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BACKGROUND: Among the reconstructive methods for treating dissecting posterior circulation aneurysms, there are stent-assisted coiling (SAC), and sole stenting (SS) therapy. Despite SAC being widely employed when compared to SS, no study systematically analyzed the difference in their outcomes. METHODS: The authors conducted a meta-analysis of studies employing both therapies to compare their outcomes. A search was performed in January 2024, including only studies with consecutive patients submitted to SS or SAC. The studies had to have at least one of the following outcomes: complete aneurysm occlusion, complications, mortality, aneurysm recurrence, retreatment, and good clinical outcome. Odds ratio (OR) with 95% confidence interval (CI) were utilized for statistics. RESULTS: In a pooled analysis of 17 studies, comparing 173 SS and 377 SAC procedures for dissecting posterior circulation aneurysms, no significant differences were found in related mortality (OR 1.44; 95% CI 0.49-4.27); total mortality (OR 1.33; 95% CI 0.53-3.37); retreatment (OR 0.45; 95% CI 0.16-1.26); recurrence (OR 1.00; 95% CI 0.43-2.33); postoperative complete aneurysmal occlusion (OR 0.79; 95% CI 0.09-6.77); follow-up complete aneurysmal occlusion (OR 1.57; 95% CI 0.62-3.94); intraoperative complications (OR 1.04; 95% CI 0.29-3.73); postoperative complications (OR 1.22; 95% CI 0.61-2.45); hemorrhagic complications (OR 2.16; 95% CI 0.77-6.06); ischemic complications (OR 1.68; 95% CI 0.68-4.15). Good clinical outcomes significantly favored SAC (OR 0.45; 95% CI 0.23-0.86). CONCLUSIONS: The findings suggest there is no substantial basis for favoring SAC over SS across all cases. Instead, an individualized approach should be considered, according to the patient's characteristics, surgeon skills, and the available material.
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Resumen Introducción: La sobrevida de pacientes con síndrome de Marfan y compromiso de la raíz aórtica ha aumentado debido a la introducción de técnicas de reemplazo de raíz aórtica con preservación valvular. Objetivo: Identificar y caracterizar desenlaces a corto y largo plazo de pacientes con síndrome de Marfan intervenidos con la técnica de reemplazo de la raíz aórtica con reimplante de la válvula aórtica (procedimiento de Tirone David). Materiales y método: Serie de casos de pacientes con síndrome de Marfan sometidos al procedimiento quirúrgico de Tirone David entre 2002 y 2020. Se generaron curvas de Kaplan-Meier para evaluar el tiempo libre de reintervención aórtica y la sobrevida. Resultados: Se incluyeron 18 pacientes, con edad promedio de 29 años. Se identificó progresión de la enfermedad en el 35.3%, determinada por reintervención en 5 pacientes, disección aórtica tipo B en 3 pacientes, insuficiencia aórtica grave en 2 pacientes y aneurismas crónicos torácicos o abdominales en 5 pacientes. Se identificaron 3 muertes no relacionadas directamente con la progresión de la enfermedad. Conclusiones: En pacientes con síndrome de Marfan y patología de raíz aórtica, el procedimiento de reemplazo de la raíz aórtica con reimplante de la válvula aórtica es la técnica ideal por sus resultados en libertad de reintervención, sobrevida y calidad de vida. Sin embargo, la cirugía no resuelve el compromiso aórtico distal, el cual es el principal factor en la progresión de la enfermedad.
Abstract Introduction: The survival of patients with Marfan syndrome and aortic root involvement has increased over the past decades due to the introduction of valve-sparing aortic root replacement techniques. Objective: To identify and characterize the short- and long-term outcomes of patients with Marfan syndrome managed with aortic root replacement with aortic valve reimplantation (Tirone David procedure). Materials and method: A case series of patients diagnosed with Marfan syndrome who underwent the surgical procedure between 2002 and 2020. Kaplan-Meier curves were created to evaluate aortic reintervention-free time and survival. Results: 18 patients were included, the average age was 29 years; disease progression was identified in 35.3%, determined by reintervention in five patients, three patients had type B aortic dissection, two had severe aortic insufficiency and five had chronic thoracic or abdominal aneurysms. There were three deaths, not directly related to disease progression. Conclusions: In patients with Marfan syndrome and aortic root disease, aortic root replacement with aortic valve reimplantation is the ideal technique due to its results in results in avoiding the need for reintervention and improving survival and quality of life. However, surgery does not resolve distal aortic involvement, which is the main factor in disease progression.
