Some patients develop ulnar nerve compression due to rare anatomical variations or malformations. The aims of this review are to provide a comprehensive overview of anatomical structures and variations that can cause ulnar nerve compression and to evaluate treatment options. Case reports and case series about rare cases of unusual ulnar nerve compression published from January 2000 until April 2022 were obtained from databases Embase, MEDLINE, and Web of Science. A total of 48 studies describing 64 patients were included in our study. The following structures have proven to cause ulnar nerve compression: anconeus epitrochlearis, accessory abductor digiti minimi, vascular anomalies, palmaris longus, fibrous bands, and flexor carpi ulnaris. All cases except one have had a surgical release of the ulnar nerve resulting in diminished symptoms or complete recovery at follow-up. In addition to considering common compression points, it is important to be aware that proximal compression symptoms, such as pain and a positive Tinel sign at the medial elbow, may be attributed to a hypertrophic AE or vascular anomaly. Distal compression symptoms encompass swelling, along with pain and a positive Tinel sign at the distal forearm. Various structures contributing to distal compression include an accessory abductor digiti minimi muscle, an accessory or anomalous palmaris longus muscle, or an accessory or hypertrophic flexor carpi ulnaris muscle. The occurrence of fibrous bands exhibits variability, manifesting in diverse locations across the arm.Level of Evidence: IV.
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