Keywords
chondromatosis - pediatrics - shoulder - synovial
Introduction
Reichel syndrome or primary synovial chondromatosis (PSC) is a benign tumor with cartilaginous
nodules in the synovium joints.[1]
[2]
[3] The glenohumeral joint is an unusual location in PSC, particularly in pediatric
patients.[3]
[4]
[5] To our knowledge, there are only 5 cases of children reported in the literature.
Herein, we report a rare case of PSC in a 14-year-old boy with an uncommon localization
in the shoulder revealed by the chronic pain and the limited motion in the right arm.
Case Report
A 14-year-old, right-handed boy presented to the Pediatric Orthopedics department
with right shoulder pain. He complained for 14 months of a history of pain and discomfort
in his right shoulder.
He reported no symptoms of weight loss, fatigue, systemic signs, or any other arthralgia.
On physical examination, there was no obvious deformity or atrophy involving the affected
shoulder. We noted a decreased range of motion, in comparison to the uninvolved side,
with respectively: flexion to 160°, extension to 40°, abduction to 140°, adduction
to 40°, internal rotation to L4, and external rotation to 50°. Subacromial impingement
signs, as well as rotator cuff tear tests, were negative.
Plain radiographs showed multiple radio-opaque bodies distributed throughout the glenohumeral
joint, without bone defection or joint narrowing ([Fig. 1A]). Subsequent Magnetic resonance imaging (MRI) revealed a high number of calcified
intra-articular loose bodies around the joint and the biceps tendon ([Fig. 1B]). PSC was strongly suspected.
Fig. 1
A) radiograph of the right shoulder demonstrates the presence of numerous radiopaque
loose bodies in the glenohumeral joint. B) STIR magnetic resonance image shows an excess of synovial joint fluid and multiple
centers of nodular calcification around the joint and within the biceps tendon shea.
We choose a shoulder arthroscopy using a deltopectoral approach to remove the tumors
nodules. More than 50 shiny and solid bodies, with an average size of 10–15 mm, were
retrieved ([Fig. 2]). There were also several bodies within the coracoid process and the conjoint tendon.
The synovial tissue, bursas, and cartilages appeared intact. Upon removing the particles,
we complete a partial synovectomy to avoid the relapse. Histology of loose bodies
and synovium confirmed the diagnosis of PSC without any evidence of malignant transformation
([Fig. 3]).
Fig. 2 Numerous synovial Chondromatosis nodules removed from the shoulder joint (size between
10 and 15 mm).
Fig. 3 Histological image of the lesion showing multiple cartilaginous nodules composed
of clustered chondrocytes, embedded in synovium. Note the presence of focal calcification
and enchondral ossification (small arrow). The nodules are surrounded by a rim of
residual synovial tissue (large arrow). (hematoxylin-eosin, original magnification × 400).
Postoperative shoulder X-rays did not show any densities. The patient was discharged
after the surgery using the arm sling. At three months of follow-up, the patient remains
free of symptoms, and shoulder radiographs showed no recurrence of calcification.
Discussion
First mentioned by Jaffe et al.,[1] PSC is a rare benign tumor affecting the synovial cavity. It is a proliferation
of multiple cartilaginous nodules in the synovium of joints, tendon sheaths, and bursae.
According to the literature, this disorder usually occurs in men between the ages
of 30 and 50 old-years. It has been reported that the knees, hip, elbow, and wrists
are the main affected joints in descending order of frequency.[6]
[7] The involvement of the shoulder is unusual in adults, and more exceptional during
childhood. To the best of our knowledge, only five cases have been reported in the
literature[3]
[4]
[5]
[7] ([Table 1]).
Table 1
|
Age
|
Sex
|
Duration of symptom (months)
|
Trigger factor
|
Clinical presentation
|
Surgical option
|
Follow-up
Period (months)
|
Recurrence
|
|
Nashi et al.1998.[10]
|
14
|
Male
|
6
|
Sporting activities
|
- Shoulder pain
|
Under observation
|
24
|
−
|
|
Miranda et al.
2004.[7]
|
10
|
Female
|
1
|
Sporting activities
|
- Shoulder pain
- Discomfort
|
Arthrotomy
and synovectomy
|
12
|
No
|
|
Hamada et al.
