Bilateral Renal Dysplasia Research Articles

Distinguishing Features of Childhood Renal Dysplasia

Abstract Background Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia. Methods All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK). Results A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5–240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2–173) mg/m2/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5–208) months, including 12 renal transplant patients. Conclusions As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.

Bilateral renal dysplasia in a ferret (Mustela putorius furo)

AbstractRenal dysplasia is a histological diagnosis characterised by disorganised architecture of the renal cortex and medulla with disrupted nephron differentiation and primitive ducts surrounded by collarettes of the mesenchymal tissue. This condition has been reported once previously in ferrets as a component of multiple congenital abnormalities of the urogenital tract. A 1.5‐year‐old, male, neutered ferret was presented after being found minimally responsive under a fallen shelf in its enclosure. The ferret was obtunded, with mild intermittent muscle tremors and severe dehydration. Survey radiographs, including thoracic and abdominal ventrodorsal and right and left lateral views, were performed to assess for general trauma. The right renal silhouette was normal in size, and the left was moderately reduced. The capsular margins of both renal silhouettes were moderately undulating and mildly indistinct with perirenal retroperitoneal striations. No signs of trauma or other abnormalities were identified. Whole blood biochemical analysis demonstrated elevated creatinine, elevated blood urea nitrogen, hypernatraemia, hypercalcaemia, hyperphosphataemia and hyperproteinemia characterised by hyperalbuminemia and haemoconcentration. The hypernatraemia and mentation failed to improve with intravenous fluid therapy, and the ferret was euthanased. Bilateral renal dysplasia was confirmed via gross postmortem examination and histopathology.

Survival of Infants With Severe Congenital Kidney Disease After ECMO and Kidney Support Therapy.

Congenital kidney failure not only affects the homeostatic functions of the kidney, but also affects neonatal respiratory integrity. Until recently, extracorporeal membrane oxygenation (ECMO) support was not used in this population because the need for ECMO clearly established nonviability. Since 2016, 31 neonates have been admitted to the NICU at Children's of Alabama with congenital kidney failure. Five patients were placed on ECMO for severe respiratory distress unresponsive to conventional interventions. We evaluated neonates with congenital kidney failure and pulmonary hypoplasia/hypertension refractory to conventional therapies who received ECMO support within the first 9 postnatal days. We describe the pre and postnatal diagnoses, ECMO course details, dialysis modalities, complications, procedures, and long-term outcomes of these patients. All 5 patients received kidney support therapy by postnatal day 7. Diagnoses included posterior urethral valves, bilateral renal dysplasia, and autosomal recessive polycystic kidney disease. Gestational age ranged from 35.6 to 37.1 weeks. Birth weight ranged from 2740 to 3140 g. Days on ECMO ranged from 4 to 23. Four survived and are living today. Pulmonary hypertension resolved in surviving patients. Three surviving patients require no oxygen support, and 1 patient requires nocturnal oxygen. Three survivors received a kidney transplant, and 1 awaits transplant evaluation. Patients with congenital kidney failure with severe pulmonary hypoplasia/pulmonary hypertension no longer warrant a reflexive assignment of nonviability. Meticulous ECMO, respiratory, nutritional, and kidney support therapies may achieve a favorable long-term outcome. Further investigation of strategies for optimal outcome is needed.

Concurrent polycystic liver disease and bilateral renal dysplasia in a 20‐week‐old Great Dane

AbstractA 20‐week‐old, unneutered, female Great Dane was evaluated for failure to thrive and anorexia after 4 weeks of adequate caloric provision. Initial bloodwork results indicated elevated liver enzymes, azotaemia, hyperphosphataemia and non‐regenerative anaemia. Abdominal radiographs following a 24‐hour fast revealed hepatomegaly. Additional bloodwork was submitted to screen for possible infectious and immune‐mediated causes, of which none were discovered. The patient was referred to an American Veterinary College board‐certified internal medicine specialist for abdominal ultrasound, whose diagnostic findings were consistent with polycystic liver disease and bilateral renal dysplasia. Due to the grim prognosis provided by the ultrasonographic findings, the owners elected to euthanase the patient. To the authors’ knowledge, this is not only the first reported case of polycystic liver disease in a Great Dane, but its concurrence with bilateral renal dysplasia has also never been reported in any species.

