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Acta Otorrinolaringológica Española
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Vol. 57. Issue 9.
Pages 425-428 (November 2006)
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Vol. 57. Issue 9.
Pages 425-428 (November 2006)
Rinolalia abierta, un síntoma característico del síndrome velocardiofacial
Open rinolalia a typical symptom of velo-cardiofacial syndrome
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6066
C. Morales-Angulo
,1
Corresponding author
cmorales@mundivia.es

Correspondencia: Carmelo Morales Angulo Alta de Veneras, 8 39478 Puente Arce (Cantabria)
* Servicio de ORL. Hospital Sierrallana. Torrelavega. Servicio de ORL
J. Gallo-Terán
Hospital Nacional Marqués de Valdecilla. Santander
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Resumen

El síndrome velocardiofacial (VCF) es una cromosomopatía relativamente frecuente que habitualmente asocial varias manifestaciones otorrinolaringológicas, como rinolalia abierta severa, secundaria a un paladar hendido submucoso, rasgos faciales característicos y anomalías auriculares. Se presenta el caso clínico de una paciente con síndrome VCF que antes de ser diagnosticada había sido sometida a una adenoidectomía y presentando en el postoperatorio un empeoramiento severo del lenguaje. Se resumen los datos de sospecha del síndrome VCF y se propone un protocolo de estudio para conseguir un diagnóstico precoz en estos pacientes y evitar intervenciones iatrogénicas.

Palabras clave:
Síndrome Velo-Cardio-Facial
Paladar hendido submucoso
Malformaciones craneofaciales
Rinolalia abierta
Abstract

The Velocardiofacial (VCF) syndrome is a relatively frequent cromosomopathy that usually associates various otorhinolaryngological features, as hipernasal speech, typical facies and auricular anomalies. We report a patient with VCF syndrome that before being diagnosed had undergone adenoidectomy with a postoperative worsening in speech. Otorhinolaryngological clinical features of the VCF syndrome are discussed and a diagnostic protocol is proposed to achieve an early diagnosis and to prevent iatrogenic interventions in these patients.

Key words:
Velo-cardio-facial syndrome
Submucous cleft palate
Craniofacial malformation
Hypernasal speech

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