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International Journal of Surgical Pathology
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Medullary Sponge Kidney Associated With Multivessel Fibromuscular Dysplasia: Report of a Case With Renovascular Hypertension

Michele Bisceglia, MD

Department of Pathology, IRCCS "Casa Sollievo della Sofferenza" Hospital, San Giovanni Rotondo, Italy, bismi{at}libero.it

Carlos Galliani, MD

Cook Children's Hospital, Department of Pathology, Fort Worth, Texas

Medullary sponge kidney (MSK) is a congenital anomaly characterized by dilatation of the collecting ducts of Bellini associated with defective urinary acidification and concentration. Medullary nephrocalcinosis/ nephrolithiasis is the usual presentation in adults, however neonatal and childhood cases are being reported with increasing frequency. Among the conditions associated with MSK are Beckwith-Wiedemann syndrome/hemihyperplasia (13%), horseshoe kidney, congenital small kidney, hyperparathyroidism, Caroli syndrome, congenital hepatic fibrosis, Ehlers-Danlos syndrome, Marfan syndrome, immotile cilia syndrome, and arterial fibromuscular dysplasia. This article describes an adult female who underwent nephrectomy for renovascular hypertension due to multivessel fibromuscular dysplasia with small left kidney found to be associated with MSK.

Key Words: medullary sponge kidney • Lenarduzzi— Cacchi—Ricci disease • papillary duct ectasia • cystic disease of renal pyramids • renovascular hypertension • arterial fibromuscular dysplasia • small kidney

International Journal of Surgical Pathology, Vol. 16, No. 1, 85-90 (2008)
DOI: 10.1177/1066896907307237
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