Multiple talon cusps, dens in dente and concrescence in a child with hypomelanosis of Ito

Feyza Nur Gorken, Ikikarakayali Goksen, Arzu Pinar Erdem, Sinem Kuru, Elif Sepet

Department of Pediatric Dentistry, Faculty of Dentistry, Istanbul University, Istanbul, Turkey.

DOI: 10.4236/ojst.2012.23037                

Abstract

Hypomelanosis of Ito (HI) is a rare neurocutaneous disorder most likely caused by chromosomal mosaicism. HI patients may suffer from numerous clinical manifestations, but the expression of the disease is highly variable. Ophthalmologic, musculoskeletal, neurologic, and dental anomalies may be associated with the syndrome. The dental abnormalities found in HI include talon cusps, a single maxillary central in-cisor, enamel defects, hypodontia, and irregularly spaced teeth. The aim of this case report is to present multiple talon cusps, dens in dente and concrescence of maxillary permanent incisors in an 8-year-old boy affected with hypomelanosis of Ito. This unusual dental anomaly has been described in two previous reports of this neurocutaneous syndrome. HI can be difficult to diagnose, these dental findings may help to identify the syndrome in cases where other findings are minimal or atypical.

Keywords

Hypomelanosis of Ito; Incontinentia Pigmenti Achromians; Talon Cusps

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	Nehal, K.S., PeBenito, R. and Orlow, S.J. (1996) Analysis of 54 cases of hypopigmentation and hyperpigmentation along the lines of Blaschko. Archives of Dermatology, 132, 1167-1170. doi:10.1001/archderm.1996.03890340027005
[2]	Gupta, S., Shah, S., Mcgaw, A., Mercado, T., Zaslav, A.L. and Tegay, D. (2007) Trisomy 2 mosaicism in hypome- lanosis of Ito. American Journal of Medical Genetics Part A, 143A, 2466-2468. doi:10.1002/ajmg.a.31940
[3]	Olson, L.L. and Maria, B.L. (2005) Hypomelanosis of Ito. In: Maria, B.L. Ed., Current Management in Child Neurology, 3rd Edition, BC Decker, Inc., London, 476-478.
[4]	Browne, R.M. and Byrne, J.P.H. (1976) Dental dysplasia in incontinentia pigmenti achromians (Ito): An unusual form. British Dental Journal, 140, 211-214. doi:10.1038/sj.bdj.4803735
[5]	Happle, R. and Vakilzadeh, F. (1982) Hamartomatous dental cusps in hypomelanosis of Ito. Clinical Genetics, 21, 65-68. doi:10.1111/j.1399-0004.1982.tb02081.x
[6]	Bartholomew, D.W., Jabs, E.W., Levin, L.S. and Ribo-vich, R. (1987) Single maxillary central incisor and colo-boma in hypomelanosis of Ito. Clinical Genetics, 32, 370-373.
[7]	Tsutsumi, T. and Oguchi, H. (1991) Labial talon cusp in a child with incontinentia pigmenti achromians: Case report. Pediatric Dentistry, 13, 236-237.
[8]	Kumar, A. and Kuriakose, S. (2001) Incontinentia pigmentii acromians with seizure disorder: A case report. Journal of the Indian Society of Pedodontics and Preventive Dentistry, 19, 145-147.
[9]	Yoon, R.K. and Chussid, S. (2007) Dental management of a talon cusp on a primary incisor. Pediatric Dentistry, 29, 51-55.
[10]	Al-Omari, M.A., Hattab, F.N., Darwazeh, A.M. and Dummer, P.M. (1999) Clinical problems associated with unusual cases of talon cusp. International Endodontic Journal, 32, 183-190. doi:10.1046/j.1365-2591.1999.00212.x
[11]	Steffen, H. and Splieth, C. (2005) Conventional treatment of dens invaginatus in maxillary lateral incisor with sinus tract: One year follow-up. Journal of Endodontics, 31, 130-133. doi:10.1097/01.don.0000137637.94281.82