A Central Role of Telomere Dysfunction in the Formation of a Unique Translocation within the Sub-Telomere Region Resulting in Duplication and Partial Trisomy

Telomeres play a major role in maintaining genome stability and integrity. Putative involvement of telomere dysfunction in the formation of various types of chromosomal aberrations is an area of active research. Here, we report a case of a six-month-old boy with a chromosomal gain encompassing the 11q22.3q25 region identified by SNP array analysis. The size of the duplication is 26.7 Mb and contains 170 genes (OMIM). The duplication results in partial trisomy of the region in question with clinical consequences, including bilateral renal dysplasia, delayed development, and a heart defect. Moreover, the karyotype determined by R-banding and chromosome painting as well as by hybridization with specific sub-telomere probes revealed the presence of an unbalanced t(9;11)(p24;q22.3) translocation with a unique breakpoint involving the sub-telomere region of the short arm of chromosome 9. The karyotypes of the parents were normal. Telomere integrity in circulating lymphocytes from the child and from his parents was assessed using an automated high-throughput method based on fluorescence in situ hybridization (FISH) with telomere- and centromere-specific PNA probes followed by M-FISH multicolor karyotyping. Very short telomeres, as well as an increased frequency of telomere loss and formation of telomere doublets, were detected in the child’s cells. Interestingly, similar telomere profiles were found in the circulating lymphocytes of the father. Moreover, an assessment of clonal telomere aberrations identified chromosomes 9 and 11 with particularly high frequencies of such aberrations. These findings strongly suggest that telomere dysfunction plays a central role in the formation of this specific unbalanced chromosome rearrangement via chromosome end-to-end fusion and breakage–fusion–bridge cycles.

A Term Neonate with Multiorgan Dysfunction, Severe Metabolic Acidosis, and Hyperkalemia.

A Term Neonate with Multiorgan Dysfunction, Severe Metabolic Acidosis, and Hyperkalemia.

Congenital bilateral renal dysplasia in a Cavalier King Charles Spaniel

AbstractThis report describes a case of congenital bilateral renal dysplasia in a Cavalier King Charles Spaniel (CKCS) puppy, which has not been previously reported in the breed. An 8‐week‐old male intact CKCS was presented to a University Teaching Hospital for the evaluation of vomiting and lab work revealed azotemia. The puppy was treated with medical management involving fluid therapy, antibiotics, and supportive care. Due to the development of unresponsive oliguric renal failure, puppy's level of care intensified and peritoneal dialysis was initiated and continued over a 12‐day course. After initiating peritoneal dialysis, azotemia improved but the patient acutely declined after an episode of regurgitation and ultimately cardiopulmonary arrest. Postmortem examination revealed bilateral persistent fetal glomeruli, tubular dysplasia, and rare tubular necrosis. While congenital renal dysplasia has been reported in other canine breeds, to the authors’ knowledge, this is the first report of congenital renal dysplasia in a CKCS.

