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• The prevalence of prenatal ectopic kidney is quite low. • The most common locations of ectopic kidneys are the iliac fossa and lateral pelvic areas, respectively. • In ectopic kidney cases, both kidneys are equally affected in both genders. • Blood supply to ectopic kidneys is most commonly provided by the iliac artery and then the aorta. • The most common extraurinary anomalies accompanying prenatally diagnosed ectopic kidney are cardiac anomalies. To assess the characteristics, additional structural anomalies and postnatal urinary outcome of the cases diagnosed with fetal ectopic kidneys in the prenatal period. Cases having fetal ectopic kidneys, detected from a total of 14,617 pregnant women examined by routine detailed (Group 1) or indicated (Group 2) obstetric ultrasonography (USG) in a tertiary perinatology unit were analyzed. The prevalence of the cases, time of the diagnosis, sidedness of the affected kidney, anatomical location, origins of blood supply, additional urinary or extraurinary anomalies, and urinary complications during the postnatal follow-up period were investigated. We have detected 33 fetuses with ectopic kidneys in our cohort. The prevalence of fetal ectopic kidney was 0.22 %, with a median (min.-max.) diagnosis time of 21.3 (17.6–34) weeks. In the group in whom indicated USG was performed, the time of diagnosis was later compared to routine detailed USG (p = 0.04) group. There was no difference in terms of gender [male, (n = 14), female (n = 19), p = 0.38] and the sidedness of the ectopic kidneys (p = 0.38). The location of ectopic kidneys was most frequent in the iliac fossa (n = 20, 60.6 %) and in the lateral pelvic areas (n = 13, 39.3 %). The blood supply origin of ectopic kidneys was the common iliac artery in 22 (66.6 %), whereas the aorta in 11 cases (33.3 %). There was an additional urinary anomaly in 8 cases (24 %), an extraurinary structural anomaly, most commonly cardiac, and/or a soft marker for aneuploidy were presented in 16 cases (48 %). The most common urinary complication in the postpartum period was vesicoureteral reflux (n = 5). Ectopic kidney in the prenatal period is a rare structural anomaly that can equally affect both genders and both kidneys. Prenatal diagnosis is important for the diagnosis of additional anomalies and follow-up of postnatal complications. [ABSTRACT FROM AUTHOR]

Objectives and background: Congenital malformations of the kidney and urinary tract (CAKUT) have a prevalence of 4–60 in 10,000 livebirths and constitute for 40–50% of all end stage pediatric kidney disease. CAKUT can have a genetic background due to monogenetic inherited disease, such as PKD or ciliopathies. They can also be found in combination with extra-renal findings as part of a syndrome. Upon detection of genitourinary malformations during the fetal anomaly scan the question arises if further genetic testing is required. The purpose of this study was to determine the phenotypic presentation of CAKUT cases and the results of exome analysis (WES). Methods: This is a retrospective analysis of 63 fetal cases with a diagnosis of CAKUT or DSD at a single center between August 2018 and December 2022. Results: A total of 63 cases (5.6%) out of 1123 matched CAKUT phenotypes including renal parenchyma malformations. In 15 out of 63 WES analysis a pathogenic variant was detected (23.8%). In fetuses with isolated CAKUT the rate of detecting a pathogenic variant on exome sequencing was five out of 44 (11.4%). Ten out of 19 fetuses (52.6%) that displayed extra-renal findings in combination with CAKUT were diagnosed with a pathogenic variant. Conclusions: WES provides an increase in diagnosing pathogenic variants in cases of prenatally detected CAKUT. Especially in fetuses with extra-renal malformations, WES facilitates a gain in information on the fetal genotype to enhance prenatal counselling and management. [ABSTRACT FROM AUTHOR]

Boning Li,1,* Tingting Zhu,1,* Fenghua Ma,2 Yuqing Zhao1 1Department of Gynecology, the Obstetrics and Gynecology Hospital of Fudan University, Shanghai, People’s Republic of China; 2Department of Radiology, the Obstetrics and Gynecology Hospital of Fudan University, Shanghai, People’s Republic of China*These authors contributed equally to this workCorrespondence: Yuqing Zhao, Department of Gynecology, the Obstetrics and Gynecology Hospital of Fudan University, No. 128, Shenyang Road, Yangpu District, Shanghai, 200090, People’s Republic of China, Tel +86-13817678883, Fax +86-021-33189900, Email yyqingzz@126.comAbstract: An ectopic kidney is a rare congenital defect that is often asymptomatic, but can be incidentally discovered during imaging examinations. Moreover, the morphological characteristics and laboratory features of ectopic kidneys are nonspecific, which may lead to misleading diagnostic approaches, particularly when there are additional factors, such as infection, obstruction, or other anomalies. A 43-year-old female presented with a mass in the left adnexal area. She had septate uterus and a history of congenital urinary incontinence. Ultrasound and MRI findings indicated that the mass was a cyst originating from the ovary. However, it is possible that the lump was derived from the urinary system. To confirm the diagnosis, laparoscopy was performed, followed by pathological examination, which confirmed the presence of an ectopic kidney with a single-system ectopic ureter. The patient underwent nephroureterectomy, and her symptoms successfully resolved, leading to a favorable prognosis. This case report highlights a rare case involving an ectopic kidney with a vaginal ectopic ureter that initially presented as an adnexal cyst and caused urinary dribbling. This case emphasizes the importance of early recognition and accurate diagnosis in women with similar symptoms.Keywords: ectopic kidney, ectopic ureter, urinary incontinence, pelvic mass, urogenital malformation

