International Journal of Clinical Pediatric Dentistry

Register      Login

VOLUME 6 , ISSUE 2 ( May-August, 2013 ) > List of Articles

CASE REPORT

Gorlin and Goltz Syndrome: A Case Report with Surgical Review

Rakesh N Bahadure, Eesha Surendraji Jain, Gautam P Badole

Keywords : Basal cell carcinoma, Gorlin syndrome, Nevoid basal cell carcinoma, Odontogenic keratocysts, Palmar/Plantar pits, Bifid rib, Internal strabismus

Citation Information : Bahadure RN, Surendraji Jain E, P Badole G. Gorlin and Goltz Syndrome: A Case Report with Surgical Review. Int J Clin Pediatr Dent 2013; 6 (2):104-108.

DOI: 10.5005/jp-journals-10005-1199

License: CC BY-NC 4.0

Published Online: 01-12-2017

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


Abstract

Gorlin and Goltz syndrome are a very complex syndrome and a multisystemic process that is characterized by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and/or plantar pits and calcification of the falx cerebri. Along with these major features a great number minor features have also been described which involves numerous skeletical, dermatology related, neurological, ophthalmological and reproductive anomalies. It exhibits high penetrance and variable expressivity. Presented here is the case of Gorlin-Goltz in a 12 years old male patient which was diagnosed through its oral and maxillofacial manifestations. Treatment of odontogenic keratocyst was done by enucleation without primary suturing. Iodoform dressing was kept to enhance the healing and to reduce the recurrence of the lesion. It is important to provide the early diagnosis for detection of clinical and radiological manifestations in young patients and for provision of advice concerning preventive treatment like protection of the skin from the sunlight and genetic sensitivity testing so that possible complications associated with this syndrome can be prevented. How to cite this article: Bahadure RN, Jain ES, Badole GP. Gorlin and Goltz Syndrome: A Case Report with Surgical Review. Int J Clin Pediatr Dent 2013;6(2):104-108.


PDF Share
  1. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cyst and bifid rib: A syndrome. New Engl J Med 1960 May 5;262:908-912.
  2. Jarisch W. Zurlehre von den hautgeschwulsten. Archivfür Dermatologie und Syphilis 1894;28:163-165.
  3. Ward WH. Naevoid basal celled carcinoma associated with a dyskeratosis of the palms and soles. A new entity. Aust J Dermatol 1960 Dec;5:204-208.
  4. Agaram NP, Collins BM, Barnes L, Lomago D, Aldeeb D, Swalsky P, Finkelstein S, Hunt JL. Molecular analysis to demonstrate that odontogenickeratocysts are neoplastic. Arch Pathol Lab Med 2004 Mar;128(3):313-317.
  5. Straith FE. Hereditary epidermoid cyst of the jaws. Am J Orthod Oral Surg 1939;25:673-677.
  6. Bettley FR. Two cases of multiple nevoid basal cell ephiteliomata? Porokeratosis de Mantoux. Br J Dermatol 1953 Jun;65(6):219-221.
  7. A 43-year-old man with multiple recurrent basal cell carcinomas. Baltimore: Johns Hopkins University School of Medicine; c2000-2007 [updated: 2007 Aug 10; cited: 2007 Aug 30]. Available from: DermAtlas.org.
  8. Gu XM, Zhao HS, Sun LS, Li TJ. PTCH mutations in sporadic and Gorlin-syndrome-related odontogenic keratocysts. J Dent Res 2006 Sep;85(9):859-863.
  9. Ramaglia L, Morgese F, Pighetti M, Saviano R. Odontogenic keratocyst and uterus bicornis in nevoid basal cell carcinoma syndrome: Case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006 Aug;102(2):217-219.
  10. 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 May;130(5):669-674.
  11. Díaz-Fernández JM, Infante-Cossío P, Belmonte-Caro R, Ruiz- Laza L, García-Perla-García A, Gutiérrez-Pérez JL. Basal cell nevus syndrome. Presentation of six cases and literature review. Med Oral Patol Oral Cir Bucal. 2005 Apr 1;10 Suppl 1:E57- E66.
  12. Marsh A, Wicking C, Wainwright B, Chenevix-Trench G. DHPLC analysis of patients with nevoid basal cell carcinoma syndrome reveals novel PTCH missense mutations in the sterolsensing domain. Hum Mutat 2005 Sep;26(3):283.
  13. Rayner CR, Towers JF, Wilson JS. What is Gorlin's syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirty-seven patients. Br J Plast Surg 1977 Jan;30(1):62-67.
  14. White JC. Multiple benign cystic ephiteliomata. J Cutan Dis 1894;12:477-481.
  15. Barreto DC, Chimenos Kustner E. New considerations about the diagnosis of odontogenic keratocysts. Med Oral 2001 Nov- Dec;6(5):350-357.
  16. Reyes Macias JF, Bagán Sebastián JV. Síndrome de Gorlin- Goltz Revisión de la literatura y reporte de uncaso. Rev Europ Odonto Estomatol 2002 March;14(2):105-112.
  17. Weber AL. Imaging of cysts and odontogenic tumors of the jaw. Definition and classification. Radiol Clin North Am 1993 Jan;31(1):101-120.
  18. Voorsmit RA, Stoelinga PJ, van Haelst UJ. The management of keratocysts. J Maxillofac Surg 1981 Nov;9(4):228-236.
  19. Lo Muzio L, Nocini PF, Savoia A, Consolo U, Procaccini M, Zelante L, Pannone G, Bucci P, Dolci M, Bambini F, Solda P, Favia G. Nevoid basal cell carcinoma syndrome. Clinical findings in 37 Italian affected individuals. Clin Genet 1999 Jan;55(1):34-40.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.