Precision oncology in neurofibromatosis type 1: quantification of differential sensitivity to selumetinib in plexiform neurofibromas using single-cell RNA sequencing.

PURPOSE: Selumetinib is an FDA-approved targeted therapy for plexiform neurofibromas in neurofibromatosis type 1(NF1) with durable response rates seen in most, but not all patients. In this proof-of-concept study, we demonstrate single-cell RNA sequencing(scRNAseq) as a technique for quantifying drug response to selumetinib at the single cell level. METHODS: scRNAseq data from neurofibroma biopsies was obtained from a public genomics repository. Schwann cell populations were identified through standard clustering techniques and single-cell selumetinib sensitivity was quantified on a scale of 0(resistant) to 1(sensitive) based on the expression pattern of a 500 gene selumetinib sensitivity signature from the BeyondCell sensitivity library. RESULTS: A total of seven plexiform neurofibromas were included in our final analysis. The median absolute number of Schwann cells across samples was 658 cells (IQR: 1,029 cells, Q1-Q3: 135 cells to 1,163 cells). There was a statistically significant difference in selumetinib sensitivity profiles across samples (p < 0.001). The tumor with the highest median selumetinib sensitivity score had a median selumetinib sensitivity score of 0.64(IQR: 0.14, Q1-Q3: 0.59-0.70, n = 112 cells) and the tumor with the lowest median selumetinib sensitivity score had a median score of 0.37 (IQR: 0.21, Q1-Q3: 0.27-0.48, n = 1,034 cells). CONCLUSIONS: scRNAseq of plexiform neurofibroma biopsies reveals differential susceptibilities to selumetinib on a single cell level. These findings may explain the partial responses seen in clinical trials of selumetinib for NF1 and demonstrate the value of collecting scRNAseq data for future NF1 trials.

Hirayama Disease: Surgical Restoration of Hand Function.

PURPOSE: Hirayama disease (HD) is a rare, nonfamilial, self-limiting, progressive lower cervical myelopathy, resulting in debilitating distal upper-extremity motor deficits, mimicking high ulnar neuropathy, lower trunk brachial plexopathy, or C8-T1 radiculopathy. Although most literature focuses on pathophysiology and prevention of disease progression, there remains limited discussion regarding treatment to improve upper-extremity function in patients with stable disease. The upper-extremity manifestations of HD are reviewed along with surgical options for restoring hand function. METHODS: A retrospective review of patients with HD who underwent reconstruction to improve hand function was undertaken. Demographic data, preoperative electrodiagnostic and electromyographic, and physical examination findings were collected. Outcome data involved postoperative grip, pinch, and functional assessment documented on clinical visits. Qualitative descriptions of the surgical techniques are described. RESULTS: Among six patients identified, four met the inclusion criteria and underwent tendon transfers and selected joint arthrodeses. All patients were diagnosed as teenagers, were right hand-dominant, and three were male. Unilateral symptoms were present in one patient and were bilateral in the rest. All patients were treated with tendon transfers for thumb opposition, grasp, anticlaw, and thumb interphalangeal joint arthrodesis. All patients had postoperative grip strength improvement. The average follow-up was 3.2 years. CONCLUSIONS: Hirayama disease is a rare disease often managed by spine surgeons and neurologists who may be unaware of options for restoring hand function deficits. Technical strategies and outcomes of improving hand function in HD have not been adequately described. Surgical options to improve hand function are tailored to the deficits and include tendon transfers, select joint arthrodeses, and/or tenodeses. Risk of disease progression and expectations following hand reconstruction must be managed carefully. TYPE OF STUDY LEVEL OF EVIDENCE: Therapeutic V.

Validation of the Root Analysis Score for C5 Viability in Patients With Pan-Brachial Plexus Injury.

