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Urolithiasis represents the most frequent complication of horseshoe kidneys. All known approaches for stone disease management have been reported in these patients, including Extracorporeal Shockwave Lithotripsy, ureteroscopy, Percutaneous Nephrolithotomy, open surgery and laparoscopy. The purpose of this systematic review is to compare Percutaneous Nephrolithotomy with other least-invasive procedures in patients with horseshoe kidneys. Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Guidelines, PubMed®, Scopus® and Cochrane® primary databases were systematically screened, from inception to 29 May 2023. Both prospective and retrospective comparative studies including patients with horseshoe kidneys and urolithiasis, who underwent Percutaneous Nephrolithotomy and other treatments for stones management, were eligible. Non comparative studies and case series were also included. A total of 10 studies met inclusion criteria and were included in final qualitative synthesis. In total 583 patient underwent Percutaneous Nephrolithotomy and other treatments for stone management in included studies. Stone free rates ranged from 57.1% to 88% for Percutaneous Nephrolithotomy, from 55.6% to 100% for ureteroscopy, from 27.3% to 83.3% for Extracorporeal Shockwave Lithotripsy and was 100% for pyelolithotomy and 71.4% for Percutaneous Nephrolithotomy in combination with ureteroscopy. Although percutaneous Nephrolithotomy was associated with more complications when compared to other treatment modalities, most of them were Grade I or Grade II ones, according to the Clavien-Dindo Classification System. Management of urolithiasis can be efficiently and safely accomplished in almost all cases of horseshoe kidneys. Extracorporeal Shockwave Lithotripsy, ureteroscopy, Percutaneous Nephrolithotomy and pyelolithotomy all represent excellent choices for the treatment of stones in these patients, being feasible, efficient and safe. Percutaneous Nephrolithotomy was associated with the highest stone-free rates, but also with the highest complication rates. Access should be ideally performed via the upper poles. Optimal stone management in patients with horseshoe kidneys depends on stone burden, stone location and surgeon's preference. [ABSTRACT FROM AUTHOR]

Background: Horseshoe kidney is the most common renal fusion anomaly, and Wilms tumor is the most frequent renal malignancy in children. The occurrence of Wilms tumor in association with horseshoe kidney is a scarce anomaly. However, the arising of a teratoid type, which is a rare variant of Wilms tumor in a horseshoe kidney, is exceptionally unique. Case presentation: This report presents a 5-year-old male admitted with horseshoe kidney involved by a large heterogeneous calcified mass that was diagnose on biopsy as Wilms tumor blastemal dominant. According to the local and regional extension and metastatic tumor in the lungs, the patient underwent neoadjuvant chemotherapy and then surgery. Post-operative pathologic findings confirmed the diagnosis of teratoid Wilms tumor. Conclusions: The occurrence of renal anomalies associated with a malignancy might be more frequent in the clinical environment. There are numerous differential diagnoses for renal tumors and masses, but the possibility of exceptional anomalies should not be denied, and clinicians should be prepared for these occasions. Although studies propose that chemotherapy has a trivial effect on teratoid Wilms tumors, it is essential to evaluate the tumor for any possibility of regression in non-teratoid regions before proceeding to upfront tumoral resection. [ABSTRACT FROM AUTHOR]

The upper urinary tract is the most common human organ system affected by congenital anomalies. A Horseshoe kidney is a fusion anomaly, it can be described as a fusion across the midline of 2 distinct functioning kidneys. The incidence of renal tumors in a Horseshoe kidney is higher than in the normal population.We present a 60-year-old male patient with a history of Horseshoe kidney and a diagnosis of clear cell renal cell carcinoma who underwent a combined therapeutic approach, guided by interventional radiology. This approach involved selective transarterial embolization and microwave ablation. Three months after surgery and with abdominal MRI follow-up, there is evidence of a non-viable tumor, indicating a favorable response to the intervention.

Abstract Background Horseshoe kidney is the most common renal fusion anomaly, and Wilms tumor is the most frequent renal malignancy in children. The occurrence of Wilms tumor in association with horseshoe kidney is a scarce anomaly. However, the arising of a teratoid type, which is a rare variant of Wilms tumor in a horseshoe kidney, is exceptionally unique. Case presentation This report presents a 5-year-old male admitted with horseshoe kidney involved by a large heterogeneous calcified mass that was diagnose on biopsy as Wilms tumor blastemal dominant. According to the local and regional extension and metastatic tumor in the lungs, the patient underwent neoadjuvant chemotherapy and then surgery. Post-operative pathologic findings confirmed the diagnosis of teratoid Wilms tumor. Conclusions The occurrence of renal anomalies associated with a malignancy might be more frequent in the clinical environment. There are numerous differential diagnoses for renal tumors and masses, but the possibility of exceptional anomalies should not be denied, and clinicians should be prepared for these occasions. Although studies propose that chemotherapy has a trivial effect on teratoid Wilms tumors, it is essential to evaluate the tumor for any possibility of regression in non-teratoid regions before proceeding to upfront tumoral resection.

Gorham-Stout disease is a rare condition characterized by the massive osteolysis replaced with vascular or lymphatic proliferation and fibrous tissue. We report the case of a 13-year-old boy complaining of occasional lower back pain. Spinal X-ray showed scoliosis and pelvic asymmetry, CT and MRI revealed multiple osteolysis replaced by soft tissue without osteogenesis in lumbar vertebrae, sacrum, iliac bone, ischium and acetabulum and horseshoe kidney. Laboratory and clinical findings excluded all other potential causes of osteolysis and the patient was diagnosed as Gorham-Stout disease, although no lymphangioma or hemangioma were found in the specimen of right iliac bone. The report shows an unusual, multifocal Gorham-Stout disease in a 13-year-old boy with horseshoe kidney, suggesting that the typical imaging findings and raising awareness of the disease can facilitate timely diagnosis for the disease.

