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We report a rare case of non-fused renal ectopia with pelviureteric junction obstruction and multiple pelvic and renal calculi thereby discussing vascular anatomy of the non-fused ectopic kidney along with robot assisted surgical management of this rare clinical entity which amounts for good preoperative workup for best surgical and clinical outcome.

The incidence of ectopic kidney is 1 in 2100–3000 autopsies. Renal cell cancer (RCC) in pelvic kidney is a rare entity with limited case reports available in the literature. Symptoms can vary from abdominal pain to haematuria, or RCC can be detected incidentally. Contrast imaging with computed tomography (CT) angiography is recommended to determine the location of the renal mass and the anatomy of surrounding organs and major vessels feeding it. Treatment of choice is radical nephrectomy. We report a case of RCC in ectopic pelvic kidney managed with radical nephrectomy that was deemed unresectable on CT imaging.

Introduction Meyer–Rokitansky–Küster–Hauser syndrome is rare congenital abnormality in females with a blind ending vagina. There can be associated hypoplasia of uterus. The syndrome is of sporadic origin and affects the reproductive life. Adequate and early diagnosis can help to rule out other associated anomalies. Case presentation We are presenting an interesting case of Mullerian agenesis in a 16-year-old female patient who came to our gynecology department of our hospital with complaints of primary amenorrhea. On imaging, it was found that she was a case of Mayer–Rokitansky–Küster–Hauser syndrome with ectopic kidney. On physical examination, affected individuals appear normal but there was agenesis of uterus which is the common cause of primary amenorrhea. Magnetic resonance imaging findings are discussed in this article. On karyotyping and hormonal evaluation, patient hormones were in range and karyotype was 46, XX. Conclusion MRI is mainstay for diagnosis of this condition although other modalities like ultrasound can diagnose hypoplastic uterus and ectopic kidney. A rudimentary uterus hampers the reproductive functioning, and patient needs counseling for the same.

Background and Objective: Congenital renal anomalies cover a significant portion of abnormalities, which may be due to variations in number, position, shape, and size of the kidney(s). Most studies are based on structural, local, and vascular abnormalities, while few of them, to the best of our knowledge, were conducted on abnormal renal rotation (renal malrotation). Case Presentation: The patient was a 25-year-old woman who presented with right kidney pain with a history of pain in the same area. Examinations and tests were normal. The patient underwent retrograde pyelography. The right and the left kidneys were observed with excessive rotation and below the normal location, and severe hydronephrosis, respectively. Conclusion: Ectopic kidneys are accidentally diagnosed and are prone to complications such as hydronephrosis, kidney stones, Urinary Tract Infections (UTI), and urologic problems.

INTRODUCTION: Ectopic kidneys with concomitant aortoiliac aneurysmal disease have been previously reported in the literature, however its exact prevalence is unknown. The objective of this review is to summarize current knowledge on the treatment this special group of patients. METHODS: A non-systematic literature research was performed on the treatment of aortoiliac aneurysmal disease in patients with ectopic kidneys. RESULTS: Literature on the management of patients with aorto-iliac aneurysms and concomitant ectopic kidneys is limited to case reports and very small series. Treatment modalities which include open, endovascular or hybrid techniques, should preserve the variable vasculature of the ectopic kidney. Several different surgical solutions have been proposed, highlighting the uncertainty on the optimal management strategy. However, a growing number of reports suggest safety and efficacy with adapted endovascular techniques. CONCLUSION: In parallel to the general trend in the management of abdominal aortic aneurysm, it is expected that a growing number of patients will concomitant aorto-iliac aneurysm and ectopic kidneys will be treated with endovascular techniques., Angiologia e Cirurgia Vascular, Vol. 18 No. 2 (2022): June

Introduction Pelvic kidney is a rare congenital anomaly. The ectopic kidney is more susceptible to developing lithiasis. The management of this type of lithiasis is a challenge. The objective of this paper was to conduct a review of available literature on the treatment of stone in ectopic kidney. Material and methods Description of a case of transperitoneal laparoscopic pyelolithotomy for the treatment of inferior calyceal lithiasis in a right pelvic kidney. A literature review was performed by using Pubmed. The following terms and combination terms were searched: “pelvic ectopic kidney”, “ureterorenoscopy”, “extracorporeal lithotripsy”, “PCNL”, “pyelolithotomy”. We incluyed original articles, meta-analysis, review and case reports. Results 130 articles were excluded by title or duplication. 62 abstracts articles and them 50 full text articles were evaluated. Stone free rate were 75% (SLW), 85% (URSf), 85%–90% (PCNL) and 100% (laparoscopic pyelolithotomy). The literature on treatment on pelvic kidney is poor. Conclusion Factors such stone size, density and location, and upper urinary tract abnormalities, influence the choice of therapeutic approach (retrograde, percutaneous and/or laparoscopic/robotic). Laparoscopic pyelolithotomy is a safe and minimally invasive treatment option for large kidney stones with unfavorable anatomy for the endoscopic approach.

