ISSN : 2471-8041
Hamillour B, Khadri A H, Bensebti A A and Atrih Z
EHS El-Mensourah Constantine, Child and Adolescent Hospital, Algeria Child and Adolescent Hospital, Algeria
Posters & Accepted Abstracts: Med Case Rep
DOI: 10.21767/2471-8041-C2-006
Background: Goldenhar syndrome, or oculo-auriculo-vertebral syndrome, is a poly-malformative syndrome associating most often an epibulbar dermoid cyst, pretragial diverticula and vertebral anomalies. We report a rare observation of Goldenhar syndrome associating lateral labial cleft, dermoids of the occular limbus and pre-tragic excretion
Materials & Methods: This is the observation of female child 2 years old presenting a Goldenhar syndrome associating lateral cleft lip, dermoids of the occular limb and pre-tragic exchroissance and pre-auricular diverticula. Compliterary examinations, namely stromal radiograph of the echocardiography spine, did not show any associated abnormalities. The hospital were the surgery has taken is : EHS El-Mensourah Constantine, Child and Adolescent Hospital, Constantine, Algeria
Discussion: Goldenhar syndrome is a rare malformation syndrome related with an anomaly of development of the first gill arches. Although most cases of SDEG are sporadic, the modalities of autosomal recessive inheritance and the dominant have been described. Also referred to as oculo-atrial dysplasia, it is most often unilateral affecting the soft tissues and less the bone component. It usually includes peribulbar dermoids or dermolipomas, pre-auricular cuticular diverticula, abnormalities of the shape of the auricle, conductive hypoacoustics; abnormalities of the facial area and sometimes vertebral anomalies. It can be added to these malformative alterations a coloboma of the eyelid, a Duane syndromea and very rarely microphthalmia or anophthalmia.
Conclusion: Through this observation, we discuss the pathogenic aspects and clinics of this rare clinical entity and the therapeutic measures taken.
Dr.Hamillour Bochra ,Specialist, Pediatric Surgery M.D at EHS El Mensourah Hospital .Constine ,Algeria
She is board-certified in surgery and pediatric surgery, and has dedicated interest in pediatric urology and tumors , as well as neuroblastoma, childhood sarcomas, She also enjoys general pediatric surgery including plastic and neonatal care.She completed her medical degree at Constantine Algeria Medical School,and her general pediatric surgery residency at the EHS El Mensourah Constantine Hospital.
E-mail: hamillour.bochra@gmail.com
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