伴有颌骨及轻微皮肤表现的戈林-戈尔茨综合征——病例报告
Gorlin-Goltz Syndrome with Jaw and Minimal Skin Manifestations - A Case Report.
作者信息
Nikunj Anand Mohan, Mishra Biswajit, Elangovan Bathri, Jadhav Dhanashree
机构信息
Department of Oral and Maxillofacial Surgery, Government Dental College, Raipur, Chhattisgarh, India.
Department of Oral and Maxillofacial Surgery, Bharat Ratna Atal Bihari Vajpayee Medical College, Pune, Maharashtra, India.
出版信息
Ann Maxillofac Surg. 2025 Jan-Jun;15(1):110-114. doi: 10.4103/ams.ams_139_24. Epub 2025 Apr 16.
RATIONALE
Multiple odontogenic keratocysts (OKC) occurs as a component of nevoid basal cell carcinoma syndrome (NBCCS) with concomitant cutaneous, skeletal, ophthalmic and neurological abnormalities.
PATIENT CONCERNS
A 15-year-old male patient reported with chief complaint of swelling in his upper and lower jaws.
DIAGNOSIS
Orthopantomogram was taken and it showed multiple radiolucent areas present in the maxilla and mandible. Aspiration of the cystic lesion was done under local anaesthesia and it yielded a cheesy exudate which is characteristic of OKC.
TREATMENT
Complete enucleation of the cyst with chemical cauterisation with Carnoy's solution and peripheral ostectomy was done under general anaesthesia.
OUTCOME
He was under follow-up for the past 5 years, and there was no recurrence.
TAKE-AWAY LESSON: Multiple OKCs have been known to occur most commonly in syndromic cases. These multiple lesions may be the first manifestation of the NBCCS, or otherwise, they may be because of the multifocal nature of OKCs.
理论依据
多发性牙源性角化囊肿（OKC）作为痣样基底细胞癌综合征（NBCCS）的一部分出现，伴有皮肤、骨骼、眼科和神经方面的异常。
患者情况
一名15岁男性患者，主诉上下颌肿胀。
诊断
拍摄了全景曲面断层片，显示上颌骨和下颌骨存在多个透射区。在局部麻醉下对囊性病变进行抽吸，抽出了干酪样渗出物，这是OKC的特征。
治疗
在全身麻醉下，用卡诺氏液进行化学烧灼并完整摘除囊肿及周边骨切除术。
结果
在过去5年中对他进行随访，未见复发。
经验教训
已知多发性OKC最常见于综合征病例。这些多发性病变可能是NBCCS的首发表现，或者，也可能是由于OKC的多灶性。
Zotero 插件