Frequency: 1/4,000 live births. The most common cause of renal cysts in the newborn ! 90 % of cases are detected on antenatal ultrasonography: non communicating anechogenic cysts  of variable size . The pathogenesis is poorly understood.

There are typically 4 types:

-         unilateral anomaly of development

-         focal dysplasia of the upper pyelon in case of duplication

- in the context of a malformative syndrome

• chromosomal anomalies: Turner; trisomies 3Q, 9, 13, 18, 21
• syndromes: VACTERL, Kallmann, Meckel, Di George, Zellweger (see table in cystic renal diseases)

- in the context of a severe obstructive uropathy.

Bilateral form: lethal 

Unilateral form can be

-        associated with a contralateral normal and often hypertophic kidney 

-        associated with an obstruction of the pyeloureteraljunction  on the contralateral kidney or vesicoureteral reflux in the contralateral kidney (30 %)

-        associated to hypoplasia of the contralateral kidney and/or homolateral abnormalities of the genitalia: in these cases, check for a mutation in the TCF2 gene (see  glomerulokystic diseases of the kidney); risk of progressive renal failure and proteinuria.

Evolution: 

-         55-95 %: spontaneous involution  within a period of a few months to several years (median 2.1 years) 

-         13-30 %: persistence

-         0- 2 %: increase of volume

On the contrary to what has long been said, there's no increased risk of hypertension or tumor complications (except in case of  association to a syndrome predisposing to high BP).

Long term evolution: the increase of each nephron glomerular filtration can lead to a progressive depletion of renal functional reserve with microalbuminuria, glomerulosclerosis, hypertension and moderate kidney failure in adulthood.

Anesthetic implications: 

Check blood pressure and renal function; fragile (single kidney) renal function: avoid nephrotoxic agents (NSAIDs ?)  !

References : 

-         Avni F. 
Renal cystic diseases in children : new concepts. 
Pediatr Radiol 2010; 40: 939-46.

-        Brochard K, Decramer S. 
Reins kystiques de l’enfant. 
Néphrologie et Thérapeutique 2010 ; 6 : 272-9.

-        Bachetta J, Liutkus A, Dodat H, Cochat P. 
Dysplasie rénale multikystique : mise au point et information pour les parents lors du diagnostic anténatal. 
Arch Pédiatr 2008 ; 15 :1107-1115.

-        Harambat J, Dobremez E, Llanas B. 
Quoi de neuf dans la dysplasie rénale multikystique? 
Arch Pédiatr 2009 ; 16 : 696-7.

-        Salomon R. 
Syndromes avec anomalies rénales in 
Progrès en Pédiatrie : syndromes dysmorphiques par Lacombe D et Philip N, Doin 2013, p 139-50

Updated: July 2019