Müllerian anomalies in girls with congenital solitary kidney.

BACKGROUND: The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA in patients with congenital SFK. METHODS: A retrospective review was performed of patients within the Nationwide Children's Hospital system with ICD9 or ICD10 diagnostic codes for congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review. Patients with complex urogenital pathology were excluded. Renal anomaly, MA, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated. RESULTS: Congenital SFK occurred in 431 girls due to URA (209) or MCDK (222). Pelvic evaluation, most commonly by ultrasound for evaluation of abdominal pain or dysmenorrhea, occurred in 115 patients leading to MA diagnosis in 60 instances. Among 221 patients ages 10 years and older, 104 underwent pelvic evaluation and 52 were diagnosed with an MA of which 20 were obstructive. Isolated uterine or combined uterine and vaginal anomalies were the most common MA. MA were five-fold more common in patients with URA compared to MCDK. In 75% of patients, the SFK was diagnosed prior to the MA. CONCLUSIONS: The prevalence of MA in patients with congenital SFK was 24% among those age 10 years or older, and 38% were obstructive. This justifies routine screening pelvic ultrasound in girls with congenital SFK to improve early diagnosis.

Renal agenesis: A meta-analysis of its prevalence and clinical characteristics based on 15 641 184 patients.

Our objective was to analyse the newest relevant data on worldwide prevalence and associated symptoms of renal agenesis (RA). This meta-analysis builds on previous systematic reviews to include bilateral RA, its symptoms and data on gender, unilateral RA and anomaly location prevalence. Review of available data included records in English and other languages from PubMed, Embase, ScienceDirect, Web of Science, SciELO, BIOSIS, Current Content Connect Korean Journal Database and Russian Citation Index and Google. A total of 15 641 184 patients were analysed in relation to the prevalence of RA. The pooled prevalence of RA was 0.03% (95% CI: 0.03%-0.04%). Based on 500 subjects, a pooled prevalence of 47.96% (95% CI: 31.55%-64.58%) for unilateral and 52.04% (95% CI: 35.42%-68.45%) for bilateral RA has been set. Our study presents the newest generalized findings on bilateral RA. There appears to be universal disease and symptom prevalence with minor differences between world regions, although quality of future observational research should include genomic data. This will provide even further insight into the prognosis of various renal anomalies and their etiologies.

Follow up of renal outcomes in children with solitary kidney.

BACKGROUND: Solitary kidney (SK) affects 1/1000 people worldwide, and there are controversies concerning renal outcomes in these patients. This study aimed to investigate clinical findings and renal outcomes in children with SK and to compare the results for congenital (CSK) and acquired SK (ASK) groups. METHODS: The study included patients that presented to our pediatric nephrology department with SK between January 2010 and January 2021. Demographic and clinical data were recorded retrospectively. RESULTS: Of the 101 patients with SK, 71 had CSK (55 had unilateral renal agenesis and 16 had a multicystic dysplastic kidney) and 30 had ASK (17 had previously undergone unilateral nephrectomy due to a renal tumor and 13 had urological structural anomalies). There were nine patients (9%) with renal injury. The serum uric acid level was significantly higher and the estimated glomerular filtration rate was significantly lower in the patients with ASK compared with those with CSK (p = 0.005 and p < 0.001, respectively). There was a positive correlation between renal injury and the uric acid level (p < 0.001, r = 0.45). CONCLUSION: In addition to the management of blood pressure and proteinuria, it is important to control uric acid levels in patients with SK, especially those with ASK, to prevent renal injury. The ASK group has a greater risk of renal injury than the CSK group. There is a need for new markers to predict early stage renal damage in SK.

Zinner Syndrome with Papillary Neoplasm: A Rare Case Report.

One of the uncommon urogenital malformations in males, which presents late in the second or third decades of life, is the congenital malformation of the seminal vesicle. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction, and ipsilateral ejaculatory duct obstruction. Very few cases in the literature describe the malignant change in seminal vesicle cyst in Zinner syndrome. To the best of our knowledge, this is the first report of a papillary neoplasm of the seminal vesicle cyst developed in a patient with Zinner syndrome.

Retrospective evaluation of children with unilateral renal agenesis.

