Bardet-Biedl syndrome and a large concha bullosa pyocele

Int J Pediatr Otorhinolaryngol. 2014 Dec;78(12):2316-8. doi: 10.1016/j.ijporl.2014.10.012. Epub 2014 Oct 25.

Abstract

Bardet-Biedl syndrome is a rare disease involving rode cone dystrophy, polydactyly, obesity, learning disabilities, hypogonadism and renal anomalies, symptoms caused by immotile cilia dysfunction. We report the first case of this syndrome in a teenager with an endonasal mass secondary to pyocele of a concha bullosa. The patient was treated successfully with endoscopic sinus surgery. Nasal obstruction secondary to a middle turbinate pyocele in Bardet-Biedl syndrome has not been described before. The pediatrician and the rhinologist should take this entity into consideration when investigating patients with Bardet-Biedl syndrome and sinonasal symptoms.

Keywords: Bardet–Biedl syndrome; Chronic rhinosinusitis; Concha bullosa; Mucocele; Pyocele.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Bardet-Biedl Syndrome / complications*
  • Endoscopy
  • Humans
  • Male
  • Mucocele / complications
  • Mucocele / surgery*
  • Nasal Obstruction / etiology
  • Nasal Obstruction / surgery*
  • Turbinates / surgery*