Definition
Rare congenital anomaly with enlargement of the finger
Epidemiology
0.9%
Aetiology
Uncertain
Strong FHx
Pathology
All elements involved
- compared to enlargment from tumour /haemangioma
Index finger most commonly affected
- involved digit is stiff & angulated
- always large at birth compared with 2° enlargement
Classification
Primary
- true macrodactyly
- all tissues enlarged
Secondary
- neurofibromatosis
- hemangioma
- lymphangioma
- AVM
- fibrous dysplasia
- lipoma
NHx
1. Static type
- grows with child at normal rate
2. Progressive type
- progressive enlargement out of proportion to normal growth
Operative Management
Indications
Most patients will require surgery but surgery often unsuccessful
Options
Debulking procedures
- fat & skin
- may need multiple debulks
Epiphysiodesis
- when digit achieved adult length
Finger shortening
- removal of segments from P2 & P3
- has a high complication rate
- stiffness, pain, residual deformity & skin problems
Ray resection
- salvage
Usual approach
Staged procedure
- operative on one side of finger at a time
- convex side first
- remove up to 25% of phalanx
- remove half of soft tissue from each side
Epiphysiodesis once digit same size as parents digits
Uncontrolled growth
Ray resection
May get accelerated growth in adjacent digits after ray amputation