Endoscopic management of Urolithiasis on a pediatric patient with a solitary kidney and an Ileal conduit

@#Management of nephrolithiasis in patients with urinary diversions pose a unique therapeutic challenge for the following reasons: 1) retrograde ureteral access is difficult to perform through a bowel diversion and 2) percutaneous renal access becomes challenging because of inability to do a retrograde pyelogram. For this reason, image-guided access through a combined ultrasound and fluoroscopic guidance are both necessary. This clinical problem becomes even more complicated when dealing with a solitary functioning kidney. Treatment should be precise in order to avoid any complications that may progress to renal failure. Presented here is a 15-year-old male adolescent who had previously undergone a radical cystectomy with an ileal conduit for a rhabdomyosarcoma of the bladder last 2008, and complained of flank pain, fever and foul-smelling urine. Imaging studies showed left obstructive hydronephrosis with ureterolithiasis and nephrolithiasis, and an atrophic contralateral kidney. A preliminary nephrostomy tube drainage was done to recover renal function, followed later by percutaneous endoscopic stone management. Discussed here are the challenges involved in his therapy as well as the advantages of a stepwise approach including the short-term outcomes.

Hydroperitoneum: A unique complication of Supine Percutaneous Nephrolithotomy on an Ectopic Solitary Pelvic Kidney

@#A 47-year-old male complained of anuria for 2 days with elevated creatinine of 14 mg/dL on admission. Patient underwent emergent hemodialysis. Non-contrast CT showed a solitary ectopic pelvic kidney with a 2 cm. pelvolithiasis and a 1 cm upper pole calyceal stone with obstructive hydronephrosis. He therefore underwent ultrasound-guided nephrostomy tube placement. Once clinically stable, the patient underwent a multi-tract supine PCNL. Intraoperatively, the authors noted tense abdominal distention accompanied by hypotension during the procedure. A diagnosis of compartment syndrome secondary to hydroperitoneum was considered. An indwelling stent and a nephrostomy tube were placed. An abdominal pigtail drain was placed removing three liters of fluid. The patient remained intubated for 3 days. He underwent blood transfusion. He required two 2 sessions of hemodialysis postoperatively. The patient was discharged in stable condition on postoperative day 22. Hydroperitoneum is a potential complication of PCNL in ectopic pelvic kidneys. Its prompt recognition, followed by immediate aspiration of intraabdominal fluid and drain placement is life-saving.

Síndrome de Herlyn-Werner-Wunderlich: reporte de caso y revisión de la literatura / Herlyn-Werner-Wunderlich syndromne: case review and report of the literatura

RESUMEN Objetivo: Presentar un reporte de caso de síndrome de Herlyn Werner Wunderlich (SHWW) y hacer una revisión de la literatura para determinar los hallazgos clínicos e imagenológicos en estas pacientes. Materiales y métodos: Se presenta el caso de una mujer de 16 años que consultó, en un hospital de las fuerzas militares en Bogotá, por dolor pélvico recurrente, su diagnostico final fue SHWW. Se realizó una búsqueda sistemática de la literatura en las diferentes bases de datos, revisiones sistemáticas, cohortes, series de casos y reportes de casos en pacientes con diagnóstico de SHWW en cualquier edad, se obtuvo información sobre las características de presentación clínica, y las tecnologías diagnósticas más frecuentemente utilizadas. Se hace resumen narrativo de los hallazgos. Resultados: Se incluyeron 77 publicaciones, un total de 676 pacientes. Los síntomas más frecuentes fueron dismenorrea (63,9 %), seguido de dolor pélvico (35,2%). Las tecnologías diagnósticas más utilizadas fueron el ultrasonido pélvico en un 92,1% y la resonancia magnética nuclear en un 74,6% de los casos. La histeroscopia y laparoscopia son poco utilizados en el diagnóstico. Conclusión: El SHWW es una entidad poco frecuente, debe hacer parte del estudio complementario de la agenesia renal del paciente pediátrico y del diagnóstico diferencial de la dismenorrea primaria en pacientes en la adolescencia. Se requiere evaluar con estudios de cohorte más grandes la utilidad de la histeroscopia en estas pacientes.

