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.

Midureteral Hypoplasia at Congenital Midureteral Stricture / 대한비뇨기과학회지

Hydronephrosis is the most common abnormal finding of genitourinary tract detected by fetal ultrasonography. The causes of majority are attributed to ureteropelvic junction(UPJ) obstruction. The remaining are secondary to vesicoureteral reflux, megaureter, or posterior urethral valves. Congenital midureteral stricture is an unusual cause of hydronephrosis. We report a case in a one month old male baby with hydronephrosis(grade V, 20mm in AP diameter). He was diagnosed as a ureteropelvic junction obstruction. A mid ureteral stricture was identified with intraoperative anterograde pyelography. After removal of severely strictured ureter, the dilated proximal end was anastomosed to the spatulated distal ureter by microscope. Pathologic finding was subepithelial fibrosis and segmental inner smooth muscle attenuation. Ureteral stent was removed 2 months after surgery. Degree of hydronephrosis was markedly reduced on the follow up ultrasonography(14mm in AP diameter) 6 months after surgery.

The Ultrasonographic Follow up Results and the Prognosis of Prenatal Hydronephrosis / 대한비뇨기과학회지

The diagnosis of hydronephrosis has been increasing in compared to other organs anomaly since the advent of the prenatal ultrasonography, and it is important to differentiate between physiologic and pathologic hydronephrosis. We retrospectively reviewed the follow up of the postnatal ultrasonography and tried to determine the course of prenatal hydronephrosis. The pyeloplasty was indicated 27.1% of patients, in where the diameter of renal pelvis ranged from 18 to 68 mm with a mean of 41 mm and all patients had severe calyceal dilatation. Normal group in which no obstruction pattern on DTPA renal scan were 55.9% and the diameter of renal pelvis ranged from 6 to 23 mm with a mean of 13 mm. The cases of normal, close observation and indicated pyeloplasty were less than 50% and in all cases of vesicoureteral reflux, revealed urinary tract infection. The study revealed that prenatal ultrasonography alone is inadequate in determining the consistent prognostic factors of prenatal hydronephrosis. However, we determined that Pyeloplasty indicated in cases with severe dilatation of calyx and severe renal pelvis dilation. In cases with urinary tract infection. regardless of calyceal dilation and in cases with severe calyceal dilatation, voiding cystourethrogram should be performed to rule out vesicoureteral reflux. In the cases with mild to moderate dilatation, renal scan indicated to exclude a possible urinary tract obstruction. Minimal calyceal dilation can be regarded as free of urinary tract obstruction.