Variante incomum da síndrome de Herlyn- -Werner-Wunderlich: relato de caso / Unsual Herlyn-Werner-Wunderlich syndrome: case report

Malformações müllerianas correspondem a variações anatômicas do trato repro- dutor feminino. Comumente assintomáticas, o diagnóstico e a verdadeira incidên- cia são difíceis de determinar. A síndrome de Herlyn-Werner-Wunderlich, clas- sicamente descrita pela tríade útero didelfo, hemivagina cega e agenesia renal ipsilateral também pode ter variações diferentes. Em virtude da mesma origem embrionária dos tratos genital e urinário, anomalias renais devem ser investigadas nesses casos, sendo a mais comum a agenesia renal. Este artigo relata o caso de uma paciente de 18 anos, do sexo feminino, com história de piocolpo por cinco anos. Em propedêutica complementar, foi identificado útero com septação com- pleta associado a hemissepto de terço superior de vagina, formação de piocolpo e agenesia renal à direita. Apesar de não ser a definição clássica, o quadro está incluído nos casos de síndrome de Herlyn-Werner-Wunderlich.

Mullerian malformations correspond to anatomical variations of the female repro- ductive tract. Commonly asymptomatic, the diagnosis and true incidence are difficult to determine. The Herlyn-Werner-Wunderlich syndrome, classically described by the triad: uterus didelphus, blind hemivagina and ipsilateral renal agenesis, can also have different variations. Due to the same embryonic origin of the genital and urinary tracts, renal anomalies must be investigated in these cases, the most common being renal agenesis. This article reports the case of an 18-year-old female patient with a 5-year history of pyocolpus. In complementary exams, a uterus with complete septa- tion was identified, associated with a hemiseptum in the upper third of the vagina, formation of pyocolpus and renal agenesis on the right side. Despite not being the classic definition, it is included in the cases of Herlyn-Werner-Wunderlich syndrome.

Herlyn-Werner-Wunderlich syndrome: A fertility-sparing approach to a rare mullerian anomaly.

The Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by the triad of uterus didelphys, obstructed hemivagina, and renal agenesis. The typical clinical presentation involves chronic pelvic pain, dysmenorrhea, and palpable abdominal mass, related to hematocolpos/hematometra. It is a rare disease, with a challenging clinical and radiological diagnosis. Surgery is the definitive treatment. Complications such as endometriosis, infertility and chronic pelvic pain occur more frequently and severely when diagnosis and treatment are delayed. This is a case report of a twelve-year-old patient admitted to the Gynecology Department of the Federal University of Rio de Janeiro's General Hospital (HUCFF/UFRJ), in March 2021, with progressive symptoms of dysmenorrhea and abdominal distention due to palpable abdominal mass. She had a previous history of congenital solitary kidney. Magnetic Resonance Imaging (MRI) showed a double uterus with hematometra and hematocolpos on the left side, pelvic endometriosis and left renal agenesis. Conservative clinical treatment with inhibition of the hypothalamic-pituitary-ovarian (H-P-O) axis was initiated while a definitive surgical approach was being defined. In June 2022, the patient underwent left hemi-hysterectomy and salpingectomy, achieving full remission of symptoms. Given the rarity of this syndrome and its potential complications, our report aims to familiarize clinicians with it, mostly those who work with children and adolescents, so that more patients have access to early diagnosis and adequate treatment. Consequently, future fertility can be effectively preserved.

Riñón único funcionante y su seguimiento en Pediatría

Contexto: el riñón único funcionante (RUF) es una entidad que puede ser de tipo congénito y es causada por una agenesia renal unilateral o un riñón displásico multiquístico, o adquirido de forma secundaria a una nefrectomía unilateral, condicionando al paciente a un seguimiento estricto rutinario, así como en la implementación de algunas pautas de prevención en los pacientes pediátricos. Objetivo: analizar la importancia del seguimiento en los pacientes pediátricos con RUF. Metodologí:a se hace una revisión de la literatura mostrando desde la embriología, fisiología, etiología, aspectos clínicos y estudios diagnósticos en pacientes con RUF para así, posteriormente, ofrecer unas pautas de seguimiento y manejo en pacientes pediátricos. Resultados: los pacientes con RUF pueden presentar hiperfiltración glomerular compensatoria, seguida de lesión glomerular con hipertensión, albuminuria y reducción de la tasa de filtración glomerular, sin embargo, para prevenir el daño renal se deben realizar seguimientos clínico, paraclínico e imagenológico cuidadosos de todo paciente con RUF, según la condición lo amerite. Conclusiones: se hace necesaria la unificación de pautas de seguimiento en los pacientes pediátricos con RUF y hacer énfasis en aquellos factores de riesgo que predisponen a compromiso renal.