Background: Renal Anomalies are relatively common. Problems with congenital anomalies of kidney are important as they may cause renal failure. Failure in Ascent of kidney can result in a pelvic kidney alternatively, the kidneys may fuse together at their caudal poles produces Horseshoe kidney. Objectives: The present study is to evaluate renal developmental anomalies Radiologically. Material and Methods: The present study was a Cross Sectional study in Tertiary Care Hospital who were referred to radiology department for CT abdomens. 3500 abdominal CT scans were observed for a period of 6 months. Among those male CT abdominal scans are 2060 and female CT abdominal scans are 1440, from Tertiary care hospital in Andhra Pradesh and observed for renal malformations. Results: 3500 abdominal C. T scans were studied for a period of 6 months. Out of the 3500 ct's males are 2060 and females are 1440. Conclusion: Renal Anomalies all though asymptomatic and accidental finding in most of the cases thorough evaluation of the patient is utmost important to rule out serious pathological conditions prior to the arrival of symptoms. [ABSTRACT FROM AUTHOR]

Abstract Background Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and the fourth cause of death of end-stage renal disease. The disease has a prevalence of 1:400–1:1000 accounting for 10% of patients on dialysis. In most ADPKD patients, bilateral kidneys are similarly affected, with numerous fluid-filled cysts arising from different nephron segments. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic. Case presentation We report a case of a 46-year-old Ghanaian male patient who presented with left flank pain and hematuria with high BP and deranged renal function. Abdominal ultrasonography showed both kidneys to be larger than normal and had multiple cysts of varying sizes with the right kidney located in the right iliac fossa. Follow up Abdominopelvic computer tomographic scan (CT–Scan) without contrast showed enlarged kidneys with the renal parenchyma replaced by innumerable cyst of varying sizes. The right kidney was ectopically located in the right aspect of the pelvis. A diagnosis of ADPKD with right pelvic ectopic multicystic kidney was made. He was put on antihypertensives, analgesia for the left flank pain and to have follow up at the urology and nephrology departments. Conclusion In most ADPKD patients, bilateral kidneys are similarly affected. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and the fourth cause of death of end-stage renal disease. The disease has a prevalence of 1:400–1:1000 accounting for 10% of patients on dialysis. In most ADPKD patients, bilateral kidneys are similarly affected, with numerous fluid-filled cysts arising from different nephron segments. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic. Case presentation: We report a case of a 46-year-old Ghanaian male patient who presented with left flank pain and hematuria with high BP and deranged renal function. Abdominal ultrasonography showed both kidneys to be larger than normal and had multiple cysts of varying sizes with the right kidney located in the right iliac fossa. Follow up Abdominopelvic computer tomographic scan (CT–Scan) without contrast showed enlarged kidneys with the renal parenchyma replaced by innumerable cyst of varying sizes. The right kidney was ectopically located in the right aspect of the pelvis. A diagnosis of ADPKD with right pelvic ectopic multicystic kidney was made. He was put on antihypertensives, analgesia for the left flank pain and to have follow up at the urology and nephrology departments. Conclusion: In most ADPKD patients, bilateral kidneys are similarly affected. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic. [ABSTRACT FROM AUTHOR]

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Abstract Background Ectopic kidney and median arcuate ligament syndrome are both rare conditions. The clinical presentation and diagnosis of these conditions are not well studied. There are no reports on the combination of these two rare conditions. Case presentation We report a 24-year-old woman with fever, dysuria, urinary frequency and left flank pain for two days. The primary diagnoses in the clinic were left acute pyelonephritis and left hydronephrosis due to throbbing pain in the left costovertebral angle and pyuria. However, further computed tomography showed right ectopic pelvic kidney, left renal pelvis dilatation without definite ureteral lesion, good bilateral renal contrast enhancement, and compression of the celiac axis due to obstruction by the median arcuate ligament. Chronic abdominal symptoms were reported by the patient after repeat history taking. The patient’s condition was fully explained and discussed with her and her family, but they refused further therapy. After the acute pyelonephritis began improving, the patient was discharged for follow-up at our outpatient clinic. Conclusion We present an extremely rare case of a combination of two rare conditions: ectopic kidney and median arcuate ligament syndrome. No study to date has reported on the relationship between the two diseases. Given the rarity of the two conditions, no evidence or even a hypothesis exists to explain the possible etiology of their combination. More reports are required to enhance the understanding of these rare conditions.