Background To compare the safety and efficacy of mini-percutaneous nephrolithotomy (PCNL) and retrograde intrarenal surgery (RIRS) for the treatment of renal lithiasis in patients with pelvic ectopic kidney. Methods From January 2015 to October 2017, mini-PCNL and RIRS were performed in ten patients diagnosed with lithiasis in pelvic ectopic kidneys, including three cases under laparoscopy-assisted mini-PCNL. Patient demographics and perioperative characteristics (age, gender, BMI, side of pelvic kidney, stone size, stone number, stone location, special medical history, and ASA physical status classification), and operative and post-operative related details (operation time, hospital stay, blood loss, VAS, analgesic requirement, complications, and stone free outcome) were reviewed. Results Although the mean operation time of mini-PCNL (71.3 min) was shorter than RIRS (85.3 min), the mean operation time of laparoscopy assisted mini-PCNL (92 min) was longer than patients without laparoscopy-assisted mini-PCNL (55.8 min). However, the use of mini-PCNL allowed for larger lithiasis to be dealt with (1.9 cm in laparoscopy assisted mini-PCNL and 2.4 cm in mini-PCNL without laparoscopy-assist) compared with RIRS (1.2 cm). In addition, although the mean hospital-stay time, blood loss, and analgesic requirement of patients undergoing RIRS were less than those receiving mini-PCNL, the success rate of RIRS was only 50% (3/6) in comparison to 100% (7/7) for mini-PCNL. Except for pain and urinary tract infection after the operation, there were no significant intraoperative and postoperative complications, and no residual lithiasis were seen in any patient. Conclusions Although RIRS was less time-consuming and invasive, mini-PCNL can deal with the bigger lithiasis and more complex situations with a higher success rate. Both mini-PCNL and RIRS are feasible and safe treatments for pelvic ectopic kidney lithiasis with each carrying unique advantages. Hence in practice, an appropriate individualized treatment should be selected depending on patient characteristics.

Background The incidence of ectopic kidneys is 1:12,000 clinically and 1:900 postmortem. Patients with pelvic mal-rotated kidneys are more susceptible to recurrent urinary tract infections, recurrent renal stones, and renal injury. Fusion of the kidney lower poles is relatively common compared to other types of renal anomalies. Case presentation We present the case of a 36-year-old Sudanese female patient who presented with a long history of recurrent urinary tract infections unresponsive to antibiotics. Ultrasound scan revealed bilateral pelvic kidneys. Computed tomography (CT) urography confirmed bilateral ectopic fused kidneys, with the left kidney mal-rotated (renal pelvis facing upwards and laterally). Kidney infection secondary to vesicoureteral reflux was diagnosed. Antibiotics were prescribed according to culture and sensitivity. The patient responded well to ciprofloxacin. Conclusion A history of recurrent urinary tract infections without an apparent cause is highly suggestive of renal anomaly and should be investigated expediently. Ultrasonography or CT imaging may be utilized to aid in diagnosis. Early recognition may help prevent the high risk of end-stage renal failure associated with anomalies.

Renal ectopia, even though a benign condition, presents diagnostic challenges when its complications arise. Cold abscess in an abnormally sited kidney may, therefore, create a diagnostic conundrum for the clinician. We present the case of a 55-year-old male who had a suppurating ectopic kidney that mimicked an abdominal visceral mitotic lesion necessitating initial laparotomy, reviewed the literature, and highlighted the need to consider the differential diagnosis, especially in patients who have been referred from peripheral hospitals where generous antibiotic therapy has been instituted. We also recommend undertaking a preoperative split renal scintigraphy where available, especially when nephrectomy is considered.

Congenital Bochdalek hernia (BH) in an adult is rare and has an unusual presentation. They are confined to the pediatric age group with an incidence of 1:3,000 live births. It rarely persists asymptomatic until adulthood. Surgical repair by thoracic, abdominal, or thoraco-abdominal approach is the treatment of choice with diaphragmatic reconstruction in associated diaphragmatic agenesis. With only 10 cases of BH with partial diaphragmatic agenesis reported to date, we discuss the rarity, unusual presentation, and management of BH in a young adult with sickle cell disease that has not been reported in the literature.

Pelvic kidney, an ectopic renal mass caused by developmental failure, is a rare condition. Here, we report a case of laparoscopic anterior resection in a patient with a solitary pelvic kidney. A 76-year-old man was diagnosed as having rectosigmoid colon cancer. Preoperative computed tomography revealed a left ectopic kidney in his pelvis. Computed tomographic angiography and retrograde pyelography were performed preoperatively to identify the renal vessels and the ureter. To keep the surgical plane intact for complete mesocolic excision, a complete laparoscopic anterior resection was safely performed without open conversion, and there was no injury to the pelvic kidney. The patient was discharged on postoperative day eight without any complication. No deterioration of renal function was observed after surgery. This report describes a case of laparoscopic anterior resection that was safely performed without conversion to open surgery in a patient with a pelvic ectopic kidney.

