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INTRODUCTION: The aim of this study was to evaluate the efficacy and safety of innominate artery cannulation strategy with side-graft technique in proximal aortic pathologies. METHODS: A total of 70 patients underwent innominate artery cannulation with a side graft for surgery on the proximal aorta from 2012 to 2020. There were 46 men and 24 women with an average age of 56±13 years. The indications for surgery were type A aortic dissection in 17 patients (24.3%), aortic aneurysm in 52 patients (74.3%), and ascending aorta pseudoaneurysm in one patient (1.4%). The innominate artery was free of disease in all patients. Hypothermic circulatory arrest with antegrade cerebral perfusion was utilized in 60 patients (85.7%). Three patients had previous sternotomy (4.2%). The most common surgical procedure was ascending aorta with hemiarch replacement in 34 patients (48.5%). RESULTS: The mean cardiac ischemia and cardiopulmonary bypass times were 116+46 minutes and 164+56 minutes, respectively. Mean antegrade cerebral perfusion time was 27+14 minutes. The patients were cooled between 22°C and 30°C during surgery. Thirty-day mortality rate was 7.1% (five patients). One patient (1.4%) had stroke, one patient (1.4%) had temporary neurologic deficit, and eight patients (11.4%) had confusion and agitation that resolved completely in all cases. There was no local complication or arterial injury. CONCLUSION: Cannulation of the innominate artery with side graft is safe and effective for both cardiopulmonary bypass and antegrade cerebral perfusion. This technique provides satisfactory neurologic outcomes for proximal aortic surgery.
Subject(s)
Aortic Dissection , Brachiocephalic Trunk , Male , Humans , Female , Adult , Middle Aged , Aged , Brachiocephalic Trunk/surgery , Cerebrovascular Circulation , Catheterization/methods , Aorta/surgery , Aortic Dissection/surgery , Cardiopulmonary Bypass , Treatment Outcome , Aorta, Thoracic/surgeryABSTRACT
ABSTRACT Introduction: The aim of this study was to evaluate the efficacy and safety of innominate artery cannulation strategy with side-graft technique in proximal aortic pathologies. Methods: A total of 70 patients underwent innominate artery cannulation with a side graft for surgery on the proximal aorta from 2012 to 2020. There were 46 men and 24 women with an average age of 56±13 years. The indications for surgery were type A aortic dissection in 17 patients (24.3%), aortic aneurysm in 52 patients (74.3%), and ascending aorta pseudoaneurysm in one patient (1.4%). The innominate artery was free of disease in all patients. Hypothermic circulatory arrest with antegrade cerebral perfusion was utilized in 60 patients (85.7%). Three patients had previous sternotomy (4.2%). The most common surgical procedure was ascending aorta with hemiarch replacement in 34 patients (48.5%). Results: The mean cardiac ischemia and cardiopulmonary bypass times were 116+46 minutes and 164+56 minutes, respectively. Mean antegrade cerebral perfusion time was 27+14 minutes. The patients were cooled between 22°C and 30°C during surgery. Thirty-day mortality rate was 7.1% (five patients). One patient (1.4%) had stroke, one patient (1.4%) had temporary neurologic deficit, and eight patients (11.4%) had confusion and agitation that resolved completely in all cases. There was no local complication or arterial injury. Conclusion: Cannulation of the innominate artery with side graft is safe and effective for both cardiopulmonary bypass and antegrade cerebral perfusion. This technique provides satisfactory neurologic outcomes for proximal aortic surgery.
ABSTRACT
ABSTRACT The management of Type A aortic dissection has evolved over a period of a decade or so, and contemporary reports are suggesting a paradigm shift from a conservative approach to complete excision of the diseased aorta including root and distal arch. Improved cardiopulmonary bypass perfusion techniques, better understanding of the cerebral perfusion, and wide-ranging obtainability of prosthetic conduits gave surgical teams numerous choices. With improving outcomes and maturing surgical techniques, surgeons are performing extensive resections of the diseased aorta, but there is no standard protocol as far as the extent of the proximal and distal diseased aortic tissue resection is concerned. Aortic root replacement is associated with good early- and long-term outcomes and proffered solution in young and stable patients, for that reason many busy centres are endorsing total arch replacement in complex distal aortic dissections. This systemic review is discussing contemporary literature and associated pros and cons during surgical decision-making for these high-risk cases.
