Bilateral Wilms tumor in a patient with a horseshoe and duplex kidney: A case report.

The occurrence of horseshoe kidney with duplex urinary collecting systems is rare. Herein, we report a case of bilateral Wilms tumor (BWT) in a patient with a concurrent horseshoe kidney and left duplex kidney, which had not been previously reported. The patient was treated with neoadjuvant chemotherapy, followed by surgical resection and adjuvant chemotherapy. The tumor recurred 6 months postoperatively. A second resection was performed, followed by the administration of chemotherapy and radiotherapy. The patient passed away 15 months after the initial diagnosis of BWT.

Diagnosis, Management, and Challenges in Synchronous Bilateral Wilms Tumor in a Horseshoe Kidney: A Case Report.

Horseshoe kidney (HK) refers to a congenital malformation that results from fusion of both the kidneys at one pole, and is the most common renal fusion defect with an incidence of 1 in 400 to 600 individuals. Synchronous bilateral development of Wilms tumor (WT) in an HK is extremely rare. Here, we present a case of synchronous bilateral WT in an HK in an 18-month-old girl. The patient received 12 weeks of preoperative chemotherapy followed by 2-step surgical resection including nephron-sparing surgery (NSS) in both kidneys and left nephrectomy because of positive surgical margin and adjuvant chemotherapy. The patient is still in remission and asymptomatic 6 months after the completion of treatment. In this report, we discuss the treatment modalities of synchronous bilaterally located WT in HK. We conclude that successful outcomes can be obtained with preoperative chemotherapy and NSS in such cases even in the presence of advanced disease and complex anatomic conditions. In addition, the deepest tumor point can be reached during NSS, but total nephrectomy should be considered regarding the possibility of microscopic residue.

Malrotation of the midgut associated with horseshoe kidney presenting as gastric outlet obstruction in a 15-year-old boy.

Malrotation occurs in approximately 1 in 500 live births. However, the true incidence of malrotation is unknown since many asymptomatic patients fail to present. Approximately 90% of patients with malrotation are diagnosed within the 1st year of life. Eighty per cent of them are diagnosed within the 1st month of life. Nevertheless, there are recent reports of manifestations later in life both as emergency conditions and more chronic gastrointestinal symptoms. The relationship between malrotation and horseshoe kidney has not been fully understood, but few case reports have highlighted their occurrence in the same patient. The mode of presentation of this case and its association with a horseshoe kidney is the reason for this report. This was a case of malrotation associated with horseshoe kidney. He had exploratory laparotomy and Ladd's procedure. Malrotation is associated with horseshoe kidney which presented as gastric outlet obstruction. He responded well to treatment after Ladd's procedure.

Laparoscopic nephron-sparing surgery for a tumor near the isthmus of a horseshoe kidney with a complicated blood supply.

A horseshoe kidney is a congenital kidney malformation commonly associated with complications such as hydronephrosis, renal calculi, and infections of the renal pelvis. Renal cell carcinoma is extremely rare in a horseshoe kidney; once it occurs, however, it is intractable because of vascular abnormalities. This is especially true in laparoscopic nephron-sparing surgery, even for tumors of <4 cm in diameter. We herein report a case involving an asymptomatic 65-year-old man with an incidental finding of a 4-cm solid mass near the isthmus of a horseshoe kidney on B-mode ultrasonography. Preoperative computed tomography of the renal artery revealed six arterial vessels supplying the affected kidney. Laparoscopic partial nephrectomy was performed. The outcome of this case suggests that laparoscopic nephron-sparing surgery might be a successful treatment method for a horseshoe kidney but that preoperative vessel evaluation and experienced laparoscopic skills are needed.

Case Report of a Pelvic Crossed Fused Renal Ectopic Kidney.

Herein we present a case of a horseshoe kidney with crossed fused renal ectopia. Both of these pathologies are congenital anomalies; however, to date, there are few cases that present with both. In this case, discovered during routine dissection, the fused kidney was mostly left-sided and very low in the pelvis. No renal artery arose from the right wall of the abdominal aorta, and the right renal vein drained into the lower part of the inferior vena cava (IVC) where the right and left common iliac veins joined. It is essential for clinicians and surgeons to understand these types of congenital anomalies, as they could impact patient care.

