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

36 Journal of Dr. NTR University of Health Sciences 2015;4(1) 36-38

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

Synovial chondromatosis is an unusual proliferative and metaplastic disorder, which is characterized by the formation of multiple cartilaginous nodules in the synovial membrane of the joint, tendon sheath and the bursae, the etiology of the disease is uncertain.[1] It is usually mono articular, knee joint is the most commonly affected.[2] It

• ccurs twice as frequently in men than in women and

usually presents with pain and swelling during the third to the fi fth decade.[3] Multiple discreet nodules presenting as intra-articular loose bodies is the hallmark. The patient experiences a decreased range of motion, palpable swelling, effusion and crepitus.[3] Trauma, infections and fi broblast growth factors-9 (FGF-9) have been implicated in the pathogenesis.[4]

CASE REPORT

A 10-year-old girl presented to the outpatient department with 4 months history of swelling the left knee with

• ccasional pain. There was no history of trauma and

no other joints were involved. Her past history family history and general health were un-remarkable. On examination Girl had normal gait was unable to squat due to discomfort in the knee. The left knee was moderately swollen with mild tenderness over the anterior aspect. The range of motion was from full extension to 90°

• f fl
• exion. The knee was stable. One centimeter of

wasting of the quadriceps was present. All other joints were normal. The patient’s blood tests were normal serum calcium, phosphate, erythrocyte sedimentation rate, C-reactive protein, white cell count. Rheumatoid arthritis factor and antinuclear antibody were not detected. Plain radiograph revealed multiple calcifi c densities within the soft tissues around although some appeared to be in the joint, majority were in the suprapatellar pouch and popliteal fossa [Figure 1]. There was no ligamentous calcification and the growth plates were normal. To further scrutinize a magnetic resonance imaging (MRI) was taken and it showed extensive thickening of the synovium, multiple intra- articular calcific and ossific loose bodies and large calcifi ed bursal extensions, consistent with synovial chondromatosis [Figures 2 and 3]. Operation In a bloodless fi eld, a lateral parapatellar incision was made when the synovial membrane was opened straw

Access this article online Quick Response Code: Website: www.jdrntruhs.org DOI: 10.4103/2277-8632.153318

Case Case Report Report

Address for correspondence

• Dr. Kuppa Srinivas,

Government General Hospital, Kurnool, Andhra Pradesh, India. E-mail: drsrinivasmsortho@gmail.com [Downloaded free from http://www.jdrntruhs.org on Wednesday, September 28, 2016, IP: 41.66.228.183]

Srinivas, et al.: Synovial chondromatosis 37 Journal of Dr. NTR University of Health Sciences 2015;4(1)

colored fluid extruded in large amounts containing many cartilaginous loose bodies, which were 2-4 mm in diameter. Many more were attached to the synovial

• membrane. A large pedunculated mass was present

in the supra patellar pouch. This along with other pieces of synovium were excised and sent for histopathological examination [Figure 4]. The joint was washed to remove any leftover loose bodies after doing synovectomy. Wound was closed in layers patient recovered well and was discharged a week after surgery [Figures 5 and 6]. Gross pathology — Irregular surface multinodular specimen of size 11 cm × 7 cm × 3 cm. Partly covered with membrane c/s nodular and bony hard multiple gray white nodules of twenty in number [Figure 4]. Microscopy — Multiple sections studied shows synovial tissue with multiple benign looking cartilaginous nodules. Focal areas show trabeculae

• f mature bone. Macroscopic and histological

examination of the specimen confi rmed the diagnosis

• f synovial chondromatosis.

Follow-up Patient followed regularly at 4-week interval. There is no clinical, radiological evidence of recurrence, and there is no limitation in knee movements. Patient is able to walk and squat without any diffi culty.