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ABSTRACT Introduction: The aim of this study is to present a series of six cases with thoracoabdominal aneurysm treated with hybrid technique in our center. Methods: Between May 2015 and December 2018, the data of six patients with thoracoabdominal aneurysms and various comorbidities who underwent visceral debranching followed by endovascular aortic aneurysm repair were reviewed retrospectively. Results: Patients' mean age was 65.3±19.6 years. All of them were male. Comorbidities were old age, congestive heart failure, coronary artery disease, chronic obstructive pulmonary disease, previous surgical interventions, and/or esophageal hemangioma. Except for one patient who underwent coronary artery bypass grafting (inflow was taken from ascending aorta), debranching was performed from the right iliac artery. Debranching of four visceral arteries (superior mesenteric artery, celiac trunk, and bilateral renal right arteries) was performed in three patients, of three visceral arteries (superior mesenteric artery, celiac trunk, right renal artery) was performed in one, and of two visceral arteries (superior mesenteric artery, celiac trunk) was performed in two patients. Great saphenous vein and 6-mm polytetrafluoroethylene grafts were used in one and five patients, respectively, for debranching. Endovascular aneurysm repair was performed following debranching procedures as soon as the patients were stabilized. In total, three patients died at the early, mid, and long-term follow-up due to multiorgan failure, pneumonia, and unknown reasons. Conclusion: Hybrid repair of thoracoabdominal aneurysms may be an alternative to fenestrated or branched endovascular stent grafts in patients with increased risk factors for open surgical thoracoabdominal aneurysm repair; however, the procedure requires experience and care.
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Introducción. La patología del arco aórtico se ha tratado principalmente con cirugía por vía abierta, pero con una alta morbimortalidad. Las técnicas endovasculares híbridas y las reconstrucciones en "chimenea" son una técnica válida y segura para disminuir el riesgo y la mortalidad. Métodos. Se presentan dos pacientes con patología del arco aórtico y contraindicación de manejo quirúrgico abierto, atendidos en el Servicio de Cirugía Vascular, Hospital Universitario Clínica de San Rafael, Bogotá, D.C., Colombia. Resultados. Se realizaron dos procedimientos endovasculares del arco aórtico para tratar un aneurisma torácico roto y una úlcera aórtica sintomática, con cubrimiento de los troncos supra aórticos con una endoprótesis y canalización de los vasos supra aórticos con prótesis cubiertas y uso de la "técnica de chimenea", de manera exitosa. Discusión. La patología del arco aórtico es de alta complejidad y se asocia con una morbimortalidad elevada por lo que, en los últimos 20 años se han desarrollado diferentes técnicas utilizando procedimientos percutáneos. Conclusión. La "técnica de chimenea" se puede realizar de una manera mínimamente invasiva en pacientes con patología del arco aórtico, no candidatos para cirugía abierta, con resultados exitosos.
Introduction. Aortic arch pathology has been treated mainly by open surgery, but with high morbidity and mortality. Hybrid endovascular techniques and "chimney" reconstructions are a valid and safe techniques to reduce risk and mortality. Method. Two patients with pathology of the aortic arch and contraindication for open surgical management, treated at the Vascular Surgery Service, Hospital Universitario Clínica de San Rafael, Bogotá, Colombia, are presented. Results. Two endovascular aortic procedures were performed successfully to treat a ruptured thoracic aneurysm and a symptomatic aortic ulcer, with coverage of the supra-aortic trunks with an endoprosthesis and cannulation of the supra-aortic vessels with covered prostheses and use of the "chimney technique". Discussion. The pathology of the aortic arch is highly complex and is associated with high morbidity and mortality, being the reason that during the last 20 years, different techniques have been developed using percutaneous procedures. Conclusions. The "chimney technique" can be performed with successful results in a minimally invasive manner in patients with pathology of the aortic arch, who are not candidates for open surgery
Subject(s)
Humans , Aorta, Thoracic , Aortic Aneurysm , Endovascular Procedures , Aortic Rupture , Aortic DissectionABSTRACT
The management of Type A aortic dissection has evolved over a period of a decade or so, and contemporary reports are suggesting a paradigm shift from a conservative approach to complete excision of the diseased aorta including root and distal arch. Improved cardiopulmonary bypass perfusion techniques, better understanding of the cerebral perfusion, and wide-ranging obtainability of prosthetic conduits gave surgical teams numerous choices. With improving outcomes and maturing surgical techniques, surgeons are performing extensive resections of the diseased aorta, but there is no standard protocol as far as the extent of the proximal and distal diseased aortic tissue resection is concerned. Aortic root replacement is associated with good early- and long-term outcomes and proffered solution in young and stable patients, for that reason many busy centres are endorsing total arch replacement in complex distal aortic dissections. This systemic review is discussing contemporary literature and associated pros and cons during surgical decision-making for these high-risk cases.
