A renal aplasia case mimicking radiologically as unilateral renal agenesis in a child with spina bifida, atresia ani and unilateral undescended testis: a case report.

BACKGROUND: As a result of the failure of embryogenic kidney formation, a condition can occur where not a single kidney appears and this phenomenon is known as unilateral renal agenesis (URA). Both aplastic and dysplastic kidney are different from renal agenesis, atrophy and renal hypoplasia. However, from this case report it can be seen that there are similarities, both radiologically and macroscopically, between cases of unilateral renal aplasia and renal agenesis. CASE PRESENTATION: A 2 year old Javanese boy came to the health facility with complaints of recurrent fever and urinary tract symptoms such as dysuria and straining. Computerized Tomography (CT) scan of the abdomen and urography showed agenesis of the left kidney and a probable spina bifida. Cystourethrography examination was done and showed grade 5 voiding, then retrograde pyelography was performed with the diagnosis of unilateral renal agenesis was made because there was no visible left side collecting system even though there was a duplication in the left ureter. The next examination was carried out by histopathology and immunohistochemistry after resection of the left side of the ureter and the diagnosis increasingly pointed towards renal aplasia after primitive renal structures were found. CONCLUSIONS: Renal agenesis and aplastic kidney are difficult to differentiate macroscopically and radiologically. Nevertheless, from this case report, we try to provide some interesting points to differentiate cases of unilateral renal agenesis from Renal Dysplasia which presents as unilateral renal aplasia.

GFR measurements and ultrasound findings in 154 children with a congenital solitary functioning kidney.

BACKGROUND: Multicystic dysplastic kidney (MCDK) and unilateral renal agenesis (URA) are the most common reasons for a congenital solitary functioning kidney (SFK). We aimed to assess the presence of abnormalities in the congenital SFK and evaluate kidney function using chrome EDTA (CrEDTA) measurements. METHODS: We retrospectively reviewed the medical records of 154 children with MCDK and URA in the period from 2005 to 2022 to analyze results from ultrasound scans and CrEDTA glomerular filtration rate (GFR) examinations. RESULTS: Of 154 children with a solitary kidney due to MCDK (62%) or URA (38%), abnormalities on the congenital SFK were found in 13 children (8%). The abnormalities spontaneously resolved in 6 children (46%). The most common abnormality was hydronephrosis. Compensatory hypertrophy was found in 17% of the children within the first 6 months of life. 116 children (90%) had a standard GFR (sdGFR) above 75% of expected for the age. Out of those with a sdGFR below 75% of expected, 3 (23%) had abnormalities in the congenital SFK. There was no difference in sdGFR between children with MCDK and URA. CONCLUSIONS: Our study is the first using CrEDTA for GFR measurements and suggests that most children with a congenital SFK due to MCDK or URA have a kidney function within expected for the age. Compensatory hypertrophy of the SFK is found in a minority of children within the first six months of life, suggesting that this process is developing over time. The prevalence of abnormalities in the SFK seems low, however those with abnormalities (e.g. hydronephrosis) are at higher risk of reduced sdGFR.

Prevalence of reflux nephropathy in Iranian children with solitary kidney: results of a multi-center study.

BACKGROUND: Given the importance of the function of the remnant kidney in children with unilateral renal agenesis and the significance of timely diagnosis and treatment of reflux nephropathy to prevent further damage to the remaining kidney, we aimed to determine the prevalence of reflux nephropathy in this subgroup of pediatric patients. METHODS: In general, 274 children referred to pediatric nephrologists in different parts of Iran were evaluated, of whom 199 had solitary kidney and were included in this cross-sectional study. The reasons for referral included urinary tract infection (UTI), abnormal renal ultrasonography, being symptomatic, and incidental screening. Demographic characteristics, including age and gender were recorded. History of UTI and presence of vesicoureteral reflux (VUR) were evaluated. RESULTS: Of the 274 children evaluated in this study with the mean age (SD) of 4.71 (4.24) years, 199 (72.6%) had solitary kidney. Among these, 118 (59.3%) were male and 81 (60.7%) were female, 21.1% had a history of UTI, and VUR was present in 23.1%. The most common cause of referral was abnormal renal ultrasonography (40.2%), followed by incidental screening (21.1%), being symptomatic (14.1%), and UTI (5.5%). In 116 children (58.3%), the right kidneys and in 83 (41.7%) the left kidneys were absent. Besides, 14.6% of the participants had consanguineous parents and 3% had a family history of solitary kidney. Upon DMSA scan, the single kidney was scarred in 13.1%, of which only 7.5% were associated with VUR. In addition, proteinuria and hematuria were observed in 6.5% and 1.5% of children, respectively. CONCLUSIONS: The prevalence of reflux nephropathy was 7.5% in children with solitary kidney with a male predominance. Given the relatively high prevalence of reflux nephropathy in these children, screening for VUR in the remnant kidney appears to be essential in this population.

