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.

Síndrome de cascanueces y agenesia renal derecha. Una rara asociación / Nutcracker Syndrome and Right Renal Agenesis. A Rare Association

Introducción: El síndrome de cascanueces se considera una anomalía infrecuente y poco pensada en la práctica médica; su incidencia no está bien establecida debido a su sintomatología variada. La agenesia renal unilateral se estima entre 1/2500 y 1/4000 nacidos vivos. Objetivos: Describir una paciente de 18 años con sospecha prenatal de agenesia renal derecha confirmada por imágenes después del nacimiento, que ingresó por proteinuria. Presentación del caso: Paciente de 18 años con agenesia renal derecha conocida, en la que un examen de orina detectó proteinuria desde los 13 años. En su estudio se clasificó como proteinuria ortostática y evolutivamente refirió dolor lumbar izquierdo ligero y transitorio. En el ultrasonido renal, realizado para valorar crecimiento del riñón único, se detectó dilatación de la vena renal izquierda. Se repitió el estudio ecográfico para precisar ángulo aorto-mesentérico y dilatación de vena renal izquierda, y se confirmó el síndrome de cascanueces. Conclusiones: La asociación entre agenesia renal derecha y síndrome de cascanueces, resulta extremadamente rara, y, cuando se presenta con proteinuria ortostática y dolor lumbar ocasional, debe seguirse en forma expectante, pero no se necesita tratamiento quirúrgico en la mayoría de los casos(AU)

Introduction: Nutcracker syndrome is considered an infrequent and poorly thought out anomaly in medical practice; its incidence is not well established due to its varied symptomatology. Unilateral renal agenesis is estimated to be between 1/2500 and 1/4000 live births. Objectives: To describe an 18-year-old female patient with suspected pre-natal imaging-confirmed right renal agenesis after birth, who was admitted due to proteinuria. Case presentation: An 18-year-old female patient with known right renal agenesis, in whom a urine test detected proteinuria from the age of 13. In the study it was classified as orthostatic proteinuria and evolutionarily she referred mild and transient left low back pain. Renal ultrasound, performed to assess single kidney growth, showed dilation of the left renal vein. The ultrasound study was repeated to specify aorto-mesenteric angle and left renal vein dilation, and nutcracker syndrome was confirmed. Conclusions: The association between right renal agenesis and nutcracker syndrome is extremely rare, and, when it presents with orthostatic proteinuria and occasional low back pain, it should be followed expectantly, but surgical treatment is not needed in most cases(AU)

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.

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.

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.

Management of Aortoiliac Aneurysms with Atypical Renal Artery Anatomy.

BACKGROUND: Renal artery anomalies occur at a rate of 1-2% and present a challenge to vascular surgeons performing aortic surgery. We describe adjuncts used to manage such anatomic variants. METHODS: A single surgeon registry of all abdominal aortic aneurysms repaired in an academic center was retrospectively reviewed. Patients with prior renal transplants, congenital pelvic kidneys, or horseshoe kidneys were included. Open repair was reserved for patients with no endovascular or hybrid repair options. RESULTS: Over an 8-year period, 18 patients were identified (renal transplant n = 9, horseshoe kidney n = 3, congenital pelvic kidney n = 6). All transplant patients were treated with endovascular repair. Four required cross-femoral bypasses, 1 for retrograde allograft perfusion after aorto-uni-iliac (AUI) procedure to the contralateral external iliac artery and 3 for contralateral limb perfusion after endograft extension into iliac artery ipsilateral to allograft. Three transplant patients required carotid access due to severe iliofemoral occlusive disease or allograft origin off the internal iliac artery. Two horseshoe kidney patients underwent open repair with direct reimplantation of accessory renal arteries, whereas 1 underwent endovascular repair with exclusion of an isthmus branch. Of the congenital single/pelvic kidney cohort, 2 underwent open repair with renal reimplantation, 2 underwent endovascular aneurysm repair, 1 was treated with AUI and cross-femoral bypass, and one was treated with a staged iliorenal bypass and subsequent fenestrated endovascular repair. Intravascular ultrasound was used to minimize contrast use in patients with chronic renal insufficiency (Cr > 1.5 mg/dL, n = 6). Over a mean follow-up of 31 months (range, 1-110), there were no aortic deaths or reintervention, no decline in renal function (measured by serum creatinine and glomerular filtration rate), and 100% patency of the preserved renal arteries. CONCLUSIONS: Atypical renal anatomy should not preclude repair of aortic aneurysms. Repair of such aneurysms is safe and achieves good long-term outcomes with the use of the described techniques.

Sonographic Image of Solitary Kidney in Wilms Tumour Survivors.

BACKGROUND/AIMS: This study presents an analysis of the sonographic and laboratory parameters of solitary kidney in Wilms tumour survivors (TWs) and compares these parameters with those of healthy individuals. METHODS: Fifty-three TWs who completed treatment for Wilms tumour and 44 healthy individuals were enrolled. The study protocol consisted of completing a medical history, sonographic examination of the solitary kidney, estimation of glomerular filtration rate (eGFR) by the Schwartz or MDRD formulas, albumin urine excretion and BP measurement. RESULTS: Sonographic signs of kidney damage were observed in 22 (41,5%) TWs. The most frequently detected abnormalities are hyperechoic rings around renal pyramids (28,3% TWs). Hypertrophy of the solitary kidney occurred in 71,7% of cases. The mean volume of the solitary kidney was 77% of the sum of the two kidney volumes in the control group. The median eGFR in the TWs group was 117 with 25Q-105,5, 75Q-130 ml/min/1,73 m2 vs 131,8 with 25Q-124, 75Q-140 ml/min/1,73 m2 in the control group (p=0,000). Six TWs (11,3%) had a value of eGFR below 90 ml/min/1,73 m2. Increased urine albumin excretion (> 30 mg/g) was observed in 7 TWs (13,2%) and in 3 (6,8%) individuals in the control group. CONCLUSION: Ultrasonographic abnormalities in solitary kidney of TWs are frequent. The most frequently detected abnormalities are hyperechoic rings around renal pyramids. Sonographic examination of TWs ought to be performed not only to detect tumour recurrence but also to assess the signs of kidney damage and their progression.