BACKGROUND: Children born with unilateral renal agenesis (URA) are thought to have a risk of developing hypertension, proteinuria, and progressive chronic kidney disease (CKD). The present study aimed to evaluate the long-term prognosis and clinical characteristics of children with URA. METHODS: The study included 171 patients aged < 18 years diagnosed as URA who were followed-up for ≥ 1 year and 121 healthy controls matched for age, gender, and BMI. RESULTS: Median age at diagnosis was 2 years (IQR: 1 month-16 years) and the incidence of URA in males (65.4%) was higher than in females. Among the patients, 21 (12.2%) had other urinary system anomalies. It was noted that 2.3% of the patients had proteinuria, 15.2% had hyperfiltration, and 2.9% had CKD. Hypertension based on ambulatory blood pressure monitoring (ABPM) was diagnosed in 18 (10.5%) of the patients, of whom 10 had masked hypertension. Diastolic blood pressure in the URA patients was significantly higher than in the healthy controls. The incidence of hypertension and CKD was significantly higher in the patients with other urinary system anomalies. CONCLUSIONS: Patients with a single functional kidney should be periodically evaluated throughout their lifetime for urine protein, blood pressure, and kidney functions. The most remarkable finding of this study is the importance of the use of ABPM for evaluating blood pressure in pediatric URA patients, especially for the detection of masked hypertension and the non-dipper phenomenon, which cannot be achieved with office blood pressure measurement.

The clinical characteristics of Chinese patients with unilateral renal agenesis.

BACKGROUND: We aimed to investigate the clinical characteristics of Chinese patients with unilateral renal agenesis. METHODS: We enrolled patients with unilateral renal agenesis diagnosed radiologically at the Department of Nephrology from January 2008 to January 2016. Patients with a solitary kidney due to nephrectomy or renal atrophy due to secondary factors were excluded. Clinical data were recorded and analyzed. RESULTS: In this study, 118 Chinese patients with unilateral renal agenesis were recruited, and the gender ratio (male/female) was 1.11:1. A total of 14 (11.9%) patients had additional abnormalities, 15 (12.7%) had a family history, and 30 (25.4%) presented with renal insufficiency. Kidney length, serum creatinine level and estimated glomerular filtration rate were significantly different between patients with and without family history (P < 0.05, respectively). Gender showed a significant difference between patients with and without other abnormalities. Kidney length and the incidence of proteinuria, hematuria, hypertension, and hyperuricemia were significantly different between patients with and without renal insufficiency. Logistic regression analysis revealed that family history was associated with severe renal failure (OR = 7.11, 95% CI 1.52-33.25). CONCLUSION: Renal insufficiency is common in patients with unilateral renal agenesis. Patients with renal insufficiency have shorter kidney lengths and a higher incidence of proteinuria, hypertension, hematuria, and hyperuricemia. Family history is considered a risk factor for severe renal failure.

Life with one kidney.

Life with a solitary functioning kidney (SFK) may be different from that when born with two kidneys. Based on the hyperfiltration hypothesis, a SFK may lead to glomerular damage with hypertension, albuminuria and progression towards end-stage renal disease. As the prognosis of kidney donors was considered to be very good, having a SFK has been considered to be a benign condition. In contrast, our research group has demonstrated that being born with or acquiring a SFK in childhood results in renal injury before adulthood in over 50% of those affected. Most congenital cases will be detected during antenatal ultrasound screening, but up to 38% of cases of unilateral renal agenesis are missed. In about 25-50% of cases of antenatally detected SFK there will be signs of hypertrophy, which could indicate additional nephron formation and is associated with a somewhat reduced risk of renal injury. Additional renal and extrarenal anomalies are frequently detected and may denote a genetic cause for the SFK, even though for the majority of cases no explanation can (yet) be found. The ongoing glomerular hyperfiltration results in renal injury, for which early markers are lacking. Individuals with SFK should avoid obesity and excessive salt intake to limit additional hyperfiltration. As conditions like hypertension, albuminuria and a mildly reduced glomerular filtration rate generally do not result in specific complaints but may pose a threat to long-term health, screening for renal injury in any individual with a SFK would appear to be imperative, starting from infancy. With early treatment, secondary consequences may be diminished, thereby providing the optimal life for anyone born with a SFK.

Association of Unilateral Renal Agenesis With Adverse Outcomes in Pregnancy: A Matched Cohort Study.

