Yenidoğanda Galen Veni Anevrizmal Malformasyonlu Vakaların Radyolojik ve Klinik Özellikleri, Endovasküler Tedavi Yönetimi: On Yıllık Deneyim

Year 2015, , 16 - 23, 01.01.2015

Sinan Tüfekci Asuman Çoban Beril Yaşa Meltem Bor Mehmet Barburoğlu Serra Sencer Zeynep İnce

https://doi.org/10.5222/j.child.2015.16

Abstract

Amaç: Galen veni anevrizmal malformasyonu GVAM tanısı ile 2005-2015 yılları arasında izlenen yenidoğanların klinik özellikleri, tanı, tedavi yaklaşımı ve sonuçlarını değerlendirmek.Gereç ve Yöntem: GVAM tanısı alan 8 hastanın klinik başvuru, izlem, tanı ve tedavi yaklaşımı retrospektif olarak dosya kayıtlarından elde edilddi. Yaşayan 4 hastadan 3’ünün nörolojik izlemi değerlendirildi, diğer vaka ile ilgili bilgiler ailenin başka şehire taşınmasından dolayı telefon ile görüşülerek elde edildi. Bulgular: Merkezimizde tedavi edilen sekiz hastadan yedisinin erkek 7, kız 1 antenatal tanısı vardı. Vakaların klinik izleminde ilk günlerde ağır kalp yetersizliği ve pulmoner hipertansiyon sık olup, daha sonra hipotansiyon, çoklu organ yetersizliği, hidrosefali ve konvülsiyon geliştiği görüldü. Hastalarda MR görüntüleme ve MR anjiyografi ile GVAM ve besleyici damar haritası gösterildi. Dört vaka transarteriyel embolizasyon tedavisi ile başarılı olarak tedavi edildi. Benzer tedavi metodlarına rağmen, üç vaka kaybedildi, bir hasta embolizasyon işlemi yapılamadan kaybedildi.Sonuç: GVAM karmaşık anatomi, patofizyoloji, ciddi nörolojik sekele yol açan özelliği ile yüksek mortalite ve morbiditeye sahiptir. Agresif tıbbi destek ve erken endovasküler embolizasyon tedavisi ile yüksek riskli yenidoğanlarda da prognoz iyileştirilebilir

Keywords

Yüksek debili kalp yetersizliği, endovasküler tedavi, galen ven anevrizmal malformasyonu, yenidoğan

Radiological and Clinical Features of Vein of Galen Aneurysmal Malformation in Newborn, Endovascular Interventional Treatment Method: 10 Years Experience

Abstract

Objective: To assess the clinical features, diagnosis, management strategies and results of newborns born between the years 2005-2015 with Vein of Galen Malformation VGAM .Material and Method: Eight newborn patients with diagnosis of VGAM were assessed retrospectively in terms of clinical admission symptoms, diagnosis, treatment strategies and follow-up based on the information retrieved from patient files. Three of four patients who survived had neurological assessment while the information about the remaining patient whose family moved to another city were obtained via phone call.Results: Seven out of 8 patients 1 female, and 7 male patients had antenatal diagnosis. In all cases severe heart failure and pulmonary hypertension were present from the first days of their life, later on development of hypotension, multiorgan failure, hydrocephalus and seizures was seen. VGAM and its feeder arteries were mapped by cranial magnetic resonance imaging and magnetic resonance angiography. Transarterial embolization therapy was performed successfully in four patients. Despite similar treatment modalities three of these patients died, and one patient was lost before initiation of embolization procedure.Conclusion: VGAM has high mortality and morbidity rates because of its complicated anatomy, pathophysiology leading to severe neurological sequelae. Prognosis in high risk neonates can be improved with aggressive medical support and early endovascular embolization therapy

Keywords

High-outflow heart failure, endovascular therapy, vein of Galen aneurysmal malformation, neonate

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