PDF(Pubmed)
Abstract：
Gorlin-Goltz syndrome (GGS) among Indians is rarely reported. Since 1960, only 38 cases having 48 patients of Gorlin-Goltz syndrome have been identified in the Indian population. It is crucial to diagnose this illness early because it can be connected to a malignant lesion like fibrosarcoma, leiomyosarcoma or rhabdomyosarcoma. The four patients in this case series were identified and treated in our department between 2019 and 2023. The average patient age was around 20 years old. Jaw swelling and tooth movement were the two most typical presenting concerns. Odontogenic keratocysts (100%), palmer pits (100%), plantar pits (50%), calcification of falx cerebri (50%), and rib abnormalities (50%), were the most prevalent characteristics. None of the patients had basal cell cancer, cleft lip, or medulloblastoma. Multiple odontogenic keratocysts were present in three cases, whereas a single odontogenic keratocyst (OKC) was seen in one patient. Patients were managed with either marsupialization or enucleation, depending on the size of the cyst. Two cases with a large cyst size were marsupialized by using a modified obturator. Two cases with small cysts were managed with enucleation of the cyst followed by chemical cauterization. Recurrence was seen in two cases. In one patient, we noticed the formation of a new cyst. A GGS diagnosis can be made by having a systemic evaluation of the patient. A thorough examination of the patient should be performed in every histopathology-diagnosed case of OKC. This will help to miss the syndromic cases. The treatment part should be conservative, like marsupialization with an obturator in a large cyst. The obturator helps maintain patient hygiene and prevents regular visits for changing dressings. Small-sized cysts can be managed with enucleation and chemical cauterization. Radical resection should be avoided.
摘要：
很少报道印度人的Gorlin-Goltz综合征（GGS）。自1960年以来，在印度人口中仅发现38例Gorlin-Goltz综合征患者48例。早期诊断这种疾病至关重要，因为它可能与纤维肉瘤等恶性病变有关，平滑肌肉瘤或横纹肌肉瘤。该病例系列中的四名患者在2019年至2023年之间在我们部门进行了鉴定和治疗。患者平均年龄约为20岁。颌骨肿胀和牙齿移动是两个最典型的问题。牙源性角化囊肿（100%），Palmer坑(100%),足底坑(50%)，大脑镰状钙化（50%），和肋骨异常（50%），是最普遍的特征。这些病人都没有基底细胞癌,唇裂,或者髓母细胞瘤.3例患者出现多发性牙源性角化囊肿，而在一名患者中发现了单个牙源性角化囊肿（OKC）。患者接受有袋化或眼球摘除治疗，取决于囊肿的大小。使用改良的闭孔器对2例囊肿大小较大的病例进行了有袋化。对2例小囊肿病例进行了囊肿摘除，然后进行化学烧灼。在两个病例中发现了复发。在一个病人中,我们注意到一个新的囊肿的形成。可以通过对患者进行系统评估来进行GGS诊断。在每个组织病理学诊断的OKC病例中，都应对患者进行彻底检查。这将有助于错过综合征病例。治疗部分应保守，比如在一个大囊肿里有一个闭孔的有袋化.闭塞器有助于保持患者卫生并防止定期访问更换敷料。小尺寸囊肿可以通过摘除和化学烧灼来管理。应避免根治性切除。
