RESUMO
Aortopathies associated to Marfan syndrome (MFS) are important causes of maternal death during pregnancy. We present a 27-year-old and 24-week pregnant MFS woman who arrived to the emergency department with increasing abdominal pain; an obstetric ultrasound showed an Abdominal Aortic Aneurysm (AAA), a multislice computed tomography angiography (CTA) confirmed and demonstrated a 7.3 centimeter (cm) infrarenal AAA without evidence of dissection. A multidisciplinary committee determined that an open repair would lead to a significantly high maternal-fetal morbidity and mortality. Although endovascular repair (EVAR) in MFS patients remains controversial, an urgent bridge therapy was considered to be the best option. She was transferred to the angiography suite for EVAR to prevent AAA rupture and ensure a satisfactory pregnancy. The patient and fetus presented no complications during the procedure and were discharged 3 days later. She continued her pregnancy without eventualities and an elective C-section was performed on week 36. A CTA imaging at 12 months revealed type 1A and 3 endoleaks, we decided to perform endograft explant and a definitive open repair, there were no complications during the procedure, the patient is currently asymptomatic. Our case illustrates a complex decision and management that successfully avoid morbidity and mortality of a MFS mother and her product; additionally, this experience reinforces the need for lifelong and close surveillance in these patients.
Assuntos
Aneurisma da Aorta Abdominal , Implante de Prótese Vascular , Procedimentos Endovasculares , Síndrome de Marfan , Adulto , Aneurisma da Aorta Abdominal/diagnóstico por imagem , Aneurisma da Aorta Abdominal/etiologia , Aneurisma da Aorta Abdominal/cirurgia , Implante de Prótese Vascular/efeitos adversos , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Síndrome de Marfan/complicações , Síndrome de Marfan/diagnóstico , Gravidez , Gestantes , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Endovascular repair has become the gold standard for the treatment of infrarenal abdominal aortic aneurysmal disease. Branched and fenestrated commercially custom-made devices have been developed as a treatment option for short necks or juxtarenal aneurysms. However, the lack of availability in some countries and centers, manufacturing time (6-8 weeks requirements), urgent setting in ruptured abdominal aortic aneurysms, and elevated costs make them not a widespread option. Hereby, we expose our step-by-step technique of physician modified Gore Excluder to treat complex aneurysms. Physician-modified endovascular graft (PMEG) has emerged as an alternative to open repair for the treatment of complex aortic aneurysms. The ultimate goal of fenestrations is to increase the proximal sealing zone length. The Gore C3 delivery system allows repositioning of the graft by constraining the proximal part after a back-table physician modification; the PMEG technique with the Gore Excluder C3 delivery system can be used for complex aortic aneurysms repair as an alternative treatment option.
Assuntos
Aneurisma da Aorta Abdominal/cirurgia , Implante de Prótese Vascular/instrumentação , Prótese Vascular , Procedimentos Endovasculares/instrumentação , Desenho de Prótese , Aneurisma da Aorta Abdominal/diagnóstico por imagem , Humanos , Resultado do TratamentoRESUMO
INTRODUCTION: Endovascular therapy represents a less invasive alternative to open surgery for reconstruction of the portal vein (PV) and the spleno-mesenteric venous confluence to treat Portal hypertension. The objective of this study is to determine if the Model for End-Stage Liver Disease (MELD) score is a useful method to evaluate the risk of morbidity and mortality during endovascular approaches. MATERIAL AND METHODS: Patients that underwent endovascular reconstruction of the PV or spleno-mesenteric confluence were identified retrospectively. Data were collected from November 2011 to August 2016. The MELD score was calculated using international normalized ratio, serum billirubin and creatinine. Patients were grouped into moderate (≤ 15) and high (> 15) MELD. Associations of the MELD score on the postprocedural morbidity, mortality and vessels patency were assessed by two-sided Fisher's exact test. RESULTS: Seventeen patients were identified; MELD score distribution was: ≤ 15 in 10 patients (59%) and > 15 in 7 (41%). Even distribution of severe PV thrombosis was treated in both groups, performing predominately jugular access in the high MELD score group (OR 0.10; 95%; CI 0.014-0.89; p = 0.052) in contrast to a percutaneous transhepatic access in the moderate MELD score group. Analysis comparing moderate and high MELD scores was not able to demonstrate differences in mortality, morbidity or patency rates. CONCLUSION: MELD score did not prove to be a useful method to evaluate risk of morbidity and mortality; however a high score should not contraindicate endovascular approaches. In our experience a high technical success, good patency rates and low complication rates were observed.