MP44-19 PRENATAL AND EARLY POSTNATAL OUTCOMES FOR FETUSES WITH ANATOMIC OR FUNCTIONAL RENAL AGENESIS

You have accessJournal of UrologyPediatric Urology II (MP44)1 Sep 2021MP44-19 PRENATAL AND EARLY POSTNATAL OUTCOMES FOR FETUSES WITH ANATOMIC OR FUNCTIONAL RENAL AGENESIS Eniola Ogundipe, Nicholas Behrendt, Colton Leavitt, Regina Reynolds, and Vijaya Vemulakonda Eniola OgundipeEniola Ogundipe More articles by this author , Nicholas BehrendtNicholas Behrendt More articles by this author , Colton LeavittColton Leavitt More articles by this author , Regina ReynoldsRegina Reynolds More articles by this author , and Vijaya VemulakondaVijaya Vemulakonda More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002065.19AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: With the advent of novel fetal interventions, including amnioinfusion, there has been increased interest in fetal intervention for previously “lethal” anomalies such as bilateral renal agenesis or other CAKUT diagnoses associated with in utero renal failure (functional renal agenesis). While there have been rare reports of successful births following intervention in these cases, there is a paucity of data regarding the risks, benefits, and outcomes of intervention. To address this gap, we reviewed our experience with fetal intervention for anatomic or functional renal agenesis. METHODS: A retrospective review was conducted for patients referred to the Colorado Fetal Care Center (CFCC) for evaluation of complex CAKUT anomalies. Patients were included if they had severe oligohydramnios associated with bilateral renal agenesis, renal dysplasia, or other etiologies consistent with primary renal failure. Eligibility for amnioinfusion was determined by a multidisciplinary team including social work and psychology. Data collected included patient demographics, eligibility for fetal intervention, survival to birth, ventilatory support, survival to dialysis, and 30-day mortality. RESULTS: 14 patients met eligibility criteria. Of these, 50% were deemed eligible for serial amnioinfusion. Age did not differ between eligible and ineligible patients (mean 29 years). 86% (6/7) of eligible patients identified as white compared to 57% (4/7) of ineligible patients. 86% (6/7) of eligible patients were married, while 43% (3/7) of those ineligible were married.Postnatal data was available for 5/7 eligible cases and 2/7 ineligible cases. In the eligible group, respiratory intervention occurred in 3/5 cases. 2/5 cases survived to dialysis. In the ineligible group, respiratory intervention occurred in ½ cases. 0/2 cases survived to dialysis. 30-day mortality for the eligible group was 60% (3/5) compared to 100% (2/2) for the ineligible group. CONCLUSIONS: Individuals carrying a pregnancy complicated by anatomic or functional renal agenesis face a difficult choice when considering intervention. In this small cohort, patients considered eligible were more likely to be white and married, suggesting potential implicit bias or other confounding social variables. While these diagnoses are considered lethal, intervention is associated with significant morbidity and mortality. These findings reinforce that treatment of these cases should be considered experimental and large-scale multicenter trials are needed to determine the optimal indications for prenatal intervention. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e800-e800 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Eniola Ogundipe More articles by this author Nicholas Behrendt More articles by this author Colton Leavitt More articles by this author Regina Reynolds More articles by this author Vijaya Vemulakonda More articles by this author Expand All Advertisement Loading ...

Homozygous WNT9B variants in two families with bilateral renal agenesis/hypoplasia/dysplasia.

WNT9B plays a key role in the development of the mammalian urogenital system. It is essential for the induction of mesonephric and metanephric tubules, the regulation of renal tubule morphogenesis, and the regulation of renal progenitor cell expansion and differentiation. To our knowledge, WNT9B has not been associated with renal defects in humans; however, WNT9B-/- mice have renal agenesis/hypoplasia and reproductive tract abnormalities. We report four individuals from two unrelated consanguineous families with bilateral renal agenesis/hypoplasia/dysplasia and homozygous variants in WNT9B. The proband from Family 1 has bilateral renal cystic dysplasia and chronic kidney disease. He has two deceased siblings who presented with bilateral renal hypoplasia/agenesis. The three affected family members were homozygous for a missense variant in WNT9B (NM_003396.2: c.949G>A/p.(Gly317Arg)). The proband from Family 2 has renal hypoplasia/dysplasia, chronic kidney disease, and is homozygous for a nonsense variant in WNT9B (NM_003396.2: c.11dupC/p.(Pro5Alafs*52)). Two of her siblings died in the neonatal period, one confirmed to be in the context of oligohydramnios. The proband's unaffected brother is also homozygous for the nonsense variant in WNT9B, suggesting nonpenetrance. We propose a novel association of WNT9B and renal anomalies in humans. Further study is needed to delineate the contribution of WNT9B to genitourinary anomalies in humans.

Elevated sweat chloride test: is it always cystic fibrosis?