Objective To better understand why children born with cloacal anomalies are at a high risk of renal insufficiency, this study aims to determine baseline renal volume in children with cloacal anomalies compared to controls. We hypothesized children with cloacal anomalies would be born with less renal volume. Methods An IRB approved database of children with cloacal anomalies was reviewed. Controls were female patients with two-vessel umbilical cord or preauricular tags who underwent screening renal ultrasound. Children were included if they had a renal ultrasound in the first 3 months of life. Cloacal exstrophy, horseshoe and cross-fused ectopic kidneys were excluded. Total and individual kidney volumes were compared between the two groups. Results The study cohort consisted of 109 patients, 46 (42.2%) cloaca patients and 63 (57.8%) controls. In unadjusted analyses, average total renal volume for cloaca and control patients was 22.4 cm3 versus 25.5 cm3 respectively (p=0.1006), and there was no significant difference when adjusting for age (p=0.3915). The estimated difference in renal unit volume between cloaca patients without solitary kidneys and controls was -1.6 cm3 (95% C.I.: -3.6, 0.4; p=0.1201), and there was no significant difference when adjusting for age (p=0.4725). The age-adjusted difference in renal unit volume between cloaca patients with solitary kidney and controls was 1.8 cm3 (95% CI: -1.1, 4.8; p=0.2148). Conclusions Children with cloacal anomalies have similar baseline renal volumes as children without cloacal anomalies. Therefore, the increased risk of renal insufficiency in this patient population appears to be due to renal injury post-natally.

Background: Bilateral Ectopic Kidney is uncommon and usually diagnosed during a routine medical checkup. It could be misdiagnosed as an abdominopelvic mass. In-depth knowledge of its presentation will aid in its management. A poor anatomical relation of the kidneys with other abdominopelvic organs could lead to renal complications and surgical errors. This is of gynaecological importance and worthy of discussion for a lady of her reproductive age. Case presentation: A 20-year-old nulliparous lady of African descent presented with a long-standing history of dull lower abdominal pain. The pain has been intermittent and occasionally associated with a sensation of movement in the lower abdomen. She has a regular and normal menstrual cycle and has no lower urinary tract symptoms. Physical examination was unremarkable with intact secondary sexual characteristics. The abdomen was soft, non-tender and without any organomegaly. Abdominal ultrasonography, renal pyelogram and urinalysis were carried out after her consent was sought in accordance with the institution’s protocol. Urine analysis was normal and culture was negative. However further imaging revealed a bilateral pelvic ectopic kidney. Conclusion: Most of the cases reported are usually unilateral ectopic kidneys. It is, therefore, our belief that this information will be useful to medical practitioners such as surgeons, urologists, radiologists and gynaecologists. Such a diagnosis will enable clinicians to follow up with the client and preserve the renal function whiles preventing iatrogenic injuries during surgical procedures.

Failure of mature kidney to reach its natural location in renal fossa is termed as renal ectopia. Ectopic kidney can be found in pelvic, iliac, abdominal, and thoracic location. Pelvic ectopia has been estimated to occur in 1 of 2100 to 3000 autopsies. In contrast, ectopic ureters are commonly associated with complete renal duplication. Commonest presentation in females in continuous urinary incontinence with normal voiding habits as ectopic ureter open below the bladder neck in urethra or vagina. An ectopic kidney with ectopic ureter is extremely rare congenital anomaly. We report a 36-year-old woman presenting with left lower abdomen pain with no history of fever, dysuria, or urinary incontinence. On evaluation, she was found to have left nonfunctioning ectopic pelvic kidney with ectopic ureter opening in the vestibule of the vagina, which was managed with laparoscopic nephroureterectomy. One should suspect an ectopic ureter in a female presenting with continuous urinary incontinence since birth. However, diagnosis is challenging when clinical presentation is unusual with no urinary incontinence as seen in the index case. Detailed local examination in correlation with imaging is key for diagnosis and rule out other congenital anomalies. Laparoscopic approach in such clinical scenario is a safe and feasible option.

Renal ectopia is a rare congenital anomaly that mostly occurs in the pelvic area. An ectopic kidney is usually associated with other anomalies such as a malrotation. We report the case of a 15-year-old male who consulted after a blunt abdominal trauma. A left iliac renal ectopia was incidentally discovered. This ectopic kidney was associated with a malrotation, and a pyelo-ureteral duplication. Iliac renal ectopia should be dissociated from other abdominal renal ectopias, and its association with other renal malformations should be further investigated.