BACKGROUND: Data regarding the effect of a solitary kidney during pregnancy have come from studies of living kidney donors. We evaluated the risk for adverse pregnancy outcomes in women with a single kidney from renal agenesis. STUDY DESIGN: Matched cohort study. SETTING & PARTICIPANTS: Using data from 7,079 childbirths from an integrated health care delivery system from 1996 through 2015, we identified births from women with renal agenesis. Only first pregnancies and singleton births were included. After excluding those with diabetes and kidney disease, 200 women with renal agenesis were matched 1:4 by age (within 2 years), race, and history of hypertension to women with 2 kidneys. PREDICTOR: Renal agenesis defined by International Classification of Diseases, Ninth Revision (ICD-9) codes prior to pregnancy. OUTCOMES: The primary outcome was adverse maternal outcomes, including preterm delivery, delivery by cesarean section, preeclampsia/eclampsia, and hospital length of stay. Adverse neonatal end points were considered as a secondary outcome and included low birth weight (<2,500g) and infant death/transfer to acute inpatient facility. RESULTS: Mean gestational age at delivery was 37.9±2.1 weeks for women with renal agenesis compared to 38.6±1.8 weeks for women with 2 kidneys. Compared with women with 2 kidneys, those with renal agenesis had increased risk for preterm delivery (OR, 2.88; 95% CI, 1.86-4.45), delivery by cesarean section (OR, 2.11; 95% CI, 1.49-2.99), preeclampsia/eclampsia (OR, 2.41; 95% CI, 1.23-4.72), and length of stay longer than 3 days (OR, 1.81; 95% CI, 1.18-2.78). Renal agenesis was not significantly associated with increased risk for infant death/transfer to acute facility (OR, 2.60; 95% CI, 0.57-11.89) or low birth weight after accounting for preterm delivery (OR, 2.11; 95% CI, 0.76-5.88). LIMITATIONS: Renal agenesis was identified by ICD-9 code, not by imaging of the abdomen. CONCLUSION: Women with unilateral renal agenesis have a higher risk for adverse outcomes in pregnancy.

Identification of 8 Novel Mutations in Nephrogenesis-Related Genes in Chinese Han Patients with Unilateral Renal Agenesis.

BACKGROUND: Few genetic studies have focused on unilateral renal agenesis (URA), which is a disorder with insidious clinical manifestations and a tendency to result in renal failure. We aimed to detect pathogenic mutations in nephrogenesis-related genes, which were identified by a literature review conducted among a large cohort of Chinese Han patients with URA. METHODS: Totally, 86 unrelated URA patients were included. All URA patients were diagnosed by employing radiological methods. Patients with a solitary kidney owing to nephrectomy or renal atrophy due to secondary factors were excluded. Nine (10.5%) patients had a family history of abnormal nephrogenesis. Fifteen (17.4%) had other malformations in the urogenital system. All coding exons and adjacent intron regions of 25 genes were analyzed using next-generation sequencing and validated by Sanger sequencing and 100 ethnically matched healthy controls. RESULTS: Ten conserved mutations (9 missense mutations and 1 deletion mutation) were identified in SALL1, EYA1, RET, HNF1B, DSTYK, WNT4, and SIX5. All mutations were novel or rare (frequency <0.1%) in the public databases and absent from the 100 healthy controls. Nine patients carried mutations in candidate genes. Most of the patients carried one single heterozygous mutation, except for 2, who respectively carried compound heterozygous mutations and 2 single heterozygous mutations. In addition, 2 patients shared the same mutation in DSTYK. CONCLUSION: A total of 10.5% of our URA cases could be explained by mutations in our candidate genes. The mutations in nephrogenesis-related genes in the Chinese Han patients with URA had a decentralized distribution without any hotspot mutations.

Spontaneous one-kidney rats are more susceptible to develop hypertension by DOCA-NaCl and subsequent kidney injury compared with uninephrectomized rats.

