Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population.

PURPOSE: Peripheral nerve sheath tumors (PNSTs) are rare in pediatric patients, especially in the brachial plexus. Research on PNSTs is lacking. This article presents a retrospective cohort study of pediatric patients diagnosed and treated with PNSTs, specifically brachial plexus tumors. METHODS: All pediatric patients intervened in a single center between 2007 and 2023 with brachial plexus tumors were systemically analyzed. RESULTS: Eleven pediatric patients with 14 brachial plexus PNSTs were studied. The gender distribution was 64% female and 36% male, with an average age of 10.7 years. Ninety-one percent had a previous NF-1 diagnosis. Right brachial plexus presented a higher prevalence (64%). Pain, Tinel's sign, and stiffness masses were common during diagnosis. Motor deficits were noted in 43% of the patients. Surgery was indicated for symptoms, particularly pain and rapid growth, increasing malignancy risk. Due to suspected malignancy, an en bloc resection with safety margins was performed. Among the patients, 57% received a histopathological diagnosis of MPNST (malignant peripheral nerve sheath tumor). Treatment included radiotherapy and chemotherapy. Clinical follow-up was conducted for all cases, involving clinical and oncological evaluations for all MPNSTs. CONCLUSIONS: This article present a series of pediatric brachial plexus tumors, especially in NF-1, and emphasizes the importance of thorough evaluation for this group. Swift diagnosis is crucial in pediatrics, enabling successful surgery for small lesions with limited neurological symptoms, improving long-term outcomes. Prompt referral to specialized services is urged for suspected masses, irrespective of neurological symptoms. Benign tumor postsurgical progression shows better outcomes than MPNSTs, with complete resection as the primary goal. Needle-guided biopsy is not recommended.

Core outcomes in nerve surgery: development of a core outcome set for brachial plexus and upper extremity nerve injuries.

OBJECTIVE: When considering traumatic brachial plexus and upper extremity nerve injuries, iatrogenic nerve injuries, and nontraumatic nerve injuries, brachial plexus and upper extremity nerve injuries are commonly encountered in clinical practice. Despite this, data synthesis and comparison of available studies are difficult. This is at least in part due to the lack of standardization in reporting and a lack of a core outcome set (COS). Thus, there is a need for a COS for adult brachial plexus and upper extremity nerve injuries (COS-BPUE). The objective of this study was to develop a COS-BPUE using a modified Delphi approach. METHODS: A 5-stage approach was used to develop the COS-BPUE: 1) consortium development, 2) literature review to identify potential outcome measures, 3) Delphi survey to develop consensus on outcomes for inclusion, 4) Delphi survey to develop definitions, and 5) consensus meeting to finalize the COS and definitions. The study followed the Core Outcome Set-STAndards for Development (COS-STAD) recommendations. RESULTS: The Core Outcomes in Nerve Surgery (COINS) Consortium comprised 23 participants, all neurological surgeons, representing 13 countries. The final COS-BPUE consisted of 36 data points/outcomes covering demographic, diagnostic, patient-reported outcome, motor/sensory outcome, and complication domains. Appropriate instruments, methods of testing, and definitions were set. The consensus minimum duration of follow-up was 24 months, with the consensus optimal time points for assessment being preoperatively and 3, 6, 12, and 24 months postoperatively. CONCLUSIONS: The COINS Consortium developed a consensus COS and provided definitions, methods of implementation, and time points for assessment. The COS-BPUE should serve as a minimum set of data that should be collected in all future neurosurgical studies on adult brachial plexus and upper extremity nerve injuries. Incorporation of this COS should help improve consistency in reporting, data synthesis, and comparability, and should minimize outcome reporting bias.

When biopsy goes wrong: a case series of misdiagnoses and complications from biopsies of masses of unknown origin potentially originating from a peripheral nerve.

