertaken to assess the frequency of complications in children who underwent nephrectomy (n=40) versus those who were treated conservatively (n=164). Six patients required antihypertensive treatment, 30 (13% of girls, 17% of boys) had at least one episode of urinary tract infection. The number of clinical complications did not differ in patients who underwent nephrectomy in comparison to those who did not. The dysplastic kidney decreased in size in 65% of kidneys with repeated ultrasound values; no change occurred in 16%, while an increase in maximal diameter was observed in 19%. Contralateral kidney length of more than 2 standard deviation scores (SDS) was seen in 30% of patients. There is evidence for a slight impairment of renal function in the whole study group given by a median of serum creatinine level of 0.63 SDS in all patients available for analysis. Among those 35 patients with contralateral anomalies (mainly obstructive changes and vesico-ureteral reflux), all 3 patients with contralateral changes suggestive of hypoplasia and 22% with obstruction, but only 1/7 (14%) with reflux showed elevated serum creatinine level >2 SDS. Conclusion Renal function seems to be slightly impaired in patients with unilateral multicystic renal dysplasia. The apparent tendency to regression of the dysplastic kidney and no difference in the number of complications justify a conservative management rather than operative intervention. Received: 9 May 1997 / Accepted in revised form: 16 February 1998     

儿童肾发育不良的诊治分析

目的总结儿童肾发育不良的临床特点，探讨合理的诊治方法。方法回顾性分析我院1997年6月2006年6月间66例肾发育不良患儿的临床表现、影像学检查、治疗方法及预后情况。结果就诊年龄1个月～15．8岁，平均4．8岁，男20例，女46例，左侧38例，右侧28例，临床表现主要为正常分次排尿同时有排尿间滴尿，B超检查可以确定发育不良肾的位置、大小、形态，以及合并的其他畸形，静脉肾盂造影（IVP）检查多表现为患侧肾不显影、患肾无功能，65例患儿接受了发育不良肾及输尿管切除术，1例患儿因患侧肾与对侧正常肾融合而接受了患侧输尿管切除术，其中经腹腔镜切除32例，术中见发育不良的肾脏的大小、形态和位置异常，术后绝大部分预后良好。结论超声结合静脉尿路造影是诊断肾发育不良的有效影像学检查方法。对于有临床表现及体征的肾发育不良的患儿，肾和输尿管切除术是有效的治疗方法。腹腔镜微创手术在肾发育不良的治疗中具有一定优势。     

Renal outcome of children with one functioning kidney from birth. A study of 99 patients and a review of the literature

In patients with a single functioning kidney, renal function was assessed at regular intervals over a period of 10 years. Serum creatinine, glomerular filtration rate (GFR), blood pressure, and urinary protein-creatinine ratio were assessed at the age of 2, 5 and 10 years. Between January 1980 and December 2005, 99 such patients were diagnosed in the first year of life. They were divided into three groups: A, patients with multicystic kidney disease and a normal contralateral kidney (n = 36); B, patients with a normal solitary kidney without uropathy (n = 20); and C, patients with obstructive uropathy and one nonfunctioning kidney (n = 43). Serum creatinine levels increased significantly with increasing age in every group. In group C, serum creatinine was significantly elevated compared with group A in all age categories (p = 0.043, p = 0.019, p = 0.001 respectively). Median figures of GFR remained within normal limits over the 10-year period. GFR was significantly lower in group C compared with group A (p = 0.001, p = 0.009, p = 0.019 respectively) and B in all age categories (p = 0.013, p = 0.002, p = 0.016 respectively). There were no changes in blood pressure over time and no differences among the three groups were observed. At the age of 10 years, the patients in group C had a significantly higher median urinary protein-creatinine ratio (p = 0.022) than those in groups A and B. There was also an increasing level of proteinuria with increasing age in group C (p = 0.002). In conclusion, renal function was stable over time in all patients, but children with obstructive uropathy have a lower median GFR and higher serum creatinine level for the whole study period. Hypertension was exceptionally observed in group C, with obstructive uropathy, as was an elevated urinary protein-creatinine ratio.     

