RESUMEN
We report the case of a 53-year-old woman who presented with massive hemoptysis. Computed tomographic angiography revealed an anomalous vessel arising from the abdominal aorta, coursing anteriorly and through the diaphragm, and feeding the left upper lobe. At operation the vessel was found to anastomose to the left upper lobe lingula, which contained multiple vascular abnormalities and arteriovenous fistulas. The vessel was ligated, and the affected portion of the left upper lobe was resected. Anomalous systemic arterial supply of an upper lobe is an especially rare form of a Pryce type 1 abnormality. Recognition of these unusual anatomic variants is crucial to successful treatment and avoidance of adverse events.
Asunto(s)
Aorta Abdominal/anomalías , Angiografía por Tomografía Computarizada/métodos , Hemoptisis/etiología , Pulmón/irrigación sanguínea , Malformaciones Vasculares/diagnóstico por imagen , Aorta Abdominal/diagnóstico por imagen , Enfermedad Crítica , Diafragma/irrigación sanguínea , Servicio de Urgencia en Hospital , Femenino , Estudios de Seguimiento , Hemoptisis/diagnóstico , Humanos , Ligadura/métodos , Pulmón/cirugía , Persona de Mediana Edad , Neumonectomía/métodos , Enfermedades Raras , Resultado del Tratamiento , Malformaciones Vasculares/complicaciones , Malformaciones Vasculares/cirugíaRESUMEN
The efficacy of thymectomy and the optimal surgical technique in the treatment of myasthenia gravis (MG) remain controversial. Long-term outcomes are lacking and remission rates are based on small populations. We reviewed our institutional experience of thymectomy for MG focusing on long-term outcomes, complete stable remission (CSR), improvement of symptoms, after transcervical, transsternal, thoracotomy, and VATS thymectomy. A retrospective review of a prospectively maintained database of 3017 patients from 1941-2013 with MG was performed. Patients who underwent thymectomy with follow-up data including age at the time of surgery, sex, date of onset of symptoms, date of surgery, Osserman classification before and after surgery, surgical technique, date of remission, and status at last follow-up were included in the analysis. CSR and prognostic factors were analyzed by crude rate, Kaplan-Meier estimate, chi-squared test, Wilcoxon test, and a Cox proportional model. Overall, 1002 thymectomy patients with complete data were analyzed, and 35.5% (n = 355) derived benefit from surgery. Crude rate CSR was 19% (n = 191) and an additional 16% (n = 164) symptomatically improved requiring less medication after thymectomy. Also, 58% (n = 580) were stable after resection, and 6.7% (n = 67) developed progressive disease. Kaplan-Meier estimates of CSR were 27.7%, 36.7%, and 47.3% at 10, 25, and 40 years, respectively. On multivariate analysis, transsternal technique, thymoma, and preoperative Osserman classification were significantly associated with failure to achieve CSR. Thymectomy provides long-term CSR in 47.3% of patients with long-term follow-up. Patients with MG should be offered thymectomy when possible.