primary synovial osteochondromatosis

Case presentation of 22/F with Rt knee Pain:

Case presentation of 22/F with Rt knee Pain Presented by: Saswat Prasad 2 nd year resident Dept. of orthpaedics , NMCTH 1

Presentation :

Presentation 20 yrs/ F c/o pain & swelling over the rt knee Duration – 3 yrs Pain – was gradual in onset Relieved by rest & aggravated by walking & physical activities Swelling developed gradually, ass. with physical activity Also there is Hx of locking of the knee 2

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No Hx of trauma No Hx of similar comp. in other jts No Hx of fever No Hx of discharge from the jt No Hx of night sweats or night pains No Hx of burning micturation , urethral Dis. No Hx of any drug abuse No Hx of loss of apetitte , or loss of wt. Normal bowel & bladder habits 3

Rx Hx:

Rx Hx No Hx of any medication taken for the pain No Hx of any jt aspiration or I/A inj Hx of massage done on & off Also hot fomentation was done during episodes of intense pain 4

Past, Family & Personnel Hx:

Past, Family & Personnel Hx No past Hx of any chrnic illnesses No Hx of similar complains in the family No Hx of any chronic illnesses in the family Does not smoke or drink alcohol Takes a mixed diet Has no known allergies 5

General Examination:

General Examination GC was fair No icterus , pallor, cyanosis, lympadenopathy Chest – B/L vesicular breath sounds heard CVS – S1+ S2+ Mo P/A – Soft non tender, no organomegaly 6

Local Examination:

Local Examination Ex from the front Attitude – neutral Swelling + Wasting + more on the medial side Patella central, & shape b/l sym No visible scars, sinuses, venous prominences 7

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From the sides No prominence of the fibular head Wasting of the vastus lateralis From the back No fullness of the popliteal fossa 8

On palpation:

On palpation Increased local temp Swelling Crepitus + Tenderness + over lateral & medial aspect of rt knee Synovium was thickened, and doughy Patellar Tap – ve Transillumination – ve 9

Measurements :

Measurements Rt Lt Apparent length 86 86 True length 77 77 Girth of thigh 44 46 Girth of calf 31 31 Range of motion 0 – 120 0 - 130 10

Special Tests:

Special Tests Galleazi’s test Varus & Valgus tests – ve Lachman’s test – ve Drawer tests – ve McMurry’s test – ve Apley’s distraction & compression test – ve Patellar grind test – ve 11

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All routine investigations Plain X ray of the Rt knee AP & LAT views were sent 12

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Differential Diagnosis:

Differential Diagnosis Trauma-related: Fracture with avulsed fragment Fragmentation of meniscus with calcification Degenerative joint disease related: Degenerative joint disease with detached spur Synovial proliferation: Pigmented villonodular synovitis Synovial osteochondromatosis 15

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Neoplastic : Synovial chondrosarcoma Other: Osteochondritis dissecans Sequestrum from osteomyelitis Neuropathic (Charcot) joint 16

SYNOVIAL OETEOCHONDROMATOSIS:

SYNOVIAL OETEOCHONDROMATOSIS LITERATURE REVIEW 17

Introduction :

Introduction Monoarticular diseasse , with a benign course Though in a few cases malignant transformation have been reported. C harac . By synovial membrane proliferation & metaplasia Incidence 2 – 4 folds higher in males All age groups can be affected (20-50) 18

Introduction:

Introduction Cartilaginous metaplasia , within syn. Mem Metaplastic synovium organizes into nodules Following trauma nodules are shed into the joints Similar process have also been identified in tendon sheaths and bursal sacs 19

Fate of these free nodules:

Fate of these free nodules Float freely in the joint synovial fluid Which also provides nutrition to these bodies Progressive enlargement and ossification may occur with time May occupy enitre jt. Space Infiltrate into surrounding tissue Can reattach to the synovium and often even get reabsorbed 20

Etiology :

