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Laryngotracheoesophageal cleft type 1
Disease definition
A congenital respiratory tract anomaly characterized by a supraglottic, interarytenoid cleft above the vocal folds with moderate respiratory symptoms.
ORPHA:93938
Classification level: Subtype of disorder- Synonym(s):
- LTEC I
- LTEC1
- Laryngo-tracheo-esophageal cleft type 1
- Prevalence: Unknown
- Inheritance: -
- Age of onset: Infancy, Neonatal
- ICD-10: Q32.1
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
Summary
Epidemiology
Prevalence of this form of LC is difficult to ascertain because of the moderate nature of the condition, but it is thought to be rare.
Clinical description
Clinical signs include stridor, a toneless or hoarse cry, swallowing disorders such as aspirations, cough, dyspnea, cyanosis during feeding and gastro-esophageal reflux.
Etiology
The causes underlying development of this anomaly are unknown.
Management and treatment
In children with mildly symptomatic type 1 LC, management includes maintaining adequate ventilation, feeding with thickened food, treatment of gastro-esophageal reflux, and maintaining a postprandial upright position. Children with moderate symptoms may also benefit from nasogastric tube feeding. Treatment initially involves conservative measures such as anti-reflux therapy, thickened feeds, and maneuvers during feeding to prevent aspiration. In cases of failure of a conservative approach, surgical correction of the cleft, using an endoscopic treatment approach, is recommended.
Detailed information
Professionals
- Summary information
- Polski (2011, pdf)
- Review article
- English (2011)
Additional information