Abstract Background We previously described the KINSSHIP syndrome, an autosomal dominant disorder associated with intellectual disability (ID), mesomelic dysplasia and horseshoe kidney, caused by de novo variants in the degron of AFF3. Mouse knock-ins and overexpression in zebrafish provided evidence for a dominant-negative mode of action, wherein an increased level of AFF3 resulted in pathological effects. Methods Evolutionary constraints suggest that other modes-of-inheritance could be at play. We challenged this hypothesis by screening ID cohorts for individuals with predicted-to-be damaging variants in AFF3. We used both animal and cellular models to assess the deleteriousness of the identified variants. Results We identified an individual with a KINSSHIP-like phenotype carrying a de novo partial duplication of AFF3 further strengthening the hypothesis that an increased level of AFF3 is pathological. We also detected seventeen individuals displaying a milder syndrome with either heterozygous Loss-of-Function (LoF) or biallelic missense variants in AFF3. Consistent with semi-dominance, we discovered three patients with homozygous LoF and one compound heterozygote for a LoF and a missense variant, who presented more severe phenotypes than their heterozygous parents. Matching zebrafish knockdowns exhibit neurological defects that could be rescued by expressing human AFF3 mRNA, confirming their association with the ablation of aff3. Conversely, some of the human AFF3 mRNAs carrying missense variants identified in affected individuals did not rescue these phenotypes. Overexpression of mutated AFF3 mRNAs in zebrafish embryos produced a significant increase of abnormal larvae compared to wild-type overexpression further demonstrating deleteriousness. To further assess the effect of AFF3 variation, we profiled the transcriptome of fibroblasts from affected individuals and engineered isogenic cells harboring + / + , KINSSHIP/KINSSHIP, LoF/ + , LoF/LoF or KINSSHIP/LoF AFF3 genotypes. The expression of more than a third of the AFF3 bound loci is modified in either the KINSSHIP/KINSSHIP or the LoF/LoF lines. While the same pathways are affected, only about one third of the differentially expressed genes are common to the homozygote datasets, indicating that AFF3 LoF and KINSSHIP variants largely modulate transcriptomes differently, e.g. the DNA repair pathway displayed opposite modulation. Conclusions Our results and the high pleiotropy shown by variation at this locus suggest that minute changes in AFF3 function are deleterious.

Objective: We assessed the safety and feasibility of minimally invasive robot-assisted surgery for horseshoe kidney (HSK). Method: A prospectively maintained data set for consecutive patients undergoing robotic kidney surgery was reviewed for patients with HSK. Cases were performed by experienced robotic surgeons, across two high-volume centres between 2016 and 2020. Results: Seven patients underwent robotic surgery for HSK, comprising three partial nephrectomies for renal masses, one nephroureterectomy and three benign nephrectomies for non-functioning kidneys. The median age was 53 (interquartile range (IQR) = 47–60) years and median body mass index (BMI) was 25 (IQR = 25–26.5). Median console time was 120 (IQR = 118–215) minutes and median estimated blood loss was 150 (IQR = 125–250) mL. The median pre- and post-operative estimated glomerular filtration rate (eGFR) was 76 (IQR = 72–90) and 71 (IQR = 60–81), respectively. There were no higher-grade complications (Clavien–Dindo III–IV) and one Clavien–Dindo grade II complication (wound infection treated with IV antibiotics). Median length of stay (LOS) was 2 days and there were no 30-day readmissions. Negative margins were achieved in 75% of tumour resections. Conclusion: We report one the largest series of robot-assisted surgery on HSK. Robotic surgery is safe and feasible for HSK in centralised high-volume centres with acceptable perioperative outcomes. Established benefits of minimally invasive surgery, such as reduced LOS and low complication rates, were demonstrated. Level of evidence: 4 [ABSTRACT FROM AUTHOR]

Background: Robot-assisted partial nephrectomy (RAPN) has become the standard treatment for small renal tumors, including highly complex cases. However, applying RAPN to renal tumors in the horseshoe kidney (HSK) is clinically challenging due to malformations and complex blood supply. Herein, we present two cases of RAPN in patients with HSK treated using selective artery clamping methods. Case Reports: A 61-year-old male with a 15 mm renal tumor located on the upper pole of the right HSK was referred to our Department. The patient underwent RAPN via the transperitoneal approach, following a threedimensional computed tomography (3D-CT) assessment. Additionally, before surgery, we confirmed which renal arteries would be clamped in surgery by examining the kidney regions supplied by each renal artery. The second patient referred to our Department, a 45-year-old male, had a 46 mm renal tumor located on the isthmus of the HSK. His tumor received blood supply from two renal arteries, with the bilateral collecting systems converging and forming a ureter on 3D-CT. The patient underwent RAPN through an intraperitoneal approach in the semi-lateral position, with port placement lower than in standard RAPN. Pathological examinations revealed clear-cell renal cell carcinoma with negative surgical margins in both cases. Both patients had no recurrences or metastases at 53 and 13 months postsurgery, respectively. Conclusion: We present cases successfully treated with RAPN with selective artery clamping methods for HSK using 3D-CT without encountering complications, even in isthmus tumors. [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]

Background: We previously described the KINSSHIP syndrome, an autosomal dominant disorder associated with intellectual disability (ID), mesomelic dysplasia and horseshoe kidney, caused by de novo variants in the degron of AFF3. Mouse knock-ins and overexpression in zebrafish provided evidence for a dominant-negative mode of action, wherein an increased level of AFF3 resulted in pathological effects. Methods: Evolutionary constraints suggest that other modes-of-inheritance could be at play. We challenged this hypothesis by screening ID cohorts for individuals with predicted-to-be damaging variants in AFF3. We used both animal and cellular models to assess the deleteriousness of the identified variants. Results: We identified an individual with a KINSSHIP-like phenotype carrying a de novo partial duplication of AFF3 further strengthening the hypothesis that an increased level of AFF3 is pathological. We also detected seventeen individuals displaying a milder syndrome with either heterozygous Loss-of-Function (LoF) or biallelic missense variants in AFF3. Consistent with semi-dominance, we discovered three patients with homozygous LoF and one compound heterozygote for a LoF and a missense variant, who presented more severe phenotypes than their heterozygous parents. Matching zebrafish knockdowns exhibit neurological defects that could be rescued by expressing human AFF3 mRNA, confirming their association with the ablation of aff3. Conversely, some of the human AFF3 mRNAs carrying missense variants identified in affected individuals did not rescue these phenotypes. Overexpression of mutated AFF3 mRNAs in zebrafish embryos produced a significant increase of abnormal larvae compared to wild-type overexpression further demonstrating deleteriousness. To further assess the effect of AFF3 variation, we profiled the transcriptome of fibroblasts from affected individuals and engineered isogenic cells harboring + / + , KINSSHIP/KINSSHIP, LoF/ + , LoF/LoF or KINSSHIP/LoF AFF3 genotypes. The expression of more than a third of the AFF3 bound loci is modified in either the KINSSHIP/KINSSHIP or the LoF/LoF lines. While the same pathways are affected, only about one third of the differentially expressed genes are common to the homozygote datasets, indicating that AFF3 LoF and KINSSHIP variants largely modulate transcriptomes differently, e.g. the DNA repair pathway displayed opposite modulation. Conclusions: Our results and the high pleiotropy shown by variation at this locus suggest that minute changes in AFF3 function are deleterious. [ABSTRACT FROM AUTHOR]

Abdominal aortic aneurysm with concomitant horseshoe kidney is exceedingly rare. Although open repair was previously the treatment, endovascular aortic repair has become an increasingly popular option. In the current endovascular era, complex aortic pathologies are treatable with selective use of multiple advanced techniques. We present a unique case involving complex endovascular repair of abdominal aortic aneurysm complicated by presence of horseshoe kidney with central renal artery in a patient where an open approach was prohibited.

