[A Case of Sigmoid Colon Cancer with Horseshoe Kidney].

We reported a case of sigmoid colon cancer with horseshoe kidney. A 79-year-old man had lower abdominal pain and underwent colonoscopy. The results of colonoscopy revealed sigmoid cancer. Preoperative computed tomography revealed horseshoe kidney. He underwent radical laparoscopic surgery. The histopathological diagnosis was pStage Ⅱa(The 9th Edition). He has not recurred 22 months later after operation. Surgery for colorectal cancer with congenital anomalies of the urinary tract requires attention to intraoperative secondary injuries. Therefore, preoperative evaluation using 3D-CT is useful tool for safety. Operating the proper dissecting normal layer would make safe laparoscopic operation possible without unexpected injuries.

Robot-assisted laparoscopic surgery for rectal cancer in a patient with a horseshoe kidney: A case report.

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.

Laparoscopic retroperitoneal heminephrectomy for renal cell carcinoma in horseshoe kidney: a case report and review of the literature.

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.

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.

Supine mini percutaneous nephrolithotomy in horseshoe kidney.

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.

Wilms Tumor in Child With Trisomy 18 and Horseshoe Kidney.

Trisomy 18 is associated with several congenital malformations, including horseshoe kidney. It can be full, partial, or mosaic, and mosaicism is often associated with lesser severity and longer life expectancy, placing patients at greater risk of developing neoplasms or malignancies. One common tumor among children with Trisomy 18 is Wilms tumor, which is also associated with renal congenital abnormalities such as horseshoe kidney. We present a case describing the occurrence of these three characteristics: development of Wilms tumor in a patient with Trisomy 18 and a horseshoe kidney and discuss treatment with regards to these conditions.

Horseshoe Kidney: 500 Years From the First Report in the Literature.

Horseshoe kidney or ren arcuatus is the most common renal fusion anomaly, with an incidence of 1:500 in the normal population and a male predominance of 2:1. In >90% of cases, the fusion occurs along the inferior pole. It may vary in location, orientation, and arterial and venous anatomy. In 1522, Berengario da Carpi described this renal malformation for the first time in his masterpiece "Isagogae breves" (Introduction to Anatomy). He reported the results of a postmortem examination in the public autopsy room of the University of Bologna, describing "kidneys that are continuous as if they were a kidney, with two emulsifying veins, two emulsifying arteries, two ureteral outlets." In 1564, Leonardo Botallo described and illustrated the features of this atypical anatomical representation, and later, in 1602, Leonard Doldius added further details by examining this anatomical feature during an autopsy. In 1761, Giovanni Battista Morgagni discussed this condition not only as a rare anatomical curiosity found only in necroscopy but also discussed its physiological aspect. In the nineteenth century, with the advent of renal surgery, the horseshoe kidney played a more important role in urological diagnosis and treatment, and its identification became more frequent. With the advent of pyelography, imaging reports of the horseshoe kidney allowed a more accurate representation of the anatomical variants, which was particularly useful in preoperative assessment and outcomes. Berengario da Carpi laid the foundation for a better knowledge of this anatomical anomaly. Five hundred years after the first report in the literature, relevant advances have been made in the management of complications associated with horseshoe kidney and in diagnosis, confirming the need to monitor individuals with this condition who are at higher risk of developing chronic kidney disease.

Implications of Tumor Surgery in Horseshoe Kidneys: A Comparative Study of Outcomes and Surgical Practices.

OBJECTIVE: To contrast surgical outcomes of Horseshoe Kidney (HSK) patients with localized renal masses suspected of cancer with nonfused nonectopic kidney patients, emphasizing safe surgical practices for HSKs. METHODS: The study examined solid tumors from the Mayo Clinic Nephrectomy registry between 1971 and 2021. Each HSK case was matched to three non-HSK patients based on various factors. The outcomes measured included complications within 30days of surgery, change in estimated glomerular filtration rate, and overall, cancer-specific, and metastasis-free survival rates. RESULTS: Thirty of the 34 HSKs had malignant tumors compared with 90 of the 102 patients in the nonfused nonectopic referent cohort. Accessory isthmus arteries were present in 93% of HSK cases, with 43% exhibiting multiple arteries and 7% with 6 or more arteries. Estimated blood loss and surgery duration were significantly higher in HSKs (900 vs 300 mL, P = .004; 246 vs 163 minutes, P < .001, respectively). The HSK group demonstrated an overall complication rate of 26% (vs 17% in referents, P = .2) and a median change in estimated glomerular filtration rate at 3months of - 8.5 (vs -8.1 in referents, P = .8). At 5-year follow-up, survival rates for HSK patients were 72%, 91%, and 69% for overall, cancer-specific, and metastasis-free survival, respectively. The corresponding rates were 79%, 86%, and 77%, respectively, for matched referent patients (P > .05). CONCLUSION: HSK tumor management is technically challenging with higher blood loss; however, the data demonstrate comparable outcomes for patients with HSK tumors, including complications and survival, to those without HSKs in experienced centers.

