Hypertension and multiple arterial changes in a child with Klippel Trenaunay syndrome

Abstract

Klippel-Trenaunay syndrome (KTS) is an inherited disorder which essentially affects the venous system. Arterial malformations accompanying venous pathologies are extremely rare. We report a 10-year-old boy of KTS patient presented with hypertension and renal atrophy who have also multiple arterial (renal, pulmonary and internal carotid) pathologic features. J Clin Exp Invest 2014; 5 (4): 610-613

Keywords

• Hypertension, arterial changes, Klippel-Trenaunay syndrome

Klippel-Trenaunay sendromlu bir çocukta hipertansiyon ve multipl arteriyel değişiklikler

Öz

Klippel-Trenaunay sendromu (KTS) özellikle venöz sistemi etkileyen kalıtsal bir hastalıktır. Venöz patolojilere eşlik eden arteriyel malformasyonlar oldukça nadirdir. Biz burada hipertansiyon ve renal atrofi nedeni ile başvuran, multipl arteriyel (renal, pulmoner ve internal karotid) patolojik özelliklere sahip KTS'lu 10 yaşında bir erkek hastayı sunduk.

Anahtar Kelimeler

• Hipertansiyon, arteriyel değişiklikler, Klippel-Trenaunay sendromu

References

1. Klippel M, Trenaunay P. Du noevus variquex osteohy- pertrophique. Arch Gen Med (Paris) 1900;185:641- 672.
2. Oduber CE, van der Horst CM, Hennekam RC. Klippel- Trenaunay syndrome diagnostic criteria and hypoth- esis on etiology. Ann Plast Surg 2008;60:217-223.
3. Jolobe OM. Klippel-Trenaunay syndrome. Postgrad Med J 1996;72:347-348.
4. Samuel M, Spitz L. Klippel-Trenaunay syndrome: clini- cal features, complications, and management in chil- dren. Br J Surg 1995;82:757-762.
5. Lee A, Driscoll D, Gloviczki P, et al. Evaluation and management of pain in patients with Klippel-Trenau- nay syndrome: a review. Pediatrics 2005;115:744- 749.
6. Uğurlucan M, Yerebakan C, Alpagut U, et al. Klippel- Trenaunay-Weber syndrome. Wien Med Wochenschr 2008;158:402-404.
7. Proesmans W, Van Damme B, Marchal G, et al. Klippel- Trenaunay syndrome with systemic hypertension and chronic renal failure. Ann Pediatr (Paris) 1982;29:671.
8. Berry SA, Peterson C, Mize W, et al. Klippel-Trenaunay syndrome. Am J Med Genet 1998;79:319-326.

9. Bartels C, Claeys L, Ktenidis K, et al. Weber syndrome associated with a brachial artery aneurysm. A case re- port. Angiology 1995;46:1039-1042.
10. Sharma S. Multifocal intradural spinal AVF and renal artery aneurysms in a case of Klippel Trenaunay Syn- drome (KTS). J Neuroimaging 2010;20:386-389.
11. Pourhassan S, Grotemeyer D, Klar V, et al. The Klip- pel-Trenaunay syndrome associated with multiple vis- ceral arteries aneurysms. Vasa 2007;36:124-129.
12. Goldstein SJ, Lee C, Young AB, et al. Aplasia of the cervical internal carotid artery and malformation of the circle of Willis associated with Klippel-Trenaunay syn- drome. Case report. J Neurosurg 1984;61:786-789.
13. Plouin PF, Perdu J, La Batide-Alanore A, et al. Fibro- muscular dysplasia. Orphanet J Rare Dis 2007;2:28.