Department of Medicine, Surgery, and Health Sciences, University of Trieste, Via dell'Istria 65/1, 34100, Trieste, Italy.
Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.
Ital J Pediatr. 2022 Jul 30;48(1):139. doi: 10.1186/s13052-022-01332-4.
Primary Synovial Chondromatosis (PSC) is a rare benign tumor of the synovial membrane in which cartilage metaplasia produces calcific loose bodies within the articular space. Only a few cases are reported in the pediatric population and its etiology remains unknown. This condition typically affects large weight-bearing joints with pain, swelling and decrease range of motion. Due to its slow progressions, delayed diagnosis is frequent and differential diagnosis should consider other chronic arthritis and malignancies. While arthroscopic removal of loose bodies is the current treatment up to now, the association of partial or complete synovectomy is debated.
We report about a 14-year-old girl with a long-lasting right shoulder pain, especially during movements or exercise, localized tenderness and hypotonia of the glenohumeral joint. No previous trauma was mentioned. Blood exams, Mantoux test and plain radiography of the right shoulder were unremarkable. Ultrasound imaging revealed echogenic and calcified bodies stretching the glenohumeral joint and dislocating the long head of biceps tendon. Magnetic resonance showed a "rice-grain" pattern of the right shoulder. From an arthroscopic surgery, multiple loose white bodies were removed within the synovial membrane, and synovial chondromatosis was confirmed by histological analysis. At one month follow up visit, the patient completely recovered without pain.
Synovial chondromatosis is a very uncommon cause of mono articular pain in children, especially when it affects shoulder. Pediatricians should keep in mind this condition to avoid delayed diagnosis and treatment, even in consideration of the low risk of malignant transformation. Through this case, we would highlight common diagnostic pitfalls and treatment of synovial chondromatosis.
原发性滑膜软骨瘤病(PSC)是一种罕见的滑膜膜良性肿瘤,软骨化生在关节腔内产生钙化的游离体。这种情况在儿科人群中仅有少数病例报道,其病因仍不清楚。这种疾病通常影响大承重关节,表现为疼痛、肿胀和运动范围减小。由于其进展缓慢,常导致诊断延迟,鉴别诊断应考虑其他慢性关节炎和恶性肿瘤。尽管关节镜下切除游离体是目前的治疗方法,但部分或完全滑膜切除术的选择仍存在争议。
我们报告了一名 14 岁女孩,她长期右肩关节疼痛,尤其是在运动或锻炼时,局部压痛和盂肱关节无力。没有提到以前的创伤。血液检查、曼托试验和右肩关节的普通 X 线片均未见异常。超声成像显示在盂肱关节处有回声增强和钙化的游离体,且肱二头肌长头腱脱位。磁共振成像显示右肩关节有“米粒”样表现。通过关节镜手术,在滑膜内切除了多个白色的游离体,组织学分析证实为滑膜软骨瘤病。在一个月的随访中,患者完全恢复,没有疼痛。
滑膜软骨瘤病是儿童单关节疼痛的一种非常罕见的原因,尤其是当它影响肩部时。儿科医生应牢记这种疾病,以避免诊断和治疗的延迟,即使考虑到恶性转化的风险较低。通过这个病例,我们将强调滑膜软骨瘤病的常见诊断误区和治疗方法。