Variants in the degron of AFF3 are associated with intellectual disability, mesomelic dysplasia, horseshoe kidney, and epileptic encephalopathy.

The ALF transcription factor paralogs, AFF1, AFF2, AFF3, and AFF4, are components of the transcriptional super elongation complex that regulates expression of genes involved in neurogenesis and development. We describe an autosomal dominant disorder associated with de novo missense variants in the degron of AFF3, a nine amino acid sequence important for its binding to ubiquitin ligase, or with de novo deletions of this region. The sixteen affected individuals we identified, along with two previously reported individuals, present with a recognizable pattern of anomalies, which we named KINSSHIP syndrome (KI for horseshoe kidney, NS for Nievergelt/Savarirayan type of mesomelic dysplasia, S for seizures, H for hypertrichosis, I for intellectual disability, and P for pulmonary involvement), partially overlapping the AFF4-associated CHOPS syndrome. Whereas homozygous Aff3 knockout mice display skeletal anomalies, kidney defects, brain malformations, and neurological anomalies, knockin animals modeling one of the microdeletions and the most common of the missense variants identified in affected individuals presented with lower mesomelic limb deformities like KINSSHIP-affected individuals and early lethality, respectively. Overexpression of AFF3 in zebrafish resulted in body axis anomalies, providing some support for the pathological effect of increased amount of AFF3. The only partial phenotypic overlap of AFF3- and AFF4-associated syndromes and the previously published transcriptome analyses of ALF transcription factors suggest that these factors are not redundant and each contributes uniquely to proper development.

Horseshoe kidney with teratoid type of Wilms tumor: a rare case report.

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.

Super Mario sign at somatostatin receptor PET/CT.

68Ga-DOTA NOC PET-CT imaging has been shown to have high accuracy for the evaluation of neuroendocrine tumours. We present the case of a 59-year-old male with well differentiated gastric neuroendocrine tumour (grade II) treated with surgery. 68Ga-DOTA NOC PET/CT was performed to rule out metastasis. 68Ga-DOTA NOC showed physiological uptake in the bilateral adrenal and horseshoe kidney appearing as the famous character Super Mario. There is no evidence of any abnormal somatostatin avid lesion.

Non-papillary prone percutaneous nephrolithotomy for renal abnormalities: single-institution experience.

PURPOSE: To evaluate the effectiveness and safety of non-papillary prone PCNL for the treatment of patients with renal abnormalities. METHODS: An observational retrospective cohort study including PCNL cases of patients with renal abnormalities was performed. The following inclusion criteria were applied: renal stones > 1.5 cm with maximal diameter, anatomical malformations of affected kidney (malrotated kidneys, horseshoe kidneys and kidneys with complete duplicated systems, medullary sponge kidney), patients treated with standard (30Fr) PCNL or mini-PCNL (22Fr). The lithotripsy was performed using the Lithoclast Master or the Lithoclast® Trilogy (EMS Medical, Nyon, Switzerland). RESULTS: Overall, 57 patients, 35 males, and 22 females with any renal malformation underwent non-papillary prone PCNL. Our study included 25 patients with horseshoe kidneys, 21 with malrotated kidneys, 9 with kidneys with duplicated pelvicalyceal systems and 2 with medullary sponge kidneys. The mean cumulative stone size was 36 ± 1.4 mm and most of the stones were in the lower calyceal group (36.9%) and in the pelvis (27.2%). The stone-free rate (SFR) was 84.2% and the mean hospitalization time was 2.7 ± 0.7 days. In total, postoperative complications were developed in six patients (10.5%), half of them presenting fever and the other half requiring blood transfusion (Grade II). CONCLUSION: The PCNL is the method of choice for treating large stones in anomalous kidneys. The generally accepted panacea that only a papillary puncture is safe is questioned by our results. Based on our experience, a non-papillary puncture proved to be a safe and effective procedure.

Giant left pheochromocytoma with vascular anomalies and pelvic horseshoe kidney: a case report.

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.

Recurrent deep venous thrombosis of lower extremities as a result of compression of large horseshoe kidney cysts in double inferior vena cava - Successfully treatment with sclerotherapy.

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.

Spectrum of congenital anomalies of the kidney and urinary tract (CAKUT) including renal parenchymal malformations during fetal life and the implementation of prenatal exome sequencing (WES).

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.

EVAR in Patients With Abdominal Aortic Aneurysm and Horseshoe Kidney: A Systematic Review.

