			Home About us Contact

Goldenhar Syndrome (goldenhar + syndrome)

Distribution by Scientific Domains

Medical Sciences	62%

Selected Abstracts

Effect of mandibular distraction osteogenesis on developing molars

ORTHODONTICS & CRANIOFACIAL RESEARCH, Issue 4 2007
M Kleine-Hakala
Structured Abstract Authors,,, Kleine-Hakala M, Hukki J, Hurmerinta K Objective,,, To observe the effect of mandibular distraction osteogenesis (DO) on developing molars. Design,,, Descriptive clinical study. Setting,,, University hospital setting. Seventeen children (mean age 7.6 years) with various syndromes (hemifacial/craniofacial microsomia, Goldenhar syndrome, Treacher Collins syndrome, Nager syndrome and Pyle,Bakwin,Krida syndrome) participated. Experimental variable,,, Severely retrognathic lower jaws were distracted (mean 30 days) with an extraoral bicortically fixed DO device. Outcome measure,,, Consecutive panoramic tomograms were analysed after a mean follow-up period of 3.6 years, range 1,6.9 years. Results,,, The mandibular molars were affected by DO in 13 of the 17 patients which included 18 of 63 mandibular molars studied. Structural changes included root malformations, hindered tooth development and the destruction of tooth follicles. Positional changes such as shifted and tilted teeth were also found. Three injured teeth failed to erupt. These changes were because of splitting of the tooth follicle during the osteotomy (22%), piercing of the tooth follicle by the pin (39%) or migration of tooth germ towards the newly created bone (39%). Fifteen per cent of first molars, 43% of second molars and 31% of third molars were affected during the distraction process. Of all dental injuries, 44% were noticed while the appliance was in place. A further 17% of injuries were noted between 3 months and 1 year postoperatively and 33% during the second postoperative year. Conclusions,,, Although dental injuries are a minor disadvantage compared with the vast benefits offered by DO, focusing on these drawbacks might lead to re-consideration of the type of the device as well as the timing of DO. [source]

Goldenhar syndrome and intubation with the fiberoptic broncoscope

PEDIATRIC ANESTHESIA, Issue 8 2008
Onur ÖZlü md
No abstract is available for this article. [source]

Accessory Tragus: Report of Two Cases and Review of the Literature

PEDIATRIC DERMATOLOGY, Issue 5 2000
Thomas Jansen M.D.
In the vast majority of cases it is an isolated developmental defect not associated with other abnormalities. However, the remote possibility exists that it could be associated with other abnormalities of the first and second branchial arch. Accessory tragus is a consistent feature of the oculoauriculovertebral syndrome (Goldenhar syndrome). When correctly identified, surgical excision of accessory tragus is quite simple and rarely results in any complications. [source]

Goldenhar syndrome associated with prenatal maternal Fluoxetine ingestion: Cause or coincidence?

BIRTH DEFECTS RESEARCH, Issue 7 2010
Chantal Farra
Abstract Goldenhar syndrome, also known as oculo-auriculo-vertebral spectrum, is a complex, heterogeneous condition characterized by abnormal prenatal development of facial structures. We present the occurrence of Goldenhar syndrome in an infant born to a woman with a history of prenatal Fluoxetine ingestion throughout her pregnancy. Because this is the first reported case associating maternal Fluoxetine intake with fetal craniofacial malformations, a potential mechanism of injury is discussed. The propositus, a male born from nonconsanguinous parents, had facial asymmetry with right microtia and mandibular hypoplasia; he also had bilateral hypoplastic macula, scoliotic deformity of the thoracic spine, and ventricular septal defect. The mother was under treatment with Fluoxetine 20 mg/day prior to conception and maintained the same dosage throughout her pregnancy. The drug is a selective serotonin re-uptake inhibitor, the most widely prescribed for the treatment of depression. The occurrence of developmental aberrations may be caused by a profound serotonin receptor suppressive state in utero leading to aberrant clinical manifestations of the first and second branchial arches. Despite the very many limitations of case reporting of teratogenic events, it remains an important source of information on which more advanced research is based. Birth Defects Research (Part A) 2010. © 2010 Wiley-Liss, Inc. [source]

Descriptive epidemiologic features shared by birth defects thought to be related to vascular disruption in Texas, 1996,2002,

BIRTH DEFECTS RESEARCH, Issue 6 2008
Tasneem Husain
Abstract BACKGROUND:In utero vascular disruptions are thought to be associated with a variety of birth defects. This study examined the descriptive epidemiology of several of those defects using data from a large birth defects registry. METHODS: Data on birth defects ascertained from pregnancies in 1996,2002 were obtained from the Texas Birth Defects Registry. Using Poisson regression, we calculated crude and adjusted associations between maternal and infant characteristics and birth defects thought to be related to vascular disruption. We repeated the analysis using isolated cases and cases occurring in mothers <20 years. RESULTS: The most commonly shared pattern was observed for plurality and five defects: large intestinal atresia (PR 3.67; CI: 1.63,7.13), renal agenesis (PR 2.05; CI: 1.55,2.65), transverse limb deficiency (PR 1.85; CI: 1.28,2.57), porencephaly (PR 5.18; CI: 2.40,9.87), and Goldenhar syndrome (PR 3.45; CI: 1.04,8.53). Hispanics had the highest prevalence of gastroschisis (PR 1.21; CI: 1.05,1.40), transverse limb deficiency (PR 1.19; CI: 1.01,1.40), microtia/anotia (PR 2.22; CI: 1.83,2.70), and Poland anomaly (PR 1.90; CI: 1.26,2.93). Male infants were at greatest risk for renal agenesis (PR 1.58; CI: 1.40,1.80), porencephaly (PR 1.66; CI: 1.03,2.72), and Poland anomaly (PR 1.52; CI: 1.05,2.21). CONCLUSIONS: Our study confirmed findings in previous studies, but also uncovered several new associations. Birth Defects Research (Part A), 2008. © 2008 Wiley-Liss, Inc. [source]

Goldenhar syndrome and neuroblastoma: a chance association?

ACTA PAEDIATRICA, Issue 10 2003
C Michel-Adde
Oculoauriculovertebral dysplasia, also called Goldenhar syndrome, includes several anomalies: epibulbar dermoids or lipodermoids, microtia, mandibular hypoplasia, vertebral, skeletal, cardiac and kidney anomalies, among others. Tumours have also been observed in patients with oculoauriculovertebral dysplasia. We report the first case of oculoauriculovertebral dysplasia associated with a neuroblastoma. This tumour consists of cells identical to early migratory neural crest cells in the embryo. Several theories have been proposed regarding the pathogenetic explanation of oculoauriculovertebral dysplasia. Currently, some researchers have suggested a deficiency in mesodermal formation or defective interaction between neural crest and mesoderm as a possible aetiology. Conclusion: It is suggested that the case reported here is an additional argument for an anomaly in neural crest cell migration or interaction with the mesoderm in the pathogenesis of oculoauriculovertebral dysplasia. [source]