Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome

Kaneko, Kazunari; Someya, Tomonosuke; Ohtaki, Risako; Yamashiro, Yuichiro; Yamataka, Atsuyuki; Iizuka, Y.; Fukumura, Yuki; Suda, K

doi:10.1007/s00431-003-1312-x

Cited by 42 publications

(18 citation statements)

References 18 publications

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“…Riela and Roach [10 ]mentioned in their review that moyamoya syndrome could be associated with coarctation of the aorta, although the site of the lesion was not discussed. We could find no reports of moyamoya disease associated with midaortic syndrome, except for the case of an infant with congenital fibromuscular dysplasia manifesting moyamoya syndrome and stenosis of the abdominal aorta and renal arteries [11]. Although the term ‘midaortic syndrome’ was not used, the clinical symptoms of their case including renovascular hypertension, and angiographic findings may indicate midaortic syndrome because midaortic syndrome has been depicted as a stenotic vascular disorder involving the abdominal aorta and its branches, frequently demonstrating renovascular hypertension [2].…”

Section: Discussionmentioning

confidence: 99%

Moyamoya Disease Associated with Midaortic Syndrome

Korematsu¹,

Yoshioka²,

Maruyama³

et al. 2006

Pediatr Neurosurg

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We report a 1-year-old girl who presented moyamoya disease associated with midaortic syndrome. She had been treated for cardiac failure and severe hypertension due to midaortic syndrome until she suffered seizure and repeated cerebral ischemic attack. Cerebral angiography revealed stenosis of the bilateral internal carotid artery at its terminal portion. She was successfully treated with encephaloduroarteriosynangiosis, and ischemic attack ceased postoperatively. This is the first report of moyamoya disease with midaortic syndrome. Although cerebral ischemic attack has been effectively managed by encephaloduroarteriosynangiosis, renovascular hypertension is still difficult to control.

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Section: Discussionmentioning

confidence: 99%

Moyamoya Disease Associated with Midaortic Syndrome

Korematsu¹,

Yoshioka²,

Maruyama³

et al. 2006

Pediatr Neurosurg

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“…She developed progressive left ventricular hypertrophy together with complaints of fatigue, abdominal pain and headache. In addition, we were afraid of cerebrovascular complications, in specific intracranial hemorrhages, which are reported in several cases of FMD and renovascular hypertension (9,10). To get a better control over her blood pressure, we decided to perform a nephrectomy of the most affected (right) kidney when she was 5.5 years old.…”

Section: Case Reportmentioning

confidence: 99%

Renal Transplantation for Fibromuscular Dysplasia

Knops¹,

Cornelissen²,

Monnens

2011

American Journal of Transplantation

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This is the first report that presents renal transplantation after bilateral nephrectomy as the final treatment for severe renovascular hypertension due to fibromuscular dysplasia (FMD). We describe the history of a 1-year-old girl who suffered from renovascular hypertension due to FMD. Imaging revealed multiple bilateral stenoses of the renal artery extending into the distal branches. The hypertension proved unresponsive to pharmacologic treatment and the intrarenal peripherally located stenoses rendered a conventional approach such as transluminal or surgical angioplasty not feasible. At the age of 5 years, a unilateral nephrectomy of the most affected kidney was performed, but she remained hypertensive and developed progressive cardiomyopathy and retinopathy. At the age of 6 years the remaining kidney was removed, followed by a living related renal transplantation with a kidney donated by her mother. Posttransplantation, she developed mild hypertension due to a postanastomotic stenosis, which was easily controlled with antihypertensives. Now 8 years after transplantation, she has experienced no further blood pressure related problems. Although there is a risk of recurrence of FMD after performing a living related transplantation, our report suggests that this procedure is relatively safe, provided appropriate preoperative evaluation and follow up is performed.

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“…Others have proposed a dystrophic theory according to which myocyte death could be due to some genetic defects [8]. To support this theory some altered genes have been described [9][10][11][12][13][14][15][16][17][18][19]. Another possible explanation for ARVD pathobiology is ischemia.…”

Section: Discussionmentioning

confidence: 99%

“…Coronary fibromuscular dysplasia has been considered as rare. A number of reports have yet documented small coronary artery FMD in patients with sudden cardiac death [16][17][18][19][20]. Furthermore, left main stem coronary artery FMD induces ischemic left ventricular dysfunction [21].…”

Section: Two Unusual Cases Of Sudden Death In Children 71mentioning

confidence: 99%

Two Unusual Cases of Sudden Death in Children

Osterheld

Perez²

2013

OJPathology

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We report two unusual cases of sudden unexpected death in children. Histopathologic examination showed intimal fibroplasias, a variant of fibromuscular dysplasia of the right coronary artery associated in both cases with fatty infiltration of the right ventricular myocardium. The significance of this particular combination of two lesions known to induce a sudden death in young people is discussed.

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Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome

Abstract: Our case suggests that the nature of fibromuscular dysplasia is congenital in origin and its aetiology, at least in some cases, is a systemic abnormality of vascular development.

Cited by 42 publications

References 18 publications

Moyamoya Disease Associated with Midaortic Syndrome

Moyamoya Disease Associated with Midaortic Syndrome

Renal Transplantation for Fibromuscular Dysplasia

Two Unusual Cases of Sudden Death in Children

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