PURPOSE: A systematic review of all patients that have been reported in the literature with abdominal aortic aneurysm (AAA) concomitant with horseshoe kidney (HSK) treated electively by endovascular aneurysm repair (EVAR) is presented. A new grouping system for describing HSK vasculature is implemented. MATERIALS AND METHODS: We searched for published manuscripts using the Medical Subject Headings terms "abdominal aortic aneurysm," "AAA," "EVAR," "endovascular aneurysm repair," and "horseshoe kidney" in PubMed, Google Scholar, Scopus, and National Center for Biotechnology Information databases. Inclusion criteria include all published material of patients with AAA with HSK treated electively by an endovascular approach. We excluded patients who were treated by a hybrid or open repair or patients with ruptured AAA. Statistical analysis was carried out using SPSS Statistics version 25 (IBM Corp, Armonk, New York) software. RESULTS: A total of 50 patients from 30 studies were included for analysis. Males made up 88% (n=44) of the population. The median age for this cohort was 70 years (range: 47-86 years). Median aneurysmal diameter was 6.0 cm (range: 4.0-10.3 cm). The median operative time for endovascular repair was 84 minutes (range: 40-332 minutes). The most common graft used was Zenith, used in 40% (n=20) of the cases, followed by Endurant in 14% (n=7). The overall complication rate was 14% (n=7). The median follow-up was 19 months (range: 1-108 months). While comorbidities did not appear to impact outcomes significantly, median operative times for smokers were higher than those in nonsmokers, 84 versus 118 minutes, respectively (p=0.048). Univariate linear regression modeling of aneurysmal size with age, operative time, and length of stay revealed a significant coefficient association between aneurysmal size and operative times. After adjusting for comorbidities and aneurysmal size, prior history of chronic kidney disease significantly increased odds for renal infarction. CONCLUSION: This review presents the most complete data set possible of patients with concomitant HSK and AAA treated by an endovascular approach. Furthermore, the A + B + C classification for grouping the HSK vasculature is implemented. This systematic review suggests EVAR to be an excellent option with low complication rates for the treatment of AAA in patients with HSK.

Confirmation of Xp22.11 Duplication as a Germline Susceptibility Alteration in a Wilms Tumor Arising in Horseshoe Kidney.

BackgroundThere is strong evidence of a genetic contribution to Wilms tumor, such as WT1 gene variation or epigenetic changes at chromosome locus 11p15. A previous genome wide association study (GWAS) of Wilms tumor identified other significant association loci including Xp22. Case report: A 4-year-old girl developed a Wilms tumor of the left isthmus of a horseshoe kidney. Chromosomal microarray analysis (CMA) of peripheral blood showed a 563 kb copy number gain at Xp22.11 that included PRDX4 and ZFX. PRDX4 has been shown to play an active role in the tumorigenesis of malignant neoplasms in various organs. Beckwith-Wiedemann methylation analysis and WT1 sequencing were negative. Whole exome sequencing of peripheral blood revealed pathogenic variant in PMS2 gene (c.765C > A), which is consistent with Lynch syndrome. Conclusion: We report a case of Wilms tumor with germline Xp22.11 duplication which further supports this locus as germline susceptibility alteration for Wilms Tumor.

[Modern endourological technologies new opportunities in the treatment of patients with urinary tract anomalies and in complex clinical cases].

The clinical examples of the use of modern endoscopic minimally invasive technologies in patients with urinary tract anomalies and in complex clinical cases are presented in the article. The techniques and features of percutaneous nephrolithotomy, retrograde intrarenal surgery (RIRS), antegrade ureteroscopy using f lexible scope in this category of patients are discussed. The paper confirms the efficiency and necessity of further development of endourological methods for the treatment of urolithiasis.

Renal outcomes in adult patients with horseshoe kidney.

BACKGROUND: Horseshoe kidney (HSK) is a congenital disorder that is usually asymptomatic, but that increases the risks of kidney stones and infectious disease. However, renal outcomes such as end-stage renal disease (ESRD) in patients with HSK remain unclear. METHODS: In total, 146 patients with HSK (age of ≥20 years) from two tertiary hospitals were included in this study. Control individuals who underwent medical check-ups were selected by matching for age, sex, serum creatinine level, hypertension and diabetes. The hazard ratios (HRs) for the risks of ESRD and all-cause mortality were calculated after adjustment for multiple variables. RESULTS: The proportions of HSK-related complications for obstruction, kidney stones, urinary tract infection and urogenital cancer were 26, 25, 19 and 4%, respectively. During the median follow-up period of 9 years (maximum 32 years), the incidence of ESRD was 2.6/10 000 person-years. The risk of ESRD in patients with HSK was higher than in control individuals [adjusted HR = 7.6; 95% confidence interval (CI) 1.14-50.47]. All-cause mortality did not differ between the two groups (adjusted HR = 0.6; 95% CI 0.08-4.29). CONCLUSIONS: Patients with HSK are at risk of ESRD, which may be attributable to the high prevalence of complications. Accordingly, these patients should be regarded as having chronic kidney disease and require regular monitoring of both kidney function and potential complications.

