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[Gorham's disease. Report of 2 cases associated with bilateral pleural effusion]
A Padua y Gabriel1, L Peraza-Martínez, J Peralta-Sánchez
1Servicio de Neumología, Centro Médico Nacional, Torreón, Coahuila.
Abstract:
The clinicopathologic characteristics of two patients with Gorham's disease and bilateral recidivant pleural effusions are reported. The first case corresponded to a nine year-old girl with osteolysis in her right clavicle and ribs. The second case was a 55 year-old male with osteolytic lesions in ribs, cranium, pelvis and femurs; a thymic cyst was also documented. In both patients, biopsies were performed from ribs, which showed proliferating, benign, thin walled small vessels that extensively replaced the intertrabecular spaces. Thoracocentesis, instillation of talcum and radiation therapy were used in the former patient and no recidivant pleural effusions have developed after one year. The second patient was not treated with radiation therapy, persisted with pleural effusions and finally died two years after the diagnosis. Because Gorham's disease is rare, it was not immediately suspected in the cases described here. Its association with pleural effusion is even rarer and has been mentioned only occasionally in the literature. The utility of radiation therapy in Gorham's disease is commented.