Yarık Damaklı Yenidoğan Olgumuzda Jarcho-Levin Sendromu - Olgu Sunumu

Yıl 2007, Cilt: 5 Sayı: 3, 125 - 128, 01.12.2007

Tolga Altuğ Şen Osman Öztekin Ayşegül Bükülmez Reşit Köken Tevfik Demir Adnan Narcı

Öz

Jarcho-Levin Sendromu segmental kostovertebral deformitelerin bulunduğu, çok sayıda sayısal ve yapısal vertebra ve kosta anomalilerinin yer aldığı konjenital bir sendromdur. Bu sendromda göğüs duvarının görünümü adeta “yengeç”e benzemektedir. Kostaların ve vertebraların yaygın deformiteleri ve anormal birleflme göstermeleri solunum sıkıntısına ve sık tekrarlayan solunum yolu enfeksiyonlarına neden olmaktadır. Spondilotorasik displazide baflta hemivertebra olmak üzere vertebradeformiteleri, spondilokostal displazide kosta deformiteleri görülürken, her ikisi de Jarcho-Levin sendromunun alt grubu olarak değerlendirilmektedir. Sık görülen bir tablo olmadığı için yarık damakla doğmufl olan spondilotorasik displazisi olan yenidoğan olgusu sunulmştur

Anahtar Kelimeler

Spondilotorasik dizostozis, yarık damak, yenidoğan

A Newborn With Cleft Palate Who Was Diagnosed As Jarcho-Levin Syndrome

Öz

Jarcho-Levin syndrome is a congenital, segmental costo-vertebral deformation with multiple vertebral and numerical orstructural rib abnormalities resulting in the 'crab-like' aspect of the chest. The clinical picture composed of thoracal vertebral and costal malformations and the affected vertebrae and costa were abnormally fused with each other and resulted inrespiratory difficulties with recurrent respiratory infections. In spondylothoracic dysplasia, malformed vertebral bodiesincluding hemivertebral are the major anomalies where as spondylocostal dysplasia refers to intrinsic anomalies of the ribsand both of them are defined as subgroups of Jarcho-Levin syndrome. Since it is a very rare entity, a newborn diagnosed asspondylothoracic dysplasia associated with cleft palate is presented

Anahtar Kelimeler

Spondylothoracic dysostosis, cleft palate, newborn

Kaynakça

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