Is carpal tunnel decompression warranted for HNPP?

Abstract

The role of carpal tunnel decompression surgery for patients that have hereditary neuropathy with liability to pressure palsy (HNPP) is currently unknown. Since recovery from carpal tunnel compression is often associated with remyelination or nodal reconstruction rather than axonal regeneration, it is uncertain whether the PMP22 deletion associated with HNPP interrupts myelin or nodal reconstitution. We describe two patients with genetically confirmed HNPP and symptomatic carpal tunnel syndrome that had clinical and electrophysiological improvement after surgical decompression. The findings indicate a capacity for conduction repair in HNPP. They also suggest a need for further investigation and discussion around whether to offer carpal tunnel decompression to symptomatic HNPP patients.

Inherited Neuropathies

“…The role of surgery for compressive neuropathies in HNPP remains unclear, and emphasis should be placed on preventing injury. 46…”

Carpal tunnel syndrome in inherited neuropathies: A retrospective survey

“…Data are lacking regarding outcomes of interventions for CTS in inherited neuropathies to inform development of standards of care for the upper extremity in different forms of CMT and hereditary neuropathy with liability to pressure palsies (HNPP). The role of carpal tunnel release in patients with inherited neuropathy is currently unknown . This study evaluates CTS symptom severity and therapy outcomes in patients with inherited neuropathy.…”

“…The role of carpal tunnel release in patients with inherited neuropathy is currently unknown. 8 This study evaluates CTS symptom severity and therapy outcomes in patients with inherited neuropathy.…”

Rapid improvement of hereditary neuropathy with liability to pressure palsies following cubital tunnel release

“…Taggart and Allen reported a patient with moderate–severe ulnar neuropathy at the elbow who recovered completely 6 months after release . Two patients with moderate–severe median neuropathy at the wrist improved after flexor retinaculum release …”

“…Entrapment neuropathies in hereditary neuropathy with liability to pressure palsies (HNPP) are usually managed conservatively by avoiding pressure and bracing, although recovery is often incomplete . Evidence that surgical release of nerves at entrapment sites is beneficial in HNPP is limited . We describe 3 cases of patients with HNPP and moderate ulnar neuropathy at the elbow with conduction block who had complete and rapid recovery after cubital tunnel release surgery.…”