Synovial chondromatosis in a young child: A rare presentation Kuppa - PDF document

[Downloaded free from http://www.jdrntruhs.org on Wednesday, September 28, 2016, IP: 41.66.228.183] Case Case Report Report Synovial chondromatosis in a young child: A rare presentation Kuppa Srinivas, Dema Rajaiah, Yerukala Ramana, Puppula Kiran Kumar Department of Orthopaedics, Kurnool Medical College, Kurnool, Andhra Pradesh, India ABSTRACT Synovial chondromatosis is a benign cartilaginous metaplasia of the synovium, which may affect any synovial joint. Knee joint is the most commonly involved joint presenting with pain swelling and restricted movements. It usually occurs aged 30 to 50 years and is extremely rare in children. Diagnosis is made by radiographs, computed tomography, magnetic resonance imaging and on surgery. Key words: Synovial chondromatosis, young child, knee joint INTRODUCTION occasional pain. There was no history of trauma and no other joints were involved. Her past history family Synovial chondromatosis is an unusual proliferative history and general health were un-remarkable. and metaplastic disorder, which is characterized by the On examination formation of multiple cartilaginous nodules in the synovial Girl had normal gait was unable to squat due to membrane of the joint, tendon sheath and the bursae, the discomfort in the knee. The left knee was moderately etiology of the disease is uncertain. [1] It is usually mono swollen with mild tenderness over the anterior aspect. articular, knee joint is the most commonly affected. [2] It The range of motion was from full extension to 90° occurs twice as frequently in men than in women and of fl exion. The knee was stable. One centimeter of usually presents with pain and swelling during the third wasting of the quadriceps was present. All other joints to the fi fth decade. [3] Multiple discreet nodules presenting were normal. as intra-articular loose bodies is the hallmark. The patient experiences a decreased range of motion, palpable The patient’s blood tests were normal serum calcium, swelling, effusion and crepitus. [3] Trauma, infections and phosphate, erythrocyte sedimentation rate, C-reactive fi broblast growth factors-9 (FGF-9) have been implicated protein, white cell count. Rheumatoid arthritis factor in the pathogenesis. [4] and antinuclear antibody were not detected. CASE REPORT Plain radiograph revealed multiple calci fi c densities within the soft tissues around although some appeared A 10-year-old girl presented to the outpatient department to be in the joint, majority were in the suprapatellar with 4 months history of swelling the left knee with pouch and popliteal fossa [Figure 1]. There was no ligamentous calcification and the growth plates Address for correspondence were normal. To further scrutinize a magnetic Dr. Kuppa Srinivas, resonance imaging (MRI) was taken and it showed Government General Hospital, Kurnool, Andhra Pradesh, India. E-mail: drsrinivasmsortho@gmail.com extensive thickening of the synovium, multiple intra- articular calcific and ossific loose bodies and large Access this article online calci fi ed bursal extensions, consistent with synovial Quick Response Code: Website: chondromatosis [Figures 2 and 3]. www.jdrntruhs.org Operation DOI: In a bloodless fi eld, a lateral parapatellar incision was 10.4103/2277-8632.153318 made when the synovial membrane was opened straw 36 Journal of Dr. NTR University of Health Sciences 2015;4(1) 36-38

[Downloaded free from http://www.jdrntruhs.org on Wednesday, September 28, 2016, IP: 41.66.228.183] Srinivas, et al. : Synovial chondromatosis colored fluid extruded in large amounts containing examination of the specimen con fi rmed the diagnosis many cartilaginous loose bodies, which were 2-4 mm of synovial chondromatosis. in diameter. Many more were attached to the synovial membrane. A large pedunculated mass was present Follow-up in the supra patellar pouch. This along with other Patient followed regularly at 4-week interval. There is pieces of synovium were excised and sent for no clinical, radiological evidence of recurrence, and histopathological examination [Figure 4]. The joint there is no limitation in knee movements. Patient is was washed to remove any leftover loose bodies able to walk and squat without any dif fi culty. after doing synovectomy. Wound was closed in layers patient recovered well and was discharged a week DISCUSSION after surgery [Figures 5 and 6]. Synovial chondromatosis involves major joints such Gross pathology — Irregular surface multinodular as knee, hip, shoulder, elbow and temporomandibular specimen of size 11 cm × 7 cm × 3 cm. Partly covered joint. [5] Other rare sites are distal radio ulnar joint, with membrane c/s nodular and bony hard multiple acromioclavicular joint facet joint, metacarpophalengeal gray white nodules of twenty in number [Figure 4]. joint and the spine. Presents as monoarticular pathology Microscopy — Multiple sections studied shows with pain and swelling along with the presence of intra synovial tissue with multiple benign looking capsular lesions. Lesions can invade the capsule and cartilaginous nodules. Focal areas show trabeculae present as extra capsular masses. [6] of mature bone. Macroscopic and histological Figure 2: Magnetic resonance imaging of left knee - coronal plane Figure 1: X-ray of left knee Figure 3: Magnetic resonance imaging of left knee-coronal plane Figure 4: Biopsy specimen Journal of Dr. NTR University of Health Sciences 2015;4(1) 37