Subject(s)
Aortic Dissection , Humans , Acute Disease , Aortic Dissection/surgery , Aorta/surgery , Cardiopulmonary Bypass , Perfusion , Aorta, Thoracic/surgeryABSTRACT
INTRODUCTION: The aim of this study is to present a series of six cases with thoracoabdominal aneurysm treated with hybrid technique in our center. METHODS: Between May 2015 and December 2018, the data of six patients with thoracoabdominal aneurysms and various comorbidities who underwent visceral debranching followed by endovascular aortic aneurysm repair were reviewed retrospectively. RESULTS: Patients' mean age was 65.3±19.6 years. All of them were male. Comorbidities were old age, congestive heart failure, coronary artery disease, chronic obstructive pulmonary disease, previous surgical interventions, and/or esophageal hemangioma. Except for one patient who underwent coronary artery bypass grafting (inflow was taken from ascending aorta), debranching was performed from the right iliac artery. Debranching of four visceral arteries (superior mesenteric artery, celiac trunk, and bilateral renal right arteries) was performed in three patients, of three visceral arteries (superior mesenteric artery, celiac trunk, right renal artery) was performed in one, and of two visceral arteries (superior mesenteric artery, celiac trunk) was performed in two patients. Great saphenous vein and 6-mm polytetrafluoroethylene grafts were used in one and five patients, respectively, for debranching. Endovascular aneurysm repair was performed following debranching procedures as soon as the patients were stabilized. In total, three patients died at the early, mid, and long-term follow-up due to multiorgan failure, pneumonia, and unknown reasons. CONCLUSION: Hybrid repair of thoracoabdominal aneurysms may be an alternative to fenestrated or branched endovascular stent grafts in patients with increased risk factors for open surgical thoracoabdominal aneurysm repair; however, the procedure requires experience and care.
Subject(s)
Aortic Aneurysm, Abdominal , Aortic Aneurysm, Thoracic , Blood Vessel Prosthesis Implantation , Endovascular Procedures , Humans , Male , Middle Aged , Aged , Aged, 80 and over , Female , Aortic Aneurysm, Thoracic/surgery , Aortic Aneurysm, Thoracic/etiology , Blood Vessel Prosthesis Implantation/methods , Blood Vessel Prosthesis , Aortic Aneurysm, Abdominal/surgery , Aortic Aneurysm, Abdominal/etiology , Endovascular Procedures/adverse effects , Retrospective Studies , Follow-Up Studies , Treatment Outcome , Stents , Prosthesis DesignABSTRACT
BACKGROUND: Intracranial dissecting aneurysms (IDAs) are rare vascular lesions usually arising from the posterior circulation. The anterior cerebral artery (ACA) is an unusual location for this pathology. Even rarer is the occurrence of a transient de novo third cranial nerve (CN) palsy after flow-diverting device (FDD) treatment of an ACA dissecting aneurysm. CASE DESCRIPTION: A middle-aged man with a prior history of hypertension was admitted to our emergency department with severe headache and loss of consciousness after sexual intercourse. Imaging revealed a subarachnoid hemorrhage with stenosis of the left A1 segment of the ACA. Cerebral digital subtraction angiography confirmed a dissecting aneurysm of the left A1 segment. The aneurysm was treated with an FDD (Pipeline Shield). Transient isolated incomplete third CN palsy was documented 12 h after treatment. No evidence of ischemic or hemorrhagic strokes was found. The condition improved after a few days of empiric steroid treatment. CONCLUSION: An FDD is a suitable alternative for the treatment of a ruptured IDA of the anterior circulation. Some infrequent complications associated with the device, such as de novo cranial neuropathies, are yet to be studied.
