Presentation
Known Alagille syndrome with left sided vesicoureteric reflux and hydronephrosis. General check-up with abdominal ultrasound.
Patient Data
Right-sided renal dysplasia (image 1), identified as a general increased echogenicity of the kidney parenchyma. No hydronephrosis on the right.
Left-sided moderate hydronephrosis due to known longstanding vesicoureteric reflux (image 2). Unlike the right kidney, the left renal parenchyma does not have an increased parenchymal echogenicity.
Case Discussion
Alagille syndrome is an autosomal dominant condition. The manifestations include cholestatic liver disease, cardiac disease, craniofacial and skeletal abnormalities and renal abnormalities.
Around 40% of cases have renal involvement. Renal manifestations visible with ultrasound include renal dysplasia (focal or generalized increased echogenicity), hydronephrosis (caused by vesicoureteric reflux or ureteropelvic junction obstruction), renal artery stenosis and duplex collecting system.
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