Abdullah Barış Akcan MD, Ayşe ANIK, Semiha TERLEMEZ, Tuba ÖZDEMİR, MÜNEVVER KAYNAK TÜRKMEN

A Newborn with Dandy-Walker Malformation and Aortic Coarctation

Dandy-Walker malformasyonu (DWM) posterior kraniyal fossanın nadir görülen bir anomalisidir ve beraberinde beyin veya sistemik malformasyonlar sıklıkla eşlik edebilmekte ve prognozu etkileyebilmektedir. DWM'ye eşlik eden kardiyak anomalilerde bulunmaktadır. Bu anomalilerin tanınması, klinik tanı ve uygun cerrahi tedavinin düzenlenmesine yardımcı olur. Aort koarktasyonu ve Dandy-Walker varyantı birlikteliği çok nadir olarak görülmektedir. Burada, DWM ve aort koarktasyonu olan bir yenidoğanı sunuyoruz. Amacımız DWM'nin kardiyak anomalilerle birlikte olabileceğini vurgulamaktır. Kardiyak konjenital defektler genellikle kötü prognozla ilişkilidir. Bu tür özellikler, klinisyeni bu tür hastalarda sadece serebral yapılara değil kardiyovasküler anomalilere de kapsamlı tarama yapmaları konusunda uyarmalıdır.

Dandy-Walker Malformasyonu ve Aort Koarktasyonu

Dandy-Walker malformation (DWM) is a rare anomaly of the posterior cranial fossa. Concomitant brain or systemic malformations are frequent and can influence the prognosis. DWM is associated with cardiac abnormalities. Recognition of these anomalies are important for diagnosis and accurate surgical management. Association of aortic coarctation with DWM is extremely rare. Here, we report a newborn with DWM and aortic coarctation. Our aim was to emphasize that DWM can be associated with cardiac anomalies. Cardiac congenital defects are often associated with a poor prognosis. These kinds of features should alert the clinician to consider extensive screening in these patients not only for cerebral structures but also for cardiovascular abnormalities.

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1. Ten Donkelaar HJ, Lammens M, Wesseling P, Thijssen HO, Renier WO. Development and developmental disorders of the human cerebellum. J Neurol 2003; 250: 1025-36.
2. Butts T, Green MJ, Wingate RJ. Development of the cerebellum: simple steps to make a "little brain". Development 2014; 141: 4031-41.
3. Hirsch JF, Pierre-Kahn A, Renier D, Sainte-Rose C, Hoppe-Hirsch E. The Dandy-Walker malformation. A review of 40 cases. J Neurosurg 1984; 61: 515-22.
4. Roth P, Cohen A. Management of hydrocephalus in infants and children. In: Tindall G et al. editors: The Practice of Neurosurgery, Baltimore, 1996, Williams&Wilkins, p.2707.
5. Akgul A, Babaroglu S, Bahar I, Bokesoy I, Birincioglu L, Cobanoglu A. An usual combination: aortic arch coarctation associated with Dandy-Walker variant. Int J Cardiol 2006; 113: 258-60.
6. Barkovich AJ, Kios BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cyst like malformations based on the results of multiplaner MR imaging. AJR Am J Roentgenol 1989; 153: 1289-300.
7. Has R, Ermish H, Yuksel A, Ibrahimoglu L, Yildirim A, Sezer HD, et al. Dandy-Walker malformation: a review of 78 cases diagnosed by prenatal sonography. Fetal Diagn Ther 2004; 19: 342-7.
8. Venturini E, Magni L, Pucci G, Mazzinghi F. A late presentation of Dandy-Walker malformation and aortic coarctation. J Cardiovasc Med (Hagerstown) 2009.
9. Kontopoulos EV, Quintero RA, Salihu HM, Bornick PW, Allen MH. Dandy-Walker syndrome and monochorionic twins: insight into a possible etiological mechanism. J Matern Fetal Neonatal Med 2008; 21: 839-42.
10. Sasaki-Adams D, Elbabaa SK, Jewells V, Carter L, Campbell JW, Ritter AM. The Dandy-Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatr 2008; 2: 194-9.
11. Güven AS, Oflaz MB, Kaya A, Bolat F, Gülsever O, Şalk İ, et al. Fallot pentalojisi'nin eşlik ettiği dandy-walker varyasyonu: olgu sunumu. Cumhuriyet Tıp Derg 2014; 36: 400-3.
12. Kolbel N, Wisser J, Kirmanavisius J, Bolthauser E, Stallmach T, Huch A, et al. Dandy-Walker malformation: prenatal diagnosis and outcome. Prenat Diagn 2000; 20: 318-27.