Introduction Congenital diaphragmatic eventration is characterized by the elevation of the diaphragm, causing a protrusion of the intraabdominal viscera into the affected hemithorax and resulting in respiratory distress. Diaphragmatic eventration with an intrathoracic ectopic kidney is a very rare disorder with the incidence of 0.25% of all ectopias. Presentation of case A 16-day-old male presented with chief complaint of respiratory distress. His plain chest X-ray showed intestinal gases in the right diaphragm and elevation of the right diaphragm. Intraoperative findings revealed elevation of the right diaphragmatic dome and visceral displacement, including the ileum, transverse colon, and right lobe of the liver. Subsequently, hemidiaphragm plication was conducted. Two weeks after surgery, the patient suffered from respiratory distress again. Computed tomography (CT) scanning revealed right diaphragmatic elevation and an ectopic kidney inside the right hemithorax. During the second operation, there were no longer elevation of the right diaphragmatic dome nor any other organ displacement. Moreover, we decided to let the intrathoracic kidney remain in place. The outcome was good during the postoperative period and six months after surgery. Discussion Eventration of diaphragm with an intrathoracic ectopic kidney should be considered as a differential diagnosis in neonate patients with respiratory distress accompanied by a thoracic mass. Conclusion Congenital diaphragmatic eventration with an intrathoracic ectopic kidney is a very rare disorder, requiring a personalized surgical repair to achieve a good outcome. CT scanning may help confirm the diagnosis, particularly to define the dome elevation and the intrathoracic organ precisely., Highlights • CT-scan should be performed to establish the diagnosis of eventration of diaphragm and to find an incidental, very rare intrathoracic finding, such as an ectopic kidney. • Diaphragmatic eventration with an intrathoracic ectopic kidney is a very rare disorder, requiring personalized surgical repair to achieve a good outcome. • Ectopic kidney might be left intrathoracic without any complications and not require any surgical treatment.

Background Congenital anomalies of the urinary system are very common and have extremely varied presentation. Among them, the most rarely found structural anomaly is the pancake kidney. When both kidneys are fused along their medial surfaces to form a round-shaped single renal mass, it is termed as pancake kidney. In this case report, a pancake kidney was incidentally detected in a girl. The majority of individuals who have pancake kidney are usually asymptomatic but surgeons should be aware of coexisting malformation of other organs and its potential risk of developing malignancy. Case presentation A 12-year-old Bengali girl presented to our out-patient department with mild, dull aching, lower abdominal pain and dysuria. She had no history of fever, hematuria, menstrual abnormality, pelvic inflammatory disease, or trauma. Urine examination showed traces of albumin and 10–12 pus cells/high-power field. She had normal kidney function test but a digital X-ray of her kidney, ureter, and bladder region failed to demonstrate bilateral renal tissue shadows. Ultrasonography of her whole abdomen showed normal intra-abdominal organs except for empty bilateral renal fossa. A multi-detector computed tomography scan of her whole abdomen revealed one round-shaped mass measuring approximately 9 cm (vertical) × 10 cm (horizontal) in the pelvic cavity. That mass was finally identified as a pancake kidney. She was prescribed antibiotics based on urine culture and sensitivity test that cured her symptoms. She was advised to follow-up regularly in our out-patient department to evaluate her kidney function and to rule out any neoplastic change. Conclusions This condition can be managed conservatively, if the individual remains asymptomatic, by regular monitoring of renal function. Surgeons should remain alert for the development of infections, any obstructive manifestations leading to calculus formation, and any malignant changes. The individual should be careful in avoiding trauma to low-lying pelvic kidney. Extensive surgeries should be avoided and only selective procedures should be done so that the patient may lead a normal lifestyle.

The accessory spleen is a congenital disability characterized by a separated ectopic splenic parenchyma. Wandering spleen (WS) is a rare condition where the spleen, free from its ligaments, is allowed to move inside the abdomen, predisposing the patient to life-threatening complications due to torsion of the vascular pedicle; splenic infarction, portal hypertension, bleeding and acute abdomen may occur. WS or Accessory Spleen (AS) is rarely suspected at presentation since symptoms are usually not specific, and definitive diagnosis is often reached only by imaging technologies such as color flow ultrasonography and angio-spiral computed tomography. The size is rarely more than 4cm. Patients rarely present symptoms, and the diagnosis can be accidental when faced with ectopia in radiological or intraoperative investigations. It is an essential clinical entity since abdomen-pelvic tumors must always be considered as differential diagnoses, requiring detailed evaluation. Surgical treatment is indicated for symptomatic patients or those with complications. On the other hand, the ectopic kidney with rotation abnormality also consists of a singular entity. Congenital anomalies of the upper urinary tract occur in 3%-4% of those born, most of which are followed up clinically. The present study discusses a case of symptomatic intrapelvic WS associated with an ectopic kidney with rotation anomaly, a rare union, and still with scarce reports in the literature. This study aimed to present the case of a patient with an isolated oversized accessory spleen and a review of the literature.

Background An intrathoracic kidney is a very rare form of ectopic kidney. Though increasingly recognized in the literature, impact on renal function is less well described. We report the case of a 67-year-old South Asian gentleman who presented with intrathoracic kidney and chronic kidney disease. We carried out a systematic review of the available literature on intrathoracic kidney, in order to characterize the typical clinical features, and describe likely clinical course and possible renal and extra-renal complications associated with this form of ectopia. Materials and methods A structured search using PubMed identified all relevant published case reports from 1988 to 2018, with search restricted to papers in English, and to adult cases only (> 18 years of age). 124 records were identified, and after screening for eligibility, 34 case reports were analyzed. Results Median age was 53.5 years, with no gender predominance. 68% (27/34) of cases were symptomatic. 29% (10/34) had a significant complication associated with their intrathoracic kidney, with 3 cases with either documented chronic or end-stage kidney disease. 26% (9/34) required surgical intervention. Conclusion Though previously regarded as a benign entity, results from our systematic review, bearing in mind susceptibility to publication bias, suggests an appreciable risk of symptoms, complications, and in the minority a risk to kidney function. We recommend close biochemical and imaging surveillance of affected patients, with low threshold for intervention in those with renovascular stenosis, reflux, or hydronephrosis.

