Serdar CEYLANER, S. Suna OĞUZ, Dilek DİLLİ, Uğur DİLMEN, Avidan KIZILELMA, Nilüfer GAZİOĞLU, Ömer ERDEVE
Nöral tüp defektinin eşlik ettiği spondilotorakal tip jarcho-levin sendromu: bir olgu sunumu
Jarcho-Levin sendromu anormal kostovertebral segmentasyon, “yengeç şeklinde” toraks kafesi görünümüne neden olan ve omurga yapısını etkileyen kostovertebral bileşkede kostaların bilateral komplet füzyon defektleri ile karakterize konjenital bir sendromdur. Bu sendromun spondilotorasik ve spondilokostal dizostoz olmak üzere iki alt tipi vardır. Spondilotorasik tipte vertebra deformiteleri, spondilokostal tipte ise kosta anomalileri daha sık gözlenir. Jarcho-Levin sendromuna nöral tüp defektleri, kardiyak, renal ve gastrointestinal anomaliler eşlik edebilir. Bu yazıda meningomiyelosel ve hidrosefali eşlik eden spondilotorasik tip Jarcho-Levin sendromu tanısı alan bir yenidoğan olgu nadir görülmesi nedeniyle literatür bilgileri ışığında sunuldu.
Spondylothoracal type jarcho- levin syndrome associated with neural tube defect: a case report
Jarcho-Levin syndrome is a congenital disorder characterized by abnormal vertebral segmentation, defects affecting spine formation with complete bilateral fusion of the ribs at the costovertebral 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 gastrointestinal anomalies. In this report, a newborn diagnosed to be Spondylothorasic type Jarcho-Levin syndrome and associated with meningomyelocele and hydrocephalus was presented with literature highlight because of its rarity.
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- Başlangıç: 1947
- Yayıncı: Erkan Mor
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