Coarctation of the Abdominal Aorta

Onat, Teoman; Zeren, E.

doi:10.1159/000166249

Cited by 55 publications

(28 citation statements)

References 8 publications

(16 reference statements)

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“…The location of the aortic lesion in idiopathic MAS is inter-renal in 19-52% of cases, supra-renal in 11-40%, infra-renal in 19-25% and diffuse in 12% [3,13]. In the 102 patients reviewed here, the renal arteries were involved in 91% of cases, of which 67% were bilaterally affected.…”

Section: Discussionmentioning

confidence: 94%

“…Only ten autopsy cases were reported until the introduction of angiography in the 1940s. In the following 20 years, 116 cases of MAS of different etiologies were published, 91 of which were reviewed in 1969 [13]. Our report describes six cases of idiopathic MAS and an additional 96 reported cases excluding those in the review by Onat and Zeren [13].…”

Section: Discussionmentioning

confidence: 94%

“…Published cases were included in the review when there were at least two cases of idiopathic MAS in a series. A review of 91 cases by Onat and Zeren in 1969 was excluded since it did not distinguish between idiopathic and secondary causes of MAS [13]. Data from the cases in the literature and our cases were combined for analysis.…”

Section: Methodsmentioning

confidence: 99%

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Idiopathic mid-aortic syndrome in children

et al. 2008

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Mid-aortic syndrome (MAS) is an uncommon condition characterized by narrowing of the abdominal aorta and stenosis of its major branches. Our goal was to illustrate the presentation, diagnosis and management of six new cases of idiopathic MAS together with 96 cases of idiopathic MAS from the literature. The mean age of the 102 cases was 14.3 years (19 days to 49 years). Our patient who presented at 19 days of age is the youngest reported to date. Clinical presentations included hypertension (94%), claudication (17%), renal failure (4%) and intestinal ischemia (1%). Angiography was the diagnostic imaging study of choice. Renal arteries were involved in 91% of patients, while the superior mesenteric artery and celiac artery were involved in 35%. Thirteen percent of cases were managed medically, and the remainder was treated surgically. Our experience shows that initial conservative blood pressure management of idiopathic MAS is feasible unless medical control of hypertension is unsatisfactory, renal function is at risk or there are symptoms of claudication or intestinal ischemia. Careful timing and planning of a surgical intervention is possible for most cases and may, in select cases, be considered after completion of puberty to allow growth to be completed.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 94%

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Idiopathic mid-aortic syndrome in children

et al. 2008

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“…Stenosis of the renal arteries is common (60-90%), with less common involvement of the coeliac and superior mesenteric arteries (20-40%), and infrequent involvement of the inferior mesenteric arteries. [8,9,10] The exact cause is unknown in most cases though it is probably due to an insult during intrauterine life. Whilst intimal and subintimal fibrosis and fragmentation of the elastic media are seen, arteritis and atherosclerosis are characteristically absent in this condition.…”

Section: Discussionmentioning

confidence: 99%

Middle Aortic Syndrome (MAS) –A Rare Cause Of Hypertension In A Child with Rheumatic Heart Disease

Ali¹

2017

jmscr

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“…Our two cases presented with neurofibromatosis and systemic arterial hypertension had stenotic lesions of renal arteries evaluated as midaortic syndrome (MAS). Neurocutaneous syndromes are the cause of MAS in 5% cases [12]. The location of lesion in MAS is inter-renal in 19-52% of cases, supra-renal in 11-40%, infrarenal in 19-25% and diffuse in 12% [13].…”

Section: Discussionmentioning

confidence: 99%

Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

Çonkar¹,

Özdemir²,

Bulut³

et al. 2015

Pau Med J

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Neurofibromatosis type 1 (NF1), an autosomal dominant disorder, is characterized by various clinical manifestations as a result of dysplasia of neuroectodermal and mesodermal tissues. Cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas with a variable clinical expression, iris Lisch nodules, and multiple tumors of central and peripheral nervous system, also other systems may be involved. NF1 patients develop hypertension due to renovascular diseases, mid-aortic syndrome, or pheochromocytoma. In pediatric NF1 patients elevated blood pressure is usually due to renal artery stenosis, generally involving the origin or the proximal tract of the vessel, and is associated in 25% of patients with coarctation of the abdominal aorta. Several patients with NF1 and hypertension have been reported in the literature, showing the extreme variability in anatomical lesions. Although NF1 is a rare disease, it is an important cause of renovasculer hypertension in childhood. Abdominal bruit is an alerting physical finding in diagnosis of renal artery stenosis. We here presented our two cases diagnosed with hypertension while being screened for NF1 to emphasize the importance of blood pressure measurement in children with NF1 screening. Also we suggest that hypertensive NF1 patients should be examined in terms of stenosis in the major branch vessels of the aorta and renal arteries. Pam Med J 2015;8(3):259-262Key words: Neurofibromatosis, hypertension, children. ÖzetNörofibromatozis tip 1 otozomal dominant geçiş gösteren nöroektodermal ve mezodermal dokuların displazisi sonucu ortaya çıkan klinik bulgular ile karekterizedir. Cafe-au-lait denilen karekteristik pigmente deri lezyonu, aksiller ve inguinal çillenme, deri nörofibromaları, Lisch nodülleri denilen pigmente iris hamartomaları, merkezi sinir sistemi tümörleri, periferik sinir sistemi tümörleri ve diğer sistemleri ilgilendiren tümörler görülebilir. Nörofibromatoz tip 1 de hipertansiyon renovasküler hastalıklar, mid-aortik sendrom veya feokromasitomaya bağlı olarak gelişebilir. Çocukluk yaşlarında nörofibramatoza bağlı hipertansiyonun nedeni genellikle renal arter stenozu olup genellikle damarın proksimalini veya orijini ilgilendirir. Hastaların %25'inde aort koarktasyonu ile birliktedir. Nörofibromatozis nadir görülen bir hastalık olsada çocukluk çağında ki renovasküler hipertansiyonun önemli nedenlerinden biridir. Nörofibramatoz tip 1 nedeniyle izlenirken hipertansiyon tespit edilen iki olgumuzu NF 1 izleminde kan basıncı ölçümleri ile tarama yapılmasının önemini belirtmek ve aort ana dallarının ve renal arterlerin hipertansiyon tespit edilen olgularda stenoz açısından incelenmesinin önemini vurgulamak için sunduk. 2015;8(3):259-262 Pam Tıp Derg

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Coarctation of the Abdominal Aorta

Cited by 55 publications

References 8 publications

Idiopathic mid-aortic syndrome in children

Idiopathic mid-aortic syndrome in children

Middle Aortic Syndrome (MAS) –A Rare Cause Of Hypertension In A Child with Rheumatic Heart Disease

Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

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