CLOVES Syndrome Diagnosis and Treatment in an Adult Patient.

CLOVES syndrome is a rare, nonheritable sporadic overgrowth disorder. In the world 130-200 cases have been reported. This is the first case of CLOVES described in Portugal, which had been not been diagnosed for the last 36 years. With this paper, the authors look to highlight the clinical features of this syndrome so that it does not go unrecognized in daily practice. The authors also underline the efficacy and safety of sirolimus, and that this treatment should not be denied, even in adult patients.

DEEP VEIN ULTRASOUND-GUIDED THROMBOLYSIS.

Catheter directed thrombolysis is a minimally invasive procedure that results in a significant reduction in venous obstruction after deep vein thrombosis. The technique implies the performance of phlebographies to monitor the thrombolysis progression. The objective of this paper is to describe the use of vascular ultrasound to follow the thrombus lysis and to adjust the catheter position according to the progression of the thrombolysis. This is the first case reported describing the application of ultrasound to monitor the thrombolysis. A 36-year woman was admitted ilio-femoral vein. The ipsilateral great saphenous vein was the percutaneous access to perform the anterograde thrombolysis. The procedure, including the control of thrombus lysis, as well as catheter progression was performed under ultrasound guidance. The thrombolysis took 52 hours. The patient has two years of follow-up without any complaint. The use of vascular ultrasound to monitor the thrombolysis has several theoretical advantages in patients with adequate biotopes. It minimizes the number of venograms and can be performed at the bedside. It is effective and inexpensive.

RECURRENT INGUINAL LYMPHOCELE - A THERAPEUTIC CHALLENGE.

Recurrent lymphocele after repair of crural hernia without resolution after several aspirations and injections of sclerosing agents. Reintervention with lymphatic marking (injection of patent blue dye at the interdigital level, Figure 1 a) Lymphatic marking through injection of patent blue dye at the interdigital level. b) Surgical approach of the inguinal region. Figure 1), followed by en bloc removal of the ganglia of the saphenofemoral junction and the lymphocele capsule (Figure 2). No evidence of recurrence at 24 months of follow- up.

Endometriosis Unusual Aetiology Of Inguinal Swelling.

A 42 years-old female presented with right inguinal swelling with one year of evolution. Magnetic resonance imaging was suggestive of inguinal endometriosis adherent to femoral vessels. Due to the rarity of this pathology (prevalence 0.3-0.6%), clinical suspicion is essential. Surgical excision is the treatment of choice.

Differences In Anthropometric Measures Between Critical Limb Threatening Ischaemia And Intermittent Claudication In Patients Undergoing Aorto Bifemoral Bypass.

OBJECTIVE/BACKGROUND: Peripheral artery disease (PAD) is an important manifestation of systemic atherosclerosis. Obesity is a risk factor for atherosclerosis and for cardiovascular events. However, the relationship between obesity and PAD is unclear. We hypothesized that anthropometric measures of adiposity, in particularly of central obesity will be associated with PAD severity, in patients undergoing aorto-bifemoral bypass. METHODS: A prospective observation study was conducted. From 2009 and 2012 a total of 46 males who underwent aorto-bifemoral bypass were enrolled prospectively. 17 with intermittent claudication (IC) and 29 with chronic limb threatening ischemia (CLTI). They were followed for 5 years. The anthropometric measures, weight, body mass index (BMI), waist circumference (WC), waist-to-hip ratio (WHR) and the seric levels of hemoglobin, triglycerides, and albumin were recorded. The mortality and cardiovascular events in following five years were also registered. RESULTS: The groups did not differ in the mean age (IC 60.69±7.46 versus CLTI 64.51 ±8.42 years, p=0.712), diabetes (IC 18% versus CLTI 45%, p=0.06), hypertension (IC 70% versus CLTI 52%, p=0.21), hypercholesterolemia (IC 18% versus CLTI 45%, p=0.47) and smoking habits prevalence (IC 100% versus CLTI 86%, p=0.11). The anthropometric measures: weight, WC and WHR were significant lower in CLTI compared to IC patients (IC 72.74±9.84 Kg versus CLTI 65.92±10.89 Kg, p=0.043; IC 98.65±8.19 cm versus CLTI 89.38±15.91 cm, p=0.017; IC 1.06±0.06 versus CLTI 1.01±0.06, p=0.038). The serum levels of hemoglobin, albumin and triglycerides were also lower in CLTI patients (IC 14,40±1.63g/dL versus CLTI 13.3±1.89g/dL, p=0.048; IC 4.6±0.81g/dL versus CLTI 4.3± 0.67g/dL, p=0.007; IC 212±95.60mg/Dl versus CLTI 111±41.53 mg/dL, p=0.001). No relation was found between the anthropometric measures at admission and the cardiovascular events or mortality at five years. CONCLUSION: CLTI patients had lower anthropometric measures of obesity, when compared to IC patients. These results could be explained by the fact that CLTI patients with severe atherosclerotic disease are in a state of chronic inflammation, with consequent cardiometabolic demands and catabolism.