ABSTRACT Objective: To report a case of Herlyn-Werner-Wunderlich syndrome (HWWS) and conduct a review of the literature to determine clinical and imaging findings in these patients. Material and methods: A 16-year-old female patient who presented to a military hospital in Bogota complaining of recurrent pelvic pain, receiving a final diagnosis of HWWS. A systematic search was conducted in the different databases of systematic reviews, cohort studies, case series and case reports of patients of any age diagnosed with HWWS. Clinical presentation characteristics and the most frequent diagnostic technologies used were obtained. A narrative summary of the findings is presented. Results: Overall, 77 publications with a total of 676 patients were included. The most frequent symptom was dysmenorrhea (63.9%), followed by pelvic pain (35.2%). The most frequently used diagnostic technologies were pelvic ultrasound in 92.1% of cases and nuclear magnetic resonance in 74.6%. Hysteroscopy and laparoscopy are seldom used for diagnosing this condition. Conclusion: HWWS is an infrequent disease condition. It must be part of the work-up for renal agenesis in pediatric patients and of the differential diagnosis in primary dysmenorrhea in adolescence. The role of hysteroscopy in this condition must be further assessed in larger cohort studies.

Mini-percutaneous nephrolithotomy in a child with multiple urogenital anomalies and a solitary duplex kidney

ABSTRACT Purpose: To report a case of successful removal of right staghorn renal calculi in a 3-year-old girl with Arnold-Chiari malformation and multiple urogenital anomalies. Case report: A 3-year-old female child with the diagnosis of Arnold-Chiari type 2 malformation was referred to our clinic due to presence of 9 kidney stones with a total volume of 10743mm3. The total of the longest diameters of all stones was calculated as 11.4cm. The patient had a urogenital septum, bifid bladder, and duplicated collecting system on the right side. An 18F Amplatz sheath was placed and mini-percutaneous nephrolithotomy was performed successfully by laser and pneumatic lithotripter. Any residual urinary tract stones or urinary tract infection were not detected during the 6th-month follow-up. Conclusion: Urolithiasis requires a thorough understanding of the underlying causes, as well as an effective and minimally invasive treatment. It is important for urologists to understand the complexity of the optimal stone management in pediatric patients in order to maximize treatment efficacy and minimize morbidity. We conclude that it is essential to treat urolithiasis in a single session in children with urogenital anomalies and accompanying congenital anomalies who have past surgical history.

Quiste enterogénico y agenesia renal en la edad adulta / Enterogenic cyst and renal agenesis in adulthood

Introducción: Los quistes enterogénicos son malformaciones producidas durante el período de diferenciación y desarrollo embriológico del intestino primitivo. Objetivo: Presentar una malformación digestiva infrecuente en la edad adulta. Caso clínico: Paciente de 58 años que acude por dolor abdominal y aumento de volumen en hipogastrio. Al examen físico se palpa masa en hipogastrio de 15 cm de diámetro. La tomografía axial computarizada simple y contrastada mostró una masa tumoral en la excavación pélvica hipodensa con densidad de 18 UH, pared gruesa, de 2 cm, que realza con el contraste y agenesia renal derecha. El estudio anatomo-patológico informa quiste enterogénico. Discusión: La clínica de las duplicaciones intestinales es inespecífica, el dolor abdominal recurrente y la presencia de masa abdominal, son frecuentes. El diagnóstico preoperatorio de las duplicaciones intestinales es infrecuente, depende de la disponibilidad de estudios de imagen y la sospecha clínica. Es infrecuente la asociación de quiste enterogénico y agenesia renal. Conclusión: Se presenta una paciente masculino con una masa pélvica de etiología dudosa y agenesia renal derecha, en el que se diagnosticó un quiste enterogénico. La asociación de quiste enterogénico y agenesia renal es una malformación rara en el adulto(AU)

