Midaortic syndrome and hypertension in childhood

doi:10.1016/0022-3468(95)90555-3

Journal of Pediatric Surgery

Volume 30, Issue 2, February 1995, Pages 164-172

https://doi.org/10.1016/0022-3468(95)90555-3 Get rights and content

Abstract

Midaortic syndrome is a form of fibromuscular hyperplasia that involves the abdominal aorta including the renal and visceral branches. Symptomatology depends on the degree and location of vessel narrowing. This report updates the authors' experience and details 17 operative cases, the largest reported series. Seventeen of the 45 patients who have been operated on for severe renovascular hypertension have had this syndrome. Ages have ranged from 5 months to 15 years (average, 9.7 years). Signs and symptoms have included various manifestations of malignant hypertension, congestive heart failure, oliguric renal failure, and claudication. Intestinal angina has not been noted despite celiac and/or superior mesenteric arterial involvement. Although balloon angioplasty was performed in two patients, lasting results were not achieved. One primary nephrectomy was performed. The other 16 patients had vascular reconstructions including aortoaortic bypass grafting (n = 12), with bilateral renal artery bypasses (n = 9) or unilateral renal bypass (n = 3) or bilateral renal bypass alone (n = 4). The majority had associated visceral artery narrowing, but excellent collaterals have been present so no visceral reconstructions have been required. All renal artery bypasses have been with reinforced saphenous vein. Results: In the average follow-up period of 48 months, 12 of the 17 patients have been cured of hypertension, and the other five have improved. Claudication, congestive failure, and renal failure have been alleviated. Thus far, reinforcement of the saphenous vein grafts has prevented aneurysmal degeneration and graft loss. Conclusion: These results indicate that aggressive single-stage reconstruction is the best approach for these children.

References (22)

JE Deal et al.
Renovascular disease in childhood
J Pediatr
(1992)
A Senning et al.
Coarctation of the abdominal aorta
J Thorac Cardiovasc Surg
(1960)
HD Berkowitz et al.
Renovascular hypertension in children
J Vasc Surg
(1989)
PW Meacham et al.
Chronic mesenteric ischemia in childhood and adolescence
J Vasc Surg
(1985)
W Schlessinger
Merkwurdirge verschkiessung der aorta
Wenscher ges. Helikund
(1835)
R Quain
Partial contraction of the abdominal aorta
Trans Path Soc London
(1847)
PK Sen et al.
The middle aortic syndrome
Br Heart J
(1963)
LM Graham et al.
Abdominal aortic coarctation and segmental hypoplasia
Surgery
(1979)
EG Harrison et al.
Pathologic classification of renal arterial disease in renovascular hypertension
M Takayasu
A case with unusual changes of the central vessels in the retina
Nippon Ganka Gakkai Zasshi
(1908)

P Lagneau et al.

Surgical treatment of Takayasu's disease

Ann Surg

(1987)