Reflux nephropathy in the 1st year of life — the role of infection

The importance of infection and vesico-ureteric reflux in the development of reflux nephropathy is generally accepted. Widespread use of antenatal ultrasound scanning has identified dilated fetal urinary tracts, allowing prompt investigation and treatment in the early post-natal period; some of these children have vesico-ureteric reflux. This study compares renal parenchymal damage, demonstrated by uptake of technetium ^99m dimercaptosuccinic acid (DMSA) in two groups. The first included babies less than 6 months old who had presented with their first urinary tract infection and had vesico-ureteric reflux. The second included those in whom a dilated urinary tract had been identified at antenatal ultrasound and post-natal investigation revealed vesico-ureteric reflux, but who had no evidence of urinary tract infection. Renal damage was present in 68% of group 1 and 29% of group 2 when DMSA scanning was used to detect scarring (P = <0.05). Our results support the proposal that infection is a major factor in development of renal damage in patients with vesico-ureteric reflux. Identification by antenatal ultrasound scanning allows early therapeutic intervention before renal damage occurs. Offprint requests to: D. C. S. Gough     

Postnatal management of isolated mild pelvic dilatation detected in antenatal period

AIM: Mild antenatal renal pelvic dilatation (ARPD) revealed by prenatal ultrasound (US) raises the question whether or not screening for vesicoureteral reflux (VUR) is mandatory. The aim of our study was to suggest guidelines for postnatal management of infants with mild ARPD defined as an antero-posterior (AP) dilatation >5 and <10 mm. METHOD: Therefore we assessed the value of postnatal US at day 30 to predict VUR, the incidence of VUR at day 30 and the rate of spontaneous resolution at 1 year. Two hundred (200) infants with ARPD were included and had renal US and voiding cystourethrography (VCUG) at day 30. If VUR was present, VCUG was repeated 1 year later. RESULTS: Incidence of VUR was 10% (20/200) at day 30 after birth and only 3% (6/200) 1 year later. VUR at day 30 was twice as frequent in children with postnatal dilatation (11%) than in nondilated kidneys (6%). CONCLUSIONS: Considering the low incidence of VUR at 1 year, screening for VUR in mild ARDP seems not to be justified. However follow-up by US to detect increase in dilatation and clinical monitoring for signs of urinary infection is required.     

The impact of perinatal urinoma formation on renal function: our experience and review of the literature

Introduction: A urinoma is a fluid mass consisting of extravasated urine in the perirenal space. Its impact on renal function was analysed. Methods: All cases of prenatal and neonatal urinoma reported in the literature were analysed as well as two cases recently observed in our department. Results: A review of all prenatally diagnosed urinomas revealed 25 cases, for a total of 30 renal units. The cause of obstruction was: lower urinary tract (LUT) obstruction in 16 cases (62.5%), upper urinary tract (UUT) obstruction in seven cases (29.5%) and unknown in two cases (8%). A preserved renal function was found in only 30% of all cases. Furthermore, prognosis for renal function was better in posterior urethral valve (PUV) patients than in uretero‐pelvic junction obstruction (UPJO) patients. In addition, we analysed 35 published cases of urinoma with a neonatal onset, without prenatal diagnosis, and secondary to LUT obstruction in 27 cases (77%), UUT obstruction in seven cases (20%), while the underlying cause was unknown in one cases (3%). A preserved renal function was observed in 84% of cases with UUT obstruction and in 80% of cases presenting a LUT obstruction. Conclusions: Prognosis concerning renal function seems to be mainly related to two factors: age at presentation and underlying diagnosis. Decompression of the urinary tract by urine extravasation produces the best results in terms of preservation of the renal function in LUT obstruction (75% in prenatal and 84% in postnatal cases); UUT obstruction however is associated with a good prognosis in the neonatal period (80% of preserved renal function) but with a severe impairment (only 20% of preserved renal function) in prenatally detected cases.     

Heminephrectomy by pelvicalyceal mucosal stripping of the dysplastic renal pole

A new technique of heminephrectomy by splitting the dysplastic pole in the longitudinal plane and stripping the mucosa from the pelvis and calyces using diathermy is described. By the use of this technique, inadvertent opening of the calyces of the normal hemikidney is avoided and bleeding is substantially reduced.