Introduction: Enterogenic cysts are malformations produced during the period of differentiation and embryological development of the primitive intestine. Objective: To report an rare digestive malformation in adulthood. Clinical case report: A 58-year-old patient came for abdominal pain and increased volume in the hypogastrium. On physical examination, a 15-cm diameter mass was palpated in the hypogastrium. Simple and contrast computerized axial tomography showed a tumor mass in the hypodense pelvic excavation with a density of 18 HU, a thick wall of 2 cm, which was enhanced with contrast and right renal agenesis. The anatomo-pathological study reports an enterogenic cyst. Discussion: The symptoms of intestinal duplications are nonspecific, recurrent abdominal pain and the presence of an abdominal mass are frequent. The preoperative diagnosis of intestinal duplications is occasional, it depends on the availability of imaging studies and clinical suspicion. The association of enterogenic cyst and renal agenesis is uncommon. Conclusion: A male patient is reported, with a pelvic mass of doubtful etiology and right renal agenesis, in whom an enterogenic cyst was diagnosed. The association of enterogenic cyst and renal agenesis is a rare malformation in adults(AU)

Cambios fisiológicos y morfológicos tempranos en el riñón remanente de donantes vivos / Early physiological and morphological changes in the remnant kidney of living donors

RESUMEN Con el objetivo de describir los cambios funcionales y morfológicos tempranos en el riñón remanente de donantes vivos, se realizó un estudio retrospectivo en el Hospital Cayetano Heredia, en el que se incluyeron 55 individuos. De las historias clínicas, se obtuvieron los datos clínicos y demográficos, así como la depuración de creatinina, la proteinuria, la presión arterial y las dimensiones renales a los 1, 2, 3, 6 y 12 meses después de la donación del riñón. La edad media fue de 40,88 (±9,84) años; el 80% eran mujeres y el índice de masa corporal medio fue de 25,68 (±3,5) kg/m2. Se utilizaron modelos lineales y cuadráticos para estudiar las variables fisiológicas y morfológicas. Durante el tiempo de seguimiento, la tasa de filtración glomerular, la proteinuria, la presión arterial diastólica y la longitud de los riñones mostraron cambios significativos (p < 0,05).

ABSTRACT In order to describe the early functional and morphological changes in the remnant kidney of living donors, a retrospective study was carried out at the Cayetano Heredia Hospital. Data from 55 individuals was included. Clinical and demographic data were obtained from the clinical records, as well as data for creatinine clearance, proteinuria, blood pressure and renal dimensions at 1, 2, 3, 6 and 12 months after kidney donation. The mean age was 40.88 (±9.84) years; 80% were women and the mean body mass index was 25.68 (±3.5) kg/m2. Linear and quadratic models were used to study physiological and morphological variables. During the follow-up time, glomerular filtration rate, proteinuria, diastolic blood pressure, and kidney length showed significant changes (p < 0.05).

Síndrome de OHVIRA, a propósito de un caso / A case of OHVIRA syndrome

Resumen Introducción: El síndrome de obstrucción hemivaginal y anomalía renal ipsilateral (OHVIRA, por sus siglas en inglés) o también llamado síndrome de Herlyn-Werner-Wunderlich es una rara malformación mulleriana que consiste en la presencia de útero didelfo, obstrucción hemivaginal y anomalía renal ipsilateral. La incidencia de malformaciones mullerianas son del 2 al 3%, y la menos común es el síndrome de OHVIRA. Objetivo: Descripción de un caso excepcional de alteraciones mullerianas poco conocido. Presentación del caso: Adolescente de 14 años con historia de dolor pélvico, dismenorrea e infecciones urinarias a repetición. Se estudió con gammagrafía renal que reveló agenesia renal derecha y resonancia magnética pélvica que mostró útero didelfo con hematocolpos derechos. Se confirmaron los hallazgos por laparoscopia diagnóstica. Conclusiones: Este síndrome es poco conocido. Debe sospecharse en casos de agenesia renal, dolor pélvico crónico y alteraciones menstruales en pacientes postpuberes. El abordaje es multidisciplinario.

