Renal Fusion Research Articles

Rim em ferradura com cistos e ureter único: relato de caso

Horseshoe kidney is the most common of all renal fusion anomalies, occurring in approximately 0.25% of the general population. Horseshoe kidney with only a single ureter is a rare anomaly. A 60-year-old man was admitted to hospital for routine health screening. His family history was negative for kidney diseases, and there was no abnormality in his physical examination. A computed tomography (CT) scan revealed an atypical horseshoe kidney with cysts and three-dimensional spiral CT reconstruction showed the presence of a single ureter. The patient has since been followed up for two years without any signs of clinical disease. We report a rare case of a patient with a horseshoe kidney with cysts and a single ureter that was diagnosed incidentally.

Technical Considerations for Transabdominal Aortic Reconstruction with Renal Fusion and Ectopia: Case Series

Aortic diseases associated with renal anomalies can present special challenges during surgery of the abdominal aorta. The aim of this paper is evaluation of morbidity and definition of optimal management according to the clinical histories of 30 patients with horseshoe and ectopic kidneys who underwent surgical procedures on the abdominal aorta over a 20-year period. Twenty-two of them had horseshoe kidney and eight had ectopic kidney. Indications for surgery included aortic aneurysms in 25 patients and aortoiliac occlusive disease in 5. Preoperative diagnostics were performed in all cases by means of computed tomography and angiography (except in the cases with ruptures) associated with duplex ultrasonography. In patients with ectopic kidney a Carrel patch technique was used for the reimplantation of the renal arteries into the body of the bifurcated (four cases) or tubular (four cases) Dacron graft in five (62.5%) cases whereas aortorenal bypass with Dacron graft was obtained in three cases (37.5%). Sixteen patients from the horseshoe kidney group required renal revascularization (reimplantation using Carrel patch in 10 patients and aortorenal bypass using Dacron graft in 6 patients). Two patients, both with ruptured abdominal aortic aneurysms died after the operation. In other cases the average follow up period was 6.2 years (6 months to 17 years). In one case control, angiography after 6 months revealed an occluded left renal artery that was reimplanted by Carrel patch but without repercussions on the renal function. Aortic surgery in patients with renal anomalies can be safely performed without increased mortality.

Abdominal mass due to crossed renal ectopia and fusion

Background: A 22-year-old woman was referred to the department of Radiology, because of a painless mass at the right hemiabdomen.

Pelvic Cake Kidney Drained by a Single Ureter Associated With Unicornuate Uterus

The pelvic cake kidney in which there is complete fusion of both kidneys into a single renal parenchymal mass has been described as the rarest of renal fusion anomalies. Pelvic cake kidney most commonly drains via 2 separate ureters, with fewer than 10 reports demonstrating a cake kidney drained by a single ureter. We present the first ever reported case of a pelvic cake kidney drained by a single ureter in association with a unicornuate uterus. These findings were identified in a young female patient who underwent pelvic magnetic resonance imaging for pelvic pain.

Autosomal dominant polycystic kidney disease complicating renal ectopia and managed with renal transplantation.

A 65-year-old lady developed fever and right-sided abdominal pain. Her medical history included renal transplantation for end-stage renal disease (ESRD) secondary to autosomal dominant polycystic kidney disease (ADPKD). Infection of a cyst within her native right kidney or liver was suspected. Computed tomography of her abdomen and pelvis showed both native kidneys to be grossly enlarged and polycystic. The left native kidney arose from within the pelvis. The transplanted kidney was visible in the right iliac fossa (Figure ​(Figure11). Fig. 1 Computed tomography showing a renal transplant lying in the right iliac fossa and a polycystic left native kidney arising from within the patient's pelvis. ADPKD is the fourth most common cause of ESRD [1], affecting ∼1 in 1000 live births [2]. It is characterized by the accumulation of fluid-filled cysts in the kidney and other organs. Renal ectopy and fusion are relatively common congenital anomalies. Renal ectopy results from the disruption of the normal embryological migration of the kidneys from the pelvis to the retroperitoneal renal fossa (at the level of the second lumbar vertebra). It may result from abnormalities of the ureteral bud and metanephric blastema, genetic anomalies, teratogenic influences or from anomalous vasculature acting as a barrier to ascent [3]. The incidence of pelvic kidney is 1 in 5000 [4]. Most cases are asymptomatic. Renal fusion occurs when a portion of one kidney is fused with the other. The most common fusion anomaly is the horseshoe kidney. Approximately 20 cases of polycystic kidney disease complicating a horseshoe kidney have previously been reported [5]. However, to our knowledge, this is the first reported case of ADPKD complicating a pelvic kidney. Nephrectomy may be considered prior to renal transplantation in patients with ADPKD in the presence of recurrent infection, suspected malignancy or extension of the native polycystic kidney into the potential pelvic surgical site. In cases complicated by a pelvic kidney, the notoriously variable vasculature of anomalous kidneys may complicate this procedure. Conflict of interest statement. None declared.