Introduction Congenital and acquired renal diseases, which can produce renal insufficiency during the neonatal period, may be classified according to their ultrasonographic (US) characteristics: increased parenchymal echogenicity (renal parenchymal diseases, angiotensin-converting enzyme inhibitor fetopathy, and cortical necrosis), cystic disease (glomerulocystic kidney disease, autosomal recessive polycystic renal disease, multicystic dysplastic kidney, and cystic renal dysplasia), obstructive uropathies (ureteropelvic junction obstruction and posterior urethral valves), infections (candidal infections), and renal agenesis. The high-resolution sector and linear-array transducers allow the characterization of the underlying pathologic conditions in many cases. Findings of the renal parenchymal disease will vary on Doppler US, and during the acute phase, diastolic flow can be decreased, absent, or reversed. In patients with glomerulocystic kidney disease, US shows bilaterally enlarged kidneys with diffusely increased echogenicity and retention of a reniform contour, loss of corticomedullary differentiation, and cortical cysts. Obstruction of the ureteropelvic junction, the most common cause of hydronephrosis in neonates, can be seen on US as a dilated renal pelvis with dilated and communicating calices, lack of dilatation in the distal portion of the ureter, changes of renal dysplasia with increased echogenicity of the renal parenchyma, and parenchymal cysts, depending on the severity and duration of the obstruction. The high-resolution US provides an improved characterization of the renal parenchyma and a more precise description of renal architecture. The aim of this work was to evaluate the benefit of routinely performing an abdominal ultrasound on newborns to detect possible renal abnormalities, which may be missed antenatally. Patients and methods This was a longitudinal study of 200 consecutive apparently normal neonates at Damietta University Hospital. The authors performed an abdominal ultrasound on newborns to detect possible renal abnormalities. Results In this study, the incidence of renal abnormality was found in 23 (11.5%) neonates; most abnormalities were found in males (78.3%). Moreover, the majority of abnormalities were seen in a young age group less than 20 days. In this study, regarding the ultrasound abnormality findings, three (13%) neonates had left hydronephrosis G II, with left pelviureteric junction obstruction, two (8.7%) neonates had bilateral hydronephrosis with right pelviureteric junction obstruction, two (8.7%) neonates had left multicystic dysplastic kidney and left mild nephropathy, two (8.7%) neonates had an ectopic kidney, two (8.7%) neonates had an enlarged kidney, two (8.7%) neonates had right mild multicystic dysplastic kidney and left mild hydronephrosis, and two (8.7%) neonates had right mild hydronephrosis and dilated right ureter, with right vesicoureteric reflex. There was a statistically significant difference between positive cases of ultrasound abnormality and the sex of the newborn. There was a significant statistical relation between positive cases of ultrasound abnormality and the age of the newborn. Conclusion This study has been able to demonstrate an 11.5% incidence of various types of urinary anomalies in this sample.

Scarce data are available on pelvic ectopic renal parenchymal perforation. However, this complication might lead to serious consequences. Clinicians should pay attention to the early identification and treatment of this complication. We herein report the first case of pelvic ectopic renal parenchymal perforation caused by a double-J stent after ureteroscopy. Compared with previously reported cases of renal parenchymal perforation not involving an ectopic kidney, our case involved no renal capsule hematoma and no other serious complications. Our primary management strategy was to review relevant examinations and tests, perform close monitoring, and instruct the patient to stay in bed. The patient recovered smoothly after the ureteral stent was removed 1 month postoperatively.

Urinary system anomalies are cases that can be encountered in the clinic. In the anatomy laboratory, we observed that a different vessel was separated from the aortic bifurcation during routine dissection. As a result of the vascular follow-up, we determined that the male cadaver had a pelvic localized ectopic kidney. As a result of the vascularization, we found that there is an ectopic kidney with pelvic location in the male cadaver. One of the most common forms of congenital renal ectopia is pelvic localized ectopic kidney. When the dissection was detailed, the right undescended testicle accompanying the right ectopic kidney was detected. In conclusion we are of the opinion that knowing such anomalies and variations will guide physicians before surgical procedures.

Introduction Cross-fused renal ectopia is a rare congenital malformation, and few cases of urolithiasis have been treated by retrograde ureteroscopic lithotripsy. We report a case of urolithiasis in right-to-left and superior-type cross-fused renal ectopia, successfully treated by the retrograde approach. Case presentation A 69-year-old woman with two 14-mm renal stones in cross-fused renal ectopia underwent retrograde ureteroscopic lithotripsy. Although we did not recognize the anomaly preoperatively, we could diagnose urolithiasis in the ectopic kidney intraoperatively. The patient had no complication, and follow-up computed tomography after 3 months showed only a 5-mm renal stone. Conclusion Although urolithiasis in patients with cross-fused renal ectopia is a challenging condition for the urologist, the retrograde approach is a safe and effective treatment method. It is necessary to carefully examine the urinary tract especially in a patient with a history of unidentified solitary kidney.

