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Background: Upper limb lymphedema (ULL) is a common and deliberating complication for breast cancer survivors (BCSs). Breast cancer survivors with ULL reported a wide range of symptoms. However, little is known about symptom patterns and interrelationships among them. This study was designed to explore symptom clusters and construct symptom networks of ULL-related symptoms among BCSs and to identify the core symptoms., Methods: This study is a secondary data analysis using datasets from three cross-sectional studies of BCSs in China. A total of 341 participants with maximum interlimb circumference ≥2 cm and complete responses in Part I of the Breast Cancer and Lymphedema Symptom Experience Index were included. Symptom clusters were identified through principal component analysis, and multiple linear regression analysis was employed to explore factors associated with severity of overall ULL-related symptoms. A contemporaneous network with 20 frequently reported symptoms were constructed after controlling for covariates., Results: Three symptom clusters, including lymph stasis symptom cluster, nerve symptom cluster, and movement limitation symptom cluster, were identified. Postsurgery time, axillary lymph node dissection, and radiotherapy were associated with the severity of ULL-related symptoms. Tightness (r s = 1.379; r scov = 1.097), tingling (r s = 1.264; r scov = 0.925), and firmness (r s = 1.170; r scov = 0.923) were the most central symptoms in both networks with and without covariates., Conclusions: Breast cancer survivors with ULL experienced severe symptom burden. Tightness, tingling, and firmness were core symptoms of ULL among BCSs. Our findings demonstrated that the assessment and targeted intervention of specific core symptoms might help to relive effectively the burden of ULL-related symptom among BCSs., (© 2024. Society of Surgical Oncology.)

Background/aim: Osteoarthritis (OA) is a prevalent degenerative joint disease that significantly impacts quality of life, particularly when affecting the hands. However, whether patients with OA are associated with higher risk of developing upper limb disorders, specifically trigger finger (TF) and carpal tunnel syndrome (CTS), remains unclear. This study aimed to evaluate the risk of upper limb disease in OA patients., Patients and Methods: Using the US Collaborative Network, a subset of the TriNetX research network, we identified patients diagnosed with OA and matched them 1:1 with non-OA controls based on propensity scores. Matching covariates included age, sex, race, and comorbidities. The cohort consisted of 1,554,182 patients in each group. The hazard ratio of TF and CTS, as well as related surgical interventions, was assessed over a 5-year follow-up period., Results: Patients with OA had a 1.30-fold increased risk of TF [95% confidence interval (CI)=1.27-1.33] and a 1.50-fold increased risk of CTS (95%CI=1.48-1.53) compared to controls. The hazard ratios for undergoing surgical interventions were 1.61 for TF (95%CI=1.51-1.71) and 1.97 for CTS (95%CI=1.78-2.19). These risks remained significant across various sensitivity analyses and stratifications according to age and sex., Conclusion: OA significantly increases the risk of TF and CTS. These findings highlight the need for vigilant monitoring and management of upper limb disorders in OA patients to improve overall patient care and outcomes. Future research is warranted to focus on pathological mechanisms of OA and their impact on upper limb health to develop targeted interventions., (Copyright © 2024, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Clinical Scenario: Thoracic outlet syndrome is quite challenging to diagnose. Currently, there are myriad diagnostic procedures used in the diagnosis of all types of thoracic outlet syndrome. However, controversy exists over which diagnostic procedures produce accurate findings. Clinical Question: Can clinical diagnostic tests accurately diagnose patients presenting with symptoms of thoracic outlet syndrome? Summary of Key Findings: A thorough literature search returned 6 possible studies; 3 studies met the inclusion criteria and were included. Two studies supported the use of clinical diagnostic tests for the diagnosis of thoracic outlet syndrome. One study reported high false-positive rates among clinical diagnostic tests for thoracic outlet syndrome. One study reported that clinical diagnostic test findings correlate to provocative positioned magnetic resonance imaging findings. Clinical Bottom Line: There is moderate evidence to support the use of the Halstead maneuver (also known as the costoclavicular maneuver or exaggerated military brace test), Wright's test, Cyriax Release test, and supraclavicular pressure test to have good diagnostic accuracy for the provocation of symptoms in patients presenting with upper extremity pathology. However, these clinical diagnostic tests do not appear to allow for the differential diagnosis of thoracic outlet syndrome exclusively. The use of the Adson's test and Roos test should be discontinued for the differential diagnosis of thoracic outlet syndrome. Strength of Recommendation: Grade B evidence exists to support the accuracy of the Halstead maneuver, Wright's test, Cyriax Release test, and supraclavicular pressure test for the diagnosis of upper extremity pathology in general. Grade C evidence exists for the use of these clinical diagnostic tests to exclusively diagnose thoracic outlet syndrome. [ABSTRACT FROM AUTHOR]

We investigate the etiology of amyotrophic lateral sclerosis (ALS) in a 35-year-old woman presenting with progressive weakness in her left upper limb. Prior to sequencing, a comprehensive neurological work-up was performed, including neurological examination, electrophysiology, biomarker assessment, and brain and spinal cord MRI. Six months before evaluation, the patient experienced weakness and atrophy in her left hand, accompanied by brisk reflexes and Hoffman sign in the same arm. Electroneuromyography revealed lower motor neuron involvement in three body regions. Neurofilament light chains were elevated in her cerebrospinal fluid. Brain imaging showed asymmetrical T2 hyperintensity of the corticospinal tracts and T2 linear hypointensity of the precentral gyri. Trio genome sequencing identified a likely pathogenic de novo variant in the KIF1A gene (NM_001244008.2): c.574A>G, p.(Ile192Val). Pathogenic variants in KIF1A have been associated with a wide range of neurological manifestations called KIF1A-associated neurological diseases (KAND). This report describes a likely pathogenic de novo variant in KIF1A associated with ALS, expanding the phenotypic spectrum of KAND and our understanding of the pathophysiology of ALS.

Background: CIC-rearranged sarcomas (CRS) represent a new entity of undifferentiated small round cell sarcoma belonging to the Ewing-like sarcomas family. CRS are the most common type. Fusion partners for the CIC gene include DUX4, FOXO4, and the recently recognizedNUTM1. Rare cases of CIC::NUTM1 sarcoma in pediatric patients have recently been reported in brain, kidney, bone, and soft tissues. However, such cases have not been identified in the soft tissues of the limbs., Case Presentation: We reported a case of CIC::NUTM1 sarcoma located in the right upper limb of an 18-year-old man. The tumor displayed morphologic features typical of CIC::DUX4 sarcomas, with small- to medium-sized round cells, a lobular pattern, focal spindling, myxoid stroma, and patchy necrosis. The tumor diffusely expressed NUTM1, was positive for WT1cter at weak to moderate intensity, and was focally positive for CD99, while it was negative for keratins, EMA, P40, MyoD1, myogenin, NKX2.2, BCOR, and pan-TRK. Fluorescence in situ hybridization analyses revealed cleavage of the CIC and NUTM1 genes., Conclusion: CIC::NUTM1 sarcomas represent a novel molecular variant of CRS with a preference for the central nervous system and younger pediatric persons. Its morphology and phenotype may be mistaken for NUT carcinomas, and the behavior is more progressive than other forms of CRS. For this rare and newly discovered gene fusion variant, it is necessary to integrate molecular and immunohistochemical findings with morphologic features in the diagnosis of undifferentiated neoplasms., (© 2024. The Author(s).)