Mensur Mohammed Ahmed,* Mubarek Bargicho Adem,* Adugna Getachew Mideksa,* Tolesa Yadeta Huluka Department of Surgery Urology Division, Saint Paul’s Hospital Millennium Medical College, Addis Ababa, Ethiopia*These authors contributed equally to this workCorrespondence: Mensur Mohammed Ahmed, Tel +251978191741, Email mensurm69@gmail.comAbstract: Pyonephrosis is characterized by infective hydronephrosis accompanied by suppurative destruction of the renal parenchyma, resulting in partial or complete impairment of renal function. Patients present with wide range of symptoms, they can be asymptomatic in 15% but most of them present with septic features like fever, chills, and flank pain. Urinary tract infections, functional and anatomic obstruction, and anatomic anomalies such as pelvic and horseshoe kidneys are common etiologic factors. We report a 30 years old female patient with giant pyonephrosis in an ectopic kidney. She presented with acute toxic symptoms after she has been experiencing progressive abdominal swelling and pain for a couple of years. Simple nephrectomy was done four weeks after she has been initially treated with percutaneous drainage of the pus and intravenous antibiotics.Keywords: giant pyonephrosis, ectopic kidney, percutaneous nephrostomy, simple nephrectomy

Urologists commonly make use of ureteral double J stents in endourology. If a DJ stent is forgotten to be removed, then complications happen in the form of encrustations, infection, migration, renal dysfunction and hydronephrosis. Ectopic pelvic Kidney is a relatively rare congenital anomaly caused by lack of ascent of the kidney. We report a very rare case of forgotten DJ stent in an ectopic kidney in a 55 years old patient with diabetes mellitus with history of left sided open pyelolithotomy done 21 months back. Redo open surgery was done to remove DJ stent along with stone removal.

Bochdalek hernia (BH) is a congenital diaphragmatic defect primarily diagnosed in neonates and is usually left-sided. Adult diagnosis, especially of right-sided BH is exceedingly rare and usually presents with symptoms. Till now, only 31 cases have been diagnosed to be right-sided BH along with intrathoracic kidney. This report presents a 26-year-old asymptomatic male who was incidentally diagnosed with a massive right congenital diaphragmatic hernia. Imaging revealed severe abdominal herniation, a right intrathoracic ectopic kidney, and a right liver lobe hypoplasty along with hypertrophied left liver lobe extending down to the pelvic cavity. Several surgeons were consulted, with controversial opinions on whether elective surgery should be performed or withheld. Due to the high risk associated with surgery and the patient’s choice, the surgery was deferred. For almost 18 months, the patient did not report any symptoms or complications. This case highlights the rarity of asymptomatic right-sided BH in adults and the challenges in determining a management approach. Also, it proposes a conservative approach for such patients as a management modality. Most extensive diagrammatic defect reported is estimated to have a neck defect of 10 cm. Most BH cases have been treated surgically and reports on the outcome of a conservative approach are exceedingly rare. In such cases, patient preferences and a thorough risk assessment play vital roles in decision-making regarding conservative versus surgical approaches.

The management of traumatic injuries in patients with ectopic kidneys presents special challenges. There is a paucity of literature regarding optimal strategies for renal salvage. We describe a case of a patient who presented in hemorrhagic shock after a motor vehicle collision. On initial operative exploration, he was found to have a large retroperitoneal mass. Subsequent imaging demonstrated a large retroperitoneal hematoma and an ectopic kidney. The patient was successfully treated with a combination of open renorrhaphy and endovascular angioembolization. This case demonstrates the importance of a multidisciplinary approach to treating these complex injuries.

Background: Ectopic kidney and median arcuate ligament syndrome are both rare conditions. The clinical presentation and diagnosis of these conditions are not well studied. There are no reports on the combination of these two rare conditions. Case presentation: We report a 24-year-old woman with fever, dysuria, urinary frequency and left flank pain for two days. The primary diagnoses in the clinic were left acute pyelonephritis and left hydronephrosis due to throbbing pain in the left costovertebral angle and pyuria. However, further computed tomography showed right ectopic pelvic kidney, left renal pelvis dilatation without definite ureteral lesion, good bilateral renal contrast enhancement, and compression of the celiac axis due to obstruction by the median arcuate ligament. Chronic abdominal symptoms were reported by the patient after repeat history taking. The patient's condition was fully explained and discussed with her and her family, but they refused further therapy. After the acute pyelonephritis began improving, the patient was discharged for follow-up at our outpatient clinic. Conclusion: We present an extremely rare case of a combination of two rare conditions: ectopic kidney and median arcuate ligament syndrome. No study to date has reported on the relationship between the two diseases. Given the rarity of the two conditions, no evidence or even a hypothesis exists to explain the possible etiology of their combination. More reports are required to enhance the understanding of these rare conditions. [ABSTRACT FROM AUTHOR]

