Surgical and non-surgical management of thoracic and cervical paraganglioma.

Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal gland. We describe our current protocol as a multidisciplinary team for the management of cervical and thoracic PGLs. Surgery is generally considered the treatment of choice as it offers the best chance for cure. For resection of thoracic PGLs, video-assisted thoracoscopic surgery (VATS) is the main surgical approach, while open thoracotomy is preferred in case of tumors > 6cm, lacking confirmation of a plane of separation with adjacent structures, or with technical difficulties during VATS. In cervical PGLs, the surgical approach should be individualized according to location, mainly based on the Glasscock-Jackson and the Fisch-Mattox classifications. Surgery is the treatment of choice for most cervical and thoracic PGLs, but radiotherapy or observation could be more suitable options in unresectable cervical and thoracic PGLs or when resection has been incomplete.

High serum copeptin may be a marker of an increased carotid intima-media thickness in asymptomatic patients with type 1 diabetes.

We aimed to study the association of copeptin with carotid intima-media thickness in 60 patients with type 1 diabetes (T1DM-patients). Our results suggest that copeptin might improve the stratification of cardiovascular risk in T1DM-patients. Further research is needed to determine the value in identifying carotid disease of this biochemical marker.

Fasting serum copeptin and asymptomatic peripheral artery disease: No association in patients with type 1 diabetes mellitus.

OBJECTIVE: As copeptin is associated with lower-extremity amputation in patients with type 1 diabetes mellitus (T1DM), our study aimed to address the putative association between copeptin and asymptomatic peripheral artery disease (aPAD) in those patients. DESIGN AND METHODS: This observational cross-sectional study included 112 patients with T1DM from a larger cohort (ClinicalTrials.gov: NCT02910271), selected (1:2) as per the presence of aPAD (n = 37) or not (n = 75). aPAD was evaluated by ankle-brachial index (ABI), toe-brachial index (TBI), and peripheral Doppler ultrasound. The two groups of patients were matched by age, gender distribution and duration of T1DM. Fasting serum copeptin was measured by high-sensitivity ELISA, and its relationships with clinical and biochemical variables as well as aPAD were evaluated too. RESULTS: The study population was aged 42 ± 8 years, duration of T1DM was 27 ± 7 years, and mean HbA1c was 7.7 ± 1.1%. No significant differences in copeptin concentrations were found between patients with or without aPAD (16.9 ± 10.8 vs 17.3 ± 14.7 pmol/L, respectively; P = 0.462). Considering all patients as a whole, copeptin correlated with systolic blood pressure (SBP; ρ = -0.209, P = 0.027), eGFR ρ = -0.271, P = 0.004), and serum sodium (ρ = -0.208, P = 0.027), but not with ABI (ρ = -0.068, P = 0.476). Stepwise multiple linear regression analysis (R2: 0.059; P = 0.035) retained SBP (ß: -0.219, 95% CI: -1.391; -0.089) as the only significant predictor of copeptin concentration. CONCLUSION: As serum copeptin does not appear to be associated with aPAD in patients with T1DM, further studies are now needed to elucidate whether it has any other potential role to play in the subclinical vascular disease of this patient population.

Association of Cardiovascular Autonomic Dysfunction With Peripheral Arterial Stiffness in Patients With Type 1 Diabetes.

CONTEXT: Cardiovascular autonomic neuropathy (CAN) appears to contribute to peripheral arterial stiffness (AS) in type 1 diabetes. Whether CAN in patients with AS is associated with concomitant asymptomatic peripheral arterial disease (aPAD) remains unclear. OBJECTIVE: To assess the risk of CAN in patients with type 1 diabetes and AS and its potential association with atherosclerosis. DESIGN: Cross-sectional study. SETTING: Type 1 diabetes clinic in an academic hospital. PATIENTS: Two hundred sixty-four patients with type 1 diabetes. INTERVENTION: AS was defined as an ankle-brachial index (ABI) >1.2, aPAD by the toe-brachial index and Doppler sonography, and CAN by blood pressure and heart rate responses to active standing and Ewing and Clarke tests. MAIN OUTCOME MEASURES: Odds of having CAN among patients with AS. Odds for CAN were also calculated as a function of the presence of AS and concomitant aPAD. RESULTS: The study population's mean age was 35 ± 11 years, with a duration of disease of 19 ± 10 years and mean hemoglobin A1c of 7.5% ± 1.3%. Seventy-three patients (28%) had peripheral AS, of whom 28 showed aPAD. The prevalence of CAN among patients with AS was 48% but it was only 23% in subjects with normal ABI (OR: 3.1 [1.7; 5.4]). Concomitant aPAD increased the OR for CAN (OR: 4.5 [2.0; 10.1]). After adjustments for aPAD and relevant cardiovascular risk factors, AS remained associated with parasympathetic dysfunction. CONCLUSIONS: In type 1 diabetes, both peripheral AS and atherosclerosis were associated with CAN. A simple method, such as the ABI, may identify a subset of patients with undiagnosed dysautonomia.

