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A rare case of Proteus syndrome

Published online by Cambridge University Press:  18 February 2008

N D Zingade  and
N N Zingade
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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

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References

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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
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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