Gürkan KAYABAŞOĞLU, Mahmut Sinan YILMAZ, Ali Fuat VARLI, Mehmet GÜVEN

Melkersson-Rosenthal Syndrome in Pediatric Age Group

Melkersson-Rosenthal sendromu (MRS); periferik fasiyal paralizi, fasiyal ödem ve cografik dil triadı ile karakterize bir hastalıktır. Hastalık yüz, dudaklar, oral kavite ve fasiyal sinirde ödeme yol açan granülomatöz patoloji ile seyreder. Sendromun bulgularının kendiliğinden veya medikal tedaviyle gerileyebilmesine karşın, bazı hastalarda progresif seyredebilmekte ve cerrahi tedavi (fasiyal sinir dekompresyonu) gerektirmektedir. Klasik belirtiler triadının görülmesi nadirdir ve genellikle monosemptomatik veya oligo semtomatik tutulum izlenir ayrıca hastalığın nadir görülmesinden dolayı tanı koymada güçlükler veya gecikmeler söz konusu olmaktadır. Bulgulardan bir veya ikisi ile biyopside granülamotöz keilitin varlığı kesin tanı için yeterlidir. Melkerson Rosenthal sendromu tekrarlayan fasiyal paralizilerin ayırıcı tanısında düşünülmesi gereken bir hastalıklardandır ve bu yazıda Melkersson Rosenthal sendromu tanısı konulan bir pediatrik hasta sunulmuştur

Anahtar Kelimeler:

Melkersson Rosenthal Sendromu, orofasiyal ödem, tekrarlayan fasiyal paralizi

Melkersson-Rosenthal Syndrome in Pediatric Age Group

Melkersson-Rosenthal Syndrome (MRS) is a disease characterized by peripheral facial paralysis, facial edema and fissured tongue. Appearance of this triad is rare, generally monosymptomatic or oligosymptomatic involvement is observed and difficulties or delays in diagnosis can occur due to scarcity of the disease. One or two of the findings and presence of cheilitis granulomatosa in biopsy are sufficient for the diagnosis. Melkersson Rosenthal Syndrome should be considered in differential diagnosis of recurrent facial paralysis. Although findings of the syndrome can regress spontaneously or with medical treatment, it can take a progressive course in some patients, requiring surgical treatment (facial nerve decompression). In this article a pediatric case with diagnosis of Melkersson Rosenthal Syndrome is presented.

Keywords:

Melkersson-Rosenthal syndrome, orofacial edema, recurrent facial paralysis.,

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