There is little clinical data of how hypertension may influence individuals with nephron deficiency in the context of being born with a single kidney. We recently developed a new rat model (the heterogeneous stock-derived model of unilateral renal agenesis rat) that is born with a single kidney and exhibits progressive kidney injury and decline in kidney function with age. We hypothesized that DOCA-salt would induce a greater increase in blood pressure and therefore accelerate the progression of kidney injury in rats born with a solitary kidney compared with rats that have undergone unilateral nephrectomy. Time course evaluation of blood pressure, kidney injury, and renal hemodynamics was performed in the following six groups of animals from weeks 13 to 18: 1) DOCA-treated rats with a solitary kidney (DOCA+S group), 2) placebo-treated rats with a solitary kidney, 3) DOCA-treated control rats with two kidneys (DOCA+C group), 4) placebo-treated control rats with two kidneys, 5) DOCA-treated rats with two kidneys that underwent uninephrectomy (DOCA+UNX8 group), and 6) placebo-treated rats with two kidneys that underwent uninephrectomy. DOCA+S rats demonstrated a significant rise (P < 0.05) in blood pressure (192 ± 4 mmHg), proteinuria (205 ± 31 mg/24 h), and a decline in glomerular filtration rate (600 ± 42 µl·min(-1)·g kidney weight(-1)) relative to the DOCA+UNX8 (173 ± 3 mmHg, 76 ± 26 mg/24 h, and 963 ± 36 µl·min(-1)·g kidney weight(-1)) and DOCA+C (154 ± 2 mmHg, 7 ± 1 mg/24 h, and 1,484 ± 121 µl·min(-1)·g kidney weight(-1)) groups. Placebo-treated groups showed no significant change among the three groups. An assessment of renal injury markers via real-time PCR/Western blot analysis and histological analysis was concordant with the measured physiological parameters. In summary, congenital solitary kidney rats are highly susceptible to the induction of hypertension compared with uninephrectomized rats, suggesting that low nephron endowment is an important driver of elevated blood pressure, hastening nephron injury through the transmission of elevated systemic blood pressure and thereby accelerating decline in kidney function.

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.

Herlyn-Werner-Wünderlich syndrome: Two case report. / Síndrome de Herlyn-Werner Wünderlich:reporte de dos casos.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

A variant of Zinner syndrome with ectopic ureteral insertion into the seminal vesicle.

Zinner syndrome comprises a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction, which can be accompanied by additional abnormalities of the genitourinary tract in some cases. Patients may be asymptomatic or present with urinary, reproductive, and/or local pain symptoms. Diagnosis is most commonly achieved via MRI. Here, we present the case of an 18-year-old male previously diagnosed with unilateral renal agenesis, who presented with testicular and penile pain, along with urinary urgency and frequency. MRI of the abdomen and pelvis revealed all three components of Zinner syndrome as well as an ectopic ureter emptying into the seminal vesicle. Our case adds to the existing limited literature on this rare syndrome and broadens the understanding of how this syndrome can present both clinically and radiologically.

Unilateral renal agenesis: a systematic review on associated anomalies and renal injury.

BACKGROUND: Unilateral renal agenesis (URA) is associated with other congenital anomalies of the kidney and urinary tract (CAKUT) and extra-renal anomalies. However, the reported prevalences of these anomalies are highly variable. We estimated the prevalence of associated CAKUT and extra-renal anomalies in patients with URA. Furthermore, we determined the prevalence of renal injury in URA patients. METHODS: We conducted a systematic review of English and non-English articles using PubMed and Embase.com. Included studies reported at least one of the following items: incidence of URA, gender, side of URA, prenatal diagnosis, performance of micturating cystourethrogram, associated CAKUT, urinary tract infection or extra-renal anomalies. Studies that described a mean/median glomerular filtration rate (GFR) and proportions of patients with hypertension, micro-albuminuria or a decreased GFR were also included. RESULTS: Analyses were based on 43 included studies (total number of patients: 2684, 63% male). The general incidence of URA was 1 in ∼2000. Associated CAKUT were identified in 32% of patients, of which vesicoureteral reflux was most frequently identified (24% of patients). Extra-renal anomalies were found in 31% of patients. Hypertension could be identified in 16% of patients, whereas 21% of patients had micro-albuminuria. Ten per cent of patients had a GFR<60 mL/min/1.73 m2;. CONCLUSIONS: These aggregate results provide insight in the prevalence of associated anomalies and renal injury in patients with URA. Our systematic review implicates that URA is not a harmless malformation by definition. Therefore, we emphasize the need for clinical follow-up in URA patients starting at birth.

Pregnancy Outcomes in Patients with Obstructed Hemivagina Ipsilateral Renal Agenesis.