Horseshoe kidney with PLA2R-positive membranous nephropathy.

BACKGROUND: Horseshoe kidney (HSK) is a common congenital defect of the urinary system. The most common complications are urinary tract infection, urinary stones, and hydronephrosis. HSK can be combined with glomerular diseases, but the diagnosis rate of renal biopsy is low due to structural abnormalities. There are only a few reports on HSK with glomerular disease. Here, we have reported a case of PLA2R-positive membranous nephropathy occurring in a patient with HSK. CASE PRESENTATION: After admission to the hospital due to oedema of both the lower extremities, the patient was diagnosed with nephrotic syndrome due to abnormal 24-h urine protein (7540 mg) and blood albumin (25 g/L) levels. Abdominal ultrasonography revealed HSK. The patient's brother had a history of end-stage renal disease due to nephrotic syndrome. Therefore, the patient was diagnosed with PLA2R-positive stage II membranous nephropathy through renal biopsy under abdominal ultrasonography guidance. He was administered adequate prednisone and cyclophosphamide, and after 6 months of treatment, urinary protein excretion levels significantly decreased. CONCLUSION: The risk and difficulty of renal biopsy in patients with HSK are increased due to structural abnormalities; however, renal biopsy can be accomplished through precise positioning with abdominal ultrasonography. In the literature, 20 cases of HSK with glomerular disease have been reported thus far. Because of the small number of cases, estimating the incidence rate of glomerular diseases in HSK is impossible, and the correlation between HSK and renal pathology cannot be stated. Further studies should be conducted and cases should be accumulated to elucidate this phenomenon.

Investigation of Factors Related to Percutaneous Nephrolithotomy Failure in Horseshoe Kidneys and Comparison of Three Stone Scoring Systems in Prediction of Outcomes.

INTRODUCTION: We aimed to identify factors affecting percutaneous nephrolithotomy (PNL) outcomes among patients with horseshoe kidney (HSK) and assess the predictive accuracy of the Clinical Research Office of the Endourological Society (CROES), Guy's Stone Score (GSS), and S.T.O.N.E. scoring systems. METHODS: Data from 98 patients with HSK who underwent PNL between November 2010 and January 2020 were evaluated. Patients were divided into the stone-free (SF) and non-SF groups and compared according to demographic data, stone and surgical characteristics, and stone scoring systems. Multivariate logistic regression analysis was performed to identify factors associated with SF status. RESULTS: Among the included patients, 87 were male and 11 were female (mean age: 47.37 ± 14.42 years). The SF rate was 84.7% (83 patients). Group analysis identified GSS (p < 0.001), CROES score (p < 0.001), S.T.O.N.E. score (p = 0.014), stone burden (p = 0.045), and multiplicity (p < 0.001) as factors associated with SF status. Among our cohort, 10 patients developed complications. All scoring systems were significantly correlated with SF status (CROES: r = -0.442, p < 0.001; GSS: r = 0.442, p < 0.001; S.T.O.N.E.: r = 0.250, p = 0.013), while CROES score was identified as an independent factor associated with SF status (95% CI: 0.937-0.987; p = 0.003). CONCLUSIONS: PNL is an effective method for treating nephrolithiasis among patients with HSK. Moreover, stone-related factors, such as larger size, multiplicity, and complexity, were associated with procedural failure. Finally, the CROES nomogram was a better predictor of SF status compared with other scoring systems.

Laparoscopic abdominal perineal rectal resection for rectal cancer with a horseshoe kidney using preoperative 3D-CT angiography: a case report.