The peripheral atherosclerotic profile in patients with type 1 diabetes warrants a thorough vascular assessment of asymptomatic patients.

AIMS: Epidemiological data on subclinical atherosclerotic disease in type 1 diabetes mellitus (DM1) are scarce. We aimed to estimate the subclinical atherosclerosis profile of asymptomatic patients with DM1 and an abnormal ankle-brachial index (ABI). MATERIAL AND METHODS: In a cross-sectional design (ClinicalTrials.gov Identifier: NCT02910271), we estimated ABI in 289 consecutive asymptomatic patients with DM1. An abnormal ABI led to measurements of toe-brachial index (TBI) and peripheral doppler ultrasound (DUS) to diagnose peripheral artery disease (PAD) and/or atherosclerotic carotid plaques (ACP). RESULTS: A reduced (≤0.9) or increased (>1.2) ABI was detected in 17 (6%) and 75 (26%) patients, respectively. PAD was confirmed by TBI and DUS in 9 (53%) patients with a reduced ABI and 28 (37%) patients with an increased ABI, resulting in a 12.8% (9.4-17.2) prevalence of asymptomatic PAD. Fourteen patients with an abnormal ABI also exhibited ACP [4.8% (2.9-7.9)], with 64% of these patients showing bilateral disease. Artery stenosis was mild or moderate in 21% and 29% of patients, respectively. Thus, 46 [16% (12-21)] patients showed asymptomatic PAD, ACP, or both. According to our data, we would have to explore three asymptomatic patients with DM1 and normal pulses to unmask one case of PAD, and seven asymptomatic patients showing abnormal ABI values to detect one carotid disease. CONCLUSIONS: Peripheral artery disease is often undiagnosed in asymptomatic patients with DM1. However, its presence may change medical management in a substantial percentage of cases, highlighting the potential benefit of a thorough vascular assessment on these patients.

Enfermedad de Ménétrier y edemas bilaterales en un paciente joven / Menetrier’s disease and bilateral oedemas in a young patient

Introducción. La enfermedad de Ménétrier es una gastropatía hiperplásica de aparición excepcional, que se caracteriza por un engrosamiento marcado de los pliegues gástricos, a expensas fundamentalmente de una hiperplasia foveolar. La presentación clínica más frecuente es dolor epigástrico, náuseas o vómitos. Se ha descrito una elevada prevalencia de infección por Helicobacter pylori (90%), y se ha comprobado una mejoría clínica, analítica e histológica tras la erradicación de éste. Caso clínico. Varón de 45 años que consultó por edemas de dos meses de evolución en los miembros inferiores. La ecografía Doppler de los miembros inferiores y la linfogammagrafía isotópica fueron normales. En la analítica se observó una marcada hipoproteinemia, y entre los estudios adicionales destacaba una serología positiva para H. pylori. Se le realizó un tránsito esofagogastroduodenal donde se observó un engrosamiento marcado de los pliegues gástricos, que se confirmó con gastroscopia y biopsia. El examen anatomopatológico objetivó la existencia de una gastritis crónica mixta con hiperplasia foveolar focal, junto con la presencia de H. pylori. Se instauró un tratamiento erradicador, y el paciente mostró una mejoría significativa de los edemas y una normalización de la cifra de proteínas. Conclusión. Presentamos un caso de enfermedad de Ménétrier, cuya principal manifestación clínica –a diferencia de lo habitual– son los edemas periféricos. Cabe reseñar la importancia de incluir las gastroenteropatías pierdeproteínas dentro del diagnóstico diferencial de edemas periféricos sin causa vascular