2005.[4]
|
14
|
Female
|
18
|
Sporting activities
|
- Shoulder pain
- Discomfort
|
Arthroscopy
|
36
|
No
|
|
Kirchoff et al.
2008.[3]
|
14
|
Male
|
12
|
No
|
- Shoulder pain
- Palpable mass
|
Arthrotomy
and synovectomy
|
9
|
No
|
|
Sinikumpu et al.
2020.[5]
|
14
|
Male
|
12
|
Sporting activities
|
- Shoulder pain
- Stiffness
- Palpable mass
|
Arthrotomy
and synovectomy
|
12
|
No
|
|
The present case
2021
|
14
|
Male
|
14
|
No
|
- Shoulder pain
- Discomfort
|
Arthroscopy
and synovectomy
|
5
|
No
|
Based on the underlying pathogenesis, synovial chondromatosis may be primary or secondary.
The PSC, called idiopathic synovial osteochondromatosis or Reichel syndrome, usually
occurs in a previously healthy joint.
In contrast, secondary osteochondromatosis is a sequela of intra-articular pathology
as osteochondral fracture, osteochondritis dissecans, and osteoarthritis.[4]
[6]
[8]
In our case, the young-onset, the absence of a history of trauma, and the unremarkable
results of blood tests strengthen the diagnosis of PSC.
It is noteworthy that histopathologic analysis is mandatory to distinguish between
these conditions.[4]
[6]
[9] In a series of 136 presumed synovial osteochondromatosis, Villain et al showed that
the histopathological patterns are different. In the present case, the histologic
evaluation revealed multiple cartilaginous nodules arranged in clusters and embedded
in the synovium.[9]
Clinical presentation is often nonspecific.[2]
[3]
[6] As a result, patients may experience long symptoms delays before the final diagnosis.
Like the current case, most children with PSC of the shoulder were diagnosed between
10 to 14 years old with a diagnosis delay ranging from 1 to 18 months from symptoms
onset.[3]
[4]
[5]
[7]
In a recent literature review of cases with osteochondromatosis occurring in the shoulder,
the most common reported symptoms were mainly shoulder pain, uncomfortable feeling
during exercises, and locked joint movement.[7] Our report is the following data in the literature. Interestingly, a palpable bony
mass may occur, as described in two children with PSC of the shoulder.[3]
[5]
Radiographic features vary according to the degree of ossification. In the later stages
of the disease, the plain radiographs showed a characteristic image with multiple
intraarticular radio-opacities. These calcifications are frequently very similar and
uniform in size with a typical nest-like arrangement. Thus, plain radiographs may
be normal in the earlier stages (30% of cases).[8] Sometimes, the diagnosis overlaps between differential diagnostics such as osteosarcoma
and chondrosarcoma.
Hence, MRI plays a pivotal role in confirming the diagnosis by revealing intrasynovial
hypointense nodules on T1 and T2-weighted images. MRI also aids in the management
of surgical approaches.[2]
[3]
[5]
The optimal therapeutic management of the disease requires surgical removal of any
loose bodies.[3]
[6]
[7]
[8] Partial synovectomy, optional but often recommended, may decrease the recurrence
rate.[5]
[6]
[8] Histopathological analysis of the loose bodies and synovial tissue is mandatory
as a malign transformation may occur in up to 5%.[2]
[3]
The choice of surgical procedure is still a matter of debate.[6] Open surgery remains the mainstay of treatment and is highly recommended in cases
of osteochondromatosis with soft-tissue involvement and limited anatomic space access.[3] Moreover, this approach was often preferable in pediatric patients with shoulder
involvement.[3]
[5]
[10] In line with Hamada et al, we opted for shoulder arthroscopy using the deltopectoral
approach.[4]
According to the literature, recurrence is common and ranges between 15% and 30%.[5]
[6] It's noteworthy to mention that no recurrence of calcification was reported among
pediatric patients with PSC of the shoulder.[3]
[5]
[10] In our case, the short duration of follow-up was not sufficient to make a definitive
conclusion.
The present case illustrates a rare entity of PSC that combined the intra and extraarticular
involvement of the shoulder. MRI is a powerful key for early diagnosis. The management
of this affection, like in adult patients, is based on the chondromyxoid bodies removed
through open or arthroscope-assisted surgery. Histological analysis is mandatory since
a malign transformation might occur.