Objective: This study aims to report a renal tumor in a horseshoe kidney successfully treated with an open left nephrectomy. Case(s) Presentation: A 3 years old girl with a lump in the left abdomen and intermittent hematuria was referred to our hospital. Whole abdominal contrast CT scan showed the presence of a left renal mass in a horseshoe kidney that infiltrates the left renal artery and is attached to the spleen. The patient underwent preoperative chemotherapy followed by surgical resection. Discussion: In a horseshoe kidney, renal tumors are uncommon. The limited kidney mobilization and access to the renal hilum and the abnormal and highly variable vasculature make surgical treatment of a tumor in a horseshoe kidney extremely difficult. Conclusion: Open surgery continues to be the treatment of choice for horseshoe kidney tumors due to their anatomical complexity, particularly in cases where the tumor is difficult to eradicate. Keywords: Renal tumor, horseshoe kidney, surgical management.

Objective: We aimed to evaluate the clinical features, accompanying surgical problems, and renal development outcomes during nephrological follow-up in patients with horseshoe kidney (HSK). Method: We retrospectively reviewed the medical records of 24 patients with HSK who underwent surgery in our pediatric surgery clinic between 2015 and 2023. Results: Sixteen of the patients were boys and eight were girls. The mean age was 77.3 (1.5-192) months. The mean follow-up period was 48 (12-120) months. HSK was found incidentally in 10 patients and diagnosed prenatally in seven patients. Eleven children had bladder dysfunction and six patients were diagnosed with spina bifida. Thirteen of the patients were found to have frequent urinary tract infections. Anderson-Hynes pyeloplasty for ureteropelvic junction stenosis, ureteroneocystostomy for vesicourethral reflux (VUR) and ureterovesical stricture, vesicourethral injection sting for VUR, upper pole heminephrectomy for nonfunctioning dual system, Holmium laser-guided lithotripsy and unilateral nephrectomy, isthmusectomy and contralateral kidney nephron-sparing surgery were required due to bilateral Wilms tumor. During the postoperative follow-up, three patients continued to have urinary tract infections, five developed renal scarring, three developed proteinuria and four developed hypertension. A total of three patients, including two patients operated for Wilms tumor, had elevated cystatin-C levels and developed chronic kidney disease (CKD). Conclusion: Patients with HSK should be followed up for urologic abnormalities that may require surgery and postoperative urinary tract infection and scar formation in the kidneys. In our study, it was demonstrated that surgical intervention alone cannot prevent CKD.

A 52‐year‐old, Japanese man presented to the hospital with a complaint of anal bleeding, and detailed examination resulted in a diagnosis of locally advanced rectal cancer. The patient underwent total neoadjuvant therapy followed by short‐course radiation therapy and consolidation chemotherapy, which provided a partial response. After preoperative contrast‐enhanced computed tomography showed a horseshoe kidney, robot‐assisted, precise, laparoscopic, low anterior resection with D3 dissection and ileostomy construction was performed. The horseshoe renal isthmus was elevated surrounding the inferior mesenteric artery, and the left ureter and seminal vessels ran in front of the kidney. The hypogastric nerve traveled ventral to the horseshoe kidney. With robotic surgery, it was possible to perform more precise surgery while recognizing vascular and nerve anatomy in a rectal cancer patient with a horseshoe kidney due to good three‐dimensional visibility and articulated forceps manipulation. [ABSTRACT FROM AUTHOR]

Introduction Historically, percutaneous nephrolithotomy (PCNL) in horseshoe kidney (HSK) patients has been performed in the prone position. Nevertheless, thanks to the spread of the supine PCNL technique for patients with urinary stones and normal renal anatomy, some retrospective studies have already reported on supine PCNL and HSK, showing the effectiveness and safety of the procedure. Herein we report our experience with supine PCNL in a subset of patients with urolithiasis. Material and methods Prospective data were collected for all HSK patients who underwent supine PCNL at our institution from June 2016 to June 2023. Stone volume was reported as the volume of a single stone or the sum of the volumes of multiple stones on computed tomography (CT) images. Patients were reported to be stone-free if there were no stones on postoperative non-contrast CT (NCCT) exam. Peri-/postoperative complications were reported according to the Clavien-Dindo classification system. The primary endpoint of the study was stone-free rate (SFR) and the secondary endpoints were Clavien-Dindo complications Grade I or higher. Results A total of 35 patients met the inclusion criteria and were enrolled in the study. Forty-eight procedures were analyzed. SFR was 72.9% at 1-month follow-up. In 11 out of 48 procedures (22.9%) Clavien-Dindo Grade I-II complications were recorded. In one case Clavien-Dindo Grade IIIa complication was observed. Conclusions In this prospective study of 35 HSK patients who underwent 48 procedures, supine PCNL was safe and effective, with minimal morbidity. [ABSTRACT FROM AUTHOR]

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Objective: To analyze the outcomes of single-session bilateral PCNL in a horseshoe kidney patient with bilateral staghorn stones in terms of safety and effectiveness. Case(s) Presentation: A 64-year-old woman with bilateral staghorn stones on HSK. She had an anesthesia status of ASA 2, with type 2 diabetes mellitus (uncontrolled with oral medication), 47% of ejection fraction from echocardiography and her body mass index was 21.7 kg/m2. A single session of bilateral PCNL was performed, with 150 minutes of surgery and total blood loss of ± 50 cc. A 4.7 Fr DJ stent was placed on the right kidney, a 5 Fr ureteral catheter was left in the left kidney, and a 16 Fr foley catheter was placed as a right and left nephrostomy. Control plain abdominal x-ray showed no residual stones. Postoperatively, the patient was hospitalized for 3 days, there were no significant complaints and discharged in good condition. Discussion: The procedure was performed in the prone position for two reasons. First, is to avoid intestinal injury during puncture and dilatation, the second is to simplify the positioning of the C arm, as well as the endourology monitor, and the other instruments. Conclusion: Single-session bilateral PCNL for HSK patients with bilateral staghorn stones, in our experience, is quite safe and effective. Keywords: Horseshoe kidney, single-session bilateral percutaneous nephrolithotomy (PCNL), staghorn stone.