For patients with upper limb melanoma, the significance of specific death is more important than that of all-cause death, and traditional survival analysis may overestimate the mortality rate of patients. Therefore, the nomogram model for predicting the specific mortality risk of melanoma in the upper limbs was developed. A population with melanoma in the upper limbs, diagnosed from 2010 to 2015, were selected from the National Cancer Institute database of Surveillance, Epidemiology, and End Results (SEER). The independent predictive factors of specific death were confirmed by the competing risk model of one-factor analysis and multi-factor analysis, and the nomogram was constructed according to the independent predictive factors. 17,200 patients with upper limb melanoma were enrolled in the study (training cohort: n = 12,040; validation cohort: n = 5160). Multi-factor analysis of the competing risk model showed that age, marital status, gender, tumor stage, T stage, M stage, regional lymph node surgery information, radiotherapy, chemotherapy, mitotic cell count, ulcer and whether there were multiple primary cancers, were independent factors affecting the specific death of upper limb melanoma patients (P < 0.05). The nomogram has good predictive ability regarding the specific mortality risk of melanoma in the upper limbs, and could be of great help to formulate prognostic treatment strategies and follow-up strategies that are conducive to survival., (© 2024. The Author(s).)

Background: Anatomic and functional descriptions of trunk and breast lymphedema following breast cancer treatment are emerging as indicators of lymphatic dysfunction. Indocyanine green-lymphangiography has been instrumental in characterizing this dysfunction in the extremity and can be applied to other regions. Previous work has established a validated Pittsburgh Trunk Lymphedema Staging System to characterize such affected areas. This study aims to identify risk and protective factors for the development of truncal and upper extremity lymphedema using alternative lymphatic drainage, providing implications for medical and surgical treatment., Methods: Patients undergoing revisional breast surgery with suspicion of upper extremity lymphedema between 12/2014 and 3/2020 were offered lymphangiography. The breast and lateral/anterior trunks were visualized and blindly evaluated for axillary and inguinal lymphatic flow. A linear-weighted Cohen's kappa statistic was calculated comparing alternative drainage evaluation. Binomial regression was used to compute relative risks (RRs). Significance was assessed at alpha = 0.05., Results: Eighty-six sides (46 patients) were included. Twelve sides underwent no treatment and were considered controls. Eighty-eight percent of the noncontrols had alternative lymphatic flow to the ipsilateral axillae (64%), ipsilateral groins (57%), contralateral axillae (20.3%), and contralateral groins (9.3%). Cohen's kappa for alternative drainage was 0.631 ± 0.043. Ipsilateral axillary and contralateral inguinal drainage were associated with reduced risk of developing truncal lymphedema [RR 0.78, confidence interval (CI) 0.63-0.97, P = 0.04; RR 0.32, CI 0.13-0.79, P = 0.01, respectively]. Radiation therapy increased risk of truncal and upper extremity lymphedema (RR 3.69, CI 0.96-14.15, P = 0.02; RR 1.92, CI 1.09-3.39, P = 0.03, respectively). Contralateral axillary drainage and axillary lymph node dissection were associated with increased risk of upper extremity lymphedema (RR 4.25, CI 1.09-16.61, P = 0.01; RR 2.83, CI 1.23-6.52, P = 0.01, respectively)., Conclusions: Building upon previous work, this study shows risk and protective factors for the development of truncal and upper extremity lymphedema. Most prevalent alternative channels drain to the ipsilateral axilla and groin. Ipsilateral axillary and contralateral inguinal drainage were associated with reduced risk of truncal lymphedema. Patients with radiation, axillary dissection, and contralateral axillary drainage were associated with increased risk of upper extremity lymphedema. These findings have important clinical implications for postoperative manual lymphatic drainage and for determining eligibility for lymphovenous bypass surgery., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Primary lymphoedema, axillary web syndrome (AWS) and yellow nail syndrome may be related. Mr B is a 66-year-old gentleman with genital lymphoedema and lymphoedema of all four extremities. In 2023, he was diagnosed with non-Hodgkin lymphoma and also underwent cardiac surgery. In November 2023, he completed an inpatient rehabilitation at the Földi clinic in Germany, where he received intensive treatment for his lymphoedema and was also diagnosed with bilateral AWS. The presence of AWS in a patient with primary lymphoedema and no history of axillary surgery is unique. Although AWS typically presents after axillary surgery, this case highlights that it can also occur in patients without lymph node surgery. While the precise cause of this presentation of AWS is not known, it may be connected to yellow nail syndrome or potentially the recent chemotherapy treatment. This article will describe the clinical case, highlighting the need for further research on AWS present in primary lymphoedema.

Introduction: Upper extremity (UE) desmoid tumors are locally aggressive neoplasms with high recurrence rates. Our study sought to analyze the demographics and treatment strategies of UE desmoid tumors and identify risk factors for recurrence., Materials and Methods: A retrospective review of 52 patients with histologically confirmed UE desmoid tumors treated at our institution between 1990 and 2015 was conducted. Survival was assessed using the Kaplan-Meier method and the Cox proportional hazards model was used for risk factor analysis., Results: For the entire cohort, median age was 40 (29-47) years, 75% were female, and 48% had local recurrence. The median tumor size was 45 (15-111) cm 3 on imaging. Twenty-two patients had a previous resection. The most common treatments were surgery alone (50%) and surgery with adjuvant radiotherapy (21%). Tumor size ≥5 cm and tumor volume ≥40 cm 3 on imaging were associated with increased recurrence (p = 0.006 and p = 0.005, respectively). Age and sex were not associated with local recurrence. Patients with a tumor size ≥5 cm were 2.6 times more likely to present with recurrence. At the 10-year mark, a lower local recurrence-free survival was seen in patients with tumors ≥5 cm (72.2% vs. 36.3%, p = 0.042) or ≥40 cm 3 (67.2% vs. 32.7%, p = 0.034)., Conclusion: In our study, only tumor dimensions appeared to modify recurrence risk., (© 2023 Wiley Periodicals LLC.)