OBJECTIVE: Biopsies of peripheral nerve tumors (PNTs) are often used to plan an efficient treatment strategy. However, performing a biopsy is controversial when the mass is likely to be a benign PNT (BPNT). The aim of this study was to evaluate the side effects of biopsies in patients with potential PNTs. METHODS: A retrospective and cross-sectional study was conducted on 24 patients who underwent biopsy of a mass of unknown origin potentially originating from a peripheral nerve (MUOPON), performed in nonspecialty services, and who were later referred to the authors' service for resection of their lesion between January 2005 and December 2022. The patients were evaluated for pain score, presence of a motor or sensory deficit, biopsy diagnosis, and definitive histopathological postsurgical diagnosis. RESULTS: The location of the tumor was supraclavicular in 7 (29.2%) patients, in the axillary region in 3 (12.5%), in the upper limb in 7 (29.2%), and in the lower limb in 7 (29.2%). Twenty-one (87.5%) patients were evaluated by MRI before biopsy, and 3 (12.5%) underwent ultrasound. One patient did not have an examination before the procedure. Based on the biopsy findings, 12 (50%) analyses had an inconclusive histopathological result. The preexisting pain worsened, as measured 1 week after biopsy, in all patients and had remained unchanged at the first evaluation by the authors (median 3 months, range 2-4 months). In 1 case, the open biopsy had to be interrupted because the patient experienced excruciating pain. Four (16.7%) patients developed motor deficits. Subsequent surgery was hampered by scar formation and intratumoral hemorrhage in 5 (20.8%) patients. The initial diagnosis obtained by biopsy differed from the final histopathological diagnosis in all patients, of whom 21 (87.5%) had BPNTs, 2 (8.3%) malignant peripheral nerve sheath tumors, and 1 (4.2%) an ancient schwannoma. CONCLUSIONS: Biopsies of PNTs are controversial and may result in misdiagnosis, neuropathic pain, or neurological deficit due to axonal damage, and they may also hinder microsurgical resection when if performed when not indicated. Indications for biopsy of an MUOPON must be carefully considered, especially if BPNT is a possible diagnosis.

Core outcomes in nerve surgery: development of a core outcome set for ulnar neuropathy at the elbow.

OBJECTIVE: Ulnar neuropathy at the elbow (UNE) is common, affecting 1%-6% of the population. Despite this, there remains a lack of consensus regarding optimal treatment. This is primarily due to the difficulty one encounters when trying to assess the literature. Outcomes are inconsistently reported, which makes comparing studies or developing meta-analyses difficult or even impossible. Thus, there is a need for a core outcome set (COS) for UNE (COS-UNE) to help address this problem. The objective of this study was to utilize a modified Delphi method to develop COS-UNE. METHODS: A 5-stage approach was utilized to develop COS-UNE: stage 1, consortium development; 2, literature review to identify potential outcome measures; 3, Delphi survey to develop consensus on outcomes for inclusion; 4, Delphi survey to develop definitions; and 5, consensus meeting to finalize the COS and definitions. The study followed the Core Outcome Set-STAndards for Development (COS-STAD) recommendations. RESULTS: The Core Outcomes in Nerve Surgery (COINS) Consortium comprised 21 participants, all neurological surgeons representing 11 countries. The final COS-UNE consisted of 22 data points/outcomes covering the domains of demographic characteristics, diagnostics, patient-reported outcomes, motor/sensory outcomes, and complications. Appropriate instruments, methods of testing, and definitions were set. The consensus minimum duration of follow-up was 6 months, with the consensus optimal timepoints for assessment identified as preoperatively and 3, 6, and 12 months postoperatively. CONCLUSIONS: The authors identified consensus data points/outcomes and also provided definitions and specific scales to be utilized to help ensure that clinicians are consistent in their reporting across studies on UNE. This COS should serve as a minimum set of data to be collected in all future neurosurgical studies on UNE. The authors hope that clinicians evaluating ulnar neuropathy will incorporate this COS into routine practice and that future studies will consider this COS in the design phase.

