Aneurisma de aorta torácica em paciente com esclerose tuberosa

Geiger, Martin Andreas; Cantador, Alex Aparecido; Guillaumon, Ana Terezinha

doi:10.1590/1677-5449.160017

Cited by 6 publications

(3 citation statements)

References 7 publications

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“…While it is possible that this patient's thrombus developed secondary to a prothrombotic state as a result of an unidentified malignancy such as a pancreatic neuroendocrine tumor, 2 3 4 it remains unlikely and unsubstantiated in her presentation. Previous reports have identified rare patients with TSC who developed vascular abnormalities such as aortic aneurysm or rupture, 5 6 which could present a possible source for initial thrombotic growth in this patient, however, there was no structural deformity of the aorta identified in any imaging modality utilized throughout her hospital course.…”

Section: Discussionmentioning

confidence: 67%

Aortic Thrombus Embolization in A Patient with Tuberous Sclerosis

Wolf

Rhoads

Gomes

et al. 2021

Journal of Pediatric Neurology

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Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder affecting many organ systems. Patients commonly develop a variety of benign tumors as well as neurological disease, including seizures, autism, and cognitive delay. We report here the case of an adolescent patient with TSC and a history of mild COVID-19 who presented with a 1-day history of altered mental status. The patient was found to have ischemic cerebral infarction of the right MCA and ACA territories. Initial angiography showed an occlusion of the right internal carotid artery without a demonstrable etiology, with follow-up echocardiography and angiography revealing a large aortic thrombus. The patient was not a candidate for thrombus removal due to her cerebral infarct and received medical anticoagulation. Thrombosis progressed to involve the left ICA, with left cerebral infarction and subsequent death. Aortic thrombus embolization as a cause of cerebrovascular accident (CVA) is a novel finding in the setting of TSC and should be considered for pediatric patients with CVA of unknown etiology. It is unclear whether this was related to the prior COVID-19 infection.

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Section: Discussionmentioning

confidence: 67%

Aortic Thrombus Embolization in A Patient with Tuberous Sclerosis

Wolf

Rhoads

Gomes

et al. 2021

Journal of Pediatric Neurology

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“…2 Although these entities do not occur frequently, they can risk patients’ lives. 4 Multiple case reports have associated TSC with AA since 1966 5 however only 4 cases involved the thoracoabdominal aortic segment (Table 1). 6-9…”

Section: Discussionmentioning

confidence: 99%

Open Emergency Repair of a Thoracoabdominal Aortic Aneurysm on a 12-Year-Old Boy With Tuberous Sclerosis

Corso-Ramírez

López

Flórez

et al. 2023

Vasc Endovascular Surg

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Tuberous sclerosis complex is a neurocutaneous syndrome caused by an autosomal dominant genetic disorder. This condition can lead to the expression of many vascular anomalies especially, in the pediatric population. Likewise, it has been linked with aortic aneurysm development. We report a case of a 12-year-old boy who presented a 97 × 70 mm Crawford type IV thoracoabdominal aortic aneurysm. Satisfactory open surgical repair was performed with an 18-mm multibranched dacron tube graft. Clinical and imaging findings revealed a de novo tuberous sclerosis diagnosis. The patient was discharged uneventfully during a 1-month follow-up.

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“… 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. 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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Aneurisma de aorta torácica em paciente com esclerose tuberosa

Cited by 6 publications

References 7 publications

Aortic Thrombus Embolization in A Patient with Tuberous Sclerosis

Aortic Thrombus Embolization in A Patient with Tuberous Sclerosis

Open Emergency Repair of a Thoracoabdominal Aortic Aneurysm on a 12-Year-Old Boy With Tuberous Sclerosis

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

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