Giant cell tumour (GCT) accounts for 5% of all primary bone tumours. GCT in the distal third of ulna is quite rare. We present a case of recurrent GCT in distal third of ulna with malignant features involving tenosynovium. The case was treated by wide resection of tumour and on follow up, patient recovered well with no evidence of further recurrence. Considering the features, according to the literature reviewed, is the first case of its type., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Uncommon Nerve Compression Syndromes: In regard to the complex anatomical relationship of peripheral nerves and muscles, tendons, fasciae as well as their long course within those anatomical structures and additional close contact to bony structures, they are prone to suffer from local compression syndromes. Hence creating a vast majority of entrapment syndromes - well described in literature for almost every single nerve. The purpose of this article is to give an overview of symptoms, signs, diagnostic studies and treatment options, addressing especially the less known syndromes. Compression syndromes of the upper arm and shoulder region include the suprascapular nerve syndrome the compression of the axillary nerve within the spatium quadrilaterale and the compression of the long thoracic nerve at the chest wall. The upper extremity offers a variety of infrequent entrapment syndromes, as the pronator teres syndrome and anterior interosseus syndrome, both resulting from pressure to the median nerve in the forearm. Compression neuropathy in the course of the radial nerve in the distal upper extremity is also known as supinator syndrome. Guyon's canal syndrome is the ulnar side equivalent to the well-known carpal tunnel syndrome. In the case of a Cheiralgia paresthetica, a compression of a sensory branch of the superficial radial nerve can be seen. In the lower extremities, a variety of nerves especially in the groin and thigh area can be compressed as they pass through the narrow spaces between the abdominal muscles or underneath the inguinal ligament. Compression of the lateral femoral cutaneous nerve is the most common syndrome. Compression syndromes of the femoral and obturator nerves are most often iatrogenic. Pain around the knee, especially the lateral part and following orthopedic procedures of the knee, can arise from a compression or a lesion of a small infrapatellar branch of the saphenous nerve. Another probably underdiagnosed syndrome is piriformis syndrome, resulting from an entrapment of the sciatic nerve as it passes through certain muscular structures. In the distal lower extremity, the peroneal and tibial nerves can be compressed at multple sites, clinically known as peroneal nerve paralysis resulting from nerve compression around the fibular head, the anterior and posterior tarsal tunnel syndrome, and Morton's metatarsalgia., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Introduction: The humerus is a common site of metastases and primary tumors. For some patients with a segmental defect and/or diaphyseal cortical destruction a cemented intercalary device may provide a more reliable construct, however data on their use is limited., Methods: We reviewed 43 (28 male and 15 female) patients treated with an intercalary humeral spacer at a single tertiary referral center between 1989 and 2022. Humeral lesions were most commonly secondary to metastatic disease (n = 29, 68%), with 25 (58%) patients presenting with a pathologic fracture. Mean age and body mass index were 66 years and 27.9 kg/m 2 . First generation taper joint device were used in 22 patients and second-generation lap device in 21 patients., Results: Following reconstruction the 2-year overall survival was 30%. Mechanical complications occurred in 11 patients, most commonly aseptic loosening (n = 6, 14%). With death as a competing risk, the cumulative incidence of mechanical failure was 28% at 2-years postoperative. Following the procedure, mean Musculoskeletal Tumor Society scores was 70% and mean shoulder elevation was 87°., Conclusion: Reconstruction of the humeral diaphysis with an intercalary endoprosthesis provides restoration of function of the upper extremity, however, is associated with one in four patients having mechanical failure., (© 2023 Wiley Periodicals LLC.)

Objective: To analyze the effect the LYMPHA technique on the incidence of upper limb lymphedema in patients with breast cancer after complete axillary lymph node dissection., Material and Methods: There were 89 patients with breast cancer and signs of metastatic lesion of axillary lymph nodes who underwent complete axillary lymph dissection. In group 1 (41 patients), the LYMPHA technique was used simultaneously with lymph node dissection; in group 2 (48 patients) - lymph node dissection alone., Results: The follow-up period was 1 year. The LYMPHA technique prolonged surgery and decreased duration of postoperative lymphorrhea. The incidence of upper limb lymphedema was 9.8% and 22.9%, respectively., Conclusion: The LYMPHA technique was effective for prevention of upper limb lymphedema after complete axillary lymph node dissection in the treatment of breast cancer.

Introduction: Pilomatrixoma is a benign skin neoplasm that is common in children and is often misdiagnosed. This study aimed to summarize the clinical and pathological features of pilomatrixoma in children., Methods: Data on demographic information, clinical and pathological features, diagnosis, and treatment of 171 patients with pilomatrixoma from Shenzhen Baoan Women's and Children's Hospital were collected and analyzed retrospectively., Results: The mean age of the patients was 5.7 (standard deviation [SD] = 3.9) years old, and there were 2 age peaks (≤1 year old, 5-11 years old) and 2 age valleys (2-4 years old, ≥12 years old). The mean disease course was 9.3 (SD = 14.1) months, 69.0%, 86.5%, and 95.3% of the patients' disease course in 6 months, 12 months, and 24 months, respectively. The mean tumor volume was 0.6 (SD = 1.0) cm3, and 81.3% of the patients' tumor volume ≤1.0 cm3. Tumors were distributed sequentially in the head and neck (77.2%), upper limbs (12.9%), trunk (7.6%), and lower limbs (2.3%). The correct rates of clinical and ultrasonic diagnosis were 50.9% and 38.6%, respectively. The two most common pathological features of pilomatrixoma were shadow cells (99.4%) and basaloid cells (94.7%). There were no significant differences in age, disease course, or tumor volume between the male and female patients (p &gt; 0.05). The age and tumor volume of the patients in different body parts were significantly different (P1 = 3.10E-05 and P2 = 5.60E-05, respectively). The correlation between the disease course and tumor volume was positively significant (p ≤ 0.05). There was a significant correlation between the disease course and tumor volume in patients with tumors at upper limbs (p = 0.03)., Conclusion: The age of children with pilomatrixoma presented 2 peaks and 2 valleys. Most patients had disease courses in 24 months and with tumor volumes ≤1.0 cm3. The correct rates of clinical and ultrasonic diagnosis were relatively low. The head and neck were the most common distribution sites of pilomatrixoma, and shadow cells and basaloid cells were the most common pathological features. The tumor volume was positively correlated with disease course in patients with pilomatrixoma., (© 2024 S. Karger AG, Basel.)

Abstract: Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Parosteal osteosarcomas are low-grade bony malignancies that are treated primarily with surgical resection and reconstruction. This report discusses a unique case of a pediatric patient who presented with a parosteal osteosarcoma of the distal radius causing extensive erosive mass effect and growth disturbance of the adjacent ulna. Likely due to their slow-growing nonaggressive nature, parosteal osteosarcomas have not been previously described to abut adjacent bony structures through direct contact. The patient presented in a significantly delayed manner due to social circumstances, inadvertently revealing this novel behavior. This report reviews this rare case and describes the current understanding of this tumor., Case Presentation: The patient is a 13-year-old male who presented with a parosteal osteosarcoma of his distal radius. He presented with a palpable wrist mass and wrist stiffness. He presented in a delayed manner with advanced local disease due to social factors. Imaging revealed an osseous radial mass that abutted the ulna and likely stunted its growth. The patient ultimately underwent complex resection and allograft reconstruction of both his distal radius and ulna. Intraoperative pathology was confirmed to have negative tumor margins. Allograft reconstruction of the radius and ulna was performed utilizing patient-specific custom cutting guides. At the 6-month postoperative visit, the patient had no recurrence of the mass, minimal pain, and had almost regained range of motion of the extremities. Clinical radiographs at the 6-month postoperative visit demonstrated allograft incorporation., Conclusions: A previously unreported case of pediatric parosteal osteosarcoma of the distal radius with erosion of the adjacent ulna through direct contact is presented. The challenges in and the importance of arriving at a definitive diagnosis in a timely manner for the proper treatment of this malignancy are emphasized., (© 2023. The Author(s).)

Purpose: We aimed to examine potential associations between post-surgical upper limb morbidity and demographic, medical, surgical, and health-related fitness variables in newly diagnosed individuals with breast cancer., Methods: Participants were recruited between 2012 and 2019. Objective measures of health-related fitness, body composition, shoulder range of motion, axillary web syndrome, and lymphedema were performed within 3 months of breast cancer surgery, and prior to or at the start of adjuvant cancer treatment., Results: Upper limb morbidity was identified in 54% of participants and was associated with poorer upper limb function and higher pain. Multivariable logistic regression analysis identified mastectomy versus breast-conserving surgery (odds ratio [OR] 3.51, 95% confidence interval [CI] 2.65-4.65), axillary lymph node dissection versus sentinel lymph node dissection (OR 2.67, 95% CI 1.73-4.10), earlier versus later time from surgery (OR 1.58, 95% CI 1.15-2.18), and younger versus older age (OR 1.01, 95% CI 1.00-1.03) as significantly associated with a higher odds of upper limb morbidity, while mastectomy (OR 1.57, 95% CI 1.10-2.25), axillary lymph node dissection (OR 2.20, 95% CI 1.34-3.60), lower muscular endurance (OR 1.10, 95% CI 1.01-1.16) and higher percentage body fat (OR 1.04, 95% CI 1.00-1.07) were significantly associated with higher odds of moderate or greater morbidity severity., Conclusions: Upper limb morbidity is common in individuals after breast cancer surgery prior to adjuvant cancer treatment. Health-related fitness variables were associated with severity of upper limb morbidity. Findings may facilitate prospective surveillance of individuals at higher risk of developing upper limb morbidity., (© 2023. Society of Surgical Oncology.)

