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

Year 2015, Volume: 15 Issue: 1, 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

References

• barkovich AJ. Vein of Galen aneurysmal malformation. Diagnostic imaging. Pediatric Neuroradiology. Salt Lake City, Utah: Amirsys, Elsevier 2007;7-28.
• Embryological basis of some aspects of cerebral vascular fis- tulas and malformations. J Neurosurg 1996;85:1-8. http://dx.doi.org/10.3171/jns.1996.85.1.0001
• Hydrodynamics in vein of Galen malformations. Childs Nerv Syst 1992;8:111-7. http://dx.doi.org/10.1007/BF00298261
• Galen aneurysmal malformations: critical analysis of the lite- rature with proposal of a new classification system. J Neurosurg Pediatrics 2013;12:293-306. http://dx.doi.org/10.3171/2013.5.PEDS12587
• Microneurosurgery: AVM of the Brain, Clinical Considerations, General and Special Operative Techniques, Surgical Results, Nonoperated Cases, Cavernous and Venous Angiomas. Neuroanesthesia. Stuttgart: Georg Thieme, 1998; IIIB: pp 354.
• Dommergues M. Perinatal three-dimensional color power Doppler ultrasonography of vein of Galen aneurysms. J Ultrasound Med 2003;22:1357-62. of prenatally diagnosed vein of Galen aneurysmal malforma- tion: retrospective study and review of the literature.
• Ultrasound Obstet Gynecol 2012;40:652-8. http://dx.doi.org/10.1002/uog.11188
• G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery 2006;59(suppl):184- http://dx.doi.org/10.1227/01.NEU.0000237445.39514.16
• Goldstein b, Giroir b, Randolph A. The Members of the International Consensus Conference on Pediatric Sepsis. International Pediatric Sepsis Conference: definitions for sep- sis and ongan dysfunction in pediatrics. Pediatr Crit Care Med 2005;6:2-8. http://dx.doi.org/10.1097/01.PCC.0000149131.72248.E6 frawley GP, Dargaville PA, Mithchell PJ, Tress bM, loghnan P. Clinical course and medical management of neo- nates with severe cardiac failure related to vein of Galen malformation. Arch Dis Child Fetal Neonatal Ed 2002;87: 9. http://dx.doi.org/10.1136/fn.87.2.F144
• Ozanne A. Intracranial aneurysms in children aged under 15 years: review of 59 consecutive children with 75 aneurysms. Childs Nerv Syst 2005;21:437-50. http://dx.doi.org/10.1007/s00381-004-1125-x
• Neurology India March 2004;52:43-53. clinical features of vein of Galen malformations. J
• Neurointervent Surg. doi:10.1136/neurintsurg-2013-011005
• Heuchan AM, bhattacharyha J. Superior vena cava flow and management of neonates with vein of Galen malformati- on. Arch Dis Child Fetal Neonatal Ed 2012;97:344-7. http://dx.doi.org/10.1136/fetalneonatal-2011-300766 urysmal malformation: Prenatal diagnosis and early endovas- cular management. JCMA 2011;74:134-7. http://dx.doi.org/10.1016/j.jcma.2011.01.029
• Mc Elhinney Db, Halbach VV, Silverman nH, Dowd cf, Hanley fl. Congenital cardiac anomalies with of Galen mal- formations in infants. Arch Dis Child 1998;78:548-51. http://dx.doi.org/10.1136/adc.78.6.548 of vein of Galen malformations:a review based on five neuro- surgically treated cases and literature reports. Zentralbl Neurochir 1999;60:172-82.
• Systolic heart murmur as first manifestation of high output heart failure due to the vein of Galen malformation. Arch Cardiol Mexico 2012;82(3):214-7. http://dx.doi.org/10.1016/j.acmx.2012.04.002 gement of vein of galen aneurysm malformation: A series of two case reports. JPN 2011;6:32-5. fullerton HJ, Aminoff AR, ferriero DM, et al.
• Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations. Neurology 2003;61:1386-90. http://dx.doi.org/10.1212/01.WNL.0000094322.12621.02
• Geibprasert S, Krings T, Armstrong D, et al. Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations. Childs Nerv Syst ;26:35-46. http://dx.doi.org/10.1007/s00381-009-0959-7
• K. Anesthetic management of a neonate with vein of Galen aneurismal malformations and severe pulmonary hypertensi- on. Paediatr Anaesth 2005;15:525-8. http://dx.doi.org/10.1111/j.1460-9592.2005.01471.x