Horseshoe Kidney Protection with Histidine-Tryptophan-Ketoglutarate Solution during Surgical Abdominal Aortic Aneurysm Repair.

CASE REPORT: We report an unusual case of a 65-year-old male patient with horseshoe kidney, who underwent a successful open repair for an abdominal aortic aneurysm (AAA). The accessory renal arteries were perfused with histidine-tryptophan-ketoglutarate (HTK) solution (Custodiol®; Dr. Franz-Kohler Chemie GmbH, Bensheim, Germany) during the vascular reconstruction. There were no creatinine and estimated glomerular filtration rate (eGFR) modifications in the postoperative time. In the literature, only two cases of Custodiol solution for kidney protection during aortic surgery are reported. To the best of our knowledge, this is the first case of Custodiol perfusion for horseshoe kidney protection. DISCUSSION: The concomitant presence of horseshoe kidney and an AAA requires a specific preoperative planning. This is necessary to define the appropriate surgical procedure and strategy. The onset of acute kidney injury is an aspect that must always be taken into consideration during aortic surgery, even more in the case we are reporting. Indeed, despite the complexity of the kidney anatomy, the use of Custodiol solution allowed a proper maintenance of the perioperative renal function, as shown by the postoperative levels of creatinine and eGFR. CONCLUSIONS: Preoperative planning and organ preservation are crucial in AAA open repair, especially in the presence of congenital anomalies such as horseshoe kidney.

Crossed-fused renal ectopia with renal calculi: Two case reports and a review of the literature.

RATIONALE: Crossed renal ectopia (CRE) is a rare congenital anomaly that is frequently associated with gastrointestinal, cardiovascular, genital and bone malformations. To the best of our knowledge, only 35 cases of crossed renal ectopia involving calculi and 30 cases of CRE associated with renal carcinoma have been reported to date. PATIENT CONCERNS: Here, we present 2 cases of crossed renal ectopia. A 59-year-old woman with diabetes presented to our hospital with abdominal pain. The second patient was a 24-year-old woman who complained with abdominal pain with a duration of 1 day. DIAGNOSES: On the basis of abdominal ultrasonography, we suspected a solitary kidney both in the two patients. Combined with retrograde pyelography and 3D computed tomography, case 1 was diagnosed as an S-shaped right-to-left crossed-fused ectopic kidney with many stones in the left (normal) renal pelvis and case 2 was confirmed to have lump right-to-left crossed-fused renal ectopia with two 3-mm stones in the renal pelvis of the 2 kidneys. INTERVENTIONS: Case 1 underwent percutaneous nephrolithotomy while case 2 refused to undergo surgery and underwent conservative treatment for pain relief. OUTCOMES: Two patients have been followed up and have no stones recurrence. LESSONS: Crossed fused renal ectopia is easily misdiagnosed as a solitary kidney. CRE is so rare that the recognition of the disease needs to be improved and effective treatment should be taken timely. According to the two cases and literature review, minimally invasive surgery has become increasingly common to treat CRE with stones and carcinoma.

Riñón en herradura: casualidad o embriopatía por fenilcetonuria materna / Horseshoe kidney: Chance or embryopathy due to maternal phenylketonuria

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Intraoperative utilization of advanced imaging modalities in a complex kidney stone case: a pilot case study.

PURPOSE: Despite the increasing use of advanced 3D imaging techniques and 3D printing, these techniques have not yet been comprehensively compared in a surgical setting. The purpose of this study is to explore the effectiveness of five different advanced imaging modalities during a complex renal surgical procedure. METHODS: A patient with a horseshoe kidney and multiple large, symptomatic stones that had failed Extracorporeal Shock Wave Lithotripsy (ESWL) and ureteroscopy treatment was used for this evaluation. CT data were used to generate five different imaging modalities, including a 3D printed model, three different volume rendered models, and a geometric CAD model. A survey was used to evaluate the quality and breadth of the imaging modalities during four different phases of the laparoscopic procedure. RESULTS: In the case of a complex kidney procedure, the CAD model, 3D print, volume render on an autostereoscopic 3D display, interactive and basic volume render models demonstrated added insight and complemented the surgical procedure. CAD manual segmentation allowed tissue layers and/or kidney stones to be made colorful and semi-transparent, allowing easier navigation through abnormal vasculature. The 3D print allowed for simultaneous visualization of renal pelvis and surrounding vasculature. CONCLUSIONS: Our preliminary exploration indicates that various advanced imaging modalities, when properly utilized and supported during surgery, can be useful in complementing the CT data and laparoscopic display. This study suggests that various imaging modalities, such as ones utilized in this case, can be beneficial intraoperatively depending on the surgical step involved and may be more helpful than 3D printed models. We also present factors to consider when evaluating advanced imaging modalities during complex surgery.