Background: The Solitary functioning kidney (SFK) is an entity that can be congenital type caused by unilateral renal agenesis or multicystic dysplastic kidney, or acquired secondary to unilateral nephrectomy conditioning to a strict routine follow-up, as well as the implementation of some prevention guidelines in pediatric patients. Purpose: To analyze the importance of follow-up in pediatric patients with a single functioning kidney. Methodology: A review of the literature is made showing embryology, physiology, etiology, clinical aspects and diagnostic studies in patients with SFK in order to subsequently offer guidelines for follow-up and management in pediatric patients. Results: Patients with SFK may present compensatory glomerular hyperfiltration, followed by glomerular injury with hypertension, albuminuria and reduced glomerular filtration rate, however, to prevent renal damage a careful clinical, paraclinical and imaging follow-up of every patient with SFK should be performed as the condition warrants. Conclusions: It is necessary to unify follow-up guidelines in pediatric patients with SFK and to emphasize those risk factors that predispose to renal compromise.

Herlyn-Werner-Wunderlich syndrome: Case report / Síndrome de Herlyn-Werner-Wunderlich. Reporte de caso

Abstract Introduction: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation of the urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis, which is usually diagnosed after menarche. It is treated by resecting the vaginal septum and draining the obstructed hemivagina and has a favorable postoperative prognosis. Case presentation: This is the case of 22-year-old woman with a 9-year history of dysmenorrhea, pelvic pain, and irregular menstrual cycles that started after she had her first menstruation, who visited the gynecology service of a secondary care hospital in Sogamoso, Boyacá (Colombia). The patient reported having visited multiple specialists and being treated with vitamin E, metformin, and contraceptives, without experiencing an improvement of symptoms and signs. After being assessed, and taking into account ultrasound and MRI findings, she was diagnosed with HWW syndrome. She underwent a colpotomy plus resection of right paracervical mass of approximately 60x60mm and vaginal septum, achieving complete resolution of symptoms. Finally, 10 months after the surgery, she reported being pregnant without complications. Conclusion: HWW syndrome is a rare malformation that should be considered as a differential diagnosis in women of any age with paramesonephric duct anomalies, pelvic pain, dysmenorrhea, and presence of masses in the genital tract, since its early diagnosis and timely treatment considerably improve the quality of life of these patients by reducing the severity of symptoms, decreasing the incidence of complications, and improving obstetric prognosis.

Resumen Introducción. El síndrome de Herlyn-Werner-Wunderlich (SHWW) es una malformación congénita rara del tracto urogenital que se caracteriza por la triada útero didelfo, hemivagina obstruida y anomalía renal ipsilateral, y que suele diagnosticarse después de la menarquia. Su tratamiento consiste en resección del tabique vaginal y drenaje de la hemivagina obstruida, con un buen pronóstico postquirúrgico. Presentación del caso. Mujer de 22 años con un cuadro clínico de 9 años de evolución que inició cuando tuvo su primera menstruación, consistente en dismenorrea, dolor pélvico y ciclos menstruales irregulares, quien asistió al servicio de ginecología de un hospital de segundo nivel en Sogamoso, Boyacá (Colombia). La paciente reportó haber visitado múltiples especialistas y recibido tratamiento con vitamina E, metformina y anticonceptivos, sin mejoría de los síntomas y signos. Luego de ser valorada, y teniendo en cuenta los hallazgos en ecografía y resonancia magnética, fue diagnosticada con SHWW, por lo que se le realizó colpotomía más resección de masa paracervical derecha de aproximadamente 60x60mm y de tabique vaginal, lográndose la resolución completa de los síntomas. Finalmente, 10 meses después de la cirugía, la joven refirió encontrarse en estado de embarazo, sin presentar complicaciones. Conclusión. El SHWW es una malformación poco común que debe considerarse como diagnóstico diferencial en mujeres de cualquier edad con anomalías de los conductos paramesonéfricos, dolor pélvico, dismenorrea y masa en el tracto genital, pues su diagnóstico temprano y un tratamiento oportuno mejoran considerablemente la calidad de vida de estas pacientes al reducir la severidad de los síntomas, disminuir la incidencia de complicaciones y mejorar el pronóstico obstétrico.

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.

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

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 Bogotá 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.

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.

Renal length z-score for the detection of dysfunction in children with solitary functioning kidney.

AIM: To evaluate whether renal length z-scores predict renal dysfunction in children with a solitary functioning kidney (SFK). METHODS: In a single-centre retrospective cohort of children with SFK, we correlated body mass index z-scores, extracellular volume and lean body mass to renal length z-scores. We grouped these z-scores to other markers of renal dysfunction (proteinuria, hypertension, extracellular volume and abnormal estimated glomerular function rate [eGFR]) and analysed renal length z-score with multivariate analysis, receiver-operated characteristics (ROC) plots and Youden's index to determine an appropriate cut-off. RESULTS: 111 children had a median follow-up 5.08 years, eGFR 80.8 mL/min/1.73 m2 , and age at last follow-up 7.4 (3.8-13.4 years). The median renal length z-scores of those without any renal dysfunction (n = 37, 25.1%) were greater (+3.66, interquartile range 3.02-4.47) than those with renal dysfunction (median 3.11, interquartile range 1.76-4.11, P = .0107, Mann-Whitney test). Using a cut-off of z-score of >+1.911, the odds ratio for having no renal dysfunction was 0.07 (95% CI 0.002-0.459, P = .0010). However, accuracy of the renal length z-score was poor (ROC curve 0.6488). CONCLUSION: In this cohort of children with SKF, using the renal length z-score as a biomarker of renal dysfunction at 7 years of age is not recommended.