STUDY OBJECTIVE: Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) is a rare congenital Müllerian anomaly. We sought to explore the impact of OHVIRA on pregnancy outcomes. METHODS: A retrospective chart review identified 8 patients with an OHVIRA diagnosis and at least 1 documented pregnancy. Data collected included demographic characteristics, age at diagnosis and repair, gynecologic history, and pregnancy outcomes. Descriptive analysis was performed. RESULTS: Twenty-three pregnancies were identified among the 8 patients (range 1-5 pregnancies per patient), resulting in 9 term births, 6 preterm births, and 8 first-trimester spontaneous abortions. The most common pregnancy complications were fetal growth restriction (2/15) and preeclampsia with severe features (2/15). The average gestational age at delivery was 37 2/7 weeks; however, 60% (9/15) of live births were at term. Two patients delivered after going into preterm labor, one of whom also had preterm premature rupture of membranes. Of the 15 live births, there were 10 cesarean deliveries, 4 vaginal deliveries, and 1 forceps-assisted vaginal delivery. Three patients (30%) underwent primary cesarean deliveries due to fetal malpresentation. Five live births were repeat cesarean deliveries, and 2 were unscheduled cesareans, at 34 and 36 weeks, due to preeclampsia with severe features. CONCLUSION: This study is among the first to examine pregnancy outcomes in patients with OHVIRA. Among these patients, there were 23 total pregnancies, with higher incidences of spontaneous abortion, preterm birth, breech presentation, and cesarean delivery compared with the general population. This descriptive data set addresses a knowledge gap to help clinicians more effectively advise patients with OHVIRA about pregnancy outcomes.

Pancreatic solid pseudopapillary neoplasm with concomitant left unilateral renal agenesis and bicornuate uterus: a case report.

A solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor of the pancreas. Concomitant SPN with urogenital anomalies is a very rare presentation. Case Presentation: A 16-year-old female presented with a chief complaint of abdominal pain 30 days back. Solid pseudopapillary neoplasm (SPN) of the pancreas was diagnosed with the aid of ultrasonography and contrast-enhanced computed tomography of the abdomen and pelvis. Incidentally, concomitant left unilateral renal agenesis and bicornuate uterus were also detected in radiological findings. The patient underwent spleen-preserving distal pancreatectomy, and SPN was confirmed with the histopathological report. Discussion: Symptomatic SPN patients present with an abdominal mass and pain or very rarely jaundice. Most of the SPNs are benign. Complete surgical excision results in more than 95% cure. SPN with concomitant urogenital anomalies is extremely rare, and their concurrent occurrence can be better attributed to Wnt signaling pathway owing to their similar pathogenic mechanism. Conclusion: The solid pseudopapillary tumor has an excellent prognosis if timely resected. Proper evaluation of the patient with imaging is necessary to suspect and diagnose SPN who has urogenital anomalies and vice versa.

Should we screen for Müllerian anomalies following diagnosis of a congenital renal anomaly?

INTRODUCTION: Despite the well-established embryological relationship in the development of renal and Müllerian structures, no clear guidelines exist regarding screening for Müllerian anomalies (MA) in the setting of a renal anomaly (RA). Delayed diagnosis of MA can have significant reproductive consequences. OBJECTIVE: To investigate the prevalence of coexisting MA in patients with congenital RA. STUDY DESIGN: This is a retrospective cohort study of females age 12-35 years with a diagnosis of RA, identified by diagnosis codes, who were followed for care between 2013 and 2020. Data were collected on demographics, medical history, clinical presentation, and imaging studies. Descriptive statistics were used to summarize the data. This study was IRB approved. RESULTS: A total of 465 patients were included in this study, of whom 326 patients (70.3%) had a pelvic evaluation during the study period. Of these 326 patients, 125 (38.3%) were found to have coexistent MA. About one-third of patients who underwent pelvic evaluation due to pain were found to have MA. For 69.6% (87/125) of patients with MA, the RA was diagnosed prior to the MA. The average age at time of RA diagnosis was 6.4 ± 8.8 years and the average age of MA diagnosis was 16.4 ± 6.9 years. Forty-eight (38.4%) patients had obstructive anomalies. Of the Müllerian obstructions, 93.8% were treated with urgent surgery and the remainder started on hormonal suppression. The prevalence of MA was dependent on the RA diagnosis (Figure). Of patients with a solitary kidney, 67.1% were diagnosed with MA. For other parenchymal RA, the prevalence of MA was 20-23%. In patients with solitary kidney, uterus didelphys was the most common MA (52.1%). Thirty percent of patients with a solitary kidney were diagnosed with an obstructive MA. CONCLUSIONS: In this study, 38% of patients with RA who underwent a pelvic evaluation were found to also have MA. Our study shows the strongest association between MA and solitary kidney, but also emphasizes a significant risk with other RA. Almost 40% of patients with diagnosed MA were found to have an obstruction that required urgent treatment. Delays in diagnosis and treatment of Müllerian obstructions can be detrimental for future reproductive health, due to risk of chronic pain, infertility, infection, and endometriosis. Given the high prevalence of MA in patients with RA, especially those with congenital solitary kidney, routine screening with pelvic ultrasound should be performed around the age of expected menarche.