Background: Trapezial pommel, or ulnar osteophyte positioned at the vertex of the saddle-shaped facet, is a consistent structural appearance in osteoarthritis (OA) of the first carpometacarpal. This study investigates its relation to radiographic measures (modified Eaton staging and thumb OA [ThOA] index) and wear patterns (trapezial surface morphology and cartilaginous eburnation)., Methods: In all, 137 whole trapezia were explanted from 116 patients and evaluated for Eaton staging, morphology (saddle, cirque, or dish), and eburnation (degree of cartilaginous effacement) of the articular surface of the trapezium. In total, 131 Robert's views and 126 stress views were reviewed by 2 blinded senior surgeons for ThOA index and pommel size. Statistical analyses included Spearman correlation and linear regression., Results: Standardized pommel size achieved good intrarater reliability (correlation coefficient: 0.80-0.98) and moderate interrater reliability (correlation coefficient: 0.60-0.67). The ThOA index and pommel size were significantly correlated across Robert's ( r s = 0.51) and stress views ( r s = 0.64). The ThOA index better distinguished between stages compared with pommel size. All the radiographic measures inversely correlated with preserved cartilage and varied across morphologies. Pommel size differed significantly between dish and saddle, and the ThOA index was significantly different between all morphologies when using stress views., Conclusions: We reliably quantified the pommel feature and demonstrated significant correlations with other radiographic and topologic measures of arthritic disease. If future studies can demonstrate that the pommel is a pathogenic process in ThOA and its correction can curb disease progression, the identification of the pommel feature may help guide targeted intervention., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Benign tumours of the upper extremity are common in hand surgeons' practice. The most commonly diagnosed are giant-cell tumours of the tendon sheath and lipomas., The Objective: of this study was an investigation into the distribution of tumours in the upper limb, their symptomatology and outcomes of surgery, particularly regarding the rate of recurrence., Material and Methods: A total of 346 patients, 234 women (68%) and 112 men (32%), who had undergone surgery for tumours located in the upper extremity which were not ganglion cysts were enrolled into the study. The follow-up assessment was performed at a mean of 21 months (range 12-36) post-operatively., Results: The most common tumour in this study was giant cell tumour of the tendon sheath - 96 cases (27.7%), followed by lipoma - 44 cases (12.7%). Most lesions - 231 (67%) were localized in the digits. A total of 79 (23%) recurrences were noted, the most common after surgery for rheumatoid nodules - 43.3% and the giant-cell tumours of the tendon sheath - 31.3%. The independent factors increasing risk of recurrence following the tumour's resection were: histological type of the lesion - the giant-cell tumour of the tendon sheath (p=0.0086) and the rheumatoid nodule (p=0.0027), as well as a combination of incomplete (non-radical) and not "en block" resection of tumours. A brief review of the literature referring to the presented material is offered., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Background: The classic type of epithelioid sarcoma (ES) is a rare, aggressive soft tissue neoplasm that most commonly affects the distal upper extremities of young patients. This study aimed to assess clinical features and provide a long-term report of the oncological outcome., Methods: We retrospectively analyzed our clinical database for patients with ES of the distal upper extremities., Results: Twenty-three patients with ES of the distal upper extremity were treated surgically between January 1990 and August 2018. ES affected most commonly the palmar side of young patients. The most common site affected by a sarcoma was the wrist in 47.8% of cases, followed by metacarpals and fingers with 34.8% and 17.4%, respectively. Most of the patients were treated according to the protocols of interdisciplinary tumor boards with multimodal therapy. A local recurrence was observed in 7 patients (30.4%). The 5 - and 10-year recurrence-free survival was 80.4% (95% confidence interval [CI]: 68.6-76.8) and 60.9% (95% CI: 53.5-68.3), respectively. The 5- and 10-years disease-specific survival was 89.9% (95% CI: 87-92.8) and 61.9% (95% CI: 56.5-67.3), respectively. Five patients (21.7%) had metastasis in regional lymph nodes., Conclusion: The classic type of ES represents a group of high-grade sarcomas, which affect the dominantly distal upper extremity. Specific clinical, diagnostic, and oncological characteristics make it difficult to diagnose and therapy. Wide tumor resection as a part of multimodal therapy remains a more viable and common treatment option for patients with ES on distal extremities. High rates of lymph node metastasis are typical for ES.