This pictorial presentation is a range of computed tomography (CT) images of fusion and developmental abnormalities of the kidney. A brief discussion of each abnormality is presented. [ABSTRACT FROM AUTHOR]

Introduction and Objective: Laparoscopy is the most widely followed approach in ablative or reconstructive kidney surgeries. The aim of this study is to assess the utility and safety of laparoscopic approach in pelvic ectopic kidney surgeries. Methods: Between July 1, 2021 and June 30, 2022, 8 patients with pelvic kidneys; 4 with pelviureteric junction obstruction, 3 with pelvic stones, and 1 nonfunctioning kidney underwent laparoscopic pyeloplasty, pyelolithotomy, and nephrectomy, respectively. The records of all 8 patients were evaluated retrospectively for analyzing the operating time, blood loss, postoperative hospital stay, intra- and postoperative complications, surgical difficulty, and the success in completing the case laparoscopically. The patients were followed for at least 6 months to know the outcome. After pyeloplasty the improvement in function and drainage as well were recorded. Results: Of 8 cases, 6 (75%) were completed laparoscopically. One pyelolithotomy and 1 pyeloplasty patient were converted to open surgery. The median operative time was 180 (140–240) minutes, median blood loss was 100 (50–300) mL, and median hospital stay was 4 (3–6) days. One patient, who had open conversion, had Clavien grade I complication in the form of prolonged fever. Pyeloplasty patients at 6 months follow-up showed improvement in symptoms as well as function. Conclusion: The laparoscopic approach has obvious benefits in pelvic surgeries. Laparoscopy for ectopic pelvic kidneys are challenging due to abnormal anatomy of vessels and kidneys. Proper exposure of kidneys and exact identification of vessels can accomplish laparoscopic procedure in ectopic kidneys successfully with patients having nil complications and early convalescence. [ABSTRACT FROM AUTHOR]

Abstract Background Endometriosis mainly occurs in female pelvic organs. Endometriosis in the kidney is extremely rare. Case presentation We herein describe a case of a 19-year-old girl with occasional mild abdominal pain associated with an ectopic left kidney. SPECT-CT showed no abnormal radioactive distribution in the left pelvis, suggesting loss of function of the ectopic kidney. Laparoscopic left ectopic kidney resection was subsequently performed. Histopathology revealed endometriosis of the ectopic left kidney. Conclusions In female patients with clinical manifestations of abdominal pain and gross hematuria, the possibility of renal endometriosis should be considered.

Abstract Background Thoracic kidney (TK) presenting with symptomatic urolithiasis is a rare case scenario. Retrograde intrarenal surgery (RIRS) for calculus in TK is not reported in the literature. We report a case of partial staghorn calculus in TK managed by RIRS. Case presentation A young adult male presented with back pain and was found to have right TK and bilateral partial staghorn calculus. He also had right cryptorchidism with intraabdominally located testis. He underwent staged bilateral RIRS and laparoscopic right orchiectomy. Follow-up ultrasound imaging showed complete stone clearance in both kidneys. Conclusions RIRS is safe and effective treatment option, in the management of urolithiasis in TK. The anatomical abnormalities in TK did not play any hindrance role in the ureteral access, renal maneuverability of the flexible ureterorenoscope and subsequent drainage of the stone dust after RIRS.

A 21-year-old woman presented with polyuria, fragility fractures, and a history of recurrent renal calculi, which was also present in her maternal aunt. Examination revealed an oval palpable mass in the neck. Biochemistry revealed a grossly elevated serum calcium, PTH, and serum alkaline phosphatase with low serum phosphorous, suggestive of primary hyperparathyroidism. Ultrasonography of the neck and parathyroid scintigraphy localized a large lesion arising from the right posterior and inferior aspect of the thyroid gland, suggesting a parathyroid tumor. Parathyroid carcinoma was suspected based on the severe clinical manifestations. A computed tomography scan of the abdomen revealed cysts in the kidneys, bilateral medullary nephrocalcinosis, left ectopic-pelvic kidney, and lytic lesions in the iliac bone. The patient underwent a right inferior parathyroidectomy with normalization of serum calcium postoperatively. Histopathologic examination revealed a parathyroid adenoma, which was contrary to the expectation. Whole exome sequencing in the index case revealed a novel 99-bp heterozygous insertion, likely pathogenic variant in the exon 2 of CDC73 gene causing hyperparathyroidism jaw tumor syndrome. Here, we report a rare case of hyperparathyroidism jaw tumor syndrome that presented with severe hypercalcemia, renal cysts, and an ectopic-pelvic kidney without jaw tumor or uterine abnormalities. [ABSTRACT FROM AUTHOR]