Management of locally advanced mesonephric carcinoma of the cervix in the setting of Mullerian Duct anomaly spectrum and unilateral renal agenesis: A case report and review of the literature.

Cervical mesonephric adenocarcinoma is a rare histologic cervical carcinoma variant arising from remnants of the mesonephric duct. Few clinical cases have been reported in the literature, and given the low rate of occurrence, the optimal management strategy is unknown. Most reported cases involve patients with either early stage (FIGO I) or metastatic disease. Herein, we report the only known case of locally advanced, node-positive cervical mesonephric carcinoma in a 55-year old woman with Mullerian duct anomaly of the uterus, obstructed hemivagina, and ipsilateral renal agenesis. To our knowledge, this would be the first case report with the concurrence of both rare entities. We review the treatment paradigm in this patient, and the literature, including radiotherapy and brachytherapy techniques.

Reducing Unnecessary Imaging in Children With Multicystic Dysplastic Kidney or Solitary Kidney.

BACKGROUND AND OBJECTIVES: Children with isolated unilateral multicystic dysplastic kidney (MCDK) or congenital solitary kidney (CSK) undergo serial renal ultrasonography with variable frequency until they are transitioned to adult care. A growing body of literature suggests the value of frequent ultrasonography in this population is limited, providing no benefit to overall outcomes. Despite emerging evidence, ultrasound remains overused, resulting in avoidable health care expenditures and unnecessary use of resources. With our initiative, we aimed to improve quality of care by reducing avoidable ultrasounds in these children. METHODS: This was a single-center, prospective, interrupted time series of children <18 years with ultrasound-confirmed isolated unilateral MCDK or CSK in the outpatient nephrology clinic to evaluate the effect of a decision-making algorithm on the proportion of children receiving an avoidable ultrasound. An algorithm depicting a consensus, evidence-based protocol for managing pediatric MCDK or CSK was refined through content expert feedback and usability testing to standardize frequency of ultrasonography. Ultrasounds were deemed necessary after birth, at 6 months, and at 2, 5, 10, and 15 years. Differences pre- and postintervention were determined by using a U chart and t and F tests for significance. RESULTS: The algorithm resulted in a 47% reduction (P < .001) in the proportion of avoidable ultrasounds ordered in children with MCDK and CSK. This reduction was sustainable over a 6-month period and would result in at least $46 000 annual savings. CONCLUSIONS: Introduction of a clinical decision-making algorithm was associated with a reduction in avoidable ultrasound testing. Improving adherence across providers may allow for an even more pronounced reduction.

Ex-Vivo Repair of Complex Hilar Renal Artery Aneurysms and Auto-Transplantation of Solitary Kidney.

Renal artery aneurysms (RAA) are rare and challenging to repair. We present a case of a 48-year-old female with solitary right kidney who had complex aneurysms near the renal hilum. CT angiogram showed fibromuscular dysplasia (FMD) features of the renal artery and 2 saccular aneurysms measuring 2.3 cm and 1 cm. An additional small lower pole renal artery added to the complexity of the case. Ex-vivo aneurysm repair was performed after the kidney was removed and flushed with preservation solution. This was followed by auto-transplantation to the right external iliac vessels. The patient did well postoperatively without need for dialysis with serum creatinine returning to normal 5 weeks after.

