Proteus Syndrome: A Rare Entity

Madan Nanjappa; R Keerthi; SV Kumaraswamy; G Anup Kumar

doi:10.5005/jp-journals-10015-1023

SEARCH WITHIN CONTENT

Online First

Proteus Syndrome: A Rare Entity

Madan Nanjappa, R Keerthi, SV Kumaraswamy, G Anup Kumar

Citation Information : Nanjappa M, Keerthi R, Kumaraswamy S, Anup Kumar G. Proteus Syndrome: A Rare Entity. World J Dent 2010; 1 (2):113-116.

DOI: 10.5005/jp-journals-10015-1023

Published Online: 01-12-2011

ARTICLE
REFERENCES

Abstract

The Proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations, sometimes involving the face. Common manifestations include partial gigantism, congenital lipomas, and plantar hyperplasia. In this report, we describe a case of an 18-year-old male patient with Proteus syndrome, who had pronounced hemifacial hypertrophy, exostoses in the left parietal region, and enlargement of the inferior alveolar nerve and mandibular canal in the affected region. The dental development of the affected left mandible and maxilla was characterized by extremely premature development and eruption of the permanent teeth. The proposed multidisciplinary management of the patient and a review of literature of the Proteus patients, who exhibited manifestation in the craniofacial region is discussed in this report.

PDF Share

A newly recognized Harmartomatous syndrome. In O'Donnell JJ, Hall BD (Eds). Penetrance and variability in malformation syndromes. Birth Defects 1979;15(5B):291-96.
The Proteus syndrome, partial gigantism of the hands and feet, nevi, hemihypertrophy, subcutaneous tumours, macrocephaly, skull anomalies and possible accelerated growth and visceral affections. Eur J Pediatr 1983;140:5-12.
Craniofacial and dental manifestations of Proteus Syndrome: A Case Report: Cleft Palate–Craniofacial Journal, March 2002;39(2):233-45.
Proteus syndrome: Diagnostic criteria, differential diagnosis, and patient evaluation. Am J Med Genet 1999;84:389-95.

PDF Share