Assuntos
Procedimentos Endovasculares , Hipertensão Portal/cirurgia , Oclusão Vascular Mesentérica/cirurgia , Veias Mesentéricas/cirurgia , Procedimentos de Cirurgia Plástica , Veia Porta/cirurgia , Veia Esplênica/cirurgia , Trombose Venosa/cirurgia , Adulto , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Feminino , Humanos , Hipertensão Portal/diagnóstico , Hipertensão Portal/mortalidade , Hipertensão Portal/fisiopatologia , Masculino , Oclusão Vascular Mesentérica/diagnóstico por imagem , Oclusão Vascular Mesentérica/mortalidade , Oclusão Vascular Mesentérica/fisiopatologia , Veias Mesentéricas/diagnóstico por imagem , Veias Mesentéricas/fisiopatologia , Pessoa de Meia-Idade , Pressão na Veia Porta , Veia Porta/diagnóstico por imagem , Veia Porta/fisiopatologia , Portografia , Procedimentos de Cirurgia Plástica/efeitos adversos , Procedimentos de Cirurgia Plástica/mortalidade , Estudos Retrospectivos , Fatores de Risco , Veia Esplênica/diagnóstico por imagem , Veia Esplênica/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Grau de Desobstrução Vascular , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/mortalidade , Trombose Venosa/fisiopatologiaRESUMO
Portal vein (PV) thrombosis (PVT) in the absence of liver disease or thrombophilia is rare. We report a 57-year-old male with a history of stage 3 chronic kidney disease who presented at the emergency department 18 months after abdominal surgery with progressive abdominal pain and distention. Computed tomography revealed PVT with multiple collaterals and moderate ascites. He had undergone partial gastrectomy and gastrojejunal anastomosis at an outside facility for gastrointestinal stromal tumors that caused an iatrogenic stenotic lesion in the PV. The patient underwent balloon angioplasty and endovascular deployment of an 8 mm × 100 mm Viabahn covered stent (W. L. Gore and Associates, Flagstaff, Arizona) in the extrahepatic PV via a transhepatic approach; the device allowed complete restoration of prograde portal flow with clinical improvement. At 6 months from the intervention, he remains symptom-free with normal liver function tests and patent endoprosthesis on antiplatelet therapy.
Assuntos
Angioplastia com Balão , Gastrectomia/efeitos adversos , Doença Iatrogênica , Veia Porta , Trombose Venosa/terapia , Angioplastia com Balão/instrumentação , Angiografia por Tomografia Computadorizada , Constrição Patológica , Humanos , Masculino , Pessoa de Meia-Idade , Flebografia/métodos , Veia Porta/diagnóstico por imagem , Veia Porta/fisiopatologia , Portografia/métodos , Desenho de Prótese , Stents , Fatores de Tempo , Resultado do Tratamento , Grau de Desobstrução Vascular , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/etiologia , Trombose Venosa/fisiopatologiaRESUMO
Introducción: El higroma quístico es una dilatación difusa de los conductos linfáticos. Puede diagnosticarse prenatalmente a través de una ecografía obstétrica. La incidencia aproximada es de 1/6,000 nacidos vivos y de 1/750 abortos espontáneos. Esta lesión puede presentarse a nivel cervical en la parte inferolateral del cuello, donde aparece con grandes cavidades únicas o multiloculares. En general, se producen por la falta de conexión de los vasos linfáticos con los sacos linfáticos yugulares, o de estos con el sistema de drenaje venoso. Caso clínico: Con el fin de enfatizar sobre la notificación de estas enfermedades y las opciones de tratamiento no quirúrgico, se presenta una paciente con higroma quístico cervical (cara lateral del cuello) con compromiso de la vía aérea y digestiva por la extensión del tumor. Se trató con etanol puro por medio de múltiples infiltraciones guiadas por ultrasonido. Conclusiones: Dependiendo de las características de la lesión, el tratamiento puede ser quirúrgico, farmacológico o mixto. Cuando la extensión es importante o se relaciona con órganos vitales, la mejor opción de tratamiento es, en primer lugar, reducir el tamaño de la lesión y el compromiso de los órganos contiguos. Esto se hace por medio de escleroterapia. Posteriormente, de ser necesario, se realiza cirugía.