Introduction: Horseshoe kidney (HK) is an anatomical variant characterised by abnormalities in the position, rotation, and vascular supply of the kidney, with functioning renal masses on both sides of the vertebral column fused together at the isthmus. Due to the altered pattern of kidney vasculature, endovascular aortic repair for aortic abdominal aneurysm (AAA) in the presence of HK requires vascular anatomy specific planning. Report: A 68 year old male, with multiple comorbidities, presented with an asymptomatic AAA and HK. The kidney vasculature was characterised by the presence of three arteries: two arising laterally at the same level and a third polar artery arising from below. The polar artery was 6 mm in diameter and larger than the other two; therefore, in order to preserve this artery, a custom-made device with a single side branch was implanted below the main renal arteries. A balloon expandable covered stent was used to complete the side branch into the polar renal artery. The follow-up computed tomography angiography revealed a successful outcome, with total aneurysm exclusion, branched graft patency, no endoleak, and unchanged renal function. Discussion: This case report shows a possible surgical solution for a case of HK with AAA and the importance of accurate endovascular planning. Large polar arteries, if present, need to be preserved, and custom-made devices in the modern endovascular era permit that. This approach could represent the best option for complicated patients.

Neuroendocrine tumors of the kidney are exceedingly rare. We report the first case of robotic-assisted partial nephrectomy for such tumors in horseshoe kidneys. A 65-year-old woman was incidentally found to have a 27 mm renal mass in the isthmus of her horseshoe kidney during computed tomography. Based on contrast-enhanced computed tomography results, we initially suspected renal cell carcinoma originating from the horseshoe kidney. Subsequently, robotic-assisted partial nephrectomy with isthmus transection was performed. Intraoperatively, we adjusted the port position for camera insertion and the patient's positioning to facilitate better visualization for dorsal isthmus and vessel dissection. Pathological examination and immunohistochemical analysis revealed a well-differentiated neuroendocrine tumor. Therefore, robotic-assisted partial nephrectomy is a safe and effective approach for managing neuroendocrine tumors in the isthmus of horseshoe kidneys. Given the nonspecific clinical presentation of renal neuroendocrine tumors and their rarity, the optimal management of these tumors remains controversial. [ABSTRACT FROM AUTHOR]

Abstract Introduction In this case report, we demonstrate our technique of a retroperitoneal laparoscopic heminephrectomy for a T1b right hilar tumor in a horseshoe kidney. Case presentation A 77-year-old Vietnamese woman presented to the hospital because of right flank pain. On presentation, her serum creatinine was 0.86 mg/dL and glomerular filtration rate was 65.2 mL/minute/1.73 m2. According to her renal scintigraphy, glomerular filtration rates of the right and left moieties were 24.2 and 35.5 mL/minute, respectively. Computed tomography imaging demonstrated a 5.5 × 5.0 cm solid hilar mass with a cT1bN0M0 tumor stage was in the right moiety. After discussion, the patient elected a minimally invasive surgery to treat her malignancy. The patient was placed in a flank position. We used Gaur’s balloon technique to create the retroperitoneal working space, and four trocar ports were planned for operation. Three arteries were dissected, including two arteries feeding the right moiety, one artery feeding the isthmus, and one vein, which was clipped and divided by Hem-o-lok. The isthmusectomy was performed with an Endostapler. Consequently, the ureter was clipped and divided. Finally, the whole right segment of the horseshoe kidney was mobilized and taken out via the flank incision. Results The total operative time was 250 min with an estimated blood loss of 200 mL. The patient's serum creatinine after surgery was 1.08 mg/dL, and glomerular filtration rate was 49.47 mL/minute/1.73 m2. The patient was discharged on postoperative day #4 without complication. Final pathologic examination of the tumor specimen revealed a Fuhrman grade II clear cell renal cell carcinoma, capsular invasion, with negative surgical margins. After a three-month follow-up, the serum creatinine was 0.95 mg/dL, and glomerular filtration rate was 57.7 mL/minute/1.73 m2. Local recurrence or metastasis was not detected by follow-up computed tomography imaging. Conclusions Retroperitoneal laparoscopic heminephrectomy is a safe and feasible technique for patients with renal cell carcinoma in a horseshoe kidney and may be particularly useful in low income settings without access to robotic technology.

Abstract Background Pheochromocytoma is a neuroendocrine tumor, and its treatment is dependent on surgical resection. Due to the wide availability of cross-sectional imaging, pheochromocytomas are commonly seen as small tumors less than 10 cm in size and are mostly treated with minimally invasive surgery. Their concomitant presence with horseshoe kidney or other anatomical and vascular anomalies is rare. Herein, we present a surgically complex giant pheochromocytoma case who underwent an open left radical adrenalectomy. Case presentation A 41-year-old Hispanic female presented with a 12 × 8 cm left hypervascular adrenal mass, pelvic horseshoe kidney, and severely dilated large left retro-aortic renal vein which branched into a left adrenal vein, congested left ovarian vein, and left uterine plexus. She was managed with insulin and metformin for uncontrolled diabetes with an A1c level of 9% and doxazosin for persistent hypertension. Clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and normetanephrine. The pre-operative ACTH was within normal range with a normal dexamethasone suppression test and 24-hour urine free cortisol. The adrenalectomy of the highly aggressive adrenal mass was performed via open approach to obtain adequate surgical exposure. Due to the large size of the tumor and its significant involvement with multiple adjacent structures, coordination with multiple surgical teams and close hemodynamic monitoring by anesthesiology was required for successful patient outcomes including preservation of blood supply to the pelvic horseshoe kidney. The histopathological diagnosis was pheochromocytoma with negative surgical margins. The patient was followed at 1, 4, 12, and 24 weeks postoperatively. She had a normal postoperative eGFR and was able to discontinue antihypertensive and antidiabetic medications at four weeks. She had transient adrenal insufficiency, which resolved at five months. The horseshoe kidney was intact except for a minimal area of hypo-enhancement in the left superior renal moiety due to infarction, which was significantly improved at six months. Conclusion Our patient had a giant pheochromocytoma with anatomical variations complicating an already surgically challenging procedure. Nonetheless, with multiple provider collaboration, detailed pre-operative surgical planning, and meticulous perioperative monitoring, radical resection of the giant pheochromocytoma was safe and feasible with successful postoperative outcomes.

