Is Response to a Pre-implant Diagnostic Peripheral Nerve Block Associated With Efficacy After Peripheral Nerve Stimulation Implantation? A Ten-Year Enterprise-Wide Analysis.

OBJECTIVES: This study aimed to assess whether patient response to targeted diagnostic peripheral nerve block before peripheral nerve stimulator (PNS) device implantation is associated with efficacy after PNS implantation. MATERIALS AND METHODS: The electronic medical records from the Mayo Clinic Enterprise (three quarternary care medical centers and additional satellite medical centers) were reviewed to identify patients who underwent PNS implantation between January 2014 and January 2023. A primary outcome of interest was to assess whether administration of a preimplant diagnostic peripheral nerve block predicted pain relief at three months and six months after temporary and permanent PNS implantation. Another primary outcome was to investigate whether there was an association between the pain relief from a preimplant diagnostic peripheral nerve block and pain relief after three and six months after temporary or permanent PNS therapy. Linear regression analysis was conducted for outcomes of interest. RESULTS: Of 193 eligible patients who underwent PNS therapy, a total of 173 patients were included in the final analysis and were stratified into either the temporary PNS cohort (n = 112) or the permanent PNS cohort (n = 61). Overall, 77.5% of all patients (134/173) underwent a preimplant diagnostic peripheral nerve block and reported a mean percentage relief of 70.1 ± 27.0 from the diagnostic block. Of patients in the temporary PNS cohort, there was no difference in postimplant percentage pain relief between patients who received a diagnostic block (n = 93) and control patients (n = 19) at three months (35.4 ± 36.0 vs 49.8 ± 36.1, respectively; ß -14.45, 95% CI -32.98 to 4.07, p = 0.125) or at six months (23.3 ± 30.8 vs 45.7 ± 40.0, respectively; ß -22.39, 95% CI -46.86 to 2.08, p = 0.072). Of patients in the permanent PNS cohort, there was no difference in postimplant percentage pain relief between patients who received a diagnostic block (n = 41) and control patients (n = 20) at three months (42.4 ± 34.3 vs 43.2 ± 42.4, respectively; ß -0.79, 95% CI -23.56 to 21.99, p = 0.945) or at six months (44.3 ± 35.0 vs 38.8 ± 40.9, respectively; ß 5.42, 95% CI -20.04 to 30.88, p = 0.669). Pain relief from preimplant diagnostic blocks was associated with postimplant pain relief from temporary PNS at three months (ß 0.33, 95% CI 0.04-0.61, p = 0.025). However, pain relief from preimplant diagnostic blocks did not predict postimplant pain relief from temporary PNS at six months, or permanent PNS at three months and six months. CONCLUSIONS: Administration of a diagnostic block is not associated with superior pain relief at three or six months after PNS implantation to that of an approach without diagnostic block. Pain relief from a diagnostic block may potentially predict short-term pain relief after temporary PNS therapy, although future prospective studies are warranted to evaluate the prognostic utility of diagnostic blocks.

Peripheral Nerve Stimulation for the Treatment of Phantom Limb Pain: A Case Series.

This case series aims to highlight the efficacy of peripheral nerve stimulation (PNS) in the treatment of phantom limb pain, as well as provide an alternative method for the treatment of this pain syndrome. In this report, we describe three amputee patients with severe phantom limb pain who obtained substantial analgesia and improvement in physical functionality after implantation of a temporary PNS device. Future studies should assess predictors of successful response or poor response to PNS therapy, such as mental health, environmental stressors, coping skills, and procedural factors. These factors may facilitate an individualized approach for each patient to ensure appropriate candidacy for PNS and better prognosis. Considering that patients in our cohort did not achieve long-lasting benefit after removal of temporary PNS, future research should assess if patients with phantom limb pain would benefit from permanent PNS, rather than temporary PNS.

Ultrasound-guided percutaneous peripheral nerve stimulation for chronic refractory neuropathic pain: a unique series.