An ectopic kidney is defined as an atypically placed kidney, due to improper migration from the fetal pelvis, during embryogenesis. The presented CT scan of 72-year-old male with pain and visible hematuria reveals that the right kidney is located in the pelvis. The ectopic kidney has malrotation with a calcified artery and 2 veins. One draining in the right common iliac vein and the other connected to the left common iliac vein—near the bifurcation of vena cava inferior. Usually, pelvic ectopy is asymptomatic. However, it may lead to elevated blood pressure, increased risk of stone formation, infections, and traumatism, due to the atypical anatomical position. Variations in the anatomy of the kidney and its vascular supply are of clinical importance. It is possible to encounter a radiological, surgical, or cancer case, such as the presented.

Crossed Fused Renal Ectopia (CFRE) is a very rare anomaly of the kidneys with a prevalence of 1 in 1000 live births. In this condition, both kidneys are fused together on one side of the spine. Usually, CFRE is an asymptomatic condition and is mostly detected incidentally, but sometimes the patient may develop various renal signs and symptoms. We report a case of acute pyelonephritis in a crossed fused right renal ectopia which was diagnosed by renal ultrasound and confirmed by computed tomography in a 75-year-old female.

Ectopic kidney is a congenital anomaly of renal position, which is commonly asymptomatic. Symptoms arising from pelvic kidneys can closely mimic pathological processes of nearby organs and vice versa. We report an 18-year-old girl who presented with acute onset of a painful lump in the right iliac fossa. Although the clinical features mimicked an appendicular abscess, the judicious use of cross-sectional imaging aided in the proper diagnosis. Pyonephrosis of a right-sided lumbar kidney was confirmed on further imaging and nephrectomy was performed. This case has been presented to highlight the significance of renal imaging in patients suspected of an appendicular abscess.

Pulmonary agenesis is an extremely rare congenital malformation with an estimated frequency of 1 in 10000-15000 autopsies and 34 per million live births. In 50% cases it is associated with other malformations i.e. cardiovascular, musculoskeletal, gastrointestinal and renal.We report a rare case of a male neonate with right pulmonary agenesis associated with VACTERL sequence (L5 hemi-vertebra, right absent kidney with left ectopic kidney and right radial agenesis). This association was never reported in published literature. Keywords: Congenital abnormalities neonate agenesis VACTERL.

Crossed fused renal ectopia (CFRE) is an uncommon developmental anomaly of the genitourinary system whereby the two kidneys are located on the same side of the body and joined together, while the ureter of the ectopic kidney still enters the bladder in its normal side. CFRE has variable clinical presentations and usually discovered incidentally when patients are investigated for abdominal complaints. Treatment may be conservative when renal function is preserved with no associated symptomatic urological complications. Highlighted here is the unusual presentation of left-to-right CFRE with acute pyelonephritis as an appendix mass in a 19-year-old female.

The present case study presented a rare autopsy based finding on Crossed fused renal ectopia Crossed fused kidney place in an unusual area are the congenital developmental malformation of urinary tract. There are many variants of ectopic renal presentation. In this malformation both the kidneys are anatomically located at one side of the body over each other and inferior CFRE is the most frequent type observed. The condition is generally asymptomatic and diagnosed as an incidental radiology finding and Multi Detector Computed Tomography (MDCT), urography is the investigation of choice. Autopsy based kidney specimens with gross anomalies can be rich source of knowledge about renal pathology. It is concluded that ectopic kidney is an accidental finding, but awareness regarding this issue and its probable signs and symptoms among general population is the need of hour.

Thoracic ectopic kidney with congenital diaphragmatic hernia is a rare congenital malformation. We report a case of right thoracic ectopic kidney associated with congenital diaphragmatic hernia in a 4-year-old girl. Chest X-ray indicated the right diaphragmatic distention and contrast-enhanced CT showed thoracic ectopic kidney. We chose thoracoscopic approach for a complete anatomical repair. The bottom of the lower lobe of the right lung adhered to the hernia sac. Right thoracic ectopic kidney was pushed into abdominal cavity and congenital diaphragmatic defect was closed. Postoperative hemodynamics and renal function were normal. We review similar literatures and summarize surgical experience.

Renal ectopy is a rare phenomenon, which its diagnosis may be delayed due to an asymptomatic condition. The ectopic kidney may even have a normal functional state; however, because of occurring kidney blockade, the risk for kidney stones and even failure may be raised. In many cases, the diagnosis is completely incidental and is based on the request of radiography and sometimes, CT scan even for other reasons. In the therapeutic approach, in cases of kidney blockage, or urine backing up to the kidney, the surgical intervention is highly recommended by excisional removing the mass. Herein, we described a case of ectopic intrathoracic kidney initially presented with mild respiratory distress that was ultimately diagnosed by CT scanning and removed surgically.

