Bilateral En-Block Horseshoe Kidney Laparoscopic Nephrectomy / Nefrectomía bilateral en bloque en riñón en herradura

Horseshoe kidney (HSK) has a prevalence of 1 in every 500 individuals. The management of patients with HSK is usually conservative, except in the presence of symptoms such as obstruction, stones, glomerulopathies, and tumors. In the following case report, we describe how a bilateral en-block transmesenteric laparoscopic nephrectomy in supine position was performed. A 5-year-old boy, with proximal hypospadias and early onset of chronic kidney disease due to focal segmental glomerulosclerosis on biopsy, underwent a genetic molecular evaluation that confirmed a pathogenic mutation at the WT-1 gene. Due to the increased risk of developing Wilms tumor, he underwent a bilateral transmesenteric nephrectomy. In a five-minute video, we describe how we performed an en-block transperitoneal and transmesenteric laparoscopic nephrectomy with special attention to patient positioning, including the feasibility of performing the dissection of the left renal hilum and isthmus with the patient in supine with no need for repositioning, and then moving to the dissection of the right renal hilum and completion of the procedure. The case herein reported enables us to describe the technical key-points to perform a bilateral en-block laparoscopic nephrectomy with shorter operative time and reduction of blood loss by preserving the entire specimen, without the need for an isthmus transection.

El riñón en herradura (RH) tiene una prevalencia de 1 en cada 500 individuos. El manejo del RH es usualmente conservador, excepto cuando genera síntomas como obstrucción, litiasis, glomerulopatías o tumores. Con este reporte de caso, describimos como se realizó una nefrectomia bilateral en bloque transmesentérica en un paciente con riñón en herradura. Un paciente de 5 años de edad, con hypospadias proximal y desarrollo temprano de enfermedad renal crónica por glomeruloesclerosis focal segmentaria, fue sometido a un estudio molecular que confirmó la presencia de una mutación en el gen WT-1. Dado el alto riesgo de desarrollar tumor de Wilms, se decidió realizar una nefrectomía transperitoneal laparoscópica bilateral. En un video de cinco minutos, describimos como se realizó una nefrectomía transperitoneal y transmesentérica en bloque, con especial atención al posicionamiento del paciente, incluso la viabilidad de realizar la disección del hilio renal izquierdo y el istmo con el paciente en supino, sin necesidad de reposicionarlo, y, después, la disección del hilio renal derecho y el fin del procedimiento. El caso reportado nos permite describir los puntos clave técnicos para realizar una nefrectomía laparoscópica bilateral en bloque con un tiempo operativo más corto y reducción del sangrado al preservar todo el espécimen, sin la necesidad de realizar la transección del istmo.

Association between Genitourinary and Congenital Heart Defects: A 52-year Case-Control Study of the Latin American Collaborative Study of Congenital Malformations (ECLAMC) / Asociación entre cardiopatías congénitas y malformaciones congénitas urinarias: Un estudio de casos y controles de 52 años del Estudio Colaborativo Latino Americano de Malformaciones Congénit

Background Congenital urological anomalies are present in 4.3/10 thousand newborns, and their association with other anomalies may increase the overall mortality and disability. The present study establishes the risk of having congenital urological anomalies presenting associated cardiopathies. Methods We conducted a retrospective case-control study using the Latin American Collaborative Study of Congenital Malformations (Estudio Colaborativo Latino Americano de Malformaciones Congénitas, ECLAMC, in Spanish). The analysis included all registered cases of congenital urological malformation from 1967 to 2019. Patients with or without associated heart defects were included for the statistical analysis. Odds ratios (ORs) were calculated using a 95% confidence interval (95% CI). We compared the variables with the Chi-squared test and analysis of variance (ANOVA). The statistical analysis was performed using the Statistical Package for the Social Sciences (IBM SPSS Statistics for Windows, IBM Corp., Armonk, NY, United States) software, version 27.0. Values of p < 0.05 were considered statistically significant. Results A total of 7,767,161 newborns were evaluated, and 17,834 genital and upper urinary tract malformations were identified. Of these, 64.2% were genital anomalies, and 35.8% were abnormalities of the upper urinary tract. Genitourinary malformations and concomitant congenital heart defects (GU + C) were observed in 3.5% of the cases. Subjects with GU + C had a higher number of malformations (4.59 ± 2.3) than patients without heart defects (1.53 ± 1.58) (p < 0.000). The OR was of 3.61 (range: 1.86­7.00) for cloacal exstrophy, of 4.01 (range: 3.14­5.12) for imperforate anus, of 5.52 (range: 3.92­7.78) for horseshoe kidney, and of 13.7 (range: 6.65­28.22) for trisomy 21 (Down syndrome) with malformations of the upper urinary tract. Conclusion The association of congenital heart defects with urological anomalies is higher for complex congenital anomalies such as imperforate anus, cloacal exstrophy, and ho