Verónica Judith Picos-Cárdenas,1 Evelia Leal-Ugarte,2 Juan Pablo Meza-Espinoza2 1Laboratorio de Genética, Facultad de Medicina, Universidad Autónoma de Sinaloa, Culiacán, Sinaloa, C. P. 80030, México; 2Facultad de Medicina Matamoros, Universidad Autónoma de Tamaulipas, Matamoros, Tamaulipas, C. P. 87300, MéxicoCorrespondence: Juan Pablo Meza-Espinoza, Facultad de Medicina Matamoros, Universidad Autónoma de Tamaulipas, Matamoros, Tamaulipas, México, Tel +528682044000, Email sirol1073@yahoo.com.mx

Introduction: In this case report, we demonstrate our technique of a retroperitoneal laparoscopic heminephrectomy for a T1b right hilar tumor in a horseshoe kidney. Case presentation: A 77-year-old Vietnamese woman presented to the hospital because of right flank pain. On presentation, her serum creatinine was 0.86 mg/dL and glomerular filtration rate was 65.2 mL/minute/1.73 m2. According to her renal scintigraphy, glomerular filtration rates of the right and left moieties were 24.2 and 35.5 mL/minute, respectively. Computed tomography imaging demonstrated a 5.5 × 5.0 cm solid hilar mass with a cT1bN0M0 tumor stage was in the right moiety. After discussion, the patient elected a minimally invasive surgery to treat her malignancy. The patient was placed in a flank position. We used Gaur's balloon technique to create the retroperitoneal working space, and four trocar ports were planned for operation. Three arteries were dissected, including two arteries feeding the right moiety, one artery feeding the isthmus, and one vein, which was clipped and divided by Hem-o-lok. The isthmusectomy was performed with an Endostapler. Consequently, the ureter was clipped and divided. Finally, the whole right segment of the horseshoe kidney was mobilized and taken out via the flank incision. Results: The total operative time was 250 min with an estimated blood loss of 200 mL. The patient's serum creatinine after surgery was 1.08 mg/dL, and glomerular filtration rate was 49.47 mL/minute/1.73 m2. The patient was discharged on postoperative day #4 without complication. Final pathologic examination of the tumor specimen revealed a Fuhrman grade II clear cell renal cell carcinoma, capsular invasion, with negative surgical margins. After a three-month follow-up, the serum creatinine was 0.95 mg/dL, and glomerular filtration rate was 57.7 mL/minute/1.73 m2. Local recurrence or metastasis was not detected by follow-up computed tomography imaging. Conclusions: Retroperitoneal laparoscopic heminephrectomy is a safe and feasible technique for patients with renal cell carcinoma in a horseshoe kidney and may be particularly useful in low income settings without access to robotic technology. [ABSTRACT FROM AUTHOR]

Background: Pheochromocytoma is a neuroendocrine tumor, and its treatment is dependent on surgical resection. Due to the wide availability of cross-sectional imaging, pheochromocytomas are commonly seen as small tumors less than 10 cm in size and are mostly treated with minimally invasive surgery. Their concomitant presence with horseshoe kidney or other anatomical and vascular anomalies is rare. Herein, we present a surgically complex giant pheochromocytoma case who underwent an open left radical adrenalectomy. Case presentation: A 41-year-old Hispanic female presented with a 12 × 8 cm left hypervascular adrenal mass, pelvic horseshoe kidney, and severely dilated large left retro-aortic renal vein which branched into a left adrenal vein, congested left ovarian vein, and left uterine plexus. She was managed with insulin and metformin for uncontrolled diabetes with an A1c level of 9% and doxazosin for persistent hypertension. Clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and normetanephrine. The pre-operative ACTH was within normal range with a normal dexamethasone suppression test and 24-hour urine free cortisol. The adrenalectomy of the highly aggressive adrenal mass was performed via open approach to obtain adequate surgical exposure. Due to the large size of the tumor and its significant involvement with multiple adjacent structures, coordination with multiple surgical teams and close hemodynamic monitoring by anesthesiology was required for successful patient outcomes including preservation of blood supply to the pelvic horseshoe kidney. The histopathological diagnosis was pheochromocytoma with negative surgical margins. The patient was followed at 1, 4, 12, and 24 weeks postoperatively. She had a normal postoperative eGFR and was able to discontinue antihypertensive and antidiabetic medications at four weeks. She had transient adrenal insufficiency, which resolved at five months. The horseshoe kidney was intact except for a minimal area of hypo-enhancement in the left superior renal moiety due to infarction, which was significantly improved at six months. Conclusion: Our patient had a giant pheochromocytoma with anatomical variations complicating an already surgically challenging procedure. Nonetheless, with multiple provider collaboration, detailed pre-operative surgical planning, and meticulous perioperative monitoring, radical resection of the giant pheochromocytoma was safe and feasible with successful postoperative outcomes. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Although anatomical and vascular abnormalities of the horseshoe kidney might be challenging, complete preoperative imaging evaluations and accurate organ‐sparing surgical planning can lead to much lower complications. Horseshoe kidney (HK) is one of the most common renal fusion anomalies. Renal carcinoids are rarely reported in HK patients. Here, we described a rare case of advanced right renal cell carcinoma (RCC) along with proximal left ureter stone in a 41‐year‐old man who presented with a complaint of turbid urine. Early blood tests revealed a blood urea nitrogen of 44 mg/dL and serum creatinine of 1.35 mg/dL. The urine analysis showed microscopic hematuria (6–8 RBCs) and few calcium oxalate crystals. The imaging evaluations revealed an HK anomaly with a solid mass on the right side and a 4 mm stone in the proximal left ureter. The findings suggested RCC which was confirmed by histopathology examination. Consequently, the patient was scheduled for an organ‐preserving open surgery of a right kidney tumor with concomitant left ureterolithotomy. The 16‐month follow‐up showed no urological complications, metastasis, or tumor proliferation. Although the anatomical and vascular abnormalities of HK might be challenging, organ‐sparing surgical treatment should be considered in feasibly resectable tumors. Complete preoperative imaging evaluations to identify the characteristics of HK, as well as accurate surgical planning, can lead to much lower complications. [ABSTRACT FROM AUTHOR]

To present a patient with horseshoe kidney and bilateral squamous cell carcinoma (SCC) which has not been reported so far. A 61-year-old woman presented with abdominal mass and recent episodes of gross hematuria. Imaging revealed malignant lesion of lower calyces of the right kidney and isthmus of horse-shoe kidney with midline crossing to the left side. Finally, the patient underwent bilateral enbloc radical nephroureterectomy and pathology evaluation was compatible with bilateral squamous cell carcinoma. This is the first report of bilateral SCC in horseshoe kidney which was managed via open enbloc radical nephroureterectomy.