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Middle aortic syndrome (MAS), an uncommon cause of secondary hypertension, is defined by obstructive narrowing of the abdominal aorta and ostia of its major branches like the renal and splanchnic arteries. Most of the cases of MAS are categorized as idiopathic; however, genetic disorders like Williams syndrome, mucopolysaccharidosis, neurofibromatosis type 1 (NF1), and Alagille syndrome, and acquired inflammatory diseases such as Takayasu arteritis and other nonspecific arteritis can also lead to MAS. MAS is commonly seen in children and young adults presenting with severe hypertension, congestive heart failure, renal failure, or severe leg claudication. The diagnosis of MAS on CT, MR, and conventional angiography is fairly straightforward. However, the spectrum of sonographic findings in MAS can be varied. Since ultrasound is frequently utilized as a first-line investigation for secondary causes of hypertension, it is especially crucial to understand the sonographic features of MAS. Here, we report a case of a young female who presented to our hospital with severe hypertension. On the Renal Doppler scan, the only clue of the renovascular etiology of her secondary hypertension was the “tardus-parvus waveform’’ in the intrarenal arteries.
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Middle aortic syndrome presents with segment narrowing of the descending thoracic and abdominal aorta. A common manifestation is uncontrolled hypertension, which can lead to severe aortic regurgitation in the long term. We have presented the case of a 31-year-old woman with worsening heart failure symptoms and longstanding uncontrolled hypertension. Echocardiography revealed severe aortic regurgitation. Aortic computed tomography showed severe stenosis of the aorta at the diaphragm level. Stent graft implantation was performed, followed by Bentall surgery 1 year later. Endovascular stent graft implantation of the descending aorta can be used safely as a bridging surgery for the Bentall procedure to reduce the patient’s blood pressure and relieve heart failure symptoms.
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Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta. Patients with disease refractory to medical management will usually require either endovascular therapy or surgery with use of prosthetic graft material for bypass or patch angioplasty. We report our early experience with a novel approach using a tissue expander (TE) to lengthen the normal native arteries in children with MAS, allowing primary aortic repair without the need for prosthetic graft material.
We conducted a retrospective review of patients with MAS undergoing the TE-stimulated lengthening of arteries (TESLA) procedure at our institution from 2010 to 2014. Data are presented as mean (range).
Five patients aged 4.8 years (3-8 years) underwent the TESLA procedure. Stages of this procedure include the following: stage I, insertion of retroaortic TE; stage II, serial TE injections; and stage III, final repair with excision of aortic stenosis and primary end-to-end aortic anastomosis. Stage II was completed in 4 months (1-9 months) with 12 (7-20) TE injections. Goal lengthening was achieved in all patients. Stage III could not be completed in one patient because of extreme aortic inflammation, which precluded safe excision of the aortic stenosis and required use of a prosthetic bypass graft. The other four patients completed stage III with two (one to three) additional vessels also requiring reconstruction (renal or mesenteric arteries). At 3.2 years (1-6 years) of follow-up, all patients are doing well.
The TESLA procedure allows surgical correction of MAS without the need for prosthetic grafts in young children who are still growing.
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Duplex Ultrasound Findings Before and After Surgery in Children and Adolescents with Renovascular Hypertension
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Citation Excerpt :
Therefore, surgical procedures are often required, with the goal of reconstructing the anatomy. Surgical procedures include renal artery re-implantation, arterial reconstruction with autologous or synthetic grafts, auto-transplantation and nephrectomy (Bleacher et al. 1997; Chalmers et al. 2000; Lillehei and Shamberger 2001; O'Neill et al. 1995; Stadermann et al. 2010; Stanley et al. 2006). The purpose of this article is to describe DUS findings before and after surgery in children and adolescents with renovascular disease.
We report our experience with duplex ultrasound in young patients with renal artery stenosis (RAS) or middle aortic syndrome (MAS) before and after surgery (1995 and 2009). Of 36 patients (mean age: 13 ± 7 y), 21 had RAS and 15 had MAS. For patients with RAS, the V_max in the affected artery was 350 ± 111 cm/s before surgery and 145 ± 55 cm/s after surgery. The resistance index was 0.46 ± 0.1 in the post-stenotic kidney and increased to 0.60 ± 0.08 after revascularization. Determination of the flow profile in the iliac artery revealed triphasic flow. In individuals with MAS, V_max in the aorta was 323 ± 98 and the resistance index in both kidneys was low, even in the absence of RAS. The flow profile in the iliac arteries was monophasic before surgery and became triphasic after surgery. Duplex ultrasound is useful for the evaluation of children and young adults both pre- and post-surgery. Duplex ultrasound criteria for RAS in adults appear to be applicable in children and young adults also. The diagnostic evaluation of suspected renal vascular disease should include assessment of the aorta and the flow profile in the iliac arteries, as this could help differentiate between aortic and isolated renal artery stenosis.
Lower-limb ischemia in the young patient: Management strategies in an endovascular era
2012, Annals of Vascular Surgery
The aim of this paper is to review the potential role of endovascular interventions for young patients with lower-limb ischaemia.
A literature search was performed of PubMed and Medline databases using appropriate search terms and limits. Case reports, retrospective studies, and prospective studies evaluating treatment of lower-limb ischemia in patients aged <50 years were scrutinized. Articles published between 1966 and 2010 were included in this review.
Premature atherosclerosis is likely to represent the commonest cause of lower-limb ischemia in patients aged <50 years, although the incidence of nonatherosclerotic causes such as popliteal entrapment syndrome, cystic adventitial disease, and arteritis in these patients is greater than in older patients. As with older patients, endovascular interventions may be beneficial for patients with symptoms secondary to atherosclerosis. At present, the perceived durability of open surgery may be more preferable to patients aged <50 years, but the development of new endovascular technology is challenging this view. Endovascular interventions such as catheter-directed thrombolysis have specific roles in the treatment of lower-limb ischemia due to nonatherosclerotic causes.
The use of angioplasty and stenting procedures to treat young patients with lower-limb ischemia is increasing and has specific roles. However, many developmental causes and nonatherosclerotic conditions still require primary surgical correction.

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^☆: Presented at the 25th Annual Meeting of the American Pediatric Surgical Association, Tucson, Arizona, May 14–17, 1994.

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Midaortic syndrome and hypertension in childhood☆

Abstract

J Pediatr

J Thorac Cardiovasc Surg

J Vasc Surg

J Vasc Surg

Merkwurdirge verschkiessung der aorta

Wenscher ges. Helikund

Partial contraction of the abdominal aorta

Trans Path Soc London

The middle aortic syndrome

Br Heart J

Abdominal aortic coarctation and segmental hypoplasia

Surgery

Pathologic classification of renal arterial disease in renovascular hypertension

A case with unusual changes of the central vessels in the retina

Nippon Ganka Gakkai Zasshi

Surgical treatment of Takayasu's disease

Ann Surg