Surgery as an Effective Therapy for Ulnar Nerve Neuropathic Pain Caused by Gunshot Wounds: A Retrospective Case Series.

BACKGROUND: Peripheral nerve injuries remain a major medical problem worldwide and are associated with multiple causes, including gunshot wounds (GSWs), which are the second most common cause of brachial plexus injuries in peacetime and the main, or only, cause reported in wartime studies. The ulnar nerve (UN) is one of the most affected nerves. Peripheral nerve trauma may cause intense neuropathic pain, which is very difficult to control. Particularly UN gunshot injuries may impact individual daily life, as injuries to this nerve result in both sensory and motor deficits within the hand. We evaluated the improvement of neuropathic pain after surgical treatment in a consecutive series of 20 patients with UN injury due to GSWs. METHODS: This single-center, retrospective, consecutive case series included 20 patients with UN injuries due to GSWs, who presented with excruciating neuropathic pain and underwent surgical treatment between 2005 and 2017. RESULTS: Of injuries, 13 occurred in the right upper limb (65%); 12 patients had a high UN injury (60%). Regarding associated injuries, 8 patients had bone injuries (40%), and 4 patients had arterial injuries (20%). A neuroma in continuity was detected in 8 cases (40%), and 4 patients (20%) had shrapnel lodged within the UN. All patients had severe neuropathic pain and functional deficit, with a mean visual analog scale score of 8.45 ± 1.4 and a mean reduction of 6.95 points 12 months after surgery; 10 patients (50%) had a British Medical Research Council score ≥M3. CONCLUSIONS: Surgery is an effective treatment for neuropathic pain from GSWs. Early isolated external neurolysis is associated with better pain management and functional outcomes postoperatively.

Gamma Irradiation Processing on 3D PCL Devices-A Preliminary Biocompatibility Assessment.

Additive manufacturing or 3D printing applying polycaprolactone (PCL)-based medical devices represents an important branch of tissue engineering, where the sterilization method is a key process for further safe application in vitro and in vivo. In this study, the authors intend to access the most suitable gamma radiation conditions to sterilize PCL-based scaffolds in a preliminary biocompatibility assessment, envisioning future studies for airway obstruction conditions. Three radiation levels were considered, 25 kGy, 35 kGy and 45 kGy, and evaluated as regards their cyto- and biocompatibility. All three groups presented biocompatible properties, indicating an adequate sterility condition. As for the cytocompatibility analysis, devices sterilized with 35 kGy and 45 kGy showed better results, with the 45 kGy showing overall improved outcomes. This study allowed the selection of the most suitable sterilization condition for PCL-based scaffolds, aiming at immediate future assays, by applying 3D-customized printing techniques to specific airway obstruction lesions of the trachea.

Giant anterior sacral meningocele associated with hydroureteronephrosis and renal injury: illustrative case.

BACKGROUND: Anterior sacral meningocele (ASM) is a defect in the closure of the neural tube. Patients can be asymptomatic or present with genitourinary, neurological, reproductive, or colorectal dysfunction. Magnetic resonance imaging (MRI) is the gold standard test because it can assess communication between the spinal subarachnoid space and the lesion and identify other abnormalities. Surgical correction is the definitive treatment because untreated cases have a mortality rate of more than 30%. OBSERVATIONS: A 24-year-old woman with Marfan syndrome presented with polyuria, recurrent urinary tract infections, and renal injury for 3 months along with a globose abdomen, with a palpable mass in the middle and lower third of the abdomen that was massive on percussion. MRI showed an ASM consisting of two cystic lesions measuring 15.4 × 14.3 × 15.8 and 6.7 × 6.1 × 5.9 cm, respectively, compressing the distal third of the right ureter and causing a hydroureteronephrosis. Drainage and ligature of the cystic lesion were performed. The urinary outcome was excellent, with full recovery after surgery. LESSONS: ASM should be suspected in all abdominal masses with progressive symptoms in the setting of Marfan syndrome. Computed tomography and MRI are important to investigate genitourinary anomalies or other types of dysraphism to guide the best surgical approach.