Introducción Las malformaciones congénitas urológicas están presentes en 4,3/10 mil, y su asociación con otros defectos puede aumentar la mortalidad global y la discapacidad. Este estudio analiza la presentación de las malformaciones congénitas urológicas asociadas a las cardiopatías congénitas. Métodos Este es un estudio retrospectivo de casos y controles que usa el Estudio Colaborativo Latinoamericano de Malformaciones Congénitas (ECLAMC). Se incluyeron todos los casos registrados de malformaciones congénitas urológicas de 1967 a 2019, y todos los casos con y sin defectos cardiacos. Se calculó la razón de disparidad (RD) usando un intervalo de confianza del 95% (IC 95%). Se probó la hipótesis con el Chi-cuadrado y análisis de la varianza (analysis of variance, ANOVA, en inglés). Se realizó el análisis estadístico por medio del programa Statistical Package for the Social Sciences (IBM SPSS Statistics for Windows, IBM Corp., Armonk, NY, Estados Unidos), versión 27.0. Se consideró la significancia estadística con valores de p < 0,05. Resultados Se evaluaron 7.767.161 recién nacidos, y se identificaron 17.834 malformaciones genitales y del tracto urinario superior. De estas, 64,2% fueron genitales, y 35,8%, de vías urinarias superiores. El 3,5% de los casos tenían malformaciones genitourinarias y defectos cardiacos congénitos concomitantes (GU + C). Aquellos con GU + C tenían mayor número de malformaciones (4,59 ± 2,3) que los pacientes sin defectos cardiacos (1,53 ± 1,58) (p < 0,000). La RD fue de 3.61 (rango: 1,86­700) para la extrofia cloacal, de 4,01 (3,14­5,12) para el ano imperforado, de 5,52 (3,92­7,78) para el riñón en herradura, y de 13,7 (6,65­28,22) para la trisomía 21 (síndrome de Down) con malformaciones del tracto urinario superior. Conclusión La asociación entre defectos cardiacos congénitos y anomalías urológicas es significativa en malformaciones congénitas complejas como el ano imperforado, la extrofia cloacal, y el riñón en herradura. Los pacientes con síndrome de Down y malformaciones urológicas tienen la mayor probabilidad.

Aneurisma de aorta abdominal sintomático associado a rim em ferradura / Symptomatic abdominal aortic aneurysm in a patient with a horseshoe kidney

Resumo O rim em ferradura é a anomalia congênita renal mais comum, ocorrendo em 0,15 a 0,25% de nascidos vivos. A associação de rim em ferradura com aneurisma de aorta abdominal é rara, estando presente em apenas 0,12% dos pacientes submetidos a tratamento dos aneurismas de aorta abdominal. Este desafio terapêutico consiste em um caso de um paciente portador de um aneurisma de aorta abdominal sintomático associado a rim em ferradura. A irrigação do rim em ferradura dava-se por meio de quatro artérias, sendo que duas delas emergiam diretamente do saco aneurismático. O caso foi solucionado por meio de cirurgia aberta, em caráter de urgência, com acesso transperitoneal, interposição de enxerto de Dacron bifurcado aorto bi-ilíaco e reimplante das duas artérias renais anômalas no corpo principal do Dacron. O paciente teve boa evolução pós-operatória, recebendo alta da unidade de tratamento intensivo no 3º dia e alta hospitalar no 8º dia, mantendo níveis normais de creatinina sérica.

