Multicystic dysplasia in the upper component of the complete duplex kidney

Abstract

Four cases of neonatal multicystic dysplasia involving the upper part of a complete duplex kidney are presented. This malformation, suspected on antenatal ultrasound examination, was confirmed by postnatal IVP and ultrasound. This emphasizes the need to perform an IVP when the diagnosis of multicystic kidney is suggested by ultrasound. Conservative surgery may then be achieved, as in three of the four cases.

Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome

“…In the 29 previously reported cases of segmental MCDK, 16 were first detected because of an abnormal prenatal ultrasound, with a definitive diagnosis made antenatally or postnatally. As demonstrated in our first 3 cases, prenatal ultrasound may show cystic kidney disease, duplex collecting system, ureterocele, renal mass, or hydronephrosis [2,4,10,12].…”

“…Voiding cystourethrography is indicated for evaluation of VUR, especially in children with duplex collecting system. The DMSA renal scintigraphy can be used to evaluate renal function both in areas involved by segmental MCDK as well as in functioning portions of the kidney that may be obscured by a large cystic dysplastic portion [2,5,12] and which may therefore be overlooked by sonographic evaluation. This would be particularly important if nephrectomy were contemplated.…”

“…Subsequent authors have used the terms segmental or focal MCDK to describe this finding [4]. With only 28 pediatric cases [2,[4][5][6][7][8][9][10][11][12][13][14][15] and 1 adult case [16] reported, there is limited understanding of the presentation and natural history of segmental MCDK. The purpose of this study was to report our experience with the diagnosis of segmental MCDK, with particular emphasis on the initial manifestations, associated anomalies, imaging, management, and follow-up.…”

Bilateral multicystic kidneys ? an unusual case

“…Segmental/partial multicystic dysplasia in a duplex with a contralateral MCDK has only previously been described in one child, but without further elaboration [13]. As an isolated finding, segmental MCDK appears to occur more commonly in females, almost invariably affecting the upper moiety and from a small series, are reported to occur in 3 of 4 times on the left side [14,15,16].…”

Conservative Management of Segmental Multicystic Dysplastic Kidney in Children

“…However, we still have a limited understanding of segmental MCDK due to the limited number of studies and reports on this disease entity. 2,3,[7][8][9][10][11][12][13][14] Review of previous studies showed M A N U S C R I P T A C C E P T E D ACCEPTED MANUSCRIPT 2 that only 39 cases have been reported worldwide, and most were treated by routine surgical resection. Herein, we focused on conservative management of this relatively rare disease by investigation of involution, associated anomalies requiring surgery, and complications of segmental MCDK.…”