PURPOSE: Pan-brachial plexus injury patients present a reconstructive challenge. The root analysis score, developed from parsimonious multivariable modeling of 311 pan-brachial plexus injury patients, determines the probability of having a viable C5 nerve based on four categories: positive C5 Tinel test, intact C5 nerve on computed tomography myelogram, lack of hemidiaphragmatic elevation, and absence of midcervical paraspinal fibrillations. METHODS: Root analysis scores were calculated for a separate cohort of patients with pan-brachial plexus injuries. Scores were validated by the presence or absence of a graftable C5 root, based on supraclavicular exploration and intraoperative electrophysiologic testing. Receiver operating characteristic curve, accuracy, and concordance statistic of the scores were calculated. Patients were divided into three root analysis score cohorts: less than 50 (low), 50-75 (average), and 75-100 points (high) based on dividing the score into quartiles and combining the lowest two. The probability, sensitivity, and specificity of each cohort having an available C5 nerve were based on the intraoperative assessment. RESULTS: Eighty patients (mean age, 33.1 years; 15 women and 65 men) were included. Thirty-one patients (39%) had a viable C5 nerve. The root analysis calculator had an overall accuracy of 82.5%, a receiver operating characteristic of 0.87, and a concordance statistic of 0.87, demonstrating high overall predictive value; 6.5% of patients with a score of less than 50 (94% sensitivity and 43% specificity), 16.1% of patients with a score of 50-75 (94% sensitivity and 67% specificity), and 77.4% of patients with a score of 75-100 (77% sensitivity and 90% specificity) had a graftable C5 nerve. CONCLUSIONS: The root analysis score demonstrated high accuracy and predictive power for a viable C5 nerve. In patients with a score of less than 50, the necessity of supraclavicular root exploration should balance patient factors, presentation timing, and concomitant injuries. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnosis II.

High Resolution MRI Confirms Torsional Injury as Mechanism for Combined Ankle and Common Fibular (Peroneal) Nerve Injuries.

Common fibular nerve (CFN) injury due to ankle fracture is an underreported complication. The authors have proposed that torsional injury to the ankle can be translated along the interosseous membrane (IOM), producing tension on the CFN at the fibular neck. A 23-year-old woman presented to our clinic for left foot drop. Three months prior, the patient sustained a fall with left ankle inversion injury while running. She was diagnosed with a minor ankle fracture and placed in an orthopaedic boot. Unfortunately, her swelling worsened and one week later the patient was diagnosed with foot drop, which was further corroborated with EMG studies showing severe CFN injury localizing to the fibular neck. Because of the lack of recovery, she underwent decompression of the CFN. She experienced immediate symptomatic relief. High resolution imaging in this case supports our previous mechanism for indirect trauma to the ankle resulting in CFN injury. (Journal of Surgical Orthopaedic Advances 33(1):053-055, 2024).

Superficial Branch of the Radial Nerve Versus Sural Nerve Grafting After Traumatic Adult Brachial Plexus Injury.

The purpose of this study was to compare two sources of nerve graft for brachial plexus reconstruction: the denervated superficial branch of the radial nerve (SBRN) and the sural nerve. Ninety-seven patients who underwent brachial plexus reconstruction with denervated SBRN nerve (24 patients with 24 grafts) or with sural nerve grafting (73 patients with 83 nerve grafts) were included. The two groups were compared with respect to postoperative muscle reinnervation, disabilities of the arm, shoulder, and hand (DASH) scores. In the SBRN group, only four (17%) of the nerve grafts provided grade III or higher muscle function. In the sural nerve group, 31 (37%) of the nerve grafts provided grade III or higher muscle function. Smoking had a negative impact on muscle recovery. Denervated SBRN grafts are associated with inferior outcomes when compared with sural nerve grafts in the treatment of traumatic adult brachial plexus injuries. (Journal of Surgical Orthopaedic Advances 33(2):080-083, 2024).

Characterizing T-cell dysfunction and exclusion signatures in malignant peripheral nerve sheath tumors reveals susceptibilities to immunotherapy.