Retrograde intrarenal surgery for stones associated with renal anomalies: caliceal diverticulum, horseshoe kidney, medullary sponge kidney, megacalycosis, pelvic kidney, uretero-pelvic junction obstruction.

PURPOSE OF REVIEW: Nowadays, due to the increase of imaging diagnosis, we identify easily renal anomalies, and we can choose between a wide range of armamentarium to treat symptomatic stones in those challenging cases. However, there is a lack of evidence and consensus on its use. The aim of this narrative review is to collect all the available data about safety and efficacity of retrograde intrarenal surgery (RIRS) in the treatment of kidney stones associated to a renal anomaly. RECENT FINDINGS: Renal anomalies are uncommon findings and even more if it has to be associated with renal stones. After a literature review of the past 2 years, there are a small number of studies that compare the outcomes in patients who have been treated with minimally invasive modalities and they are mainly focus on RIRS. SUMMARY: It is of extreme importance to know the advances on the stone treatment in anomalous kidneys. With the development of new laser technologies, RIRS is becoming a more interesting technique with high success rate and safety. Further studies are needed to make an accurate statement about the adequate surgical technique for each renal anomaly and also, clinical trials using new laser technologies.

Nephron-sparing surgery for primary mucinous adenocarcinoma of renal pelvis in horseshoe kidney: A case report.

RATIONALE: Mucinous adenocarcinoma of the renal pelvis is extremely rare in malignancy of the renal pelvis, and <100 cases have been reported so far. However, horseshoe kidneys appear to be more prone to this mucinous gland metaplasia. Due to the lack of a specific diagnostic basis, it is difficult to confirm the diagnosis before surgery, which is easy to cause misdiagnosis. PATIENT CONCERNS: We report a 64-year-old male patient who found a cystic mass in the left kidney during physical examination. CT examination showed a horseshoe kidney with a cystic mass 9.5 × 8.0 cm in front of the left kidney, lacking obvious diagnostic features. DIAGNOSIS: It was misdiagnosed as cystic teratoma before the operation, and was diagnosed as mucinous adenocarcinoma of the renal pelvis through pathological examination after the operation. INTERVENTIONS: Resection of the tumor by nephron-sparing surgery and postoperative chemotherapy. OUTCOMES: No tumor recurrence was found at 6 years of follow-up. After 7 years, the patient had multiple metastases in the abdominal wall and peritoneum, and no tumor recurrence was found in the urinary system. The patient received chemotherapy again and survived well. LESSONS: The prognosis of nephron-sparing tumor resection for MRAP is not significantly different from that of radical nephroureterectomy + bladder cuff excision. Because it can reduce the risk that patients can not tolerate follow-up chemotherapy due to abnormal renal function after surgery, which may be more beneficial in patients with kidney abnormalities or chronic disease.

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.

Laparoscopic nephron-sparing surgery for horseshoe kidney complicated with renal hemangioma: a case report and literature review.

In very rare cases, horseshoe kidney can present simultaneously with renal hemangioma. Nephron-sparing surgery (NSS) is a minimally invasive surgical procedure for horseshoe kidney with tumor, but it is difficult to operate and has not been reported. We report a 71-year-old female with imaging examination revealed an unexpected horseshoe kidney malformation and a cystic mass in the right kidney before her bladder cancer surgery. After one and a half years of follow-up, the mass was progressively enlarged, and was classified as Bosniak type III. After evaluating the anatomical structure around the lesion by 3D reconstruction computed tomography, she received cystoscopic right double J ureteral catheter placement combined with retroperitoneal laparoscopic partial nephrectomy of the right kidney. After a 3-month follow-up, no tumor recurrence was observed, with normal kidney function. Retroperitoneal laparoscopic NSS of horseshoe kidney with hemangioma is feasible and adequate preoperative 3D reconstruction imaging is the guarantee of safe and smooth operation.

Is there a correlation between urolithiasis in horseshoe kidneys and isthmus thickness or renal angulation?