Renal length z-score for the detection of dysfunction in children with solitary functioning kidney.

AIM: To evaluate whether renal length z-scores predict renal dysfunction in children with a solitary functioning kidney (SFK). METHODS: In a single-centre retrospective cohort of children with SFK, we correlated body mass index z-scores, extracellular volume and lean body mass to renal length z-scores. We grouped these z-scores to other markers of renal dysfunction (proteinuria, hypertension, extracellular volume and abnormal estimated glomerular function rate [eGFR]) and analysed renal length z-score with multivariate analysis, receiver-operated characteristics (ROC) plots and Youden's index to determine an appropriate cut-off. RESULTS: 111 children had a median follow-up 5.08 years, eGFR 80.8 mL/min/1.73 m2 , and age at last follow-up 7.4 (3.8-13.4 years). The median renal length z-scores of those without any renal dysfunction (n = 37, 25.1%) were greater (+3.66, interquartile range 3.02-4.47) than those with renal dysfunction (median 3.11, interquartile range 1.76-4.11, P = .0107, Mann-Whitney test). Using a cut-off of z-score of >+1.911, the odds ratio for having no renal dysfunction was 0.07 (95% CI 0.002-0.459, P = .0010). However, accuracy of the renal length z-score was poor (ROC curve 0.6488). CONCLUSION: In this cohort of children with SKF, using the renal length z-score as a biomarker of renal dysfunction at 7 years of age is not recommended.

Reversible Electrochemotherapy (ECT) as a Treatment Option for Local RCC Recurrence in Solitary Kidney.

A 61-year-old female underwent a right radical nephrectomy and a left nephron sparing surgery in 2014 due to renal cell carcinoma. A renal cell carcinoma local recurrence, 28 mm in size, centrally located in the left kidney was treated using cryoablation in 2016. In November 2018, computed tomography (CT) scan showed three nodules (maximum size 15 × 11 mm) in the left kidney, and CT-guided needle biopsy was performed. For multifocal recurrence and the anatomical site of these three nodules, a simultaneous reversible electrochemotherapy treatment was performed in April 2019. At 6-month CT control, no evidence of residual disease was found. Electrochemotherapy could be used to treat locoregional renal cell carcinoma recurrence when other ablative techniques are not suitable. LEVEL OF EVIDENCE: Level 4, Case Report.

Cystic Dysplasia of the Rete Testis: Does Pathophysiology Guide Management?

Cystic dysplasia of the rete testis (CDT) is a rare, benign, cause of testicular mass in the pediatric population. The mass appears on sonography as multiple small cysts of varying size surrounded by normal or compressed testicular tissue. CDT is often associated with other genitourinary anomalies, commonly presenting with agenesis or dysplasia of the ipsilateral kidney. The pathophysiology and the management remains controversial. We report a case of a 3-year-old presenting with an enlarged testicular mass later presumed to be CDT associated with ipsilateral renal agenesis, review the literature, and propose an evaluation and management algorithm.

The comparison of the resistivity index values in the ultrasonographic evaluation of a unilateral atrophic/hypoplastic kidney.

Background: In the study, we aimed to determine the sensitivity of the renal resistivity index (RI) in differentiating hypoplastic and atrophic kidneys in patients with small-sized kidneys, and to evaluate its capacity to predict the renal involvement confirmed by the DMSA scintigraphy.Material and methods: We retrospectively reviewed the ultrasonography (US) and DMSA findings, and medical records of pediatric patients with unilateral diminutive kidneys followed between January 2017 and June 2018. The RI measurements were performed twice, and the mean RI was calculated for each kidney of all patients.Results: Sixty-three (male/female, m/f = 28/35) pediatric patients aged 107.2 ± 49.4 months (range 14-206 months) were included in this study. The DMSA scintigraphy revealed abnormal changes to atrophic kidneys in 38 patients and hypoplastic kidneys in 25. There were no differences between the groups with atrophy and hypoplasia by age, gender, urine density, and creatinine. The patient group with atrophic kidneys had a mean RI of 0.55 ± 0.21, and patients with hypoplastic kidneys had a mean RI of 0.67 ± 0.03. The mean RI and systolic/diastolic rates of the patients with atrophy were significantly lower than of the patients with hypoplastic kidneys (p = 0.042 and p = 0.048, respectively). There was a positive correlation between RI and DFR in the group with atrophy (r = 0.461, p = 0.016), but this was not the case for the group with hypoplastic kidneys (r= -0.066, p = 0.889).Conclusions: The resistivity index might be very useful for differentiating atrophy and hypoplasia in patients with unilateral small kidneys and can be used instead of scintigraphic evaluation.

