Thursday 27 September 2018

Early diagnosis Gorlin-Goltz syndrome: A Rare Case Report: SCSOAJ - Lupine Publishers

Gorlin-Goltz syndrome, is an uncommon, autosomal dominant inherited disorder, which is characterized by numerous basal cell carcinoma (known as nevoid basal cell carcinoma syndrome (NBCCS)). The common manifestations include multiple odontogenic keratocysts in the jaws are usually one of the first manifestations of the syndrome, macrocephaly, frontal boosing, multiple basal cell nevi on the skin with an early age onset, palmar pits, bifid or splayed ribs, high arched palate, calcified diaphragm sellae, calcifications of the falx cerebri. Although all the manifestations may not be present in all the patients. We reported here a child patient, a 8-year-old child boy visited maxillofacial department in Rizgary teaching hospital for right premaxillary swelling emphasizing its clinical and radiographic manifestations of Gorlin -Goltz syndrome with no family history, the patient has multiple odontogenic keratocysts, rib anomalies, calcifications of the falx cerebri, frontal and temporal boosing, macrocephaly and thick eye brows. This study highlights the importance of maxillofacial surgeon in the early diagnosis, treatment and closes follow up is important to prevent long-term complication including malignancy and oromaxillofacial deformation and destruction.

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