CONGENITAL RADIOULNAR SYNOSTOSIS

STEVEN R. BOYEA, M.D., Resident, Orthopaedic Surgery

WILLIAM MACKENZIE, M.D., Attending, Pediatric Orthopaedic Surgery

20 March 1996

CLINICAL CASE PRESENTATION

ORTHOPAEDIC DEPARTMENT

THE ALFRED I. DUPONT INSTITUTE

WILMINGTON, DELAWARE

CASE HISTORY:

The patient is a 11 year old who presented for evaluation of his left arm. He states he has always had limited range of motion of his left arm, however, his mother states that it was not until recently she became concerned. The patient is right hand dominant. He denies any weakness or loss of feeling in his left hand or arm. He states that some movements are more difficult than others especially around the elbow. The child has no significant past medical history and has had a normal developmental history except for the left arm. He denies any trauma to the elbow or fractures/dislocations. This does not cause him any functional problems and he has no pain.

PHYSICAL EXAM:

The left arm demonstrates a shoulder with FROM and strength 5/5. The elbow has a flexion from 0 to 135 degrees, but the forearm is fixed in 45 degrees of pronation without active or passive supination. The wrist has FROM with some hypermobility, especially in radial and ulnar deviation. The strength is 5/5 in elbow flexion/extension, wrist flexion/extension and grip. He is neurovascularly, intact. The right arm also demonstrates normal motion at the elbow with flexion from 0-135 degrees but limited supination from 0-50 degrees and pronation from 0-20 degrees. The remainder of his exam was normal.

RADIOGRAPHS:

These demonstrate the left elbow to have a bony synostosis of the proximal radius and ulna, distal to the proximal radial physis but with anterior dislocation of the radial head. There is no evidence of a bony synostosis or dislocation in the right elbow. Internal Rotation , External Rotation , AP , Lateral .

ASSESSMENT:
TREATMENT:

CONGENITAL RADIOULNAR SYNOSTOSIS

Embryology
Epidemiology
Clinical Features
Radiographic Features
Classification
Management

Observation

Surgical correction

Methods

Taking down the synostosis and interposition of soft tissue

Kelikian/Doumanian swivel

Derotational osteotomy distal to synostosis site

Derotational osteotomy through the synostosis mass

Derotational osteotomy with the Ilizarov Method

  1. osteotomy through the synostosis
  2. fixation proximally with hybrid fixation
  3. fixation distally with half pins
  4. Ilizarov device with rotation/translation hinges
  5. rotation at a rate of 4 degrees per day
  6. low risk of vascular injury because of slow rotational correction
  7. REFERENCES:
  8. Bolano, L.E. Congenital Proximal Radioulnar Synostosis: Treatment with the Ilizarov Method. J Hand Surg 19A: 977-978, 1994.
  9. Charvat, K.A.; Hornstein, L.; Oestreich, A.E. Radio-ulnar synostosis in Williams syndrome: A frequently associated anomaly. Pediatr Radiol 21: 508-510, 1991.
  10. Cleary, J.E.; Omer, G.E., Jr. Congenital proximal radio-ulnar synostosis: Natural history and functional assessment. J Bone Joint Surg 67-A: 539-545, 1985.
  11. Kelikain, H.; Doumanian, A. Swivel for proximal radio-ulnar synostosis. J Bone Joint Surg 39-A: 945-952, 1957.
  12. Khalil, I.; Vizkelety, T. Osteotomy of the synostosis mass for the treatment of congenital radio-ulnar synostosis. Arch Orthop Trauma Surg 113: 20-22, 1991.
  13. Mital, M.A. Congenital radioulnar synostosis and congenital dislocation of the radial head. Orthop Cln N Am 7: 375-383, 1976.
  14. Simmons, B.P.; Southmayd, W.W.; Riseborough, E.J. Congenital radioulnar synostosis. J Hand Surg 8: 829-838, 1983.
  15. Wiley, J.J.; Loehr, J.; McIntyre, W. Isolated dislocation of the radial head. Orthop Rev 20: 973-976, 1991.


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