Retrospective study to identify risk factors for chronic kidney disease in children with congenital solitary functioning kidney detected by neonatal renal ultrasound screening.

To evaluate the prognostic significance of factors frequently associated with a reduction in renal mass, such as prematurity, low birth weight, and congenital anomalies of kidney and urinary tract (CAKUT), in patients with solitary functioning kidney (SFK) and investigate signs of early renal injury due to glomerular hyperfiltration damage or dysplasia in the remaining kidney.Retrospective observational study of congenital SFK diagnosed and followed at a tertiary care hospital over a period of 10 years in which 32,900 newborns underwent routine neonatal abdominal ultrasound screening. We analyzed age at diagnosis, sex, gestational age, anthropometric measurements at birth and prenatal and neonatal ultrasound findings, in addition to follow-up data corresponding to imaging findings (ultrasound, micturating cystourethrography, dimercaptosuccinic acid renal, and scintigraphy), ipsilateral CAKUT, compensatory hypertrophy, and renal injury in the form of albuminuria, blood pressure, and estimated glomerular filtration rate (eGFR).In total, 128 congenital SFK cases were detected (1 per 257 live births). Of these, 117 (91.4%) were diagnosed by neonatal ultrasound screening and 44.5% of these had been previously identified by prenatal ultrasound. Neonatal ultrasound had a specificity of 100% and a sensitivity of 92.1%. Forty-five patients (35.2%) had ipsilateral CAKUT and the most common type was urinary collecting system anomalies (75.5%). Over a median follow-up of 6.3 years (1-10 years), compensatory renal hypertrophy was observed in 81 patients (63.7%), most of whom had ipsilateral CAKUT (76.1% vs 56.6% of patients without ipsilateral CAKUT). Albuminuria and hypertension were observed in 3.12% and 5% of patients, respectively, and both were associated with ipsilateral CAKUT (P < .05). In addition, 75% of albuminuria cases (P = .031), 83.3% of hypertension cases (P = .004), and 100% of decreased eGFRcases (P = .031) were significantly associated with CAKUT (renal parenchymal anomaly category), being the strongest predictor of GFR the presence or absence of CAKUT.Neonatal ultrasound screening is useful for the early diagnosis of SFK. The presence of ipsilateral CAKUT should be evaluated in all patients with SFK as congenital anomalies of the renal parenchyma are associated with a poorer prognosis. Because morbidity from CAKUTs may not develop until adulthood, patients should be closely followed throughout life.

[Congenital megacalycosis in a girl with unilateral renal agenesis]. / Wrodzone kielichonercze u dziewczynki z jednostronna agenezja nerki.

Renal agenesis occurs in pediatric population with the incidence 1:500- 2000 children. It is more often diagnosed in boys and on the left side of the body. Renal agenesis may be isolated or it may be a part of complex malformation syndrome. Megacalycosis is a very rare anomaly of urinary tract associated with abnormal structure of the kidney pyramids. AIM: The aim of the study was to present for the first time in the medical literature the case of a girl with unilateral renal agenesis and megacalycosis. CASE REPORT: A girl, born at term in good general medical condition, and with normal birth weight was admitted to the hospital because of urinary tract infection caused by E.coli. Antenatal abdominal ultrasounds were normal. In a diagnostic, repeated ultrasound studies, unilateral, left renal agenesis and the righ-sided megacalycosis were found. The right kidney had dilated collecting system, with normal size of renal pelvis and enlarged calyces up to 26 mm. The kidney function was normal. Voiding cystourethrography excluded vesicoureteral reflux. Dynamic scintigraphy 99mTc-EC showed the lack of function of the left kidney, postinflammatory changes and dilation of collecting system without signs of obstruction. During two-years follow up we didn't observe clinical relapse of urinary tract infection. Blood pressure and kidney function were normal. CONCLUSIONS: Complex congenital anomalies of the kidney and the urinary tract (CAKUT) can be diagnosed at any age. Normal antenatal abdominal ultrasound does not exclude CAKUT. Every patient with congenital abnormalities of the kidney and the urinary tract requires long-term follow up, because of increased risk of chronic kidney disease.

Renocolic fistula secondary to urothelial carcinoma.

A 77-year-old man presented with watery, bloody diarrhoea, symptomatic anaemia and signs of sepsis. He was well known to our unit with a history of extensive low-grade urothelial carcinoma involving a solitary kidney. CT performed on admission demonstrated a new finding of renocolic fistula. Due to his multiple medical and surgical comorbidities conservative management was elected. He passed away after 1 year of follow-up.

Pediatric Ureteroscopy.

In recent years, the incidence of nephrolithiasis in the pediatric population appears to be increasing. 1 This has placed a new emphasis on surgical management of stones in children. In the past, extracorporeal shockwave lithotripsy was the preferred management technique for stones in children. 2 More recently, though, advances in endoscopy have allowed ureteroscopy to be adapted to the pediatric population and this approach has become more frequently utilized not just for lower ureteral calculi but also for proximal ureteral and renal stones.