Abstract Introduction: The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as the Herlyn-Werner-Wunderlich syndrome, is a rare müllerian anomaly characterized by didelphic uterus, hemivaginal obstruction, and ipsilateral renal anomaly. The incidence of müllerian malformations is 2% - 3%, OHVIRA being the least common of these cases. Objective: To describe an exceptional case of a müllerian disorder. Case presentation: A 14-year-old adolescent with a history of pelvic pain, dysmenorrhea, and recurrent urinary infections. Renal gammagraphy analysis revealed right renal agenesis, and pelvic magnetic resonance imaging showed a didelphic uterus with right hematocolpos. These findings were confirmed by diagnostic laparoscopy. Conclusions: This syndrome is not well known. Renal anegesis, chronic pelvic pain, and menstrual abnormalities in post-pubertal patients must be presumed as indicators of OHVIRA, whose treatment requires a multidisciplinary approach.

Long-term analysis of safety and efficacy of standard percutaneous nephrolithotomy in patients with solitary kidneys / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the long-term efficacy and safety of ultrasound-guided percutaneous nephrolithotomy (PCNL) in the treatment of patients with solitary kidney stones.@*METHODS@#The clinical data of 22 patients with solitary kidney stones treated with PCNL in Peking University People's Hospital from September 2008 to June 2014, with the follow-up data of more than 5 years were analyzed retrospectively. Perioperative indicators, postoperative stone free rate (SFR) and incidence of complications were recorded. Ultrasonography was used to evaluate the long-term stones recurrence rate. Serum creatinine and estimated glomerular filtration rate (eGFR) were used to assess the long-term renal function.@*RESULTS@#In this group of 22 patients, the average age was (50.3±11.8) years, with 10 cases of anatomic solitary kidneys, 12 functional solitary kidneys, and the median stone diameter was 1.65 (1.1-3.9) cm. All the patients had multiple stones, including 7 cases of staghorn stones. The median pre-operative serum creatinine was 104.5 (60.0-460.0) μmol/L, and the mean eGFR was (60.3±29.4) mL/min, showing no statistically significant difference compared with that before surgery. The mean operative time was (88.2±42.0) min, and there were 11 cases of single-channel and double-channel PCNL. The median serum creatinine on the first day after surgery was 102.0 (63.0-364.0) μmol/L, and the mean eGFR was (58.0±25.1) mL/min. The mean postoperative hospital stay was (8.7±5.2) days. In this group, 5 patients (22.7%) presented short-term complications, among which 4 patients presented postoperative infection and massive hemorrhage at the same time, which improved after conservative treatment, and 1 patient presented pleural injury and improved after closed thoracic drainage. Two patients (9.1%) developed long-term complications, and ureteral stricture occurred 3 months after operation, which improved after balloon dilatation. The median follow-up time was 6.2 (4.7-11.1) years. The median serum creatinine at the last follow-up was 104.0 (72.4-377.0) μmol/L, and the mean eGFR was (60.1±23.7) mL/min, showing no statistically significant difference compared with that before surgery. Renal function decreased in 6 patients (27.3%). Initial and final SFR were 72.7% and 100%, respectively. During the 6.2-year follow-up, 9 patients (40.9%) experienced recurrence of kidney stone. After stone recurrence, 13 lithotomy surgeries were performed, and the SFR by the latest follow-up was 63.6%.@*CONCLUSION@#This study had the longest follow-up time for patients with solitary kidney stones after PCNL reported at home and abroad. Ultrasound-guided standard PCNL was safe and effective in the treatment of solitary kidney stones. Long-term follow-up results showed that the recurrence rate of kidney stones was still high, but the long-term renal function was stable after operation, and some patients showed mild renal function decline.