Abstract Horseshoe kidney is the most common congenital renal anomaly, occurring in 0.15-0.25% of newborns. The association of a horseshoe kidney with an abdominal aortic aneurysm is rare. Only 0.12% of patients requiring abdominal aortic repair have a horseshoe kidney. This therapeutic challenge constitutes a patient presenting with a symptomatic abdominal aortic aneurysm and a horseshoe kidney. The horseshoe kidney was supplied by 4 renal arteries, 2 of which emerged from the aneurysmal sac. The patient underwent urgent open repair, with transperitoneal exposure, interposition of a bifurcated aorto-bi-iliac Dacron graft and re-implantation of the 2 anomalous renal arteries on the Dacron main body. Postoperatively, the patient was discharged from the intensive care unit on day 3, and discharged home on day 8, maintaining normal serum creatinine.

Role of the three-dimensional printing technology in complex laparoscopic renal surgery: a renal tumor in a horseshoe kidney

ABSTRACT Purpose: To report our initial experience using a patient-specific 3D-printed renal tumor model for the surgical planning of a complex heminephrectomy in a horseshoe kidney. Materials and Methods: We selected a clinical case for a complex laparoscopic surgery consisting in a 53 year-old male presenting a local recurrence of a renal tumor in a horseshoe kidney with aberrant vascularisation previously treated with a laparoscopic partial nephrectomy. He is now proposed for a laparoscopic left heminephrectomy. Along with conventional imaging, a real-size 3D-printed renal model was used to plan de surgical approach. The perioperative experience of the surgical team was recorded. Results: The surgical team found the patient-specific 3D printed model useful for a better understanding of the anatomy and an easier surgical planning. Conclusion: The use of patient-specific 3D-printed renal models seem to be helpful for the surgical planning in complex renal tumors.

Crossed renal ectopia with fusion in a female feline: case report / Ectopia renal cruzada com fusão em uma gata: relato de caso

A 3 year old female feline of mixed breed was sent to the diagnostic imaging sector under suspicion of bleeding due to ovariohysterectomy. An abdominal ultrasonography was performed to confirm the initial suspicion. However, no signs of bleeding were found, instead it was observed that the left renal silhouette had two pelvises and was elongated and larger than normal. The right kidney was not found. Excretory urography was requested to evaluate the condition of the ureters. The final diagnosis was crossed renal ectopia with fusion in an asymptomatic cat with no changes in renal function.(AU)

Uma gata, sem raça definida, com três anos de idade, foi encaminhada para o setor de diagnóstico por imagem sob suspeita de hemorragia devido à ovário-histerectomia. Foi realizada ultrasonografia abdominal para confirmar a suspeita inicial, mas nenhum sinal de hemorragia foi encontrado; visibilizou-se, entretanto, a silhueta renal esquerda alongada, com presença de duas pelves. O rim direito não foi encontrado. Solicitou-se exame de urografia excretora para avaliação de ureteres. O diagnóstico foi de ectopia renal cruzada com fusão em um felino assintomático e sem alterações na função renal.(AU)

Anatomical and clinical aspects of horseshoe kidney: a review of the current literature / Aspectos anatómicos y clínicos del riñón en herradura: una revisión de la literatura actual

SUMMARY: Horseshoe kidney (HSK) is the most common kidney malformation. This review analyses the literature surrounding the etiology, morphology and clinical anatomy of these kidney fusion anomalies. A systematic literature search was carried out using the Science Direct and Scopus applications. HSK is characterized by three anatomic abnormalities: ectopia, malrotation and vascular changes. The study put emphasis especially on blood supply and upper urinary tract changes in HSK's patients. Arterial blood supply was analysed not only basing on Graves pattern, but also a new model of supply created on horseshoe kidneys computed tomography was taken into account. In addition, study presents clinical aspects of especially significant pathology of HSK, like hydronephrosis, stones and urinary tract infection. This study focuses also on congenital anomalies associated with horseshoe kidney.

