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A 3-year-old boy with synovial chondromatosis of the distal radioulnar joint

Issue: July 2019

ByMilan Stevanovic, MD, PhD

ByAdam Lindsay, MD, MS

ByNima Kabirian, MD

ByVenus Vakhshori, MD

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An otherwise healthy 3-year-old boy presented to the pediatric urgent care clinic 2 days after his mother noticed left distal forearm swelling in the child without a known inciting event. The patient appeared to have no pain, skin changes or constitutional symptoms. Neurovascular status and range of motion of the wrist were intact. There was mild tenderness to palpation of an approximately 2-cm, firm fixed mass deep to the subcutaneous tissues at the level of the distal radioulnar joint. Laboratory evaluation was unremarkable. Radiographs revealed soft tissue swelling about the wrist without focal calcifications or cortical irregularity (Figure 1). Ultrasound was remarkable for an ill-defined heterogeneous soft-tissue lesion in the left wrist with mild adjacent cortical thickening and minimal periosteal reaction, without evidence of hypervascularity (Figure 2). One week later, the patient underwent MRI with and without gadolinium contrast which demonstrated a 2 cm (anteroposterior) by 2 cm (longitudinal) by 1.3 cm (transverse) heterogeneous non-enhancing mass that was mildly hyperintense on T1 and T2, diffusely bright on short-TI inversion recovery (STIR), with associated inflammatory change interposed between the distal radius and ulna in the volar aspect of the wrist, just distal to the pronator quadratus. There was no bony involvement, but there was reactive bony remodeling of the distal ulna and radius (Figure 3).

What is the next step in diagnosis, treatment?

See answer on the next page.

Source: Venus Vakhshori, MD

Biopsy, surgical excision of the mass via a volar approach

The patient remained asymptomatic. To clarify the diagnosis, an ultrasound-guided core needle biopsy was performed 2 months after presentation. Histology revealed a chondromyxoid lesion with no high-grade features. Surgical excision of the mass was performed 3.5 months after initial presentation using a volar approach to the wrist. The lesion was found deep to the pronator quadratus at the level of the distal radioulnar joint. The mass was well encapsulated. The mass was resected en bloc with attached capsule and measured 2 cm by 2.1 cm by 1 cm (Figure 4). Histology revealed a marble-white mass with firm, cartilage-like consistency and homogenous white material with pinpoint central hemorrhage less than 1 mm in diameter. The associated capsule showed synovial tissue with chronic inflammation. Pathology was consistent with synovial chondromatosis.

The patient recovered uneventfully. He had intact range of motion and neurovascular status. He had no recurrence at 12 months after surgery.

Discussion

Synovial chondromatosis is an idiopathic condition characterized by metaplasia of the synovium, leading to intra- or extra-articular osteocartilaginous bodies that can vary in size. These loose bodies may or may not be visible on radiographs depending on the presence of calcification. Intra-articular synovial chondromatosis is typically monoarticular and primarily affects large joints. Extra-articular tenosynovial chondromatosis is more common in the flexor tendons of the fingers. It is most common in the third to fifth decades of life and affects men more frequently than women. Synovial chondromatosis carries a low, 2.5% risk of malignant transformation to chondrosarcoma.

Synovial chondromatosis has been documented in the metacarpophalangeal and interphalangeal joints of the hand. When smaller joints are involved, younger women are more commonly affected. In adults, few cases of synovial chondromatosis of the distal radioulnar joint have been reported in the literature. This condition is rare in the pediatric population. Since 1968, only two dozen cases of pediatric synovial chondromatosis have been reported in the literature (Table 1), most of which involved large joints, including the knee, hip and shoulder. Other reported locations include the biceps tendon sheath, cervical spine, elbow, ankle and the sternoclavicular and acromioclavicular joints. The hand is an exceedingly uncommon location for synovial chondromatosis and only two cases have been reported in pediatric patients. To our knowledge, no cases involving the wrist have been reported. Additionally, all reported patients have been aged at least 5 years, which makes our patient the youngest reported case of primary synovial chondromatosis to date.

In 1977, James W. Milgram, MD, classified synovial chondromatosis into three phases: active intrasynovial disease without loose bodies; transitional lesions with active intrasynovial proliferation and free loose bodies; and multiple free osteochondral bodies without intrasynovial disease. Synovial chondromatosis is treated with removal of loose bodies to prevent joint destruction from abrasion. Additionally, synovectomy is sometimes performed. Milgram concluded that if the disease is in the third phase at the time of surgery, synovectomy may not be needed and the patient may be treated solely with loose body resection. F. Murphy and colleagues advocated for synovectomy due to the risk of recurrence. Recurrence rates in adults have been reported to be similar with or without synovectomy, ranging from 11% to 23%, more commonly in large joints. As such, some authors argue against synovectomy in young children, especially if pain and swelling are not present and symptoms are primarily mechanical. Despite this, in the pediatric cases reviewed, most patients underwent synovectomy. In our case, the loose body was resected along with the surrounding synovial tissue which showed chronic inflammation.

Key points are as follows:

• Synovial chondromatosis is a benign metaplastic condition of the synovium which can cause intra- or extra-articular cartilaginous loose body formation; and
• Synovial chondromatosis is rare in children and most common in middle-aged adults.

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• For more information:
• Nima Kabirian, MD; Adam Lindsay, MD, MS; Milan Stevanovic, MD, PhD; and Venus Vakhshori, MD, can be reached in the department of orthopaedic surgery, Keck School of Medicine at the University of Southern California, 1520 San Pablo St., Suite 2000, Los Angeles, CA 90033. Kabirian’s email: nima.kabirian@med.usc.edu. Lindsay’s email: adam.lindsay@med.usc.edu. Stevanovic’s email: stevanov@med.usc.edu. Vakhshori’s email: venus.vakhshori@med.usc.edu.
• Edited by Joshua D. Johnson, MD, and Nicholas A. Trasolini, MD. Johnson is a chief resident in the department of orthopedic surgery at Mayo Clinic in Rochester, Minnesota. He will be a musculoskeletal oncology fellow at MD Anderson following residency. Trasolini is the administrative chief resident in the department of orthopaedic surgery at Keck Medical Center of the University of Southern California and will be a sports medicine fellow at Rush University Medical Center following residency. For information on submitting Orthopedics Today Grand Rounds cases, please email: orthopedics @healio.com.

Disclosures: Kabirian, Lindsay, Stevanovic and Vakhshori report no relevant financial disclosures.