Turkish Neurosurgery 2013 , Vol 23 , Num 2
Spondylocostal Dysostosis Associated with Type I Split Cord Malformation and Double Nipple on One Side: A Case Report
Muhammet Bahadr YILMAZ1, Aysegul KAYMAK2, Gokhan KURT1, Ferda Emriye PERCIN2, Kemali BAYKANER1
1Gazi University, Faculty of Medicine, Division of Pediatric Neurosurgery, Department of Neurosurgery, Ankara, Turkey
2Gazi University, Faculty of Medicine, Department of Medical Genetics, Ankara, Turkey
DOI : 10.5137/1019-5149.JTN.4503-11.4 Jarcho Levin syndrome is a rare genetic disorder characterized by multipl vertebral and costal anomalies at birth. Jarcho Levin syndrome includes two phenotypic groups: spondylothoracic dysostosis and spondylocostal dysostosis. The prognosis of spondylothoracic dysostosis has worse than spondylocostal dysostosis, because of respiratory complications. Associated malformations include those of the congenital heart disease, urogenital malformation, skeletal anomalies and neural tube defects. We present a patient with spondylocostal dysostosis, who also had type I split cord malformation, tethered cord, scoliosis and double nipple on the right. Although the association of spondylocostal dysostosis and type I split cord malformation is very rare, double nipples on one side is no previously reported. Keywords : Spondylocostal dysostosis, Spondylothoracic dysostosis, Jarcho Levin syndrome, Congenital scoliosis, Accessory nipple, Type I split cord malformation
Corresponding author : Muhammet Bahadr Ylmaz, bahadiryilmaz@yahoo.com.tr