Abstract
Klippel-Trenaunay Weber Sendromu (KTWS) kutanöz
vasküler malformasyonlar, kemik ve yumuşak doku hipertrofisi ve varisli
damarlar ile karakterize konjenital bir sendromdur. KTWS'de hem periferik hem
de viseral tutulum görülebilir ve bu önemli bir morbidite ve mortalite kaynağı
olabilir. Pulmoner emboli, tromboflebit, kanama, staz dermatiti ve kutanöz
ülserasyon gibi komplikasyonların önlenmesinde erken tanı önemlidir.
Kliniğimize olası KTWS tanısı ile 9
günlük yenidoğan sunuldu. Çocuklukta bu hastalığın erken teşhisi önemlidir ve
venöz yetmezliği önlemek için uygun destek tedavisi başlatılır.
References
- 1- Darmstadt GL. Vasculer Disorders. In:Behrman RE, Kliegman RM, Arin AM, Nelson Textbook Of Pediatrics, Philadelphia: W:B Saunders Company, 2000:1978.
- 2- Whelan AJ, Watson MS, Porter FD, Steiner RD, Klippel Trenauy Weber Syndrome associated with a 5:11 balanced translocation. Am J Med Genet 1995;59:492
- 3- Hızarcıoğlu M, Gülez P, Bilger Ütük E, Kayserili E, Yener H, klippel trenaunay Weber Sendromu: Bir olgu sunumu ve literatür taraması . Türkiye klinileri pediyatri 2003;12: 93-95
- 4- Gloviczki P, Hollier LH, Telander RL, Kaufman B, Bianco AJ, Stickler GB. Surgical implications of Klippel-Trenaunay syndrome. Ann Surg 1983;197:353-62.
- 5- Mulliken J, Young A. Combined vascular malformations. In: Mulliken J, editor. Vascular birthmarks: hemangiomas and malformations. Philadelphia: Saunders; 1988; 159-72
- 6- Esterly NB. Diseases of the skin. In: Behrmann RE, Kliegman RM, Nelson WE, Vaughan VC, editors. Nelsontextbook of pediatrics. 20th ed. Philadelphia: W.B. Saunders Co; 2016;1621-85.
- 7- Karalezli A, Sevgili S, Ernam Turgut D, Hasanoğlu A, Hasanoğlu HC. Pulmonary embolism in a patient with Klippel-Trenaunay-Weber syndrome. Tuberk Toraks 2006;54:281-7.
Abstract
Klippel-Trenaunay Weber Syndrome (KTWS) is a
congenital syndrome characterized by cutaneous vascular malformations, bone and
soft tissue hypertrophy, and varicose veins. Both peripheral and visceral
involvement can be observed in KTWS and this can be a major source of morbidity
and mortality. Early diagnosis is important in preventing complications such as
pulmonary embolism, thrombophlebitis, bleeding, stasis dermatitis and cutaneous
ulcerations.Herein we presented a 9-day newborn with possible KTWS. That it is
important early diagnosis of this disease in childhood and appropriate
supportive treatment is started to prevent venous insufficiency.
References
- 1- Darmstadt GL. Vasculer Disorders. In:Behrman RE, Kliegman RM, Arin AM, Nelson Textbook Of Pediatrics, Philadelphia: W:B Saunders Company, 2000:1978.
- 2- Whelan AJ, Watson MS, Porter FD, Steiner RD, Klippel Trenauy Weber Syndrome associated with a 5:11 balanced translocation. Am J Med Genet 1995;59:492
- 3- Hızarcıoğlu M, Gülez P, Bilger Ütük E, Kayserili E, Yener H, klippel trenaunay Weber Sendromu: Bir olgu sunumu ve literatür taraması . Türkiye klinileri pediyatri 2003;12: 93-95
- 4- Gloviczki P, Hollier LH, Telander RL, Kaufman B, Bianco AJ, Stickler GB. Surgical implications of Klippel-Trenaunay syndrome. Ann Surg 1983;197:353-62.
- 5- Mulliken J, Young A. Combined vascular malformations. In: Mulliken J, editor. Vascular birthmarks: hemangiomas and malformations. Philadelphia: Saunders; 1988; 159-72
- 6- Esterly NB. Diseases of the skin. In: Behrmann RE, Kliegman RM, Nelson WE, Vaughan VC, editors. Nelsontextbook of pediatrics. 20th ed. Philadelphia: W.B. Saunders Co; 2016;1621-85.
- 7- Karalezli A, Sevgili S, Ernam Turgut D, Hasanoğlu A, Hasanoğlu HC. Pulmonary embolism in a patient with Klippel-Trenaunay-Weber syndrome. Tuberk Toraks 2006;54:281-7.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Health Care Administration |
| Journal Section | Makaleler |
| Authors | |
| Publication Date | March 28, 2018 |
| Submission Date | December 14, 2017 |
| Acceptance Date | March 27, 2018 |
| Published in Issue | Year 2018 Volume: 13 Issue: 1 |