Introduction: A congenital intrathoracic kidney (ITK) is a rare anomaly that is recognized to have four causes: renal ectopia with an intact diaphragm, diaphragmatic eventration, diaphragmatic hernia, and traumatic diaphragmatic rupture. We report a case of a prenatal-diagnosed ITK related to a congenital diaphragmatic hernia (CDH) and conducted a systematic review of all cases of the prenatal diagnosis of this association. Case presentation: A fetal ultrasound scan at 22 gestational weeks showed left CDH and ITK, hyperechoic left lung parenchyma, and mediastinal shift. The fetal echocardiography and karyotype were normal. Magnetic resonance imaging at 30 gestational weeks confirmed the ultrasound suspicion of left CDH in association with bowel and left kidney herniation. The fetal growth, amniotic fluid, and Doppler indices remained within the normal range over time. The woman delivered the newborn via an at-term spontaneous vaginal delivery. The newborn was stabilized and underwent non-urgent surgical correction; the postoperative course was uneventful. Conclusions: CDH is the rarest cause of ITK; we found only eleven cases describing this association. The mean gestational age at diagnosis was 29 ± 4 weeks and 4 days. There were seven cases of right and four cases of left CDH. There were associated anomalies in only three fetuses. All women delivered live babies, the herniated kidneys showed no functional damage after their surgical correction, and the prognosis was favorable after surgical repair. The prenatal diagnosis and counseling of this condition are important in planning adequate prenatal and postnatal management in order to improve neonatal outcomes. [ABSTRACT FROM AUTHOR]

Abstract Background Situs inversus totalis is a very rare congenital anatomical variation, in which all thoracic and abdominal organs are right-left inverted. This condition is associated with an increased risk of organ malformations including ectopic kidney, which is a very rare combination. Case presentation A 56-year-old male presented with colicky left iliac pain associated with nausea, vomiting, and irritative lower urinary symptoms. The patient has a medical history of recurrent lower urinary infections and a family history of situs inversus totalis. Radiological images demonstrated dextrocardia, situs inversus totalis of all the abdominal organs, and an ectopic pelvic kidney on the left side, with 4 stones inside it. Left nephrectomy was performed due to extensive renal damage. At discharge and during follow-up, the patient's condition was satisfactory and stable. Conclusions The ectopic kidney may present diagnostic and therapeutic challenges when associated with situs inversus.

Anatomic variations in the urogenital tract have generally been diagnosed through intravenous urography as a modality of choice. In recent years, computerized tomography (CT) urogram has replaced the traditional intravenous imaging of the genitourinary tract. Hematuria, tumoral mass, obstructive uropathy, and congenital collecting system abnormalities are indications for CT imaging. In this report, we present a young woman with intermittent right flank pain and recent urinary tract infection. Her history was also positive for spontaneous abortion. She was referred to the Radiology Clinic for a CT urography. Our aim, in this case report, is to highlight the role of CT urography in the early diagnosis of anatomical variations of the urogenital system and appropriate prevention of clinical progression.

Background: Endometriosis mainly occurs in female pelvic organs. Endometriosis in the kidney is extremely rare. Case presentation: We herein describe a case of a 19-year-old girl with occasional mild abdominal pain associated with an ectopic left kidney. SPECT-CT showed no abnormal radioactive distribution in the left pelvis, suggesting loss of function of the ectopic kidney. Laparoscopic left ectopic kidney resection was subsequently performed. Histopathology revealed endometriosis of the ectopic left kidney. Conclusions: In female patients with clinical manifestations of abdominal pain and gross hematuria, the possibility of renal endometriosis should be considered. [ABSTRACT FROM AUTHOR]

Flexible ureteroscopy (fURS) is a well-established procedure for treating multiple upper-urinary tract pathologies, particularly renoureteral lithiasis. Endoscopes have undergone significant advancements, including miniaturization, improved optics, and increased maneuverability. In addition, advancements in accessory instruments, such as the performance of laser fibers, guidewires, and extraction probes, have played a significant role in improving the overall performance of flexible ureteroscopy procedures. However, despite these advancements, unique circumstances can make achieving optimum results during flexible ureteroscopy challenging. These include congenital renal anomalies (horseshoe kidneys, ectopic kidneys, rotation anomalies), as well as the unique intrarenal anatomy (infundibulopelvic angle, infundibular length) or the specifications of the endoscope in terms of maneuverability (active and passive deflection). This review explored challenging scenarios during flexible ureteroscopy procedures in the pyelocaliceal system. [ABSTRACT FROM AUTHOR]

Abstract Background Cephalad renal ectopia is rare. Ectopic kidneys besides being prone to various pathologies are occasionally associated with other congenital abnormalities. To the best our knowledge, at the time of this writing, only ten cases of bilateral subdiaphragmatic renal ectopia had been reported. Case presentation: We present a rare case of bilateral subdiaphragmatic ectopic kidneys incidentally discovered during evaluation of the abdominal pain. In addition, our patient had associated anomalies of the liver, spleen and mesocardia. Conclusions Recognition of this condition is important for accurate diagnosis, surgical and/or intervention planning, as well as identifying other associated anomalies.

