Science News: Peripheral Nerve Compression Syndromes in Children

Submitted by: Rebecca O'Bryan, MD
Edited by: Niranjan N. Singh, MD, DM

Gallone G, Di Gennaro GL, Farr S. Peripheral nerve compression syndromes in children. J Hand Surg Am. 2020;45(9):857-863. doi:10.1016/j.jhsa.2020.04.028


Summary: Non-traumatic neuropathies of the upper limb are rare in children. Diagnostic and treatment parameters are lacking high quality evidence and guidelines. Carpal tunnel syndrome (CTS) was noted in children with lysosomal storage diseases, and ultrasound (US) was found to be more sensitive than nerve conduction studies (NCSs) for diagnosis; however, NCS was preferred for follow up post operatively. CTS was also noted in children with HNPP. In a small study of 12 children with PMP22 mutation/hereditary neuropathy with pressure palsy (HNPP), all showed electrophysiological evidence of nerve entrapment, and 7 had bilateral limb involvement. HNPP should be strongly suspected in children with bilateral CTS. Idiopathic CTS in children has been associated with playing musical instruments, sports, obesity, and abnormal anatomy. Cubital tunnel syndrome in children is rare, and is associated with sports participation, wheelchair/surgical/sleep positioning, and cubitus varus deformity, as well as HNPP. Children who present with clinical ulnar neuropathy at the elbow (UNE) for the most part failed conservative management, and went on to have surgical intervention. Outcomes were better in children with nontraumatic presentation. Congenital constriction band syndrome has also been described. These usually arise in utero, and require very prompt surgical intervention including excision of the affected nerve. Radial neuropathies in children have also been described, especially associated with labor and delivery. Patients tend to recover well without surgical intervention; however, surgical intervention is needed when compression is evident.

Comments: This article presented a nice summary of the development of upper extremity neuropathies in children. Evidence was lacking for the best path to diagnosis in both cubital tunnel and radial neuropathies in this population. Some attention was paid to diagnosis using NCS/EMG, but the bias of this article was that due to the technical difficulty of NCS in children, US or other modalities may be preferred. The article did not even address diagnosis modalities for cubital tunnel and radial neuropathies in children. While the article is very useful in reviewing treatment and presentation of these entities, more information and education would be beneficial for our surgical colleagues as to the adjunctive diagnostic value of NCS in children and adolescents.