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Bifid uvula is a fissure type embryopathy affecting the uvula at the back of the soft palate.
ORPHA:99771Classification level: Disorder
- Bifidity of the uvula
- Uvular cleft
- Prevalence: Unknown
- Inheritance: Multigenic/multifactorial or Not applicable
- Age of onset: Infancy, Neonatal
- ICD-10: Q35.7
- ICD-11: LA42.2
- OMIM: -
- UMLS: C0266122
- MeSH: -
- GARD: -
- MedDRA: -
The prevalence is unknown.
The cleft in the uvula is median. The clinical forms range from a simple posterior notch to the complete duplication of the uvula. Bifid uvula has no clinical repercussions.
This embryopathy appears in the 7th to 12th week of pregnancy following an error in the fusion of the palatine process, which usually involves a fusion that occurs from the front to back of the hard palate, the soft palate and then the uvula. Genetic, environmental and toxic factors are all possible causes.
Although bifid uvula is usually isolated, it can also been seen in syndromic forms. Usually discovered by chance, it is important to eliminate any association with a submucous cleft velum. A submucous cleft velum is a specific form of cleft palate where a cleft in the muscular tissue is covered by the oral and nasal mucosa.
Management and treatment
When bifid uvula is isolated, there are no clinical repercussions and it can be considered as a familial genetic trait. In the isolated forms, once an associated submucous cleft has been ruled out, there is no therapeutic management necessary in the absence of clinical manifestations.
A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Português (2011)