Ali KAYA, İsmail ŞALK, Fatih BOLAT, Ahmet Sami GÜVEN, Füsun Dilara İÇAĞASIOĞLU, Osman GÜLSEVER, Özlem AKKAR BOZOKLU, Mehmet Burhan OFLAZ

Fallot Pentalojisi'nin eşlik ettiği Dandy-Walker varyasyonu: Olgu sunumu

Dandy-Walker varyasyonu, 4. ventrikül ve sisterna magna arasındaki devamlılıkla sonuçlananvermisin parsiyel agenezisi ile karakterize santral sinir sisteminin nadir bir anomalisidir.Literatürde ilk kez 1988de Kohyama ve ark. [10] tarafından bildirilen Dandy-Walker varyasyonuve Fallot Tetralojisi birlikteliği daha sonraki yıllarda birkaç vaka sunumu şeklinde bildirilmiştir.Biz bu yazıda, Dandy-Walker varyasyonu ve Fallot Pentalojisi olan 2 günlük bir erkek hastayıbildiriyoruz.

Dandy-Walker variant associated with pentalogy of f allot: Case report

Dandy-Walker variant is a rare anomaly of the central nervous system that is characterized bypartial agenesis of vermis and results in persistence of the 4. ventricule and cisterna magna. Firstly,in 1988 Kohyama et al. [10] reported the coexistence of Dandy-Walker variant and tetralogy ofFallot. Afterwards, it is reported as several case reports in the literature. In this case report, wereport a 2 days-old male patient with Dandy-Walker variant and pentalogy of Fallot.

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1. Millen KJ, Gleeson JG. Disorders of Cerebellar and Brainstem Development. In: Swaiman KF, Ashwal S, Ferriero DM, Schor NF, eds. Swaimans Pediatric neurology: principles and practice. 5th ed. Philadelphia: Elsevier Saunders 2012; 160-5.
2. Barkovich AJ. Congenital Malformations of the Brain and Skull. Pediatric Neuroimaging. 4th ed. Philadelphia: Lippincott Williams&Wilkins 2005; 387-91.
3. Tadakamadla J, Kumar S, Mamatha GP. Dandy-Walker malformation: An incidental finding. Indian J Hum Genet 2010; 16: 33-5.
4. Ozdemir O, Polat A, Cinbis M, Kurt F, Kucuktasci K, Kiroglu Y. Dandy- Walker's variant and tetralogy of Fallot with atrial septal defect and patent ductus arteriosus and primary hypothyroidy--a new association. Indian J Pediatr 2009; 76: 433-5.
5. 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.
6. Poetke M, Frommeld T, Berlien HP. PHACE syndrome: new views on diagnostic criteria. Eur J Pediatr Surg 2002; 12: 366-74.
7. Leonardi ML, Pai GS, Wilkes B, Lebel RR. Ritscher-Schinzel cranio-cerebello- cardiac (3C) syndrome: report of four new cases and review. Am J Med Genet 2001; 102: 237-42.
8. Hsieh CC, Hsu JJ, Lo LM, Hsieh TT, Soong YK. Non-mosaic trisomy 20 in cultures of amniotic fluid from a fetus with serious congenitale malformation. J Formos Med Assoc 1992; 91: 543-4.
9. Hirsch JF, Kahn AP, Renier D, Rose CS, Hirsch EH. The Dandy-Walker malformation. J Neurosurg 1984; 61: 515-22.
10. Kohyama J, Watanabe S, Iwakawa Y, Fukuda C. A case of Dandy-Walker malformation: consideration on the teratogenic period and sleep structures. Brain Dev 1988; 10: 392-6.