Etiology Unknown Various factors have been proposed such as trauma, nutrition, aseptic necrosis, interupted blood supply, infections, defects of bone metabolism, inflammatory arthritis 21

Etiology :

Etiology Jones(1924) theory The pathology may have existed in the synovium was 1 st proposed by Jones(1924) Untill then it was assumed that these bodies originated from the articular surface 22

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In the past, the etiology was considered purely metaplastic or a true neoplastic disease. However, currently known molecular studies suggest that primary synovial chondromatosis is believed to be due to dysregulation of Hedgehog signalling . HopyanS , Nadesan P, Yu C, Wunder J, Alman BA. Dysregulation of hedgehog signalling predisposes to synovial chondromatosis . J Pathol2005; 206:143–150 23

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Also chromosome 6 abnormalities, identified at cytogenetic and molecular cytogenetic analyses, have been a recurrent finding in primary synovial chondromatosis , results that indicate the disease is a neoplastic condition. BuddinghEP , Krallman P, Neff JR, Nelson M, Liu J, Bridge JA. Chromosome 6 abnormalities are recurrent in synovial chondromatosis . Cancer Genet Cytogenet2003;140:18–22 24

Classification:

Classification Usually classified into Primary & Secondary Primary – normal joints, monoarticular involvement, pts in 3 rd & 5 th decade Secondary – common in older pts, more than 1 jt involved, 25

Miligram’s Sequence:

Miligram’s Sequence Miligram ( 1977) sequence of 3 phases Phase I – active syn. Ds w/o loose bodies Phase II – active syn proliferation with e few loose bodies as well Phase III – multiple free loose bodies, with no demonstrable syn. Ds 26

Signs & Symptoms:

Signs & Symptoms Usually large jts involved(> 50% knee) Often asymptomatic Chronic Progressive pain Swelling of the affected jt Hx of locking of the jt 27

Signs & Symptoms:

Signs & Symptoms Joint effusion Associated muscle wasting Articular tenderness Articular crepitus Limited range of motion 28

Diagnosis :

Diagnosis Plain Radiographs Typical finding is of multiple, smooth, oval-shaped calcified masses They have a characteristic popcorn-ball appearance of calcified cartilage. They may pass from the main joint cavity into a neighboring soft tissue Articular surface is usually intact, except in secondary SOC 29

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Lateral knee radiograph shows extensive areas of intraarticular calcification about the knee A few Extra Articular calcification can also be visualized 30

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Anteroposterior radiograph of the hip Shows multiple calcified masses about the hip (arrows) with joint space narrowing resulting from secondary osteoarthritis 31

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CT Scan CT is best utilized in earlier stages CT can effectively detect non-calcified masses or those with only minimal calcification, which allows to distinguish this condition from a simple joint effusion. 32

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MR appearance depends on composition of the intra- articular body. Entirely cartilaginous bodies will appear isointense to muscle on T1 and hyperintense to muscle on T2 weighted images. Partly calcified intra- articular bodies demonstrate foci of absent signal on all pulse sequences. Like CT arthrography , MR with gadolinium may be used to detect intra- articular bodies that have not yet calcified. 33

Gross Features:

Gross Features Cartilaginous bodies or osteocartilaginous bodies with central ossification. They are typically spherical in shape. Sizes range from several millimeters to several centimeters in diameter. The synovium demonstrates villous hyperplasia, which imparts a wrinkled appearance on gross examination. C artilaginous bodies attached to the synovium . 34

Histological Findings:

Histological Findings Loose bodies have a chondroid matrix Cells have an eosinophilic cytoplasm Large nucleii , mitoses rarely seen Synovium show villous hypertrophy Subintima is oedematous 35

Differential Diagnosis:

Differential Diagnosis Trauma-related: Fracture with avulsed fragment Fragmentation of meniscus with calcification Degenerative joint disease related: Degenerative joint disease with detached spur Synovial proliferation: Pigmented villonodular synovitis 36