[Downloaded free from http://www.jdrntruhs.org on Wednesday, September 28, 2016, IP: 41.66.228.183] Srinivas, et al. : Synovial chondromatosis Figure 5: Postoperative clinical photograph showing suture line Figure 6: Postoperative X-ray of left knee Occurs in two forms: necrotic change and rarely mitotic fi gures. Radiological 1. Primary form which results from metaplasia of the evidence of bony invasion or soft tissue invasion on MRI synovium, which produces multiple loose bodies should raise suspicion of malignant change recurrence within the joint they progress to calcify loose bodies ranges from 3.2% to 22.2%, respectively. Radiotherapy which are of the same size. Eventually, they embed in is useful for recurrent lesions and inhibition of FGF-9 the synovium and they do not fl oat freely in the joint. has been suggested as nonoperative treatment of primary 2. Secondary form is much more common believed to synovial chondromatosis. be secondary to trauma which causes shedding of bits of articular cartilage resulting in loose bodies ACKNOWLEDGMENTS in the joint. They may or may not calcify. Unlike primary these loose bodies are of different sizes and We are thankful to the Superintendent, Government General Hospital, Principal, Kurnool Medical College, Kurnool generally fewer in number. Generally, osteoarthritis and other staff members and faculty of the Department is present due to articular cartilage damage. of Orthopedics, Kurnool Medical College, Kurnool. We specially thank Dr. O. Sujith Postgraduate in Orthopedics Milgram staging for his contribution to this study. Early-active intra synovial disease but no loose bodies. [7] REFERENCES Transitional-active disease and loose bodies. 1. Je ff reys TE. Synovial chondromatosis. J Bone Joint Surg Br 1967; Late-multiple loose bodies, but no intra synovial 49:530-4. disease. The current patient had stage three disease. 2. Ackerman D, Le � P, Galat DD Jr, Parvizi J, Stuart MJ. Results of total hip and total knee arthroplasties in patients with synovial chondromatosis. J Arthroplasty 2008;23:395-400. X-rays may show loose bodies in the joint but can 3. Temple HT, Gibbons CL. Tumors and tumor-related conditions about the knee. In: Bulstrode C, Buckwalter J, Carr A, Marsh L, Fairbank J, underestimate the size of the lesion. MRI helps to Wilson-Macdonald J, et al ., editors. Oxford Textbook of Orthopaedics de fi ne the extent of the lesion, identify involvement of and Trauma. Vol. 2. Oxford: Oxford University Press; 2002. p. 1153-4. 4. Robinson D, Hasharoni A, Evron Z, Segal M, Nevo Z. Synovial other structures such as adjacent marrow, soft tissues, chondromatosis: The possible role of FGF 9 and FGF receptor 3 in its neurovascular structures and differentiating it from pathology. Int J Exp Pathol 2000;81:183-9. 5. Boya H, Pinar H, Ozcan O. Synovial osteochondromatosis of other synovial disorders. the suprapatellar bursa with an imperforate suprapatellar plica. Arthroscopy 2002;18:E17. 6. Sim FH, Dahlin DC, Ivins JC. Extra-articular synovial chondromatosis. Surgical excision is the preferred treatment. [2] On a clinical J Bone Joint Surg Am 1977;59:492-5. basis malignant transformation needs to be considered if 7. Milgram JW. Synovial osteochondromatosis: A histopathological study of thirty cases. J Bone Joint Surg Am 1977;59:792-801. there is a rapid recurrence following synovectomy, sudden exacerbation of the symptoms or extension of the process How to cite this article: Srinivas K, Rajaiah D, Ramana Y, Kumar PK. beyond the joint capsule occur. Microscopically features Synovial chondromatosis in a young child: A rare presentation. J NTR Univ Health Sci 2015;4:36-8. include chondrocytic arrangement in sheets without Source of Support: Nil. Con fl ict of Interest: None declared. clustering architecture, a myxoid change in the stroma, 38 Journal of Dr. NTR University of Health Sciences 2015;4(1)