A Rare Cause of Ureteropelvic Junction Obstruction

No abstract available.

Anomalías nefro-urologicas en el riñón contralateral de pacientes con displasia renal multiquística unilateral / Urological and nephrological abnormalities in the contralateral kidney in patients with unilateral multicystic dysplastic kidney

La displasia renal multiquística unilateral (DRMU) es la enfermedad quística renal más frecuente en pediatría. En estos pacientes, la función renal depende exclusivamente del riñón único funcionante contralateral. El objetivo de este trabajo retrospectivo fue determinar la prevalencia y tipo de anomalías nefro-urológicas en el riñón contralateral funcionante de 103 pacientes con DRMU atendidos en el Hospital Nacional de Pediatría Prof. Dr. Juan P. Garrahan entre 1995-2008. Se analizaron sólo aquellos niños que tenían ecografía renal y vesical, cistouretrografía miccional (CUGM) y centellograma renal con ácido dimercaptosuccínico (DMSA-Tc99m). Por ecografía se encontraron anomalías en el riñón contralateral funcionante en el 18.5%. Las más frecuentes fueron dilatación de la vía urinaria (6.8%) y aumento de la ecogenicidad (6.8%). La CUGM detectó la presencia de reflujo vesicoureteral en el riñón contralateral en 11 niños (10.7%), la mayoría leve a moderado. El centellograma renal con DMSA-Tc99m demostró escaras en 12 niños (11.6%). Los resultados muestran que el 23.3% de los pacientes con DRMU tienen alguna anomalía nefro-urológica en el riñón único funcionante contralateral.

Diagnóstico prenatal de la displasia renal multiquística (DRMQ) o potter tipo II: presentación de dos casos / Prenatal diagnosis of multicyst renal dysplasia (MCRD) or potter type II: presentation of two cases

La displasia renal multiquística (DRMQ) también conocida como riñón multiquístico o Potter tipo II,es una anomalía del desarrollo embrionario en la cual el parénquima renal está sustituido por tejidorenal no funcionante constituido por quistes que no se comunican entre sí. Es la segunda causa más común de masa abdominal en el neonato luego de la hidronefrosis. Su incidenciaes de 1 de cada 4300 nacidos vivos y es más frecuente en hombres que en mujeres (2,4:1) siendo en los primeros más frecuente su presentación unilateral, mientras que en mujeres se ve principalmente la forma bilateral(3). Es rara su forma bilateral, la cual es incompatible con la vida. La localización más común es del lado izquierdo.La DRMQ forma parte de las anomalíasdisplásicas de presentación esporádica, aunque no se ha podido descartar la posible implicación de factores genéticos en su origen existiendo en la literatura trabajos que suponen una herencia autosómica dominante e incluso se ha visto asociaciones con diferentes síndromes como Dandy Walker, Meckel, Saldino-Noonan, Apert, Jeune y Zellweger, como así también condefectos cromosómicos tipo trisomías odeleciones cromosómicas. El riesgo derecurrencia estimado oscila entre un 3-5%. Debido a que las principales malformaciones renales y del tracto urinario son de naturalezaobstructiva o se presentan como lesionesquísticas, la evaluación ultrasonográfica prenatal es un método no sólo sensible sino también específico en la detección precoz de las patologías que lo afectan. Su diagnóstico temprano permite predecir la probable evoluciónde la gestación y sus posibles complicaciones así como también su manejo perinatal. Presentamos el caso de dos pacientes con DRMQ.

