Dysplasia: renal multicystic
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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
- 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