Hourglass-like constriction (HLC) is an uncommon spontaneous mononeuropathy that is typically characterised by a sudden onset of pain followed by palsy, affecting branches of the radial (posterior interosseous nerve) and median nerves (anterior interosseous nerve). HLC of the radial nerve (RN) is rare, with only a few reported cases. Here, we report a case of a man who presented with acute wrist and finger drop due to the HLC of the RN. Surgery was recommended 5 months after clinical observation, when the lesion was resected and primarily repaired, resulting in satisfactory recovery. There is still much that remains unknown about HLC, especially for RN. The current understanding points out an inflammatory disease that should be treated conservatively for 3-7 months. The surgical technique depends mostly on the severity and extent of constriction; however, considering only RN constrictions, primary repair by neurorrhaphy or nerve grafts resulted in better functional outcomes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction:  Fractures of the upper extremity are common traumatic injuries in children. Nerve lesions are a rare but typical complication of these fractures. Additional to physical, electrophysiological, and sonographic examinations, magnetic resonance neurography (MRN) can be used to assess the degree and exact localization of nerve damage. This retrospective study was conducted to evaluate the potential role of this examination technique for children and to test a proposed MRN classification of traumatic nerve injury according to Chhabra in a pediatric cohort., Materials and Methods:  Pediatric patients undergoing MRN for traumatic nerve injury from January 2016 to December 2020 were retrospectively identified. A total of 12 consecutive patients with sufficient clinical data, an MRN, and if available follow-up examination were enrolled and analyzed., Results:  In 10 of 12 cases one or more nerve lesions could be identified by MRN using the classification proposed by Chhabra et al. MRN was used to assess nerve injuries, imaging results were compared with clinical course. Clinical follow-up examinations of 10 patients showed an overall good clinical recovery, even in one case with severe trauma and nerve surgery., Conclusion:  MRN as a noninvasive procedure can help in the evaluation of nerve injury, especially for the identification of lower grade nerve damage and to objectify suspected nerve damage in case of uncertain clinical examination results; thus, can help in decision making whether surgical revision or conservative treatment is preferable., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: The lateral upper arm channel is an accessory lymphatic pathway that drains the upper extremity by means of the deltopectoral groove and supraclavicular nodes, thereby bypassing the axilla. Its variable connectivity to the forearm has not been studied in vivo., Methods: Indocyanine green (ICG) lymphography was performed preoperatively to map the superficial and functional arm lymphatics in breast cancer patients without clinical or objective evidence of lymphedema. A retrospective review was performed to extract demographic, ICG imaging, and surgical data., Results: Sixty patients underwent ICG lymphography before axillary lymph node dissection between June of 2019 and October of 2020. In 59%, the lateral upper arm lymphatic channel was contiguous with the forearm (long bundle). In 38%, the lateral upper arm lymphatic channel was present but not contiguous with the forearm (short bundle). In 3%, the lateral upper arm pathway was entirely absent. Seven patients developed at least one sign of lymphedema during postoperative surveillance, of which 71% demonstrated the short bundle variant., Conclusion: Although the lateral upper arm pathway is most often present, its connections to the forearm are frequently absent (short bundle), which, in this pilot report, appears to represent a potential risk factor for the development of lymphedema., Clinical Question/level of Evidence: Risk, V., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Objective: To investigate the value of high frequency ultrasound in diagnosis of neuralgic amyotrophy., Materials and Methods: From January 2010 to December 2020, the ultrasonographic images of 117 patients with neuralgic amyotrophy diagnosed by the Department of Neurology and hand & foot surgery of Shandong Provincial Hospital Affiliated to Shandong First Medical University were retrospectively analyzed. The ultrasonographic features were summarized., Results: High frequency ultrasound could clearly show the degree of the affected nerves: No ultrasonic findings were found in 12 cases (10%). The affected nerves were thickening and hypoechogenicity with loss of normal fascicular definition in 28 cases (24%). The affected nerves showed hourglass-like changes, including constriction and torsion in 77 cases (66%). In addition, ultrasound can determine the extent of the lesion, and microvascular imaging can display small blood flow signal within the nerve. There was a significant statistical difference between the diameter of the thickened nerve fascicle and the diameter of the nerve fascicle at the corresponding site of the contralateral normal limb., Conclusions: High frequency ultrasound is a valuable imaging method for diagnosis of neuralgic amyotrophy., (© 2022. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Background: Breast cancer-related lymphedema (BCRL) is a limiting sequelae of breast cancer treatment that may negatively impact 30% to 50% of high-risk breast cancer survivors. Risk factors for development of BCRL include axillary lymph node dissection (ALND), and recently, axillary reverse lymphatic mapping and immediate lymphovenous reconstruction (ILR) at time of ALND have been implemented to prevent BCRL. Reliable anatomy of neighboring venules has been commented on in the literature; however, little information exists about anatomical location of local lymphatic channels amenable for bypass., Methods: After institutional review board approval, patients who underwent ALND with axillary reverse lymphatic mapping and ILR at a tertiary cancer center from November 2021 to August 2022 were applicable for this study. The location and number of lymphatic channels used for ILR were identified and measured intraoperatively with the arm abducted to 90 degrees and soft tissue under no tension. Four measurements were taken to localize each lymphatic and were based on relationship with reliable anatomic landmarks including 4th rib, anterior axillary line, and lower border of the pectoralis major muscle. Demographics, oncologic treatments, intraoperative factors, and outcomes were prospectively maintained., Results: Twenty-seven patients met inclusion for this study by August 2022 with a total of 86 lymphatic channels identified. Patients were on average 50 ± 12 years old with a body mass index of 30 ± 6 and had an average of 1 vein and 3 identifiable lymphatic channels amenable to bypass. Seventy percent of lymphatic channels were found in a cluster of 2 or more channels. The average horizontal location was 4.5 ± 1.4 cm lateral to the 4th rib. The average vertical location was 1.3 ± 0.9 cm from the superior border of the 4th rib., Conclusions: These data comment upon intraoperatively identified and consistent location of upper extremity lymphatic channels used for ILR. These lymphatic channels are often found in clusters with 2 or more lymphatic channels at the same location. Such insight may aid in easier intraoperative identification of amenable vessels for the unexperienced surgeon, decrease in intraoperative time, and higher success of ILR., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Plexiform fibrohistiocytic tumors are rare, low-to-moderate malignant soft tissue tumors that occur primarily in children and adolescents and are located on the upper extremity. The diagnosis must be made histologically. We report on a young woman who presented a growing, painless lesion on the cubital fossa. Histopathology as well as the standard of treatment are discussed., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Introduction: Magnetic resonance imaging (MRI) stage 1 (early stage) upper extremity lymphedema is characterized by fluid infiltration in the subcutaneous tissues that does not exceed 50% of the extremity circumference at any level. The spatial fluid distribution in these cases has not been detailed and may be important to help determine the presence and location of compensatory lymphatic channels. The aim of this study is to determine whether there was a pattern of distribution of fluid infiltration in patients with early-stage lymphedema that could correspond to known lymphatic pathways in the upper extremity., Methods: A retrospective review identified all patients with MRI stage 1 upper extremity lymphedema who were evaluated at a single lymphatic center. Using a standardized scoring system, a radiologist graded the severity of fluid infiltration at 18 anatomical locations. A cumulative spatial histogram was then created to map out regions where fluid accumulation occurred most and least frequently., Results: Eleven patients with MRI stage 1 upper extremity lymphedema were identified between January 2017 and January 2022. The mean age was 58 years and the mean BMI was 30 m/kg2. One patient had primary lymphedema and the remaining 10 had secondary lymphedema. The forearm was affected in nine cases, and fluid infiltration was predominantly concentrated along the ulnar aspect, followed by the volar aspect, while the radial aspect was completely spared. Within the upper arm, fluid was primarily concentrated distally and posteriorly, and occasionally medially., Conclusions: In patients with early-stage lymphedema, fluid infiltration is concentrated along the ulnar forearm and the posterior distal upper arm, which aligns with the tricipital lymphatic pathway. There is also sparing of fluid accumulation along the radial forearm in these patients, suggesting a more robust lymphatic drainage along this region, possibly due to a connection to the lateral upper arm pathway., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Personal Neglect (PN) is a disorder in which patients fail to attend or explore the contralateral side of their body. An increasing number of studies have considered PN as a form of body representation disorder frequently observed following damage to parietal areas. The extent and the direction of the body misrepresentation is still unclear with recent studies suggesting a general reduction of contralesional hand size. However, little is known about the specificity of this representation and whether the misrepresentation also generalises to other body parts. We explored the features of the representation of the hands and face in a group of 9 right brain damaged patients with (PN+) and without PN (PN-), when compared to a healthy control group. For this, we used a body size estimation task with pictures, in which patients were required to choose the one that most closely matched the perceived size of their body part. We found that PN + patients showed a labile body representation for both hands and face, having a larger distorted representational range. Interestingly, in comparison with PN + patients and healthy controls, PN- patients also showed misrepresentation of the left contralesional hand which could be related to impaired motor performance of their upper limb. Our findings are discussed within a theoretical framework suggesting a reliance on multisensory integration (body representation, ownership, and motor influences) for an ordered representation of the size of the body., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objectives: The aim of this literature review was to synthesise and report current practice in evaluation and reporting of scar outcomes in hand and wrist clinical research., Methods: A systematic search from inception to 2022 was conducted using three electronic databases. English language randomized controlled trials and observational cohort studies reporting standardised scar outcome measures and/or scar symptoms, appearance, impairment, function, or mental health outcomes in patients with hand and wrist scars were included. Two independent reviewers determined study eligibility and performed data extraction of a priori identified scar outcome domains. Data analysis included descriptive statistics and identification of discordance in taxonomy., Results: Fifty-nine studies were included. Elective surgery cohorts were the most frequently included clinical population (n = 28; 47%) followed by burns (n = 16; 27%). Six different standardised scar outcome measures were reported by 25% of studies however only 7% of studies utilised a patient-reported measure. Scar symptoms were the most frequently reported outcome domain (81%); but taxonomy was incongruous, constructs lacked working definitions required for generalisability and outcome measurement was variable and unreported. Nineteen different measures of scar appearance and structure were reported by 30 (51%) of studies however only nine (23%) were patient-reported. Seven different hand function PROMs were reported by 25 (43%) studies. Person-centred domains including scar acceptability (12%), mental health impact (5%), and social participation (4%) were rarely reported., Conclusions: This review highlights that evaluation and reporting of hand and wrist scar outcomes is not standardised, assessment methods and measures are under-reported and there is discordance in taxonomy. Evaluation is not person-centred, rather it is dependent on clinician assessment. Domains including scar acceptability, mental health, and social participation are rarely addressed. A stakeholder consensus derived hand and wrist scar core outcome measurement set will promote standardisation and underpin improvements in clinical research quality, transparency, and rigour., (© 2023. The Author(s).)