LINEAR ENDOSONOGRAPHY IN LUNG CANCER: A COMPREHENSIVE REVIEW.

The role of endobronchial ultrasound (EBUS) and trans-esophageal endobronchial ultrasound (EUS-B) in lung cancer is well established and scientifically validated. There is increasing data that endosonography is a crucial tool for the diagnosis of central lung lesions, and mediastinal staging and restaging of non-small cell lung cancer patients. The present article reviews the technical aspects of EBUS and EUS-B and focus on the last published research regarding its value in lung cancer.

Central airway obstruction: is it time to move forward?

INTRODUCTION: Central airway obstruction (CAO) represents a pathological condition that can lead to airflow limitation of the trachea, main stem bronchi, bronchus intermedius or lobar bronchus. MAIN BODY: It is a common clinical situation consensually considered under-diagnosed. Management of patients with CAO can be difficult and deciding on the best treatment approach represents a medical challenge. This work intends to review CAO classifications, causes, treatments and its therapeutic limitations, approaching benign and malign presentations. Three illustrative cases are further presented, supporting the clinical problem under review. CONCLUSION: Management of CAO still remains a challenge. The available options are not always effective nor free from complications. A new generation of costume-tailored airway stents, associated with stem cell-based therapy, could be an option in specific clinical situations.

3D Printed Poly(𝜀-caprolactone)/Hydroxyapatite Scaffolds for Bone Tissue Engineering: A Comparative Study on a Composite Preparation by Melt Blending or Solvent Casting Techniques and the Influence of Bioceramic Content on Scaffold Properties.

Bone tissue engineering has been developed in the past decades, with the engineering of bone substitutes on the vanguard of this regenerative approach. Polycaprolactone-based scaffolds are fairly applied for bone regeneration, and several composites have been incorporated so as to improve the scaffolds' mechanical properties and tissue in-growth. In this study, hydroxyapatite is incorporated on polycaprolactone-based scaffolds at two different proportions, 80:20 and 60:40. Scaffolds are produced with two different blending methods, solvent casting and melt blending. The prepared composites are 3D printed through an extrusion-based technique and further investigated with regard to their chemical, thermal, morphological, and mechanical characteristics. In vitro cytocompatibility and osteogenic differentiation was also assessed with human dental pulp stem/stromal cells. The results show the melt-blending-derived scaffolds to present more promising mechanical properties, along with the incorporation of hydroxyapatite. The latter is also related to an increase in osteogenic activity and promotion. Overall, this study suggests polycaprolactone/hydroxyapatite scaffolds to be promising candidates for bone tissue engineering, particularly when produced by the MB method.

Terminal Anatomy of Phrenic Nerve: A Deeper Look at Diaphragm Innervation Patterns.

BACKGROUND: Traumatic brachial plexus injuries are devastating lesions, and neurotization is an usually elected surgical therapy. The phrenic nerve has been harvested as a motor fibers donor in brachial plexus neurotization, showing great results in terms of motor reinnervation. Unfortunately, these interventions lack solid evidence regarding long-term safety and possible late respiratory function sequelae, raising crescent concerns after the COVID-19 pandemic onset and possibly resulting in reduced propensity to use this technique. The study of the distal anatomy of the phrenic nerves may lead to a better understanding of their branching patterns, and thus the proposition of surgical approaches that better preserve patient respiratory function. METHODS: Twenty-one phrenic nerves in 10 formalized cadavers were scrutinized. Prediaphragmatic branching patterns were inspected through analysis of the distance between the piercing site of the nerve at the diaphragm and the cardiac structures, number of divisions, and length from the point where the main trunk emits its branches to the diaphragm. RESULTS: The main trunk of the right phrenic nerve reaches the diaphragm near the inferior vena cava and branches into 3 major divisions. The left phrenic nerve reaches the diaphragm in variable locations near the heart, branching into 2-5 main trunks. Moreover, we noticed a specimen presenting 2 ipsilateral parallel phrenic nerves. CONCLUSIONS: The right phrenic nerve presented greater consistency concerning insertion site, terminal branching point distance to this muscle, and number of rami than the left phrenic nerve.

