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Nevoid Basal Cell Carcinoma Syndrome Presenting with Neck Pits and Café au Lait Patches

  • Sathesh Balasundram1,*,
  • Ferdinand Jesudian Kovilpillai1
  • Colin Hopper1

1Oral and Maxillofacial Surgery Unit, Eastman Dental Institute, University College London, London, United Kingdom

2Head of Oral Surgery Unit, Taiping Hospital, Taiping, Perak, Malaysia.

DOI: 10.17796/jcpd.35.1.r7t6t25372830426 Vol.35,Issue 1,January 2011 pp.95-100

Published: 01 January 2011

*Corresponding Author(s): Sathesh Balasundram E-mail: satbala@yahoo.com

Abstract

A 10- year-old patient presented with a slow growing jaw swelling. The initial general examination did not reveal any significant findings. Method: Conservative enucleation of the cyst confirmed it to be an odontogenic keratocyst. The patient remained asymptomatic for the following 2 years and subsequently presented cystic lesions in jaws with displaced teeth. These cysts were enucleated and were confirmed to be odontogenic keratocysts . The patient has been on regular follow up since then and subsequent scans have shown further occurrence of cysts in the jaws with displacement of the third molars. Results: Clinical examination also revealed macrocephaly, fronto-parietal bossing, pitting on palmar and plantar surfaces, calcification of falx cerebri and splayed ribs, confirming the diagnosis of Nevoid Basal Cell Carcinoma Syndrome. He also presented with a café au lait patch and skin pits on the neck. The family history was negative for features of nevoid basal cell carcinoma syndrome. Conclusion: Nevoid basal cell carcinoma syndrome is a condition that can cause significant morbidity if not detected early. Over the years this syndrome has presented with many other non specific phenotype presentation, of which the current finding may be one of. This calls for meticulous assessment and examination of patients and a standardized protocol in screening and managing these patients that may facilitate a more beneficial outcome for the patient.

Keywords

Gorlin Goltz syndrome, nevoid basal cell carcinoma, odontogenic keratocyst

Cite and Share

Sathesh Balasundram,Ferdinand Jesudian Kovilpillai,Colin Hopper. Nevoid Basal Cell Carcinoma Syndrome Presenting with Neck Pits and Café au Lait Patches. Journal of Clinical Pediatric Dentistry. 2011. 35(1);95-100.

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