The natural history of solitary post-nephrectomy kidney in a pediatric population

ABSTRACT Introduction: Children with a solitary post-nephrectomy kidney (SNK) are at potential risk of developing kidney disease later in life. In response to the global decline in the number of nephrons, adaptive mechanisms lead to renal injury. The aim of this study was to determine the prevalence and time of onset of high blood pressure (HBP), proteinuria, glomerular filtration rate (GFR) disruption and renal tubular acidosis (RTA) in children with SNK. Materials and methods: After obtaining the approval from our institution's ethics committee, we reviewed the medical records of patients under 18 years of age who underwent unilateral nephrectomy between January 2005 and December 2015 in three university hospitals. Results: We identified 43 patients, 35 (81.4%) cases of unilateral nephrectomy (UNP) were due to a non-oncologic pathology and Wilm's tumor was identified in 8 (18.6%) cases. In patients with non-oncologic disease, 9.3% developed de novo hypertension, with an average time of onset of 7.1 years, 25% developed proteinuria de novo, with an average time of onset of 2.2 years. For GFR, 21.8% presented deterioration of the GFR in an average time of 3.4 years. Ten (43.5%) patients developed some type of de novo renal injury after UNP. Patients with oncologic disease developed the conditions slowly and none of them developed proteinuria. Conclusions: Taking into account the high rate of long term postoperative renal injury, it can be considered that nephrectomy does not prevent this disease. The follow-up of children with SNK requires a multidisciplinary approach and long-term surveillance to detect renal injury.

Incidencia de ectasia pielocalicial neonatal en el Hospital Metropolitano de Quito, 2014 a 2018: continuación de un estudio / Pyelocalyceal incidence of neonatal ectasia in Hospital Metropolitano of Quito, from 2014 to 2018: the next part of the study

Objetivo: determinar la incidencia de ectasia pielocalicial e hidronefrosis en el Hospital Metropolitano de Quito durante el período 2014-2018. Métodos: se realizó un estudio analítico retrospectivo de incidencia de ectasia pielocalicial en el Hospital Metropolitano de Quito. Se presentaron 7 casos de neonatos diagnosticados de esta entidad durante un período de 4 años 5 meses, desde 2014 a 2018 (incidencia: 0,18%) en el Hospital. Edad: 25 meses promedio; 5 de sexo masculino y 2 de sexo femenino; 57% diagnosticados durante el período prenatal y 43% en el periodo neonatal. Resultados: de los neonatos diagnosticados por ecografía prenatal, en 100% se ratificó el diagnóstico ecográfico posterior al nacimiento. En este estudio se encontró que 85,7% padecía ectasia I-II, 0% ectasia III-IV y en 14,28% no se pudo identificar el tamaño de la dilatación. El 71,42% presentó asociación con hidronefrosis, 14,28% con doble sistema pielocalicial, 42,85% megauréter, 14,28% agenesia renal. Localización: 57,14% izquierdo, 28,57% derecho y 14,28% bilateral. De los 7 pacientes, 14% acudió al Hospital por fiebre, 57% para control diagnóstico prenatal, 14% por control posnatal y 14% por hematuria. Conclusiones: la ectasia pielocalicial es una entidad de importancia que debe ser diagnosticada durante la etapa prenatal y confirmada en la etapa neonatal para lograr su correcto y oportuno manejo.

Objective: Determine the incidence of pyelocalyceal ectasia at Hospital Metropolitano of Quito from 2014 to 2018. Methods: A retrospective analytical study of pyelocalyceal ectasia incidence was performed at the Metropolitan Hospital of Quito. There were 7 patients with this disease from 2014 to 2018 (incidence of 0,18%) at the Hospital. The average age was 25 months. Of this, 5 were male and 2 female, 57% were diagnosed during prenatally and 43% of neonatal form. Results: From the group of patients that were diagnosed by prenatal ultrasound, 100% the diagnosis was confirmed by ultrasound performed within a few days of birth. In this study it was found that 85,7% had I-II ectasia grade, 0% ectasia III-IV, and 14,28% could not be identified due to lack of information about the size of expansion. Of the cases, 71,42% was associated with hydronephrosis, 14,28% with double pyelocalicial system, 42,85% megaureter, 14,28% renal agenesis. 57% affected the left side, 28% the right side and 14% afected bilaterally. From the 7 patients, 14% went to hospital due to fever, 57% for prenatal diagnosis, 14% for postnatal control, and 14% for hematuria. Conclusions: The pyelocalyceal ectasia is an important entity that must be diagnosed during the prenatal stage and confirmed in newborns for its correct management.

