RESUMO
The upper urinary tract is the most common human organ system affected by congenital anomalies. A Horseshoe kidney is a fusion anomaly, it can be described as a fusion across the midline of 2 distinct functioning kidneys. The incidence of renal tumors in a Horseshoe kidney is higher than in the normal population. We present a 60-year-old male patient with a history of Horseshoe kidney and a diagnosis of clear cell renal cell carcinoma who underwent a combined therapeutic approach, guided by interventional radiology. This approach involved selective transarterial embolization and microwave ablation. Three months after surgery and with abdominal MRI follow-up, there is evidence of a non-viable tumor, indicating a favorable response to the intervention.
RESUMO
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.
Assuntos
Humanos , Masculino , Pré-Escolar , Biópsia , Insuficiência Renal Crônica , Rim Fundido , Nefrectomia , Glomerulosclerose Segmentar e Focal , Decúbito Dorsal , Litíase , Glomerulonefrite , Hipospadia , Rim , NeoplasiasRESUMO
Objective: To report the outcomes of percutaneous nephrolithotomy (PCNL) in horseshoe kidneys (HSK) in 12 institutions worldwide and evaluate the impact of patient position during operation. Methods: We carried out a retrospective analysis of PCNL procedures performed between 2008 and 2018 in patients with HSK. Pre-, peri-, and postoperative data were collected, and a subgroup analysis was performed according to patient position. Success was defined as an absence of >4-mm fragments. Values of p < 0.05 were considered significant. Results: We analyzed 106 procedures. The transfusion, complication, and immediate success rates (ISRs) were 3.8%, 17.5%, and 54.7%, respectively. The final success rate (FSR) increased to 72.4% after a mean of 0.24 secondary procedures. Logistic regression showed that higher body mass index (BMI) and stone size were significantly associated with residual fragments ≥4 mm. Sixty-seven patients (63.2%) were treated in prone and 39 (36.8%) in supine position. The prone group had a significantly higher BMI than the supine group (30.1 vs 27.7, p = 0.024). The transfusion, complication, and ISRs between the prone and supine groups were 4.5% vs 2.6% (p = 0.99), 16.9% vs 18.4% (p = 0.99), and 52.5% vs 69.2% (p = 0.151), respectively. Surgical time was significantly longer in the prone group (126.5 vs 100 minutes, p = 0.04). Upper pole was the preferred access in 80.3% of the prone group and 43.6% of the supine group (p < 0.001). The prone group had significantly more Clavien 2 complications than the supine (p = 0.013). The FSR in the prone and supine groups increased to 66.1% and 82.1% after 0.26 and 0.21 secondary procedures, respectively. No complications higher than Clavien 3 occurred. Conclusion: PCNL in patients with HSK is safe and effective with a low complication rate. Higher BMI and stone size negatively impacted outcomes. Supine PCNL may be an option for treating kidney stones in patients with HSK.
Assuntos
Rim Fundido , Cálculos Renais , Nefrolitotomia Percutânea , Nefrostomia Percutânea , Rim Fundido/cirurgia , Humanos , Cálculos Renais/cirurgia , Nefrolitotomia Percutânea/efeitos adversos , Nefrostomia Percutânea/efeitos adversos , Posicionamento do Paciente , Decúbito Ventral , Estudos Retrospectivos , Decúbito Dorsal , Resultado do TratamentoRESUMO
Horseshoe kidney (HSK) is a rare anatomic anomaly that poses surgical challenges in the setting of abdominal aortic aneurysm repair. An endovascular approach is safer for patients yet carries technical challenge because of variable renal vasculature. We present the case of a patient with an infrarenal abdominal aortic aneurysm and concomitant HSK who underwent successful repair with a custom fenestrated endograft with preservation of a midaortic renal artery. Complex endovascular aneurysm repair options for HSK include chimneys and fenestrated stent grafts. We suggest that the availability of custom fenestrated grafts and ongoing skill enhancement among vascular surgeons may make this approach more favorable.