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Se describe el caso de un paciente de 70 años que consultó por cefalea súbita, tipo trueno, sin alteración del estado de consciencia, acompañada de dolor torácico de una hora de evolución y de baja intensidad. A su ingreso fue enfocado como cefalea en trueno, que es clasificada, en cuanto a la atención, como bandera roja. La medición de troponina fue negativa y una tomografía de cráneo fue leída como normal. Desde el ingreso presentaba signos vitales normales, cuando iba a ser dado de alta se torna hipotenso (completamente asintomático) y por su síntoma cardinal (cefalea), que se asoció a dolor torácico leve y no anginoso, se solicitó angiotomografía toracoabdominal, con la que se demostró aneurisma disecante de la aorta. Con la presentación de este caso, se busca resaltar la importancia en el servicio de urgencias de la asociación de la cefalea tipo trueno, con condiciones vasculares como la disección aórtica.
We describe the case of a 70-year-old patient, who seeks medical advice due to sudden, thunder headache, without alteration of the state of consciousness, accompanied by chest pain of 1 hour of evolution and of low intensity. Upon his admission, the patient was treated as a thunderclap headache, which is considered a red flag. His troponin was negative, and his head tomography was interpreted as normal. From admission he had normal vital signs, but when he was going to be discharged, he became hypotensive (completely asymptomatic) and due to his cardinal symptom (headache) that was asso-ciated with mild non-anginal chest pain, a thoracoabdominal angioCT was requested, with which dissecting aneurysm of the aorta was evidenced. With the presentation of this case, we seek to highlight the importance of the association of thunder-type headache with possible vascular conditions such as aortic dissection in the emergency department.
Descrevemos o caso de uma paciente de 70 anos que consultou por quadro de cefaleia súbita, tipo trovão, sem alteração do estado de consciência, acompanhada de dor torácica de uma hora de evolução e de baixa intensidade. Na admissão, foi tratado como cefaleia em trovoada, que é classificada, em termos de atenção, como bandeira vermelha. A me-dição da troponina foi negativa e uma tomografia de crânio foi lida como normal. Desde a admissão apresentava sinais vitais normais, quando ia receber alta ficou hipotenso (totalmente assintomático) e devido ao seu sintoma cardinal (cefaleia), que se associou a dores torácicas ligeiras e não anginosas, foi solicitada angiografia toracoabdominal, com cujo aneurisma dissecante da aorta foi demonstrado. Com a apresentação deste caso, o objetivo é destacar a importância no pronto-socorro da associação da cefaleia do tipo trovão com afecções vasculares como a dissecção da aorta.
Subject(s)
Humans , Aortic Dissection , Aorta , Chest Pain , Angiography , HeadacheABSTRACT
Persistent embryological connections between the anterior and posterior circulations are rare entities. Persistent hypoglossal artery is the second most common persistent carotid-basilar anastomosis. As it is often associated with hypoplasia of vertebral arteries, it poses a challenge during endovascular interventions. We present a case of a 32-year-old woman who presented with occipital headache of four weeks' duration. Magnetic Resonance Angiography showed hypoplastic vertebral arteries with a persistent hypoglossal artery arising from the cervical segment of the left internal carotid artery and supplying the entire posterior circulation, associated with a dissecting aneurysm of the right posterior cerebral artery. Endovascular parent vessel occlusion was performed for the dissecting posterior cerebral artery aneurysm by navigating the guide catheter, microwire, and microcatheter through the persistent hypoglossal artery because the vertebral arteries were hypoplastic. Post-intervention, the patient did not develop any neurological deficit and was discharged in a stable condition.
Conexões embriológicas persistentes entre as circulações anterior e posterior são entidades raras. A artéria hipoglossa persistente é a segunda anastomose carotídeo-basilar persistente mais comum. Como está frequentemente associada à hipoplasia das artérias vertebrais, apresenta um desafio durante as intervenções endovasculares. Apresentamos o caso de uma mulher de 32 anos que apresentou cefaleia occipital com duração de quatro semanas. A angiografia por ressonância magnética mostrou artérias vertebrais hipoplásicas com artéria hipoglossa persistente surgindo do segmento cervical da artéria carótida interna esquerda e suprindo toda a circulação posterior com um aneurisma dissecante da artéria cerebral posterior direita. A oclusão endovascular do vaso parental foi realizada para o aneurisma da dissecção da artéria cerebral posterior pela passagem de cateter guia, microfio e microcateter pela artéria hipoglossa persistente, pois as artérias vertebrais eram hipoplásicas. Após a intervenção, a paciente não apresentou déficit neurológico e recebeu alta em uma condição estável.
