Related Experiment Video
Updated: Jun 25, 2026

04:08
Metacarpal Small Incision for Carpal Tunnel Syndrome
Published on: April 5, 2024
Neurofibromatosis contributing to carpometacarpal instability
Maximillian Soong1, Edward Akelman
1Department of Orthopedic Surgery, Lahey Clinic, 41 Mall Rd, Burlington, MA 01805, USA.
Orthopedics
|February 20, 2009
Summary
Neurofibromatosis type 1 can cause rare hand instability, leading to carpometacarpal joint dislocation. This case highlights successful percutaneous pin fixation for this unique orthopedic complication.
Area of Science:
- Orthopedic Surgery
- Medical Genetics
Background:
- Neurofibromatosis is a genetic disorder known for causing spinal and long bone abnormalities.
- Skeletal manifestations in the hand are infrequently reported in neurofibromatosis patients.
Observation:
- A 29-year-old male with neurofibromatosis type 1 presented with acute right hand pain and deformity.
- Physical examination revealed significant swelling due to a plexiform neurofibroma and radiographic evidence of dorsal dislocation of the 4th and 5th carpometacarpal joints.
- Imaging also showed erosions, cystic changes, and articular rounding suggestive of underlying bony abnormalities.
Findings:
- Initial closed reduction and splinting failed due to immediate redislocation, indicating significant carpometacarpal instability.
- Percutaneous pin fixation was successfully employed to stabilize the joints, carefully avoiding the adjacent neurofibroma.
- The patient achieved a stable reduction and returned to full function within 6 months post-procedure.
Implications:
- This case underscores the potential for neurofibromatosis to cause severe carpometacarpal instability.
- Percutaneous fixation offers a viable treatment option for such injuries, particularly when avoiding extensive dissection near neurofibromas is crucial.
- Highlights the importance of considering hand abnormalities in the orthopedic evaluation of neurofibromatosis patients.
