Off-the-Shelf Strategy for Endovascular Aneurysm Repair of Ectopic, Dominant, Renal Artery Originating From the Aortic Bifurcation: Case Report.

INTRODUCTION: An ectopic renal artery originating from the aortic bifurcation can strongly interfere with surgical or endovascular intervention in the infrarenal aorta. CASE: We present an infrarenal symptomatic aneurysm with a right ectopic/pelvic kidney associated with 1 renal artery originating from the aortic bifurcation. RESULT: During an endovascular aortic repair (EVAR) procedure, the dominant ectopic right kidney was perfused by an iliac branch device (IBD) used as an iliac extension. However, out of instructions for use (IFU), this is an "off-the-shelf" resolution for this type of rare case. CONCLUSION: The use of an "off-the-shelf" IBD to perform an EVAR with an ectopic renal artery, in patients unfit for open surgery, is a potentially valuable option.

A rare case of a combination of ectopic kidney and medial arcuate ligament syndrome: a case report.

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.

Monochorionic Twin Discordance for Horseshoe Lung and Tricuspid Atresia.

Background: The horseshoe lung is a congenital malformation in which the bases of the right and the left lung are fused. Case report: We describe a monochorionic twin gestation with malformation discordance. The abnormal twin had a horseshoe lung with hypoplasia of the right lung, tricuspid atresia, cleft lip, and a pelvic right kidney. Conclusion: The discordance of anomalies in this monochorionic twin suggests that a postzygotic mutation, epigenetic change, or environmental factors may be responsible for these malformations.

Embryology of the urogenital tract; a practical overview for urogynecologic surgeons.

INTRODUCTION AND HYPOTHESIS: Urinary tract anomalies are one of the most common birth defects. Nevertheless, they prove challenging to diagnose as a result of variable presenting symptoms. We aimed to perform a review of urogenital tract development, highlight common congenital upper urinary tract anomalies encountered by urogynecologists and tools to facilitate diagnosis. METHODS: Multiple searches were performed utilizing resources such as PubMed and the TriHealth library database to access publications related to embryology of the urinary tract and urinary tract anomalies. Each citation was reviewed. RESULTS: Congenital urinary tract anomalies account for up to 20% of all birth defects and occur more often in females. The true incidence of these malformations is unknown as some can remain clinically insignificant throughout life. In addition, patients may present with non-specific complaints such as urinary tract infections, nephrolithiasis or urinary incontinence. Therefore, unsuspected anomalies pose a risk of delayed diagnosis and potential injury during urogynecologic surgery. Imaging modalities such as computed tomography or magnetic resonance imaging are the most common diagnostic tests. Management and treatment options range from observation to surgical resection with the goal of optimizing long-term functionality and prevention of chronic sequelae. CONCLUSION: Patients with urinary tract anomalies can present with vague complaints often encountered by urogynecologists. It is crucial to understand the embryologic development of urinary tract anomalies to help facilitate diagnosis and guide care within the office and operating room setting.

Chromosomal microarray findings in pregnancies with an isolated pelvic kidney.

Objective To examine the risk for abnormal chromosomal microarray analysis (CMA) results among fetuses with an apparently isolated pelvic kidney. Methods Data from all CMA analyses performed due to an isolated pelvic kidney reported to the Israeli Ministry of Health between January 2013 and September 2016 were retrospectively obtained. Risk estimation was performed comparing the rate of abnormal observed CMA findings to the general population risk, based on a systematic review encompassing 9272 cases and on local data of 5541 cases. Results Of 120 pregnancies with an isolated pelvic kidney, two gain-of-copy number variants suggesting microduplication syndromes were demonstrated (1.67%). In addition, three variants of unknown significance were detected (2.5%). Conclusion The risk for clinically significant CMA findings among pregnancies with an isolated single pelvic kidney was not significantly different compared to both control populations. The results of our study question the practice of routine CMA analysis in fetuses with an isolated pelvic kidney.

Locally advanced carcinoma of the cervix associated with pelvic kidney treated with intensity-modulated radiotherapy: Overcoming a therapeutic challenge.

The simultaneous occurrence of carcinoma of the cervix and pelvic kidney is rare. As the pelvic kidney occupies the conventional radiation portal for carcinoma of the cervix, treatment of these patients with radiation presents a therapeutic challenge. A 48-year-old stage IIIB cervical carcinoma patient with an incidental diagnosis of pelvic kidney was treated with radical chemoradiotherapy using intensity-modulated radiotherapy with concurrent weekly cisplatin, followed by intracavitary radiotherapy. The bilateral kidney dose was restricted within a tolerance limit of 16.6 Gy. At the 18-month follow-up, the patient was disease free and had no deterioration in kidney function. Intensity-modulated radiotherapy provided the necessary means for delivering radical radiation doses in this case scenario with adequate sparing of the kidney.

Prenatal diagnosis and outcomes for fetuses with suspected pelvic kidney.

OBJECTIVE: Evaluation of demographic characteristics and postnatal outcomes of patients with suspected fetal pelvic kidney diagnosis followed in a tertiary center. METHODS: This retrospective study was conducted in Ankara Bilkent City Hospital perinatology clinic between 2020-2023. Demographic features, prenatal ultrasound findings, and postnatal outcomes were reported. RESULTS: Pelvic kidney localization was on the left in 11(55 %) patients, on the right in 7(35 %), and bilateral in 2(10 %) patients in prenatal ultrasonography. The gender of the 12(60 %) fetuses were male and 8(40 %) of them were female. The pelvic kidney was an isolated finding in 8(40 %) fetuses, additional findings were present in the remaining 12(60 %) fetuses. Pelvic kidney was confirmed postnatally by ultrasound in all 18 fetuses. However two cases with prenatal ultrasound findings resulted in intrauterine fetal demise and the final diagnosis could not be confirmed as the parents refused autopsia. Cases were divided into 3 groups according to postnatal follow-up duration as 0-12 months (n = 7), 12-24 months (n = 7) and 24-44 months (n = 4). Atrial septal defect was the most common accompanying abnormality in the postnatal period (n = 4). Smaller kidney size (n = 7), vesicoureteral reflux (n = 3), and impaired renal function (n = 3) were the most common postnatal complications. CONCLUSION: Pelvic kidney can be diagnosed in fetal abnormality screening ultrasound and postnatal follow-up should be performed closely for the assessment of renal functions.

A congenital renal anomaly concomitant with a vascular emergency: a case of an ectopic pelvic kidney accompanied by aortoiliac aneurysms.

Introduction and importance: Ectopic pelvic kidneys are an extremely rare congenital renal system anomaly. This embryological occurrence transpires between the 4th and 8th gestational weeks, and is seen in 1 in every 2100-3000 births. Moreover, research articles indicate an incidence of ectopic pelvic kidneys ranging from 0.033 to 0.047%. The co-occurrence of this anomaly with an abdominal aortic aneurysm is an even rarer finding. Case presentation: We report a rare case involving a 62-year-old Middle Eastern male with a right ectopic pelvic kidney. The condition initially manifested as vague abdominal discomfort in the periumbilical region. The pain evolved into a continuous, localized, and insidious sensation. This was escorted by the perception of a pulsatile abdominal mass. Preoperative radiology illustrated a right ectopic pelvic kidney with concomitant aortoiliac aneurysms. Clinical discussion: Via a successful surgical intervention, the abdominal aortic aneurysms were repaired via synthetic Dacron grafts and the renal perfusion to the ectopic pelvic kidney was secured. Moreover, the aneurysmal wall underwent a full histopathological analysis, and the results of which indicated an atherosclerotic cause. Conclusion: Ectopic pelvic kidneys are an exceptionally rare congenital anomaly, especially when coupled with life-threatening co-occurrences like an abdominal aortic aneurysm. The need for timely surgical interventions is critical, and this topic requires comprehensive documentation as a guide for surgical professionals. This unique case is the first documented instance in the country. It emphasizes the fundamental role of proper intraoperative techniques in repairing aneurysmal conditions while preserving the function of the ectopic kidney.

Complex case of ureteropelvic junction obstruction with bifid renal pelvis in ectopic kidney: A rare combination.

INTRODUCTION: Renal pelvis bifidity, ectopic pelvic kidney, and ureteropelvic junction obstruction are rare urinary tract anomalies resulting from embryological developmental variations. Their coexistence in one kidney is exceedingly uncommon. CASE PRESENTATION: An 18-year-old male with no prior medical history presented with right-sided lumbar pain. A CT scan revealed bilateral hydronephrosis, with the left kidney being malrotated and ectopically positioned in the pelvis. Dynamic renal scintigraphy confirmed bilateral ureteropelvic junction obstruction. Surgical management involved laparoscopic pyeloplasty for the right UPJ and open surgery for the left ectopic kidney with bifid pelvis. DISCUSSION: The combination of pelvic kidney, renal pelvis bifidity, and bilateral ureteropelvic junction obstruction is exceptionally rare. Diagnosis often occurs incidentally or when symptoms related to these anomalies emerge. Imaging and dynamic renal scintigraphy play crucial roles in diagnosis. Individualized surgical management is essential for positive outcomes. CONCLUSION: This case highlights the need for individualized management in complex urological cases involving rare anatomical variations. Surgeon experience and a comprehensive understanding of such anomalies are crucial for successful outcomes.