During the last two decades, with the advent of recent technology, peripheral nerve stimulation has become an appealing modality at the forefront of pain management. In this case series, we document the clinical rationale and technical considerations on three of the most challenging cases, refractory to previous interventions, that were treated by our team with an ultrasound-guided percutaneous peripheral nerve stimulator targeting the musculocutaneous, bilateral greater occipital and subcostal nerves. At the 6-month follow-up, all patients experienced greater than 50% relief of baseline pain, with a near-complete resolution of pain exacerbations. Furthermore, to our knowledge, this is the first report of an ultrasound-guided percutaneous technique of a peripheral nerve stimulator targeting the musculocutaneous and subcostal nerves.

Peripheral nerve stimulation is a new tool used in the treatment of peripheral nerve pain. In this study, we share our experience using this technology in three unusual, difficult-to-treat chronic nerve pain presentations, targeting the musculocutaneous, bilateral greater occipital and subcostal nerves. All patients were asked about how pain levels had changed since the peripheral nerve stimulation device had been implanted. In every case, patients reported a decline in their pain level from day one. After 6 months of peripheral nerve stimulator use, all patients reported a greater than 50% pain relief.

Incidence of Temporary Peripheral Nerve Stimulator Lead Tip Retention: A Retrospective Review of 80 Lead Placements.

OBJECTIVES: The primary objective of this study was to determine the current rate of lead fracture during temporary percutaneous peripheral nerve stimulator (PNS) lead removal at the Mayo Clinic Rochester Division of Pain Medicine. MATERIALS AND METHODS: A retrospective review of electronic medical records was performed for patients implanted with a temporary percutaneous PNS device between January 1, 2018, and December 31, 2020. Patients were included if they underwent temporary percutaneous PNS system implant, with planned lead removal at 60 days. Data collection included date of implant, diagnosis, peripheral nerve target(s), number of leads, and lead tip status at the time of removal (intact vs fractured). RESULTS: Fifty patients underwent a total of 80 temporary percutaneous PNS leads placed during the time frame analyzed. Of the 80 temporary percutaneous PNS leads implanted, there were five lead fractures at the time of intentional lead removal. CONCLUSIONS: This retrospective review of 50 patients with 80 temporary percutaneous PNS leads implanted for chronic peripheral neuropathic pain resulted in a 6.25% rate of retained lead fragment at the time of lead removal by the provider at the end of 60-day treatment. This fracture and retention rate is consistent with previous published retrospective data on PNS, which has shown a 3% to 21% lead fracture rate during intentional lead removal.

A case for ultrasound-guided peripheral nerve stimulation in intractable anterior cutaneous nerve entrapment syndrome.

Aim: This case report documents the use of peripheral nerve stimulation in the setting of entrapment of the anterior cutaneous branches of the intercostal nerves, with pain rated by the patient as severe during exacerbation episodes. Materials & methods: Under ultrasound guidance, two permanent leads were implanted caudad to cephalad, along and superficial to the lateral aspect of the rectus abdominis, distal to the umbilicus (1 lead per side). Results: At the 6 month follow-up, the patient reported near complete resolution of baseline pain, as well as fewer, sporadic pain exacerbation episodes, rated as mild-to-moderate. Conclusion: This case report suggests that peripheral nerve stimulation might be a valuable treatment option for previously intractable abdominal pain due to entrapment of the anterior cutaneous branches.

Anterior cutaneous nerve entrapment syndrome is a peculiar, a largely disregarded pain condition. Current management algorithms rely mostly on local injections followed by surgical anterior neurectomy. This case report presents a case of longstanding, anterior cutaneous nerve entrapment syndrome, unresponsive to first-line treatment, that was successfully treated with peripheral nerve stimulation technology targeting the anterior cutaneous branches.

Real-world evidence of sustained improvement following 60-day peripheral nerve stimulation treatment for pain: a cross-sectional follow-up survey.