BACKGROUND: A horseshoe kidney is a congenital malformation involving the fusion of the bilateral kidneys and is often accompanied by anomalies of the ureteropelvic and vascular systems. When performing resection of colorectal cancer in a patient with horseshoe kidney, damage to the ureter or excessive renal arteries should be avoided. To achieve this purpose, comprehensive preoperative anatomical assessments and surgical planning are important. Here, we report a case of a laparoscopic abdominal perineal rectal resection for lower rectal cancer with a horseshoe kidney. CASE PRESENTATION: A 79-year-old woman presented with bloody stool and was diagnosed with advanced lower rectal cancer, immediately above the rectal dentate line, without metastasis. A preoperative computed tomography (CT) scan revealed a horseshoe kidney, while a three-dimensional CT (3D-CT) angiography revealed aberrant excess renal artery from the aorta to the renal isthmus. The left ureter ran in front of the isthmus of the horseshoe kidney and presented calculus formation. Laparoscopic abdominal perineal rectal resection was performed with D3 lymph node dissection. During the operation, we mobilized the sigmoid colon mesentery via a medial approach and preserved the left ureter, the left gonadal vessels, and the hypogastric nerve plexus in the retroperitoneum in front of the horseshoe kidney. CONCLUSIONS: We report a rare case of rectal cancer surgery in a patient with a horseshoe kidney. We discuss the anatomical peculiarities of a horseshoe kidney, such as excess renal arteries, inferior vena cava, ureter, gonadal vessels, and nerves, that should be preserved according to the literature. We suggest that preoperative 3D-CT angiography is both useful for revealing the relationship between the vascular system and a horseshoe kidney and helpful when performing laparoscopic surgery for a left-sided colon and rectal cancer to avoid intraoperative injury.

Outcomes of ureteroscopy for stone disease in anomalous kidneys: a systematic review.

INTRODUCTION: Treatment of stone disease in anomalous kidneys can be challenging. As ureteroscopy (URS) has advanced, the number of studies reporting on outcomes of URS for stone disease in anomalous kidneys has increased. Our objective was to perform a systematic review of the literature to evaluate the outcomes of URS for stone disease in this group of patients. METHODS: A Cochrane style review was performed in accordance with the PRISMA guidelines using Medline, EMBASE, CINAHL, Cochrane Library, Scopus and individual urologic journals for all English language articles between inception and June 2018. RESULTS: Fourteen papers (413 patients) with a mean age of 43 years and a male to female ratio of 285:128 were included. The underlying renal anomaly was horseshoe kidney (n = 204), ectopic kidney (n = 117), malrotation (n = 86), cross fused ectopia (n = 2) and others (n = 2). With a mean stone size of 16 mm (range 2-35 mm), the majority of stones were in the lower pole (n = 143, 34.6%) or renal pelvis (n = 128, 31.0%), with 18.9% (n = 78) having stones in multiple locations. Treatment modality included the use of flexible ureteroscope in 90% of patients and ureteral access sheath used in 11 studies. With a mean operative time of 61.3 min (range 14-185 min), the initial and final SFR was 76.6% (n = 322) and 82.3% (n = 340), respectively. The overall complication rate was 17.2% (n = 71), of which 14.8% were Clavien I/II and the remaining 2.4% were Clavien ≥ III complications. CONCLUSION: Although ureteroscopy in patients with anomalous kidneys can be technically challenging, advancements in endourological techniques have made it a safe and effective procedure. In these patients the stone-free rates are good with a low risk of major complications.

Laparoscopy combining with ureteroscopy for horseshoe kidney accompanying with duplicate kidney and a ureteral calculus: a case report.

BACKGROUND: Horseshoe kidney (HSK) is a common renal fusion anomaly, occurring in about 1 in 400-600 individuals. In addition, the incidence of duplicated collecting system is about 0.8%. CASE PRESENTATION: This report documents an extremely rare case, which was treated by multiple procedures in the same operative session to accomplish laparoscopic amputation of the HSK isthmus, resection of duplicate kidney and ureteroscopic lithotripsy. CONCLUSION: Results showed that minimally invasive surgery with use of multiple endoscopes may be a feasible choice for this patient population with complicated comorbid renal conditions.

Surgery for the complex Wilms tumour.

The prognosis of children with Wilms tumour has greatly improved since the introduction of adjuvant radiotherapy and chemotherapy more than 70 years ago with a current overall long-term survival approaching 90%. Before this, surgery was the only option with around 20% survival, even in low-risk categories. The focus is now on management options for those patients in specific subgroups, who continue to have lower event-free survival and who suffer from the long-term effects of treatment. These include those with anaplastic histology, bilateral Wilms, biologically high-risk tumours and those that relapse (Aldrink et al. in J Pediatr Surg 54(3):390-397, 2019). The major advances that have already been made in risk assessment and tailoring the chemotherapy/radiotherapy to achieve maximum advantage with minimum toxicity and long-term morbidity have been predicated on safe and complete resection and staging of the tumour. There are, however, still surgical challenges faced with respect to resection of 'complex' tumours; thus, surgeons need to understand their role and advance their expertise in the overall treatment of children with Wilms tumour. These include very large tumours not responding to chemotherapy, ruptured tumours, tumours with intravascular invasion into the inferior vena cava (± 10%), heart and hepatic veins (± 4%) and Wilms tumours in horseshoe kidneys (< 1%) (Kieran and Ehrlich in Urol Oncol https://doi.org/10.1016/j.urolonc.2015.05.029, 2015). This review describes surgical strategies and techniques used in these situations, gleaned from the authors' experience in the surgical management of over 300 children with Wilms tumours in our centres over the last 30 years.