Abstract: Trichoepithelioma is a benign adnexal neoplasm of follicular germinative cells, with bulbs, papillae, and sheaths of perifollicular connective tissue as signs of follicular differentiation. Accordingly, trichoepithelioma may arise in any hair-bearing location, mostly on the face. That is why trichoepithelioma cannot appear in glabrous skin, and, although the dorsum of the hands and feet are a hair-bearing area, acral location is exceptional. We report the first case of trichoepithelioma localized in the finger of a 79-year-old man. It was a brown-pink, firm, 7-mm diameter, solid papule on the dorsal aspect of his left index finger. The lesion was completely asymptomatic, and he remembered to have it for many years. We describe this case highlighting its rare anatomical location and correlate its dermoscopic features with the histopathological appearance., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: A previous study reported a new ultrasonography (US) measurement technique to evaluate the cross-sectional area (ΔCSA) of lymphedema in the upper extremity. This ΔCSA correlated well with parameters, such as the circumference, volumetry, and bioimpedance analysis (BIA) in healthy people and upper extremity lymphedema patients. This study examined whether a US measurement technique is clinically useful in patients with lymphedema in the lower extremity. Methods and Results: Forty patients diagnosed with unilateral lower extremity lymphedema were enrolled in this study. The subjects' leg circumference, BIA, isokinetic strength, and ΔCSA were examined on the same day. The leg circumference was measured at 15 cm above the knee (AK) and below the knee (BK) crease using a tape measure. BIA was performed by a trained physical therapist, and the data of impedance ( Z ) at 1 and 5 kHz of each side of the lower limbs and extracellular water (ECW) were used. A fully experienced physician measured soft tissue thickness, the distance between the skin and the fascia of the muscle, three times each at the anterior, medial, posterior, and lateral aspects of the bilateral legs by US at 15 cm AK and BK. The amount of soft tissue in the ΔCSA was calculated using the designed formula from the mean values of the thicknesses. Each parameter was calculated as the ratio of the sound side to the lesion side. The Pearson and Spearman correlation coefficients were used to assess the significance of these parameters. The ratio of ΔCSA measured at 15 cm AK and BK showed strong positive correlations with the circumference difference at the same level (rho = 0.790, p  = 0.000, and rho = 0.882, p  = 0.000, respectively). In addition, it showed moderate or strong correlations with the ratio of Z at 5 and 1 kHz in the BIA of the lower limbs (AK15, r  = -0.511, p  = 0.001 and r  = -0.497, p  = 0.001; BK15, r  = -0.780, p  = 0.000 and r  = -0.756, p  = 0.000, respectively). Although ECW and body mass index showed weak positive correlations with the ratio of ΔCSA measured at 15 cm BK, there was no significant correlation between the ratio of ΔCSA and the isokinetic muscle strength. Conclusion: The ΔCSA results showed moderate-to-strong correlations with other conveniently used methods except for the isokinetic muscle strength. As the US ΔCSA technique could measure lymphedema status with a structural consideration, it could also be recommended as a conventional measurement method in patients with upper and lower extremity lymphedema.

Purpose: Axillary lymph node dissection and radiotherapy have been associated with pain, physical symptoms, and decreased functional abilities in the upper extremity. This study aimed to evaluate the potential effects of the proprioceptive neuromuscular facilitation (PNF) technique on muscle strength, pain and functionality in this patient group in comparison with progressive resistance training (PRT)., Methods: The study was conducted with a randomized clinical trial design. Sixty-six women were included in the study and randomly divided into three groups: the PNF group (n = 22), the PRT group (n = 22), and the control group (n = 22). The participants were evaluated at the baseline and after eight weeks of treatment. Outcome measures were determined as pain (the Visual Analog Scale), upper extremity strength (isokinetic dynamometer), functionality (the Disabilities of the Arm, Shoulder and Hand questionnaire), and perception of change (the Global Rating of Change Scale)., Trial Registration: ClinicalTrials.gov identifier: NCT05288036., Results: The results showed statistically significant changes in both treatment groups in terms of shoulder flexors/extensors, abductor/adductors, internal/external rotators strength/power/endurance measurement, pain, and functionality (p < 0.05). Concerning functionality and perception of change, the PNF group had a statistically significantly higher improvement compared to the remaining two groups (p < 0.05)., Conclusion: PNF is an effective technique in increasing upper extremity muscle strength, reducing pain during rest and activity, and improving functionality in patients receiving breast cancer treatment., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest related to the publication of this manuscript., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: Brain-derived neurotrophic factor (BDNF) promotes activity-dependent neuroplasticity and is released following aerobic-exercise., Objective: Feasibility and efficacy of 1.Moderate-Intensity Cycle-Ergometer-Training (MI-ET) and 2.Low-Intensity Circuit-Training (LI-CT) on BDNF-serum-concentration in chronic-stroke and consequently efficacy of motor-learning in varying BDNF-concentrations (neuroplasticity being the substrate for motor-learning) via upper-limb robotic-training (RT) in both groups., Methods: Randomised-control feasibility-study. 12-week, 3x/week intervention, 17 chronic-stroke-survivors randomized into: (1) MI-ET&RT or (2) LI-CT&RT. Both groups completed 40 mins MI-ET or LI-CT followed by 40 mins RT. Feasibility outcomes: (1) screening and enrollment-rates, (2) retention-rates, (3) adherence: (i) attendance-rates, (ii) training-duration, (4) adverse events. Primary clinical outcomes: 1. serum-BDNF changes pre-post training (immediate) and pre-training basal-levels over 12-weeks (long-term). 2.upper-limb performance with Action-Research-Arm-Test (ARAT). Additionally, feasibility of an embedded health economic evaluation (HEE) to evaluate health-costs and cost-effectiveness., Outcomes: cost-questionnaire return-rates, cost-of-illness (COI) and Health-Utitility-Index (HUI)., Results: 21.5% of eligible and contactable enrolled. 10 randomized to MI-ET and 7 to LI-CT. 85% of training-sessions were completed in MI-ET (306/360) and 76.3% in LI-CT-group (165/216). 12-weeks: Drop-outs MI-ET-10%, LI-CT-43%., Clinical Outcomes: No significant changes in immediate or long-term serum-BDNF in either group. Moderate-intensity aerobic-training did not increase serum-BDNF post-stroke. Individual but no group clinically-relevant changes in ARAT-scores. HEE outcomes at 12-weeks: 100% cost-questionnaires returned. Group-costs baseline and after treatment, consistently favouring MI-ET group. COI: (1-year-time-frame): MI-ET 67382 SD (43107) Swiss-Francs and LI-CT 95701(29473) Swiss-Francs., Conclusion: The study is feasible with modifications. Future studies should compare high-intensity versus moderate-intensity aerobic-exercise combined with higher dosage arm-training.

