title: Rare Association of Klippel-Trenaunay Syndrome with Large Pulmonary Embolism and Asymmetrical Emphysematous Bullae.
abstract: Klippel-Trenaunay syndrome is a rarely encountered congenital disease characterized by a triad of enlarged arteries and veins, limb hypertrophy and capillary malformations. We are presenting an interesting case of a 23-year male who had been previously diagnosed to have Klippel-Trenaunay syndrome. The patient presented with large pulmonary embolism after having undergone laser surgery for varicose veins. The diagnostic chest computed tomography (CT) performed also revealed the co-existence of severe destructive pulmonary parenchymal disease involving large areas of the pulmonary parenchyma and formation of large emphysematous bullae having an asymmetric involvement of the left lung field. The patient was managed with thrombolysis with a bolus fibrinolytic agent and subsequently underwent an inferior vena cava (IVC) filter implantation to prevent further episodes of embolism in the presence of a compromised lung. The association of Klippel-Trenaunay syndrome with recurrent pulmonary embolism and unexplained pulmonary parenchymal disease leading to almost complete destruction of large areas of lung fields is interesting and has never been previously described.
authors.affiliation: Department of Cardiology, Care Hospital, Surat, Gujarat, India.
authors.forename: Navin
authors.initials: N
authors.lastname: Agrawal
authors.affiliation: Department of Cardiology, Care Hospital, Surat, Gujarat, India.
authors.forename: Apurva
authors.initials: A
authors.lastname: Vasavada
mesh_terms: D005341:Fibrinolysin / Q000008:administration & dosage; D005343:Fibrinolytic Agents / Q000008:administration & dosage; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000175:diagnosis; D008297:Male; D011183:Postoperative Complications; D011655:Pulmonary Embolism / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000209:etiology*; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014648:Varicose Veins / Q000601:surgery*; D055815:Young Adult
chemical_list: D005343:Fibrinolytic Agents; D005341:Fibrinolysin