ABSTRACT
RESUMEN La disección de la aorta abdominal tiene una baja incidencia, se produce a partir de una laceración, desgarro o rotura intimal, con la consecuente separación longitudinal de las capas de la pared y la aparición de una falsa luz con flujo en su interior. Se presenta el caso de un hombre de 67 años de edad con antecedentes de hipertensión arterial, sin tratamiento regular, que acudió al cuerpo de guardia del Hospital Salvador Allende (La Habana, Cuba) por presentar dolor abdominal con irradiación a fosa ilíaca izquierda y espalda, de una semana de duración, que no aliviaba con los analgésicos habituales. Se le realizó ultrasonido abdominal y se encontró una dilatación aneurismática de la aorta abdominal, con signos de disección hacia la ilíaca derecha; por lo que se realizó angiotomografía que confirmó el diagnóstico. Se realizó baipás aorto-ilíaco con fenestración en aorta abdominal y anastomosis término-terminal en ambas arterias ilíacas.
ABSTRACT Abdominal aortic dissection has a low incidence. It may happen when a small tear or rupture occurs in the tunica intima, dividing the wall layers and forming a false channel, or lumen with blood flow inside. We present the case of a 67-year-old man with a history of high blood pressure, with no regular treatment, who sought care at the Hospital Salvador Allende (Havana, Cuba) as he presented with a week-long abdominal pain radiating to the left iliac fossa and back, which was not relieved by the usual analgesics. An abdominal ultrasound was performed which found an aneurysmal dilatation of the abdominal aorta, with signs of dissection towards the right iliac artery; therefore, computed tomography angiography (CTA) was performed and the diagnosis was confirmed. He underwent aorto-iliac bypass with abdominal-aortic-fenestration and end-to-end anastomosis in both iliac arteries.
Subject(s)
Aorta, Abdominal , Diagnostic Imaging , Tomography, X-Ray Computed , Dissection , Aortic DissectionABSTRACT
Abstract Persistent embryological connections between the anterior and posterior circulations are rare entities. Persistent hypoglossal artery is the second most common persistent carotid-basilar anastomosis. As it is often associated with hypoplasia of vertebral arteries, it poses a challenge during endovascular interventions. We present a case of a 32-year-old woman who presented with occipital headache of four weeks' duration. Magnetic Resonance Angiography showed hypoplastic vertebral arteries with a persistent hypoglossal artery arising from the cervical segment of the left internal carotid artery and supplying the entire posterior circulation, associated with a dissecting aneurysm of the right posterior cerebral artery. Endovascular parent vessel occlusion was performed for the dissecting posterior cerebral artery aneurysm by navigating the guide catheter, microwire, and microcatheter through the persistent hypoglossal artery because the vertebral arteries were hypoplastic. Post-intervention, the patient did not develop any neurological deficit and was discharged in a stable condition.
Resumo Conexões embriológicas persistentes entre as circulações anterior e posterior são entidades raras. A artéria hipoglossa persistente é a segunda anastomose carotídeo-basilar persistente mais comum. Como está frequentemente associada à hipoplasia das artérias vertebrais, apresenta um desafio durante as intervenções endovasculares. Apresentamos o caso de uma mulher de 32 anos que apresentou cefaleia occipital com duração de quatro semanas. A angiografia por ressonância magnética mostrou artérias vertebrais hipoplásicas com artéria hipoglossa persistente surgindo do segmento cervical da artéria carótida interna esquerda e suprindo toda a circulação posterior com um aneurisma dissecante da artéria cerebral posterior direita. A oclusão endovascular do vaso parental foi realizada para o aneurisma da dissecção da artéria cerebral posterior pela passagem de cateter guia, microfio e microcateter pela artéria hipoglossa persistente, pois as artérias vertebrais eram hipoplásicas. Após a intervenção, a paciente não apresentou déficit neurológico e recebeu alta em uma condição estável.
