Terminal Phocomelia
Definition
Complete absence of some part distal to some point on the upper extremity
Incidence
1:20 0000
98% unilateral
Aetiology
Usually unknown ? AR trait
Thalidomide
Sites
Commonest level is proximal forearm
Also
- fingers / midcarpal / wrist / mid-forearm / mid arm
Management
Non-operative / Prosthesis
Passive terminal device started 3/12
Active device as child starts to use above device
Krukenberg procedure
Radius and ulna formed into pincers
Only in blind / bilateral
Intercalary Phocomelia
Definition
Loss of intercalated segment
- hand suspended from trunk
- loss of elbow joint
- deformed hand with only 3-4 digits
Aetiology
Seen with thalidomide
Often bilateral
Three types
1. Complete
- hand to trunk
2. Distal
- hand to arm
3. Proximal
- forearm to trunk