Shoko Hara1, Kazuhide Shimizu2, Tadashi Nariai2, Mitsuhiro Kishino3, Toshifumi Kudo4, Tomoyuki Umemoto5, Motoki Inaji2, Taketoshi Maehara2. 1. Department of Neurosurgery, Tokyo Medical and Dental University, Tokyo, Japan. Electronic address: shara.nsrg@tmd.ac.jp. 2. Department of Neurosurgery, Tokyo Medical and Dental University, Tokyo, Japan. 3. Department of Diagnostic Radiology, Tokyo Medical and Dental University, Tokyo, Japan. 4. Department of Vascular Surgery, Tokyo Medical and Dental University, Tokyo, Japan. 5. Department of Cardiovascular Medicine, Tokyo Medical and Dental University, Tokyo, Japan.
Abstract
BACKGROUND: The de novo occurrence of renal artery stenosis in renal arteries that were angiographically confirmed to be normal in the past has never been reported before in patients with moyamoya disease. CASE DESCRIPTION: During the long-term follow-up of pediatric patients with moyamoya disease, we observed 3 patients who developed de novo renal artery stenosis in arteries that had been angiographically confirmed to be normal 1 year after the surgery (7 years on average, ranging from 4 to 11 years). All of these patients were neurologically stable after successful indirect bypass surgery during childhood. However, more than 10 years after the surgery (15 years on average, ranging from 14 to 23 years), they developed hypertension and were found to have de novo renal artery stenosis, which was ameliorated by endovascular angioplasty. During the follow-up after angioplasty, 1 patient experienced a recurrence of hypertension and required a second and third angioplasty for restenosis. Another patient died of intracranial hemorrhage 2 years after angioplasty. In the 2 surviving patients, gene analysis of the ring finger protein 213 (RNF213; p.R4810K) point mutation, the susceptibility gene for moyamoya disease in the Asian population, was positive for the heterozygous variant. CONCLUSIONS: De novo renal artery stenosis might develop in initially normal arteries during long-term follow-up, particularly among pediatric patients with moyamoya disease. Considering the extracranial manifestations of moyamoya disease, clinicians should keep in mind that de novo renal artery stenosis could emerge later in their life. Thus, it is crucial to continue to follow these patients for decades, even if the patients are neurologically stable after bypass surgery. Monitoring for blood pressure and the de novo occurrence of renal artery stenosis is important to prevent hypertension-related morbidity and mortality, such as intracranial hemorrhage, in this disease population.
BACKGROUND: The de novo occurrence of renal artery stenosis in renal arteries that were angiographically confirmed to be normal in the past has never been reported before in patients with moyamoya disease. CASE DESCRIPTION: During the long-term follow-up of pediatric patients with moyamoya disease, we observed 3 patients who developed de novo renal artery stenosis in arteries that had been angiographically confirmed to be normal 1 year after the surgery (7 years on average, ranging from 4 to 11 years). All of these patients were neurologically stable after successful indirect bypass surgery during childhood. However, more than 10 years after the surgery (15 years on average, ranging from 14 to 23 years), they developed hypertension and were found to have de novo renal artery stenosis, which was ameliorated by endovascular angioplasty. During the follow-up after angioplasty, 1 patient experienced a recurrence of hypertension and required a second and third angioplasty for restenosis. Another patient died of intracranial hemorrhage 2 years after angioplasty. In the 2 surviving patients, gene analysis of the ring finger protein 213 (RNF213; p.R4810K) point mutation, the susceptibility gene for moyamoya disease in the Asian population, was positive for the heterozygous variant. CONCLUSIONS: De novo renal artery stenosis might develop in initially normal arteries during long-term follow-up, particularly among pediatric patients with moyamoya disease. Considering the extracranial manifestations of moyamoya disease, clinicians should keep in mind that de novo renal artery stenosis could emerge later in their life. Thus, it is crucial to continue to follow these patients for decades, even if the patients are neurologically stable after bypass surgery. Monitoring for blood pressure and the de novo occurrence of renal artery stenosis is important to prevent hypertension-related morbidity and mortality, such as intracranial hemorrhage, in this disease population.
Authors: Amélie Pinard; Maximillian D J Fiander; Alana C Cecchi; Andrea L Rideout; Mohamed Azouz; Stuart M Fraser; P Daniel McNeely; Simon Walling; Sarah C Novara; Anna C E Hurst; Dongchuan Guo; Sandhya Parkash; Michael J Bamshad; Deborah A Nickerson; Anthony M Vandersteen; Dianna M Milewicz Journal: Neurology Date: 2021-02-10 Impact factor: 9.910