Bir vaka üzerinden bir sendromun analizi: Gorlin-Goltz sendromu
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Date
2013-05-31
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Uludağ Üniversitesi
Abstract
Gorlin-Goltz sendromu (GGS), otozomal dominant geçiş gösteren nadir bir genetik hastalıktır. Bu sendromun klasik triadını meydana getiren bulgular; maksillomandibular odontojenik keratokistler, çok sayıda bazoselüler karsinom ve çeşitli iskelet anomalilerinin varlığıdır. Sendro mun erken döneminde tanı koydurucu ana bulgu, oral kavitede multipl keratokistler olduğundan özellikle kulak burun boğaz uzmanları ve diş hekimlerinin hastalık hakkında bilgi sahibi olması önemlidir. Bu sayede hastalarda daha sonra görülebilecek deri ve organ kanserlerinin erken tanısı ve tedavisi multidisipliner şekilde mümkün olabilmektedir. Bu olguda kliniğimize mandibula ve maksillada multipl kistik lez yonlar nedeniyle başvuran ve tanısında GGS düşünülen 13 yaşında bir kız hasta bildirilmektedir.
Gorlin-Goltz Syndrome (GGS) is a rare genetic disease transmitted in autosomal dominant trait. The classical triad of this syndrome involves the existence of maxillomandibular odontogenic keratocysts, multiple basocellular carcinoma and various skeletal abnormalities. Since the existence of multiple keratocysts in the oral cavity is the main finding at the early phase of disease, especially otolaryngologists and medical dentists should be aware of this clinical entity. Thus the early diagnosis and appropriate treatment of skin and solid organ malignancies in these patients can be achieved in a multidisciplinary fashion. In this case, we presented a 13 years old girl patient who admitted to our department with the complaints of multiple cystic lesions in mandibula and maxilla and had the diagnosis of GGS.
Gorlin-Goltz Syndrome (GGS) is a rare genetic disease transmitted in autosomal dominant trait. The classical triad of this syndrome involves the existence of maxillomandibular odontogenic keratocysts, multiple basocellular carcinoma and various skeletal abnormalities. Since the existence of multiple keratocysts in the oral cavity is the main finding at the early phase of disease, especially otolaryngologists and medical dentists should be aware of this clinical entity. Thus the early diagnosis and appropriate treatment of skin and solid organ malignancies in these patients can be achieved in a multidisciplinary fashion. In this case, we presented a 13 years old girl patient who admitted to our department with the complaints of multiple cystic lesions in mandibula and maxilla and had the diagnosis of GGS.
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Keywords
Gorlin Goltz Sendromu, Eksizyon, Odontojenik keratokist, Gorlin Goltz Syndrome, Excision, Odontogenic keratocyst
Citation
Akça, M. E. vd. (2013). "Bir vaka üzerinden bir sendromun analizi: Gorlin-Goltz sendromu". Uludağ Üniversitesi Tıp Fakültesi Dergisi, 39(2), 123-126.