Goldenhar's Syndrome (Oculo-Auriculo-Vertebral Dysplasia) with Congenital Facial Nerve Palsy

Clinical aspects of oculo-auriculo-vertebral dysplasia.

Oculo-auriculo-vertebral (Goldenhar's) syndrome.

Goldenhar's syndrome (Goldenhar, I952) consists of epibulbar dermoids or lipodermoids, auricular appendages, pretragal blind fistulas, and vertebral anomalies. A number of other deformities associated with this condition have been summarized by Bowen, Collum, and Rees (I97I). Our case is presented because of the presence of an aberrant salivary gland in the nose. This complication has not been ...

[Oculo-auriculo-vertebral spectrum in patients with congenital heart defects].

BACKGROUND there have been few studies evaluating the frequency of oculo-auriculo-vertebral spectrum (OAVS) in patients with congenital heart defects (CHDs). OBJECTIVE to verify the frequency of OAVS in a sample of patients with major heart malformations. METHODS we evaluated a prospective cohort of patients with CHD admitted in a pediatric cardiac intensive care unit (ICU) in Brazil. The d...

Cranial Nerve Abnormalities in Oculo-Auriculo-Vertebral Spectrum.

BACKGROUND AND PURPOSE Cranial nerve abnormalities might be observed in hemifacial microsomia and microtia (oculo-auriculo-vertebral spectrum), but the rate, features, and relationship with functional impairment or phenotype severity have not yet been defined. This study aimed at investigating absence/asymmetry, abnormal origin, morphology and course of cranial nerves, and presence/asymmetry of...

Goldenhar syndrome (oculo-auriculo-vertebral dysplasia) in a twin new born baby.

Fhe Oculo-auriculo-vertebral dyspla~ia, described for the first time by Goldenhar 1 is a disorder affecting the structures which develop from I and II branchial archs. It is characterised by epibulbar dermoid cyst and preauricular skin appendages with or without vertebral defects. 2 This syndrome has always been reported in single born babies from India 2-4 but in western literature 3 cases hav...