Gorlin-Goltz Syndrome with Jaw and Minimal Skin Manifestations - A Case Report.

Q2 Dentistry

Annals of Maxillofacial Surgery Pub Date : 2025-01-01 Epub Date: 2025-04-16 DOI:10.4103/ams.ams_139_24

Anand Mohan Nikunj, Biswajit Mishra, Bathri Elangovan, Dhanashree Jadhav

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Abstract

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.

Abstract Image

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Gorlin-Goltz综合征伴颌骨及轻微皮肤表现1例报告。

理由：多发牙源性角化囊肿（OKC）是网状基底细胞癌综合征（NBCCS）的一个组成部分，并伴有皮肤、骨骼、眼科和神经系统异常。患者关注：一名15岁的男性患者报告了他的上颚和下颚肿胀的主诉。诊断：骨断层扫描显示上颌和下颌骨多发透光区。在局部麻醉下对囊性病变进行抽吸，并产生一种干酪样渗出物，这是OKC的特征。治疗：在全身麻醉下，用卡诺伊氏液化学烧灼完全摘除囊肿并行周围骨切除术。结果：随访5年，无复发。结论：已知多个OKCs最常见于综合征病例。这些多发病变可能是NBCCS的首次表现，或者，它们可能是因为OKCs的多灶性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annals of Maxillofacial Surgery Medicine-Surgery

CiteScore

1.20

自引率

0.00%

发文量