An 11-year-old male presented to the orthopedic clinic with painless inability to supinate his left forearm. The patient’s mother reported she first noticed a difference in his left upper extremity mobility when he was 6 years old, but he was not reporting any discomfort or symptoms at the time. The patient’s father is also known to have a congenital upper extremity deformity.
Forearm radiographs taken in anteroposterior and lateral views demonstrated a short segment of osseous synostosis between the proximal radius and ulna, with mild bowing of the radius. Otherwise, the radial head was normally located. There was no evidence of fracture. Patient positioning limitations while the radiograph was being obtained further increased suspicion of radioulnar synostosis as a cause of the patient’s symptoms. According to the Cleary classification, these radiographs are consistent with type II proximal radioulnar synostosis.
Background and clinical perspective
Congenital proximal radioulnar synostosis is a rare malformation of bone development in which the proximal radius and ulna fuse [1]. It is bilateral in 60-80% of cases and often diagnosed in childhood after beginning sports when the child notices decreased rotational passive and active movements [2,3]. Symptoms of radioulnar synostosis involve restricted pronation and supination of the forearm which is typically non-painful. The exact etiology is unclear, but appears to be X-linked dominant with paternal inheritance [1]. Radioulnar synostosis may also develop following trauma and has been suggested to develop in relation to posterior radial head dislocation [1].
Imaging perspective
Anteroposterior and lateral radiographs of the forearm and elbow are recommended for evaluation of proximal radioulnar synostosis. A bony or fibrous synostosis may be visible, in addition to a wide and bowed radius, narrow and straight ulna, and possible dislocation or malformation of the radial head. Classification is determined by the Cleary Classification of Congenital Proximal Radioulnar Synostosis [2,3]. Type I includes fibrous synostosis with a normal anatomical location of the radial head. Type II involves osseous synostosis with a normal anatomical location of the radial head. Type III involves osseous synostosis with a hypoplastic and posteriorly dislocated radial head. Type IV involves short osseous synostosis with an anteriorly dislocated mushroom shaped radial head.
Outcome
Treatment options include observation or surgical intervention. Observation is the most common treatment especially in asymptomatic or unilateral cases. Operative treatment may be indicated in patients with severe or bilateral deformities limiting activity. Synostosis excision with soft tissue interposition may be performed to provide active forearm rotation. The increase in forearm rotation is typically only mild and recurrence is common. Alternatively, forearm derotational osteotomy may be performed to place the forearm in a more functional position. This procedure usually results in improved forearm position and patient function with a low rate of recurrence [4]. Surgical interventions carry a risk of refusion, compartment syndrome, or neurologic deficits [5].
Take home message
Congenital proximal radioulnar synostosis is a rare condition resulting in the proximal radius and ulna fusing, resulting in reduced range of rotational movements. Imaging will demonstrate a bony or fibrous synostosis and may involve dislocation of the radial head. Treatment can include observation, synostosis excision, or forearm derotational osteotomy.
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Congenital proximal radioulnar synostosis
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Based on the provided anteroposterior and lateral X-ray images of the forearm, it can be seen that there is a fusion or close contact between the proximal radius and ulna in the left forearm, suggesting an abnormal structure of the proximal radioulnar joint. Specific radiological features include:
Combining the patient’s history (limited forearm rotation noticed since age 6, no significant pain, and a paternal history of congenital upper limb deformity) and the radiological findings, the following potential diagnoses are considered:
Taking into account the patient’s age, clinical presentation (restricted rotation with no pain), family history (father has a similar congenital deformity), and imaging findings, the most likely diagnosis is:
Congenital Proximal Radioulnar Synostosis.
For congenital proximal radioulnar synostosis, treatment strategies can be divided into conservative management and surgical intervention, chosen according to the patient’s functional requirements and the severity of the deformity.
Rehabilitation and Exercise Prescription Recommendations:
This report is a reference analysis and cannot replace in-person consultation or professional medical advice. Treatment and rehabilitation plans should be carried out under the supervision of orthopedic specialists and rehabilitation physicians.
Congenital proximal radioulnar synostosis