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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In uncomplicated cases, the treatment of the syndrome varies with age and systemic associations and it is mainly cosmetic. The diagnosis is based on clinical data: Goldenhar’s syndrome is a rare condition described initially in the early ‘s.

The exact aetiology is not known. The characteristics observed were: Cardiovascular malformations in oculoauriculovertebral dysplasia Goldenhar syndrome. The influence of other factors, including sindrome de goldenhar environment, during pregnancy has been also blamed. The high frequency of congenital heart disease in oculo-auriculo-vertebral dysplasia Goldenhar’s syndrome.

Goldenhar first described this condition in as a disease that presents a combination of several anomalies such as dermal epibulbar tumors, peri-auricular appendices and malformation of the ears. There is a global consensus that the diagnostics can’t be based sindrome de goldenhar in radiological or laboratory results, but consider the clinic aspects and associated with the birth systemic conditions and radiological findings.

The condition was documented in by Belgian ophthalmologist Maurice Goldenhar — Chromosomal sindrome de goldenhar are verified and theories suggest a disorder of the superior part of neural cells as the cause of the illness. We taught the mother how to monitor the child, and asked her to return once a month for 6 months to evaluate the prognosis of the lesion.

Prevalence ranges from 1 in 3, to 5, live births. The surgical procedure was performed under general anesthesia, with sindrome de goldenhar removal of the dermoid cysts until the corneal surface, where the epithelial layerwas removed.


Goldenhar syndrome

The mother reported in directed anamnesis that the diagnosis was given by the pediatrics, genetics and otolaryngology in the first year of her son’s life. On general examination, the patient did not reveal any systemic disease and her IQ for her age was normal.

Acessado em 22 de abril sindrome de goldenhar We inferred from this study that publications related to rare incident diseases such as sindrome de goldenhar one described in this report are important, considering that such descriptions provide greater assistance to professionals who may be dealing with this type of patient and thus can better goldenbar them, facilitating their work and giving a faster and more accurate diagnosis.

Case Report 2 An eight-year-old male patient came to our Department of Oral Medicine and Radiology with the complaint of deviation of mouth towards the right side on opening.

sindrome de goldenhar

X-ray examination of the skull and vertebral sindrlme did not show abnormalities. Greenword RD et al. Auditory and facial nerve dysfunction in patients with hemifacial microsomia.

Goldenhar syndrome – Wikipedia

J Craniofac Gen Develop Biol ;3: On the other hand, the sindroms of 1st and 2nd branchial arcs have been observed in children born from mothers who were exposed to thalidomide, primidone and retinoic acid, in addition to diabetic mothers 15, Other syndromes associated with sundrome preauricular tragic include Treacher — Collins syndrome, Wolf — Hirschhorn syndrome cervicooculoacoustic syndromeTownes-Brocks syndrome and Delleman syndrome. Clinical manifestations in 17 Greek patients with Goldenhar sindrome de goldenhar.

There was mild deviation of the tongue towards the right side. Ocular abnormalities include limbal dermoids, strabismus. Abnormalities of chromosomes have been identified sindrome de goldenhar.

Goldenhar’s syndrome: case report

Also a possibility of sindrlme of these bones exists, with defect of fusing of the zigomatic arc and agenesia of the palatine bones.

Male-female ratio is sindrome de goldenhar to be 3: Microtia appears to represent its less complex manifestation, however, several facial and systemic abnormalities may also be observed sindrome de goldenhar. J Med Gen ; This condition can be inherited in an autosomal dominant manner.


Regarding biomicroscopy, it was presentin both eyes, with the appearance of a high, whitish, vascularized tumor lesionwith hair follicles across its surface Figures 12 and 3. The patients present greater susceptibility for diabetes, hyperlipidemia and for sindrome de goldenhar embryopahtological effect caused by rubella. In sindrome de goldenhar literature we see that its etiology is characterized by involvement of the first branchial arches, and there are reported cases suggesting the existence of genetic factors, withrecessive autosomal and dominant autosomal pattern of dde.

PA view of vertebrae revealed no abnormalities. These examinations must be carried through after three years of age. It is characterized by a combination of anomalies: Most of the authors consider the presence of ear anomalies microtia and appendices in the ear necessary for diagnosis.

Published online Apr The ingestion of some drugs sondrome as cocaine, thalidomide, retinoic acid, and tamoxifen by sindrome de goldenhar mother were also related to the development of the disease 4. Sometimes, the collaboration of speech therapists and dentists is necessary when there are malfunctions of the palate and dental anomalies.

The correct therapeutic strategy may consider if the malformation is unilateral go,denhar bilateral and if presents association sindrome de goldenhar another digenetic syndrome. Heavy alcohol consumption during pregnancy is also one of the aetiologies of this syndrome [ 7 ]. In some cases they can coexist with periauricular fistulas.

Some patients present a wide variety of anomalies. Dental development was normal. Cotin G, Lacombe H.