HDR syndrome in a Colombian woman with a genital tract malformation: first case report in Latin America / Síndrome HDR en una mujer colombiana con una malformación genitourinaria: primer reporte de caso en Latinoamérica

ABSTRACT Objectives Hypoparathyroidism, sensorineural deafness and renal disease (HDR) syndrome, also known as Barakat syndrome, is an autosomal dominant transmission hereditary disease with a wide range of penetrance and expressivity. Haploinsufficiency of the GATA3 two finger zinc transcription factor is believed to be its cause. This is the first time this orphan disease is reported in Latin America, so the publishing of this report is expected to raise awareness on these types of syndrome, that are usually underdiagnosed in our region, which in turn causes an increase in the years lost to disability (YLDs) rates, as well as higher costs to be assumed by public health systems. Methods A 36-year-old Colombian woman diagnosed with parathyroid gland agenesis was referred from the Endocrinology Service to the Outpatient Service. According to her medical record, in the past she had developed hypocalcaemia, left renal agenesis, hypoparathyroidism, bicornate uterus and sensorineural hearing loss. Through a genetic analysis a pathological mutation on the short arm of the GATA 3 gen (c.404dupC, p Ala136 GlyfsTER 167) was confirmed, which led to a HDR syndrome diagnosis. Discussion This case proves that there is a possibility that mutations described in other continents may be developed by individuals from our region. Regardless of ethnicity, Barakat syndrome should be considered as a possible diagnosis in patients presenting the typical triad that has been described for this condition, since there could be underdiagnosis of this disease in Latin-America due to the lack of knowledge on this condition in said region, and that genetic counseling in these patients is of great importance for the implications of the syndrome in future generations.(AU)

RESUMEN Objetivos El síndrome de hipoparatiroidismo, sordera neurosensorial y displasia renal (HDR) también llamado síndrome de Barakat, es una enfermedad hereditaria de transmisión autosómica dominante con amplia penetrancia y expresividad genética. El síndrome es causado por la haploinsuficiencia del factor de transcripción de dedos de Zinc GATA3. Esta es la primera vez que esta enfermedad huérfana es reportada en latinoamerica, y buscamos generar consciencia de la presencia de estas enfermedades, las cuales usualmente son infradiagnósticadas en nuestro medio y llevan a un aumento de años perdidos por discapacidad y costos para el sistema de salud pública. Métodos Una mujer colombiana de 36 años ingresó a consulta externa de genética referida por el servicio de endocrinología por una agenesia de paratiroides. La paciente tenía antecedentes de hipocalcemia, agenesia renal izquierda, hipoparatiroidismo, sordera neurosensorial y útero bicorneo. Se realizó un análisis genético que confirmo una mutación patológica en el brazo corto del gen GATA3 (c.404dupC, p Ala136 GlyfsTER 167) diagnóstica del síndrome de Barakat. Discusión Este caso demuestra la posibilidad de existencia de mutaciones descritas en otros continentes en nuestra población. Sin importar la etnia, el síndrome de Barakat debe ser estudiado en pacientes que presenten la triada típica, ya que podría existir un infra diagnóstico de la enfermedad secundario al desconocimiento de la misma en Latinoamérica y teniendo en cuenta la importancia que tiene la consejería genética en estos pacientes por las implicaciones de la enfermedad en futuras generaciones.(AU)

Solitary functioning kidney in children: clinical implications / Rim solitário em funcionamento em crianças: implicações clínicas

ABSTRACT Introduction: Children with solitary functioning kidney (SFK) are prone to develop long term problems, which are not well represented in the literature. The extent to which the presence of associated congenital anomalies of kidney and urinary tract (CAKUT) further de-stabilize renal function is to be addressed. Objective: This study was conducted to evaluate the etiology, presentation, presence of CAKUT, and renal damage in children with SFK. Methods: All children with SFK who presented to the department of pediatric surgery from March 2014 to May 2016 were included in the study. Children with malignancy were excluded from the study. Results: Of the 20 patients with SFK, 14 (70%) had primary SFK (8 with agenesis and 6 with multicystic dysplastic kidney), 6 (30%) belonged to secondary SFK group, among them 3 had pelviureteric junction obstruction, 2 had posterior urethral valves and 1 had vesicoureteric reflux. Eight (40%) had associated CAKUT, 4 (20%) were asymptomatic while 8 (40%) had UTI and 6 (30%) had hypertension. Ten (50%) patients had reduced glomerular filtration rate (GFR) suggesting compromised renal function. Conclusion: Children with SFK have high morbidity especially when associated with ipsilateral CAKUT. Long-term periodical follow up is essential in these patients to improve clinical outcome.

