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.

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