Objective: This study presents real-world data from a cross-sectional follow-up survey of patients who previously received 60-day peripheral nerve stimulation (PNS) treatment for pain. Materials & methods: A survey including validated pain and other related outcome measures was distributed to patients who previously underwent implantation of temporary PNS leads for 60-day PNS treatment. Results: Among survey respondents who were at least 3 months from the start of treatment, most reported sustained clinically significant improvements in pain and/or quality of life, with the length of follow-up at the time of survey completion ranging from 3 to 30 months. Conclusion: These real-world data support recent prospective studies indicating that 60-day percutaneous PNS provides significant and sustained relief across a wide range of pain conditions.

This study presents the findings from a survey that was sent to patients who previously received a 60-day peripheral nerve stimulation (PNS) treatment for their chronic pain. Patients were asked about their current pain levels, how their quality of life and physical function have changed since their PNS treatment, and whether they had changed their usage of pain medications. The survey showed that most patients who were at least 3 months from the start of the PNS treatment continued to have meaningful pain relief and/or improvement in their quality of life. This information is consistent with clinical studies that were previously published and supports that the 60-day PNS treatment can provide patients with long-term relief of chronic pain.

Comparative Effectiveness of Landmark-guided Greater Occipital Nerve (GON) Block at the Superior Nuchal Line Versus Ultrasound-guided GON Block at the Level of C2: A Randomized Clinical Trial (RCT).

OBJECTIVES: The purpose of this single center, prospective randomized controlled trial was to compare clinical outcomes between an ultrasound-guided greater occipital nerve block (GONB) at the C2 vertebral level versus landmark-based GONB at the superior nuchal line. METHODS: Patients with occipital neuralgia or cervicogenic headache were randomized to receive either a landmark-based GONB with sham ultrasound at the superior nuchal line or ultrasound-guided GONB at the C2 vertebral level with blinding of patients and data analysis investigators. Clinical outcomes were assessed at 30 minutes, 2 weeks, and 4 weeks postinjection. RESULTS: Thirty-two patients were recruited with 16 participants in each group. Despite randomization, the ultrasound-guided GONB group reported higher numeric rating scale (NRS) scores at baseline. Those in the ultrasound-guided GONB group had a significant decrease in NRS from baseline compared with the landmark-based GONB group at 30 minutes (change of NRS of 4.0 vs. 2.0) and 4-week time points (change of NRS of 2.5 vs. -0.5). Both groups were found to have significant decreases in Headache Impact Test-6. The ultrasound-guided GONB had significant improvements in NRS, severe headache days, and analgesic use at 4 weeks when compared with baseline. No serious adverse events occurred in either group. CONCLUSIONS: Ultrasound-guided GONBs may provide superior pain reduction at 4 weeks when compared with landmark-based GONBs for patients with occipital neuralgia or cervicogenic headache.

Ultrasound-guided transversus abdominis plane block vs trigger point injections for chronic abdominal wall pain: a randomized clinical trial.

ABSTRACT: The primary aim of this randomized clinical trial is to investigate the effects of ultrasound-guided transversus abdominis plane (TAP) vs ultrasound-guided trigger point injections (TPIs) on numerical rating scale pain scores at month 3 follow-up in patients with a chronic abdominal wall pain. The primary outcome measure was the difference in mean numeric rating scale pain scores between the TAP and TPI groups at month 3 in an intent-to-treat (ITT) analysis. A total of 60 patients were randomized 1:1 to receive an ultrasound-guided TAP block (n = 30) or an ultrasound-guided TPI (n = 30). No significant group differences in baseline demographic or clinical characteristics were observed. The mean baseline pain score for the TAP and TPI groups was 5.5 and 4.7, respectively. In the ITT analysis at month 3, the between-group difference in pain scores was 1.7 (95% confidence interval, 0.3-3.0) favoring the TPI group. In a secondary per-protocol analysis, the between-group difference in pain scores was 1.8 (95% confidence interval, 0.4-3.2) favoring the TPI group. For the ITT and per-protocol analyses, the group differences in pain scores were consistent with a medium effect size. The main finding of this randomized clinical trial is that adults with chronic abdominal wall pain who received a TPI reported significantly lower pain scores at month 3 follow-up compared with patients who received a TAP block.

