An intracranial hemorrhage in a patient with Klippel-Trenaunay syndrome and Hemophilia B association

Year 2015, Volume: 4 Issue: 4, 387 - 390, 01.11.2015

Zühal Örnek İbrahim Etem Pişkin Mehmet Karacı

https://doi.org/10.5505/abantmedj.2015.50023

Abstract

Klippel-Trenaunay syndrome KTS , a rare congenital disorder, is characterized by cutaneous vascular malformation, soft tissue-bony hypertrophy and varicose veins. KTS does not only show peripheral involvement, but also visceral involvement may be observed which is an important source of morbidity and mortality. Intracranial hemorrhage in KTS is extremely rare and usually traced to an underlying arteriovenous malformation or fistula. We report a case that KTS in a patient presented with intracranial hemorrhage was bound to hemophilia B.

Keywords

Klippel-Trenaunay syndrome, haemophilia B, intracranial haemorrhage

İntrakraniyal kanamalı bir olguda Klippel-Trenaunay sendromu ve Hemofili B birlikteliği

Abstract

Klippel-Trenaunay Sendromu KTS , kutanöz vasküler malformasyonlar, kemik ve yumuşak doku hipertrofisi ve variköz venler ile karakterize ender konjenital bir sendromdur. KTS’de hem periferik hem de visseral tutulum gözlenebilir ve bu önemli morbidite ve mortalite kaynağı olabilir. KTS de intrakranial kanamaya oldukça nadir rastlanır ve altında genellikle arteriovenöz malformasyonlar veya fistül mevcuttur. Bu olgu KTS’lu bir hastadaki intrakranial kanamanın sebebinin Hemofili B olması nedeniyle sunulmuşur.

Keywords

Klippel-Trenaunay sendromu, Hemofili B, intrakranial kanama

References

• 1. Klippel M, Trenaunay P. Dunaevusvariquexosteo hypertrophique. Arch GenMed 1900;3:641-72.
• 2. Petzold A, Bischoff C, Conrad B. Repetitive cerebral bleeding in an adult with KlippelTrénaunay Syndrome. J Neurol 2000; 247: 389- 91.
• 3. al-Salman MM. Klippel-Trenaunay syndrome: clinical features, complications, and management. Surg Today 1997;27: 735-40.
• 4. Hizarcioğlu M, Gülez P, BilgerÜtük E, Kayserili E, Yener H. Klippel Trenaunay Weber Sendromu: Bir Olgu Sunumu ve Literatür Taraması, T Klin Pediatri 2003;12:93-6.
• 5. Oduber CE, GerdesVE,van der Horst CM, Bresser P. Vascular malformations as underlying cause of chronic thromboembolism and pulmonary hypertension. J Plast Reconstr Aesthe tSurg. 2009;62: 684-9
• 6. Samuel M, Spitz L. Klippel Trenaunay syndrome: clinical features, complication and management in children. Br J Surg 1995;83: 757-61.
• 7. Mazoyer E, Enjolras O, Laurian C, Houdart E, Drouet L. Coagulation abnormalities associated with extensive venous malformations of thelimbs: differentiation from KasabachMerritts yndrome. Clin Lab Haematol. 2002;24: 243–51
• 8. Wilson CL, Song LM, Chua H, Ferrara M, Devine RM, Dozois RR, Nehra V. Bleeding from cavernous angiomatosis of the rectum in Klippel-Trenaunay syndrome: report of three cases and literatüre review. Am J Gastroenterol. 2001; 96:2783-8.
• 9.Kasper CK. Hereditary plasmaclotting factor disorders and their management. Haemophilia 2000; 6(Suppl.1); 13-27.
• 10- Reinertsen I, Jakola AS, Selbekk T, Solheim O. Validation of model-guidedplacement of external ventricular drainsInt J Comput Assist Radiol Surg. 2014;9:777-84.