Multicystic Lesion in Jaws: A Rare Case Report on Gorlin-Goltz Syndrome

Authors

  • Pradeep Acharya BPKIHS, Dharan
  • Ashok Dongol BPKIHS, Dharan
  • Anjani Kumar Yadav BPKIHS, Dharan
  • Vivek kumar Mahato BPKIHS, Dharan
  • Rishikesh Pratap Sah JMC-Teaching Hospital, Ramdaiya, Janakpur
  • Mehul R Jaisani BPKIHS, Dharan

DOI:

https://doi.org/10.3126/jmcjms.v5i2.19017

Keywords:

Odontogenickeratocyst, Gorlin-Goltz Syndrome, Decompression, Enucleation

Abstract

Background and Objectives: Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder, which is characterized by multiple odontogenickeratocysts (OKC) and basal cell carcinomas, skeletal, dental, ophthalmic and neurological abnormalities.

Material and Methods: Thirteen years old male patient came with swelling of left maxilla obliterating buccal vestibule with pus discharge for 1 month. The orthopantomograph and computed tomography scan demonstrated multiple lytic lesions in maxilla and mandible.  Incisional biopsy was suggestive of odontogenickeratocyst (OKC). Besides multiple OKCs; bifid ribs, palmer pits and hypertelorism were present supporting for the diagnosis as Gorlin-Goltz Syndrome.

Results: The classical treatment of KCOT with enucleation with curettage or resection would have resulted in significant morbidity. Therefore we first decompressed and then enucleated the cysts.

Conclusion: Gorlin-Goltz syndrome is a rare entity and the multiple KCOT can be managed with decompression followed by enucleation.

Janaki Medical College Journal of Medical Sciences (2017) Vol. 5(2): 46-50

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Author Biographies

Pradeep Acharya, BPKIHS, Dharan

Oral and Maxillofacial Surgery

Ashok Dongol, BPKIHS, Dharan

Oral and Maxillofacial Surgery

Anjani Kumar Yadav, BPKIHS, Dharan

Oral and Maxillofacial Surgery

Vivek kumar Mahato, BPKIHS, Dharan

Oral and Maxillofacial Surgery

Rishikesh Pratap Sah, JMC-Teaching Hospital, Ramdaiya, Janakpur

Dental Surgery

Mehul R Jaisani, BPKIHS, Dharan

Oral and Maxillofacial Surgery

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Published

2018-01-16

How to Cite

Acharya, P., Dongol, A., Yadav, A. K., Mahato, V. kumar, Sah, R. P., & Jaisani, M. R. (2018). Multicystic Lesion in Jaws: A Rare Case Report on Gorlin-Goltz Syndrome. Janaki Medical College Journal of Medical Science, 5(2), 46–50. https://doi.org/10.3126/jmcjms.v5i2.19017

Issue

Section

Case Report