Fact-sheet: Multicystic dusplastic kidney


Updated on 09/17/2021 at 12:16 PM

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Definition

Association of ureteral atresia with non-functioning or poorly functioning cystic dysplasia of the kidney. 1/4300 live births (relatively common). This malformation results either from: 1) a unilateral developmental anomaly, the most common form, related to failure of union between the ureteral bud and the metanephros during embryologic development. Lethal in utero in bilateral forms due to absent renal function (oligohydramnios+++) Associated urogenital anomalies to look for+++: - contralateral vesicoureteral reflux associated in 30% of cases - pyeloureteral junction (7 to 15%) - ureterovesical junction (6%) - genital malformations, ectopic ureteral insertion Systematic workup at birth - ultrasound of the contralateral kidney Absence of contralateral renal hypertrophy should raise suspicion of an associated condition - retrograde and voiding cystography looking for ipsilateral or contralateral reflux Multicystic dysplasia Schematic representation 2) or a focal anomaly of the upper moiety of a duplicated system differential diagnosis: cystic tumor++ 3) or occurs in a syndromic context: polymalformative syndrome * Meckel syndrome (renal anomaly, encephalocele, and polydactyly) * VACTERL (vertebral anomalies, imperforate anus, cardiac malformations, tracheoesophageal fistula, renal and limb anomalies) 4) associated with severe uropathy: posterior urethral valves and severe pyeloureteral junction syndrome. Pathophysiologic hypothesis: obstructive hyperpressure that would induce a defect in ureteral bud induction and severe dysplastic lesions when occurring early in pregnancy.

Radiography

Non-functioning kidney on IVU

Ultrasound

Multiple cysts of variable size arranged haphazardly
with no communication between them, sometimes with persistence of islands of renal parenchyma,
but rarely a reniform shape
No identifiable renal sinus

Nuclear medicine

Non-functioning kidney on renal scintigraphy