Abstract：
The coexistence of calcium pyrophosphate dihydrate crystal deposition (CPP) and synovial chondromatosis (SC) in the temporomandibular joint (TMJ) is rarely reported. CPP disease (CPPD) is complex arthritis synonymous with excessive pyrophosphate production and variable aberrations in mineral and organic phase metabolism of the joint cartilage, leading to local inundated CPP and crystal deposition of partially deciphered predispositions. Meanwhile, SC is a rare benign synovial joint proliferative disease of unclear etiology and has a low risk of malignant transformation. However, SC manifests severe joint disability and dysfunction because of connective tissue metaplasia of the synovial membrane, which forms cartilaginous nodules with or without calcifications or ossifications. These nodules often detach and form intra-articular loose bodies and very rarely within extraarticular spaces.
We report the case of a 61-year-old man to expand the body of literature on these unusual coexisting arthropathies of the TMJ. The patient presented to our hospital in 2020 with complaints of pain in the right TMJ and trismus for over 6 months. Radiographic assessments of the TMJ provided a preoperative provisional diagnosis of SC. However, the histopathology of the open biopsy revealed tumor-like lesions comprising several deposits of rhomboid and rod-shaped crystals that displayed positive birefringence in polarized light, confirming a coexistence of CPPD. A second-stage operation was performed for the complete removal of the loose bodies and chalk-like lesions including synovectomy. No evidence of recurrence was recorded after a follow-up of nearly 1.5 years.
Isolated CPPD and SC of the TMJ are prevalent in the literature however, monoarticular coexistence of these diseases is rare, due to the lack of consistency in the diagnostic criteria in clinical practice. Moreover, optimal treatment depends on several considerations. This report delineated the molecular etiopathology and underscored the need for continued deciphering of the causal mechanisms of coexisting CPPD and SC of the TMJ. In addition, the importance of confirmatory testing for accurate diagnosis, and appropriate management of these diseases were discussed.
We report the case of a 61-year-old man to expand the body of literature on these unusual coexisting arthropathies of the TMJ. The patient presented to our hospital in 2020 with complaints of pain in the right TMJ and trismus for over 6 months. Radiographic assessments of the TMJ provided a preoperative provisional diagnosis of SC. However, the histopathology of the open biopsy revealed tumor-like lesions comprising several deposits of rhomboid and rod-shaped crystals that displayed positive birefringence in polarized light, confirming a coexistence of CPPD. A second-stage operation was performed for the complete removal of the loose bodies and chalk-like lesions including synovectomy. No evidence of recurrence was recorded after a follow-up of nearly 1.5 years.
Isolated CPPD and SC of the TMJ are prevalent in the literature however, monoarticular coexistence of these diseases is rare, due to the lack of consistency in the diagnostic criteria in clinical practice. Moreover, optimal treatment depends on several considerations. This report delineated the molecular etiopathology and underscored the need for continued deciphering of the causal mechanisms of coexisting CPPD and SC of the TMJ. In addition, the importance of confirmatory testing for accurate diagnosis, and appropriate management of these diseases were discussed.
摘要：
背景：颞下颌关节（TMJ）中焦磷酸钙二水合物晶体沉积（CPP）和滑膜软骨瘤病（SC）共存的报道很少。CPP疾病（CPPD）是复杂的关节炎，与过度的焦磷酸盐产生和关节软骨的矿物质和有机相代谢中的可变畸变同义。导致局部淹没的CPP和部分破译倾向的晶体沉积。同时,SC是一种罕见的良性滑膜关节增殖性疾病，病因不明，恶变风险低。然而,由于滑膜结缔组织化生，SC表现出严重的关节残疾和功能障碍，形成有或没有钙化或骨化的软骨结节。这些结节经常分离并形成关节内松散体，很少在关节外间隙内。
方法：我们报道了一名61岁男子的病例，以扩大有关这些不寻常的TMJ并存关节病的文献。该患者于2020年到我们医院就诊，抱怨右TMJ和三联肌疼痛超过6个月。TMJ的影像学评估提供了SC的术前临时诊断。然而,开放活检的组织病理学显示肿瘤样病变，包括几个菱形和杆状晶体沉积物，在偏振光下显示正双折射，确认CPPD共存。进行了第二阶段的手术，以完全切除松散的身体和粉笔样病变，包括滑膜切除术。在近1.5年的随访后，没有记录到复发的证据。
结论：分离的TMJCPPD和SC在文献中很普遍，这些疾病的单关节共存是罕见的，由于临床实践中诊断标准缺乏一致性。此外，最佳治疗取决于几个方面的考虑。该报告描述了分子病因学，并强调需要继续破译TMJ的CPPD和SC共存的因果机制。此外,验证性测试对准确诊断的重要性，并讨论了这些疾病的适当管理。
方法：我们报道了一名61岁男子的病例，以扩大有关这些不寻常的TMJ并存关节病的文献。该患者于2020年到我们医院就诊，抱怨右TMJ和三联肌疼痛超过6个月。TMJ的影像学评估提供了SC的术前临时诊断。然而,开放活检的组织病理学显示肿瘤样病变，包括几个菱形和杆状晶体沉积物，在偏振光下显示正双折射，确认CPPD共存。进行了第二阶段的手术，以完全切除松散的身体和粉笔样病变，包括滑膜切除术。在近1.5年的随访后，没有记录到复发的证据。
结论：分离的TMJCPPD和SC在文献中很普遍，这些疾病的单关节共存是罕见的，由于临床实践中诊断标准缺乏一致性。此外，最佳治疗取决于几个方面的考虑。该报告描述了分子病因学，并强调需要继续破译TMJ的CPPD和SC共存的因果机制。此外,验证性测试对准确诊断的重要性，并讨论了这些疾病的适当管理。
