Open Repair for Abdominal Aortic Aneurysm in a Young Boy with Tuberous Sclerosis and Review of the Literature

Dueppers, Philip; Duran, Mansur; Grabitz, K.; Schelzig, Hubert

doi:10.1016/j.avsg.2016.06.025

Cited by 14 publications

(20 citation statements)

References 15 publications

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“…The association of these symptoms with aneurysmal aortic injury is rarely observed, but it should always be considered for differential diagnosis. 4,10 Whatever the cause and location, aortic aneurysms are life-threatening due to the risk of rupture. [1][2][3][4] In our patient's case, systemic arterial hypertension, which had been diagnosed 2 years prior, was the associated cardiovascular risk factor.…”

Section: Discussionmentioning

confidence: 99%

“…15 Three treatment options are available for AAA patients: conventional open surgery, minimally invasive endovascular repair, or optimized medical treatment based on active surveillance and control of risk factors. [3][4][5][6][7][8][9][10] The best option depends on such factors as the patient's clinical condition, the anatomical characteristics of AAA, and the presence of symptoms or signs of complications. 12,13,16 In our patient, drug treatment was started based on a non-selective beta-blocker and statin, in addition to the usual symptomatic medications.…”

Section: Discussionmentioning

confidence: 99%

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Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

Reis¹,

Melo²,

Oliveira³

et al. 2021

International Journal of Cardiovascular Sciences

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Takayasu's arteritis is a type of primary systemic vasculitis that affects medium and large arteries, including the aorta and its main branches, as well as the pulmonary and coronary arteries. Although rare in children, it is the third most common vasculitis in the pediatric population, often with delayed diagnosis due to the nonspecific presentation of clinical symptoms in its initial phase. This is a case of a 16-year-old girl with a giant ruptured abdominal aortic aneurysm, who needed surgery on an emergency basis. The etiological aspects involved in aneurysms in young patients are also addressed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

Reis¹,

Melo²,

Oliveira³

et al. 2021

International Journal of Cardiovascular Sciences

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“…In children, it is recommended the largest possible graft to allow proper vascular growth. Oversizing graft is one of the most important techniques [4][5][6][7][8]. Anticoagulation or anti-platelet therapy is difficult to conclude if it is necessary to use it, due to limited experience reported [3].…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Abdominal Aortic Aneurysm in a 3-Year-Old Girl with Tuberous Sclerosis Complex

Alain¹,

Hamzeh²,

A³

et al. 2020

SCR

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The tuberous sclerosis complex is a multisystem disorder affecting children and adults. The association between abdominal aortic aneurysm and tuberous sclerosis is rare. We present 3-year-old girl with a pulsatile abdominal mass in the pediatrician´s medical review. An abdominal evaluation showed a 6cm infrarenal abdominal aortic aneurysm. The patient underwent open repair with a 14-mm Dacron tube graft. The histological examination revealed connective tissue proliferation, fibrosis, and calcification of the aortic wall. Frequent follow-ups with ultrasounds showed no alterations. Due to the high risk of rupture, early surgical repair is the first-line therapy.

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“…Altogether, there are fewer than 40 cases of children with TSC and aortic aneurysms reported in the literature, most commonly described in the infrarenal aorta, and some presenting with rupture and a fatal outcome. 9 , 10 , 11 , 12 , 13 Guided by these experiences, it is recommended to search for, and exclude, aortic and large artery aneurysms in children diagnosed with TSC and, when present, to manage with close surveillance or surgery. 9 In the majority of the reports, open repair has been applied using either Dacron, polytetrafluoroethylene, or biological conduits; an endovascular procedure for a saccular aneurysm in the descending thoracic aorta of a young adult has also been described.…”

Section: Aortic Aneurysms In Tscmentioning

confidence: 99%

“… 14 Because recurrent, anastomotic aneurysms and new ones have been encountered in children with TSC, continued surveillance after repair is recommended. 10 …”

Section: Aortic Aneurysms In Tscmentioning

confidence: 99%

Resolving the biological paradox of aneurysm formation in children with tuberous sclerosis complex

Hedin

Brunnström

Dahlin

et al. 2021

JVS-Vascular Science

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Aortic aneurysms are rare manifestations in children with tuberous sclerosis complex (TSC) with life threating implications. Although an association between TSC, aortic and other aneurysms has been recognized, mechanistic insights explaining the pathophysiology behind aneurysm development and genetic aberrations in TSC have so far been lacking. Here, we summarize existing knowledge on aneurysms in TSC and present a case of a 2-year-old boy with an infrarenal aortic aneurysm, successfully treated with open aortic reconstruction. Histologic examination of the excised aneurysm wall showed distortion of vessel wall structure with loss of elastin and a pathologic accumulation of smooth muscle cells. Until now, these pathologic features have puzzled researchers as proliferating smooth muscle cells would rather be expected to preserve vessel wall integrity. Recent reports exploring the biological consequences of the dysregulated intracellular signaling pathways in patients with TSC provide plausible explanations to this paradox, which may support the development of future therapeutic strategies.

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Open Repair for Abdominal Aortic Aneurysm in a Young Boy with Tuberous Sclerosis and Review of the Literature

Cited by 14 publications

References 15 publications

Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

Surgical Treatment of Abdominal Aortic Aneurysm in a 3-Year-Old Girl with Tuberous Sclerosis Complex

Resolving the biological paradox of aneurysm formation in children with tuberous sclerosis complex

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