Subject(s)
Humans , Female , Adult , Arteriovenous Anastomosis/surgery , Posterior Cerebral Artery/surgery , Aortic Dissection/surgery , Vertebral Artery/pathology , Magnetic Resonance Angiography , Endovascular Procedures , Headache , Aortic Dissection/diagnostic imagingABSTRACT
BACKGROUND: Fibromuscular dysplasia affects generally renal artery, causing renovascular hypertension. The most classical angiographic pattern, string-of-beads, can be found in cervical and more rarely in other arteries. With the advance of endovascular procedures techniques, the number of open surgeries is decreasing, and complications related to the selective catheterization of diseased vessels are increasing. CASE REPORT: A 37-year-old man presenting with subarachnoid hemorrhage was submitted to angioplasty for dissecting aneurysms of vertebral artery with a good outcome. Several arteries were angiographically diagnosed with the dysplasia (renal, carotid, femoral), and some complications like stenosis, dissection, arteriovenous fistula, and dissecting aneurysm occurred in sequence. CONCLUSIONS: FMD of cervical arteries is usually asymptomatic. There are no guidelines or protocols to cervical FMD treatment, being indicated only for the complications. Because of the vessels fragility, a several spontaneous or post endovascular procedure complications can be disastrous.
Subject(s)
Aortic Dissection , Fibromuscular Dysplasia , Subarachnoid Hemorrhage , Adult , Carotid Arteries , Fibromuscular Dysplasia/complications , Fibromuscular Dysplasia/diagnostic imaging , Humans , Male , Vertebral ArteryABSTRACT
Background Fibromuscular dysplasia (FMD) affects predominantly the cervical and renal arteries and may cause the classical angiographic pattern of string-of-beads. The diagnosis is increasing with the advances of imaging techniques. Case Report A 37-year-old man presenting with subarachnoid hemorrhage due to a dissecting aneurysm of the vertebral artery was treated by angioplasty with stent, with good outcome. All of the cervical and renal arteries were diseased and showed dysplasia and/or ectasias. Conclusions There are no guidelines or protocols to treat patients with FMD.
Subject(s)
Humans , Male , Adult , Subarachnoid Hemorrhage/complications , Carotid Stenosis/etiology , Endovascular Procedures/rehabilitation , Fibromuscular Dysplasia/complications , Aortic Dissection/diagnostic imaging , Angioplasty/methods , Vascular Malformations/complicationsABSTRACT
Abstract Objective: To investigate whether axillary artery cannulation has supremacy over innominate artery cannulation in thoracic aortic surgery. Methods: A comprehensive search was undertaken among the four major databases (PubMed, Excerpta Medica dataBASE [EMBASE], Scopus, and Ovid) to identify all randomized and nonrandomized controlled trials comparing axillary to innominate artery cannulation in thoracic aortic surgery. Databases were evaluated and assessed up to March 2017. Results: Only three studies fulfilled the criteria for this meta-analysis, including 534 patients. Cardiopulmonary bypass time was significantly shorter in the innominate group (P=0.004). However, the innominate group had significantly higher risk of prolonged intubation > 48 hours (P=0.04) than the axillary group. Further analysis revealed no significant difference between the innominate and axillary groups for deep hypothermic circulatory arrest time (P=0.06). The relative risks for temporary and permanent neurological deficits as well as in-hospital mortality were not significantly different for both groups (P=0.90, P=0.49, and P=0.55, respectively). Length of hospital stay was similar for both groups. Conclusion: There is no superiority of axillary over innominate artery cannulation in thoracic aortic surgery in terms of perioperative outcomes; however, as the studies were limited, larger scale comparative studies are required to provide a solid evidence base for choosing optimal arterial cannulation site.
Subject(s)
Humans , Male , Female , Aorta, Thoracic/surgery , Axillary Artery/surgery , Catheterization/methods , Brachiocephalic Trunk/surgery , Postoperative Complications , Catheterization/adverse effects , Catheterization/mortality , Treatment Outcome , Hospital MortalityABSTRACT
Mucopolysaccharidoses (MPS) are disorders characterized by impaired glycosaminoglycan (GAG) catabolism as a consequence of a deficiency or the absence of lysosomal enzymes directly involved in their degradation. Multiple organ systems are involved in MPS, including the cardiovascular system. Recently, aortic root dilatation (ARD) has been described in these patients. Thus, we reviewed aortic root diameter measurements in 69 MPS patients from a single center from 2000 to 2016. Aortic root diameter z scores were calculated based on data published by Colan et al. according to the body surface area (BSA) determined using the Haycock formula. The overall incidence of ARD in MPS patients was 39.1%. Higher mean z scores were present in patients with MPS IVA and VI when compared to MPS I and II. Aortic root z scores were higher in older MPS IVA patients, which may suggest a progressive ARD change in this MPS type. No significant differences were found before and after enzyme replacement therapy (ERT) in 11 patients with available data (2 with MPS I; 4 with MPS II; 2 with MPS IVA, and 3 with MPS VI). This work provides further evidence that ARD is common in different types of MPS, being especially evident in MPS IVA, but with a significant occurrence also in MPS VI.