Peripheral Nerve Stimulation and Clinical Outcomes: A Retrospective Case Series.

PURPOSE: Peripheral nerve stimulation (PNS) is a rapidly expanding field within neuromodulation; however, there is limited data on therapeutic efficacy. This study describes the indications and clinical outcomes for patients undergoing PNS for chronic pain states. PATIENTS AND METHODS: This is a retrospective case series of adults undergoing PNS implantation from 2004 to 2017 at an academic medical center. The primary outcomes were changes in numeric rating scale (NRS) pain scores, opioid utilization in oral morphine milligram equivalent (MME), and self-reported patient functioning at 6 months postoperatively. Infectious and device-related complications were also assessed. RESULTS: A total of 72 patients underwent PNS implantation, including 59 patients that received a preceding PNS trial (59/78; 76% progression rate) and 13 that did not receive a PNS trial. The most common indication for stimulation was occipital neuralgia (47%) followed by lower-extremity neuropathies (17%). PNS implantation was associated with 6-month reductions in pain scores (7 [6, 8] baseline vs. 4 [2, 5] 6 months; P < 0.001) and opioid utilization (eg, median 60 [31, 104] vs. 18 [0, 52] MME among those with baseline opioid use; P < 0.001). Median functional improvement was 73% (50%, 88%). Seven patients (10%) suffered a postoperative surgical site infection at a median of 50 (30, 124) days, of which five devices were removed. CONCLUSION: Peripheral nerve stimulation was associated with reduced pain scores, lower opioid utilization, and improved patient function at 6 months. These data support PNS as a potentially effective nonopioid analgesic modality in chronic pain, though prospective multicenter evaluation is warranted to evaluate longer-term outcomes.

Cooled radiofrequency ablation provides extended clinical utility in the management of knee osteoarthritis: 12-month results from a prospective, multi-center, randomized, cross-over trial comparing cooled radiofrequency ablation to a single hyaluronic acid injection.

BACKGROUND: Safe and effective non-surgical treatments are an important part of the knee osteoarthritis (OA) treatment algorithm. Cooled radiofrequency ablation (CRFA) and hyaluronic acid (HA) injections are two commonly used modalities to manage symptoms associated with knee OA. METHODS: A prospective 1:1 randomized study was conducted in 177 patients comparing CRFA to HA injection with follow-ups at 1, 3, 6 and 12 months. HA subjects with unsatisfactory outcomes at 6-months were allowed to crossover and receive CRFA. Knee pain (numeric rating scale = NRS), WOMAC Index (pain, stiffness and physical function), overall quality of life (global perceived effect = GPE, EQ-5D-5 L), and adverse events were measured. RESULTS: At 12-months, 65.2% of subjects in the CRFA cohort reported ≥50% pain relief from baseline. Mean NRS pain score was 2.8 ± 2.4 at 12 months (baseline 6.9 ± 0.8). Subjects in the CRFA cohort saw a 46.2% improvement in total WOMAC score at the 12-month timepoint. 64.5% of subjects in the crossover cohort reported ≥50% pain relief from baseline, with a mean NRS pain score of 3.0 ± 2.4 at 12 months (baseline 7.0 ± 1.0). After receiving CRFA, subjects in the crossover cohort had a 27.5% improvement in total WOMAC score. All subjects receiving CRFA reported significant improvement in quality of life. There were no serious adverse events related to either procedure and overall adverse event profiles were similar. CONCLUSION: A majority of subjects treated with CRFA demonstrated sustained knee pain relief for at least 12-months. Additionally, CRFA provided significant pain relief for HA subjects who crossed over 6 months after treatment. TRIAL REGISTRATION: This trial was registered on ClinicalTrials.gov, NCT03381248. Registered 27 December 2017.