PURPOSE: Malignant peripheral nerve sheath tumors (MPNSTs) are malignant tumors that arise from peripheral nerves and are the leading cause of mortality in Neurofibromatosis Type 1 (NF1). In this study, we characterized whether transcriptomic signatures of T-cell dysfunction (TCD) and exclusion (TCE) that inversely correlate with response to immune checkpoint blockade (ICB) immunotherapy exist in MPNSTs. METHODS: MPNST transcriptomes were pooled from Gene Expression Omnibus (GEO). For each sample, a tumor immune dysfunction and exclusion (TIDE) score, TCD and TCE subscores, and cytotoxic T-cell(CTL) level were calculated. In the TIDE predictive algorithm, tumors are predicted to have an ICB response if they are either immunologically hot (CTL-high) without TCD or immunologically cold (CTL-low) without TCE. TIDE scores greater than zero correspond with ICB nonresponse. RESULTS: 73 MPNST samples met inclusion criteria, including 50 NF1-associated MPNSTs (68.5%). The average TIDE score was + 0.41 (SD = 1.16) with 22 (30.1%) predicted ICB responders. 11 samples were CTL-high (15.1%) with an average TCD score of + 0.99 (SD = 0.63). Among 62 CTL-low tumors, 21 were predicted to have ICB response with an average TCE score of + 0.31(SD = 1.20). Age(p = 0.18), sex(p = 0.41), NF1 diagnosis (p = 0.17), and PRC2 loss(p = 0.29) were not associated with ICB responder status. CONCLUSIONS: Transcriptomic analysis of TCD and TCE signatures in MPNST samples reveals that a select subset of patients with MPNSTs may benefit from ICB immunotherapy.

Sural nerve biopsy utility by masked assessment of individual histologic preparations.

BACKGROUND AND AIMS: Comprehensive study of sural nerve biopsy utility based on individual histopathologic preparations is lacking. We aimed to quantify the value of different histologic preparations in diagnosis. METHODS: One hundred consecutive sural nerves were studied by standard histological preparations plus graded teased nerve fibers (GTNF), immunohistochemistry, and epoxy-semithin morphometry. Three examiners scored the individual preparations separately by a questionnaire of neuropathic and interstitial abnormalities, masked to the biopsy number, versus a gold-standard of all preparations. Multivariate modeling was utilized to determine best approach versus the gold-standard. RESULTS: Highest confidence (range 8-9 of 10) and inter-rater reliability (99%) for fiber abnormalities came from GTNF, and interstitial abnormalities from paraffin stains (range 7-8, 99%). Vasculitic neuropathy associated with GTNF axonal degeneration (moderate to severe 79%) with OR 3.8, 95% CI (1.001-14.7), p = .04, but not significantly with the other preparations. Clinicopathologic diagnoses associated with teased fiber abnormalities in chronic inflammatory demyelinating polyradiculoneuropathy, 80% (8/10); amyloidosis, 50% (1/2); adult-onset polyglucosan disease 100% (1/1). GTNF and paraffin stains significantly correlated with fiber density determined by morphometric analysis (GTNF: OR 9.9, p < .0001, paraffin: OR 3.8, p = .03). GTNF combined with paraffin sections had highest accuracy for clinicopathologic diagnoses and fiber density with 0.86 C-stat prediction versus morphometric analysis. Pathological results lead to initiation or changes of immunotherapy in 70% (35/50; initiation n = 22, reduction n = 9, escalation n = 4) with the remaining having alternative intervention or no change. INTERPRETATION: Nerve biopsy paraffin stains combined with GTNF have highest diagnostic utility, confidence, inter-rater reliability, improving accuracy for a pathologic diagnosis aiding treatment recommendations. Immunostains and epoxy preparations are also demonstrated useful supporting consensus guidelines. This study provides class II evidence for individual nerve preparation utility.

Plexal intraneural perineurioma: an analysis of the clinicoradiologic presentation of this rare variant.

