ABSTRACT
El síndrome de Swyer-James o de MacLeod es una enfermedad infrecuente que asocia hipoperfusión pulmonar y atrapamiento aéreo con enfisema. En la edad adulta suele pasar desaparecido por su escasa sintomatología y puede diagnosticarse tras el hallazgo en una radiografía simple de tórax una imagen de hiperclaridad pulmonar unilateral. Presentamos un caso que destaca por su inhabitual forma de presentación clínica con hemoptisis masiva, no referida en la literaturamédica previamente según nuestra revisión, y que fue controlada con éxito mediante angiografía y embolización de los vasos anómalos. Después de cuatro años de seguimiento en la consulta externa no volvió a ocurrir de nuevo ningún episodio de hemoptisis y la paciente realiza su vida normal
Swyer-James or MacLeod syndrome is an infrequent disease associated with pulmonary hypoperfussion, air trapmentand emphysema. At adult age this disease can pass unnoticed because of its scarce symptoms but can be diagnosed in a plain chest radiograph showing a unilateral pulmonary hyperlucency. We present a case with an unusual clinic debut: massive hemoptysis. Such way of presentation was not refered previously in the medical literature according to our review.The patient was successful managed through angiography and embolization of the anomalous vessels. After four years of follow-up at the outpatient clinic no hemoptysis occurred again, and she can keep a normal activity life1
Subject(s)
Humans , Female , Adult , Hemoptysis/etiology , Lung, Hyperlucent/diagnosis , Lung, Hyperlucent/complications , Embolization, Therapeutic/methodsABSTRACT
OBJECTIVES: While the short-term results of lung volume reduction surgery are known, follow-up over several years has not often been described. The purpose of the present study was to describe results in terms of functional improvement, dyspnea, quality of life, and mortality over a 4-year period in patients with advanced emphysema. PATIENTS AND METHODS: Fourteen successive patients were enrolled between 1996 and 2000 and studied prospectively for 4 years. All patients served as their own controls and initially received pulmonary rehabilitation and medication. Preoperative data were used as baseline and were compared to postoperative data over 4 years. The data analyzed were: functional improvement (forced expiratory volume in 1 second [FEV1]), quality of life, dyspnea, and patient loss due to death or referral to a lung transplantation program. RESULTS: Patients with advanced emphysema (mean FEV1 [SD]: 22.8% [11%] of predicted) were studied. Postoperative mortality was 14%. Overall mortality (postoperative plus deaths due to respiratory insufficiency) was 28% at 1 year and 35% at 4 years. Two patients died of cancer and 5 were referred for transplantation. At 3 months, FEV1 had improved more than 15% in 9 patients (64%); the improvement was maintained in 43% of patients at 1 year and 7% at 4 years. Improvement in dyspnea paralleled improvement in FEV1. Overall, at 3 months mean FEV1 had improved 41.9% (68%), transitional dyspnea index 2.7 (3), and quality of life questionnaire score 1 (0.9). Thus, improvements were considerable, but there was great variation. Preoperative mean decrease in FEV1 was 50 (32) mL/y, and postoperative decrease 194 (70) mL/y. CONCLUSIONS: With the inclusion criteria used, there was considerable variation in the results. Significant overall functional improvement was maintained in 50% of the patients 1 year following surgery and in 7% 4 years after surgery. Given such results, together with a surgical mortality rate of 14% and overall mortality of 28% in the first year, we believe that the criteria for using lung reduction surgery should be revised.
