Cervical cystic lymphangioma in a patient with blue rubber bleb nevus syndrome: clinical case report and review of the literature.

We present the case of a 9-year-old boy diagnosed with blue rubber bleb nevus syndrome, who showed a very large left cervical cystic lymphangioma. He was previously subjected to various treatments for lesions in the intestinal tract including blood transfusions for anemia, sclerosis, enterotomies or resections. The tumor was resected without any complications and the anatomopathologic report confirmed this diagnosis. The blue cavernous hemangioma syndrome (or blue rubber bleb nevus syndrome) is a rare disease characterized by cavernous angiomas involving the skin and gastrointestinal tract. Several cases of cystic lymphangiomas associated with this syndrome have been published recently and lymphomatous differentiation has been identified in the cells of cutaneous lesions. Given their common embryological origin, we underscore the importance of bearing in mind that it is possible for different types of vascular malformations to coexist in the same patient.

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

Infragenicular bypass graft for limb salvage using polytetrafluoroethylene and distal vein cuff as the first alternative in patients without ipsilateral greater saphenous vein.

The objectives of this study were to evaluate the results of polytetrafluoroethylene infragenicular bypass grafts with a distal interposition vein cuff in patients with critical limb ischemia in the absence of ipsilateral greater saphenous vein. From January 1997 to June 2002, 58 consecutive below-knee bypass grafts with PTFE and distal interposition vein cuff were performed in 57 patients with a median age of 70.8 years. The distal anastomosis was located at the infragenicular popliteal artery in 18 cases and at tibial vessels in 40. Primary patency, secondary patency, and limb salvage were analyzed using the Kaplan-Meier method. During a median follow-up of 14.4 months (range, 1-50) 26 cases of graft occlusion and 19 major amputations were registered. The primary and secondary patency rates at 12, 24, and 36 months were 57%, 54%, and 47% and 61%, 58%, and 50%, respectively. Limb salvage rates reached 69%, 69%, and 59% at 12, 24, and 36 months. When below-knee revasculanzation is required in patients with limb-threatening ischemia, in the absence ipsilateral greater saphenous vein, PTFE grafts with a distal vein cuff are a reasonable substitute with acceptable long-term patency and limb salvage rates.