Hipernefroma en riñon en herradura

We report a clinical case of renal carcinoma in horseshoe kidney and some anatomical particularities during surgery.We describe the case of a 54 year old man who presented pain and prostatism. A renal tumor in horseshoe kidney was found in the diagnostic tests.We performed surgical treatment saving the left kidney .Horseshoe kidney is the most frequent fusion abnormality of the kidney. Renal carcinoma in this type of renal fusion is similar to those with normal anatomy. Anatomical particularities should be taken into account during surgery.

Percutaneous Nephrolithotomy in Renal Anomalies of Fusion, Ectopia, Rotation, Hypoplasia, and Pelvicalyceal Aberration: Uniformity in Heterogeneity

Percutaneous nephrolithotomy (PCNL) in renal fusion, ectopia, renal rotational anomalies, renal hypoplasia, and pelvicaliceal aberration for complex calculi is challenging for fear of inaccessibility, vascular, colonic, and splanchnic injury. We report our experience with 48 such patients. From 2002 to July 2007, 48 patients (mean age, 28 years) underwent PCNL for mean stone size of 39 mm. Fourteen patients had malrotated kidneys, 12 patients had bifid renal pelvis with complete or incomplete double ureter, 10 horseshoe kidneys, 8 hypo plastic kidneys, 3 with crossed fused ectopia, and 1 had pelvic kidney. After appropriate evaluation, the procedure was performed in one session either in prone or supine position with or without nephrostomy tube making amendments to the conventional procedure as deemed necessary befitting the requirements. Complete stone clearance was achieved in 39 patients (81%) after single session of PCNL. Seven patients treated subsequently with extracorporeal shockwave lithotripsy (SWL) for residual stone also became stone free, achieving final stone-free status of 96%. Three patients had more than one tract creation; 25 patients had this procedure in supine position. Mean hospital stay was 3 days (range, 2-7 days). Three patients required transfusion, and two had perinephric collection. No incidence of bowel, vascular, and splanchnic injury was recorded. Stone analysis showed calcium oxalate composition in 83% calculi. Universal application of PCNL in all diverse situations like renal fusion, renal ectopia, renal malrotation, renal hypoplasia, and pelvicaliceal variations yields comparable results demanding firm resolve and mandatory surgical skill on behalf of the surgeon.

Conjoined twins with a horseshoe kidney

Conjoined twins are rare variants of monozygotic twins. There are many types of conjoined twins, and they are usually classified at the point at which they are fused. Our patient fitted into a thoraco-omphalopagus type, where there was fusion of the thorax as well as mid-trunk [3]. The most common type of renal fusion anomaly is the horseshoe kidney. It consists of 2 distinct functioning kidneys on each side of the midline, and they are more likely to be connected at the lower poles [4]. We describe here a 30 year-old patient who presented to the antenatal clinic of Chris Hani Baragwanath hospital in Soweto, South Africa at 34+ weeks gestation. Foetal magnetic resonance imaging revealed thoraco-omphalopagus conjoined twins with complex thoracic and abdominal structures, including a horseshoe kidney that was confirmed on post-natal computed tomography.

Horseshoe Kidney in an 80 Year Old with Chronic Kidney Disease

This image illustrates a horseshoe kidney from an elderly man that was being evaluated for chronic kidney disease associated with diabetes. Horseshoe kidney is the most common renal fusion abnormality, with an incidence of 1/400 worldwide and a male-to-female ratio of 2:1 and is not a cause of impaired kidney function. One theory of the abnormal fusion of the kidneys is that the metanephric blastemas close together before renal capsule maturation, resulting in fusion. The normal embryonic ascent of the fused kidney is disrupted by the inferior mesenteric artery, leaving the adult horseshoe kidney in a lower position than normal. About 90% of the time, the lower poles are fused and the excretory renal units and ureters are maintained separately.

Retroperitoneoscopic Nephrectomy for a Horseshoe Kidney with Unilateral Severe Hydronephrosis and Ureteral Hypoplasia

Jung Woo Lee, Seung Hyun You, Dong Yup Han, Hee Jong Jeong, Doo Young Choi, Yeon Kyun Oh From the Departments of Urology, Pediatrics, Wonkwang University School of Medicine, Iksan, Korea A horseshoe kidney is the most common renal fusion anomaly. It is well known that horseshoe kidneys may be associated with many urological problems, including calculi, vesicoureteral reflux, and ureteropelvic junction obstruction. However, a horseshoe kidney with unilateral severe hydronephrosis and ureteral hypoplasia is very rare. We report an 11year-old female who underwent a retroperitoneoscopic nephrectomy for a horseshoe kidney with severe hydronephrosis and unilateral ureteral hypoplasia. (Korean J Urol 2009;50:512-515) 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏

COMPLETE RENAL FUSION (“CAKE”/“LUMP” KIDNEY)

British Journal of UrologyVolume 43, Issue 4 p. 391-394 COMPLETE RENAL FUSION (“CAKE”/“LUMP” KIDNEY) R.N. Ssrivastava M.R.C.P.(Ed.), R.N. Ssrivastava M.R.C.P.(Ed.) Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this authorMeharban Singh M.D., Meharban Singh M.D. Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this authorO. P. Ghai M.D., O. P. Ghai M.D. Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this authorUma Sethi M.B., Uma Sethi M.B. Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this author R.N. Ssrivastava M.R.C.P.(Ed.), R.N. Ssrivastava M.R.C.P.(Ed.) Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this authorMeharban Singh M.D., Meharban Singh M.D. Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this authorO. P. Ghai M.D., O. P. Ghai M.D. Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this authorUma Sethi M.B., Uma Sethi M.B. Departmentas of Pædiatrics and pathology, All India Insitute of Medical, New Delhi, IndiaSearch for more papers by this author First published: August 1971 https://doi.org/10.1111/j.1464-410X.1971.tb12058.xCitations: 7AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume43, Issue4August 1971Pages 391-394 RelatedInformation

Imaging of horseshoe kidneys and their complications

Horseshoe kidney is the most common renal fusion anomaly and the patients are prone to a variety of complications, such as stone disease, pelviureteric junction (PUJ) obstruction, trauma, infections and tumours. As result of the abnormal anatomy of a horseshoe kidney, imaging and treatment pathways vary substantially from the normal kidney. In this review, we describe the role of modern imaging in depicting horseshoe kidneys and their complications, in tandem with the role the interventional radiologist plays in treating these patients.

Sixteen Years of Experience with Stone Management in Horseshoe Kidneys

Introduction: Horseshoe kidney is the commonest congenital renal fusion anomaly, and is often complicated by urolithiasis. We focus on our 16 years of experience with stone management in horseshoe kidneys. Materials and Methods: We reviewed the progress of 44 patients treated between 1987 and 2002. Shock wave lithotripsy (SWL) was used in 25 patients; the average stone surface area was 91 (range 10–1,600) mm² and average follow-up was 36.5 (range 1–91) months. 19 patients underwent percutaneous nephrolithotomy (PCNL); the average stone surface area was 197 (range 6–2,400) mm². Follow-up data are available for 8 patients and the average follow-up was 42.3 (range 3–144) months. Results: In the SWL group the 3-month stone-free rate (SFR) was only 31%. In the PCNL group the SFR was 75% on the postoperative day-1 KUB. Complications occurred in 9 patients. Conclusions: Stone management in horseshoe kidneys is challenging: PCNL produces a higher SFR with minimal major complications and failed access. PCNL thus appears to be the preferred management option in patients with urolithiasis in horseshoe kidneys.

Incidental finding of horseshoe kidneys in whole-body 18F-FDG PET/CT evaluation

Incidental finding of horseshoe kidneys (HK) in a 77-years old patient who underwent 18-F FDG PET/CT during a check-up evaluation is presented in figures 1 and 2. In the horseshoe kidneys, the normal rotation is incomplete, so that the ureters leave the kidneys from its ventral aspect and the longitudinal axes of the kidneys converge (fig. 1A-D) 1. It is probably the most common of all renal fusion anomalies, with incidence of 3% 2 CT multiplanar and 18F-FDG PET reformatted fused images in Figure 2A-D depict the fused lower poles connected by an isthmus (in this case of renal parenchyma) lying anterior to the aorta and inferior vena cava 2. There is a higher incidence of transitional cell carcinoma in horseshoe kidneys, this is conceivably due to the presence of chronic urinary tract infections 3. Other tumours such as renal cell carcinoma, squamous cell carcinomas, Wilm's tumors, lymphomas, carcinoid tumors and sarcomas have also been reported 3.

Pancake Kidney Detected on Renal Scintigraphy

Pancake kidney is an extremely rare renal anomaly resulting from abnormal renal ascent and subsequent fusion. The authors describe a patient with pancake kidneys detected on a Tc-99m DTPA scan. The case reported depicts the scintigraphic findings in such a patient having a pancake kidney.