RESUMEN: El riñón en herradura (RH) es la malformación renal más común reportada en la literatura. Esta revisión analiza la literatura que rodea la etiología, morfología y anatomía clínica de esta anomalía de fusión renal. Se realizó una búsqueda sistemática de la literatura utilizando las aplicaciones Science Direct y Scopus. El riñón en herradura se caracteriza por tres anomalías anatómicas: ectopía, malrotación y cambios vasculares. El estudio puso énfasis especialmente en el suministro de sangre y los cambios del tracto urinario superior en los pacientes con RH. El suministro de sangre arterial se analizó no solo basándose en el patrón de Graves, sino que también se tuvo en cuenta un nuevo modelo de suministro creado en los riñones en herradura. Además, el estudio presenta aspectos clínicos de patología especialmente significativa de RH, como hidronefrosis, cálculos e infección del tracto urinario. Este estudio se centró también en las anomalías congénitas asociadas con el riñón en herradura.

Horseshoe Kidney / Riñón en herradura

Abstract We present the clinical case of a 39 year old male that presented with a violent death in the city of Medellin. During the necropsy, a horseshoe Kidney was found, which was never diagnosed according to the patients' medical history. It is a case relevant in the medical field given the unusual vasculature that was found, because it's not a usual arrangement. The lack of symptoms and its spontaneous finding during the necropsy, shows us that it's not a rare find in our environment and that it has a big impact in the surgical fields, and in occasions, with urinary tract infections. Methodology: Descriptive. Informed consent was given by the deceased relatives for the use and analysis of the medical history.

Resumen Se expone el caso de un hombre de 39 años que presenta muerte violenta en la ciudad de Medellín. Durante la necropsia se encuentra un riñón en herradura, que no tuvo diagnóstico clínico en su momento, según los hallazgos en su historial médico. Es un caso que puede tener implicaciones en el campo médico, dada la vasculature accesoria que presenta, presentación que no es comúnmente observada. La falta de sintomatología y su hallazgo espontáneo durante la necropsia hacen ver que, a pesar de la poca incidencia, no es extraño encontrar el caso en pacientes de nuestro entorno. Este tiene mayor impacto en el campo quirúrgico y, en ocasiones, en las afecciones urinarias. Metodología: descriptivo. Se obtuvo consentimiento informado, de la familia del occiso, para el estudio de su historial médico.

Flexible ureterorenoscopy in position or fusion anomaly: Is it feasible? / Ureterorrenolitotripsia flexível no tratamento de cálculos em rins anômalos: Qual a viabilidade?

Summary Objective: To analyze the results of flexible ureterorenoscopy (F-URS) with holmium laser in the treatment of kidney stones with ectopic and fusion anomalies (horseshoe kidney and rotation anomalies). Method: We reviewed data from 13 patients with fusion and ectopic renal anomalies that underwent F-URS from April 2011 to April 2017. We analyzed demographic and clinical data (age, gender, BMI, anatomical abnormality, location and dimension of the renal calculi) and perioperative data (method of treatment, stone-free rate, number of days with DJ catheter and perioperative complications). Results: The mean stone size was 12.23 +/- 5.43 mm (range 6-22mm), located in the inferior (58.33%) and middle (16.76%) calyceal units, renal pelvis (16.67%) and multiple locations (8.33%). All 13 patients were treated with Ho-Yag laser, using dusting technique (25%), fragmentation and extraction of the calculi (58.33%) and mixed technique (16.67%). We did not have any severe perioperative complication. After 90 days, nine patients (75%) were considered stone free. Conclusion: Our data suggest that F-URS is a safe and feasible choice for the treatment of kidney stones in patients with renal ectopic and fusion anomalies.

Resumo Objetivo: Analisar os resultados da ureterorrenolitotripsia flexível (ULT-F) no tratamento de cálculos em rins com anomalia de posição e de fusão (rins em ferradura e rins com vício de rotação). Método: Realizamos a coleta prospectiva dos dados de 13 pacientes com anomalias de fusão e de posição submetidos a ULT-F entre abril de 2011 e abril de 2017. Analisaram-se dados clínicos (idade, gênero, IMC, anormalidades anatômicas, dimensão e localização dos cálculos) e perioperatórios (método de tratamento do cálculo, índice de stone free, tempo de cateter DJ e complicações perioperatórias). Resultados: Nos 13 pacientes, os cálculos mediam em média 12,23 mm +/- 5,43 mm (variando de 6 a 22 mm), em sua maioria distribuídos em apenas um grupo calicinal (58.33% em grupo calicial inferior, 16.67% em grupo calicial médio, 16,67% em pelve e 8,33% em múltiplos cálices). Todos os pacientes foram tratados com utilização de laser Ho-Yag, com fragmentação e retirada de cálculos em sete casos (58,33%), pulverização em três casos (25%) e técnica mista em dois casos (16,67%). Não houve complicações intraoperatórias ou pós-operatórias graves. Após 90 dias, nove pacientes tornaram-se stone free (75%). Conclusão: A ULT-F apresenta-se como método seguro e eficaz no tratamento de litíase em rins com anomalia de posição e de fusão.

