Pediatric renal artery dysplasia: a morphologic study

Area of Science:

• Vascular Pathology
• Pediatric Nephrology
• Histopathology

Background:

• Renal artery stenosis in children can result from dysplasia.
• Previous classifications of renal artery dysplasia included fibroplasia and hyperplasia.

Purpose of the Study:

• To histologically examine renal artery dysplasia in pediatric patients.
• To characterize the types and frequencies of renal artery dysplasia.
• To investigate associations between dysplasia types and clinical presentation.

Main Methods:

• Light microscopy examination of renal arteries from 12 pediatric patients.
• Inclusion of surgical resections and autopsy cases.
• Clinical data review for patient demographics and associated anomalies.

Main Results:

• Medial fibrous dysplasia was the most frequent type (6 cases).
• Perimedial elastic dysplasia was observed in 4 cases, exclusively in bilateral disease.
• Intimal fibrous dysplasia and medial muscular dysplasia were less common.
• Extrarenal vascular involvement by dysplasia was noted in autopsy cases.
• No distinct histological pattern of dysplasia was associated with neurofibromatosis.

Conclusions:

• Renal artery dysplasia in children exhibits diverse histological patterns.
• Medial fibrous dysplasia and perimedial elastic dysplasia are significant types.
• The type of dysplasia may correlate with the laterality of renal artery stenosis.
• Dysplasia can affect extrarenal vessels.
• Neurofibromatosis did not show a specific renal artery dysplasia subtype.