Horseshoe kidney and associated anomalies: Single institutional review of 20 cases.

BACKGROUND: Horseshoe kidney (HSK) is a common renal fusion anomaly. We undertook this study to discuss various anomalies associated with HSKs. The objective of the study is to study various anomalies associated with HSKs and to assess the need for surgical intervention in patients with these anomalies. PATIENTS AND METHODS: This is a retrospective cohort study of twenty patients who presented to our institute with the diagnosis of HSKs. The data were analysed with regard to age at presentation, symptoms, associated anomalies, investigations and surgical intervention. RESULTS: Twenty patients were included in this study. They were referred either with a diagnosis of HSK or were diagnosed during investigations for symptoms and during workup for associated anomalies. Eleven patients were incidentally diagnosed and were asymptomatic, and there were no associated anomalies diagnosed in these patients. Nine patients were symptomatic and were diagnosed with various associated anomalies. Our results concur with recent literature review which suggests that nearly half of the patients with HSKs have associated anomalies. In the present series, all symptomatic patients with associated anomalies required surgical intervention. CONCLUSION: HSKs being a common fusion anomaly, necessitates a prompt and thorough search of the search for any associated anomalies. Many of these anomalies may require surgical intervention.

Role of magnetic resonance urography in pediatric renal fusion anomalies.

Renal fusion is on a spectrum of congenital abnormalities that occur due to disruption of the migration process of the embryonic kidneys from the pelvis to the retroperitoneal renal fossae. Clinically, renal fusion anomalies are often found incidentally and associated with increased risk for complications, such as urinary tract obstruction, infection and urolithiasis. These anomalies are most commonly imaged using ultrasound for anatomical definition and less frequently using renal scintigraphy to quantify differential renal function and assess urinary tract drainage. Functional magnetic resonance urography (fMRU) is an advanced imaging technique that combines the excellent soft-tissue contrast of conventional magnetic resonance (MR) images with the quantitative assessment based on contrast medium uptake and excretion kinetics to provide information on renal function and drainage. fMRU has been shown to be clinically useful in evaluating a number of urological conditions. A highly sensitive and radiation-free imaging modality, fMRU can provide detailed morphological and functional information that can facilitate conservative and/or surgical management of children with renal fusion anomalies. This paper reviews the embryological basis of the different types of renal fusion anomalies, their imaging appearances at fMRU, complications associated with fusion anomalies, and the important role of fMRU in diagnosing and managing children with these anomalies.

Bladder agenesis, ectopic ureters and a multicystic dysplastic horseshoe kidney in one twin newborn with normal amniotic fluid index in utero.

A monochorionic-diamniotic twin baby presented with intrauterine growth restriction and anuria. The baby was found to have bladder agenesis, a pelvic dysplastic horseshoe kidney, vertebral anomalies, a ventricular septal defect and facial dysmorphisms. It was surprising to find no abnormalities in amniotic fluid indices prenatally, suggesting the possibility of urine output that declined as the pregnancy proceeded. Some degree of twin-to-twin transfusion of amniotic fluid was also possible, which could have rescued the oligohydramnios known to be associated with kidney and urinary tract abnormalities. It was also notable that there was no abnormality in respiratory function, especially since further investigations revealed close to no kidney function. The intrauterine growth restriction (IUGR) along with the multiple anomalies found made the baby unsuitable for dialysis and transplant, and the decision of transition to palliative care was made.

Aspiration Thrombectomy of Acute Atrial Fibrillation-related Renal Artery Thromboembolism in a Patient with Horseshoe Kidney.

