A rare case of Proteus syndrome

J Laryngol Otol. 2008 Mar;122(3):e7. doi: 10.1017/S002221510700151X. Epub 2008 Feb 18.

Abstract

Introduction: We report a rare case of Proteus syndrome which presented with dysphagia due to unilateral tonsillar hypertrophy.

Case report: Our case had involvement of multiple organs in addition to bony deformities, namely, enlarged right parotid gland and right testis, verrucous naevus, varicose veins and abdominal lipoma. The features unique to this case were a larger right optic disc and expansion of the inner table of the skull.

Discussion: A literature review revealed less than 100 bona fide, published cases which fit the revised diagnostic criteria proposed by Turner.

Summary: The ENT manifestations of Proteus syndrome are high arched palate, gingival hypertrophy, malocclusion and overcrowding of teeth, hyperostosis of the external auditory meatus, and low nasal bridge. Our patient had a unilateral enlargement of the tonsil causing dysphagia, which was relieved by tonsillectomy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Deglutition Disorders / pathology*
  • Deglutition Disorders / surgery
  • Facial Asymmetry / congenital*
  • Facial Asymmetry / psychology
  • Humans
  • Male
  • Palatine Tonsil / pathology*
  • Palatine Tonsil / surgery
  • Proteus Syndrome / pathology*
  • Proteus Syndrome / psychology
  • Treatment Outcome