BackgroundThe sweat chloride test (ST) is the gold standard for cystic fibrosis (CF) diagnosis in symptomatic patients, within the newborn screening and in the follow-up of CF patients during molecular therapies. However, false positives have been reported in patients with different diseases. We describe and discuss 4 cases due to different clinical conditions in which we recorded false positive ST, and the test remained altered for a period of varying length.Cases presentationCase 1: Eight months old female child suffering from constipation, recurrent vomiting and failure to thrive, family history of recurrent pancreatitis without mutations in the PRSS1 and SPINK1 genes. Both ST and fecal elastase were altered although no CFTR gene mutations were found. Due to rapid clinical deterioration, celiac disease was suspected and diagnosed by laboratory tests and intestinal biopsy. After 2 weeks of gluten-free diet ST and fecal elastase normalized.Case 2: 14 months old male suffering from bilateral renal dysplasia, episodes of metabolic alkalosis, recurrent respiratory infections and recurrent vomiting. The child had more ST positives, but no CFTR mutations were found. During follow-up, he developed sensorineural hearing loss and an atrial septic defect was found. Finally, a diagnosis of Klinefelter was made, but the ST normalized several years later.Case 3 and 4: Two boys with stubborn constipation and fecal occlusion treated with Poly Ethylene Glycol (PEG) with salts showed pathological ST. The test returned normal a few days after stopping treatment.ConclusionsWe hypotesized the possible causes of ST alteration in these conditions: in celiac disease it could be due to a transient dysregulation of the aquaporins, rapidly reversed by the diet; in Klinefelter, it may be due to stable pubertal hypoandrogenism; while, the PEG formulation itself contains salts that can temporarily alter ST.

Bladder Agenesis and Bilateral Ectopic Ureters in an Infant Male With Cystic Renal Dysplasia, Imperforate Anus, and Penoscrotal Transposition

Bladder agenesis is a rare congenital anomaly infrequently reported in the literature, with an incidence of 1/600,000 patients.1 Commonly associated with other fatal malformations, the condition is often incompatible with life.2 Prior reports estimate that over 90% of living children born with this malformation are female, owing to renal preservation resulting from low pressure drainage of urine into the vagina, uterus, and vestibule.3,4 Herein we report a rare case of an infant male born with penoscrotal transposition and end stage renal disease secondary to bilateral cystic renal dysplasia found to have concurrent bladder agenesis and bilateral ureteral ectopia.

Prevalence of asymptomatic phase of renal disease in slum school children

Routine use of diagnostic ultrasound in pregnancy has led to the detection of urologic abnormality in many fetuses. The most common abnormality detected is hydronephrosis caused by the P.U.J. obstruction followed by multicystic dysplastic kidney [4]. Bilateral renal dysplasia or agenesis may be associate with oligohydramnios. However antenatal diagnosis of posterior urethral valves may be missed unless a scan is undertaken late in pregnancy. A cross sectional study was conducted on 500 school going children of both sexes. An informed consent was obtained followed by a visit to the institution. Children were subjected to physical examination, blood pressure recording and urine was collected and further analyzed. Culture repeated on cases with significant have shown the growth of same organism in 7 cases. Thus asymptomatic bacteriuria was taken as 1.4%.E. coli (42.8%) was the most common isolated organism followed by Klebsiella (28.5%).

Resistant hypertension: drug-eluting balloon for revascularization of bilateral renal fibromuscular dysplasia.

Resistant hypertension: drug-eluting balloon for revascularization of bilateral renal fibromuscular dysplasia.

Histopathological study of nephrectomy specimens in a tertiary care hospital

Histopathological study of nephrectomy specimens in a tertiary care hospital - JDPO- Print ISSN No: - 2581-3714 Online ISSN No:- 2581-3706 Article DOI No:- 10.18231/2581-3706.2019.0007, IP Journal of Diagnostic Pathology and Oncology-IP J Diagn Pathol Oncol

Renal manifestations in children with Alagille syndrome.

Alagille syndrome (AS) is a cholestatic disease secondary to scarcity of interlobular bile ducts. It is associated with extrahepatic manifestations, and renal involvement is frequent. To describe the prevalence, type and outcome of renal pathology in children with AS. The presence and outcome of renal pathology was retrospectively studied in 21 children who met AS criteria. Renal pathology was observed in 18 patients (85.7%): (1) ultrasound variations in 7 patients (6 cases of bilateral renal dysplasia and 1 case of renal agenesis); (2) distal renal tubular acidosis in 2 patients; (3) a drop in glomerular filtration and/or proteinuria in 16 patients. The frequency of a drop in glomerular filtration was similar between patients with and without pathological kidney ultrasound findings. Our study confirms a high prevalence of renal involvement, which enhances the importance of diagnosis and renal function follow-up in children with AS.