Introduction. Ménétrier’s disease is rare hyperplastic gastropathy that is characterised by a notable thickening of the gastric folds, mainly due to foveolar hyperplasia. The most frequent clinical presentation is epigastric pain, nausea or vomiting. A high rate of prevalence of infection by Helicobacter pylori (90%) has been reported and clinical, analytical and histological improvement is observed once this has been eradicated. Case report. A 45-year-old male who visited due to a two-month history of oedemas in the lower limbs. Results of Doppler ultrasound scans of the lower limbs and isotopic lymphoscintigraphy imaging were normal. Lab tests revealed a notable hypoproteinemia and one of the most significant findings in the additional studies was positive serology for H. pylori. The upper gastrointestinal series that was performed revealed a notable thickening of the gastric folds, which was confirmed by means of gastroscopic and biopsy tests. A pathological examination revealed the existence of a chronic non-specific gastritis with focal foveolar hyperplasia, together with the presence of H. pylori. Treatment was established to eradicate the infection and the patient showed significant improvement with regard to the oedemas and normalisation of the protein count. Conclusion. We report a case of Ménétrier’s disease in which, uncharacteristically, the main clinical feature is the presence of peripheral oedemas. It is important to include protein-losing gastroenteropathies within the differential diagnosis of peripheral oedemas that have no vascular causation

Falso aneurisma arterial y Salmonella paratyphi / Arterial pseudoaneurysm and Salmonella paratyphi

Introducción. La etiología infecciosa es una causa frecuente de formación de pseudoaneurismas arteriales. Clásicamente se ha relacionado la bacteriemia por Salmonella con colonización endovascular. Caso clínico. Varón de 77 años, hipertenso, obeso, ex fumador y bebedor habitual, que acudió al servicio de urgencias por dolor, edema y empastamiento del miembro inferior derecho, con mala perfusión distal. El estudio de eco-Doppler confirmó una trombosis en la vena femoral común derecha, por lo que ingresó con la sospecha diagnóstica de flegmasia cerulea dolens. Comenzó entonces con la clínica del síndrome compartimental y picos febriles. Se le realizaron fasciotomías en los compartimentos anterior y lateral de la pierna derecha. En la tomografía axial computarizada (TAC) se observaron abscesos en el músculo psoas derecho. En los hemocultivos crecieron colonias de Salmonella paratyphi, por lo que se realizó una nueva TAC con contraste intravenoso para descartar una infección endovascular. Se visualizó una imagen nueva compatible con un pseudoaneurisma de la arteria ilíaca derecha. Se decidió la intervención quirúrgica urgente para la reparación vascular y el drenaje de los abscesos, por lo que se indicó previamente la colocación de un filtro de cava. Durante la colocación de éste, el paciente sufrió una parada cardiorrespiratoria y el posterior fallecimiento. Conclusión. En la necropsia se halló una perforación puntiforme en el ápex y el hemopericardio como probable origen del fallecimiento (AU)

INTRODUCTION. Arterial pseudoaneurysms are often produced as a result of infectious processes. Bacteraemia due to Salmonella has traditionally been related to endovascular colonisation. CASE REPORT. 77-year-old male, who was hypertensive, obese, previously a frequent smoker and drinker, and who visited the emergency department because of pain, oedema and investment of the right lower limb, with poor distal perfusion. A Doppler ultrasound scan confirmed the presence of thrombosis in the right common femoral vein, and was therefore admitted to hospital with a suspected diagnosis of phlegmasia cerulea dolens. The patient then started with a clinical picture of compartment syndrome and bouts of fever. Fasciotomies were performed in the anterior and lateral compartments of the right leg. Computerised axial tomography (CAT) scans revealed the presence of abscesses in the right psoas muscle. Colonies of Salmonella paratyphi grew in the blood cultures, so a second CAT scan was performed with intravenous contrast to preclude an endovascular infection. A new image was seen that was compatible with pseudoaneurysm of the right iliac artery. An urgent surgical intervention was performed to carry out vascular repair and to drain the abscesses, which required the prior placement of a vena cava filter. While this was being placed, the patient suffered a cardiorespiratory arrest and later died. CONCLUSIONS. The post-mortem examination revealed a pinhole perforation in the apex and haemopericardium as the probable cause of death (AU)

Evaluación de estenosis carotídea en pacientes subsidiarios de revascularización miocárdica / The evaluation of carotid stenosis in patients who can benefit from myocardial revascularisation

