Presentation and management of syndromic and non-syndromic patients with multiple odontogenic keratocysts
<h3>Objective</h3><p dir="ltr">This case series reports the clinical presentation and management of multiple <u>odontogenic keratocysts</u> (OKCs) in patients with <u>Gorlin Goltz</u> syndrome and in non-syndromic patients. </p><h3>Mate...
محفوظ في:
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| مؤلفون آخرون: | , , , |
| منشور في: |
2022
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| الموضوعات: | |
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| الملخص: | <h3>Objective</h3><p dir="ltr">This case series reports the clinical presentation and management of multiple <u>odontogenic keratocysts</u> (OKCs) in patients with <u>Gorlin Goltz</u> syndrome and in non-syndromic patients. </p><h3>Material and methods</h3><p dir="ltr">Eight patients presented with features of multiple odontogenic keratocyst at department of Oral and Maxillofacial Surgery. The diagnosis was confirmed following clinical, radiographic and histopathological examination. Initially the diagnosis of OKC was confirmed on an incisional biopsy. Major and minor criteria were followed for the diagnosis of Gorlin Goltz syndrome. Smaller cysts in all patients were enucleated and for larger cysts <u>marsupialization </u>was planned either alone or followed by <u>enucleation</u>. Patients were followed at 1 month, 3 months, 6 months and yearly interval to check for bone healing and recurrences. </p><h3>Results</h3><p dir="ltr">Association of Gorlin Goltz syndrome was identified in four patients all of whom were males with age range of 12–37 years. Among four patients that had non-syndromic OKCs, three were female and one was male with age range of 09–50 years; two patients had familial non-syndromic multiple OKCs. In syndromic patients, multiple OKCs, recognized manifestations of Gorlin Goltz syndrome were identified with variable frequency: calcification of falx cerebri and <u>chest deformity</u> (100%); <u>macrocephaly</u> with <u>frontal bossing </u>(100%);<u> hypertelorism</u> (75%); b<u>asal cell carcinoma </u>(25%); pectus deformity with flame shaped hands and feet (25%) and <u>syndactyly</u> (50%). Palmar or plantar pits, <u>cleft lip</u> or palate, ovarian<u> fibroma</u> and <u>medulloblastoma</u> was not identified in any patient. In patients with non-syndromic multiple OKCs <u>mandible</u> was more commonly involved than <u>maxilla</u>. </p><h3>Conclusion</h3><p dir="ltr">Patients with multiple OKCs, should be evaluated thoroughly and <u>basal cell</u> carcinomatous lesions should be ruled out. Meticulous follow up is vital as Gorlin Goltz syndrome is associated with<u> malignancies</u> and OKCs may be the first manifestation of this syndrome. Given the fact that OKCs associated with this syndrome have higher rate of recurrence than the isolated OKCs, long term follow up is warranted.</p><h2>Other Information</h2><p dir="ltr">Published in: Oral and Maxillofacial Surgery Cases<br>License: <a href="http://creativecommons.org/licenses/by/4.0/" target="_blank">http://creativecommons.org/licenses/by/4.0/</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1016/j.omsc.2022.100253" target="_blank">https://dx.doi.org/10.1016/j.omsc.2022.100253</a></p> |
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