FIRST REPORT OF PERSISTENT FIFTH AORTIC ARCH AND SEVERE AORTIC COARCTATION IN ASSOCIATION WITH GOLDENHAR SYNDROME

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MALAKAN RAD E.

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Paper Information

Journal: IRANIAN JOURNAL OF PEDIATRICS Year:2013 | Volume:23 | Issue:1 (SUPPLEMENT) Page(s): 2-3

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Title

FIRST REPORT OF PERSISTENT FIFTH AORTIC ARCH AND SEVERE AORTIC COARCTATION IN ASSOCIATION WITH GOLDENHAR SYNDROME

Author(s)

MALAKAN RAD E.

Keywords

GOLDENHAR SYNBDROME

PERSISTENT FIFTH AORTIC ARCH

COARCTATION

Abstract

Background: Goldenhar syndrome or oculo-auriculovertebral dysplasia is present in 1/3500 to 1/25000 live births. The most recognizable feature of this syndrome is hemifacial microsomia. It may be associated with several other craniofacial abnormalities and congenial heart diseases (CHD). Ventricular septal defect and tetralogy of Fallot have been reported as the most common CHD in these patients. However, to date, there is no report of persistent fifth aortic arch and severe coarctation in these patients. This is the first report of a 7-year-old boy with Goldenhar syndrome and persistent fifth aortic arch associated with aortic arch tortuosity and severe coarctation in the persistent fifth arch.Case presentation: The patient was a 7-year-old boy, born to a consanguineous parent as a twin pregnancy, after 7 years of infertility. On general appearance, hemifacial microsomia, bilateral dermolipoma of the eyes, deformity of both ears associated with skin tags were apparent. On cardiac examination, pulses were normal in the upper extremities, but were very weak in the lower extremities. A grade 3/6 ejection systolic murmur was heard at upper left sternal border with wide radiation to the back. Renal bruit was heard on the back. On CXR, the left upper border of the cardiac silhouette was almost absent due to the aortic arch abnormality. Aortic angiography showed common origin of all four brachiocephalic branches from the main fouth arch and a torotous, hypoplastic persistent fifth aortic arch with severe coarctation. The patient was referred to pediatric cardiac surgeon for aortic repairment.Conclusion: This is the first report of persistent fifth aortic arch and severe coarctation in a 7-year-old male patient with Goldenhar syndrome. Persistent fifth aortic arch is a rare abnormality of aortic arch with an unkown incidence.Persistent fifth aortic arch may be asymptomatic or may present itself as coarctation.

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APA: Copy

MALAKAN RAD, E.. (2013). FIRST REPORT OF PERSISTENT FIFTH AORTIC ARCH AND SEVERE AORTIC COARCTATION IN ASSOCIATION WITH GOLDENHAR SYNDROME. IRANIAN JOURNAL OF PEDIATRICS, 23(1 (SUPPLEMENT)), 2-3. SID. https://sid.ir/paper/653912/en

Vancouver: Copy

MALAKAN RAD E.. FIRST REPORT OF PERSISTENT FIFTH AORTIC ARCH AND SEVERE AORTIC COARCTATION IN ASSOCIATION WITH GOLDENHAR SYNDROME. IRANIAN JOURNAL OF PEDIATRICS[Internet]. 2013;23(1 (SUPPLEMENT)):2-3. Available from: https://sid.ir/paper/653912/en

IEEE: Copy

E. MALAKAN RAD, “FIRST REPORT OF PERSISTENT FIFTH AORTIC ARCH AND SEVERE AORTIC COARCTATION IN ASSOCIATION WITH GOLDENHAR SYNDROME,” IRANIAN JOURNAL OF PEDIATRICS, vol. 23, no. 1 (SUPPLEMENT), pp. 2–3, 2013, [Online]. Available: https://sid.ir/paper/653912/en

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