Mehmet Oğuz BORAHAN, Ömer Birkan AĞRALI, Leyla KURU, Filiz NAMDAR PEKİNER

Pseudoksantoma elastikumda kök agenezisinin klinik, histolojik ve radyolojik olarak incelenmesi

Amaç: Psödoksantoma elastikum (PXE) deri, retina ve kardiyovasküler sistemde elastik liflerin ilerleyen kalsifikasyon ve parçalanması ile karakterize, nadir görülen genetik bir hastalıktır. PXE ‘nin oftalmik ve dermatolojik belirtileri ve vasküler komplikasyonları heterojendir. Sistemik düzensizliklerin yanı sıra, bir dizi belirtiler bildirilmiştir. Bu olgu raporunun amacı PXE olan bir hastada görülen kök agenezisi sunmaktır.Vaka Sunumu: PXE tanısı konmuş 22 yaşındaki bayan hasta maloklüzyonu ve estetik şikayet nedenleriyle Marmara Üniversitesi, Diş Hekimliği Fakültesi’ne başvurmuştur. Klinik muayeneye ek olarak, panoramik radyografi ve konik ışınlı bilgisayarlı tomografide kök agenezi görüntülenmiştir. Tedavi planını mekanik periodontal tedavi ve kısa aralıklarla klinik takibi oluşturmuştur.Sonuç: Radyolojik muayene, dental ve mukozal anomaliler için yararlı ve önemlidir. Sonuç olarak, bu çalışma klinisyenler tarafından dikkate alınması gereken PXE ile birlite kök agenezisi görülen bir hastanın sunumudur.

Anahtar Kelimeler:

Psödoksantoma elastikum, kök agenezi, konik ışınlı bilgisayarlı tomografi

Clinical, histological and radiological presentation of root agenesis in pseudoxanthoma elasticum

Objectives: Pseudoxanthoma elasticum (PXE) is a rare, genetic disorder characterized by progressive calcification and fragmentation of elastic fibers in the skin, retina and cardiovascular system. The ophthalmic and dermatologic expressions of PXE and vascular complications are heterogeneous. In addition to systemic disorders, a number of manifestations have been reported. The aim of this case report is to present, for the first time, root agenesis in a patient with PXE.Case Report: A 22-year-old girl diagnosed with PXE was referred to the Faculty of Dentistry, Marmara University, with a chief complaint of malocclusion and unaesthetic appearance. In addition to clinical examination, the patient was imaged using panoramic radiography and cone-beam computed tomography, which revealed root agenesis. Treatment plan consisted of mechanical periodontal therapy and recall visits with short intervals.Conclusion: Radiologic examination is useful and important for dental and mucosal abnormalities. In conclusion, this is the first report of a patient with root agenesis coexisting with PXE which should be taken into account by clinicians.

Keywords:

Pseudoxanthoma elasticum, root agenesis, cone beam computed tomography,

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