An ectopic kidney is often found inadvertently during CT, ultrasonography, MRI, or urologic physical examination. Ectopic kidneys usually occur in the pelvis. A pelvic ectopic kidney may be misinterpreted for a pelvic tumor by less experienced physicians and surgeons. We present an extremely rare case of ectopic kidney in the deep subcutaneous region of the abdominal wall and associated with the additional abnormality of spina bifida. MRI found an ectopic kidney but failed to identify ureteropelvic drainage. Diuretic renography with 99mTc-diethylenetriaminepentaacetic acid showed normal functioning and identified nonobstructive ureteropelvic drainage of the ectopic subcutaneous kidney. [ABSTRACT FROM AUTHOR]

One of the rarest anomalies of the renal collecting system is ureteral triplication along with the presence of a complex ureteral cyst. We describe an unusual case of a 37-year-old man who presented with fever and abdominal pain. Computed tomography urography (CTU) revealed triplication of the ureter in association with renal ectopia and a ureteral cyst which was complicated by calculi formation. Following treatment with antibiotics, the patient was managed by open ureterolithotomy with resection of the ureteral cyst and tailoring of the ureter.

Introduction: Renal ectopia also known as ectopic kidney is an embryological renal abnormality characterized by abnormal anatomical location of one or both kidneys. Kidneys can vary in number, shape, size, position, rotation and vascularization. These variations are of immense importance due to their susceptibility to trauma, infection and iatrogenic injury.We reported the first case of bilateral renal ectopia associated with a bilateral accessory renal artery, a retro-aortic left renal vein and a variation of the renal pelvis. Case report: During a routine dissection of a male cadaver, showing no trace of trauma or surgical scar in the abdomen, we observed a rare case of bilateral renal ectopia and bilateral accessory renal artery. The left renal vein was retro-aortic and anastomosed with the left common iliac vein. We also observed the right renal vein drained the testicular vein, the fusion of the major calyxes (formation of the renal pelvis) outside the right kidney. These observations are of paramount importance to interventional radiologists, nephrologists and vascular surgeons. Conclusion: Renal vasculature variations are common in the population and are important not only for surgeons, but for radiologists. Therefore, a good knowledge of the multiple variations of renal vessels and renal ectopia is important for urologists and radiologists when performing kidney surgeries in kidney donors and transplants, in the end to avoid otherwise to reduce the rate of haemorrhage by accidental lesions of the accessory arteries during surgeries involving the kidneys in particular.

Abstract 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: Human kidneys develop from intermediate mesoderm, in three successive stages; pronephros, mesonephros and metanephros. The definitive kidney, which is derived from metanephros occupy sacral region initially, which ascent later, reaching the adult position by 9th week. It receives blood supply from different sources during its development and ascent. Accessory renal arteries are widely studied in adults while the same in fetuses is an understudied topic. Aims and Objectives: The present study was carried out to find out the percentage of accessory renal arteries in fetuses and associated positional congenital anomalies with it. Materials and Methods: Fifty fetuses of gestational ages ranged from 14 to 40 weeks were collected from the department of Obstetrics and Gynecology, with prior permission from Head of the department and Dean. Results: The study showed a higher percentage of accessory renal arteries in fetuses (30%) and in 2 out of 50 fetuses dissected (4%), accessory renal arteries were found to be associated with positional anomalies. One was a case of bilateral ectopic kidney whereas the other was a horseshoe kidney (HSK). Lobulation were seen in most of the kidneys and 3 cases of bilateral accessory renal arteries without any obvious anomalies were noted. Conclusion: As the advancement in prenatal diagnostic techniques, screening for accessory renal arteries in fetuses can provide a clue for associated congenital renal anomalies. Knowledge on vascular variation in fetuses will help pediatric surgeons in planning abdominal surgeries in new born and children of early ages, where vascular anatomy may be different than in adults.

The thoracic kidney is the rarest form of renal ectopia. Furthermore, it is usually asymptomatic and discovered incidentally. It is seen as a mass in the posterior mediastinum or juxta-diaphragmatic on chest radiography. A computed tomography scan or magnetic resonance imaging is usually needed for a definitive diagnosis. The thoracic kidney typically exits the retroperitoneal space through the foramen of Bochdalek.

Background: Situs inversus totalis is a very rare congenital anatomical variation, in which all thoracic and abdominal organs are right-left inverted. This condition is associated with an increased risk of organ malformations including ectopic kidney, which is a very rare combination.Case Presentation: A 56-year-old male presented with colicky left iliac pain associated with nausea, vomiting, and irritative lower urinary symptoms. The patient has a medical history of recurrent lower urinary infections and a family history of situs inversus totalis. Radiological images demonstrated dextrocardia, situs inversus totalis of all the abdominal organs, and an ectopic pelvic kidney on the left side, with 4 stones inside it. Left nephrectomy was performed due to extensive renal damage. At discharge and during follow-up, the patient's condition was satisfactory and stable.Conclusions: The ectopic kidney may present diagnostic and therapeutic challenges when associated with situs inversus. [ABSTRACT FROM AUTHOR]

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.

