RESUMEN
OBJECTIVE: This study described the occurrence and expression of pharyngeal and laryngeal anomalies in a population of patients with oculo-auriculo-vertebral spectrum (OAVS). DESIGN: Cross-sectional study using chart review, physical examination, and endoscopic evaluation of the pharynx and larynx, and perceptual and aerodynamic evaluation of speech for assessment of vocal tract function. SETTING: Academic tertiary referral center. PATIENTS: Chart review of 41 patients with a diagnosis of OAVS and clinical evaluation of 23 of the patients identified in the initial chart review, ranging in age from 9 months to 17 years. MAIN OUTCOME MEASURES: Data from chart review pertaining to airway and speech symptoms. Physical examination and endoscopic studies of pharyngeal and laryngeal structure and function and perceptual and aerodynamic evaluation of speech, resonance, and voice. RESULTS: Chart review showed a high occurrence of indicators of pharyngeal and laryngeal abnormalities such as velopharyngeal inadequacy and airway obstruction. Clinical evaluations documented a variety of abnormalities in pharyngeal and laryngeal structure and function as well as impairment in speech articulation, resonance, and voice. Presence and severity of pharyngeal and laryngeal abnormalities were not correlated with the severity of expression of the spectrum based on mandibular morphology. CONCLUSIONS: Oculo-auriculo-vertebral spectrum appears to be associated with a high occurrence of structural and functional abnormalities of the pharynx and larynx that may contribute to increased risk of airway obstruction, communication impairment, and morbidity.
Asunto(s)
Síndrome de Goldenhar/patología , Laringe/anomalías , Faringe/anomalías , Adolescente , Obstrucción de las Vías Aéreas/etiología , Niño , Preescolar , Estudios Transversales , Endoscopía , Estudios de Evaluación como Asunto , Femenino , Humanos , Lactante , Laringoscopía , Laringe/patología , Laringe/fisiopatología , Masculino , Mandíbula/anomalías , Mandíbula/patología , Faringe/patología , Faringe/fisiopatología , Examen Físico , Estudios Retrospectivos , Habla/fisiología , Trastornos del Habla/etiología , Insuficiencia Velofaríngea/etiología , Pliegues Vocales/fisiopatología , Voz/fisiología , Calidad de la Voz/fisiologíaRESUMEN
The reconstruction of a traumatic telecanthus, particularly the repositioning and securing of the medial canthal tendon, presents a challenge to the reconstructive surgeon. The adequate positioning of the medial canthal tendon for proper intercanthal distance, and apposition of the lid to the globe, is the cornerstone of a successful reconstruction. The authors have developed a technique for transnasal canthoplasty that is fast, relatively easy, and safe. Transnasally, a 16-gauge spinal needle is introduced over a preplaced K-wire using a 4-0 Bunnell stainless wire suture (Ethicon, Somerville, NJ). The medial canthal tendon is lassoed, secured, and then fixed to the contralateral nasal bone. Six patients have undergone this technique to date. The authors believe this procedure offers an improvement to existing methods.