Introduction: The Multicystic Renal Dysplasia (MCRD), also known as multicystic kidney or Potter type II, is anabnormality of the embryonic development in which the renal parenchyma is substituted by notfunctioning renal tissue made up of cysts which do not communicate with each other (7-8). It is thesecond most common cause of abdominal mass in neonates after the hydronephrosis(2). Itsincidence is 1 of every 4300 born alive and is more frequent in men than in women (2,4:1); in the first ones, being more frequent its unilateral presentation, while in women is mainly observedthe bilateral presentation (3). Its bilateral form is rare and incompatible with life. It is most commonly located on the left side. Although MCRD belongs to sporadic-presentation dysplasic abnormalities, the possible implicationof genetic factors in its origin (8) has not been ruled out there being literature works that imply autosomal dominant inheritance and there haveeven been associations to different syndromes such as Dandy Walker, Meckel, Saldino-Noonan, Apert, Jeune and Zellweger, as well as to chromosomal defects of the type of trisomies or chromosomal deletions. The estimated risk of occurrence ranges between 3% and 5%.(9) Since main renal and urinary tract malformationshave an obstructive nature or present as cystic lesions, prenatal ultrasonographic assessment is not only a sensible method, but also a specific one in the early detection of the pathologies thataffect it. Its early diagnosis allows for predicting the probable evolution of pregnancy and its possible complications, as well as its perinatal management.We present two cases of patients with MCRD.

Multicystic dysplastic kidney - A need for conservative and long term approach.

Objective. We retrospectively studied our cases of Multicystic Dysplastic Kidney (MCDK). The review was aimed at identifying the pattern of the disease in Indian Scenario and the required management thereof. Methods. We studied the clinical, radiological and nuclear scan findings of 22 patients with unilateral MCDK. They were diagnosed and/or treated in our unit from 1999 to 2007. The diagnosis was achieved by Ultrasound and further confirmed by DMSA scans. Other ancillary investigations like Micturating cystourethrogram were done if indicated. These patients were followed and followup investigations consisted of renal ultrasound, blood pressure measurement, and urinalysis and blood biochemistry. Results. A total of 22 patients (18 boys and 4 girls) with unilateral MCDK were investigated and followed for a mean period of 41 months. MCDK was detected on antenatal ultrasound only in 12(55%) and postnatally in 10(45%) babies. Mean age for postnatal diagnosis was 20 months. Follow up ultrasound revealed complete involution of MCDK in 3 patients and partial regression in 11 patients. The size of dysplastic kidney was unchanged in 4 patients and a further 4 patients underwent nephrectomy. Indications of nephrectomy were parental anxiety in 2, hypertension in 1 and palpable mass in 1. Conclusion. Large proportion (45%) of patients in presented series are diagnosed post natally contrary to western world where more than 80% are diagnosed antenatally. Uncomplicated isolated MCDK carry good prognosis with nephrectomy required in only a few patients. Association with other urological anomalies in ipsilateral/contralateral genitourinary tract is important to identify as they have worse outcome in terms of ultimate renal function. All patients with simple/complex unilateral MCDK should be advised long term follow up for the possible development of hypertension and/or hyper infiltration injury.

Rim displático multicístico: atualização / Multicystic dysplatic kidney: update

Rim displásico multicistico (RDM) é a anomalia renal cística mais freqüente na infância. Tem sido detectado por meio de ultra-sonografia pré-natal com a melhoria dos equipamentos ecográficos e com a observação detalhada da anatomia fetal. Nos últimos anos, a abordagem dessa anomalia tem mudado de cirúrgica para conduta mais conservadora. Neste estudo foram revisados os principais aspectos concementes ao RDM, com ênfase no tratamento conservador e suas possíveis complicações. Independentemente do tipo de conduta adotada, se manejo conservador ou cirúrgico, é importante ressaltar que os pacientes devem ser acompanhados com ultra-sonografia e medidas de pressão arterial por tempo prolongado, tendo em vista o pouco conhecimento acumulado, até o presente momento, acerca da evolução natural do rim multicístico.

Multicystic dysplastic kidney (MCDK) is the most common cystic anomaly diagnosed in childhood. With the improvement of the resolution of ultrasonography and detailed fetal scanning, MCKD has had its antenatal detection increased. In the last decade, the management has changed from surgical to a conservative approach. In this study, the main aspects concerning MCDK were described, emphasizing conservative management and its possible complications. Independently of the chosen management, whether conservative or surgical, it is important to point out that the patients should be followed with serial ultrasonography and blood pressure measures for a prolonged time due the incomplete knowledge of the clinical course of MCKD.