Tumor de Wilms en displasia renal multiquística

RESUMEN Introducción: La displasia renal multiquística es una anomalía congénita caracterizada por un riñón afuncional con quistes de diferentes tamaños. Es considerada como una anomalía del desarrollo, aunque se han descrito casos hereditarios. Se ha puntualizado la degeneración maligna de esta displasia, pero su asociación es muy rara. Objetivo: Describir una paciente con tumor de Wilms asociado a una displasia renal multiquística. Presentación del caso: Paciente de sexo femenino y 10 años de edad que los ultrasonidos prenatales no detectaron anomalías del tracto urinario y a los 7 años un estudio ecográfico detectó quistes en el riñón izquierdo. No cumplió el seguimiento clínico y ultrasonográfico indicado y abandonó la consulta; los 10 años asistió al médico por síntomas respiratorios agudos y al palpar el abdomen se comprobó masa en flanco izquierdo de consistencia leñosa, no dolorosa y con contacto lumbar. Se realizó biopsia renal y se diagnosticó tumor de Wilms, se impuso tratamiento citostático y se realizó nefrectomía que confirmó el diagnóstico. Entre los antecedentes familiares se informa un hermano fallecido antes de las 72 horas de nacido por displasia renal multiquística bilateral y madre con nefropatía por reflujo vesicoureteral. Conclusiones: La paciente que se describe reúne tres características raras: un tumor maligno asociado a una displasia renal multiquística, la posibilidad hereditaria por el antecedente del hermano con la displasia bilateral y un tumor de Wilms a los 10 años de edad, lo que demuestra la importancia del seguimiento en estos pacientes.

ABSTRACT Introduction: Multicystic renal dysplasia is a congenital anomaly characterized by an afunctional kidney with cysts of different sizes. It is considered a developmental abnormality, although hereditary cases have been described. The malignant degeneration of this dysplasia has been pointed out, but its association is very rare. Objective: Describe a patient with Wilms tumor associated with multicystic renal dysplasia. Case Presentation: 10-year-old female patient to whom prenatal ultrasounds did not detect urinary tract abnormalities and at age 7 an ultrasound study detected cysts in the left kidney. She did not comply with the indicated clinical and ultrasonographic follow-up and left the consultation; at 10 years old, she attended the doctor for acute respiratory symptoms and when palpating the abdomen there was a mass on the left flank of woody consistency, not painful and with lumbar contact. A renal biopsy was conducted and Wilms tumor was diagnosed; cytostatic treatment was indicated, and a nephrectomy was performed that confirmed the diagnosis. Among the family history is reported a brother who died before 72 hours of birth due to bilateral multicystic renal dysplasia and a mother with nephropathy by vesicoureteral reflux. Conclusions: The described patient has three rare characteristics: a malignant tumor associated with multicystic renal dysplasia, the hereditary possibility due to the sibling's history of bilateral dysplasia, and a Wilms tumor at 10 years of age, which demonstrates the importance of follow-up in these patients.

Flexible Fiberoptic Bronchoscopy Directed Interventions in Neonatal Intensive Care Unit

Objective: To describe the utility of flexible fiberoptic bronchoscopy for the diagnosis andmanagement in the neonatal ICU. Methods: A retrospective, medical chart review wasconducted in neonates who underwent flexible fiberoptic bronchoscopy over a period of 7years. Besides demographic data and diagnostic findings, the results of medical and/orsurgical interventions done by treating neonatologist were recorded. Results: 88bronchoscopies were performed in 83 neonates, of which 37 were done throughendotracheal tube. Indications included persistent need for mechanical ventilation (32),persistent atelectasis (21), and stridor (27). Most common airway anomalies diagnosedincluded tracheobronchomalacia (20), laryngomalacia (18), subglottic stenosis (7), choanalatresia (4), laryngeal cleft (4), and tracheoesophageal fistula (4). Surgical interventions wereundertaken in 17 cases (9 tracheostomies and 2 cases of slide tracheoplasty). Conclusion:Flexible fiberoptic bronchoscopy can be beneficial for the diagnosis and management ofneonates with persistent or undiagnosed respiratory problems.

