Introduction  |
- A congenital upper extremity deformity characterized by
- deficiency of the ulna and/or the ulnar sided carpal structures
- unstable elbow and stable wrist or vice versa
- elbow abnormalities more common than wrist abnormalities
- Epidemiology
- 5-10 times less common than radial club hand
- Associated conditions
- medical
- not associated with systemic conditions like radial club hand
- orthopaedic conditions
- PFFD
- fibula deficiency
- scoliosis
- phocomelia
- multiple hand abnormalities
- almost all patients have absent ulnar sided digits
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| Presentation |
- Symptoms
- limited function
- usually painless
- Physical exam
- shortened, bowed forearm
- decrease in elbow function
- loss of ulnar digits
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| Classification |
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| Bayne Classification |
| Type 0 |
• Deficiencies of the carpus and/or hand only
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| Type 1 |
• Undersized ulna with both growth centers present
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| Type II |
• Part of the ulna is missing (typically the distal ulna is absent) |
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| Type III |
• Absent ulna |
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| Type IV |
• Radiohumeral synostosis |
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There is a subtype of each classification that is based on the first webspace
• A = Normal
• B = Mild deficiency fo the webspace
• C = Moderate to severe deficiency of the webspace
• D = Absent webspace
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| Treatment |
- Goals
- treatment depends on multiple factors including
- hand position, thumb function, elbow stability, syndactyly
- thumb condition is most important factor to consider for treatment
- Nonoperative
- stretching and splinting
- indications
- used in early stages of treatment
- Operative
- syndactyly release and digital rotation osteotomies
- indications
- done at 12-18 months of age
- radial head resection and creation of a one-bone forearm
- indications
- Stage II to provide stability at the expense of forearm motion
- there is no good option for restoring elbow motion
- corrective procedures should not be performed until the child is at least 6 months old
- osteotomy of the synostosis
- indications
- may be required in Stage IV to obtain elbow motion
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