Management of severe hypertension in a child with tuberous sclerosis-related major vascular abnormalities.

OBJECTIVE: We report the case of a 4-year-old girl with tuberous sclerosis who presented with severe renovascular hypertension secondary to compression of the renal arteries by a large infradiaphragmatic abdominal aortic aneurysm.
DESIGN: Case report of a previously unreported case.
RESULTS: Normotension could not be achieved despite conservative treatment with three antihypertensive drugs at high doses. An aortic graft was performed successfully but failed to correct the hypertension. Postoperatively, the child experienced acute renal failure after attempting treatment with additional enalapril. Further investigations revealed ischaemia of the left kidney and stenosis of the right renal artery. Subsequently, a combination of maximum dose minoxidil, a calcium antagonist, clonidine and peripheral alpha and beta-blockers, and diuretics was used, but her blood pressure control remained poor. A bypass between the hepatic artery and the right renal artery restenosed at the proximal anastomosis. Her renovascular hypertension was finally improved with an autotransplantation of her right kidney into the right iliac fossa. Fibromuscular dysplasia and abundant collagen deposition were the predominant features seen on histology. She is currently normotensive on moderate doses of a beta-blocker, amlodipine and a diuretic.
CONCLUSION: Hypertension in this case was impossible to control medically, and the surgical approach was compromised by numerous complications. This case illustrates the challenges in the management of renovascular hypertension in a child with disease-associated vascular malformations.