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ISSN : IJCMI Volume 1 • Issue 1 • 1000101 January, 2014 Clinical case blog http://dx.doi.org/10.4172/ijcmi.1000101 International Journal of Clinical & Medical Imaging Title : Diagnose the Varicosity - Klippel Trenaunay Weber Syndrome Rushabh H. Doshi and Truptesh H. Kothari Whitney High School, Los Angeles, CA Fox Chase Cancer Center, Philadelphia, PA *Corresponding author: Truptesh H. Kothari, 8048 Oxford Avenue, B18, Philadelphia PA, 19111, USA, Tel: 215-214-1460; E-Mail: [email protected] Introduction e Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by Port-wine stain, Soſt tissue, and vascular anomalies such as varicose veins [1]. Many people with the Klippel-Trènaunay-Weber have an enlarged leg, skin ulcers, infections and other skin problems, but usually the treatment is conservative [2,3]. Discussion In 1900, noted French physicians Klippel and Trenaunay termed “naevus vasculosus osteohypertrophicus.” In 1907, Parkes Weber, unaware of Klippel and Trenaunay’s report, described a patient with the aforementioned symptoms as well as an arterio- venous malformation of the affected extremity, which he termed as “hemangiectatic hypertrophy” [4]. Some patients with KTWS have epidural hemangioma and cerebral or spinal cord arteriovenous fistulas. ere have been a few reports regarding a rupture of the epidural hemangioma resulting in progressive paraplegia. When epidural block is scheduled as part of treatment for patients with KTWS, CT scan should be performed to investigate abnormal vessels in the lumbar spinal canal [5]. Treatment Laser treatment (pulsed dye laser) for cosmetic improvement of head and neck cutaneous lesions Vascular interventions for AV Malformation Sclerotherapy, surgical stripping, phlebectomy; subfascial endoscopic ligation of perforating veins, endovenous thermal abla- tion or (rarely) deep venous reconstruction Gastrointestinal bleeding Argon plasma coagulation, Selective arterial embolization, Colectomy etc Copyright: © 2014 Doshi RH. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

inica case bo - OMICS International · The Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by Port-wine stain, Soft tissue, and vascular anomalies such as varicose veins

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Page 1: inica case bo - OMICS International · The Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by Port-wine stain, Soft tissue, and vascular anomalies such as varicose veins

ISSN : IJCMI

Volume 1 • Issue 1 • 1000101

January, 2014

Clinical case blog

http://dx.doi.org/10.4172/ijcmi.1000101

International Journal of Clinical & Medical Imaging

Title : Diagnose the Varicosity - Klippel Trenaunay Weber SyndromeRushabh H. Doshi and Truptesh H. KothariWhitney High School, Los Angeles, CAFox Chase Cancer Center, Philadelphia, PA

*Corresponding author: Truptesh H. Kothari, 8048 Oxford Avenue, B18, Philadelphia PA, 19111, USA, Tel: 215-214-1460; E-Mail: [email protected]

IntroductionThe Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by Port-wine stain, Soft tissue, and vascular anomalies

such as varicose veins [1]. Many people with the Klippel-Trènaunay-Weber have an enlarged leg, skin ulcers, infections and other skin problems, but usually the treatment is conservative [2,3].

DiscussionIn 1900, noted French physicians Klippel and Trenaunay termed “naevus vasculosus osteohypertrophicus.” In 1907, Parkes

Weber, unaware of Klippel and Trenaunay’s report, described a patient with the aforementioned symptoms as well as an arterio-venous malformation of the affected extremity, which he termed as “hemangiectatic hypertrophy” [4]. Some patients with KTWS have epidural hemangioma and cerebral or spinal cord arteriovenous fistulas. There have been a few reports regarding a rupture of the epidural hemangioma resulting in progressive paraplegia. When epidural block is scheduled as part of treatment for patients with KTWS, CT scan should be performed to investigate abnormal vessels in the lumbar spinal canal [5].

TreatmentLaser treatment (pulsed dye laser) for cosmetic improvement of head and neck cutaneous lesions

Vascular interventions for AV MalformationSclerotherapy, surgical stripping, phlebectomy; subfascial endoscopic ligation of perforating veins, endovenous thermal abla-

tion or (rarely) deep venous reconstruction

Gastrointestinal bleedingArgon plasma coagulation, Selective arterial embolization, Colectomy etc

Copyright: © 2014 Doshi RH. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Page 2: inica case bo - OMICS International · The Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by Port-wine stain, Soft tissue, and vascular anomalies such as varicose veins

Volume 1 • Issue 1 • IJCMI

*Corresponding author: Truptesh H. Kothari, 8048 Oxford Avenue, B18, Philadelphia PA, 19111, USA, Tel: 215-214-1460; E-Mail: [email protected]

• Page 2 of 2

Splenic haemangiomas Splenectomy may be considered for larger lesions.

Orthopedic interventionsLimb-length discrepancy may be treated with orthoses or orthopedic surgery, depending on its severity. De-bulking surgery

for grossly enlarged limbs is occasionally used but carries a significant risk of lymphatic and venous damage.Reference

1. Aelvoet GE, Jorens PG, Roelen LM (1992) Genetic aspects of the Klippel-Trenaunay syndrome. Br J Dermatol 126:603–607.

2. Biesecker LG, Happle R, Mulliken JB, Weksberg R, Graham JM, et al (1999) Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation. Am J med Genet 84:389–395.

3. Gloviczki P, Stanson AW, Stickler GB, Johnson CM, Toomey BJ, et al. (1991) Klippel-Trenaunay syndrome: the risks and benefits of vascular interventions. Surgery 110:469–479.

4. Camila K Jannige (2012) Klippel-Trenaunay-Weber Syndrome; Clinical Professor of Dermatology.

5. Yamada Y, Yamada K, Yamamoto K (2013) Epidural block for lower limb amputation in a patient with Klippel-Trenaunay-Weber syndrome; Kanazawa University Hospital 62: 213-236.

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Submit your manuscript at: http://www.omicsonline.org/submission *Corresponding author: Truptesh H. Kothari, 8048 Oxford Avenue, B18, Philadelphia PA, 19111, USA, Tel: 215-214-1460; E-Mail: [email protected]