Congenital renal arteriovenous malformation presenting as severe hypertension

Matrix Gla protein deficiency causes arteriovenous malformations in mice

“…Indeed, renal AVMs are rare (28) and have only been reported as case reports in patients with HHT (29), possibly because the kidneys are not routinely investigated. However, it has been shown that patients with HHT have an increased prevalence of hematuria (47% versus 12% in control subjects; ref.…”

Vascular Complications in Kidney Transplantation

“…Treatment has been recommended when bleeding persists for more than 72 h, renal function deteriorates markedly, lesion enlarges and there is suspicion of steal phenomenon. Endovascular superselective embolization is the therapeutic procedure of choice, as loss of normal parenchyma is minimal, with success rate of approximately 88% with no significant loss of allograft function (Loffroy, 2008, Tarif, 2002. In the majority of cases, successful embolization can be achieved using coils or microcoils.…”

The Role of Transarterial Embolization in the Management of Hematuria Secondary to Congenital Renal Arteriovenous Malformations

“…Congenital renal AVM is quite rare with an estimated prevalence of less than 0.04%, and AVM are increasingly seen as a consequence of the rise in the use of diagnostic imaging techniques [4,5]. Hematuria is the most common presenting complaint [6]. …”

“…AVM are composed of dysplastic subepithelial vessels (absence of elastic lamina) located in the calyceal or pelvic submucosa, which are usually due to massive hemorrhage, flank pain, hypertension and congestive heart failure [2,3,5,6,7]. …”