Spinal Cord Stimulation for Neuropathic Pain Treatment in Brachial Plexus Avulsions: A Literature Review and Report of Two Cases.

BACKGROUND: At least 50% of individuals who suffer a brachial plexus avulsion (BPA) will develop chronic pain, frequently more debilitating than their functional limitations. Similar to other neuropathic pain states, BPA pain is often refractory to pharmacological agents. Despite spinal cord stimulation (SCS) first being used for BPA in 1974, there have been no published literature reviews examining the current evidence of SCS for the treatment of neuropathic pain following BPA. In addition to a clinical review of the literature for this indication, we also share our experience with high-frequency SCS (HF-SCS) for BPA-related pain. METHODS: MEDLINE and EMBASE databases were searched. All published articles including at least one BPA individual treated with SCS for pain treatment were included. RESULTS: The initial search identified 288 articles, of which 13 met inclusion criteria for a total of 41 patients. These patients were primarily male and underwent SCS with reported improved pain scores. CASE REPORTS: HF-SCS leads were percutaneously placed in two male patients who suffered BPA from traumatic injuries. At follow-ups of 13 and eight months, respectively, both patients continued to report an improvement in their pain. CONCLUSIONS: Despite published reports showing benefit for pain control in patients with BPA, the overall low quality, retrospective evidence included in this review highlights the need for a rigorous prospective study to further address this indication.

Peri-procedural Management of Implanted Spinal Cord Stimulators in Patients Undergoing Radiofrequency Ablation: A Case Report and Manufacturer-Specific Recommendations.

INTRODUCTION: Radiofrequency ablation (RFA) and spinal cord stimulation (SCS) are 2 common modalities used in management of various pain conditions. RFA generates heat and an electromagnetic field that can theoretically interfere with the functionality and longevity of the SCS device. This is problematic for patients who need RFA and have previously undergone implantation of an SCS device. CASE DESCRIPTION: We present a case highlighting the safe use of bipolar RFA in a patient who had previously undergone implantation of a cervical SCS device. DISCUSSION: We also present device-specific industry guidelines and recommendations for performing RFA in patients following SCS device implantation.

Pain medicine fellow neuromodulation surgical skill assessment tool: a pilot.

OBJECTIVE: Spinal cord stimulation (SCS) is an evidence-based therapy for the treatment of refractory pain. Current American College of Graduate Medical Education requirements for pain medicine fellowship graduation include observation of five neuromodulation cases. Matriculating fellows have varying degrees of technical skill, training, and experience. The purpose of this study was to use an innovative skill-based assessment tool using the validated Zwisch scale to evaluate fellow surgical performance during SCS cases. DESIGN: Cross-sectional survey SETTING: Mayo Clinic, Rochester, Minnesota. SUBJECTS: Consultant faculty (10) in the Division of Pain Medicine and pain medicine fellows (5). METHODS: A list of faculty-driven neuromodulation surgical objectives was formed and a rubric was created that focused on technical approach, imaging, epidural access and wound closure. Consultants who perform surgical cases were instructed to use the Zwisch scale as a guide to evaluate fellow surgical performance during a neuromodulation case. Faculty and fellows were surveyed before and after implementation of the tool to determine their satisfaction with use of the skill assessment tool. RESULTS: The consultants reported they were more satisfied, consistent, and efficient with feedback provided to the trainee on every aspect of neuromodulation surgical cases. The improvement was most significant during the fellows' intraoperative skill assessment. The fellows indicated increased satisfaction, improved communication, and increased efficiency of feedback when the tool was utilized. The fellows reported greater intraoperative skill assessment and consistency of feedback provided. CONCLUSIONS: The diverse nature of primary specialty backgrounds observed in pain medicine fellowship training offers a unique opportunity to assess and improve fellow skill and surgical competence in the field of neuromodulation. Both faculty and fellows reported improved satisfaction, consistency and efficiency with feedback provided. Importantly, this pilot project observed that implementation of a skill assessment tool was beneficial for both the faculty and fellow as the feedback received was viewed as strongly beneficial to the educational experience.