Solitary kidney functional damage due to caesarean ureteric injury monitored for 2 years after acute management: A case report.

Caesarean section with associated ureteric injuries that damage kidney function is uncommon. Such injury in women with a solitary kidney has far-reaching effects if the diagnosis is delayed. The case report describes the successful acute management with stenting of ureteric damage after caesarean section in a woman with a solitary kidney. A 29-year-old woman presented with anuria, hypertension (174/101 mmHg) and flank pain 3 days after caesarean section. Physical examination was significant for peri-orbital oedema, left flank tenderness, pallor and delirium. A diagnosis of acute kidney injury was confirmed by elevated blood urea and creatinine levels. An ultrasound scan revealed a solitary hydronephrotic left kidney. She had successful acute management at a resource-deprived facility, with normal renal function at a 2-year follow-up. Proteinuria lasted for about three months after surgery. Recovery of solitary kidney function with acute kidney injury due to caesarean section ureteric injury may be associated with prolonged proteinuria without evidence of further functional deterioration.

Renal growth slope in children with congenital and acquired solitary functioning kidneys.

PURPOSE: This study aimed to analyze the renal growth slope in children with congenital and acquired solitary functioning kidneys. METHODS: This retrospective study included all renal ultrasonography examinations performed in children in the agenesis, multicystic dysplastic kidney (MCDK), or nephrectomy group between September 2002 and February 2019. We reviewed the images and recorded the contralateral kidney size only when there was no focal lesion. Linear mixed model or piecewise linear mixed model analyses with a time point of 24 months of age were performed. RESULTS: There were 132 patients, including 26 patients in the agenesis group, 35 in the MCDK group, and 71 in the nephrectomy group. The nephrectomy group showed the largest baseline kidney size (7.4 cm vs. 5.3 cm in the agenesis group [P<0.001] and 5.2 cm in the MCDK group [P<0.001]) and the smallest overall growth slope (0.04 cm/mo vs. 0.06 cm/mo in the agenesis group [P=0.004] and 0.07 cm/mo in the MCDK group [P<0.001]). However, considering the time point of 24 months for reaching adult renal function, there were significant changes in slope, from 0.1 cm/mo before 24 months of age to 0.03 cm/mo after 24 months of age in all three groups (P<0.001), without a significant difference among the groups. CONCLUSION: Significant changes were found in the renal growth slope before and after 24 months of age, with no significant difference between congenital and acquired solitary functioning kidneys.

Zinner Syndrome: A Case Report of a Rare Etiology of Infertility.

Infertility is a common medical issue with different etiologies. It can be related to female factors, male factors, or factors related to both partners. We report the case of a 35-year-old male patient who presented with primary infertility for 10 years. He was otherwise healthy with unremarkable past medical history. Physical examination revealed normal external genitalia with both testes were normal in size. Basic semen analysis revealed decreased sperm volume and sperm count. Abdominal ultrasound examination revealed an absent right kidney. Subsequently, the patient under a computed tomography scan that confirmed the right renal agenesis and demonstrated a well-defined right seminal vesicle cyst. Such findings were consistent with the diagnosis of Zinner syndrome. He underwent aspiration of the cyst that resulted in improvement in the sperm parameter of basic semen analysis. The case demonstrated a rare etiology of male infertility that was successfully managed conservatively. Despite its rarity, physicians should consider the developmental anomalies of the genitourinary system when encountering patients with infertility.