RESUMO
Ammonium is an important source of nitrogen for amino acid synthesis and is necessary for normal acid base balance. When ammonium concentrations are high it becomes a toxic compound. Hyperammonemia is a metabolic emergency. When underdiagnosed and not treated appropriately, it produces severe neurological sequelae and/or death. The clinical presentation of hyperammonemic encephalopathy varies, and includes from personality disorders, psychiatric disorders, confusion, irritability, lethargy, seizures to coma. Hyperammonemia occurs with an increase in ammonium production, as in intestinal hemorrhage, or with a decrease in the elimination of ammonium, such as in congenital metabolic errors, hepatic insufficiency or drug intoxication. As we can see, it may have multiple origins, but congenital errors of metabolism are always suspected as one of the causes. However, there are less frequent causes, such as urinary tract infection, especially in predisposing conditions. We describe the case of a 2-year-old boy with a history of horseshoe kidney and right ureterohydronephrosis, surgical correction of imperforate anus and rectal bladder fistula. This patient presented hyperammonemia with encephalopathy (Glasgow 7/15) while undergoing a urinary infection with Corynebacterium riegelii. Hyperammonemia is the result of the production in the dilated urinary tract of large amounts of ammonium due to bacterial urease and its subsequent reabsorption in the systemic circulation. The patient improved clinically (Glasgow 15/15) after parenteral antibiotic therapy and urinary tract clearance
El amonio es una fuente importante de nitrógeno para la síntesis de aminoácidos y necesario para el balance ácido base; si se encuentra elevado, se convierte en un compuesto tóxico. La hiperamoniemia es una urgencia metabólica; cuando no es diagnosticada y tratada de manera oportuna, produce graves secuelas de tipo neurológico o la muerte. La presentación clínica de la encefalopatía hiperamoniémica es variable, pudiéndose observar trastornos en la personalidad, trastornos psiquiátricos, confusión e irritabilidad, letargia, convulsión y coma. La hiperamoniemia se presenta por aumento en la producción de amonio, como en la hemorragia intestinal, o por disminución de la eliminación del mismo, como ocurre en los errores congénitos del metabolismo, en la insuficiencia hepática o en la intoxicación por fármacos. Puede tener múltiples orígenes, pero los errores congénitos del metabolismo son una de las causas que siempre se sospechan. Sin embargo, existen causas menos frecuentes, como la infección del tracto urinario (sobre todo en condiciones que predispongan a las mismas). Describimos aquí el caso de un niño de 2 años, con antecedentes de riñón en herradura y ureterohidronefrosis derecha, corrección quirúrgica de ano imperforado y fistula recto vesical. Este paciente presentó hiperamoniemia con encefalopatía (Glasgow 7/15) mientras cursaba una infección urinaria por Corynebacterium riegelii. La hiperamoniemia es el resultado de la producción en el tracto urinario dilatado de grandes cantidades de amonio, debido a la ureasa bacteriana y su posterior reabsorción en la circulación sistémica. El paciente mejoró clínicamente (Glasgow 15/15) después de la terapia antibiótica parenteral y desobstrucción de tracto urinario
Assuntos
Humanos , Masculino , Pré-Escolar , Infecções Urinárias , Encefalopatias , Corynebacterium , HiperamonemiaRESUMO
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.
Assuntos
Humanos , Rim Fundido , Rim/anormalidades , Rim/irrigação sanguíneaRESUMO
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.
Assuntos
Humanos , Masculino , Feminino , Rim Fundido , Rim , Circulação Renal , ColômbiaRESUMO
We present the anatomical study of a horseshoe kidney found during dissection practice at the human anatomy laboratory. The specimen consisted of a renal mass joined at its lower poles by an isthmus composed of renal parenchyma. We provide a macroscopic description of renal blood supply and the excretory system. We also discuss the anatomic and embryologic importance of this anomaly.
Se presenta el estudio anatómico de un riñón en herradura encontrado durante la práctica de disección en el Laboratorio de Anatomía Humana. La muestra consistió de una masa renal unida a sus polos inferiores por medio de un istmo de parénquima renal. Proporcionamos una descripción macroscópica de suministro sanguíneo renal y el sistema excretor. Asimismo, se discute la importancia anatómica y embriológica de esta anomalía.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Rim/anatomia & histologia , Rim/embriologia , Rim/irrigação sanguínea , Rim/ultraestrutura , Veias Renais/anormalidades , Anatomia Regional , Desenvolvimento Embrionário/genética , Dissecação/métodosRESUMO
El riñón en herradura se encuentra en uno de cada 1000 pares renales y la patología en estos riñones suele tener características anatómicas especiales que se deben tener en consideración para su abordaje quirúrgico. Presentamos el caso de un carcinoma de células claras en este tipo de fusión renal.
Horseshoe kidney has a frequency of 1 in 1000 renal paiss. Diseases in this type of kidneys have anatomical particularities that should be taken into account during surgery. We present the case of a renal cell carcinoma in this type of renal fusion.