RESUMO Introdução: Crianças com rim solitário funcional (RSF) tendem a desenvolver problemas de longo prazo, que não são bem representados na literatura. Devemos abordar o quanto a presença de malformações congênitas do rim e do trato urinário (CAKUT) desestabiliza ainda mais a função renal. Objetivo: Este estudo foi realizado para avaliar a etiologia, apresentação, presença de CAKUT e dano renal em crianças com RSF. Métodos: Todas as crianças com RSF que se apresentaram no departamento de cirurgia pediátrica de março de 2014 a maio de 2016 foram incluídas no estudo. Crianças com doença maligna foram excluídas do estudo. Resultados: dos 20 pacientes com RSF, 14 (70%) tinham RSF primário (8 com agenesia e 6 com rim displásico multicístico), 6 (30%) pertenciam ao grupo RSF secundário, dentre eles 3 tinham obstrução da junção pelveuretérica, 2 tinham valvas uretrais posteriores e 1 refluxo vesico-uretral. Oito (40%) tinham ACRTU associado, 4 (20%) estavam assintomáticos, enquanto 8 (40%) tinham ITU e 6 (30%) tinham hipertensão. Dez (50%) pacientes apresentaram redução da taxa de filtração glomerular (TFG), sugerindo comprometimento da função renal. Conclusão: Crianças com RSF apresentam alta morbidade, especialmente quando associadas à CAKUT ipsilateral. O acompanhamento periódico a longo prazo é essencial nesses pacientes para melhorar o resultado clínico.

Zinner syndrome:A case report and review of the literature / 中华男科学杂志

Objective@#To explore the clinical diagnosis and treatment of seminal vesicle cyst (SVC) associated with ipsilateral renal agenesis (Zinner syndrome) in order to promote the understanding of the disease.@*METHODS@#We retrospectively analyzed the clinical data about 1 case ofZinner syndrome diagnosed and treated in our hospital and reviewed the literature related to this disease in domestic and foreign authoritative databases.@*RESULTS@#The patient was a 23-year-old male, diagnosed with Zinner syndrome, treated bytransrectal aspiration of SVC, and discharged from hospital 3 days postoperatively. Follow-upat 6 months after discharge found that the patient no longer felt perineal discomfort in the endstage of urination, but transrectal ultrasonography of the prostate revealedthe samevolume of fluid in the left seminal vesicles as before,which indicated recurrence.@*CONCLUSIONS@#SVC associated with ipsilateral renal agenesis can be considered asZinner syndrome. Transrectal aspiration of SVCcan relieve the local symptoms of the patient but relapse may easilyoccur. Therefore it is not recommended as the first-choice treatment of the disease.

Agenesia renal unilateral e criptorquidismo ipsilateral em um felino: relato de caso / Unilateral renal agenesis and ipsilateral cryptorchidism in a cat: case report

A agenesia renal é uma afecção congênita rara na espécie felina, frequentemente associada a uma malformação reprodutiva. O presente trabalho relata o caso de um felino com agenesia renal unilateral associada a criptorquidismo ipsilateral, com ênfase no diagnóstico, tratamento e acompanhamento. O paciente foi conduzido ao Hospital de Clínicas Veterinárias da Universidade Federal do Rio Grande do Sul para avaliação de criptorquidismo. A agenesia renal foi um achado durante a ecografia abdominal do felino. Durante a laparotomia, foi confirmada a ausência do rim e ureter direito, hipertrofia do rim esquerdo e presença de um testículo ectópico. O paciente teve alta após a recuperação anestésica e se mantém clinicamente estável, transcorridos seis meses da cirurgia. A agenesia renal unilateral é uma condição compatível com a vida, contanto que o rim existente apresente funcionamento aceitável. Assim, sugere-se que a possibilidade de rim único em felinos criptorquidas deve ser investigada sempre que possível, tendo em vista a alta correlação entre essas malformações, e objetivando um acompanhamento da função renal do paciente ao longo da vida.(AU)