Meckel Gruber syndrome: A case report with review of literature

Meckel-Gruber syndrome, which is firstly described by Friedrich Meckel and Georg B. Gruber, is an autosomal recessive disorder that is characterized triad of occipital encephalocele, bilateral renal dysplasia and polydactyly. Because of the genetic heterogeneity, multiple organs can be affected. The worldwide incidence varies from 1 in 13,250 to 1 in 140,000 live births. We have reported a male baby born at 37-week gestation, who has occipital encephalocele, polydactyly and cystic dysplasia of both kidneys. As a result of these clinical findings, Meckel-Gruber Syndrome is suspected for his baby. This study is presented to draw attention to the Meckel Gruber Syndrome which has high risk of recurrence and early diagnosis by ultrasonographic screening can be confident.

Impact of Adjuvant Urinary Diversion versus Valve Ablation Alone on Progression from Chronic to End Stage Renal Disease in Posterior Urethral Valves: A Single Institution 15-Year Time-to-Event Analysis

Long-term progression to end stage renal disease of valve ablation alone vs ablation followed by additional urinary diversion were compared among children with stage 3 chronic kidney disease due to posterior urethral valves. We performed a retrospective study of children with posterior urethral valves and stage 3 chronic kidney disease treated at a single institution between 1986 and 2011. The 3 treatment groups were classified as group 1-valve ablation alone, group 2-ablation plus subsequent vesicostomy and group 3-ablation followed by ureterostomies and/or pyelostomies. Baseline demographic characteristics were analyzed. Statistical analyses compared the incidence of time to end stage renal disease among the intervention groups using the Fisher-Freeman-Halton exact test and Kaplan-Meier analysis with the log rank test. Cox regression was used to determine predictors of end stage renal disease progression. A total of 40 eligible patients were included in the study (group 1-14 patients, group 2-13 patients, group 3-13 patients). Baseline characteristics and post-intervention estimated glomerular filtration rate revealed no significant between-group differences. A statistically significant difference in progression to end stage renal disease was noted within 1 year after diagnosis of stage 3 chronic kidney disease among the treatment groups (log rank test p=0.02). However, cumulative end stage renal disease incidence at 15-year followup showed no statistical difference (log rank test p=0.628). Cox regression analysis determined that bilateral renal dysplasia (HR 2.76, 95% CI 1.21-6.30) and estimated glomerular filtration rate 60 ml/minute/1.73 m2 or greater after intervention (HR 0.23, 95% CI 0.09-0.61) were predictive of the likelihood of progression to end stage renal disease. Urinary diversion following valve ablation in children with stage 3 chronic kidney disease associated with posterior urethral valves may temporarily delay progression to end stage renal disease. However, no long-term benefit was noted from diversion in the ultimate incidence of end stage renal disease, suggesting that these interventions should be seen as a temporizing measure. Bilateral renal dysplasia and post-intervention estimated glomerular filtration rate are independent variables predicting overall chronic kidney disease progression.

Urethral dysontogenic metaplasia in cat with bilateral renal dysplasia

This paper to describe a case of dysontogenic urethral metaplasia in a one month old mongrel feline who also had bilateral renal dysplasia. Dysontogenic metaplasia in cats are scarce and this change may be associated with renal dysplasia and/or lower urinary tract. The animal had history of abdominal enlargement since birth and dysuria, eliminating urine only dropwise. Due to the poor prognosis we opted for euthanasia. At necropsy was observed enlarged and distended bladder, reduced kidneys and dilated and tortuous ureters. The urethra was thickened, hard to cut, and histologically, was replacing the connective tissue, cartilage and endochondral ossification areas, which features dysontogenic metaplasia. Both kidneys presented primitive appearance featuring dysplasia. Dysontogenic metaplasia in urinary tract feline with renal dysplasia, has not been described.