Subject(s)
Aorta, Thoracic/diagnostic imaging , Aortic Aneurysm, Thoracic/etiology , Aortic Dissection/etiology , Enzyme Replacement Therapy/methods , Mucopolysaccharidoses/complications , Adolescent , Aortic Dissection/diagnosis , Aortic Aneurysm, Thoracic/diagnosis , Echocardiography , Female , Follow-Up Studies , Humans , Male , Mucopolysaccharidoses/drug therapy , Time FactorsABSTRACT
Spontaneous basilar artery dissection is a rare condition and a diagnostic challenge with a high potential for morbidity and mortality if untreated. It has an estimated incidence of 1 to 1.5 cases per 100,000 people. Few cases have been described in the literature up to the present day. The clinical outcomes, prognosis and treatment remain uncertain. The authors report the rare case of a 55-year-old female patient who presented to the Interventional Neuroradiology service at Hospital São Marcos, Teresina, in the state of Piauí, Brazil, with a history of severe headache located in the occipital region and in the nape with no improvement using common analgesics. A magnetic resonance imaging of the brain showed a saccular dilatation in the basilar artery, and a digital cerebral angiography showed a basilar artery dissection associated with a dissecting aneurysm.
Subject(s)
Humans , Female , Middle Aged , Basilar Artery/diagnostic imaging , Vertebral Artery Dissection/diagnostic imaging , Basilar Artery/surgery , Angiography, Digital Subtraction/methods , Vertebral Artery Dissection/surgeryABSTRACT
Introduction The incidence of intracranial aneurysms in the pediatric population is low, and endovascular treatment is becoming a safe and minimally invasive treatment option. In the present study, the occurrence of special features of cerebral aneurysm in children, in comparison to adults, is also described. Case Report A 3-month-old female infant presented with progressive proptosis and divergent strabismus at the right eye, in addition to inconsolable crying. Cerebral resonance, angiotomography and angiography exams demonstrated angiodysplasia in the right internal carotid artery with two large paraclinoid dissecting aneurysms with wide neck. The right internal carotid artery was occluded with coils by endovascular approach, without detriment to the perfusion of the ipsilateral hemisphere and without neurological deficits. The patient achieved good recovery, and a late control angiotomography confirmed the exclusion of the aneurysms. Conclusion Parent artery sacrifice via endovascular approach is an effective therapeutic option, but a long-termfollow-up is necessary to avoid recurrence and bleeding.
Subject(s)
Humans , Female , Infant , Carotid Artery, Internal/surgery , Intracranial Aneurysm/surgery , Endovascular Procedures/methods , Aortic Dissection/surgery , Exophthalmos/complications , Exotropia/complications , Computed Tomography AngiographyABSTRACT
A Doença de Behçet é uma doença inflamatória caracterizada por úlceras genitais e orais recorrentes, uveítes e lesões cutâneas. O envolvimento arterial é raro, sendo mais comuns as degenerações aneurismáticas do que as oclusivas. Neste caso clínico, paciente do sexo feminino, em tratamento de doença de Behçet havia 20 anos, iniciou com dor abdominal progressiva por dois meses, com piora súbita importante, foi submetida à tomografia computadorizada, que mostrou pseudoaneurisma toracoabdominal. A paciente foi submetida a tratamento endovascular com sucesso.
Behçets disease is an inflammatory disease characterized by recurrent oral and genital ulcers, uveitis and skin lesions. Arterial involvement is rare, but when present aneurysmal degeneration is more common than occlusive disease. This report describes the clinical case of a female patient who had been receiving treatment for Behçets disease for twenty years before presenting with abdominal pain that progressed for 2 months before suddenly worsening significantly. A CT scan revealed a thoracoabdominal pseudoaneurysm. She was successfully treated with endovascular repair.