PURPOSE: Intraneural (IN) perineurioma is a rare benign peripheral nerve sheath tumor, typically presenting as a painless, progressive mononeuropathy in adolescents. A rare plexal variant has been described, although there are little data describing its clinicoradiologic features. Herein, we present the largest case series of plexal IN perineuriomas reported in the literature. METHODS: Electronic medical records (EMR) from 1990 to 2022 from a single academic institution were reviewed for a diagnosis of IN perineurioma involving the brachial or lumbosacral plexus. This identified 18 patients, of which 17 had available MR imaging. We reviewed the EMR for demographics, clinical presentation, imaging characteristics, and surgical outcomes. RESULTS: Eighteen patients were identified. Most patients were male (11/18, 61%) and first developed symptoms at the age of 9.6 years (range 7 months to 55 years). Diagnosis occurred on average at the age of 22 years (4-57 years), which is significantly earlier than distal IN perineurioma (p = 0.0096). All patients (100%, 17/17) presented with motor polyneuropathy and muscular atrophy in multiple nerve distributions, with associated sensory loss (12/17, 71%). Most plexal lesions occurred in the brachial plexus (66%, 12/18). Five (29%, 5/17) patients presented with a hand/foot discrepancy, and 5 patients (29%) had a limb length discrepancy. Five patients underwent tendon transfer, of which two had failure of tendon transfer at most recent follow-up (50%, 2/4) due to progression of neurologic loss affecting the donors. Of patients managed nonoperatively, 87% of patients (6/7) with follow-up information demonstrated disease progression with worsening motor function or sensory loss, and 2 patients demonstrated progression on imaging at most recent follow-up. CONCLUSIONS: Plexal perineurioma represents an uncommon variant of IN perineurioma that presents as a progressive motor and sensory polyneuropathy in childhood or early adolescence. Surgical management remains controversial, and tendon transfer tends to result in poor long-term surgical outcomes.

Diagnostic value of proximal cutaneous nerve biopsy in brachial and lumbosacral plexus pathologies.

BACKGROUND: Brachial and lumbosacral plexopathies can result from numerous non-traumatic etiologies, including those of inflammatory, autoimmune, or neoplastic origin, that often require nerve biopsy for diagnosis. The purpose of this study was to evaluate the diagnostic efficacy of medial antebrachial cutaneous nerve (MABC) and posterior femoral cutaneous nerve (PFCN) nerve biopsies in proximal brachial and lumbosacral plexus pathology. METHOD: Patients undergoing MABC or PFCN nerve biopsies at a single institution were reviewed. Patient demographics, clinical diagnosis, symptom duration, intraoperative findings, post-operative complications, and pathology results were recorded. Biopsy results were classified as diagnostic, inconclusive, or negative based on the final pathology. RESULTS: Thirty patients undergoing MABC biopsies in the proximal arm or axilla and five patients with PFCN biopsies in the thigh or buttock were included. MABC biopsies were diagnostic in 70% of cases overall and 85% diagnostic in cases where pre-operative MRI also demonstrated abnormalities in the MABC. PFCN biopsies were diagnostic in 60% of cases overall and in 100% of patients with abnormal pre-operative MRIs. There were no biopsy-related post-operative complications in either group. CONCLUSIONS: In diagnosing non-traumatic etiologies of brachial and lumbosacral plexopathies, proximal biopsies of the MABC and PFCN provide high diagnostic value with low donor morbidity.

PET imaging characteristics of neuromuscular choristoma and associated desmoid-type fibromatosis.