A second reported malignancy in a patient with Morquio syndrome

Morquio syndrome is a rare lysosomal storage disease that affects multiple organ systems. However, it is rarely associated with malignancy. We present the case of a 30-year old man with Morquio syndrome associated with gastric adenocarcinoma. This case also demonstrates two other findings that have not been previously described in patients with Morquio syndrome - malrotation of brainstem and cerebellum, without clinical neurologic deficit, and persistence of fetal lobulation in the kidneys.

En bloc transplantation of horseshoe kidney in Korea

Transplantation of the horseshoe kidney can be performed en bloc or split into 2 grafts according to the vascular anomaly and the existence of the urinary collecting system in isthmus. From 2011 to 2014, there were 3 horseshoe kidney transplantations in Korea and transplantations were performed at 2 different centers. The transplantations were carried out successfully for all recipients without complications. All recipients have shown good graft kidney function after transplantation. No severe complication was revealed during follow-up period. We described the surgical technique used in the en bloc method to overcome various vascular anomalies and difficulties in choosing cannulation site and postoperative complications. En bloc transplantation of a horseshoe kidney is a useful strategy for patients with end-stage renal disease, and can provide favorable outcomes compared to the transplantation of a normal kidney.

Renal cell carcinoma in a horseshoe kidney

@#Horseshoe kidney is a rare form of congenital renal malformation. It occurs in 0.25 to 3% of the population and is usually asymptomatic. Occurrence of symptoms is usually related to infection, lithiasis, and rarely, malignancy. Presented is a case of a 62-year old male with a one-year history of occasional painless hematuria associated with epigastric discomfort. On physical examination, a palpable mass was noted on the right periumbilical area, 10cm x 10cm in size and was non-tender. CT-scan with IV contrast showed 10cm x 7cm x 12cm mass on the right side of a horseshoe kidney. He underwent Radical Nephrectomy, right with Isthmusectomy. Post-operative course was unremarkable. Histopathology result showed Renal Cell Cacrcinoma, clear cell type.. Although malignancy was present in an anomalous kidney, the prognosis is the same as with normal kidneys. To date, this is the first reported case of malignancy on a horseshoe kidney in the Philippines.

Comparison of shock wave lithotripsy (SWL) and retrograde intrarenal surgery (RIRS) for treatment of stone disease in horseshoe kidney patients

ABSTRACT Objectives In this study it is aimed to compare the success and complication rates of SWL and RIRS in treatment of HSK stone disease. Materials and methods In this retrospective study data of 67 patients treated with either SWL (n=44) or RIRS (n=23) for stone disease in HSK between May 2003 to August 2014 was investigated. age, gender, stone size and multiplicity, stone free status, renal colic episodes and complication rates of the SWL and RIRS groups were compared. Results Mean age of the population was 42.5±8.2 (range: 16-78) years and mean stone size was 16.9±4.1 mm. SWL and RIRS groups were similar with regard to demographic characteristics and stone related characteristics. SFR of the SWL and RIRS groups were 47.7%(21/44 patients) and 73.9% (17/23 patients) respectively (p=0.039).Renal colic episodes were observed in 3 and 16 patients in the RIRS and SWL groups respectively (p=0.024). No statistically significant complications were observed between the SWL (8/44 patients) and RIRS (4/23) groups (p=0.936). Conclusions In HSK patients with stone disease, both SWL and RIRS are effective and safe treatment modalities. However RIRS seems to maintain higher SFRs with comparable complication rates.

