An Incidental Palatine Finding: Benign or ‘Double Trouble’?

A 12-year-old girl was seen because she had had a headache for 3 days straight. There was no history of fever, sore throat, earache, or visual aura. She did not have problems with phonation or swallowing. Her past health was unremarkable. In particular, there was no history of surgery in the oral cavity. She had had 2 episodes of otitis media in the past.

During the physical examination, bifurcation of the uvula was noted. The rest of the examination results were normal.

What’s your diagnosis?

(Answer and discussion on next page)

Answer: Bifid uvula

This patient has a bifid uvula, which refers to a partial or total bifurcation of the uvula.

PREVALENCE

Bifid uvula occurs in approximately 10% to 18% of American Indians,^1-3 10% of Japanese,² 6.8% of Chinese,² 0.4% of Israelis,⁴ 0.2% to 7.5% of white Americans,^2,5,6 and 0.2% of African Americans.²

The wide variation in reported prevalence in part might be due to the different diagnostic criteria used, such as some form of uvula cleft versus full-length uvula cleft, as well as accuracy in the detection of small degrees of bifidity. 

PATHOGENESIS

This benign condition has an autosomal dominant mode of inheritance with incomplete penetration.⁷ Bifid uvula is more common in males than in females.^5,7

The condition results from an incomplete fusion of the uvula during embryonic development.

CLINICAL MANIFESTATIONS

Physical examination usually reveals a bifurcated uvula or occasionally a stunted uvula. A bifid uvula may not always be apparent on physical examination, because mucous viscosity can hold a bifid uvula together.⁸ Floating the uvula in normal saline solution may assist in the identification of this anomaly.⁸ Bifid uvula is a marker for a submucous cleft palate.⁹ In one study, bifid uvula was present in 60% of patients with submucous cleft palate.¹⁰ Bifid uvula may be associated with an absent salpingopharyngeal fold, hypoplastic eustachian tube orifices, and hypoplastic/aplastic musculus uvulae.⁸

The condition usually is asymptomatic. Some affected patients may have a hypernasal resonant speech.⁸

Bifid uvula often is an isolated malformation but can be a component manifestation in various genetic syndromes such as Loeys-Dietz syndrome,¹¹ Stickler syndrome,¹² Apert syndrome,¹³ van der Woude syndrome,¹⁴ and 22q11.2 deletion syndrome.¹⁵

COMPLICATIONS

Affected individuals, especially those with associated submucous cleft palate, are at risk for velopharyngeal insufficiency and hypernasal speech.^9,16

Children with bifid uvula may be at slightly increased risk of otitis media. A retrospective study¹⁷ based on chart review of 44 white children with bifid uvula found that children with bifid uvula, when compared with age-matched controls (n = 86), were more likely to experience one episode of acute otitis media (64% versus 49%) and more than 3 episodes of acute otitis media (16% versus 8%) during the first year of life, but the difference did not reach statistical significance. By 3 years of age, the incidence of acute otitis media in the 2 groups was nearly equal. 

In another study⁹ involving 80 children with bifid uvula, 25 children were studied in detail, of whom 12 (48%) had a history of recurrent acute otitis or persistent otitis media with effusion. Other authors⁵ did not find an increased incidence of otitis media with effusion in children with bifid uvula.

MANAGEMENT

A bifid uvula is a benign condition. No treatment is necessary. For patients with velopharyngeal insufficiency and hypernasal speech who require adenoidectomy, hypernasality may worsen postoperatively.⁹ 

In addition, asymptomatic patients with associated submucous cleft palate can develop hypernasal speech after adenoidectomy.¹⁰ As such, adenoidectomy should be avoided in patients with bifid uvula. If adenoidectomy has to be contemplated, nasopharyngoscopic investigation prior to adenoidectomy is indicated so that patients can be counseled appropriately prior to surgery.⁹

Dr Wong is a clinical assistant professor of family medicine at the University of Calgary. Dr Barankin is medical director and founder of the Toronto Dermatology Centre.

Alexander K. C. Leung, MD—Series Editor: Dr Leung is clinical professor of pediatrics at the University of Calgary and pediatric consultant at the Alberta Children’s Hospital in Calgary.

REFERENCES:

1. Cervenka J, Shapiro BL. Cleft uvula in Chippewa Indians: prevalence and genetics. Human Biol. 1970;42(1):47-52.

2. Taylor GD. The bifid uvula. Laryngoscope. 1972;82(5):771-778.

3. Jaffe BF, De Blanc GB. Cleft palate, cleft lip, and cleft uvula in Navajo Indians: incidence and otorhinolaryngologic problems. Cleft Palate J. 1970;7:300-305.

4. Chosack A, Eidelman E. Cleft uvula: prevalence and genetics. Cleft Palate J. 1978;15(1):63-67.

5. Rivron RP. Bifid uvula: prevalence and association in otitis media with effusion in children admitted for routine otolaryngological operations. J Laryngol Otol. 1989;103(3):249-252.

6. Wharton P, Mowrer DE. Prevalence of cleft uvula among school children in kindergarten through grade five. Cleft Palate Craniofac J. 1992;29(1):10-14.

7. Achalli S, Bhat S, Ram Shetty S, Babu SG, Suvarna R. Deformities of the uvula in the oral cavity- a case series. Iran Red Crescent Med J. 2012;14(10):676-679.

8. Suryadevara AC, Tatum SA. Floating the uvula: an intraoperative method for detecting bifidity. Int J Pediatr Otorhinolaryngol. 2007;71(1):175-177.

9. Shprintzen RJ, Schwartz RH, Daniller A, Hoch L. Morphologic significance of bifid uvula. Pediatrics. 1985:75(3):553-561.

10. Reiter R, Brosch S, Wefel H, Schlömer G, Haase S. The submucous cleft palate: diagnosis and therapy. Int J Pediatr Otorhinolaryngol. 2011;75(1):
85-88.

11. Pezzini A, Del Zotto E, Giossi A, Volonghi I, Costa P, Padovani A. Transforming growth factor β signaling perturbation in the Loeys-Dietz syndrome. Curr Med Chem. 2012;19(3):454-460.

12. Lituania M, Tonni G. Bifid uvula and familial Stickler syndrome diagnosed prenatally before the sonographic “equals sign” landmark. Arch Gynecol Obstet. 2013;288(3):483-487.

13. Costa FWG, Rodrigues RR, Batista ACB, Ribeiro TR, Pereira KMA. Multiple radiopaque mandibular lesions in a patient with Apert syndrome. J Endod. 2012;38(12):1639-1643.

14. More CB, Varma S, Tailor M, Bhavsar K. Van der Woude syndrome: report of two cases with supplementary findings. Indian J Dent Res. 2013;24(3):
387-389.

15. Fagerberg CR, Graakjaer J, Heinl UD, et al. Heart defects and other features of the 22q11 distal deletion syndrome. Eur J Med Genet. 2013;56(2):98-107.

16. Oji T, Sakamoto Y, Ogata H, Tamada I, Kishi K. A 25-year review of cases with submucous cleft palate. Int J Pediatr Otorhinolaryngol. 2013;77(7):1183-1185.

17. Schwartz RH, Hayden GF, Rodriquez WJ, Shprintzen RJ, Cassidy JW. The bifid uvula: is it a marker for an otitis prone child? Laryngoscope. 1985;95(9 pt 1):1100-1102.