Tigabu Daniel,1 Yonas Abera2 1Surgery Department, Hawassa University, Hawassa, Ethiopia; 2Surgery Department, Ethiopian Defense University, Addis Ababa, EthiopiaCorrespondence: Tigabu Daniel, Email danieltigabu96@gmail.comBackground: Aphallia (absent penis) is an extremely rare congenital anomaly happening in 1 in 30 million births worldwide. It can occur alone or in combination with other congenital anomalies.Case Presentation: A two-day-old neonate presented to Arba Minch General Hospital with an absent penis. The delivery was performed at home by a 34-year-old primigravida mother with no antenatal follow-up. As the mother described, the baby cried immediately after birth. The baby was sucking initially but failed to suck hours after delivery. The neonate died of neonatal distress syndrome after two hours of resuscitation.Conclusion: Aphallia is a complex, extremely rare congenital anomaly that is commonly associated with other congenital anomalies. Sometimes, it may be associated with a lethal anomaly and requires sophisticated workup to diagnose and treat grave anomalies early.Keywords: aphallia, penis, horseshoe kidney

Adugna Getachew Mideksa, Tolesa Yadeta Huluka, Masresha Solomon Dino, Mensur Mohammed Ahmed Department of Surgery Urology Division, Saint Paul’s Hospital Millennium Medical College, Addis Ababa, EthiopiaCorrespondence: Adugna Getachew Mideksa; Mensur Mohammed Ahmed, Email adulense@gmail.com; mensurm69@gmail.comAbstract: Retrocaval ureter is a rare congenital vascular anomaly described as the passage of the ureter behind the inferior vena cava (IVC) and then turning around the IVC to attain the final lateral position. The condition is usually associated with obstruction in the ipsilateral kidney, causing different degrees of hydronephrosis and complications associated with urinary stasis, such as stone formation. Imaging has a crucial role in the diagnosis and management of retrocaval ureter. CT urography may be the procedure of choice to confirm the diagnosis and avoid retrograde ureteropyelography. Indications for treatment include flank pain, recurrent infection, hydronephrosis, and stone formation due to obstruction. Surgical management is standard and can be done through either an open, laparoscopic, or robotic approach. In this case series, we are going to see two cases of retrocaval ureter in a 56-year-old male and a 14-year-old male child who presented with a right flank of less than a couple of months duration. The first case has an associated horseshoe kidney and a solitary secondary stone. Both cases were surgically managed with open ureteral division, relocation, and ureteroureterostomy. Both have uneventful post-operative follow-ups.Keywords: retrocaval ureter, circumcaval ureter, pre-ureteral vena cava, horseshoe kidney

Horseshoe kidney is the most common genitourinary tract fusion abnormality at a frequency of 1 in 400-800 live births. PUJ obstruction is observed in up to one-third of these cases. The clinical presentation of the patients present will be either asymptomatic or may include stomach discomfort, persistent UTIs, and calculi. The PUJ obstruction is managed by surgery which can be carried out using a robotic, laparoscopic, or open technique. We hereby present a case of 16 hear old female with right sided PUJ obstruction due to high ureteric insertion in a horseshoe kidney which was effectively managed by open Anderson-Hynes Pyeloplasty.

A 48-year-old female presented with complaints of right sided vague abdominal pain associated with abdominal lump.. Computed tomography revealed horseshoe kidney with Bosniak IIF cystic lesion arising from the interpolar region of right segment of the horseshoe kidney (HSK). In view of the persistent pain, patient underwent cyst excision. The final histopathology diagnosis was mucinous cystadenoma. On follow-up at 6 months post surgery, there was no recurrence. The histopathology is very unique and rare with only a few cases recorded in literature. The inner lining of the cyst wall composed of columnar epithelium secreting mucin and at some places by transitional epithelium. This indicates that the origin of the mucinous tumour was likely to be from a sequestration of the renal pelvic urothelium in the renal parenchyma.

The most frequent genitourinary fusion abnormality described in literature is horseshoe kidney. Horseshoe kidney because of its abnormal anatomy is linked to malrotations, fluctuating blood flow, high insertion of the ureter, a tendency to develop ureteropelvic junction (PUJ) obstruction in up to onethird of patients, and these are all presentations of this condition. One-third of the patients with horseshoe kidney present with either stone disease or PUJ obstruction. In our case report, we discuss the treatment of a 38-year-old male patient who had been complaining of abdominal pain for the past few years, was found to have a horseshoe kidney and presented to us in urosepsis due to gigantic large calculus in right segment of horseshoe kidney and acute renal failure which was managed initially on emergent basis by insertion of percutaneous nephrostomy tube into the right segment followed by 2 months later by open right sided nephropyelolithotomy. The patient was relieved of all his symptoms at 3rd month follow-up and resumed his daily activities.

Percutaneous Nephrolithotomy (PCNL) has been shown as a safe and effective method for treating nephrolithiasis in horseshoe kidney patients. We report the first X-ray Free PCNL with Alken Telescopic Metal Dilator (ATMD) in horseshoe kidney.A 58-Year-Old female was diagnosed with left large staghorn stone and horseshoe kidney treated with X-ray Free PCNL with ATMD.X-ray Free PCNL with ATMD is safe and effective nephrolithiasis lithotripsy for staghorn stone in Horseshoe Kidney. This technique is the first of its kind and possibly has become an excellent alternative for urological centers without access to fluoroscopy.

Key Clinical Message Although anatomical and vascular abnormalities of the horseshoe kidney might be challenging, complete preoperative imaging evaluations and accurate organ‐sparing surgical planning can lead to much lower complications. Abstract Horseshoe kidney (HK) is one of the most common renal fusion anomalies. Renal carcinoids are rarely reported in HK patients. Here, we described a rare case of advanced right renal cell carcinoma (RCC) along with proximal left ureter stone in a 41‐year‐old man who presented with a complaint of turbid urine. Early blood tests revealed a blood urea nitrogen of 44 mg/dL and serum creatinine of 1.35 mg/dL. The urine analysis showed microscopic hematuria (6–8 RBCs) and few calcium oxalate crystals. The imaging evaluations revealed an HK anomaly with a solid mass on the right side and a 4 mm stone in the proximal left ureter. The findings suggested RCC which was confirmed by histopathology examination. Consequently, the patient was scheduled for an organ‐preserving open surgery of a right kidney tumor with concomitant left ureterolithotomy. The 16‐month follow‐up showed no urological complications, metastasis, or tumor proliferation. Although the anatomical and vascular abnormalities of HK might be challenging, organ‐sparing surgical treatment should be considered in feasibly resectable tumors. Complete preoperative imaging evaluations to identify the characteristics of HK, as well as accurate surgical planning, can lead to much lower complications.