Title: Quantifying the change of spasticity after intrathecal baclofen administration: A descriptive retrospective analysis.

OBJECTIVES: Exploratory research quantifying the change of spasticity among patients who underwent baclofen intrathecal drug delivery system (IDDS) implantation. PATIENTS AND METHODS: 88 patients with a baclofen IDDS were identified. Patient characteristics, spasticity scores pre/post intrathecal baclofen test dose, and IDDS perioperative implantation records were collected. The primary outcome was to quantify the extent to which there was a change in Modified Ashworth Scores (MAS) pre/post-intrathecal baclofen test dose administration. Secondary outcomes included the prevalence of perioperative IDDS implantation complications. RESULTS: The mean age at IDDS implant was 44.2 years (range, 19-71), and 62.5% were male. 45.5% had spasticity of spinal cord origin, 9% of cerebral origin, and 45.5% of other upper motor neuron dysfunction. Reduction of MAS in the spinal cord origin group was 2.6 (mean, 3.5 to 0.9), cerebral origin group was 2.9 (mean, 3.3 to 0.4), and other origin group was 2.5 (mean, 3.6 to 1.1). In all patients, post dural puncture headache was the most commonly reported complication at 22.7%. CONCLUSION: This report offers novel findings documenting a quantifiable change of at least two points on the MAS before and after intrathecal baclofen test dose as statistically significant and could prove to be useful information to enhance the decision making process to proceed with intrathecal baclofen beyond assessment of functional abilities.

Utilization of Leads After Permanent Implant in Spinal Cord Stimulator Systems.

OBJECTIVE: The goal of this study was to determine the frequency and clinical indications associated with implantation of single vs. dual percutaneous lead spinal cord stimulator (SCS) systems and to look further into how these leads are utilized for treatment. MATERIALS AND METHODS: A retrospective cohort analysis of all patients undergoing SCS implantation between January 2001 and December 2013 with a minimum of 2 years of clinical follow-up was performed. Number of trial leads and implanted leads was recorded. For patients with dual-lead systems, it was noted if and when the second lead was used, along with the clinical indication for lead activation. RESULTS: In the 259-patient cohort, 15.8% (n = 41) patients underwent placement of a single-lead system, 83.0% (n = 215) underwent placement of a dual-lead system, and 1.2% (n = 3) underwent placement of 3-lead systems. Placement of dual-lead systems was similar among all indication groups. Of those patients with a dual-lead system in place, 88.1% utilized both leads and average time to programming of the second lead was 2.3 months. The most common reason to activate the second lead was inadequate stimulation coverage. Five of the 41 patients with single-lead systems underwent an additional surgery to implant a second lead due to inadequate stimulation with 1 lead. CONCLUSIONS: To our knowledge this is the first descriptive analysis of the frequency of single- and dual-lead SCS systems. This report indicates that dual-lead systems are most often placed and both leads are required for optimal patient therapy.

Descriptive Cadaveric Study Comparing the Accuracy of Ultrasound Versus Fluoroscopic Guidance for First Sacral Transforaminal Injections: A Comparison Study.