Management and therapeutic options for abdominal aortic aneurysm coexistent with horseshoe kidney.

BACKGROUND: Horseshoe kidney (HSK), referring to the abnormal fusion of the lower renal poles, represents one of the most common renal anomalies. One of its most significant features is the anomalous vasculature, with a number of accessory renal arteries originating from the aorta, the mesenteric arteries, and even the iliac arteries supplying both the renal kidneys and the renal isthmus. METHODS: A literature review was performed to identify and to present the most recent data regarding classification and imaging evaluation of HSK concomitant with abdominal aortic aneurysm (AAA). Furthermore, an in-depth analysis of both open surgical and endovascular repair is made for management of this rare medical condition. RESULTS: The anomalous renal vasculature of HSK has led to the introduction of a number of classification systems, with Eisendrath's being currently the most commonly used. The concomitant presence of HSK in patients suffering from AAA plays a major role in preoperative planning, with a number of factors taken into consideration in deciding on either an open repair or an endovascular approach. Open repair requires careful decision-making between a transperitoneal and a left retroperitoneal approach to reach the aneurysm sac. In addition, technical points include the decision to divide the renal isthmus or not and the necessity of salvage or reimplantation of anomalous renal vessels. On the other hand, an endovascular approach requires careful preoperative imaging and evaluation of both the renal function and vasculature to decide on catheterization and salvage of accessory renal arteries or their exclusion. CONCLUSIONS: The concomitant presence of AAA and HSK poses a challenge for the modern vascular surgeon, who must possess all required technical skills-both endovascular and open repair-to deal accordingly with this rarely encountered medical condition. Preoperative determination of the perfusion pattern is necessary for the treatment strategy.

Robot-assisted laparoscopic pyeloplasty (RALP) in children with horseshoe kidneys: results of a multicentric study.

PURPOSE: This multicentric study aimed to report our experience with robot-assisted laparoscopic pyeloplasty (RALP) in children with horseshoe kidney (HSK). METHODS: The records of 14 patients (11 boys and 3 girls with an average age of 9 years), who underwent RALP for repair of pelvi-ureteric junction obstruction (PUJO) in HSK in five international pediatric urology units over a 5-year period, were retrospectively reviewed. A dismembered pyeloplasty with no division of isthmus was performed in all the cases. RESULTS: The average operative time including docking was 143.5 min (range 100-205). No conversions to laparoscopy or open surgery or intra-operative complications occurred. Patients were discharged on postoperative day 2 following catheter and drain removal. The JJ stent was removed at mean 33 days postoperatively. Overall success rate was 92.8%. As for postoperative complications, we recorded a urinary tract infection (UTI) and stent-related irritative symptoms, managed with medical therapy, in two patients (II Clavien) and an anastomotic stricture in one patient needing surgical revision with no further recurrence (IIIb Clavien). At follow-up, all the patients (one after redo-surgery) reported complete resolution of symptoms, improvement of hydronephrosis on ultrasound and no residual obstruction on diuretic renogram. CONCLUSIONS: Our experience suggested that RALP in HSK is safe, feasible and with good medium-term outcomes in expert hands. An accurate pre-operative planning associated with a standardized technique is key points to achieve good surgical and functional outcomes in these challenging cases. The da Vinci robot technology offers the advantages of MIS procedures and overcomes the technical challenges of laparoscopic approach.

Maximizing Nephron Mass in Horseshoe Kidney Transplantation Using Inferior Epigastric Artery: Case Report.

Due to the progressive shortage of donors, kidneys with congenital anomalies are considered for transplantation. We report a successful transplantation of a split horseshoe kidney from a deceased donor by using the inferior epigastric artery with an end-to-end anastomosis, supplying the isthmus. Thus, we preserved as much as possible the functional parenchyma for a good long-term outcome. The learning point is that the use of the right inferior epigastric artery seems to be a good solution to perfuse the lower artery in order to avoid its ligation, thus reducing the nephron mass of the graft.