Bilateral ectopic pelvic kidney is an exceptional finding and it may be associated rarely with other abnormalities of the kidney such as uretero-pelvic junction syndrome. We report a case of bilateral ectopic pelvic kidney revealed by left flanc pain with a left uretero-pelvic junction syndrome and a right ureteral duplication proved by computed tomography. A MAG3 renal scintigraphy was performed confirming the obstructive uretero-pelvic junction. An open left Anderson-Hynes pyeloplasty was performed without objectifying, preoperatively, any extrinsic compression without any postoperative complication. This case emphasizes on the possibility of the reconstructive management in such cases.

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.

Intrathoracic ectopic kidney is a very rare form of ectopic kidney. It is usually asymptomatic and diagnosed incidentally during chest investigations. A 3-year-old boy was admitted to our center with respiratory distress and fever recurrent since birth. An abdominal ultrasound, chest X-ray, intravenous pyelogram, and computed tomography revealed a right-sided intrathoracic ectopic kidney. A thoracotomy approach was used. The right intrathoracic ectopic kidney was reduced to the abdominal cavity and a diaphragm fold was performed. Subsequently, severe hydronephrosis and obstruction as a result of ureteral folding required repeated surgical intervention.

Background: Cephalad renal ectopia is rare. Ectopic kidneys besides being prone to various pathologies are occasionally associated with other congenital abnormalities. To the best our knowledge, at the time of this writing, only ten cases of bilateral subdiaphragmatic renal ectopia had been reported. Case presentation: We present a rare case of bilateral subdiaphragmatic ectopic kidneys incidentally discovered during evaluation of the abdominal pain. In addition, our patient had associated anomalies of the liver, spleen and mesocardia. Conclusions: Recognition of this condition is important for accurate diagnosis, surgical and/or intervention planning, as well as identifying other associated anomalies. [ABSTRACT FROM AUTHOR]

Background: Human kidneys develop from intermediate mesoderm, in three successive stages; pronephros, mesonephros and metanephros. The definitive kidney, which is derived from metanephros occupy sacral region initially, which ascent later, reaching the adult position by 9th week. It receives blood supply from different sources during its development and ascent. Accessory renal arteries are widely studied in adults while the same in fetuses is an understudied topic. Aims and Objectives: The present study was carried out to find out the percentage of accessory renal arteries in fetuses and associated positional congenital anomalies with it. Materials and Methods: Fifty fetuses of gestational ages ranged from 14 to 40 weeks were collected from the department of Obstetrics and Gynecology, with prior permission from Head of the department and Dean. Results: The study showed a higher percentage of accessory renal arteries in fetuses (30%) and in 2 out of 50 fetuses dissected (4%), accessory renal arteries were found to be associated with positional anomalies. One was a case of bilateral ectopic kidney whereas the other was a horseshoe kidney (HSK). Lobulation were seen in most of the kidneys and 3 cases of bilateral accessory renal arteries without any obvious anomalies were noted. Conclusion: As the advancement in prenatal diagnostic techniques, screening for accessory renal arteries in fetuses can provide a clue for associated congenital renal anomalies. Knowledge on vascular variation in fetuses will help pediatric surgeons in planning abdominal surgeries in new born and children of early ages, where vascular anatomy may be different than in adults. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

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.

Schimke Immuno-Osseous Dysplasia (SIOD) (MIM:242900) is an ultra-rare autosomal recessive pan-ethnic pleiotropic disease. Typical findings of this syndrome are steroid-resistant nephrotic syndrome, cellular immunodeficiency and spondyloepiphyseal dysplasia and facial dysmorphism. Biallelic variants in the SMARCAL1 gene cause SIOD. The five-and-half-year-old female patient was evaluated because of short stature, dysmorphism, hypercalcemia, hypophosphatemia and elevated FSH levels. Karyotype analysis and array-CGH testing were normal. Clinical Exome Sequencing was performed via next-generation sequencing to analyze genes associated with hypophosphatemia. No pathogenic variant was detected. The subsequent detection of proteinuria during her follow-up for cross-fused ectopic left kidney ultimately facilitated the diagnosis of SIOD, although no obvious spondyloepiphyseal dysplasia was detected. Re-analysis of CES revealed a novel homozygous c.2422_2427+9delinsA pathogenic variant in the SMARCAL1 . One hundred twenty-five SIOD cases from 38 literature reporting SMARCAL1 gene pathogenic variants were reviewed to investigate whether hypercalcemia, hypophosphatemia and elevated FSH levels had been previously reported in SIOD patients. This review revealed that this was the first time these findings had been reported in a SIOD patient. This report expands not only the phenotypic but also genotypic spectrum of SIOD.