BACKGROUND: Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion characterized by abnormal presence of muscle within nerve. Associated desmoid-type fibromatosis (NMC-DTF) often develops. We report 18F-fluorodeoxyglucose positron emission tomography (FDG PET) characteristics of NMC and NMC-DTF and propose that increased FDG activity within NMCs may be associated with subclinical NMC-DTF or NMC-DTF "precursor" tissue. METHODS: Our institutional database was searched for all NMC cases. Inclusion criteria were 1) confirmed diagnosis of NMC with or without biopsy, and 2) available PET and MRI studies. PET data included SUVmax and SUVmean of NMCs, contralateral limb normal skeletal muscle and unaffected nerves, and SUVmax of NMC-DTF if present. SUV values were compared using paired t-test. A p value of < 0.05 was considered statistically significant. RESULTS: Our cohort consisted of 9 patients with NMC, 8 cases involving sciatic nerve and 1 of brachial plexus. On PET imaging, all NMC-affected nerve segments showed significantly higher FDG uptake (SUVmax/mean) compared to both contralateral normal nerve and normal skeletal muscle (all P < 0.05). Similar to sporadic DTF, NMC-DTF was highly FDG-avid (average SUVmax of 4.2). SUVmax in NMC with or without concurrent NMC-DTF did not differ (p = 0.76). Within NMC-affected nerve segment, FDG activity was relatively higher in areas with low T1/T2 MR signal. CONCLUSION: All NMCs were more FDG avid compared to both normal skeletal muscle and contralateral unaffected nerve, arguing against the presence of heterotopic muscle in NMC as the source of FDG avidity. FDG avidity within NMC may reflect subclinical NMC-DTF or a precursor lesion, as NMC-DTF are highly FDG-avid, and the highest regions of FDG avidity in NMC occurred in regions with MR characteristics associated with NMC-DTF (i.e., lower T1/T2 signal). We believe that the integration of FDG PET with serial MR imaging in patient follow up will clarify its utility in both detection and surveillance of NMC-DTF.

Surgical management of peroneal nerve injuries.

BACKGROUND: Traumatic peroneal nerve injuries are typically associated with high-energy injuries. The aim of this study was to evaluate the demographics and outcomes following surgical management of peroneal nerve injuries. METHODS: Patients evaluated at a single institution with peroneal nerve injuries between 2001 and 2022 were retrospectively reviewed. Mechanism of injury, time to surgery, pre- and postoperative examinations, and operative reports were recorded. Satisfactory outcome, defined as the ability to achieve anti-gravity dorsiflexion strength or stronger following surgery, was compared between nerve grafting and nerve transfers in patients with at least 9 months of postoperative follow-up. RESULTS: Thirty-seven patients had follow-up greater than 9 months after surgery, with an average follow-up of 3.8 years. Surgeries included neurolysis (n=5), direct repair (n=2), tibial motor nerve fascicle transfer to the anterior tibialis motor branch (n=18), or interposition nerve grafting using sural nerve autograft (n=12). At last follow-up, 59.5% (n=22) of patients had anti-gravity strength or stronger dorsiflexion. Nineteen (51.4%) patients used an ankle-foot orthosis during all or some activities. In patients that underwent nerve grafting only across the peroneal nerve defect, 44.4% (n=4) were able to achieve anti-gravity strength or stronger dorsiflexion. In patients that had a tibial nerve fascicle transfer to the tibialis anterior motor branch of the peroneal nerve, 42.9% (n=6) were able to achieve anti-gravity strength or stronger dorsiflexion at last follow-up. There was no statistical difference between nerve transfers and nerve grafting in postoperative dorsiflexion strength (p = 0.51). CONCLUSION: Peroneal nerve injuries frequently occur in the setting of knee dislocations and similar high-energy injuries. Nerve surgery is not universally successful in restoration of ankle dorsiflexion, with one-third of patients requiring an ankle-foot orthosis at mid-term follow-up. Patients should be properly counseled on the treatment challenges and variable outcomes following peroneal nerve injuries.

Tibial intraneural ganglion cysts at the superior tibiofibular joint treated with joint resection alone: a proof of concept.

