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Case Reports
. 2023 Oct-Dec;14(4):317-321.
doi: 10.4103/ccd.ccd_139_23. Epub 2023 Dec 19.

Gorlin-Goltz Syndrome: Report of a Rare Case with an Update on the Review of the Literature

Shylaja Attur  1 , Jigar Kumar Patel  2 , Kailash Attur  3 , Sailesh Menat  4
Affiliations
Case Reports

Gorlin-Goltz Syndrome: Report of a Rare Case with an Update on the Review of the Literature

Shylaja Attur et al. Contemp Clin Dent. 2023 Oct-Dec.

Abstract

Keratocyst is a developmental odontogenic cyst arising from remnants/rests of the dental lamina with biologic behavior similar to benign neoplasm. The presence of multiple odontogenic keratocysts is rare and seen in Gorlin-Goltz syndrome (GGS). GGS syndrome presents with multisystem involvement and the classical triad of multiple basocellular epitheliomas, keratocysts in the jaws, and bifid ribs; that characterize the diagnosis of this syndrome. Multiple odontogenic keratocyst are the most consistent features of the syndrome in 65%-100% of affected individuals and are generally diagnosed at a very early age. Early diagnosis and proper counseling of the parent and patient might help to reduce the morbidity, encourage follow-up for timely treatment, and help in avoiding ionizing radiation that would lead to the development of malignancies.

Keywords: Gorlin-Goltz syndrome; multi-system disorder; multiple basal cell carcinoma; multiple odontogenic keratocyst.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Intraoral photograph showing, (a) Maxillary teeth and, (b) Mandibular teeth
Figure 2
Figure 2
Extraoral photograph showing (a) Frontal view with frontal bossing, and (b) Profile view with mandibular retrognathism and large circumference of the head, and (c) Hypertelorism with 38 mm inter inner-canthus width
Figure 3
Figure 3
Orthopantomagram showing three multilocular radiolucency, (a) The maxillary anterior region, (b) Mandibular anterior region and, (c) Mandibular left posterior region
Figure 4
Figure 4
Photograph with arrows showing multiple palmar pits
Figure 5
Figure 5
(a) Chest X-ray showing bifid fourth rib (arrow), Spina bifida (circle), and (b) Skull radiograph with bold white arrow showing calcification of falx cerebri
Figure 6
Figure 6
Photomicrograph showing histopathology of odontogenic keratocyst, (a) Maxillary lesion, (b) Mandibular left posterior region, and (c) Mandibular anterior region
Figure 7
Figure 7
Photomicrograph showing the budding of cystic lining into a capsule
Figure 8
Figure 8
Orthopantomagram after 1-year follow-up
See this image and copyright information in PMC

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