International Journal of Clinical Pediatric Dentistry

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VOLUME 6 , ISSUE 3 ( September-December, 2013 ) > List of Articles

CASE REPORT

Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report

Sonu Acharya, Swagatika Panda, Kanika Singh Dhull, Ranjan Sujit Sahoo

Keywords : Basal cell nevus syndrome, Carcinoma, Odontogenic keratocyst, Gorlin-Goltz syndrome

Citation Information : Acharya S, Panda S, Singh Dhull K, Sahoo RS. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013; 6 (3):208-212.

DOI: 10.5005/jp-journals-10005-1221

License: CC BY-NC 4.0

Published Online: 01-10-2018

Copyright Statement:  Copyright © 2013; The Author(s).


Abstract

Gorlin's syndrome is a rare disorder transmitted as an autosomal dominant trait. It is characterized by multiple disorders involving multiple systems. We present a case of 11-year-old male child presenting with multiple odontogenic keratocyst to the dental clinic. Retrograde diagnosis of Gorlin-Goltz syndrome was made after clinical and radiological investigation. How to cite this article: Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013;6(3):208-212.


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  1. Ozturk A, Oguz KK, Tumer C, Balci S. Neuroradiological findings in a mother and daughter with Gorlin syndrome. Clin Dysmorphol 2003;12(2):145-146.
  2. Patil K, Mahima VG, Gupta B. Gorlin syndrome: a case report. J Indian Soc Pedod Prev Dent 2005;23(4):198-203.
  3. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, Bale AE, Bale SJ. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69(3):299-308.
  4. Lo Muzio L. Nevoid basal cell carcinoma syndrome (Gorlin syndrome). Orphanet J Rare Dis 2008;3:32.
  5. Gorlin RJ. Nevoid basal cell carcinoma (Gorlin) syndrome. Genet Med 2004;6(6):530-539.
  6. Weber AL, Kaneda T, Scrivani, SJ, Aziz S. Basal cell nervous syndrome (Gorlin's or Gorli-Goltz syndrome). In: Som, Peter M.; Curtin, Hugh D., editors. Head and neck imaging. 4th ed. Vol. 2. St. Louis: Mosby; 2003. 938 p.
  7. Melo ES, Kawamura JY, Alves CA, Nunes FD, Jorge WA, Cavalcanti MG. Imaging modality correlations of an odontogenickeratocyst in the nevoid basal cell carcinoma syndrome: a family case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98(2):232-236.
  8. Kohli M, Kohli M, Sharma N, Siddiqui SR, Tulsi SPS. Gorlin- Goltz syndrome. Natl J Maxillofac Surg 2010;1(1):50-52.
  9. Manfredi M, Vescovi P, Bonanini M, Porter S. Nevoid basal cell carcinoma syndrome: a review of the literature. Int J Oral Maxillofac Surg 2004;33(2):117-124.
  10. Shanley S, Ratcliffe J, Hockey A, Haan E, Oley C, Ravine D, Martin N, Wicking C, Chenevix-Trench G. Nevoid basal cell carcinoma syndrome: review of 118 affected individuals. Am J Med Genet 1994;50(3):282-290.
  11. Jarish W. Zurlehre von den autgeschwulsten. Archiv Jur Dermatologic Syphilogic 1894;28:163-222.
  12. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid ribs. N Engl J Med 1960;262:908-912.
  13. Farndon PA, Del Mastro RG, Evans DG, Kilpatrick MW. Location of the gene for Gorlin syndrome. Lancet 1992; 339(8793):581-582.
  14. Evans DG, Ladusans EJ, Rimmer S, Burnell LD, Thakker N, Farndon PA. Complications of the naevoid basal cell carcinoma syndrome: Results of a population based study. J Med Genet 1993;30(6):460-464.
  15. Kimonis VE, Mehta SG, Digiovanna JJ, Bale SJ, Pastakia B. Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome. Genet Med 2004;6(6):495-502.
  16. Lo Muzio L, Nocini P, Bucci P, Pannone G, Consolo U, Procaccini M. Early diagnosis of nevoid basal cell carcinoma syndrome. J Am Dent Assoc 1999;130(5):669-674.
  17. Barnes L, Eveson JW, Reichart PA, Sidransky D, editors (2005) Pathology and genetics of head and neck tumors. World Health Organization classification of tumors. Lyon: IARC Press. 430 p.
  18. Levanat S, Gorlin RJ, Fallet S, Johnson DR, Fantasia JE, Bale AE. A two-hit model for developmental defects in Gorlin syndrome. Nat Genet 1996;12(1):85-87.
  19. Li JT. The Odontogenic keratocyst: a cyst, or a cystic neoplasm? J Dent Res 2011;90(2):133-142.
  20. Lo Muzio L, Nocini PF, Savoia A, Consolo U, Procaccini M, Zelante L, Pannone G, Bucci P, Dolci M, Bambini F, et al. Nevoid basal cell carcinoma syndrome. Clinical findings in 37 Italian affected individuals. Clin Genet 1999;55(1):34-40.
  21. Walter AW. Gorlin syndrome. Available at: www.emedicine. com/ped/topic890.htm
  22. Rai S, Gauba K. Jaw cyst-basal cell nevus-bifid rib syndromea rare case report. J Indian Soc Pedo Prev Dent 2007;25(3): 137-139.
  23. Jensen MJ. Gorlin's syndrome with ameloblastoma. Oral Surg 1978;45:325-326.
  24. Ramsden RJ, Barrett A. Gorlin's syndrome. J Laryngol Otol 1975;89(6):615-629.
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