Hostname: page-component-586b7cd67f-rcrh6 Total loading time: 0 Render date: 2024-11-28T07:31:17.644Z Has data issue: false hasContentIssue false
  • English
  • Français

A rare case of Proteus syndrome

Published online by Cambridge University Press:  18 February 2008

N D Zingade  and
N N Zingade
Get access Rights & Permissions [Opens in a new window]

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.

Keywords

DysphagiaHypertrophyExostosisNevusProteus Syndrome
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 3 , March 2008 , e7
Copyright
Copyright © JLO (1984) Limited 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1 Cohen, MM Jr, Hayen, PWA. Newly recognized hamartomatous syndrome. Birth Defects 1979;15:291–6Google ScholarPubMed
2 Turner, JT, Cohen, MM Jr, Biesecker, LG. A reassessment of the Proteus syndrome literature: application of diagnostic criteria on published cases. Am J Med Genet 2004;130A:111–22CrossRefGoogle ScholarPubMed
3 Biesecker, LG. The multifaceted challenges of Proteus syndrome. JAMA 2001;285:2240–3CrossRefGoogle ScholarPubMed
4 Biesecker, LG, Happle, R, Mulliken, JB, Weksberg, R, Graham, JM Jr, Viljoen, DL et al. Proteus syndrome: diagnostic criteria, differential diagnosis and patient evaluation. Am J Med Genet 1999;84:389–953.0.CO;2-O>CrossRefGoogle ScholarPubMed
5 Sahni, JK, Kumar, S, Wadhwa, V, Kathuria, G. Proteus syndrome with huge tonsillar mass causing dysphagia: a rare case. J Laryngol Otol 2006;120:408–10Google ScholarPubMed