Hacer YAPICIOĞLU YILDIZDAŞ, Sevcan ERDEM, Fadli DEMİR, Hüseyin ŞİMŞEK, Ferda ÖZLÜ

Konjenital midaortik sendromlu bir bebek

Midaortik sendrom nadir bir hastalık olup abdominal veya distal inen aortanın segmental veya difüz darlığı ile karakterizedir. Olguların çoğu idiyopatik olmakla birlikte bir kısmı da fibromüsküler displazi, dev hücreli aterit, Moyamoya hastalığı veya nörofibramatozis, Williams’ sendromu ve Alagille sendromu gibi genetik hastalıklara ikincildir. Burada 40 günlük iken hastaneye solunum sıkıntısı, kalp yetmezliği tablosunda başvuran ve midaortik sendrom tanısı alan bir olgu sunulmuştur. Olguda medikal tedavi tercih edilmiş ancak bebek iki aylık iken eksitus olmuştur.

An infant with congenital midaortic syndrome

Midaortic syndrome is an uncommon disease characterized by segmental or diffuse narrowing of abdominal or distal descending thorasic aorta. Majority of the cases are idiopathic but some are secondary to fibromuscular dysplasia, giant cell arteritis, Moyamoya disease or genetic diseases such as neurofibromatosis, Williams’ syndrome and Alagille syndrome. It is very rare in newborn period. Here we present a 40 days old infant who admitted to hospital for respiratory distress, hypertension, heart failure and was diagnosed as midaortic syndrome. She had medical treatment but unfortunately died due to heart failure when she was two months old age.

Keywords:

Midaortic syndrome, aort coarctation, infant,

PDF

___

1. Cohen JR, Birnbaum E. Coarctation of the abdominal aorta. J Vasc Surg. 1988;8:160-4.
2. Connolly JE, Wilson SE; Lawrence PL, Fujitani RM. Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg. 2002;194:774-81.
3. Rumman RK, Nickel C, Matsuda-Abenidi M, Lorenzo AJ, Langlois V, Radhakrishnan S et al. Disease beyond the arch: A systematic review of middle aortic syndrome in childhood. Am J Hypertens. 2015;28:833-46.
4. Sen PK, Kinare SG, Engineer SD, Parulkar GB. The middle aortic syndrome. Br Heart J. 1963;225:610-8.
5. Maycock W. Congenital stenosis of the abdominal aorta. Am Heart J. 1937;13:633-46.
6. Siassi B, Klyman G, Emmanoulides GC. Hypoplasia of the abdominal aorta associated with rubella syndrome. Am Dis Child. 1970;120:476-9.
7. Graham LM, Zelenock GB, Erlandson EE, Coran AG, Lindenauner SM, Stanley JC. Abdominal aortic coarctation and segmental hypoplasia. Surgery. 1979;86:519-29.
8. Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy F, Haycock GB, Taylor PR. Mid-aortic syndrome presenting in childhood. Br J Surg. 1996;83:235-40.
9. Poulias GE, Skoutas B, Doundoulakis N, Haycock GB, Taylor PR. The mid-aortic dysplastic syndrome. Surgical considerations with a 2 to 18 year follow-up and selective histopathological study. Eur J Vasc Surg. 1990;4:75-82.
10. Harmon CM, Azzuqa AA; Ranganathan S, Mahmood B. Mid-aortic syndrome in a preterm infant: a rare cause of hypertension. J Pediatr. 2015;167:492.e1.
11. Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KEC. Idiopathic mid-aortic syndrome in children. Pediatr Nephrol. 2008;23: 1135-42.
12. Zeltser I, Parnes IA, Ko H, Holzman IR, Kamenir SA. Midaortic syndrome in the fetus and premature newborn: a new etiology of nonimmune hydrops fetalis and reversible fetal cardiomyopathy. Pediatrics. 2003;111:1437-42.
13. Das BB, Recto M, Shoemaker L, Mitchell M, Austin EH. Mid-aortic syndrome presenting as neonatal hypertension. Pediatr Cardiol. 2008;29:1000-1.
14. Izraelit A, Kim M, Ratner V, Levasseur SM, Seigle R, Krishnamurthy G. Mid-aortic syndrome in two preterm infants. J Perinatol. 2012;32:390-2.
15. Terramani TT, Salim A, Hood DB, Rowe VL, Weaver FA. Hypoplasia of the descending thoracic and abdominal aorta: a report of two cases and review of the literature. J Vasc Surg. 2002;36:844–8.
16. ten Dam K, van der Palen RL, Tanke RB, Schreuder MF, de Jong H. Clinical recognition of mid-aortic syndrome in children. Eur J Pediatr. 2013;172;413-6.
17. Kim HB,Vakili K, Modi BP, Ferguson MA, Guillot AP, Potanos KM et al. A novel treatment for the midaortic syndrome. N Engl J Med. 2012;367:2361-62.