DISCUSSION

Synovial chondromatosis involves major joints such as knee, hip, shoulder, elbow and temporomandibular joint.[5] Other rare sites are distal radio ulnar joint, acromioclavicular joint facet joint, metacarpophalengeal joint and the spine. Presents as monoarticular pathology with pain and swelling along with the presence of intra capsular lesions. Lesions can invade the capsule and present as extra capsular masses.[6]

Figure 1: X-ray of left knee Figure 2: Magnetic resonance imaging of left knee - coronal plane Figure 3: Magnetic resonance imaging of left knee-coronal plane Figure 4: Biopsy specimen [Downloaded free from http://www.jdrntruhs.org on Wednesday, September 28, 2016, IP: 41.66.228.183]

38 Srinivas, et al.: Synovial chondromatosis Journal of Dr. NTR University of Health Sciences 2015;4(1)

Occurs in two forms:

• 1. Primary form which results from metaplasia of the

synovium, which produces multiple loose bodies within the joint they progress to calcify loose bodies which are of the same size. Eventually, they embed in the synovium and they do not fl

• at freely in the joint.
• 2. Secondary form is much more common believed to

be secondary to trauma which causes shedding of bits of articular cartilage resulting in loose bodies in the joint. They may or may not calcify. Unlike primary these loose bodies are of different sizes and generally fewer in number. Generally, osteoarthritis is present due to articular cartilage damage. Milgram staging Early-active intra synovial disease but no loose bodies.[7] Transitional-active disease and loose bodies. Late-multiple loose bodies, but no intra synovial

• disease. The current patient had stage three disease.

X-rays may show loose bodies in the joint but can underestimate the size of the lesion. MRI helps to defi ne the extent of the lesion, identify involvement of

• ther structures such as adjacent marrow, soft tissues,

neurovascular structures and differentiating it from

• ther synovial disorders.

Surgical excision is the preferred treatment.[2] On a clinical basis malignant transformation needs to be considered if there is a rapid recurrence following synovectomy, sudden exacerbation of the symptoms or extension of the process beyond the joint capsule occur. Microscopically features include chondrocytic arrangement in sheets without clustering architecture, a myxoid change in the stroma, necrotic change and rarely mitotic fi

• gures. Radiological

evidence of bony invasion or soft tissue invasion on MRI should raise suspicion of malignant change recurrence ranges from 3.2% to 22.2%, respectively. Radiotherapy is useful for recurrent lesions and inhibition of FGF-9 has been suggested as nonoperative treatment of primary synovial chondromatosis.

ACKNOWLEDGMENTS

We are thankful to the Superintendent, Government General Hospital, Principal, Kurnool Medical College, Kurnool and other staff members and faculty of the Department

• f Orthopedics, Kurnool Medical College, Kurnool. We

specially thank Dr. O. Sujith Postgraduate in Orthopedics for his contribution to this study.

REFERENCES

1. Jeff reys TE. Synovial chondromatosis. J Bone Joint Surg Br 1967; 49:530-4. 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.

3. Temple HT, Gibbons CL. Tumors and tumor-related conditions about the knee. In: Bulstrode C, Buckwalter J, Carr A, Marsh L, Fairbank J, Wilson-Macdonald J, et al., editors. Oxford Textbook of Orthopaedics and Trauma. Vol. 2. Oxford: Oxford University Press; 2002. p. 1153-4. 4. Robinson D, Hasharoni A, Evron Z, Segal M, Nevo Z. Synovial chondromatosis: The possible role of FGF 9 and FGF receptor 3 in its

• pathology. Int J Exp Pathol 2000;81:183-9.

5. Boya H, Pinar H, Ozcan O. Synovial osteochondromatosis of the suprapatellar bursa with an imperforate suprapatellar plica. Arthroscopy 2002;18:E17. 6. Sim FH, Dahlin DC, Ivins JC. Extra-articular synovial chondromatosis. J Bone Joint Surg Am 1977;59:492-5. 7. Milgram JW. Synovial osteochondromatosis: A histopathological study of thirty cases. J Bone Joint Surg Am 1977;59:792-801. How to cite this article: Srinivas K, Rajaiah D, Ramana Y, Kumar PK. Synovial chondromatosis in a young child: A rare presentation. J NTR Univ Health Sci 2015;4:36-8. Source of Support: Nil. Confl ict of Interest: None declared. Figure 5: Postoperative clinical photograph showing suture line Figure 6: Postoperative X-ray of left knee [Downloaded free from http://www.jdrntruhs.org on Wednesday, September 28, 2016, IP: 41.66.228.183]