Renal ectopia is a developmental anomaly with pelvic kidney being the commonest location (55%). The incidence of uretero-pelvis junction (UPJ) obstruction, nephrolithiasis, and reflux are higher in ectopic kidneys than their counterparts. We herein report a case of left ectopic pelvic kidney with large stone associated with UPJ narrowing managed successfully simultaneously with laparoscopic transmesocolic pyelolithotomy and pyeloplasty in a 45-year female. This is the largest stone reported in the literature in an ectopic pelvic kidney managed successfully with favorable outcome.

Introduction and importance Crossed fused renal ectopia is a rare congenital condition that might pose some diagnostic and therapeutic challenges to clinicians. We report a patient with a non-functional crossed fused ectopic left kidney that obstructed the orthotopic kidney in a rarely observed situation. Case presentation A 68-year-old male presented a right flank pain with fever. The diagnosis of right obstructive pyelonephritis was dressed, after biological and radiological investigations. The obstacle was a crossed ectopic left kidney in its inferior variety. The ectopic kidney was non-functional as result of an obstructive ureteral calculus. The patient had right ureteral stenting with a double-J catheter. Three months later, left nephrectomy was performed by lumbotomy. Per operative difficulties were mainly the infiltration of peri renal fat, the anarchic vascularization and the multiple small pedicles of the ectopic kidney that was also malrotated with the hilum facing anteriorly. Postoperative recovery was uneventful and the patient left the hospital after three days. Clinical discussion Crossed Fused renal ectopy is rare. As shown in this case, the ectopic kidney might cause damage to the orthotopic kidney, by compression to urinary ducts. Surgery is the main treatment option. Some difficulties related to aberrant vascularization and possible malrotation is to preview. Conclusion Crossed fused renal ectopia is uncommon renal anomaly, mostly asymptomatic. However, it may be responsible of some complications, sometimes severe. Surgery can be delicate due to vascular complexity., Highlights • Crossed renal fused ectopy is rare and asymptomatic congenital condition. Association to other malformations is possible. • The ectopic kidney may be responsible of the obstruction and the mal function of the orthotopic kidney. • This is the first case to be reported in literature. • Surgery might be difficult due to aberrant vascularization and possible malrotation of the kidney.

Among congenital renal anomalies, the ectopic kidney is a rare occurrence. Crossed fused renal ectopia (CFRE) - an even rarer subtype of ectopic kidney - is characterized by both kidneys being fused together on one side of the spine. CFRE is usually asymptomatic but can present with vague symptoms if the anomalous kidney becomes infected, is obstructed by calculus, or has a neoplastic change. There is no indication for surgical intervention if the kidney functions normally. This report presents a case of CFRE in a 31-year-old male with recurrent right flank pain resulting from a large calculus in the upper moiety of the fused kidney. The calculus was surgically removed by percutaneous nephrolithotomy (PCNL). The patient was discharged on analgesics, antibiotics, and potassium citrate tablets, with an order to follow up after one month. On follow-up, the patient's double-J (DJ) stent was removed and an X-ray was performed to rule out any complications. No signs of stones, strictures, or other complications were noticed.

Crossed fused renal ectopia is a very rare congenital ectopia and poses great challenges when performing abdominal aortic surgery because of the accompanying abnormal vessels and urinary tracts. A 79-year-old woman with an abdominal aortic aneurysm and L-shaped crossed fused renal ectopia was referred to our facility. One of the large ectopic renal arteries arose from the right common iliac artery. The aneurysm was treated with an endovascular aortic repair. The reversed chimney graft technique was applied to preserve the ectopic renal artery while elongating the distal landing zone on the right side. The patient experienced no complications such as renal dysfunction or recurrence of the abdominal aortic aneurysm during the 6-month follow-up period.

Intrathoracic kidney is a rare developmental anomaly representing

Highlights • Nephroblastoma is the most common pediatric renal tumor. • Genitourinary anomalies and syndromes frequently co-exist. • The existence of Wilms tumor in association with unilateral pelvic renal ectopia is very rare. • Nephroblastoma in a left pelvic kidney presenting as lower abdominal mass in a 10 year old is presented., Introduction Wilms tumor(nephroblastoma) is the most common renal tumor in childhood.The most frequent anomalies and syndromes associated with this tumor involve the genitourinary tract. The occurrence of Wilms tumor with renal ectopia is rare.When present,Wilms tumor is usually found in association with other forms of renal ectopia like horseshoe kidneys or crossed fused renal ectopia. However, reports of the occurrence of Wilms tumor in unilateral pelvic renal ectopia is uncommon. We report a case of Wilms tumor in a pelvic left kidney. Presentation of case A 10 year old girl presented to the out-patient department with a one year history of lower abdominal mass, pain and hematuria. Examination revealed a mass in the suprapubic region. Radiological investigations showed a tumor in a left pelvic kidney. She was managed with preoperative chemotherapy, surgery and adjuvant chemotherapy. Histopathologic examination of the nephrectomy specimen confirmed the diagnosis of nephroblastoma. Discussion The occurrence of Wilms tumor in association with renal ectopia is rare. There are few reports of tumors arising in unilateral pelvic ectopic kidneys. A multi-modal treatment approach involving the use of chemotherapy, surgery and radiotherapy (for high-risk tumors) in the management of Wilms tumor gives good outcome. Conclusion Tumors arising from a pelvic kidney should be considered as part of the differential diagnosis of lower abdominal masses in children.