BACKGROUND: Ultrasound is rarely used for guiding lumbosacral epidural steroid injections due to its technical limitations. For example, sonographic imaging lacks the ability to confirm epidural spread and identify vascular uptake. The perceived risk that these limitations pose to human subjects has precluded any large scale clinical trials to date. OBJECTIVE: To compare the accuracy of ultrasound versus fluoroscopic guidance for first sacral transforaminal epidural injections. DESIGN: Cadaveric comparative study using dichotomous outcomes. SETTING: A fluoroscopy suite and anatomic laboratory at an academic medical center. SUBJECTS: Four unembalmed adult human cadavers with no history of spinal surgery. METHODS: Eight sites were injected twice by one interventionalist, using fluoroscopic and ultrasound guidance. In the fluoroscopy arm, contrast spread was assessed using computed tomography. In the ultrasound arm, latex spread was assessed using gross anatomic dissection. Any visible evidence of epidural spread constituted a positive result. MAIN OUTCOME MEASUREMENTS: Comparison of the success of obtaining epidural contrast flow was the primary outcome measure. Secondary outcome measures included average duration, rate of intravascular uptake, and quantity of intravascular uptake. RESULTS: All injections performed in both the ultrasound arm and the fluoroscopy arm had positive epidural spread. The average duration was 3.03 minutes with fluoroscopy and 4.76 minutes with ultrasound. The rate of intravascular uptake was 37.5% with fluoroscopy and 50% with ultrasound. Within the ultrasound arm, greater intravascular spread and duration variability were recorded. CONCLUSION: Although ultrasonography can provide reliable image guidance for cannulating the first sacral foramen in cadavers, it would have limited clinical utility due to its inability to visualize relevant neurovascular structures deep to the osseus roof and exclude intravascular uptake. LEVEL OF EVIDENCE: IV.

Dexmedetomidine as Part of a Multimodal Analgesic Treatment Regimen for Opioid Induced Hyperalgesia in a Patient with Significant Opioid Tolerance.

Acute postoperative pain in patients with opioid tolerance creates a significant management challenge for anesthesiologists and pain medicine physicians. A multimodal approach is key; however other factors can complicate management such as opioid induced hyperalgesia. We present the case of a patient on large amounts of intrathecal opioids for chronic pain syndrome with opioid induced hyperalgesia after an exploratory laparotomy. Dexmedetomidine was utilized successfully as part of a controlled multimodal analgesic plan and should be a consideration for opioid tolerant patients experiencing opioid induced hyperalgesia.

Melorheostosis: A Retrospective Clinical Analysis of 24 Patients at the Mayo Clinic.

BACKGROUND: Current understanding of the clinical features of persons with melorheostosis is restricted primarily to individual case reports and small case series. OBJECTIVE: To assess the clinical features of patients with melorheostosis treated at our institution from 1972 through 2010. DESIGN: Chart review. SETTING: Tertiary academic medical center. PARTICIPANTS: Twenty-three patients with "definite" and one patient with "probable" melorheostosis based on radiographic criteria. METHODS: The eligible study cohort was identified through the Rochester Medical Index database. Further diagnostic confirmation of patients with melorheostosis was performed by radiographic review. MAIN OUTCOME MEASUREMENTS: We evaluated age at first visit to our institution, gender, affected body area, number of bones affected, presenting symptoms, surgical evaluation, and therapies provided. RESULTS: The average age at first evaluation at our clinic was 36.5 years (median 41.5 years, range 3-68 years). The female to male ratio was 4:1. The lower extremity was most commonly affected (66.6%), followed by upper extremity (33.3%), spine (16.6%), and head (8.3%). One-third of patients had involvement of a single bone; two-thirds had multiple bone involvement. Pain was the most common presenting concern (83.3%), followed by deformity (54.1%), limitation of movement (45.8%), numbness (37.5%), and weakness (25.0%). Most patients had a physician evaluation (87.5%); patients also underwent orthopedic surgery (45.8%), physical therapy (33.3%), and occupational therapy (12.5%). CONCLUSIONS: Melorheostosis is a rare sclerotic bone disease resulting in pain, deformity, and dysfunction. An interdisciplinary approach to care should include nonoperative and operative evaluation, as well as appropriate therapies. A prospective approach to evaluation, including imaging and physical examinations, would provide valuable longitudinal data. LEVEL OF EVIDENCE: IV.