Ureterocalycostomy - final resort in the management of secondary pelvi-ureteric junction obstruction: our experience

ABSTRACT: Ureterocalycostomy can be performed in patients in whom desired methods of treating secondary PUJ (Pelvi-Ureteric Junction) obstructions either failed or could not be used. In our study, one child and two adults in whom one redo-ureterocalycostomy and two ureterocalycostomies were performed for severely scarred PUJ. The causes for secondary PUJ obstruction were post-pyelolithotomy in one case, post-pyeloplasty and ureterocalycostomy for PUJ obstruction in the second patient and the third patient had long upper ureteric stricture post-ureteropyeloplasty due to tuberculosis. In all these cases ureterocalycostomy proved to be salvage/final resort for preserving functional renal unit

High-pressure balloon assessment of pelviureteric junction prior to laparoscopic "vascular hitch"

ABSTRACT Aim To assess if calibration of the ureteropelvic junction (UPJ) using a high-pressure balloon inflated at the UPJ level in patients with suspected crossing vessels (CV) could differentiate between intrinsic and extrinsic stenosis prior to laparoscopic vascular hitch (VH). Materials and Methods We reviewed patients with UPJO diagnosed at childhood or adolescence without previous evidence of antenatal or infant hydronephrosis (10 patients). By cystoscopy, a high-pressure balloon is sited at the UPJ and the balloon inflated to 8-12 atm under radiological screening. We considered intrinsic PUJO to be present where a ‘waist’ was observed at the PUJ on inflation of the balloon and a laparoscopic dismembered pyeloplasty is performed When no ‘waist’ is observed we considered this to represent extrinsic stenosis and a laparoscopic VH was performed. Patients with absence of intrinsic PUJ stenosis documented with this method are included for the study. Results Six patients presented pure extrinsic stenosis. The mean age at presentation was 10.8 years. Mean duration of surgery was 99 min and mean hospital stay was 24 hours in all cases. We found no intraoperative or postoperative complications. All children remain symptoms free at a mean follow up of 14 months. Ultrasound and renogram improved in all cases. Conclusion When no ‘waist’ is observed we considered this to represent extrinsic stenosis and a laparoscopic VH was performed. In these patients, laparoscopic transposition of lower pole crossing vessels (‘vascular hitch’) may be a safe and reliable surgical technique.

Displasia renal multiquística, ¿tratamiento conservador o nefrectomía?: is it appropriate to follow the conservative treatment or nephrectomy? / Multicystic renal dysplasia

Introducción: la displasia renal multiquística es una anomalía relativamente frecuente en la que aún se mantienen algunas discrepancias en la conducta médica a seguir. Objetivos: analizar la evolución de los quistes en un grupo de pacientes con diagnóstico prenatal de esta anomalía, en los que se siguió una conducta médica conservadora y expectante. Métodos: durante el período comprendido entre el 1º de enero de 1996 y el 30 de junio de 2011, se recibieron en la consulta de Nefrología del Hospital Pediátrico Universitario "William Soler", 22 recién nacidos o lactantes, en los que, durante el seguimiento de un embarazo normal, se diagnosticó una displasia renal multiquística u otra anomalía, que al estudiarse después del nacimiento, se demostró la displasia. En todos los pacientes se realizó ultrasonido renal al llegar al hospital y se hizo gammagrafía estática (99m tecnecio-DMSA) para precisar función renal del lado afectado y valorar las características del riñón contralateral. En todos los casos se siguió una conducta conservadora y expectante, y en 21 de estos niños se logró el objetivo de este estudio. Resultados: hubo predominio del sexo masculino en 60 %, del riñón izquierdo en 77 % y la forma "típica" de displasia en el 80 %. En 6 pacientes se encontraron otras anomalías asociadas. En 3 fetos se presentó involución prenatal de los quistes, la involución total entre 3 y 72 meses se observó en 9 (50,0 % de los estudiados después del nacimiento), mientras hubo involución parcial en 6 (33,3 %) y no hubo variación en 3 (16,7 %). Conclusiones: el tratamiento conservador debe ser la elección inicial en todo paciente con displasia renal multiquística, ya que la involución total se logra en la mayoría de los casos en un período relativamente corto.

