obstructive cystic renal dysplasia

Obstructive cystic renal dysplasia, or Potter type IV cystic renal disease, is a potential complication that can occur from prolonged obstruction of the bladder outlet or urethra during gestation.

Pathology

Ureteric obstruction during active nephrogenesis results in cystic renal dysplasia; the earlier and longer the obstruction the more severe the histopathological changes of dysplasia .

Causes 

Any obstructive renal tract pathology distally involving the renal tract can be a potential cause which include:

• posterior urethral valves
• urethral agenesis
• Duplex collecting system with obstructing ureterocoele
• congenital vesicoureteric junction obstruction

Associations

Associated anomalies may be present in up to 50% of cases can include:

• VACTERL association
• congenital heart disease
• CNS abnormalities
• congenital gastrointestinal malformations

Radiographic features 

Ultrasound

The kidneys are usually normal to small in size with highly echogenic cortices, loss of cortico-medullary differentiation, and scattered cysts (usually smaller than those seen with multicystic dysplastic kidneys) . The reniform shape is often preserved until late in the disease.

Differential diagnosis

General differential considerations include:

• multicystic dysplastic kidneys: no distal obstruction
• Meckel-Gruber syndrome: has occipital encephalocoele and polydactyly

See also

• fetal cystic renal disease

Siehe auch:

• Polydaktylie
• Meckel-Syndrom
• congenital cystic renal disease
• multicystic dysplastic kidneys

und weiter:

• angeborene renale Anomalien
• fetal cystic renal disease