Deep Vein Thrombosis in the Setting of Neurofibromatosis Type 1: Case Report

Neurofibromatosis type 1 (NF1) is a genetic syndrome which typically presents with neurological manifestations. Some of the patients may also present with vasculopathies, among which arterial aneurysms and stenosis are the most common. Deep vein thrombosis (DVT) has rarely been described, and, to the best of our knowledge, the present is the first report of DVT due to venous compression by a neurofibroma in the setting of NF1. This is the case of a 23-year-old male with NF1 who experienced DVT due to compression of the left posterior tibial veins by a large tumor arising from the tibial nerve. The DVT was acutely treated with enoxaparin and then with rivaroxaban. Two months after the diagnosis, Doppler ultrasonography showed partial recanalization and persistence of the DVT. The patient was then referred to neurosurgery for surgical resection of the tumor. There were no complications during the procedure, and the patient did not present postoperative neurological deficits. The final histopathological diagnosis was of a benign neurofibroma. After one year of follow-up with vascular surgery, the patient presented no more episodes of DVT. In case there is a tumor compressing the deep vessels of the leg and promoting DVT, surgical resection with microsurgical techniques may be curative.

Surgical Management of Symptomatic Lumbar, Sacral, and Lumbosacral Plexus Tumors: a Peripheral Nerve Unit Experience.

OBJECTIVE: Data concerning the surgical treatment of lumbosacral plexus tumors (LSPTs) is scarce. This study aims to present our experience with a series of 19 patients surgically treated for symptomatic LSPTs at our institution. METHODS: This is a retrospective study of 19 patients surgically treated for symptomatic LSPTs from 2011 to 2019. Clinical data were retrieved from medical records and consisted of age, gender, clinical presentation, location of the lesion, surgical approach, final histopathologic diagnosis, follow-up time, outcomes, and complications. RESULTS: Nineteen surgical procedures were conducted. Thirteen patients were female and six, male. The median age of patients was 45 years (range 20 to 63 years). No patients harbored genetic syndromes. Surgical treatment appears to be correlated to the reduction of pain in patients with peripheral nerve sheath tumors (PNSTs), as assessed by visual analog scale (VAS). Sixteen patients did not present with new-onset deficits during follow-up (84.2%), two of whom recovered from their preoperative deficit. Four patients presented with postoperative weakness. The histopathological diagnoses were 11 schwannomas, four neurofibromas, three metastases, and one lymphoma. CONCLUSIONS: LSPTs are rare. When surgical treatment is indicated, it usually requires multidisciplinary management. Surgery appears to be effective concerning the reduction of pain in PNSTs and may also recover neurological deficits. Iatrogenic neurological deficits are an evident risk, such that intraoperative multimodal monitoring should always be performed if available. In lesions involving the sacral plexus, we found it to be indispensable.

Anatomical feasibility of peripheral nerve transfer to reestablish external anal sphincter control - cadaveric study.