Melanotic nerve sheath tumor (MNST) is a rare variant of schwannoma. Here, we report an unusual case of multiple MNST lesions located in the upper limb nerves. The patient presented with a mass on the left wrist in 2016 and another mass on the left thumb in 2017. In both instances, magnetic resonance imaging scans confirmed multiple giant-cell tumors of the tendon sheath. Persistent pain in the left upper limb and numbness in the ring finger and little finger recurred in 2021. High-frequency ultrasound (HFUS) showed that the left brachial plexus nerves (C5-8) were widened compared with those on the contralateral side; the neuroma formed at the lateral cord, and the median nerve was markedly thickened. The surgical findings were consistent with the ultrasound results. Pathology confirmed that the tumors were malignant MNSTs. HFUS is important for preoperative diagnosis and lesion localization, even identifying some lesions that are unrecognized on magnetic resonance imaging; thus, HFUS is crucial for improving surgical strategy and decision-making., (© 2022. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Background: Secondary lymphedema is a debilitating morbidity. This study investigated the outcomes of vascularized lymph node transfer (VLNT) in elderly patients with secondary upper extremity lymphedema., Methods: Between 2008 and 2018, elderly (≥65 years) patients with secondary upper extremity lymphedema who underwent VLNT were retrospectively reviewed. Cheng's Lymphedema Grading, Taiwan Lymphoscintigraphy Staging, and indocyanine green lymphography were used to select the procedures. Outcome measurements included complications, circumferential difference, episodes of cellulitis, and the Lymphedema-Specific Quality of Life questionnaire (LYMQoL)., Results: Eleven patients with a mean age of 70.2 ± 5.3 years (range 65-80 years) who underwent VLNT survived and no major complications were encountered. At a mean follow-up of 6.5 ± 3.6 years (range 2-13 years), the mean limb circumferential difference was significantly improved from 25.6 ± 11.5% to 8.3 ± 4.2% (p = 0.016), and the mean episode of cellulitis was statistically reduced from 2.4 ± 1.3 to 0.4 ± 0.9 times/year (p = 0.007). At a follow-up of 24 months, four domains of Function (from 30.6 ± 2.8 to 14.5 ± 2.5), Appearance (from 18.2 ± 1.9 to 8.5 ± 2.1), Symptoms (from 30.4 ± 5.9 to 10.9 ± 1.0) and Mood (from 29.2 ± 4.4 to 10.7 ± 1.0), as well as overall LYMQoL score (from 3.9 ± 1.1 to 7.4 ± 0.5), showed statistical improvement (all p < 0.05)., Conclusions: VLNT for secondary upper extremity lymphedema in elderly patients significantly decreased the limb circumferential difference and frequency of cellulitis and improved quality of life without using compression garments postoperatively., (© 2022. Society of Surgical Oncology.)

Introduction: Lipoma of the nerve is an uncommon tumor, and usually has the highest incidence in the upper limb, especially in the median nerve. When the lesion is large it can cause peripheral neuropathy such as carpal tunnel syndrome. Therefore, a physical examination is paramount for correct surgery and patient preparation. In this article we present a case that was mainly diagnosed by a complete physical examination, which led to the selection of appropriate surgery for the patient.

Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a subtype of EDS caused by defective dermatan sulfate biosynthesis, characterized by multiple malformations (craniofacial features, ocular and visceral malformations) and progressive cutaneous, skeletal, vascular, and visceral fragility-related manifestations. Repeated dislocations and deformities of the joints due to joint relaxation are observed, causing serious damage to the musculoskeletal system of the whole body; however, the motor function of the upper limbs and the morphology of the bone joints have not been systematically investigated. In this study, we present a detailed and comprehensive report on upper limb lesions of 13 patients with a mean age at the first visit of 21 years. Twelve patients (92.3%) had a history of dislocation. Eleven patients (84.6%) had shoulder dislocations, and two patients (15.4%) had elbow dislocations. Four patients (30.8%) had elbow osteoarthritis, and three patients (23.1%) had distal radioulnar joint (DRUJ) osteoarthritis. The phalanges and metacarpals are thin, and the ratio of medullary cavity of the metacarpal bone decreases with age. As bone and joint deformity progresses, patients with mcEDS should be recommended to receive regular follow-up, including radiology. The present findings suggest an important role for dermatan sulfate in the maintenance of the skeletal system.

Background: Neuropathic arthropathy (NA) of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Syringomyelia, a spinal cord disease, is the leading cause of NA in the upper extremity., Objective: We present a systematic review of NA with syringomyelia cases alongside a case report of an adult with NA of the shoulder that occurs a few 4 years after a revelation and surgical management of a Chiari malformation with syringomyelia., Methods: A systematic review was conducted following PRISMA guidelines. A PubMed, Scopus, Isiknowledge, and manual search through references of relevant publications were used to identify all published case reports of NA. Data were collected from each case report on patient characteristics., Results: The systematic review identified 56 publications and 85 patients (including ours): nearly the same number of males (n = 41) and females (n = 44). The mean age was 50,69. Presentations included reduction of mobility (n = 66), swelling (n = 61) and sensory disorder (n = 63). The pain was absent in 41 cases. In the majority of reported cases 56 (65.1%), syringomyelia was revealed by neuropathic arthropathy, and eleven patients (12.9%) had a history of syringomyelia. Treatment was categorized into non-operative management (37[43.5%]), operative management (27[31.7%]). Following-up was non-reported in 31 (36%) cases. Improvement was reported more with patients who underwent a surgical approach than medical one 28.5% versus 8.1%., Conclusion: Physicians need to be more aware of this destructive joint disease, rare, and often misdiagnosed. Also, it is imperative to integrate clinical, pathological, and imaging findings for accurate diagnosis and for delivering appropriate therapy., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

A 28-year-old man with Hirayama disease presented with a peculiar hand posture called "horn hand" ("main en corne"). The patient noticed that he could not extend his 3rd and 4th fingers when extending the fingers of his right hand 1 year prior to presentation. On neurological examination, the strength of the finger extension is severely weak in the 1st, 3rd and 4th fingers, causing a drop finger posture, but it is only moderately weak in the 2nd and 5th fingers, enabling him to sustain the extension finger posture. The finger posture is like a bull's horns and is named "horn hand". Amyotrophy of the ulnar side of the right forearm, so-called "oblique amyotrophy", and amyotrophy of the right hand are observed. MRI examination reveals atrophy of the lower cervical spinal cord and compression of it in the neck flexion posture. Diagnosis of Hirayama disease (juvenile muscular atrophy of the distal upper extremity) is confirmed. Horn hand has been reported so far in some cases of lead neuropathy, chronic inflammatory demyelinating polyneuropathy, and distal myopathy; a "peripheral" horn hand is characterized by partial and selective lesion of the radial nerve or of the finger extensor muscles. Meanwhile, a "spinal" horn hand in this patient with Hirayama disease represents the partial and selective lesion of the spinal anterior horn neurons.