Introduction: multicystic renal dysplasia is a relatively frequent anomaly that still arouses some discrepancies about the medical behaviour to be followed. Objectives: to analyze the evolution of cysts in a group of patients prenatally diagnosed with this anomaly, with whom a conservative and expectant medical behaviour was adopted. Methods: from January 1st, 1996 to June 30th, 2011, the nephrology service of "William Soler" university pediatric hospital assisted 22 newborns and infants, who were diagnosed with multicystic renal dysplasia or other anomaly during the normal pregnancy follow-up, but later dysplasia was confirmed after birth. All these patients underwent renal ultrasound on arrival at hospital, and then static gammagraphy (99m technetium-DMSA) to see the renal function of the affected side and to assess the characteristics of the contralateral kidney. A conservative and expectant behaviour was observed for all the cases; the objective of this study was attained in 21 of these children. Results: males prevailed in 60 % of patients, left kidney was affected in 77 % of cases and the "typical" form of dysplasia was present in 80 % of them. Six infants presented with other related anomalies. There was prenatal regression of cysts in three children, total regression ranging 3 to 72 months in 9 infants (50 % of the infants under study after birth) whereas partial regression occurred in 6 cases (33.3 %) and 3 did not change their condition (16.7 %). Conclusions: the conservative treatment should be the initial choice for every patient with multicystic renal dysplasia since total regression is possible in most of cases in a relatively short period of time.

Displasia renal multiquística segmentaria: primeros casos registrados en Cuba / Segmental multicystic renal dysplasia: first cases registered in Cuba

La displasia renal multiquística segmentaria es un subtipo raro de displasia renal que puede confundirse prenatalmente con una displasia multiquística total, un doble sistema obstruido o una hidronefrosis por estenosis de la unión pieloureteral. Se presentan los casos de dos niñas con esta variedad de displasia. En una de ellas la imagen del ultrasonido maternofetal, e incluso el primer ultrasonido renal, se confundió con una hidronefrosis. En la otra, el ultrasonido de las 22 semanas de gestación detectó quistes renales y el primer ultrasonido renal antes de egresar de la maternidad hizo sospechar esta variedad de displasia. Se propone el control imagenológico expectante, sin realizar exéresis de la masa quística, pero atentos a las posibles complicaciones y con el consentimiento informado de los padres.

Segmental multicystic renal dysplasia is a rare subtype of renal dysplasia that may be mistake with a total multicystic dysplasia, an obstructed double system or a hydronephrosis by stenosis of pyeloureteral junction. Authors present cases of two girls presenting with this variety of dysplasia. In one of them maternal-fetal US image, and even the first renal US, was confounded with a hydronephrosis. In the other case, US of 22 weeks of pregnancy sowed renal cysts, and in the first renal US before admission in unit, raise suspicion of this variety of dysplasia. We propose the expectant imaging control, without performing exeresis of cystic mass, but we must to be attentive to possible complications, and with informed consent of parents.

Multicystic Renal Dysplasia with Ipsilateral Ectopic Ureteral Orifice and Seminal Vesicle Cyst: A case report

Renal dysplasia results from aberrant metanephric histogenesis caused fundamentally by a defect in inducer tissue or responding tissue. Dysplastic kidneys vary tremendously in gross and microscopic appearance but are characterized by abnormal organization and a mixed population of primitive structures, such as fetal or immature cartilage, dysplastic ducts, immature tubules, and undifferentiated mesenchyme. We report a case of unilateral multicystic renal dysplasia associated with an ipsilateral ectopic ureteral orifice entering a seminal vesicle cyst in a 33-year-old man. He was admitted due to primary infertility which had developed three years ago. The his semen analysis revealed oligospermia. No evidence of a family history of renal dysplasia was reported. Microscopic examination showed that the entire kidney was composed of cysts lined by flattened cells, dysplastic ducts and immature tubules surrounded by collars of spindle cells, primitive mesenchyme, and a few aberrantly formed glomeruli.