Renal agenesis is a rare disorder in feline species, commonly associated with reproductive malformation. This study aims to report the case of a cat with unilateral renal agenesis combined with ipsilateral cryptorchidism, emphasizing the diagnosis, treatment and patient follow up. The patient was taken to the Veterinary Hospital of the Federal University of Rio Grande do Sul to evaluate the cryptorchidism. The renal agenesis was an incidental finding during the abdominal ultrasound. At laparotomy, the absence of the right kidney and ureter was confirmed, hypertrophy of the left kidney and the presence of an ectopic testicle were found. The patient was discharged after recovering from anesthesia and remains clinically stable six months after surgery. The unilateral renal agenesis is a life compatible condition as long as the existing kidney has an acceptable performance. Therefore, it is suggested that the chance of a single kidney in cats whit cryptorchidism should be investigated, given the correlation between these malformations, and aiming to monitor renal function throughout the life of the patient.(AU)

Anomalías congénitas del sistema renal: agenesia renal izquierda asociada a variante arteriovenosa renal derecha y vena cava inferior / Congenital anomalies of the renal system: left renal agenesis associated with right renal arteriovenous variant and inferior vena cava

Este trabajo describe la variante anatómica en un caso incidental de disección en la Escuela de Medicina de la Universidad de Costa Rica, el cual presenta agenesia renal izquierda con variante arteriovenosa renal derecha que consta de cinco arterias renales y dos venas renales, asociado a la variante anatómica del origen de la vena cava inferior ya que es superior a la arteria mesentérica inferior.

This work describes the anatomic variation from an incidental case of dissection founded at Costa Rica’s University School of Medicine, which presents left renal agenesia with a right arteriovenous variant which consists of five renal arteries and two renal veins, related to the anatomic variant from the inferior cava vein, this variation its superior to the inferior mesenteric arterie.

Agenesia renal unilateral a propósito de un caso que se presentó en el Hospital Provincial General Latacunga Servicio de Pediatría / Unilateral renal agenesis regarding a case that was presented at the General Provincial Hospital Latacunga Pediatric Service

Las malformaciones congénitas inherentes a las estructuras renales y conductos ureterales están asociados con defectos en la formación del brote ureteral o divertículo metanéfrico. La agenesia renal puede ser unilateral (izquierda o derecha), con una frecuencia de 1: 500 o bilateral del 0.3: 1000. La agenesia renal congénita unilateral se caracteriza por no presentar sintomatología mientras que la bilateral es incompatible con la vida. la agenesia renal unilateral generalmente se descubre durante el ultrasonido prenatal de rutina, durante el estudio de pacientes con otras anomalías, o que presentan problemas del tracto urinario recurrentes. Se analiza un caso de dos hermanos con la misma malformación. Uno de ellos tubo una operación para ano imperforado a los dos días de edad. se enfatiza la importancia de una valuación completa de cada uno de los pacientes pediátricos.

Congenital malformations inherent in the renal structures and ureteral ducts are associated with defects in the formation of the ureteral bud or metanephric diverticulum. Renal agenesis can be unilateral (left or right), with a frequency of 1: 500 or bilateral of 0.3: 1000. Unilateral congenital renal agenesis is characterized by no symptoms, while bilateral is incompatible with life. Unilateral renal agenesis is usually discovered during routine prenatal ultrasound, during the study of patients with other abnormalities, or who have recurrent urinary tract problems. A case of two brothers with the same malformation is analyzed. One of them had an operation for an imperforate anus at two days of age. The importance of a complete evaluation of each of the pediatric patients is emphasized.