Background: Lymphedema in breast cancer survivors is a very common condition which progressively may lead to entrapment  neuropathy. In lymphedema there is accumulation of fluid due to removal of lymph nodes which causes stretching of nerve fibres within the skin, compression on top of the nerve bundle leading to nerve entrapment. This will increase the neural mechanosensitivity and functional impairment of shoulder as a protective neural response to movement or traction., Methods: This study was carried out by assessing the total 72 breast cancer survivor women, with lymphedema. Out of 72, 28 of women underwent lumpectomy, 12 underwent quadrantectomy and 32 underwent unilateral mastectomy.  These subjects were assessed for neural tissue mobility by taking pain assessment using visual analogue scale (VAS), range of motion (ROM) using goniometer, lymphedema measurement using an inch tape. The neural tissue mobility for  median nerve, ulnar nerve and radial nerve was measured using limb tension test., Result: The result obtained from this study showed that neural tissue mobility was significantly impaired in breast cancer survivors with lymphedema. The result of the upper limb tension tests showed 32 women with mild lymphedema had median nerve affected on the involved side 54.1%, about  21 women had moderate lymphedema with 75% of women had median and 25% ulnar nerve affected with median nerve affected in majority of women. Only 19 women with severe lymphedema had all the three nerves affected., Conclusion: This study of women who have undergone surgical intervention for breast cancer concludes that there was significant amount of neural tissue impairment noted to mechanical provocation test post operatively after 6 months of surgery. The study suggests that severity of lymphedema was directly related to the nerves affected due to neural tissue impairment.

Background: We aimed to analyze the computed tomography (CT) and magnetic resonance imaging (MRI) findings of gouty arthritis primarily involving the large joints of the upper limbs, signal or density characteristics of the tophi, growth patterns, involvement of the adjacent joints, and differentiation from other lesions occurring in this area and to discuss the causes of misdiagnosis., Methods: CT and MRI data were collected from 14 patients with gouty arthritis, primarily involving the shoulder and elbow joints, and their imaging features were analyzed., Results: All the patiens were ranged from 28-85 years old, and the tophi deposition can be observed on either CT or MRI.The tophi deposition apperas as slightly higher density nodules or masses on CT images,or nodules or masses on MRI with isosignal/hypointensity on T1WI and hyperintensity on T2WI. Five patients showed narrowing of the affected joint space, four had different degrees of bone erosion under the articular surface, eight developed joint effusion, and all showed surrounding soft tissue swelling. The tophi grew around the joint, with anterolateral and posterolateral tophi predominantly in the shoulder joint and dorsal tophi predominantly in the elbow joint on the MRI, with compression and edema of the surrounding soft tissues., Conclusions: Gouty arthritis occurs in the large joints of the upper limbs and is characterized by fluid accumulation in the joint capsule and the formation of tophi. These tophi are usually large, with subcutaneous bone resorption and erosion, with or without cartilage destruction. However, extensive edema appeared in the soft tissue around the tophi, but the edema only produced pressure without any obvious signs of soft tissue infiltration, which may be distinguished from the joint tumor. In addition, the gout incidence rate is increased in young patients. Therefore, when the patient has a large joint mass, it is important to confirm whether there is a history of gout., (© 2022. The Author(s).)

Background and Objectives: It is difficult to predict poststroke outcome for individuals with severe motor impairment because both clinical tests and corticospinal tract (CST) microstructure may not reliably indicate severe motor impairment. Here, we test whether imaging biomarkers beyond the CST relate to severe upper limb (UL) impairment poststroke by evaluating white matter microstructure in the corpus callosum (CC). In an international, multisite hypothesis-generating observational study, we determined if (1) CST asymmetry index (CST-AI) can differentiate between individuals with mild-moderate and severe UL impairment and (2) CC biomarkers relate to UL impairment within individuals with severe impairment poststroke. We hypothesized that CST-AI would differentiate between mild-moderate and severe impairment, but CC microstructure would relate to motor outcome for individuals with severe UL impairment., Methods: Seven cohorts with individual diffusion imaging and motor impairment (Fugl-Meyer Upper Limb) data were pooled. Hand-drawn regions-of-interest were used to seed probabilistic tractography for CST (ipsilesional/contralesional) and CC (prefrontal/premotor/motor/sensory/posterior) tracts. Our main imaging measure was mean fractional anisotropy. Linear mixed-effects regression explored relationships between candidate biomarkers and motor impairment, controlling for observations nested within cohorts, as well as age, sex, time poststroke, and lesion volume., Results: Data from 110 individuals (30 with mild-moderate and 80 with severe motor impairment) were included. In the full sample, greater CST-AI (i.e., lower fractional anisotropy in the ipsilesional hemisphere, p < 0.001) and larger lesion volume ( p = 0.139) were negatively related to impairment. In the severe subgroup, CST-AI was not reliably associated with impairment across models. Instead, lesion volume and CC microstructure explained impairment in the severe group beyond CST-AI ( p 's < 0.010)., Discussion: Within a large cohort of individuals with severe UL impairment, CC microstructure related to motor outcome poststroke. Our findings demonstrate that CST microstructure does relate to UL outcome across the full range of motor impairment but was not reliably associated within the severe subgroup. Therefore, CC microstructure may provide a promising biomarker for severe UL outcome poststroke, which may advance our ability to predict recovery in individuals with severe motor impairment after stroke., (© 2022 American Academy of Neurology.)

Purpose: Upper limb masses are one of the most common presentations in the outpatient setting. However, there is a paucity of information in the literature regarding the epidemiology of upper-extremity masses in the pediatric population. The aim of this study was to determine the differential diagnoses and clinical characteristics in children aged ≤18 years presenting with upper limb masses., Methods: A retrospective review of patients aged ≤18 years managed at our institution over a 5-year period was performed. We obtained information on patient demographics, clinical features, radiological investigations, intraoperative features, and histology. We also evaluated the correlation between the clinical and histopathological diagnoses in patients who underwent surgery., Results: There were 141 upper limb masses in our series. The mean age at presentation was 12 years (SD, 5 years). The most common presenting complaints were asymptomatic swelling, pain, and paresthesia. The majority (128; 91%) of tumors were benign; there were 5 locally aggressive tumors and 6 malignant tumors. The dominant hand was involved in 60 patients. Of 140 patients, 43 (31%) patients had surgery. The procedures included incisional biopsy, excisional biopsy, wide resection, and reconstruction. One patient required a below elbow amputation for an osteosarcoma., Conclusions: Ten percent of cases represented malignant or locally aggressive disease. We have used our observations to develop a clinical algorithm that can assist clinicians in evaluating and managing children and adolescents presenting with upper-extremity masses., Type of Study/level of Evidence: Prognostic IV., (Copyright © 2022 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Background: The lymphatic system consists of the superficial and deep lymphatic system. Several diagnostic methods are used to assess the lymphatic system. Lymphoscintigraphy and indocyanine green lymphography are widely applied, both showing disadvantages, such as a poor resolution and lack of field of view. Magnetic resonance lymphography (MRL) shows satisfactory temporal and spatial resolution. The aim of this study was to assess both the superficial and deep lymphatic system in the upper extremity of healthy subjects, using an MRL protocol. Methods and Results: Ten healthy volunteers underwent an MRL examination, using a three Tesla MRI unit. Water-soluble gadolinium was used as a contrast agent. MRL images were evaluated by an experienced radiologist on image quality, enhancement of veins and lymphatic vessels, and characteristics of the latter. Overall image quality was good to excellent. In all subjects, veins and lymphatic vessels could be distinguished. Superficial and deep lymphatic vessels were seen in 9 out of 10 subjects. Lymphatic vessels with a diameter between 0.9 and 4.3 mm were measured. Both veins and lymphatic vessels showed their characteristic appearance. Enhancement of veins was seen directly after contrast agent injection, which decreased over time. Lymphatic vessel enhancement slowly increased over time. Mean total MRL examination (room) time was 110 minutes (81 minutes scan time). Conclusions: The MRL protocol accurately visualizes both deep and superficial lymphatic vessels showing their characteristic appearances with high spatial resolution, indicating the MRL can be of value in diagnosing and staging peripheral lymphedema.