Case Report

Spondylothoracal Type Jarcho- Levin Syndrome Associated With Neural Tube Defect: A Case Report

  • Nilüfer Güzoğlu
  • Dilek Dilli
  • Ş. Suna Oğuz
  • Avidan Kızılelma
  • Ömer Erdeve
  • Serdar Ceylaner
  • Uğur Dilmen

Received Date: 12.08.2009 Accepted Date: 08.06.2010 J Ankara Univ Fac Med 2010;63(2):65-67

Jarcho-Levin syndrome is a congenital disorder characterized by abnormal vertebral segmenta-tion, defects affecting spine formation with complete bilateral fusion of the ribs at the costover-tebral junction producing a “crab-like” configuration of the thorax. This syndrome is composed of two subgroups: spondylothorasic and spondylocostal dysostosis. While vertebral deformities are frequent in spondylothorasic type, costal abnormalities are common in spondylocostal type. Jarcho-Levin syndrome may be associated with neural tube defects, cardiac, renal, and gastroin-testinal anomalies. In this report, a newborn diagnosed to be Spondylothorasic type Jarcho -Levin syndrome and associated with meningomyelocele and hydrocephalus was presented with litera-ture highlight because of its rarity.

Keywords: Congenital disorder, crab-like thorax, spondylothoracic dysostosis, meningomye-locele