Background: Robot-assisted pyeloplasty is widely used in pediatric surgery because of its well-known advantages over open or laparoscopic surgery. The aim is to explore our experience and evaluate the achievements we have made. Methods: We evaluated patients undergoing robotic pyeloplasty from January 2016 to November 2021, including those who presented with a ureteropelvic junction obstruction associated with other anomalies of the kidney. The parameters examined were: age, weight, associated renal malformations, conversion rate, operative time, and intra- and postoperative complications. Results: Of 39 patients, 7 (20%) were included, of whom 5 (71%) were male and 2 (29%) were female. The mean age at surgery was 84 months (range 36–180 months), and the mean weight at surgery was 24.4 kg (range 11–40 kg). In five (71%) patients the ureteropelvic junction obstruction (UPJO) was left-sided and in two (29%) it was right-sided. In four (57%) cases, UPJO was associated with a horseshoe kidney, right-sided in one (25%) patient, and left-sided in the other three (75%). A 180° rotation of the kidney was present in one (14%) patient. Nephrolithiasis was present in two (29%) patients. The mean operative time was 160 min (range 140–240 min). The average bladder catheter dwell time was 1 day (range 2–3 days), while the average abdominal drainage dwell time was 2 days (range 2–4 days). The mean hospitalization time was 4 days (range 3–9 days). On average, after 45 days (range 30–65) the JJ ureteral stent was removed cystoscopically. No intraoperative complications were reported, while one case of persistent macrohematuria with anemia requiring blood transfusion occurred postoperatively. Conclusions: Ureteropelvic junction obstruction might be associated with other congenital urinary tract anomalies such as a duplicated collecting system, horseshoe kidney, or pelvic kidney. These kinds of malformations can complicate surgery and require more attention and accuracy from the surgeon. Our experience shows that, with regards to the robotic learning curve required for pyeloplasty, the treatment of the ureteropelvic junction in these situations does not present insurmountable difficulties nor is burdened by complications. The application of robot-assisted surgery in pediatric urology makes difficult pyeloplasties easier. [ABSTRACT FROM AUTHOR]

Horseshoe kidney is a rare congenital anomaly resulting from the fusion of the lower pole leading to medial and caudal deviation of kidneys that pose challenges to successful stone clearance. We evaluated the various patient- and treatment-related factors that can impact successful stone clearance. A total of 34 patients with 36 renal moieties with urolithiasis in horseshoe kidneys from 2015 to 2021 were included with a median age of 38 years (11–56 years). The mean calculus size was 39.27 mm (21–70 mm). Twenty-two patients had left, 10 had right, and 2 had bilateral disease. Twelve patients (33%) had previous interventions done. As per Guy stone score (GSS), 14 patients had grade IV, 16 patients had grade III, and 4 patients had grade II disease. Of these patients, 22 underwent unilateral percutaneous nephrolithotomy (PCNL), 2 underwent bilateral PCNL, 6 had pyelolithotomy, 2 had right nephrectomy, and 2 patients underwent shockwave lithotripsy (SWL). Six patients undergoing pyelolithotomy had complete clearance. In PCNL, infra-costal puncture was done uniformly. Complete clearance was seen in 12 out of 16 with superior calyceal access (75% stone-free rates, SFR), 2 out of 8 with middle calyceal puncture (25% SFR), and both the cases with inferior calyceal puncture (100% SFR). Factors affecting stone clearance are as follows: GSS, i.e., grade < IV vs ≥ IV (71.4 vs 50%, P = 0.42), STONE score < 12 vs ≥ 12 (83.3 vs 28.5%, P = 0.048), stone size < 40 vs ≥ 40 mm (50 vs 71.4%, P = 0.42). Our analysis showed that stone clearance was independent of mode of access, GSS, or stone size, although STONE score was a predictor of successful outcomes in PCNL. [ABSTRACT FROM AUTHOR]

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.

Abstract Background Horseshoe kidney is one of the most common congenital renal fusion anomalies and is characterized by abnormalities in the position, rotation, vascular supply, and ureteral anatomy of the kidney. When performing surgery for colorectal cancer in patients with horseshoe kidneys, anatomical identification is important to avoid organ injuries. Several reports on surgery for colorectal cancer with horseshoe kidneys have described the usefulness of three-dimensional (3D) computed tomography (CT) angiography for detecting abnormalities in vascular supply. However, few reports have focused on the prevention of ureteral injury in surgery for colorectal cancer with horseshoe kidney, despite abnormalities in the ureteral anatomy. Here, we report a case in which laparoscopic sigmoid colon resection for sigmoid colon cancer with a horseshoe kidney was safely performed using fluorescent ureteral catheters. Case presentation A 60-year-old Japanese man presented to our hospital testing positive for fecal occult blood. Colonoscopy revealed sigmoid colon cancer, and CT confirmed a horseshoe kidney. The 3D-CT angiography showed aberrant renal arteries from the aorta and right common iliac artery, and the left ureter passed across the front of the renal isthmus. A fluorescent ureteral catheter was placed in the left ureter before the surgery to prevent ureteral injury. Laparoscopic sigmoid colon resection with D3 lymph node dissection was performed. The fluorescent ureteral catheter enabled the identification of the left ureter that passed across the front of the renal isthmus and the safe mobilization of the descending and sigmoid colon from the retroperitoneum. The operative time was 214 min, with intraoperative bleeding of 25 mL. The patient’s postoperative course was good: no complications arose and she was discharged on the seventh postoperative day. Conclusion In patients with horseshoe kidney, the use of fluorescent ureteral catheters and 3D-CT angiography enables safer laparoscopic surgery for colorectal cancer. We recommend the placement of fluorescent ureteral catheters in such surgeries to prevent ureteral injury.