PURPOSE: Motor deficits affecting anal sphincter control can severely impair quality of life. Peripheral nerve transfer has been proposed as an option to reestablish anal sphincter motor function. We assessed, in human cadavers, the anatomical feasibility of nerve transfer from a motor branch of the tibialis portion of the sciatic nerve to two distinct points on pudendal nerve (PN), through transgluteal access, as a potential approach to reestablish anal sphincter function. METHODS: We dissected 24 formalinized specimens of the gluteal region and posterior proximal third of the thigh. We characterized the motor fascicle (donor nerve) from the sciatic nerve to the long head of the biceps femoris muscle and the PN (recipient nerve), and measured nerve lengths required for direct coaptation from the donor nerve to the recipient in both the gluteal region (proximal) and perineal cavity (distal). RESULTS: We identified three anatomical variations of the donor nerve as well as three distinct branching patterns of the recipient nerve from the piriformis muscle to the pudendal canal region. Donor nerve lengths (proximal and distal) were satisfactory for direct coaptation in all cases. CONCLUSIONS: Transfer of a motor fascicle of the sciatic nerve to the PN is anatomically feasible without nerve grafts. Donor nerve length was sufficient and donor nerve functionally compatible (motor). Anatomical variations in the PN could also be accommodated.

Surgical management of pediatric patients with peripheral nerve and plexus lesions caused by stray bullets.

PURPOSE: Children and adolescents are frequent victims of gunshot wounds (GSW), either by direct intent or accidentaly. Lesions caused by stray bullets represent a specific type of accidental GSW and are usually associated with urban violence or aerial firing. We thereby present a series of surgically treated pediatric patients with peripheral nerve and brachial plexus lesions caused by stray bullets, referring to their clinical presentation, surgical procedures, and outcomes. METHODS: Retrospective study of a series of seven pediatric patients treated from 2012 to 2019 for nerve and/or plexus lesions caused by stray bullets at the Peripheral Nerve Unit of the Division of Neurosurgery of Gaffrée e Guinle University Hospital (HUGG). We used the Visual Analog Scale (VAS) to evaluate pain distress and the British Medical Research Council grading system (BMRC) to assess muscle strength. RESULTS: Patients' ages ranged from 6 to 17 years old (median of 16), and two were female. All presented preoperatively with intense pain, with a median VAS of 9 (range 7 to 10), and six also had neurological deficits. External neurolysis was conducted in all cases, whereas reconstruction with grafts was needed in four patients. All experienced improvement of pain, and those with motor deficits also experienced some level of recovery. CONCLUSION: Pediatric patients who endure lesions by stray bullets appear to present with debilitating pain, and often with motor deficits. Multidisciplinary management comprising of surgical treatment and physical and occupational therapy may ameliorate symptoms and improve quality of life, as young patients usually fare better after surgery.

Central Airway Stenosis: Opening the Path / Estenosis de la vía aérea central: abriendo el camino

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Nondiscogenic Sciatica: What Clinical Examination and Imaging Can Tell Us?

OBJECTIVE: Lesions affecting the sciatic nerve (SN) can mimic lumbar radiculopathy. In patients presenting with sciatica, approximately 10% have a nondiscogenic etiology. Through neurological examination and imaging, it may be possible to confirm nondiscogenic sciatica (NDS). This study aims to present a series of 6 patients with infragluteal NDS, highlighting clinical and imaging aspects that may suggest this diagnosis. METHODS: This is a retrospective study of 6 patients treated for NDS from 2010 to 2018. The mean and median ages were 41.2 and 38.5 years, respectively, with all patients female. RESULTS: All patients presented with sciatic pain, tenderness to deep infragluteal palpation, and a positive Tinel's sign related to the SN. Four patients were referred for surgical treatment, whereas 1 underwent pharmacological therapy and 1 underwent incisional biopsy and radiotherapy. In our series, 6 different causes for NDS were diagnosed: 1 nontumorous cause: extrauterine endometriosis and 5 tumors: metastasis from rectal adenocarcinoma, low-grade sarcoma, schwannoma, high-grade sarcoma, and myxoma. CONCLUSIONS: Differentiating between discogenic and NDS can be challenging for clinicians. When patients present with sciatic pain, a Tinel's sign related to the SN elicited at the deep infragluteal region, tenderness to deep infragluteal palpation, occasionally with an SN motor deficit and imaging findings of the lumbar spine that do not justify a discogenic source, the cause should be considered nondiscogenic and they should be scheduled for magnetic resonance imaging of the gluteal and pelvic region.