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Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal

INTRODUCTION: Klippel Trenaunay Syndrome (KTS) is a rare congenital malformation with capillary and venous malformations and soft tissue/bony overgrowth with or without lymphatic malformation. Cutaneous vascular stain, varicosities and tissue hypertrophy represent its main clinical features. Besides...

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Autores principales: Karmacharya, Robin Man, Vaidya, Satish, Bhatt, Swechha, Tamang, Ashish, Shrestha, Rohit Bhasink, Bhandari, Niroj, Paudel, Bijaya, Shah, Manish, Nepal, Gaurav
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9114462/
https://www.ncbi.nlm.nih.gov/pubmed/35600200
http://dx.doi.org/10.1016/j.amsu.2022.103732
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author Karmacharya, Robin Man
Vaidya, Satish
Bhatt, Swechha
Tamang, Ashish
Shrestha, Rohit Bhasink
Bhandari, Niroj
Paudel, Bijaya
Shah, Manish
Nepal, Gaurav
author_facet Karmacharya, Robin Man
Vaidya, Satish
Bhatt, Swechha
Tamang, Ashish
Shrestha, Rohit Bhasink
Bhandari, Niroj
Paudel, Bijaya
Shah, Manish
Nepal, Gaurav
author_sort Karmacharya, Robin Man
collection PubMed
description INTRODUCTION: Klippel Trenaunay Syndrome (KTS) is a rare congenital malformation with capillary and venous malformations and soft tissue/bony overgrowth with or without lymphatic malformation. Cutaneous vascular stain, varicosities and tissue hypertrophy represent its main clinical features. Besides, the patient can develop thromboembolic pathologies, recurrent bouts of infection, stasis eczema, limb length discrepancy and intolerable pain typical of intraosseous involvement. METHODS: Here, we report a case series of seven patients aged 10–45 who presented to our centre with clinical features suggestive of KTS. Out of them, six patients had involvement of unilateral lower limb, while only one had involvement of bilateral lower limb. They all had typical cutaneous vascular stains and underlying venous malformation, while one patient had developed complications with multiple ulcer formation. OUTCOMES: An interdisciplinary team of vascular surgeons, dermatologists, interventional radiologists, orthopaedics, and physiotherapists managed the cases. We performed an individualized treatment as per the patient's presentation, which included a combination of supportive, medical, interventional radiologic, and surgical interventions. The follow-up outcomes of all the patients revealed significant resolution of symptoms. CONCLUSION: Patients with KTS can have diverse presentations. Therefore, clinicians should ensure an individualized treatment with the involvement of a multidisciplinary team for proper management and prevention of complications.
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spelling pubmed-91144622022-05-19 Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal Karmacharya, Robin Man Vaidya, Satish Bhatt, Swechha Tamang, Ashish Shrestha, Rohit Bhasink Bhandari, Niroj Paudel, Bijaya Shah, Manish Nepal, Gaurav Ann Med Surg (Lond) Case Series INTRODUCTION: Klippel Trenaunay Syndrome (KTS) is a rare congenital malformation with capillary and venous malformations and soft tissue/bony overgrowth with or without lymphatic malformation. Cutaneous vascular stain, varicosities and tissue hypertrophy represent its main clinical features. Besides, the patient can develop thromboembolic pathologies, recurrent bouts of infection, stasis eczema, limb length discrepancy and intolerable pain typical of intraosseous involvement. METHODS: Here, we report a case series of seven patients aged 10–45 who presented to our centre with clinical features suggestive of KTS. Out of them, six patients had involvement of unilateral lower limb, while only one had involvement of bilateral lower limb. They all had typical cutaneous vascular stains and underlying venous malformation, while one patient had developed complications with multiple ulcer formation. OUTCOMES: An interdisciplinary team of vascular surgeons, dermatologists, interventional radiologists, orthopaedics, and physiotherapists managed the cases. We performed an individualized treatment as per the patient's presentation, which included a combination of supportive, medical, interventional radiologic, and surgical interventions. The follow-up outcomes of all the patients revealed significant resolution of symptoms. CONCLUSION: Patients with KTS can have diverse presentations. Therefore, clinicians should ensure an individualized treatment with the involvement of a multidisciplinary team for proper management and prevention of complications. Elsevier 2022-05-07 /pmc/articles/PMC9114462/ /pubmed/35600200 http://dx.doi.org/10.1016/j.amsu.2022.103732 Text en © 2022 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Series
Karmacharya, Robin Man
Vaidya, Satish
Bhatt, Swechha
Tamang, Ashish
Shrestha, Rohit Bhasink
Bhandari, Niroj
Paudel, Bijaya
Shah, Manish
Nepal, Gaurav
Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal
title Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal
title_full Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal
title_fullStr Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal
title_full_unstemmed Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal
title_short Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal
title_sort klippel-trenaunay syndrome: case series from a university hospital of nepal
topic Case Series
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9114462/
https://www.ncbi.nlm.nih.gov/pubmed/35600200
http://dx.doi.org/10.1016/j.amsu.2022.103732
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