BACKGROUND: Acute arterial thromboembolism to the renal arteries can be treated promptly by local thrombolysis, conventional surgical thrombectomy, or anticoagulation. METHODS: We report a patient who presented with acute loin pain as a result of atrial fibrillation-related thromboembolism to the right renal artery supplying his horseshoe kidney. He was already on warfarin treatment with international normalized ratio of 1.7 and had acute bleeding from malignant peptic ulcer disease, so thrombolysis was contraindicated. RESULTS: He underwent timely endovascular revascularization with aspiration thrombectomy, with good clinical and radiological consequence. He subsequently underwent curative partial gastrectomy and made a steady recovery. CONCLUSION: Early endovascular target-directed therapy such as intra-arterial thrombolysis and mechanical aspiration in combination with intravenous heparin therapy will result in renal salvage.

Crossed Fused Renal Ectopia Diagnosed After Discovering an Absent Left Kidney by Point-of-Care Ultrasound.

We report an incidental diagnosis of crossed fused renal ectopia diagnosed in a patient initially evaluated with an educational point-of-care ultrasound (US). We review the US technique, sonographic findings, and the relevant literature regarding renal US.

Incidence and Spectrum of Renal Complications and Extrarenal Diseases and Syndromes in 380 Children and Young Adults With Horseshoe Kidney.

OBJECTIVE: The objective of our study was to evaluate the incidence and spectrum of renal complications and of extrarenal diseases and syndromes in children with horseshoe kidney (HSK). MATERIALS AND METHODS: The clinical data and imaging studies of 380 subjects (median age, 2.8 years) diagnosed with HSK over a 24-year period were reviewed: 366 children and young adults and 14 fetuses, one of whom died in utero. All renal complications and extrarenal diseases and syndromes were documented. Patient age, initial imaging modality, follow-up imaging modalities, and imaging modality at diagnosis were recorded. RESULTS: One hundred seventy-five patients (46.1%) had renal complications. Pelvocaliectasis without an identifiable cause was the most common complication (n = 83, 21.8%) followed by vesicoureteral reflux (n = 37, 9.7%). Less common renal complications included duplex kidney, multicystic dysplastic kidney, renal stones, asymmetric renal size, ureteropelvic junction obstruction, ureteral stones, acute pyelonephritis, trauma, and renal rhabdomyosarcoma. One hundred ninety patients (50%) had extrarenal diseases or syndromes. Gastrointestinal tract (n = 62) and vertebral (n = 54) anomalies were the most common. Forty-nine patients had syndromes. Turner syndrome was the most common (n = 16) followed by caudal regression syndrome (n = 9). HSK was missed on initial imaging in 42 of 364 postnatal renal examinations and was diagnosed later. The patient groups with renal complications and with extrarenal diseases or syndromes and a missed diagnosis of HSK were significantly younger than the groups without them (p < 0.05, Wilcoxon rank sum test). CONCLUSION: Half of children with HSK have renal complications or extrarenal diseases or syndromes. The incidence is higher in younger children, and the spectrum of complications, diseases, and syndromes differs from those reported in previous studies of adults with HSK. Children with HSK, especially young children, therefore require follow-up renal examinations and extensive extrarenal workup.

[Horseshoe kidney: not a simple fusion anomaly]. / Rene a ferro di cavallo: non è una semplice anomalia di fusione. Analisi di due casi clinici e revisione della letteratura.

The horseshoe kidney is a congenital anatomical defect of the kidney that occurs in 0,25% of the population and is generally characterized by the fusion of the lower poles of the two kidneys through an isthmus and to which may be associated with urogenital and renal vascular anomalies. Asymptomatic in 1/3 of the cases and, most of time, accidentally discovered during a radiological examination, promotes nephrolithiasis, ureteropelvic junction obstruction, hydronephrosis, vesicoureteral reflux and pyelonephritis. We report two cases of patients with kidney horseshoe, characterized by the abrupt onset of a septic state with oligo-anuric acute renal failure, electrolyte and acid-base abnormalities, rapid decay of the general conditions, with detection of nephrolithiasis, hydronephrosis and acute pyelonephritis and whose clinical management resulted in a significant and synergistic nefro-urology involvment. The kidney horseshoe not represent so only a simple fusion anomaly but rather an important anatomical condition that, once diagnosed, it would be worthy of a careful clinical, radiological and laboratory surveillance, in order to prevent the potential complications that may be also particularly severe.