BACKGROUND: Intraneural ganglion cysts involving the tibial nerve are rare. Recent evidence has supported an articular (synovial) theory to explain the joint-related origin of these cysts; however, optimal operative treatment for cysts originating from the STFJ remains poorly understood. Therefore, we present a novel strategy: addressing the joint itself without addressing the articular branch and/or the cyst. METHODS: Records of patients with tibial intraneural ganglion cysts with a connection to the STFJ who were treated with a joint resection alone at a single academic institution were reviewed. The clinicoradiographic features, operative intervention, and postoperative course were recorded. RESULTS: We identified a consecutive series of 7 patients. These patients (4/7 male, 57%) were 43 (range 34-61) years of age and all presented with symptoms of neuropathy. The patients underwent resection of the synovial surfaces of the STFJ without disconnection of the articular branch or decompression of the cyst. Postoperatively, three patients regained partial motor function (43%, n=7), although four patients noted continued sensory abnormality (57%, 4/7). All six patients with postoperative MRIs had some evidence of regression of the cyst. CONCLUSIONS: This novel surgical technique serves as a proof of concept-highlighting the fact that treating the primary source (the joint origin) can be effective in eliminating the secondary problem (the cyst itself). While this study shows that this simplified approach can be employed in select cases, we believe that superior results (faster, fuller recovery) can be achieved with combinations of disconnecting the articular branch, decompressing the cyst, and/or resecting the joint.

The diagnostic utility of inspiratory-expiratory radiography for the assessment of phrenic nerve palsy associated with brachial plexus injury.

BACKGROUND: The phrenic nerve is commonly injured with trauma to the brachial plexus. Hemi-diaphragmatic paralysis may be well-compensated in healthy individuals at rest but can be associated with persistent exercise intolerance in some patients. This study aims to determine the diagnostic value of inspiratory-expiratory chest radiography compared to intraoperative stimulation of the phrenic nerve for assessing phrenic nerve injury associated with brachial plexus injury. METHODS: Over a 21-year period, the diagnostic utility of three-view inspiratory-expiratory chest radiography for identification of phrenic nerve injury was determined by comparison to intraoperative phrenic nerve stimulation. Multivariate regression analysis was used to identify independent predictors of phrenic nerve injury and having an incorrect radiographic diagnosis. RESULTS: A total of 237 patients with inspiratory-expiratory chest radiography underwent intraoperative testing of phrenic nerve function. Phrenic nerve injury was present in approximately one-fourth of cases. Preoperative chest radiography had a sensitivity of 56%, specificity of 93%, positive predictive negative of 75%, and negative predictive value of 86% for identification of a phrenic nerve palsy. Only C5 avulsion was found to be a predictor of having an incorrect diagnosis of phrenic nerve injury on radiography. CONCLUSION: While inspiratory-expiratory chest radiography has good specificity for detecting phrenic nerve injuries, a high number of false negatives suggest that it should not be relied upon for routine screening of dysfunction after traumatic brachial plexus injury. This is likely multifactorial and relates to variation in diaphragm shape and position, as well as limitations regarding static image interpretation of a dynamic process.

Hybrid tumors with perineurioma components: a systematic review of the literature and illustrative case.

PURPOSE: Hybrid peripheral nerve sheath tumors (HPNST) are a newly recognized class of peripheral nerve sheath tumor, composed of at least two areas characteristic of perineurioma, schwannoma, or neurofibroma. The literature consists only of case reports and small series; therefore, we present an illustrative case and an analysis of all reported cases of HPNST with a perineurioma component in the literature. METHODS: A systematic search of the literature was performed to identify all reported cases of hybrid perineurioma-schwannoma or perineurioma-neurofibroma in the world's literature. Individual cases were analyzed for demographics, clinical features, imaging, and outcomes. RESULTS: A total of 159 cases were identified across 41 studies. Hybrid tumors tended to present in mid-adulthood (median 38.5 years), predominantly affected females (57%, 89/156), as a painless (63%, 63/100) mass, or swelling. Ten patients (10/74, 14%) had a history of neurofibromatosis 1, and 2 patients a history of neurofibromatosis 2 (2/74, 3%). The majority (78%, 122/157) of cases occurred superficially, most commonly in the lower extremity (25%, 39/157). Perineurioma-schwannoma was the most reported (86%, 137/159) pathologic diagnosis, with 3 cases presenting with malignant features. Two cases reocurred after resection. CONCLUSION: HPNST tend to occur in mid-adulthood and present as slowly progressive, painless, superficial masses, with a heterogeneous appearance on imaging. These entities pose a unique diagnostic challenge and likely remain under-recognized in the literature and current clinical practice. They pose low risk of recurrence or malignant transformation, and future work regarding the association with neurofibromatosis and genetic profiles is needed.

