Abstract

Renal adysplasia is rare, occurring in only one per 10,000 births. Ultrasonography at 21 weeks of gestation in 33 years woman showed cystically enlarged left kidney in fetus and absent right kidney in fetus for which the pregnancy was terminated. On fetal autopsy, left kidney showed multiple tiny cysts. Microscopy of the left kidney showed features of dysplasia and confirmed the absence of right kidney and bilateral ureters. The diagnosis of renal adysplasia (multicystic dysplasia of left kidney and agenesis of right kidney) was made. This case is presented for its rarity. DOI: 10.21276/APALM.1441

Highlights

  • Renal adysplasia is defined as unilateral renal agenesis in association with dysplasia of the contralateral kidney

  • We report a case of renal adysplasia with a combination of multicystic dysplasia of left kidney and agenesis of right kidney in fetus diagnosed on autopsy

  • CAKUT includes a wide range of kidney anomalies including aplasia, hypoplasia, adysplasia, multicystic dysplasia and ureteric anomalies such as megaureter, ureteropelvic junction obstruction or incompetence, duplex kidney/ureters, and anomalies of the bladder and urethra.[3,4]

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Summary

Introduction

Renal adysplasia is defined as unilateral renal agenesis in association with dysplasia of the contralateral kidney. The term adysplasia is often used more broadly to include dysplasia, absent kidneys, and almost any other structural or positional kidney/ lower urinary tract defect.[1] We report a case of renal adysplasia with a combination of multicystic dysplasia of left kidney and agenesis of right kidney in fetus diagnosed on autopsy. Ultrasonography at 21weeks of gestation in 33years woman (G3P2L2) showed severe oligohydramnios and cystically enlarged left kidney of the fetus (Fig 1a). Left kidney was enlarged measuring 5.5x3.5x3cms and was diffusely cystic (Fig 1c). Cut surface of left kidney showed numerous cysts measuring 0.2 to 0.5cm present throughout the renal parenchyma (Fig 1d). With the findings of right renal agenesis and left sided multicystic dysplasia, the final diagnosis of renal adysplasia (Potter’s type 2) was made. The couple and their two daughters were screened and had normal genitourinary system on ultrasonography

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