Background: Supernumerary kidney is an accessory organ with its own encapsulated parenchyma, blood vessels and ureters, either separated from the normal kidney or connected to it via fibrous tissue and ectopic kidney is a migration abnormality of the kidney. Here, we have evaluated a rare case of the supernumerary and ectopic kidney with DMSA, MAG3 and also CT fusion of the images. Methods: The absolute divided renal function was calculated for each kidney by DMSA. The MAG3 scintigraphy showed no obstruction in the ureteropelvic junction. Furthermore, the renogram curve and Tmax and time to ½ values were assessed. Two months after the conventional scintigraphies, the patient was referred to a CT scan and the fusion of DMSA SPECT and CT data was generated on a workstation. Results: The ectopic supernumerary kidney was functioning very well except a small hypoactive area, visible on DMSA, which was possibly a minimal pelvicalyceal dilatation. However, consequent CT scan did not show any pathology. Conclusion: It is important to evaluate particularly complicated or rare cases with multimodality systems with 3D or fusion techniques for the accurate diagnosis.

Background: The aim of this study was to evaluate the clinical and demographic characteristics of children with renal anomaly detected by urinary system ultrasonography. Methods: The medical records of children with urinary system anomalies detected by ultrasonography in our hospital and who underwent further investigations in one year follow-up were examined retrospectively. Results: A total of 325 patients were included in the study, the ages of between 0-17 years, 179 (55.1%) of them males. The most common urinary system anomaly was uretero-pelvic junction obstruction in 103 (31.7%) patients. Other detected anomalies were vesicoureteral reflux in 82 (25.2%) patients, renal hypoplasia in 33 (10.2%), double collecting system in 30 (9.2%), posterior urethral valve in 18 (5.5%), in 14 ( 4.3%) uretero-vesical junction obstruction, 13 (4.0%) renal agenesis, 13 (4.0%) ectopic kidney, 11 (3.4%) horseshoe kidney, and 8 (2.5%) as multicystic dysplastic kidney determined. Five patients had renal failure and 10 patients had hypertension. Conclusion: It is necessary to follow up closely to determine the complications that may occur in children with urinary tract anomalies, to detect renal damage and to evaluate the additional abnormalities. Keywords: Child, Urinary tract anomaly, Renal damage, Vesicoureteral reflux.

Background This study aimed to estimate the associations of copy number variants (CNVs) with fetal kidney ultrasound anomalies. A total of 331 fetuses with kidney ultrasound anomalies who underwent prenatal chromosomal microarray analyses were enrolled. The fetuses were classified into groups with isolated and nonisolated anomalies or according to the types of kidney anomalies. Results Clinically significant CNVs were identified in 3.4% or 7.3% of fetuses with isolated or nonisolated kidney anomalies, respectively. CNVs were more frequently identified in fetuses with abnormal embryonic migration of the kidneys (6.6%) than in fetuses with malformations of the renal parenchyma (4.7%) or anomalies of the urinary collecting system (3.4%). In particular, CNVs were most frequently detected in fetuses with ectopic kidneys (9.5%) but not in fetuses with horseshoe kidneys or isolated duplex kidneys. Among these CNVs, the most common were del(17)(q12q12) (1.2%) and del(22)(q11q11) (0.6%). The dup(17)(p12p12) and del(15)(q11.2q11.2) CNVs were identified in this study but not in previous studies. The del(X)(p11.4p11.4) and del(16)(p13.3p13.3) CNVs were further implicated as associated with kidney anomalies. Conclusions Fetuses with abnormal embryonic migration of the kidneys (particularly ectopic kidneys) showed a higher frequency of clinically significant CNVs, whereas fetuses with horseshoe kidneys or duplex kidneys were less frequently associated with these CNVs.

An observational study carried out in a period of two years, in the Department of Radio-Diagnosis, Dr. D. Y. Patil Medical College, Hospital and Research centre, Pimpri, Pune. 50 patients with suspected or known cases of urinary tract calculi who came with renal colic or hematuria were subjected to CT urography. The study comprised of 23 females (46%) and 27 males (54%), thus showing male preponderance. The majority of the patients were from the age group of 12-30 years, with mean age of 38.96 years. 35 patients had urolithiasis. 22 patients (44%) had renal calculi, 7 patients (14%) had calculi at other sites and 6 patients (12%) had calculi at multiple sites. At other sites, calculi were most commonly seen in ureters, followed by PU junction, VU junction and urinary bladder. Right kidney was more commonly affected than left kidney. Obstructive changes were commonly due to calculi in ureters. Nephrographic phase was delayed in 1 patient (2%) and absent in 1 patient (2%), while excretory phase was delayed in 5 patients (10%) and absent in 2 patients (4%). Other pathological findings were identified in the study included renal cysts, reflux nephropathy, lymphocele, abscess, pyelonephritis, PUJ block, polycystic kidneys and neoplasm. The anatomical variants seen in the study included supernumerary kidneys, duplex collecting system, trifid pelvis, ectopic kidney, horse shoe kidney and retrocaval ureter.