Flexible Ureteroscopic Management of Horseshoe Kidney Renal Calculi

ABSTRACTPurpose:To evaluate the clinical efficacy of flexible ureteroscope (F-URS) combined with holmium laser lithotripter in treating renal calculi in horseshoe kidney.Materials and Methods:From November 2010 to December 2013, the medical history and charts of sixteen patients (mean age 42.9±11.6 years, range 26-66 years), including 13 males and 3 females were analyzed retrospectively. Mean stone burden was 29±8 mm (range 17-42 mm2). Mean stone digitized surface area (DSA) was 321±94 mm2 (range 180-538 mm2). Under spinal anesthesia in a modified lithotomy position with the head down, rigid ureteroscope was placed firstly into the ureter to reach the level of the pelvis, a zebra guide wire was inserted and following the removal of the rigid ureteroscope, an ureteral access sheath was positioned along the guide wire, then passed the URF P-5 flexible ureteroscope into the renal cavities over the guidewire. After locating the stones, holmium laser lithotripsy was performed.Results:The average operative time was 92±16 minutes (range 74-127 min.). No major complications were encountered. Ten patients obtained stone-free status with one session, four obtained stone-free status after two sessions. Single session stone-free rate was 62.5%, overall stone-free rate was 87.5%. Two patients have small residual stones in the lower pole.Conclusions:F-URS combined with holmium laser lithotripter and nitinol basket, is safe and effective in dealing with moderate stone diameter (<30 mm) in HSKs with high clearance rates and low complication rates.

Transarterial coil embolization in treatment of gross hematuria following self-inflicted stab wound in a horseshoe kidney / 中华创伤杂志(英文版)

Horseshoe kidney is an uncommon anomaly of the urinary system with an increased risk of injury during penetrating and blunt abdominal traumas. Self-inflicted abdominal stab wound, a rare type of abdominal injury, accounts for only a small percentage of suicidal attempts and may be infrequently encountered by physicians in trauma centers. Psychiatric disorders and alcohol or drug abuse are common risk factors in cases of self-stabbing. Here we report a rare case of self-stabbing of a horseshoe kidney. The case was a 19-year-old man with self-inflicted abdominal stab wound who was referred to our department of radiology due to re-occurred gross hematuria three days after exploratory laparotomy and surgical repair of injured abdominal organs. A horseshoe kidney was incidentally found in the patient's abdominal computed tomography. Renal angiography revealed active contrast extravasation from one of the segmental arteries. Selective transarterial embolization with a coil was successfully performed to control the hematuria.

Case Report on Horseshoe Kidney / 체질인류학회지

The authors dissected and examined a horseshoe kidney from the cadaver of a 54-year-old Korean female. The results were as follows. Other congenital anomalies or complications were not found, and no renal stones showed on plain radiography of the horseshoe kidney. The horseshoe kidney was located in the area between the 12 th thoracic vertebra and the 4th lumbar vertebra, with the superior extremity of the left kidney 10mm more inferior than that of the right. The isthmus connecting the bilateral kidneys was located at the level of the 3rd lumbar vertebra. The size of the kidney was 102mm × 52mm × 44mm (right) and 108mm × 62mm × 34mm (left), and the superoinferior and anteroposterior lengths of isthmus were 22mm and 10mm, respectively. The abdominal aorta and inferior vena cava passed posteriorly to the isthmus, with the inferior mesenteric artery and lumbar splanchnic nerve passing anteriorly. Some grooves were found on the anterior surface of the bilateral kidney. The hilum of the right kidney faced the anteromedial direction and that of the left kidney faced the anterolateral direction. At the hilar plane, the right renal arteries and veins passed anteriorly and posteriorly to the renal pelvis ; the left renal arteries passed posteriorly to the renal pelvis, with the left renal veins passing anteriorly and posteriorly. Three branches of the right renal arteries passed renal hilum, while two branches did not, and two branches of the left renal arteries passed renal hilum, while six branches did not. The two arteries arising from the aortic bifurcation were distributed to the isthmus. The number of renal veins passing the renal hilum were three in the right, and two in the left. The right and left ovarian veins drained to the renal veins. There were 12 minor calyces distributed normally in the right kidney, 13 minor calyces distributed radially in the left kidney, and 3 minor calyces in the isthmus, composed of parenchyme. The left portion of the horseshoe kidney was concluded to have developed poorly, on the basis of incomplete ascension and abnormal rotation during development, the imperfect configuration of the renal shape, and the abnormal distribution of the renal vessels and renal calyces.