Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.

Abstract

Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome. Klippel Trenaunay Syndrome (KTS) is a congenital disorder characterized by bony and soft tissue hypertrophy, capillary malformation, and varicosities (1). This syndrome has several complications such as, sepsis, hypercoagulability, rectal and bladder bleeding, venous thrombosis, and pulmonary embolism. Massive hemorrhage due to rupture of malformed leg vessels has been reported as a cause of death. We present a case of KTS where an adult Caucasian male who died suddenly at home. Clinically, the decedent had chronic pain and warmth in both legs, port wine stain, a longer and hypertrophied left leg, vascular malformation, big toes, and swollen soft tissues Autopsy revealed did not reveal cardiomegaly or intrusive coronary artery disease. A ropy, granular, organized, and formed anti-mortem blood clot is observed in the pulmonary trunk, its branches, and in the lungs. He had abnormal structure of the feet, as well as varicose veins and swelling of the legs. Grossly, the left calf circumference is greater than the right calf circumference, varicosity of the left lower extremity, bony and soft tissue hypertrophy, especially the lower left extremity, swollen and large toes, cerebral swelling. Microscopic finding of lungs includes thickened pulmonary interstitium with congestion and edema, Pulmonary arterioles showed hypertrophied muscular layers with numerous thrombi of different ages, ranging from recent to remote with complete organization of some vessels, Trichrome stains confirmed the presence of recanalization and some plexiform lesions. There is a thick muscular wall, organized thrombotic material, intimal fibrosis, and recanalization of the artery.