Introducción. La estenosis carotídea (EC) crítica es frecuente en pacientes pendientes de revascularización miocárdica (RM), aumenta el riesgo de ictus durante la cirugía y su diagnóstico es importante, aunque el tratamiento sea controvertido cuando coexisten ambas patologías. Objetivos. Estudiar la lesión carotídea en pacientes pendientes de RM y los factores asociados. Determinar la morbimortalidad de la cirugía combinada carotídea y la RM frente a cirugías separadas. Pacientes y métodos. Estudio prospectivo, observacional y consecutivo durante 16 meses; se realiza dúplex carotídeo en 140 candidatos a RM, y consideramos la EC crítica: lesión carotídea ≥ 70%, con un 72,9% varones y una edad media de 69,8 años. Realizamos análisis estadístico con SPSS 10.1, chi al cuadrado y test exacto de Fisher: p £ 0,05 es significativamente estadística. Resultados. Prevalencia de lesión carotídea: 16,5% EC ≥ 50%, 10% EC ≥ 70%, pero sólo 7,1% quirúrgicas, ya que cuatro casos fueron oclusión carotídea. 42,6% angor inestable, 5% fracción de eyección del ventrículo izquierdo < 30%. Encontramos asociación significativa entre EC crítica en este tipo de pacientes con las siguientes variables: enfermedad cerebrovascular en el 37,5% de los casos (p = 0,009); lesión de tronco coronario izquierdo (TCI) ≥ 50% en el 22,2% (p = 0,002) y no padecer diabetes (p = 0,028). Practicamos cinco cirugías secuenciales: endarterectomía carotídea (EAC) seguida de RM cuatro cirugías combinadas y una RM emergente sin EAC. La morbimortalidad de la RM en pacientes con EC < 70% fue: 0,61% mortalidad, 0,69% morbilidad –seis infartos agudos de miocardio (IAM) y tres ictus–. En EC quirúrgicas hubo un exitus, un IAM. Conclusiones. Consideramos indicación primaria el estudio con dúplex carotídeo en estos pacientes; es especialmente importante cuando existe lesión del TCI significativa (≥ 50%) y enfermedad cerebrovascular. Proponemos cirugía combinada en pacientes cardiológicamente inestables y es recomendable en lesión del TCI ³ 50%, aunque sean necesarios ensayos clínicos que mejoren la indicación

Introduction. Critical carotid stenosis (CS) is frequent among patients who are waiting for myocardial revascularisation (MR), it increases the risk of stroke during the operation and it is important to diagnose it, although when the two pathologies coexist there is some controversy about treatment. Aims. To study carotid lesions in patients waiting for MR and the associated factors. We also intended to determine the morbidity and mortality rates of combined carotid and MR surgery versus separate procedures. Patients and methods. The study was prospective, observational and consecutive over a period of 16 months; carotid duplex was performed in 140 candidates for MR and the CS was considered to be critical: carotid lesion ≥ 70%, with 72.9% males and a mean age of 69.8 years. Statistical analyses were performed with SPSS 10.1, chi squared and Fisher’s exact test. p £ 0.05 is statistically significant. Results. Prevalence rate of carotid lesions: 16.5% CS ≥ 50%, 10% CS ≥ 70%, but only 7.1% surgical, since four were cases of carotid occlusion. 42.6% unstable angina, 5% severe left ventricle ejection fraction < 30%. We found a significant association between critical CS in this kind of patients and the following variables: cerebrovascular disease in 37.5% of cases (p = 0.009); left coronary artery trunk (LCT) lesion ≥ 50% in 22.2% (p = 0.002) and not suffering from diabetes (p = 0.028). Five sequential surgical procedures were performed: carotid endarterectomy (CE) followed by MR four combined interventions and one emerging MR without CE. MR morbidity and mortality rates in patients with CS < 70% were: 0.61% mortality, 0.69% morbidity –six acute myocardial infarctions (AMI) and three strokes– and with surgical CS there was one death and one AMI. Conclusions. We consider the use of carotid duplex as a primary indication in the study of these patients; it is especially important when there is significant injury to the LCT (≥ 50%) and cerebrovascular disease. We propose combined surgery in cardiologically unstable patients and it is recommendable in LCT lesion ³ 50%, although further clinical trials are needed to improve the indication