BACKGROUND: Stone disease is a common complication of horseshoe kidneys (HSK). There are currently limited studies that examine the relationship between HSK anatomy and stone formation. We aim to determine if there is such an association by measuring the isthmus size and renal angulation in both stone and non-stone forming HSK using computed tomography (CT). METHOD: This is a retrospective study performed at a single tertiary centre. Using the radiological information system, all CT reports between 01 January 2010 and 31 December 2015 were searched for the keyword 'horseshoe' on the radiological information system. This produced a list of 285 reports. Each report and image packet of these 285 studies were reviewed to confirm the presence of an HSK and duplicate patients from multiple examinations were highlighted. One hundred and thirty-eight unique HSK patients were obtained and the studies were assessed for the presence or absence of stones. A total of 112 HSK were found; 88 of which contained no stone and 24 with stone. Angle measurements and isthmus size were measured on these kidneys. As axial images are obtained as standard in all cases, these measurements were all obtained in the axial plane. All parameters were measured and recorded manually by one person so as to reduce inter-observer variability. RESULTS: Isthmus size varied widely, measuring from 2 to 39 mm. Right renal angle ranged from 51 to 158°, left 38 to 152°. CONCLUSION: The isthmus size and renal angle measurements were not found to be significant determinants for stone disease in our patient population.

Novel mutation of SLC37A4 in a glycogen storage disease type Ib patient with neutropenia, horseshoe kidney, and arteriovenous malformation: a case report.

Glycogen storage disease type Ib (GSDIb) is an autosomal recessive disorder caused by mutations of SLC37A4 gene, which encodes glucose 6-phosphate translocase (G6PT). Malfunction of G6PT leads to excessive fat and glycogen in liver, kidney, and intestinal mucosa. The clinical manifestations of GSD1b include hepatomegaly, renomegaly, neutropenia, hypoglycemia, and lactic acidosis. Furthermore, the disorder may result in severe complications in long-term including inflammatory bowel disease (IBD), hepatocellular adenomas (HCA), short stature, and autoimmune disorders, which stem from neutropenia and neutrophil dysfunction. Here, we represent a novel mutation of SLC37A4 in a 5-month girl who has a history of hospitalizations several times due to recurrent infection and her early presentations were failure to thrive and tachypnea. Further investigations revealed mild atrial septal defect, mild arteriovenous malformation from left lung, esophageal reflux, Horseshoe kidney, and urinary reflux in this patient. Moreover, the lab tests showed neutropenia, immunoglobulin (Ig) G and IgA deficiency, as well as thrombocytosis. Whole exome sequencing revealed c.1245G > A P.W415 homozygous mutation in SLC37A4 gene and c.580G > A p.V1941 heterozygous mutation in PIK3CD gene. This study shows that manifestations of GSD1b may not be limited to what was previously known and it should be considered in a wider range of patients.

Robot-assisted modified bilateral dismembered V-shaped flap pyeloplasty for ureteropelvic junction obstruction in horseshoe kidney using KangDuo-Surgical-Robot-01 system.

PURPOSE: Horseshoe kidney (HSK) is the most common renal fusion anomaly, occurring in 0.25% of the population (1). It presents technical obstacles to pyeloplasty for ureteropelvic junction obstruction (UPJO) despite robotic assistance (2, 3). KangDuo-Surgical-Robot-01 (KD-SR-01), an emerging robotic platform in China, has yielded satisfactory outcomes in pyeloplasty (4, 5). We first describe our modified technique of robotic bilateral pyeloplasty for UPJO in HSK using KD-SR-01 system in the Lithotomy Trendelenburg position. MATERIALS AND METHODS: A 36-year-old man with HSK and bilateral UPJO suffered right flank pain due to renal calculi (Figure-1). Repeated double-J stent insertion and ureteroscopy lithotripsy did not relieve his symptoms. A robot-assisted modified bilateral dismembered V-shaped flap pyeloplasty was performed using KD-SR-01 system in the Lithotomy Trendelenburg position. RESULTS: Total operative time was 298 minutes with 50 ml estimated blood loss. There was no conversion to laparoscopic or open surgery. A follow-up of 14 months showed relieving symptoms and stable renal function. Cine magnetic resonance urography and computed tomography urography revealed improved hydronephrosis and good drainage. No intraoperative or postoperative complications occurred. CONCLUSIONS: It is technically feasible to perform a KD-SR-01-assisted modified bilateral dismembered V-shaped flap pyeloplasty in the Lithotomy Trendelenburg position for HSK. This procedure achieves managing UPJO on both sides without redocking the system and provides a wider operative field. In addition, it may be associated with better ergonomics, better cosmetic outcomes, and less possibility of postoperative bowel adhesion. However, further investigation is still warranted to confirm its safety, efficacy, and advantages over traditional procedures.