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African hair shaft and pigmented scalp have unique features that challenge diagnosis in scarring alopecia. In addition, Black patients may associate 2 or more types of hair disorders. Therefore, it is imperative to understand their findings thoroughly to establish a good diagnosis. Differential diagnosis on the frontal scalp includes traction alopecia and frontal fibrosing alopecia. Disorders such as central centrifugal cicatricial alopecia, fibrosing alopecia in a pattern distribution, discoid lupus erythematosus, and lichen planopilaris usually affect the middle scalp. Folliculitis decalvans, dissecting cellulitis, and acne keloidalis nuchae are the main differential diagnosis of the posterior scalp.
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Cicatriz , Lupus Eritematoso Discoide , Humanos , Cicatriz/etiología , Alopecia/diagnóstico , Alopecia/etiología , Cabello , Cuero Cabelludo , Lupus Eritematoso Discoide/complicaciones , Lupus Eritematoso Discoide/diagnóstico , Lupus Eritematoso Discoide/patologíaAsunto(s)
Aneurisma , Enfermedades de las Arterias Carótidas , Hipopigmentación , Humanos , Enfermedades de las Arterias Carótidas/complicaciones , Enfermedades de las Arterias Carótidas/diagnóstico por imagen , Aneurisma/complicaciones , Aneurisma/diagnóstico por imagen , Aneurisma/cirugía , Arterias CarótidasRESUMEN
ABSTRACT Introduction: Acute type A aortic dissection (AAAD) in late pregnancy is a rare but severe disease. Lack of clinical experience is the main cause of high mortality. This study tries to investigate the multidisciplinary therapeutic strategy for these patients. Case presentation: We reported three patients with AAAD in late pregnancy. Sudden chest pain was the main clinical symptom before operation. All three patients and their newborns survived through multidisciplinary approach in diagnosis and treatment. No serious complications occurred during the mid-term follow-up. Conclusion: Multidisciplinary diagnosis and treatment strategy play a crucial role in saving the lives of pregnant women with AAAD.
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INTRODUCTION: Lower body perfusion (LBP) is a technique used to provide blood perfusion to distal organs and spinal cord during circulatory arrest. However, the effect of LBP on the prognosis of aortic arch surgery, especially on postoperative renal function, remains unclear. METHODS: A total of 304 patients with acute type A aortic dissection who underwent total aortic arch replacement combined with frozen elephant trunk implantation between May 2016 and December 2021 were retrospectively analyzed. The patients were divided into LBP group (group L, n=85) and non-LBP group (group NL, n=219). Routine lower body circulatory arrest was applied during operation in group NL, and antegrade LBP combined was applied during operation in group L. Perioperative data were recorded. Propensity score matching was used for statistical analysis. RESULTS: After propensity score matching, 85 pairs of patients were successfully matched. Two groups significantly differed in circulatory arrest time (six minutes vs. 30 minutes, P=0.000), cross-clamping time (101 minutes vs. 92 minutes, P=0.010), minimum nasopharyngeal temperature (29.4ºC vs. 27.2ºC, P=0.000), and highest lactate value during cardiopulmonary bypass (2.3 µmol/L vs. 4.1 µmol/L, P=0.000). Considering the postoperative indicators, the drainage volume (450 mL vs. 775 mL, P=0.000) and the incidence of level I acute kidney injury (23.5% vs. 32%, P=0.046) in group L was lower than those in group NL. CONCLUSION: LBP resulted as a safe and feasible approach in aortic arch surgery, as it could significantly shorten the circulatory arrest time, which might reduce the incidence of postoperative level I acute kidney injury.
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Lesión Renal Aguda , Aneurisma de la Aorta Torácica , Implantación de Prótesis Vascular , Humanos , Aneurisma de la Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/complicaciones , Implantación de Prótesis Vascular/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Factores de Riesgo , Aorta Torácica/cirugía , Perfusión , Incidencia , Lesión Renal Aguda/etiología , Lesión Renal Aguda/prevención & control , Lesión Renal Aguda/epidemiologíaRESUMEN
INTRODUCTION: The objectives of this study were to investigate the main treatment strategies and long-term follow-up results of aortic dissection surgery after open-heart surgery (ADSOHS) and to analyze the risk factors that cause ADSOHS. METHODS: One hundred thirty-seven patients with ADSOHS hospitalized in our hospital from January 2009 to December 2018 were selected as the research object. Long-term follow-up results, complications, mortality, and changes of cardiac function before and after operation were used to explore the value of Sun's operation. RESULTS: The length of stay in intensive care unit of these 137 patients ranged from 9.5 to 623.75 hours (average of 76.41±97.29 hours), auxiliary ventilation time ranged from 6.0 to 259.83 hours (average of 46.16±55.59 hours), and hospital stay ranged from six to 85 days (average of 25.06±13.04 days). There were seven cases of postoperative low cardiac output, 18 cases of coma and stroke, and six cases of transient neurological dysfunction. A total of 33 patients died; 19 patients died during the perioperative period, 18 died during Sun's operation and one died during other operation; and 14 patients died during follow-up (January 2021), 12 cases of Sun's operation and two cases of other operations. CONCLUSION: ADSOHS treatment strategy is of high application value, and the risk of neurological complications and mortality is low. The main risk factors are postoperative low cardiac output, coma, stroke, and transient neurological dysfunction. The extracorporeal circulation time is relatively long. Short- and long-term follow-up effects are good, and it is worthy of clinical promotion.
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Disección Aórtica , Procedimientos Quirúrgicos Cardíacos , Humanos , Estudios de Seguimiento , Aorta Torácica/cirugía , Gasto Cardíaco Bajo , Coma , Resultado del Tratamiento , Disección Aórtica/cirugía , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/métodos , Complicaciones Posoperatorias , Estudios RetrospectivosRESUMEN
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.
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Disección Aórtica , Tronco Braquiocefálico , Masculino , Humanos , Femenino , Adulto , Persona de Mediana Edad , Anciano , Tronco Braquiocefálico/cirugía , Circulación Cerebrovascular , Cateterismo/métodos , Aorta/cirugía , Disección Aórtica/cirugía , Puente Cardiopulmonar , Resultado del Tratamiento , Aorta Torácica/cirugíaRESUMEN
INTRODUCTION: Acute type A aortic dissection (AAAD) in late pregnancy is a rare but severe disease. Lack of clinical experience is the main cause of high mortality. This study tries to investigate the multidisciplinary therapeutic strategy for these patients. CASE PRESENTATION: We reported three patients with AAAD in late pregnancy. Sudden chest pain was the main clinical symptom before operation. All three patients and their newborns survived through multidisciplinary approach in diagnosis and treatment. No serious complications occurred during the mid-term follow-up. CONCLUSION: Multidisciplinary diagnosis and treatment strategy play a crucial role in saving the lives of pregnant women with AAAD.
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Aneurisma de la Aorta , Disección Aórtica , Embarazo , Humanos , Recién Nacido , Femenino , Aneurisma de la Aorta/complicaciones , Aneurisma de la Aorta/diagnóstico por imagen , Aneurisma de la Aorta/cirugía , Resultado del Tratamiento , Disección Aórtica/complicaciones , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/cirugía , Complicaciones Posoperatorias/etiologíaRESUMEN
ABSTRACT Introduction: Lower body perfusion (LBP) is a technique used to provide blood perfusion to distal organs and spinal cord during circulatory arrest. However, the effect of LBP on the prognosis of aortic arch surgery, especially on postoperative renal function, remains unclear. Methods: A total of 304 patients with acute type A aortic dissection who underwent total aortic arch replacement combined with frozen elephant trunk implantation between May 2016 and December 2021 were retrospectively analyzed. The patients were divided into LBP group (group L, n=85) and non-LBP group (group NL, n=219). Routine lower body circulatory arrest was applied during operation in group NL, and antegrade LBP combined was applied during operation in group L. Perioperative data were recorded. Propensity score matching was used for statistical analysis. Results: After propensity score matching, 85 pairs of patients were successfully matched. Two groups significantly differed in circulatory arrest time (six minutes vs. 30 minutes, P=0.000), cross-clamping time (101 minutes vs. 92 minutes, P=0.010), minimum nasopharyngeal temperature (29.4ºC vs. 27.2ºC, P=0.000), and highest lactate value during cardiopulmonary bypass (2.3 μmol/L vs. 4.1 μmol/L, P=0.000). Considering the postoperative indicators, the drainage volume (450 mL vs. 775 mL, P=0.000) and the incidence of level I acute kidney injury (23.5% vs. 32%, P=0.046) in group L was lower than those in group NL. Conclusion: LBP resulted as a safe and feasible approach in aortic arch surgery, as it could significantly shorten the circulatory arrest time, which might reduce the incidence of postoperative level I acute kidney injury.