Stem cell therapy for traumatic brachial plexus injury.

Stem cell therapy is rapidly evolving, with preclinical studies showing various stem cell types successfully promoting peripheral nerve regeneration. Despite the lack of clinical studies demonstrating efficacy and safety, the number of commercial entities marketing and promoting this treatment direct to patients is also increasing. We describe three adult traumatic brachial plexus injury (BPI) patients who had stem cell therapies prior to consultation in a multidisciplinary brachial plexus clinic. No functional improvement was noted at long-term follow-up despite claims reported by the commercial entities. Considerations and implications of stem cell application in BPI patients are reviewed.

What is new in intraneural perineurioma?

Since the initial description of intraneural (IN) perineurioma in 1964, advances in the understanding of the clinical presentation, diagnostic imaging, pathologic features, and genetic underpinnings have changed how this pathology is managed. IN perineuriomas are rare, benign peripheral nerve sheath tumors, most frequently coming to clinical attention when patients present with painless, progressive weakness or sensory loss in adolescence or young adulthood. The gold standard of diagnosis has traditionally been with targeted tissue biopsy demonstrating "pseudo-onion bulb" formation with positive epithelial membrane antigen (EMA) staining. However, modern magnetic resonance imaging is allowing some patients to forgo biopsy. Recent genetic studies of IN perineuriomas have demonstrated common TRAF7 point mutations and rare NF2 mutations, which may present targets for diagnosis or therapy in the future. Current advances have allowed for us to provide improved patient counseling with informed understanding for various clinical scenarios. With the workup and diagnosis now clearly defined, the next frontier is for improving the lives of patients with IN perineuriomas through the interaction between restoration of functional deficits and advances in our understanding of the genetics of this entity.

7T for clinical imaging of benign peripheral nerve tumors: preliminary results.

PURPOSE: MRI has become an essential diagnostic imaging modality for peripheral nerve pathology. Early MR imaging for peripheral nerve depended on inferred nerve involvement by visualizing downstream effects such as denervation muscular atrophy; improvements in MRI technology have made possible direct visualization of the nerves. In this paper, we share our early clinical experience with 7T for benign neurogenic tumors. MATERIALS: Patients with benign neurogenic tumors and 7T MRI examinations available were reviewed. Cases of individual benign peripheral nerve tumors were included to demonstrate 7T MRI imaging characteristics. All exams were performed on a 7T MRI MAGNETOM Terra using a 28-channel receive, single-channel transmit knee coil. RESULTS: Five cases of four pathologies were selected from 38 patients to depict characteristic imaging features in different benign nerve tumors and lesions using 7T MRI. CONCLUSION: The primary advantage of 7T over 3T is an increase in signal-to-noise ratio which allows higher in plane resolution so that the smallest neural structures can be seen and characterized. This improvement in MR imaging provides the opportunity for more accurate diagnosis and surgical planning in selected cases. As this technology continues to evolve for clinical purposes, we anticipate increasing applications and improved patient care using 7T MRI for the diagnosis of peripheral nerve masses.

Triceps-to-Biceps Tendon Transfer for Restoration of Elbow Flexion in Brachial Plexus Injury.