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Neoplastic : Synovial chondrosarcoma Other: Osteochondritis dissecans Sequestrum from osteomyelitis Neuropathic (Charcot) joint 37

Treatment :

Treatment The treatment of choice for synovial chondromatosis , either intraarticular or extraarticular ( bursal or tenosynovial ), is surgical resection. Resection may be conducted both by open and arthroscopic techniques 38

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Nonsteroidal anti-inflammatory drugs can be used in pts with no mechanical sym. Transcutaneous therapies ( eg , ultrasound, thermal therapies) for reduction of inflammation. Patients with primarily mechanical symptoms do not benefit significantly from nonoperative therapy. Radiotherapy as a successful modality when used for synovial chondromatosis of the knee refractory to several previous surgical interventions .Chong CC et al. [Medline] 39

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With advancement in arthroscopic techniques, there is recent interest in the arthroscopic treatment of this disease. Technically demanding. Helps avoid the patient morbidity caused by a large, open procedure Eases postoperative rehabilitation 40

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C ontroversy exists regarding the extent of surgical treatment required Specifically, whether removal of chondral bodies alone is sufficient or whether associated synovectomy is also necessary. Shpitzer and co-workers found no difference in prognosis after a 6-year follow-up for 26 patients who underwent either removal of chondral bodies only or synovectomy as well as chondral fragment removal Maurice and colleagues showed similar results in a study of 53 patients 41

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However, other researchers have shown that the recurrence rate is higher among patients who undergo chondral body removal only Ogilvie-Harris and Saleh reported a 60% recurrence rate among patients managed with loose body removal alone. In contradistinction, patients who underwent associated synovectomy had no recurrences 42

Recurrence:

Recurrence The overall recurrence rate for intraarticular disease in larger series appears to range from 3% to 23% Recurrence may be related to incomplete resection in many cases 43

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Answer to these conflicts was given by Miligram Milgram advocated synovectomy to treat active phase 1 disease ( intrasynovial disease without chondral bodies), Synovectomy with removal of chondral fragments for phase 2 disease (transitional form with intrasynovial disease and chondral bodies), Removal of the multiple chondral bodies alone and no synovectomy for phase 3 disease (late inactive disease with chondral bodies but no synovial abnormality). 44

Malignant Transformation of Synovial Chondromatosis:

Malignant Transformation of Synovial Chondromatosis Chondrosarcoma arising from primary synovial chondromatosis has been considered an extremely rare event, particularly considering that primary synovial chondromatosis itself is an infequent condition on its own Although synovial chondrosarcoma may arise as the primary condition, the vast majority of cases are related to preexisting primary synovial chondromatosis . 45

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These patients present with pain and swelling, clinical symptoms similar to those accompanying primary synovial chondromatosis The majority of patients with malignant transformation of primary synovial chondromatosis have long-standing disease with multiple local recurrences. Because local recurrence of primary synovial chondromatosis is common hence distinguishing recurrent disease from malignant transformation may be difficult 46

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Treatment of patients with malignant transformation of primary synovial chondromatosis usually requires amputation. Despite this aggressive therapy, metastases to the lung are common and were reported in 56% of patients with follow-up in the series of Bertoni et al As with other chondrosarcomas , synovial chondrosarcoma is typically not effectively treated with chemotherapy and radiation therapy 47

Summary:

Summary The joints commonly affected with synovial chondromatosis are the knee, hip, ankle, and elbow. Pt usually present with chronic pain with or with ass. locking of the joint Can usually be diagnosed on a plain Xray Some may require further imaging Rx of choice is removal of loose bodies with synovectomy 48

Summary:

Summary The majority of patients with malignant transformation of primary synovial chondromatosis have long-standing disease with multiple local recurrences. However, rapid increase in the size of the lesion in a patient with known primary synovial chondromatosis or a rapidly deteriorating clinical course should prompt suspicion of malignant transformation and biopsy of the lesion. Metastases to the lungs are a clear sign of malignancy 49