Endovascular Stent-Graft Repair of a Large Renal Artery Pseudoaneurysm With Impending Rupture, Arising From a Solitary Kidney.

This was an unusual case of a large renal artery pseudoaneurysm with impending rupture, which was successfully treated with an endovascular stent-graft in a solitary kidney.

Primary seminal vesicle adenocarcinoma: a lethal yet cryptic malignancy with review of literature.

The rarity of primary seminal vesical adenocarcinoma (PSVA) coupled with mostly late and advanced presentation with high mortality makes it an unanticipated malignancy with poor prognosis. Although there has been sporadic reporting of cases, the dearth of literature makes standardised care a challenge. The detection has incorporated immunohistochemistry for establishing the site of origin as well as the differentiation of primary from metastatic cancer. Surgical management with seminal vesiculectomy continues to be the mainstay of treatment, but difficult anatomy and delayed intervention do lead to an increased chance of residual disease that may warrant further adjuvant chemoradiation. We present a case report where PSVA developed in a patient with Zinner syndrome-an observation that is extremely rare with a literature review of PSVA including the various aspects of management including contemporary diagnosis techniques.

Síndrome de Zinner en un paciente politraumatizado / Zinner's syndrome in a polytrauma patient

Objetivo: Reportar dos nuevos casos de Síndrome de Zinner, una variante anatómica poco prevalente y conocida, con riesgo de originar alteraciones. Método: Descripción de dos casos de Síndrome de Zinner presentados en nuestro centro, durante estudio por epididimitis aguda de repetición y tras traumatismo renal grado IV. Revisión de la literatura hasta la fecha, centrada en su diagnóstico y posibles complicaciones. Resultado: El manejo conservador del paciente politraumatizado permitió preservar el riñón, manteniendo una función renal normal. Es el primer caso de síndrome de Zinner diagnosticado en este contexto clínico. Conclusiones: El Síndrome de Zinner es una entidad poco frecuente y que pasa inadvertida en muchas ocasiones dada la escasa sintomatología que produce

Objective: To report two Zinner's syndrome cases, a rare anatomical variant with risk of complications. Methods: Analysis of two Zinner syndrome cases, detected during acute recurrent epididimytis and after a grade IV renal trauma. Review of the literature focusing on its diagnosis and possible complications. Results: The conservative management of the polytrauma patient allowed to preserve the kidney, while maintaining normal renal function. It is the first Zinner`s syndrome diagnosed in this clinical context. Conclusions: Zinner`s syndrome is a rare entity and often goes unnoticed because of the few symptoms it produces

Is renal scintigraphy really a necessity in the routine diagnosis of congenital solitary kidney?

BACKGROUND: For a definitive diagnosis of congenital solitary kidney, renal scintigraphy is suggested as being the gold standard of ruling out ectopic functioning renal tissue, possibly missed by ultrasound. The aim of our study was to test ultrasonography precision in comparison with renal scintigraphy on a larger cohort of congenital solitary kidneys. METHODS: We performed a retrospective unicenter study of children with congenital solitary kidney with no contralateral tissue, who were treated in the period from 1980 to 2017. The findings in children who underwent both abdominopelvic ultrasound and nuclear renal scintigraphy were compared and the accuracy of ultrasound was assessed. RESULTS: 99 children met the inclusion criteria of congenital solitary kidney confirmed with abdominopelvic ultrasound and nuclear renal scintigraphy. The children were predominantly male (61.6%), and the congenital solitary kidney was largely right-sided (55.5%). In 97 cases (98%), ultrasound correctly predicted the absence of functional renal tissue on one side in the renal fossa or in an ectopic location (pelvis or ipsilateral side). The calculated accuracy of abdominopelvic ultrasound in diagnosing congenital solitary kidney was therefore 98%. CONCLUSIONS: Our findings confirm that abdominopelvic ultrasound alone is accurate enough to diagnose congenital solitary kidney. It gives enough information for consideration if further radiological evaluation is still needed.