Lower Extremity Fibro-Adipose Vascular Anomaly-Case Report.

Fibro-adipose vascular anomaly (FAVA) is a painful complex vascular malformation, characterized by muscle fibrofatty infiltration, usually in lower limb, associated with contracture of the ipsilateral extremity. This article describes the first case of FAVA reported in Portugal successfully treated with surgery. A 9-year-old female was admitted complaining of a painful mass in the right leg. The MRI scan showed the presence of a 5 × 4 × 4 cm mass in the right leg consistent with FAVA located in the peroneus longus muscle. The patient underwent resection of the involved muscle. She had 6 months of follow-up without any relevant clinical event. The authors also highlight the difference between FAVA and venous malformation. Early surgery is a treatment option that can prevent long-term consequences, as contracture and movement limitation.

Neointimal Hyperplasia.

Neointimal hyperplasia is a physiologic healing response to injury to the blood vessel wall, involving all the three arterial layers and it occurs in the presence of internal (endovascular) or external (surgical) injury. It is a highly complex process involving several tissues (perivascular, vessel wall, and blood) and numerous cell lineages with multiple molecular signaling networks. So, there is a number of possible targets for inhibition of this process. There are known risk factors for Intimal Hyperplasia, such as diabetes, female gender, presence of systemic inflammation, type of arteries treated, types of surgical and endovascular materials, presence of turbulent flow and genetic status. The present paper discusses the pathophysiology of neointimal hyperplasia and the strategies to prevention and treatment of it.

[Endoanchor implantation for treatment of endoleak]. / Aplicação de Endo-âncoras para Tratamento de Endoleak.

The authors describe a clinical case of correction of a type 1A endoleak after EVAR using endo-anchors. An 85-year old female was referred due to an abdominal aortic aneurysm. The patient´s previous medical history included hypertension, dyslipidemia, renal insufficiency and multiple abdominal surgeries (appendicectomy, classic cholecystectomy and hysterectomy). The aneurysm had 7.5 cm diameter with an angulated short 14 mm neck extension. She underwent an uneventful EVAR with placement of a Medtronic® Endurant II stentgraft. One month after the procedure the abdominal computed tomographic angiography (CTA) revealed a type 1A endoleak. Correction of endoleak consisted of left renal artery stenting (the lowest one), placement of a proximal aortic cuff and 9 endo-anchors (APTUS® System) with satisfactory end result. The control CTA after re-intervention showed patency of the renal arteries and the stentgraft, aneurysm exclusion and absence of endoleaks.

Os autores descrevem um caso clínico de corecção de endoleak tipo 1A após EVAR com recurso a endo-âncoras. Trata-se de uma doente de sexo feminino, de 85 anos, com antecedentes de hipertensão, dislipidemia, insuficiência renal crónica e múltiplas intervenções abdominais (apendicectomia, colecistectomia clássica e histerectomia). A doente foi observada em consulta externa por AAA com 7,5 cm, colo de 14 mm, angulado. Submetida a EVAR com colocação de endoprótese Medtronic® Endurant II sem intercorrências de relevo. Decorrido um mês após o procedimento a angiotomografia computorizada (ATC) abdomino-pélvica revelou endoleak tipo 1A. Programada a correção do endoleak, que consistiu em stenting da artéria renal esquerda (a mais baixa), colocação de cuff aórtico proximal e de 9 endo-âncoras (Sistema APTUS®), com resultado final satisfatório. A ATC de controlo após a reintervenção mostrou permeabilidade das artérias renais e da endoprótese, exclusão aneurismática e ausência de endoleaks.

[Prevalence of abdominal aortic aneurysm in patients with chronic obstructive pulmonary disease]. / Prevalência de aneurisma da aorta abdominal em indivíduos com doença pulmonar obstrutiva crónica.