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.

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

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 identifi ed 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 oncolo-gic 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.

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.

Fibrodisplasia osificante progresiva plus por una variante patogénica del gen ACVR1: caso clínico / Fibrodysplasia ossificans progressiva: report of one case

Fibrodysplasia ossificans progressiva (FOP) or myositis ossificans, is a genetic disease, with a prevalence of 1 in 2.000.000. It is caused by pathogenic variants in ACVR1 gene and characterized by soft tissue heterotopic ossification, starting in the second decade of life. It is associated to early mortality caused by respiratory complications. It evolves in flare-ups, triggered by soft tissue injuries; therapy is symptomatic, using analgesia, steroids and diphosphonates. We report a 12-year-old female with left renal agenesis, hallux valgus and intellectual disability, presenting with a six months history of thoracic kyphosis, tender nodules in the thorax, and rigidity of right elbow and left knee. Clinical examination revealed dysmorphic facial features. A magnetic resonance showed heterotopic ossification nodules, which was confirmed with spinal radiography. These findings prompted the diagnosis of FOP. Pain treatment was started, and prednisone was used during flare-ups. The ACVR1 gene was analyzed and a pathogenic variant, p. Arg206His, was found, confirming the diagnosis of FOP.

A clinical predictive model of renal injury in children with congenital solitary functioning kidney.

BACKGROUND: Solitary functioning kidney (SFK) is an important condition in the spectrum of congenital anomalies of the kidney and urinary tract. The aim of this study was to describe the risk factors for renal injury in a cohort of patients with congenital SFK. METHODS: In this retrospective cohort study, 162 patients with SFK were systematically followed up (median, 8.5 years). The primary endpoint was time until the occurrence of a composite event of renal injury, which includes proteinuria, hypertension, and chronic kidney disease (CKD). A predictive model was developed using Cox proportional hazards model and evaluated by c statistics. RESULTS: Among 162 children with SFK included in the analysis, 132 (81.5%) presented multicystic dysplastic kidney, 20 (12.3%) renal hypodysplasia, and 10 (6.2%) unilateral renal agenesis. Of 162 patients included in the analysis, 10 (6.2%) presented persistent proteinuria, 11 (6.8%) had hypertension, 9 (5.6%) developed CKD stage ≥ 3, and 18 (11%) developed the composite outcome. After adjustment by the Cox model, three variables remained as independent predictors of the composite event: creatinine (HR, 3.93; P < 0.001), recurrent urinary tract infection (UTI) (HR, 5.05; P = 0.002), and contralateral renal length at admission (HR, 0.974; P = 0.002). The probability of the composite event at 10 years of age was estimated as 3%, 11%, and 56% for patients assigned to the low-risk, medium-risk, and high-risk groups, respectively (P < 0.001). CONCLUSION: Our findings have shown an overall low risk of renal injury for most of infants with congenital SFK. Nevertheless, our prediction model enabled the identification of a subgroup of patients with an increased risk of renal injury over time.

Functional and oncologic outcomes after nephron-sparing surgery in a solitary kidney: 10 years of experience

ABSTRACT Objectives: To evaluate functional and oncologic outcomes of partial nephrectomy (PN) in patients with a solitary kidney. Materials and Methods: A retrospective analysis of patients with a solitary kidney undergoing nephron-sparing surgery between March 2003 and March 2013 was performed. GFR was recorded before the procedure and 3 months after surgery, thus establishing a change (cGFR). Several variables that may influence cGFR were analyzed. Complications are herein described, namely bleeding, fistula, acute renal failure and end-stage renal disease (ESRD). Local recurrence and margin status are also described. Survival rates were calculated using the Kaplan Meier method (2 patients with metastasis at the time of surgery were excluded from the analysis). Results: Forty-five patients were available for analysis. Median follow-up was 27.56 months (r 3-96). Mean cGFR was-7.12mL/min (SD 2.1). Variables significantly related with lower GFR after surgery were loss of renal mass (p=0.01)) and male gender (p=0.03). Four patients (8.8%) experienced hemorrhage. Nine patients (20%) developed a urinary fistula. Only one patient with bleeding required open surgery. Two patients (4.4%) needed transient dialysis. Three patients (6.6%) developed ESRD. Four patients (8.8%) had positive surgical margins (PSMs) and four patients (88%) had local recurrence (2 of these had PSMs). Five patients (11.1%) died during follow-up. Four patients (8.8%) died because of renal cancer. Estimated 2-year overall survival, disease-free survival and cancer specific survival rates were 88.4% (CI 95% 70.5-96); 87.7% (CI 95% 68.1-96) and 92.4% (CI 95% 75-98), respectively. Conclusion: Loss of renal mass and male gender were associated with lower postoperative GFR. Our outcomes were comparable with those in the World literature.