Keep an eye out for the bifid uvula: yes please – as it is not always just an isolated congenital anomaly

Bifid uvula is typically associated with submucous cleft palate but has often otherwise been considered innocuous, apart from dental anomalies as in your recent paper.1 The generally accepted definition of a submucous cleft palate is the triad of bifid uvula, notching of the hard palate, and muscular diastasis of the soft palate, but it can occur in the absence of a bifid uvula; most patients with bifid uvula, however, have a submucous cleft.2 Bifid uvula is more common in males than in females. Rare associations include the occurrence of bifid uvula in Loeys–Dietz syndrome3 and inflammatory linear verrucous epidermal nevus syndrome.4

It is therefore important also to exclude a diagnosis of Loeys–Dietz syndrome, a recently recognized autosomal dominant condition associated with increased transforming growth factor (TGF beta) signalling in blood vessel walls, predisposing to thoracic aortic aneurysm (TAA) dissection.5 Loeys–Dietz syndrome presents with some features similar to Ehlers-Danlos/Marfan syndromes such as: