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PHACE SENDROMU

Journal Name:

  • Cerrahpaşa Tıp Dergisi

Publication Year:

  • 2004

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Hemangiomas are the benign tumor of vascular endothelium. Unlike vascular malformations, they are rarely associated with structural abnormalities. A new neurocutaneous syndrome (PHACE syndrome) has been recently described in cases with large facial hemangioma associated with structural systemic abnormalities. Observation.- We report an infant with large facial hemangioma associated with posterior fossa brain malformations ( Dandy-Walker malformation and left cerebellar hemisphere hypoplasia), internal carotid artery anomalies, silent patent ductus arteriosus, strabismus and small hepatic caverneous hemangiomas. To our knowleged there was no documented case of PHECE syndrome in Turkey.
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Abstract (Original Language): 
Yüzünde geniş hemangiomu olan dört aylık bir çocukta, beyin arka çukur malformasyonları (DWM ve sol serebellar hemisfer hipoplazisi ), internal karotis arterlerde anomali, PDA, tek gözde şaşılık ve karaciğer kavernöz hemanjiyomu gibi yapısal anomaliler saptandı ve 18 aylığa kadar izlendi.PHACE sendromu tanısı konulan bu olgu bildiğimiz kadarıyla ülkemizde yayınlanan ilk olgudur. Olgumuzda altı aylıkken lezyonlar hızla yaygınlaştığı ve büyüdüğü için üç hafta oral kortikosteroid uygulandı fakat yanıt alınmadı. Sekiz aylıkken patent ductus arteriosusu kendiliğinden kapandı. Motor ve mental gelişimi oldukça geri olarak seyreden hastada onsekiz aylıkken ayrıca biteral internal karotis arterinde anomali saptandı ve hidrosefali gelişti. Karaciğerdeki lezyonda büyüme gözlenmedi, ayrıca kanama diyatezi gelişmedi. Hemanjiomlar çocukluk çağında sık görülür ve genelde selim seyrederler. Fakat özellikle yüzünde geniş hemangiomu olan çocuklar, beyin arka çukur malformasyonları, büyük damar anomalileri ve iç organlarda olabilecek lezyonlar açısından dikkatle izlenmelidir.
FULL TEXT (PDF): 
PDF icon arastirmax-phace-sendromu.pdf
  • 1
24-28
  • Turkish

REFERENCES

References: 

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