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References

Alroyayamina S, McKnight M. Dent Update. 2015; 42:247-249
Shprintzen RJ, Schwartz RH, Daniller A, Hoch L. Morphologic significance of bifid uvula. Pediatrics. 1985; 75:(3)553-561
Yang JH, Ki CS, Han H, Song BG, Jang SY, Chung TY, Sung K, Lee HJ, Kim DK. Clinical features and genetic analysis of Korean patients with Loeys–Dietz syndrome. J Hum Genet. 2012; 57:52-56
Kumar AC, Yeluri G, Raghav N. Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation – a rare case report. Contemp Clin Dent. 2012; 3:119-122

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

From Volume 42, Issue 5, June 2015 | Page 494

Authors

Carlos Ibrahim

(KCL)

(WHO Collaborating Centre for Oral Health-General Health)

Articles by Carlos Ibrahim

Crispian Scully

CBE, DSc, DChD, DMed (HC), Dhc(multi), MD, PhD, PhD (HC), FMedSci, MDS, MRCS, BSc, FDS RCS, FDS RCPS, FFD RCSI, FDS RCSEd, FRCPath, FHEA

Bristol Dental Hospital, Lower Maudlin Street, Bristol BS1 2LY, UK

Articles by Crispian Scully

Article

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:

  • Bifid uvula and/or submucous cleft palate;
  • Arterial aneurysms;
  • Bicuspid aortic valve;
  • Osseous anomalies (eg scoliosis, club foot);
  • Hypertelorism;
  • Allergies.

    • Complications of Loeys–Dietz syndrome include a predisposition to arterial rupture, cervical spine instability and, for women, increased risks during or after pregnancy from aortic dissection or uterine rupture.

    Thus a thorough clinical examination and accurate medical history, and awareness of the systemic implications of apparently innocuous conditions, such as bifid uvula, allows for a possible diagnosis of a syndrome which can have serious implications for systemic health. Investigations, including echocardiography, head to pelvis 3D CT angiogram scan for evidence of vascular pathology, and mutation testing for Loeys–Dietz syndrome, may be indicated and the angiotensin II type 1 receptor antagonist, losartan, might be considered for prophylaxis.