Extrarenal calyces in a pelvic kidney with ureteropelvic junction obstruction in an adult male - A rare case report.

Extrarenal calyces (ERC) is a rare renal anomaly. First described in 1925, and till now, >60 cases have been reported worldwide. The association of ERC in ectopic kidneys with ureteropelvic junction obstruction (UPJO) is a very rare presentation. We encountered a case of an adult male with ERC in a pelvic kidney with UPJO, in which the dilated ERC mimicked the ureter and created intraoperative confusion.

Laparoscopic Approach for Reconstructive and Ablative Procedures in Ectopic Pelvic Kidneys: A Challenge in 8 Cases.

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.

The role of retrograde intrarenal surgery in kidney stones of upper urinary system anomalies.

INTRODUCTION: Fusion, pelvic, and duplicated urinary tract anomalies of the kidney are rarely seen. There might be some difficulties in the stone treatment, in the administration of extracorporeal shockwave lithotripsy (ESWL), retrograde intrarenal surgery (RIRS), percutaneous nephrolithotomy (PCNL), and laparoscopic pyelolithotomy procedures in these patients due to the anatomical variations in kidneys with anomalies. AIM: To evaluate RIRS results on patients with upper urinary tract anomalies. MATERIALS AND METHODS: Data of 35 patients with horseshoe kidney, pelvic ectopic kidney, and double urinary system in two referral centers were reviewed retrospectively. Demographic data, stone characteristics, and postoperative characteristics of the patients were evaluated. RESULTS: The mean age of patients (n=35, 6 women and 29 men) was 50 years. Thirty-nine stones were detected. The total mean stone surface area in all anomaly groups was found to be 140 mm2, and the mean operative time was 54.7±24.7 minutes. The rate of using ureteral access sheath (UAS) was very low (5/35). Eight patients needed auxiliary treatment after the operation. The residual rate, which was 33.3% in the first 15 days, decreased to 22.6% in the third month follow-ups. Four patients had minor complications. In patients with horseshoe kidney and duplicated ureteral systems, it was observed that the risk factor increasing the presence of residual stones was the total stone volume. CONCLUSIONS: RIRS for kidneys with low and medium stone volume anomalies is an effective treatment method with high stone-free and low complication rates.

Robot-assisted laparoscopic surgery with a fluorescent near-infrared ray ureteral catheter for a rectal cancer patient with pelvic kidney: A case report.

An 85-year-old woman presented with a stomachache after a meal and was admitted to the previous clinic. Multi-detector computed tomography (CT) of the abdomen showed wall thickening in the rectum and right ectopic pelvic kidney. Colonoscopy revealed a mass at the rectum, and a biopsy showed adenocarcinoma. CT showed no lymphadenopathy or distant metastasis. Hartmann's procedure with fluorescent near-infrared ray ureteral catheters was used to avoid causing urinary injury. Robotic surgery was performed while checking the route of the ureter in near-infrared mode. The patient was discharged on postoperative day 14 without specific complications. This case appears to be the first of robot-assisted laparoscopic surgery for a rectal cancer patient with pelvic kidney.

Hydronephrotic pelvic kidney mimicking urinary retention in an 18-year-old male.

We present a case of a hydronephrotic pelvic kidney in an 18-year-old male reporting about inability to void. Ultrasound showed a hypoechogenic mass mimicking a full urinary bladder. Anticipating urinary retention, a foley was inserted but no urin could be aspirated. Imaging showed a hydronephrotic pelvic kidney with no relevant function obstructing the urinary bladder and the contralateral ureter. Nephrectomy was performed and postoperative course was uneventful. A hydronephrotic pelvic kidney is a rare but important differential diagnosis in young men reporting lower abdominal pain and inability to void.

Endovascular reconstruction of aortoiliac occlusive disease in a case with claudication and an ectopic pelvic kidney.