A congenital defect in the diaphragm, known as a Bochdalek hernia (BH), is a condition that allows herniation of the abdominal viscera into the thorax. BH is the most common type of congenital diaphragmatic hernia (CDH) and is typically detected on the left side. An ectopic kidney is a rare condition. An intra-thoracic ectopic kidney is an extremely uncommon condition. In adult patients, the presence of BH with an intra-thoracic kidney is extremely uncommon and is often a finding discovered unintentionally. A 51-year-old male patient presented to the outpatient unit of the pulmonology department. He stated that he had been suffering symptoms such as coughing, wheezing, and breathing difficulties for one year. A chest X-ray showed a well-defined radio-opaque lesion in the lower left zone. A computed tomography (CT) scan of the chest demonstrated a defect in the posterolateral region of the left hemidiaphragm, as well as herniation of the left kidney and retroperitoneal fat in the left hemithorax. The intra-thoracic ectopic kidney was found to be normal in size and showed normal attenuation and enhancement, with the contrast being promptly excreted into the pelvicalyceal system during CT urography. Due to the hernia's small size and lack of abnormalities on CT urography, the patient was recommended a conservative treatment. A follow-up examination was performed on the patient annually. Throughout the follow-up period, there was not a single episode of kidney-related issues. To avoid unwanted image-guided biopsies and surgical procedures, it is imperative to look for intra-thoracic kidneys in patients presenting with a thoracic mass or an elevated hemi-diaphragm., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Sree Balaji Medical College and Hospital Institutional Human Ethical Committee issued approval 002/SBMC/IHEC/2023/1728. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, P et al.)

Introduction: Robot-assisted laparoscopic pyeloplasty (RALP) has been increasingly utilized in the treatment of pediatric ureteropelvic junction obstruction (UPJO) with reported success rates of >95%. Complex renal anatomy can make some cases challenging to reconstruct., Objective: To evaluate outcomes of children undergoing RALP with aberrant renal anatomy and compare it to those with simple renal anatomy., Methods: An IRB approved prospective registry was queried to retrospectively identify all patients who underwent robotic pyeloplasty at our institution from 2012 to 2022. Patients undergoing re-do pyeloplasty were excluded. Complex anatomy was defined as horseshoe kidney, ectopic/pelvic kidney, duplex collecting system, fully bifid renal pelvis and severe malrotation (≥180°). A comparative analysis of baseline demographics, pre-operative clinical/radiological characteristics, intra and post-operative details, and long-term success was performed between those patients with complex anatomy and those without., Results: Of 405 total robotic pyeloplasty's, 375 patients (378 total pyeloplasty; 353 simple, 22 complex) met inclusion criteria. 27 re-do were excluded from analysis. The complex pyeloplasty cohort included 9 horseshoe kidneys, 8 duplex collecting systems, 3 ectopic/pelvic kidneys and 2 kidneys with severe malrotation. There was no difference in age (58 vs 31 months; p = 0.38), procedure time (203 vs 207 min; p = 0.06), length of stay (1.4 vs 1.3 days; p = 0.99), or success (91.6% vs 100%; p = 0.24) between the simple and complex groups. Etiology of obstruction differed significantly between groups - high insertion was more common (3.9% vs 18.2%, p = 0.02) and intrinsic narrowing was less common (60.1% vs 36.4%, p = 0.04) in patients with complex anatomy. A multivariate logistic regression was adjusted for age, gender, etiology of obstruction, preoperative differential renal function and post-operative complications and found no difference in success between complex and simple RALP., Discussion: The findings showed no significant differences in age, procedure time, length of hospital stay, or success rates between the two groups. Specifically, the success rates were 91.6% for the complex group and 100% for the simple group (p = 0.24), indicating comparable efficacy. However, the etiology of obstruction varied significantly, with high ureteral insertion more common in the complex anatomy group (18.2% vs. 3.9%, p = 0.02) and intrinsic narrowing less common (36.4% vs. 60.1%, p = 0.04). Despite these differences, multivariate logistic regression, adjusted for confounders, confirmed no difference in success rates between the groups., Conclusion: RALP is a safe and efficacious approach in patients with complex anatomy with success rates comparable to index patients. High ureteral insertion does appear to be more common in patients with complex anatomy undergoing pyeloplasty., Competing Interests: Conflict of interest No financial disclosures or conflicts of interest., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

An ectopic kidney is often found inadvertently during CT, ultrasonography, MRI, or urologic physical examination. Ectopic kidneys usually occur in the pelvis. A pelvic ectopic kidney may be misinterpreted for a pelvic tumor by less experienced physicians and surgeons. We present an extremely rare case of ectopic kidney in the deep subcutaneous region of the abdominal wall and associated with the additional abnormality of spina bifida. MRI found an ectopic kidney but failed to identify ureteropelvic drainage. Diuretic renography with 99m Tc-diethylenetriaminepentaacetic acid showed normal functioning and identified nonobstructive ureteropelvic drainage of the ectopic subcutaneous kidney., (© 2024 by the Society of Nuclear Medicine and Molecular Imaging.)

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.