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  • Author: Orçun Toptaş x
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Papillon-Lefévre Syndrome: Case Report and Genetic Analysis


Backgroung/Aim: Papillon Lefévre syndrome is a rare autosomal recessive genodermatosis. The characteristic findings of the disease are early loss of primary and permanent teeth and palmoplantar keratoderma. Notwithstanding that many etiologic factors like genetic mutations, bacterial agents, immunologic changes have been identified, the pathogenesis has not been fully understood. Although dentists play an important role in the diagnosis and treatment of Papillon Lefévre syndrome, it is appropriate to treat the disease with a multidisciplinary approach.

Case Report: In this case report, the clinical, radiological and genetic examination of the patient with Papillon Lefévre syndrome who has a homozygous mutation in the CTSC gene will be presented.

Conclusions: Dentists should have knowledge about treatment management of these patients. Teeth can be preserved longer with early diagnosis and appropriate treatment of the disease.

Open access
Closure of Oronasal Fistula by Palatal Rotational Flap: Case Report with Two Years follow-up


Background/Aim: Hard palate fistulas are due to pathologies such as noma, syphilitic gom, leprosy, leishmaniasis, radiotherapy, removal of cysts or tumors of the sinus or palate, trauma or tooth attraction. Symptoms of palatal fistulas include hypernasality in the phonation due to nasal air escape during a speech, nasal cavity fluid flow, and infection due to food accumulation. Surgical repair of palatal perforations is technically difficult and complicated. Different surgical methods can be used depending on the size and location of the defect. Among these techniques, the locoregional flap types used are palatal flap, nasolabial flap, a buccal fat pad and tongue flap.

Case Report: In this case report, we presented a patient with oronasal fistula due to a previous surgical operation, which was surgically closed by a palatal rotational flap.

Conclusions: As a result, the oronasal communucation was relieved by successful closure of the defect and the donor area healed uneventfully.

Open access