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ABSTRACT Introduction: The objectives of this study were to investigate the main treatment strategies and long-term follow-up results of aortic dissection surgery after open-heart surgery (ADSOHS) and to analyze the risk factors that cause ADSOHS. Methods: One hundred thirty-seven patients with ADSOHS hospitalized in our hospital from January 2009 to December 2018 were selected as the research object. Long-term follow-up results, complications, mortality, and changes of cardiac function before and after operation were used to explore the value of Sun's operation. Results: The length of stay in intensive care unit of these 137 patients ranged from 9.5 to 623.75 hours (average of 76.41±97.29 hours), auxiliary ventilation time ranged from 6.0 to 259.83 hours (average of 46.16±55.59 hours), and hospital stay ranged from six to 85 days (average of 25.06±13.04 days). There were seven cases of postoperative low cardiac output, 18 cases of coma and stroke, and six cases of transient neurological dysfunction. A total of 33 patients died; 19 patients died during the perioperative period, 18 died during Sun's operation and one died during other operation; and 14 patients died during follow-up (January 2021), 12 cases of Sun's operation and two cases of other operations. Conclusion: ADSOHS treatment strategy is of high application value, and the risk of neurological complications and mortality is low. The main risk factors are postoperative low cardiac output, coma, stroke, and transient neurological dysfunction. The extracorporeal circulation time is relatively long. Short- and long-term follow-up effects are good, and it is worthy of clinical promotion.
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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.
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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
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Humanos , Aorta Torácica , Aneurisma de la Aorta , Procedimientos Endovasculares , Rotura de la Aorta , Disección AórticaRESUMEN
Intramyocardial dissecting hematoma is a form of cardiac rupture caused by myocardial infarction, percutaneous coronary intervention, or trauma. It is a cavity between myocardial fibers caused by partial rupture of the ventricular wall. Therapeutic management, including the timing for surgical approach, has not been standardized. We present a case series describing 4 patients. (Level of Difficulty: Intermediate.).
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ABSTRACT Introduction: Although endovascular correction is a promising perspective, the gold-standard treatment for thoracoabdominal aortic aneurisms and type-B dissections with visceral involvement remains open surgery, particularly due to its well-established long-term durability. This study aims to describe and evaluate public data from patients treated for thoracoabdominal aortic aneurism in the Brazilian public health system in a 12-year interval. Methods: Data from procedures performed between 2008 and 2019 were extracted from the national public database (Departamento de Informática do Sistema Único de Saúde, or DATASUS) using web scraping techniques. Procedures were evaluated regarding the yearly frequency of elective or urgency surgeries, in-hospital mortality, and governmental costs. All tests were done with a level of significance P<0.05. Results: A total of 812 procedures were analyzed. Of all surgeries, 67.98% were elective cases. There were 328 in-hospital deaths (mortality of 40.39%). In-hospital mortality was lower in elective procedures (26.92%) than in urgency procedures (46.74%) (P=0.008). Total governmental expenditure was $3.127.051,56 — an average of $3.774,22 for elective surgery and $3.791,93 for emergency surgery (P=0.999). Conclusion: The proportion of urgency procedures is higher than that recommended by international literature. Mortality was higher for urgent admissions, although governmental costs were equal for elective and urgent procedures; specialized referral centers should be considered by health policy makers.
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RESUMEN El síndrome de Marfan es una enfermedad genética autosómica dominante del tejido conectivo, caracterizada por una combinación variable de manifestaciones cardiovasculares, músculo-esqueléticas y oftalmológicas. A pesar del descubrimiento de las mutaciones causales, su diagnóstico resulta complejo, al exhibir una gran diversidad en su presentación clínica y carecer de características patognomónicas. El diagnóstico actual de síndrome de Marfan se basa en una serie de criterios clínicos y genéticos denominados Criterios Gante revisados. Se describe el caso de una paciente de 44 años de edad, con antecedentes de luxación del cristalino, miopía y escoliosis, sin antecedentes patológicos familiares y que cumplió con los criterios diagnósticos actuales. Se sugiere la pesquisa etiológica de afecciones como luxación del cristalino y escoliosis, por parte de las especialidades correspondientes, como traducción orgánica de una enfermedad sistémica como el síndrome de Marfan.
ABSTRACT Marfan syndrome is an autosomal dominant genetic disease of connective tissue, characterized by a variable combination of cardiovascular, musculoskeletal, and ophthalmologic manifestations. Despite the discovery of the causal mutations, its diagnosis is complex, as it exhibits great diversity in its clinical presentation and lacks pathognomonic characteristics. The current diagnosis of Marfan syndrome is based on a series of clinical and genetic criteria called the revised Ghent Criteria. The case of a 44-years-old female patient with a history of lens dislocation, myopia and scoliosis, with no family pathological history and who met current diagnostic criteria is described. The etiological investigation of conditions such as lens dislocation and scoliosis is suggested, by the corresponding specialties, as an organic translation of a systemic disease such as Marfan syndrome.