Successful stenting for renal artery stenosis in a patient with Alagille syndrome

Abstract

A 12-year-old girl with Alagille syndrome manifested severe hypertension caused by renal artery stenosis in a solitary functioning kidney. Percutaneous transluminal angioplasty (PTA) and stenting was performed, but the hypertension persisted. On the next day, acute renal failure occurred with the administration of angiotensin-converting enzyme inhibitor, and migration of the stent was confirmed by angiography. Thus, a second stent was placed with success. Since then, the hypertension has been controlled with anti-hypertensive medication, and the renal function has recovered to normal range.

Percutaneous Balloon Angioplasty of Renovascular Hypertension in Pediatric Cases

“…Minority of the cases with FMD have more focal stenosis that called as "atypical FMD" or "intimal fibroplasia" (2) like the case in our study (case no 19). Drug treatment, surgical or percutaneous revascularizations are different treatment choices, as mentioned before.…”

“…Since then there have been a few series of reports (5,(9)(10)(11)(15)(16)(17). There are much fewer cases reported with additional stent implantation (6,(18)(19)(20).…”

Treatment of severe renal artery stenosis by percutaneous transluminal renal angioplasty and stent implantation

“…After PTRA failed because of elastic recoil of the stenosis, a stent was implanted without complication. Adequate blood pressure was achieved with only two antihypertensive medications [105] Hirai et al treated a 12-year-old girl with Alagille syndrome and severe hypertension caused by renal artery stenosis with stent implantation [106]. Blood pressure improved immediately, but medication with a calcium channel blocker had to be adjusted several times in the follow-up of 28 months.…”

“…Nevertheless, complications of PTRA and stent implantation can occur. Early complications associated with the intervention are infection, bleeding, thrombosis, aneurysma (as in case 1), arterial dissection or even vascular rupture and finally dislocation of the stent, which is described in the case report of Hirai et al [106]. In this case, implantation of a second stent can be necessary or even a surgical procedure with removal of the stent and operative revascularization.…”

Glomerular basement membrane lipidosis in Alagille syndrome

“…In addition, mutations to the Notch2 receptor have been identified in Alagille patients without JAG1 mutations, and these mutations may be associated with increased renal involvement in this condition [3]. Renal involvement has been reported in 40-70% of Alagille cases, including cystic kidney disease, agenesis or hypogenesis of the kidney, duplicated ureters, renal artery stenosis, tubulointerstitial nephritis, and glomerular changes [1,2,[6][7][8][9][10][11]. Whereas the classic glomerular finding in Alagille syndrome is accumulation of lipid deposits in the mesangium, denoted mesangiolipidosis, glomerular and tubular basement membrane lipid deposition has been described [8,12].…”