Solitary kidney and risk of chronic kidney disease.

The renal outcome of solitary kidney remains controversial. We examined the longitudinal association of congenital or acquired solitary kidney with the development of chronic kidney disease (CKD). A cohort study was performed involving 271,171 Korean men and women free of CKD at baseline who underwent a health screening program and who were followed annually or biennially for an average of 5.4 years. Solitary kidney was determined based on ultrasonographic findings. CKD was defined as an estimated glomerular filtration rate of < 60 ml/min/1.73 m2 and/or the presence of proteinuria in two or more consecutive visits. During 1,472,519.6 person-years of follow-up, 2989 participants developed CKD (incidence rate: 2.0 per 1000 person-years). After adjustment for potential confounders, the aHR (95% CIs) for incident CKD comparing solitary kidney to the control was 3.26 (1.63-6.54). In analyses of cause-specific solitary kidney, aHR (95% CIs) for CKD comparing unilateral nephrectomy and congenital solitary kidney to the control were 6.18 (2.31-16.49) and 2.22 (0.83-5.92), respectively. The association between solitary kidney and CKD was stronger in men. Having a solitary kidney was independently associated with an increased risk of CKD development. Therefore, preventive strategies for reducing the risk of CKD are required in individuals with a solitary kidney.

Unilateral Renal Agenesis Diagnosed on Early Prenatal Trans-Vaginal Scans.

BACKGROUND: Unilateral renal agenesis is a rare finding. There are no large-scale studies reporting this finding in early pregnancy. OBJECTIVES: To evaluate the incidence of unilateral renal agenesis (URA) and of associated anomalies diagnosed by early prenatal trans-vaginal sonography. METHODS: We performed a retrospective chart review of all 59,382 trans-vaginal scans performed at 14-16 weeks gestation by a single operator at different clinics during the period 1994-2013. RESULTS: The incidence of URA was 1:1212 (49/59382 cases). Associated anomalies were diagnosed in 22 cases (45%). Renal anomalies were diagnosed in 22.4%. Extra-renal anomalies (with exclusion of a single umbilical artery or nuchal edema as isolated findings) were diagnosed in 24.5%. Ambiguous genitalia were diagnosed in 8.2%. CONCLUSIONS: URA is a rare finding in early trans-vaginal sonography. Associated anomalies are very common and should be sought.

Congenital solitary kidney size at birth could predict reduced eGFR levels later in life.

OBJECTIVES: To evaluate the impact of congenital solitary functioning kidney (CSFK) length, measured early in life, on the eGFR levels during the follow-up. STUDY DESIGN: We retrospectively selected 162 CSFK patients undergoing, within 60 days of life, renal length (RL) measurement by ultrasound. We divided the population in: Group 1 = RL ≥ 2 standard deviation score (SDS). Group 2 = RL < 2 SDS and showing RL ≥ 2 SDS during the follow-up. Group 3 = RL < 2 SDS and showing RL < 2 SDS during the follow-up. PRIMARY OUTCOME: development of eGFR below the range of normality. RESULTS: The median follow-up period of the overall population was 6.2 years (range 2-21.5 years). The cumulative proportion of patients free of primary outcome at 15 years of age was 96.4% in group 1, 64.6% in group 2, and 45.6% in group 3 (p = 0.03). The RL > 2 SDS within 60 days of life was a significant protective factor (hazard ratio = 0.13; 95% C.I. 0.02-0.97) against development of primary outcome. CONCLUSION: RL ≥ 2 SDS within 60 days of life could identify a population of CSFK with reduced risk of presenting reduced eGFR levels later in life.