PURPOSE: Restoring elbow flexion is a reconstructive priority in patients with brachial plexus injuries. This study aimed to evaluate the results and assess factors contributing to outcomes of triceps-to-biceps tendon transfer in patients presenting with delayed or chronic upper brachial plexus injury. METHODS: Patients with traumatic brachial plexus injuries undergoing triceps-to-biceps tendon transfer at a single institution's multidisciplinary brachial plexus center between 2001 and 2021 were retrospectively reviewed. The entire triceps tendon was transferred around the lateral aspect of the arm, secured to the radius with a tenodesis button, and reinforced with a side-to-side tendon transfer to the biceps tendon. Primary outcomes include the modified British Medical Research Council (mBMRC) elbow flexion strength and active elbow range of motion. RESULTS: Twelve patients (eight men and four women; mean age, 45.2 years) were included. The mean follow-up was 10.4 (range, 5-34) months. Nine patients achieved mBMRC ≥3. Five patients achieved mBMRC 4. Average active elbow flexion was 119°, with average extension deficit of 11°. There were three patients with unsatisfactory results, achieving mBMRC 2 elbow flexion. CONCLUSIONS: Triceps-to-biceps tendon transfer is an excellent tendon transfer option for restoring elbow flexion in certain patients with an adequately functioning triceps muscle, who present with a delayed or chronic brachial plexus injury. Although most patients achieved mBMRC ≥3 elbow flexion, there was an expected permanent loss of elbow active extension with a residual elbow flexion contracture. LEVEL OF EVIDENCE: Therapeutic IV.

Femoral intraneural ganglion cyst: the first confirmed case report.

BACKGROUND: Ganglion cysts affecting nerve are rare causes of neuropathy. The formation of intraneural ganglion cysts, once controversial, has recently been clarified. We describe the first modern description of a femoral intraneural ganglion cyst at the hip region. METHODS: A patient presented with a 1 year history of radiating pain, quadriceps weakness and anteromedial leg numbness was found to have a femoral intraneural cyst with a hip joint connection on MRI. RESULTS: Surgical disconnection of the articular branch led to improvement of the neuropathy and resolution of the cyst on postoperative MRI. CONCLUSIONS: The unifying articular (synovial) theory describes the joint origin of intraneural cysts, even when they occur in unusual locations, and their propagation into the parent nerve. Knowledge of this theory can improve outcomes; surgery needs to address the joint origin or capsulolabral defect lest recurrence ensue.

Nerve relationships to the sacrospinous ligament: application to suspension procedures and transgluteal approaches for nerve repair and tumor removal with three case illustrations.

BACKGROUND: Transvaginal suspension procedures often use the sacrospinous ligament (SSL), which attaches onto the ischial spine (IS). However, nerve-related sequelae (e.g., sciatic nerve injury) following such procedures have been reported. Therefore, the current anatomical study was performed to better understand these relationships. Additionally, three case illustrations of patients with injury to the sciatic nerve following sacrospinous ligament suspension procedures are included to exemplify the significance of a thorough knowledge of this anatomy. METHODS: In 20 human adult cadavers (40 sides), a gluteal dissection was performed to expose the IS and SSL and regional nerves near the greater sciatic foramen. Measurements between the IS and SSL were made between these structures and surrounding nerves. RESULTS: The average distance between the IS and sciatic nerve was 1.4 cm. From this bony part, the average distance to the S1 and S2 ventral rami was 3.1 cm and 1.9 cm, respectively. From the IS to the lumbosacral trunk, pudendal nerve, nerve to obturator internus, and superior gluteal nerve, the mean distance was 4 cm, 0.5 cm, 0.7 cm, and 4.5 cm, respectively. From the SSL to the lumbosacral trunk, S1 ventral ramus, and S2 ventral ramus, there was an average distance of 4.2 cm, 1.6 cm, and 0.8 cm, respectively. Statistically, in females, the distances from the IS and SSL to the sciatic nerve, lumbosacral trunk, superior gluteal nerve, and S1 and S2 ventral rami were shorter when compared to males. CONCLUSION: An improved understanding of the relationship between the SSL and IS and nerves near the greater sciatic foramen can lead to fewer intraoperative complications during approaches to various peripheral nerves in this region. Lastly, these relationships might help better understand the nerve injuries following pelvic suspension procedures that use the SSL.