Clinical phenotypes of hepatocyte nuclear factor 1 homeobox b-associated disease

Objective: Hepatocyte nuclear factor 1 homeobox b (HNF1B) -associated disease is an autosomal dominant inherited disorder with a variable, multi-systemic phenotype. In China, five adult probands and one child proband with HNF1B-associated disease had been reported, whereas few fetuses are described. The aims of this retrospective study were to understand about the clinical manifestations of HNF1B-associated disease and to further improve the recognition of this disorder. Method: Four patients (3 males, 1 female) and three fetuses with HNF1B mutations were included in this study. They were admitted to our hospital from January 2013 to March 2017. HNF1B mutations were detected using targeted next generation sequencing and quantitative real-time PCR or Sanger sequencing. HNF1B heterozygous deletion of exons 1-9 was found in 4 patients and 2 fetuses, and HNF1B heterozygous missense mutation in 1 fetus. These two mutations had been reported. Two patients and 1 fetus had de novo mutations. Results of renal ultrasonography with or without magnetic resonance imaging, biochemical investigations, urine routine examination and other necessary investigations in 7 cases were analyzed. Result: Three patients were Han Chinese ethnicity, and one patient was Mongolian. In patients 1 and 4, abnormal fetal kidneys were discovered by routine ultrasonography, and the age at first feature identified in Patients 2 and 3 were 13 years and 28 years. Patient 3 had normal renal function and the remainder had reduced glomerular filtration rate. In addition, patient 4 presented with nephrotic syndrome and glycosuria, patient 2 with early onset hyperparathyroidism and renal osteodystrophy, and patient 3 with diabetes mellitus. All the 4 patients had renal structural abnormalities including bilateral multiple renal cysts, dysplasia and hyperechogenic kidneys. Only patient 3 had a positive family history of renal diseases, the remainder had a negative family history of renal diseases. In 3 fetuses, prenatal ultrasound anomalies were detected during the second trimester. These 3 fetuses had hyperechogenic kidneys with or without renal cysts. Polyhydramnios was detected in only one of the 3 fetuses. Two of the 3 fetuses had a positive family history of renal diseases. Conclusion: Clinical phenotypes of HNF1B-related disease are heterogeneous, renal malformations clearly appear to be the most common manifestation, multiple renal cysts are characteristic, and patients can progress to impaired kidney function during childhood; HNF1B mutation is a differential diagnosis of fetal hyperechogenic kidneys or multiple renal cysts.

Intravesical ureteric reimplantation for primary obstructed megaureter in infants under 1 year of age

This study aimed to assess the outcome of intravesical ureteric reimplantation for primary obstructed megaureter (POM) performed in infants under 1 year of age. A retrospective cohort study was carried out of all infants undergoing intravesical ureteric reimplantation surgery for POM at two pediatric centers in the 12 years between 2003 and 2014. Outcomes were compared with those of children aged over 1 year undergoing intravesical reimplantation for POM during this period. Follow-up was until November 2015. Thirty-seven megaureters in 34 infants were reimplanted, at median age 5.2 months (Table). Operative success was 97%, with one out of 34 patients (3%) requiring reoperation for recurrent ureteric obstruction. Renal preservation was high. One patient of 34 (3%), who had bilateral congenital renal dysplasia, showed evidence of decline in renal function. Five patients of 34 (15%) had postoperative urinary tract infections (UTIs). Four (13%) of those toilet trained had some symptoms of lower urinary tract dysfunction (LUTD). Three patients (9%) who had congenitally dysplastic kidneys developed hypertension. Comparatively, 14 children with POM underwent reimplantation over 1 year of age. Operative success was 86%, with two children requiring reoperation for recurrent stenosis. Both had evidence of bladder dysfunction before surgery, and thickened bladder walls noted during surgery. One of 14 (7%) had a decline in ipsilateral renal function after recurrent stenosis. Two (14%) had postoperative UTI. Three (25%) had evidence of LUTD. There are few data about feasibility and long-term outcomes specifically of ureteric reimplantation for POM in infancy. This is the first study to document long-term outcomes of intravesical ureteric reimplantation in infancy for POM. The recently published British Association of Paediatric Urologists consensus statement supports delaying definitive reimplantation surgery for POM that requires intervention until after 1 year of age. There is little evidence presented for this recommendation. Our study confirms the safety and feasibility of intravesical ureteric reimplantation for POM in infants under age 1, with an operative success rate of 97%, and high renal function preservation rate. UTI risk improved after surgery in children >6 months old and remained stably low in those <6 months of age. Postoperative rates of LUTD were low. Hypertension occurred in only three cases with congenital renal dysplasia. Ureteric reimplantation in infants under 1 year of age is a safe and effective option for managing POM that requires intervention, and compares favorably with those undergoing reimplantation over 1 year of age.