Yarık damaklı yenidoğan olgumuzda Jarcho-Levin sendromu
Loading...
Date
2007
Authors
Şen, Tolga Altuğ
Öztekin, Osman
Bükülmez, Ayşegül
Köken, Reşit
Demir, Tevfik
Narcı, Adnan
Journal Title
Journal ISSN
Volume Title
Publisher
Uludağ Üniversitesi
Abstract
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 birleşme göstermeleri solunum sıkıntısına ve sık tekrarlayan solunum yolu enfeksiyonlarına neden olmaktadır. Spondilotorasik displazide başta hemivertebra olmak üzere vertebra deformiteleri, spondilokostal displazide kosta deformiteleri görülürken, her ikisi de Jarcho-Levin sendromunun alt grubu olarak değerlendirilmektedir. Sık görülen bir tablo olmadığı için yarık damakla doğmuş olan spondilotorasik displazisi olan yenidoğan olgusu sunulmuştur.
Jarcho-Levin syndrome is a congenital, segmental costo-vertebral deformation with multiple vertebral and numerical or structural 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 in respiratory difficulties with recurrent respiratory infections. In spondylothoracic dysplasia, malformed vertebral bodies including hemivertebral are the major anomalies where as spondylocostal dysplasia refers to intrinsic anomalies of the ribs and both of them are defined as subgroups of Jarcho-Levin syndrome. Since it is a very rare entity, a newborn diagnosed as spondylothoracic dysplasia associated with cleft palate is presented.
Jarcho-Levin syndrome is a congenital, segmental costo-vertebral deformation with multiple vertebral and numerical or structural 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 in respiratory difficulties with recurrent respiratory infections. In spondylothoracic dysplasia, malformed vertebral bodies including hemivertebral are the major anomalies where as spondylocostal dysplasia refers to intrinsic anomalies of the ribs and both of them are defined as subgroups of Jarcho-Levin syndrome. Since it is a very rare entity, a newborn diagnosed as spondylothoracic dysplasia associated with cleft palate is presented.
Description
Keywords
Spondilotorasik dizostozis, Yarık damak, Yenidoğan, Spondylothoracic dysostosis, Cleft palate, Newborn
Citation
Şen, T. A. vd. (2007). "Yarık damaklı yenidoğan olgumuzda Jarcho-Levin sendromu". Güncel Pediatri, 5(3), 125-128.