Mustafa ÖZDEMİR, Tugay TEPE, Şerif HAMİTOĞLU, Ahmet İbrahim KURTOĞLU, Ferda ÖZLÜ, Hacer YAPICIOĞLU

Dandy-Walker Malformasyonu İle Postaksiyel Polidaktili Birlikteliği: Yenidoğan Olgu Sunumu

Dandy-Walker malformasyonu, serebellar vermis agenezisi veya hipoplazisi, 4. ventrikülün kistik dilatasyonu ve posterior fossa genişlemesi ile karakterize nadir bir konjenital malformasyondur. Hastaların yaklaşık %70-90’ında postnatal hidrosefali gelişir. Dandy-Walker malformasyonun postaksiyel polidaktili ile ilişkisi ise, olası bir otozomal resesif sendrom (OMIM 220220) olarak tanımlanmıştır. Bu vakada Dandy-Walker malformasyonu tanısı alan ve postaksiyel polidaktilisi olan bir infant nadir olması nedeniyle sunuldu.

Anahtar Kelimeler:

Dandy-Walker malformasyonu, Polidaktili, Hidrosefali, Apne

Dandy-Walker Malformation With Postaxial Polydactyly: Newborn Case Report

Dandy-Walker malformation is a rare congenital malformation, characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the 4th ventricle and enlargement of the posterior fossa. Postnatal hydrocephalus develops in approximately 70-90% of the patients. The relationship of Dandy-Walker malformation with postaxial polydactyl has been defined as a possible autosomal recessive syndrome (OMIM 220220). Here, we present an infant with postaxial polydactyl accompanying Dandy-Walker malformation because of rare.

Keywords:

Dandy-Walker malformation, Polydactyly, Hydrocephalus, Apnea,

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