ABSTRACT
A 41-years-old woman with multiple arteriovenous lung malformations in a context of Rendu-Osler-Weber disease is described. The patient had a history of cutaneous and mucosal telangiectasia, frequent epistaxis and one episode of central artery embolism in the right retina. Malformations in the vascular territories of both lungs (right lower lobe and lingula) were detected by X-rays and magnetic resonance. Progressive dyspnea along with serious antecedents indicated that aggressive treatment was required. The malformations were embolized in the vascular radiology treatment center.
Subject(s)
Arteriovenous Malformations/therapy , Embolization, Therapeutic , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Telangiectasia, Hereditary Hemorrhagic/therapy , Adult , Arteriovenous Malformations/diagnosis , Embolization, Therapeutic/methods , Female , Humans , Pulmonary Artery/diagnostic imaging , Pulmonary Veins/diagnostic imaging , Radiography , Telangiectasia, Hereditary Hemorrhagic/diagnosisSubject(s)
Bronchial Hyperreactivity/complications , Bronchitis/complications , Tracheal Diseases/diagnostic imaging , Adrenal Cortex Hormones/therapeutic use , Bronchial Hyperreactivity/drug therapy , Bronchitis/drug therapy , Bronchodilator Agents/therapeutic use , Chronic Disease , Humans , Male , Middle Aged , Tomography, X-Ray Computed , Tracheal Diseases/etiologyABSTRACT
Pulmonary lymphangioleiomyomatosis (PLLM) is characterized by a benign proliferation of the smooth muscle around lymphatics, venulas and bronchiolus. It is usually fatal within 1 to 9 years. We present a case which remained with scarce symptoms despite receiving no treatment after at least 9 years of evolution of the disease. We highlight the clinical manifestations of the disease and the relevance of pulmonary biopsia with thoracotomy in order to establish the diagnosis.