BILATERAL EPIBULBAR DERMOIDS WITH ISOLATED DEXTROCARDIA- A RARE CASE WITH REVIEW OF LITERATURE OF CARDIOVASCULAR MALFORMATIONS IN GOLDENHAR SYNDROME
Journal Title: Journal of Evolution of Medical and Dental Sciences - Year 2017, Vol 6, Issue 64
Abstract
BACKGROUND Goldenhar syndrome (Oculo-auriculo-vertebral dysplasia) is a condition comprising of triad of Ocular, Otic and Vertebral anomalies. It is also associated with multiple malformations involving Heart, Lungs and Kidneys. Congenital Heart Disease has been reported in 5% - 58% of patients with Goldenhar syndrome. The most common reported cardiovascular malformations are Ventricular Septal Defects and Tetralogy of Fallot. CONCLUSION Isolated dextrocardia has never been reported as association of Goldenhar syndrome. The genetic loci responsible for Goldenhar syndrome still remains elusive, more specific and rare associations will help detect that. To the best of our knowledge, this remains the first reported case of isolated Dextrocardia with bilateral and unusual presentation of Limbal Dermoids in Goldenhar syndrome.
Authors and Affiliations
Shobhana Dube, Salil Dube, Pratyush Ranjan
Keywords
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- EP ID EP231504
- DOI 10.14260/Jemds/2017/1012
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