A Rare Case of Gorlin-Goltz Syndrome Presented to the Emergency Department as Facial Swelling
Abstract
Introduction: Gorlin-Goltz syndrome (GGS), also known as basal cell nevus syndrome, is a very rare autosomal dominant inherited disorder that is characterized by the development of numerous basal cell carcinoma. This article reports a case of GGS, emphasizing its clinical and radiographic manifestations. Case presentation: We report here the case of a 35-year-old man who visited the maxillofacial emergency department due to left facial swelling. According to his clinical and radiographic examination we diagnosed him with GGS with no family history. The patient has multiple odontogenic keratocysts, rib anomalies, calcifications of the falx cerebri, lower jaw prognathism, frontal bossing, macrocephaly, and thick eyebrows. Conclusion: A definitive diagnosis of GGS should be made by a multidisciplinary team including a maxillofacial surgeon and medical specialists. Early diagnosis, treatment, and regular follow up are important to decrease complications, including oromaxillofacial deformation and destruction, and possible malignancy.
2. Neville B, Damm D, Allen C, Bouquot J. Oral and Maxillofacial Pathology. 3rd ed. Philadelphia: WB Saunders; 2009.
3. Kimonis VE, Goldstein AM, Pastakia B, Yang M, Kase R, DiGiovanna J, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet. 1997;69(3):299-308.
4. Kolm I, Puig S, Iranzo P, Malvehy J. Dermoscopy in Gorlin‐Goltz Syndrome. Dermatol Surg. 2006;32(6):847-51.
5. Rózyło-Kalinowska I, Rózyło T, editors. Odontogenic keratocyst in Gorlin-Goltz syndrome. Ann Univ Mariae Curie Sklodowska Med. 2002;57(2):79-85.
6. Yordanova I, Gospodinov DK. A familial case of Gorlin-Goltz syndrome. J of IMAB. 2007;13(1):63-7.
7. Dixit S, Acharya S, Dixit P. Multiple odontogenic keratocysts associated with Gorlin-Goltz syndrome. Kathmandu Univ Med J. 2009;7(4):414-8.
8. Kannan KS, Sundharam SB, Manikandan R. Nevoid basal cell carcinoma syndrome. Indian J Dent Res. 2006;17(1):50.
9. Evans D, Ladusans E, Rimmer S, Burnell L, Thakker N, Farndon P. Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. J Med Genet. 1993;30(6):460-4.
10. 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.
11. Sunder SN, Babburi S, Guduguntla S, Raju P. Gorlin-Goltz syndrome: A rare case report. J Dr NTR Univ Health Sci. 2013;2(2):150.
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Issue | Vol 2 No 4 (2018): Autumn (October) | |
Section | Case (report / study) | |
PMCID | PMC6548149 | |
PMID | 31172109 | |
Keywords | ||
Basal cell nevus syndrome Case reports Odontogenic cysts Oral and maxillofacial surgeons |
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |