Outcome of Tendon Transfer for Monomelic Amyotrophy (Hirayama Disease).

Hirayama disease is a motor neuron disease predominantly affecting adolescent males. The identifying feature of Hirayama disease is unilateral forearm and intrinsic muscle weakness that spares the brachioradialis, termed "oblique atrophy." Hirayama disease progresses slowly over several years, followed by an abrupt arrest. The pathognomonic finding is the anterior displacement of the cervical spinal cord with the detachment of the posterior dura. Systematic clinical evaluation and appropriate diagnostic studies are crucial to rule out a variety of compressive, immune-mediated, and genetic disorders. We present a patient with Hirayama disease whose hand function was improved dramatically by a tendon transfer after nearly 3 years without a definitive diagnosis and call attention to the hand surgeon's role in identifying this rare disease to enable timely functional restoration.

Treatment of Painful Lower Extremity Neuromas with Processed Nerve Allograft.

BACKGROUND: Painful neuromas remain a challenge for both patients and surgeons. Despite numerous described treatments, they are often unreliable with variable outcomes. This study evaluated the use of processed nerve allografts for the treatment of painful lower extremity neuromas by either reconstruction or transposition into muscle. The null hypoth- esis was that both techniques for painful neuromas would not result in improved pain or functional outcomes. METHODS: Retrospective review was performed of 12 pa- tients treated by a single surgeon for painful lower extremity neuromas with the utilization of processed nerve allograft either with elongation of the residual nerve stump and trans- location into muscle (n = 7) or nerve reconstruction (n = 5). Patient demographics, surgical details, and outcomes data were evaluated comparing preoperative and postoperative PROMIS (Patient Reported Outcomes Measurement Infor- mation System) scores. Patients underwent preoperative workup with imaging (ultrasound and magnetic resonance imaging). Utilizing a processed nerve allograft, reconstruc- tion was performed if the proximal and distal nerve ends were identifiable, otherwise translocation to muscle was performed to preserve proximal nerve branches. RESULTS: Average follow-up was 15.2 months (SD: 11.4). Neuroma locations included intermetatarsal (n = 4), sural (n = 1), deep peroneal (n = 3), superficial peroneal (n = 4), and medial plantar (n = 1). Five patients failed a previous neuroma surgery, five patients had prior surgery in the zone of injury, one patient sustained a traumatic laceration, and one patient had a motor vehicle collision (MVC) requiring multiple previous surgeries. All but one patient had at least one prior surgery, with seven patients (five translocation, two reconstruction) having undergone a previous attempt to spe- cifically address neuroma pain. Preoperative injection when administered demonstrated improvement in pain and symp- toms in six of seven and two of two of the translocation and reconstruction groups, respectively. Preoperative ultrasound identified a neuroma in four of seven translocation and all four reconstruction patients. Pathology confirmed a neuroma in all 12 patients. Outcome data were available for 10 patients (six translocation, four reconstruction), which demonstrated a statistically significant improvement in PROMIS interference (p = 0.006), intensity (p = 0.011), pain behavior (p = 0.016), and NRS (p = 0.0004). Three patients underwent revision for recurrent neuroma: one translocation, two reconstruction. CONCLUSIONS: For patients with painful cutaneous neu- romas, translocation and reconstruction using processed nerve allografts improved pain in most patients, however, 25% required revision surgery. Three patients had neuroma occurrence requiring revision surgery, prompting caution when counseling patients about outcomes and recurrence.