ABSTRACT:
Reichel syndrome or primary synovial chondromatosis (PSC) is an uncommon benign metaplastic
condition that usually affects large joints. Though shoulder involvement was scarce,
there are only a few cases in the pediatric population. A 14- year-old boy was admitted
to the Pediatric Orthopedics department with right shoulder pain for 14 months. Imaging
revealed multiple loose bodies distributed throughout the glenohumeral joint. Upon
the arthroscopic approach, we remove all cartilaginous nodules within the glenohumeral
space and abarticular tendon. Histopathologic examination confirmed the diagnosis
of primary synovial chondromatosis. At follow-up, the patient remains free of symptoms,
and shoulder radiographs showed no recurrence of calcification. The present case illustrates
the clinical patterns, imaging features, histological findings, and therapeutic management
of shoulder primary synovial chondromatosis in a pediatric patient.
Keywords: chondromatosis; pediatrics; shoulder; synovial.
RESUMO:
A síndrome de Reichel ou condromatose sinovial primária (CSP) é uma metaplasia benigna
incomum geralmente observada em grandes articulações. O acometimento do ombro é raro
e há apenas alguns casos na população pediátrica. Um menino de 14 anos foi atendido
no departamento de Ortopedia Pediátrica com dor no ombro direito há 14 meses. Os exames
de imagem revelaram múltiplos corpos livres distribuídos por toda a articulação glenoumeral.
À abordagem artroscópica, removemos todos os nódulos cartilaginosos no interior do
espaço glenoumeral e tendão abarticular. O exame histopatológico confirmou o diagnóstico
de CSP. Ao acompanhamento, o paciente não apresenta sintomas e as radiografias do
ombro não mostraram recidiva da calcificação. Este caso ilustra os padrões clínicos,
as características em técnicas de diagnóstico por imagem, os achados histológicos
e o manejo terapêutico da CSP no ombro de um paciente pediátrico.
Palavras-chave: condromatose; ombro; pediatria; sinovial.
FIGURES
| Citation: Ferjani HL, Bettaib H, Dorra BN, Maatallah K, Jenzri M, Hamdi W. Reichel Syndrome in Children: A Case Report. 59(Suppl 2):212. doi:10.1055/s-0044-1779511 |
| Note: Work carried out at the Kassab Orthopedics Institute, Mannouba, Tunisia. |
|
Conflito de Interesses Os autores não têm conflitos de interesses a declarar. |
| Received: August 10 2022; Accepted: September 15 2022 |
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.
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).
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 literature3,4,5,7 (►Table 1).
| Age | Sex | Duration of symptom (months) | Trigger factor | Clinical presentation | Surgical option | Follow-upPeriod (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 | Arthrotomyand 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 | Arthrotomyand synovectomy | 9 | No |
| Sinikumpu et al.2020.5 | 14 | Male | 12 | Sporting activities | - Shoulder pain- Stiffness- Palpable mass | Arthrotomyand synovectomy | 12 | No |
| The present case2021 | 14 | Male | 14 | No | - Shoulder pain- Discomfort | Arthroscopyand 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.