INTRODUCTION AND IMPORTANCE: Ectopic kidney is a rare anomaly with an incidence of about 1 in 2500 birth in the population. Concurrency of pelvic kidney and iliac occlusion is rare, and the treatment is challenging because the pelvic kidney is associated with an atypical blood supply. CASE PRESENTATION: We reported a 68-year-old man with aortoiliac stenosis, right pelvic kidney, and high-risk cardiovascular comorbidities. He presented with the right lower extremity claudication that has been present for four years. Computed tomography angiograms showed total occlusion of right common and external iliac arteries. CLINICAL DISCUSSION: The old method for treating iliac artery stenosis is open surgery, which needs cross-clamping of the aorta. During this procedure, the chance of renal thrombosis and ischemia is high. Thus, the patient underwent an endovascular angioplasty that was done successfully for the patient without any disturbances in kidney function. CONCLUSION: Traditional treatment of iliac artery stenosis, especially in patients with high-risk cardiovascular comorbidities, may be associated with complications. Endovascular intervention is a safe and effective approach for treating aortoiliac occlusion in patients with ectopic pelvic kidneys. More research and case series are needed to review the results and compare the success rate of this method versus open surgery.

Development of subcapsular hematoma in the pelvic kidney during peripheral angioplasty.

Pelvic kidney is a mostly asymptomatic pathology resulting from failure of the kidneys to superior migration in fetal development. Herein, we report a 47-year-old female patient who presented with intermittent claudication in her right leg at 100 m. Significant stenosis was detected in the right superficial femoral artery and popliteal artery by computed tomography angiography. Peripheral angioplasty was performed for the treatment of consecutive lesions in the right lower extremity. Severe back pain developed during the procedure. Postprocedure computed tomography angiography showed a 35 mm wide subcapsular hematoma surrounding the pelvic kidney. The patient was followed up with conservative treatment on the first day. However, the next day, the patient's hemoglobin values decreased, and the pain persisted, thus angiography was performed. In the pelvic arteriography, an arteriovenous fistula was observed in the artery supply to the upper pole of the pelvic kidney. The fistula was closed with endovascular coil embolization. The patient who had no decrease in hemoglobin and no symptoms was discharged three days later. It is necessary to pay attention to the pelvic kidney during peripheral angiography, and it should be kept in mind that rare complications such as renal subcapsular hematoma may develop.

"Anomalías en la posición renal". Kidney position abnormalities.

Kidney development is a complex process that begins during the fifth to ninth weeks of life. Different types of renal congenital anomalies exist; however, they are rare and usually asymptomatic. Renal ectopia is a rare malformation that affects 0.01-0.05% of patients. Of all the locations, the most frequent is the pelvic (55%). We present a case of a 21-year-old man with bilateral renal ectopia.

Incidentalomas Among Healthy Nephrology Fellow Volunteers at POCUS Workshops: A Case Series.

A radiographic incidental finding (sometimes called an incidentaloma) is defined as a structure that is unintentionally found during an exam for an unrelated indication. The increased use of routine abdominal imaging is associated with a rising incidence in incidentalomas of the kidney 1. In one meta-analysis, 75% of renal incidentalomas were benign 2. However, the overall prevalence of incidental carcinomas is low at 0.2% 3. With the growing uptake of POCUS, healthy volunteers for clinical demonstrations may find themselves with new findings despite a lack of symptoms 4. Having an incidentaloma discovered during the course of a nephrology POCUS workshop is a unique experience. Herein we report our experiences of having incidentalomas discovered during the course of POCUS demonstrations.

Type 4 Ileal Atresia and Anorectal Malformation in a Neonate: A Rare Association.

Anorectal malformations and jejunoileal atresias are common causes of intestinal obstruction in neonates. Both have their own set of associated anomalies but it is extremely rare for the two to co-occur in the same patient. In this case report, we detail and describe this unusual incidence in a three-day-old neonate who was provisionally diagnosed with a case of simple imperforate anus. Per-op findings showed a type 4 Ileal atresia and an ileostomy was then created. Our experience stresses the importance of timely antenatal diagnosis and the presence of a high index of suspicion when encountering such patients. Both factors are key and crucial in determining the outcome and post-op course of the patient.

Bilaterally ectopic pelvic kidneys masquerading as horseshoe kidney in Fanconi anemia.

Fanconi anemia (FA) is a genetic disease associated with the risk of different congenital malformations, bone marrow failure, and predisposition to cancer. Congenital abnormalities of the kidney and urinary tract are not infrequent in FA with renal ectopia being one of them. The incidence of the bilateral pelvic ectopic kidney is restricted to only a few reported cases; however, its association with FA has never been reported in the literature. We present a case of Fanconi anemia in a young girl with apparently fused kidneys on a 99mTc-DMSA planar scan which was confirmed to be bilateral pelvic kidneys on hybrid cross-sectional imaging.