The purpose of this article is to highlight an innovative technique in the surgical management of aortic aneurysms in the presence of a horseshoe kidney. The technique involves an anterograde aortic bypass from the distal thoracic aorta to the major renal artery with the primary advantage to a significant reduction in renal ischemia time. [ABSTRACT FROM AUTHOR]

Objective: The percutaneous nephrolithotomy (PCNL) in Horseshoe kidneys (HSK) is usually performed in the prone position, allowing entry through the upper pole and providing good access to the collecting system. However, in patients with normal kidney anatomy, the supine position is reliable and safe in most cases, but it is unknown whether the supine position is adequate in patients with HSK. The purpose of this study was to describe the results of PCNL in HSK in three different surgical institutions and to evaluate the impact of supine position during surgery, comparing pre-operative and post-operative data, complications, and stone status after surgery. Material and Methods: Between 2017 and 2022, a total of 10 patients underwent percutaneous renal surgery for stone disease in HSK. All patients were evaluated pre- and post- operatively with non-contrast CT. we evaluated patients (age and gender), stones characteristics (size, number, side, site and density ), and outcomes. The change in haemoglobin, hematocrit, creatinine and eGFr were assessed between the most recent preoperative period and the first postoperative day. Procedure success was defined as stone-free or presence of ≤4 mm fragments (Clinically Insignificant residual Fragments – CIrF). Complications were registered and classified according to Clavien-dindo Grading System, during the 30 - day postoperative period and Clavien scores ≥ 3 were considered as major complications. Statistical analysis was performed using “r 4.2.1” software, with a 5% significance level. we also compared pre-operative and post-operative data using “wilcoxon signedrank test”. Results: No statistical difference was observed between preoperative and post-operative renal function data. At one post operative day CT scan, an overall success rate of 100% was registered. 9/10 patients were completely free from urolithiasis (stone-free rate: 90%), while 1/10 patients had ≤4 mm residual stone fragments (CIrF rate: 10%). No cases of intraoperative complications were registered. Post-operative complications were reported in 1/10 patients. A patient developed urosepsis (defined as SIrS with clinical signs of bacterial infections involving urogenital organs - Clavien-dindo Grade II) after procedure, and was treated with intravenous antibiotic therapy successfully. Conclusions: This study shows that in patients with HSK mini- PCNL in supine position allows to achieve good stone free rate with a very low morbidity. According to our series, the described technique for PCNL in HSK should be an option. Nevertheless these results must be confirmed by further studies.

Horseshoe kidney (HSK) has an incidence of about 1 in 400 or 0.25% of the general population and is seen more commonly in males as compared to women in a ratio of 2:1. Usually, Horseshoe kidney would have two normal ureters each draining separately into the bladder. We present a rare and interesting case of Horseshoe kidney with unilateral single ureter and associated anorectal malformation.

Introduction: Neoadjuvant nivolumab and cabozantinib in locally advanced renal cell carcinoma in a horseshoe kidney is a novel therapeutic approach in the preoperative setting. Methods: We report a case of a 52-year old male who presented with a large inoperable tumor of the horseshoe kidney and achieved major partial radiologic response after neoadjuvant therapy with nivolumab and cabozantinib leading to radical resection of the tumor. The patient remains tumor free on the subsequent follow-up and his renal function is only mildly decreased. The systemic treatment was complicated by hepatotoxicity leading to early nivolumab withdrawal. Results: Currently, the combination therapy based on immune checkpoint inhibitors and tyrosine kinase inhibitors represents the treatment of choice in treatment-naïve patients with metastatic renal cell carcinoma in any prognostic group. The neoadjuvant treatment approach is being tested in prospective clinical trials and results are eagerly awaited. Renal cell carcinoma in a horseshoe kidney is an uncommon finding that is always challenging. Additionally, management guidance in this patient population is lacking. In some patients neoadjuvant therapy could be the only way to preserve kidney function. The initial treatment strategy should be individualized to patient needs aiming at the radical resection of the primary tumor as the only chance of getting the tumor under control in the long term. Conclusion: Herein, we highlight the feasibility of neoadjuvant systemic therapy with nivolumab and cabozantinib allowing the subsequent performance of radical tumor resection with negative margins in a patient with advanced renal cell carcinoma in a horseshoe kidney, removing the primary tumor while sparing the patient from lifelong dialysis. [ABSTRACT FROM AUTHOR]

objective: The percutaneous nephrolithotomy (PCNL) in Horseshoe kidneys (HSK) is usually performed in the prone position, allowing entry through the upper pole and providing good access to the collecting system. However, in patients with normal kidney anatomy, the supine position is reliable and safe in most cases, but it is unknown whether the supine position is adequate in patients with HSK. The purpose of this study was to describe the results of PCNL in HSK in three different surgical institutions and to evaluate the impact of supine position during surgery, comparing pre-operative and post-operative data, complications, and stone status after surgery. Material and Methods: Between 2017 and 2022, a total of 10 patients underwent percutaneous renal surgery for stone disease in HSK. All patients were evaluated pre- and post- operatively with non-contrast CT. we evaluated patients (age and gender), stones characteristics (size, number, side, site and density ), and outcomes. The change in haemoglobin, hematocrit, creatinine and eGFr were assessed between the most recent preoperative period and the first postoperative day. Procedure success was defined as stone-free or presence of ≤4 mm fragments (Clinically Insignificant residual Fragments – CIrF). Complications were registered and classified according to Clavien-dindo Grading System, during the 30 - day postoperative period and Clavien scores ≥ 3 were considered as major complications. Statistical analysis was performed using “r 4.2.1” software, with a 5% significance level. we also compared pre-operative and post-operative data using “wilcoxon signedrank test”. results: No statistical difference was observed between preoperative and post-operative renal function data. At one post operative day CT scan, an overall success rate of 100% was registered. 9/10 patients were completely free from urolithiasis (stone-free rate: 90%), while 1/10 patients had ≤4 mm residual stone fragments (CIrF rate: 10%). No cases of intraoperative complications were registered. Post-operative complications were reported in 1/10 patients. A patient developed urosepsis (defined as SIrS with clinical signs of bacterial infections involving urogenital organs - Clavien-dindo Grade II) after procedure, and was treated with intravenous antibiotic therapy successfully. Conclusions: This study shows that in patients with HSK miniPCNL in supine position allows to achieve good stone free rate with a very low morbidity. According to our series, the described technique for PCNL in HSK should be an option. Nevertheless these results must be confirmed by further studies. [ABSTRACT FROM AUTHOR]

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Background: Horseshoe kidney is a well-known congenital anomaly and the most common anomaly of the upper urinary tract. This condition is rarely associated with anomalous inferior vena cava (IVC). Polycystic horseshoe kidney is a very rare occurrence and however IVC anomalies common with polycistic disease are an increasingly recognized risk factor for iliofemoral deep venous thrombosis. Method: We present a case of 75-year-old patient with recurrent deep vein thrombosis (DVT) of right leg as a result of compression of large horseshoe kidney cysts in double inferior vena cava. Results: Large renal cyst were successful treated percutaneously punctured for the relief of compression and received injection of acidic solutions of 95% ethanol under ultrasound guidance for prevention against re-accumulation of cyst fluid. Conclusion: Percutaneous aspiration with ultrasound guidance with injection of sclerosing solutions as a relatively simple procedure and can be the method of choice for treatment of renal cysts. Also, any recurrent deep vein thrombosis on lower extremity requires additional evaluation in the form of an ultrasound or multidetector computed tomography examination of the abdomen. [ABSTRACT FROM AUTHOR]