Background: Cystic hygroma is a diffuse dilatation of the lymphatic system, which can be prenatally diagnosed by ultrasound. The incidence is 1/6,000 live births and 1/750 spontaneous abortions. This malformation can occur at the cervical level located in the inferior lateral part of the neck where it appears with large single or multilocular cavities. It is generally caused by a lack of connection with jugular lymphatic channels or with the venous drainage system lymph sacs. Case report: In order to emphasize these diseases and non-surgical treatment options, we present a patient with a cervical cystic hygroma that compromises the airway and digestive tract due to tumor extension and treatment with pure ethanol with clinical improvement. Conclusions: Depending on the characteristics of the lesion, treatment options are surgery, pharmacological or mixed. When the extension involves vital organs, the best option is to reduce the size of the lesion and the compromise of the adjacent organ. This is done by sclerotherapy and, if necessary, surgery.
RESUMO
BACKGROUND: Cystic hygroma is a diffuse dilatation of the lymphatic system, which can be prenatally diagnosed by ultrasound. The incidence is 1/6,000 live births and 1/750 spontaneous abortions. This malformation can occur at the cervical level located in the inferior lateral part of the neck where it appears with large single or multilocular cavities. It is generally caused by a lack of connection with jugular lymphatic channels or with the venous drainage system lymph sacs. CASE REPORT: In order to emphasize these diseases and non-surgical treatment options, we present a patient with a cervical cystic hygroma that compromises the airway and digestive tract due to tumor extension and treatment with pure ethanol with clinical improvement. CONCLUSIONS: Depending on the characteristics of the lesion, treatment options are surgery, pharmacological or mixed. When the extension involves vital organs, the best option is to reduce the size of the lesion and the compromise of the adjacent organ. This is done by sclerotherapy and, if necessary, surgery.
Assuntos
Embolização Terapêutica/métodos , Exenteração Pélvica/métodos , Neoplasias Pélvicas/terapia , Tumores Fibrosos Solitários/terapia , Doença Aguda , Biópsia por Agulha , Terapia Combinada , Seguimentos , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Neoplasias Pélvicas/diagnóstico , Cuidados Pré-Operatórios/métodos , Medição de Risco , Tumores Fibrosos Solitários/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Retenção Urinária/diagnóstico , Retenção Urinária/etiologiaRESUMO
BACKGROUND: Early hepatic artery thrombosis (HAT) is a potentially lethal complication after orthotopic liver transplantation (OLT) requiring immediate intervention. AIM: To report an infrequent cause of HAT after OLT and by itself a controversial clinical entity, the median arcuate ligament celiac artery compression. CASE REPORT: A 59-year-old female with hepatitis C virus-induced cirrhosis, Child B, MELD 15, underwent cadaveric-donor OLT with complete vena cava exclusion. Type 1 hepatic artery anatomy was found both in the donor and the recipient, the gastroduodenal artery was ligated. During the first eight postoperative days, clinical and analytical evolution was satisfactory and Doppler ultrasound showed no abnormalities. On the ninth postoperative day, the patient developed hypovolemic shock due to bleeding at the hepatic artery anastomosis, surgical reconstruction was performed. Postoperative color Doppler showed absent hepatic artery flow and an angiography suggested celiac artery compression. The patient was explored again the same day, liberating the celiac artery from the median arcuate ligament and performing thrombectomy and reconstruction of the hepatic artery anastomosis. The patient made a satisfactory recovery and color Doppler showed adequate flow in the hepatic artery. She is alive, free of biliary complications and enjoying a good quality of life 12 months after transplantation. CONCLUSION: Median arcuate ligament celiac artery compression is an infrequent anatomical variant that should be intentionally evaluated in the recipient at the time of arterial reconstruction in OLT and specifically be considered in early HAT to allow recognition and effective correction.