Neonatal Presentation and Follow-up of Goldenhar Syndrome / 대한주산의학회잡지
Korean Journal of Perinatology
;
: 9-16, 2014.
Article
Dans Coréen
| WPRIM
| ID: wpr-76050
ABSTRACT
PURPOSE:
Expression patterns, associated anomalies and progress of the patients with Goldenhar syndrome from the neonatal period were systematically investigated. This allows us to evaluate the need for early diagnosis.METHODS:
This is a retrospective study of 29 infants with Goldenhar syndrome whose diagnosed in Samsung Medical Center between 1994 and 2013. Associated anomalies and procedures between neonatal group (n=13) and non-neonatal group (n=16) were systematically compared.RESULTS:
Mean gestational age in the neonatal group were 38+1+/-2+4 weeks and 3 patients (23%) were preterm infants. The average birth weight in the neonatal group were 2,853+/-544 grams. Goldenhar syndrome was mainly diagnosed by ear and face anomalies during the neonatal period. The associated anomalies in neonatal group were cardiovascular anomaly (54%), genitourinary anomaly (30%), vertebral anomaly (15%), and others (31%). About 40% of patients who had long-term follow-up revealed hearing abnormalities and about 1/4 of all patients had bilateral hearing problem, which resulted in requiring hearing aid devices. In addition, the most common procedure performed during follow-up was preauricular skin tag removal. And other procedures or surgery related to oral, eyes, and others were performed in each 1/4 of the patients. Cardiac surgery was done in 15% of total patients. Frequency of associated anomalies and performed procedures between the patients diagnosed at neonatal and non-neonatal period was not significantly different.CONCLUSION:
A multidisciplinary approach should be undertaken by multi-departments when evaluating patients with Goldenhar syndrome. In particular if the patient has an ear anomaly, careful hearing test is required in early life.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Peau
/
Chirurgie thoracique
/
Poids de naissance
/
Prématuré
/
Études rétrospectives
/
Études de suivi
/
Âge gestationnel
/
Diagnostic précoce
/
Oreille
/
Syndrome de Goldenhar
Type d'étude:
Etude diagnostique
/
Étude observationnelle
/
Étude pronostique
/
Facteurs de risque
/
Étude de dépistage
Limites du sujet:
Humains
/
Bébé
/
Nouveau-né
langue:
Coréen
Texte intégral:
Korean Journal of Perinatology
Année:
2014
Type:
Article
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