Title: Evaluation of Chest Radiograph Findings in Children's Below 5 Years over a Periods of 10 Months with a Rare Case of Congenital Vertebral Segmentation Defect with Left Lower Ribs Agenesis and Right Sided Bifid Rib - Jarcho Levin Syndrome Associated with Dextrocardia
Author: Dr Anju Devi Loukham
DOI: http://dx.doi.org/10.18535/jmscr/v3i11.32
Evaluation of chest radiograph findings in 200 children's below 5 years over a period of 10 months. We report two cases of segmentation anomaly, both are infants. 0ne of them had no other associated anomaly, only vertebral segmentation defect. Another one is a rare case with vertebral segmentation defects, ribs anomaly and associated dextrocardia which was presented on 20 days of her life with progressive respiratory insufficiency. The neonate had multiple left lower ribs agenesis and bifid rib of right first and second ribs, dorsal left hemivertebrae in the mid and lower thoracic region with mild scoliosis of lateral convexity towards the right. Left hemidiaphragm is relatively elevated resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of Jarcho Levin syndrome with thoracic insufficiency. In addition, the neonate had Cardiac outline dearranged with apparent direction of cardiac apex towards the right side (Dextrocardia).The aim of this study was to evaluate the findings of the chest radiographs after exposing the child patients to the halmful radiation , no doubt: X-rays can be helpful, even life-saving. But sometimes, they're not necessary. Aims and Objectives To study the chest radiographs findings of children's under five years over a period of 10 months as a Chest X-rays produce images of the heart, lungs, blood vessels, airways, and the bones of the chest and spine. Chest X-rays can also reveal fluid in or around the lungs or air surrounding a lung however repeated advice of x rays to a child patient should be limited as the kids are still growing, they're more sensitive to radiation. Keywords: Jarcho Levin syndrome, Dextocardia 1. Kulkarni ML, Navaz SR, Vani HN, Manjunath KS, Matani D. Jarcho-Levin syndrome. Indian J Pediatr.2006;73:245–7.(Pub Med) 2. Reyes MG, Morales A, Harris V, Barreta TM, Goldbarg H. Neural defects in Jarcho-Levin syndrome. J Child Neurol. 1989;4:51–4. (Pub Med) 3. Young S, Reyes MG, Uzoaru IL, Harris V. Arnold-Chiari malformation in Jarcho-Levin syndrome. J Child Neurol. 1989;4:229–30. (Pub Med) 4. Campbell RM, Jr, Smith MD, Mayes TC, Mangos JA, Willey-Courand DB, Kose N, et al. The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am. 2003;85-A:399–408. (Pub Med) 5. Turnpenny PD, Kusumi K. DLL3 and spondylocostal dysostosis. In: Epstein CJ, Erickson RP, editors.Inborn Errors of Development: The Molecular Basis of Clinical Disorders of Morphogenesis. New York: Oxford University Press; 2004. pp. 470–81. 6. Mortier GR, Lachman RS, Bocian M, Rimoin DL. Multiple vertebral segmentation defects: Analysis of 26 new patients and review of the literature. Am J Med Genet. 1996;61:310–9. (Pub Med) 7. Chiu WK, Kwok KM. A child with Jarcho Levin syndrome. J Pediatric Respirology Crit Care. 2009;5:8–13. 8. Turnpenny PD. Abnormal vertebral segmentation and the spondylocostal dysostosis. In: Kusumi K, Sally L, editors. The Genetics and Development of Scoliosis. New York: Springer; 2010. pp. 81–108. 9. Aurora P, Wallis CE, Winter RM. The Jarcho-Levin syndrome (spondylocostal dysplasia) and complex congenital heart disease: A case report. Clin Dysmorphol. 1996;5:165–9. (Pub Med) 10. Delgoffe C, Hoeffel JC, Worms AM, Bretagne MC, Pernot C, Pierson M. Costovertebral dysostosis and congenital heart disease. Four case-reports (author's transl) Ann Pediatr (Paris) 1982;29:135–9.(Pub Med) 11. Elier JL, Morton JM. Bizarre deformities in offspring of user of lysergic acid diethylamide. N Engl J Med. 1976; 283:395–7. (Pub Med) 12. Pang D, Dias MS, Ahab-Barmada M. Split cord malformation. Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992;31:451–80. (Pub Med)Abstract
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