E-ISSN 2534-9821
 

Case Report 


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

Altaf Hossain, Kelsie O Pitsenbarger, Lorelle S Parker, Emran W Altaf.

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 haemorrhage due to the rupture of malformed leg vessels has been reported as a cause of death. We present a case of KTS where an adult Caucasian male 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 an 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, and cerebral swelling. Microscopic finding of the lungs includes thickened pulmonary interstitium with congestion and oedema; Pulmonary arterioles showed hypertrophied muscular layers with numerous thrombi of different ages, ranging from recent to remote with the 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 artery recanalisation.

Key words: Klippel Trenaunay Syndrome, KTS, Autopsy, Pulmonary embolism, bone and soft tissue hypertrophy


 
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How to Cite this Article
Pubmed Style

Hossain A, Pitsenbarger KO, Parker LS, Altaf EW. Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. Int J Med Rev Case Rep. 2022; 6(14): 16-18. doi:10.5455/IJMRCR.172-1649095276


Web Style

Hossain A, Pitsenbarger KO, Parker LS, Altaf EW. Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. https://www.mdpub.net/?mno=9922 [Access: November 19, 2022]. doi:10.5455/IJMRCR.172-1649095276


AMA (American Medical Association) Style

Hossain A, Pitsenbarger KO, Parker LS, Altaf EW. Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. Int J Med Rev Case Rep. 2022; 6(14): 16-18. doi:10.5455/IJMRCR.172-1649095276



Vancouver/ICMJE Style

Hossain A, Pitsenbarger KO, Parker LS, Altaf EW. Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. Int J Med Rev Case Rep. (2022), [cited November 19, 2022]; 6(14): 16-18. doi:10.5455/IJMRCR.172-1649095276



Harvard Style

Hossain, A., Pitsenbarger, . K. O., Parker, . L. S. & Altaf, . E. W. (2022) Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. Int J Med Rev Case Rep, 6 (14), 16-18. doi:10.5455/IJMRCR.172-1649095276



Turabian Style

Hossain, Altaf, Kelsie O Pitsenbarger, Lorelle S Parker, and Emran W Altaf. 2022. Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. International Journal of Medical Reviews and Case Reports, 6 (14), 16-18. doi:10.5455/IJMRCR.172-1649095276



Chicago Style

Hossain, Altaf, Kelsie O Pitsenbarger, Lorelle S Parker, and Emran W Altaf. "Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.." International Journal of Medical Reviews and Case Reports 6 (2022), 16-18. doi:10.5455/IJMRCR.172-1649095276



MLA (The Modern Language Association) Style

Hossain, Altaf, Kelsie O Pitsenbarger, Lorelle S Parker, and Emran W Altaf. "Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.." International Journal of Medical Reviews and Case Reports 6.14 (2022), 16-18. Print. doi:10.5455/IJMRCR.172-1649095276



APA (American Psychological Association) Style

Hossain, A., Pitsenbarger, . K. O., Parker, . L. S. & Altaf, . E. W. (2022) Death due to Pulmonary Embolism and Pulmonary Hypertension in Klippel Trenaunay Syndrome.. International Journal of Medical Reviews and Case Reports, 6 (14), 16-18. doi:10.5455/IJMRCR.172-1649095276