Hand-Assisted Laparoscopic Heminephrectomy in Horseshoe Kidney

Background and Purpose: Horseshoe kidneys are the most common renal fusion anomalies. In surgery on normal kidneys, the use of minimally invasive techniques has decreased morbidity and improved postoperative care. However, aberrant vasculature, abnormal kidney location, and the renal isthmus present technical challenges to the laparoscopic management of horseshoe kidneys. We describe a case of heminephrectomy using hand-assisted laparoscopic techniques. Methods: A 35-year-old female patient with a horseshoe kidney was evaluated for recurrent nephrolithiasis and infections. Despite multiple previous percutaneous stone removal procedures, she continued to have symptomatic nephrolithiasis in the atrophic and obstructed right renal moiety. A transperitoneal hand-assisted laparoscopic heminephrectomy was performed on the right side. The aberrant vessels were identified and divided while the renal isthmus was divided using an endoscopic stapler. Results: The operative time was 165 minutes, while the estimated blood loss was 200 mL. There were no intraoperative complications. Postoperative pain was minimal, and the hospital stay was 4 days. The patient remains asymptomatic 6 months after her partial nephrectomy, with stable renal function. Conclusion: Hand-assisted laparoscopic heminephrectomy provides a safe treatment option for patients with an atrophic, obstructed moiety in a horseshoe kidney.

Transitional cell carcinoma in a fused crossed ectopic kidney

Nephroureterectomy is considered the reference standard treatment for invasive transitional cell carcinoma of the ureter. When this malignancy occurs in a patient with renal fusion anomaly, separating the involved kidney from its conjoint mate becomes a challenging task for the surgeon. We report a unique case in which a patient with an L-shaped left-to-right crossed ectopic kidney was diagnosed with invasive ureteral transitional cell carcinoma. The preoperative assessment in these uncommon cases should include renal angiography or computed tomography angiography to provide key information about the renal vasculature, which is essential for planning the surgical dissection and line of separation between the kidneys.

Horseshoe kidney transplantation

Even though the number of patients awaiting renal transplant is rapidly increasing, the donor pool remains relatively stable. In an attempt to increase this pool, marginal kidneys and kidneys with congenital anatomical variations are being used. Horseshoe kidneys, being the most common renal fusion anomaly, can provide a useful solution to the ever-increasing gap between demand and supply. These kidneys have been successfully transplanted en bloc into a single recipient or, alternatively they have been divided and transplanted into 2 recipients. We report a case of the successful transplantation of an en bloc horseshoe kidney into a single recipient. To the best of our knowledge this is the first of its kind in the U.K. The relevant literature is also reviewed with the aim of raising awareness about the necessity and promising outcomes of such transplants.

Mice lacking the giant protocadherin mFAT1 exhibit renal slit junction abnormalities and a partially penetrant cyclopia and anophthalmia phenotype.

While roles in adhesion and morphogenesis have been documented for classical cadherins, the nonclassical cadherins are much less well understood. Here we have examined the functions of the giant protocadherin FAT by generating a transgenic mouse lacking mFAT1. These mice exhibit perinatal lethality, most probably caused by loss of the renal glomerular slit junctions and fusion of glomerular epithelial cell processes (podocytes). In addition, some mFAT1(-/-) mice show defects in forebrain development (holoprosencephaly) and failure of eye development (anophthalmia). In contrast to Drosophila, where FAT acts as a tumor suppressor gene, we found no evidence for abnormalities of proliferation in two tissues (skin and central nervous system [CNS]) containing stem and precursor cell populations and in which FAT is expressed strongly. Our results confirm a necessary role for FAT1 in the modified adhesion junctions of the renal glomerular epithelial cell and reveal hitherto unsuspected roles for FAT1 in CNS development.

Does Retroperitoneal Lymphadenectomy For Testicular Germ Cell Tumor Require a Different Approach in the Presence of Horseshoe Kidney?

We report our management of stage II testicular nonseminomatous germ cell tumor in 2 patients with horseshoe kidney and discuss the technical challenges posed by this renal fusion anomaly. The embryology and clinical anatomy of horseshoe kidney are discussed with particular reference to the anomalous vascular pattern and routes of testicular lymphatic drainage in this setting. Modifications and innovations of the standard technique of retroperitoneal lymphadenectomy in the presence of horseshoe kidney are discussed in light of our experience with these patients at 2 major tertiary care cancer centers. The significance of contemporary advanced noninvasive radiological techniques, such as helical computerized tomographic angiography with digital 3-dimensional reconstruction and magnetic resonance angiography, in the surgical planning and safe performance of surgery is emphasized.Two young male patients treated at 2 major American teaching hospitals who had coexistent stage II testicular nonseminomatous germ cell tumor and horseshoe kidney underwent salvage retroperitoneal lymph node dissection.There was no evidence of recurrence in these 2 patients 12 and 15 months after surgery, respectively.Horseshoe kidney poses special technical problems during retroperitoneal lymphadenectomy for testicular tumors due to anomalous renal and intra-abdominal vascular patterns. Helical computerized tomography angiography is useful for meticulous surgical planning and the safe performance of surgery in this setting.