INTRODUCTION: The association of the abdominal aortic aneurysm (AAA) to the chronic obstructive pulmonary disease (COPD) is questionable. Both entities share the same risk factors and physiopathology, which is based on a persistent pro- -inflammatory response, proteolysis and excessive extracellular matrix turnover. OBJECTIVES: To determine the AAA prevalence in a sample of patients with COPD. METHODS: Identification of patients with COPD and abdominal CT, hospitalized in a level 2 hospital between January 2015 and December 2016. RESULTS: In a sample with 100 patients, 5 presented an AAA. There were no significant differences between patients with and without AAA regarding gender (100% versus 69,5% male), age (74,80±6,22 versus 71,23±12,36 years), comorbidities (myocardial infarction 0% versus 1,1%; stroke 0% versus 10,5%), cardiovascular risk factors (diabetes mellitus 20% versus 23,2%; hypertension 80% versus 50,5%) and spirometric results. Patients with AAA were significantly more medicated with acetylsalicylic acid and had more aortic thrombi (P=0,031 and P=0,002, respectively). No correlation was found between the aortic size and the forced expiratory flow in one second values. DISCUSSION: The AAA prevalence of our sample was 5%, twice the described for the Portuguese population (2,37%). This is the first study conducted in Portugal to determine the AAA prevalence in COPD patients. CONCLUSION: The study of the relationship between both diseases could contribute to a more effective screening of AAA, by determining a group with increased risk, which could lead to a reduction of the mortality rates associated with the ruptured aneurysm.

Introdução: A associação entre aneurisma da aorta abdominal (AAA) e doença pulmonar obstrutiva crónica (DPOC) é discutível. Ambas as doenças partilham os mesmos fatores de risco e fisiopatologia, baseados numa resposta pro-inflamatória persistente, proteólise e turnover excessivo da matriz extracelular. Objetivos: Determinar a prevalência de AAA numa população com DPOC. Métodos: Identificaram-se os doentes com DPOC e que realizaram TC abdominal, internados num hospital de nível 2, entre janeiro de 2015 e dezembro de 2016. Resultados: Numa amostra de 100 doentes, 5 apresentavam AAA. Comparando o grupo com e sem AAA, verificou-se inexistência de diferenças significativas relativamente ao género (100% versus 69,5% sexo masculino), idade (74,80±6,22 versus 71,23±12,36 anos), comorbilidades (DAC 0% versus 1,1%; AVC 0% versus 10,5%), fatores de risco cardiovascular (diabetes mellitus 20 % versus 23,2%; hipertensão arterial 80% versus 50,5%) e resultados de espirometria. Verificaram-se diferenças significativas relativamente à medicação com ácido acetilsalicílico e presença de trombo aórtico (P=0,031 e P=0,002, respetivamente). Não se encontrou correlação entre os valores dos diâmetros aórticos e do FEV1. Discussão: Nesta amostra, a prevalência de AAA foi de 5%, duas vezes superior à da população portuguesa (2,37%). Este é o primeiro estudo realizado em Portugal para determinar a prevalência de AAA em DPOCs. Conclusão: O estudo da relação entre as duas patologias poderá contribuir para um rastreio mais eficaz do AAA, ao identificar um grupo de risco aumentado, o que poderá levar a uma diminuição da mortalidade associada ao aneurisma em rotura.

Ventricular lead dysfunction and subclavian vein stenosis: when problems come in pairs.

The widespread and increasing use of cardiac devices implantation naturally augments the number of device related complications. Lead failure is a known complication associated with increased morbidity, mortality and need for reintervention.Chronic stenosis or occlusions of coronary, cephalic or subclavian veins, especially at the site of previous device implantation,can limit lead passage through the target vein, imposing great technical difficulties, especially in patients needing follow-up procedures such as lead revisions and/or system upgrades. Venous balloon angioplasty can be a valuable and safe tool for successful implantation, helping to avoid more complex and hazardous procedures. The authors report the case of a 65 years old male presenting with a "Sprint Fidelis" defibrillator lead malfunction and severe asymptomatic left subclavian vein stenosis after cardiac resynchronization defibrillator therapy. The patient was submitted to a successful percutaneous balloon venoplasty in order to regain adequate venous access and enable reimplantation of the right ventricular lead.