15 Apr Goldenhar’s syndrome is a rare condition which was described initially in the early s. It is characterized by a combination of anomalies. Goldenhar syndrome is one of the most common congenital anomalies of the first and second branchial arches. Its main . da síndrome de Goldenhar. Rev Bras. Síndrome de Goldenhar e a educação inclusiva. Article (PDF Available) · April with 22 Reads. DOI: /X Cite this publication.

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Acute vitamin A intoxication in the mother during pregnancy. It is caused by multifactorial inheritance, having generally unilateral alterations at continuous development 1.

Goldenhar syndrome

A Case of Goldenhar-Gorlin syndrome with unusual association of hypoplastic thumb. Pulse wave velocity recordings in sindrome de goldenhar family with ecchymotic Ehlers- Danlos syndrome. Case Report 2 An eight-year-old male patient came to our Department of Oral Medicine and Radiology with the complaint sindrome de goldenhar deviation of mouth towards the right side on opening.

Pre-auricular lumps or fistulas can be found as first manifestation, followed by the orthopedic alterations.

In some cases they can coexist with periauricular fistulas. Palatal cleft may be observed radiographically Syndromes of the head and neck.


Am J Med Genet. These changes explain the disorders of the external ear, because the first branchial sindrome de goldenhar gives rise to the primordium of the anterior part of the auriculas, and the second branchial arch to the primordium of the posterior part of the ears 7.

When bilateral, one of the sides is more affected 2. April 07, ; Sindrome de goldenhar It can also be identified by the similar ocular-auricle-vertebral dysplasia, OAV dysplasia, facial-auricle-vertebral sequence, FAV sequence, ocular-auricle-vertebral sinddrome.

Oxford University press; Prevalence ranges from 1 in 3, to 5, live births. The exact aetiology is not known.

Goldenhar syndrome

Congenital heart defects in sindrome de goldenhar with oculo-auriculovertebral spectrum Goldenhar syndrome. Other organ abnormalities include cardiac defects and renal abnormalities. It is necessary to carry through the functional examination of audition for a precocious detention of the malformations, moth unilateral and bilateral. Familiar cases with dominant autosomal inheritance with changeable expressivity have already been reported, in addition to cases of consanguinity between sindrome de goldenhar, suggesting recessive autosomal inheritance.

Am J Med Gen ; Severe axial anomalies in the oculo-auriculo-vertebral Goldenhar complex.

Reconstruction surgeries of the external ear may be performed at the age of 6 to 8 years. Oculoauriculovertebral dysplasia and variants: Ingestion of drugs such as thalidomide, retinoic acid, tamoxifen, and cocaine by pregnant mothers may be related to the development of this syndrome.


There is also a possibility of agenesis of sindrome de goldenhar bones with lack of fusion of the zygomatic arch and agenesis of sindrome de goldenhar palatine bones.

Goldenhar Syndrome – A Warning for the Otorhinolaryngologist

The feeding difficulties generally sindrome de goldenhar not persist beyond the first year of golldenhar. The surgical procedure was performed under general anesthesia, with total removal of the dermoid cysts until the corneal surface, where the epithelial layerwas removed.

Indian J Dent Res. X-ray examination of the skull and vertebral column did not show abnormalities.

Goldenhar Syndrome – Review with Case Series

J Med Gen ; Brachial arch and oro-acral disorders. The mother reported in directed anamnesis sindrome de goldenhar the diagnosis was given by the pediatrics, genetics and otolaryngology in the first year of her son’s life.

In patients with mandibular hypoplasia, reconstructions can be done by using rib bone grafts and an underdeveloped maxilla can be lengthened by bone distraction, osteogenesis.

Monozygotic twins discordant for Goldenhar syndrome.