Advances in Oncologic Shoulder Girdle Resection and Reconstruction.

The bony shoulder girdle consists of the clavicle, humerus, and scapula, which work synergistically to form a complex articulation that is essential for use of the upper extremity. The shoulder girdle is the most common location for primary and secondary bone tumors in the upper extremity, and following resection of these tumors, reconstruction of the upper extremity is challenging. Compared with those in the lower extremity, reconstructive techniques in the upper extremity have historically been unreliable and fraught with complications and poor functional outcomes. Newer reconstructive techniques using reverse total shoulder arthroplasty and functional muscle flaps have shown promise to improve outcomes while reducing complications for proximal humerus reconstructions. Despite these advancements, reconstruction following scapulectomy remains challenging and is still associated with more frequent complications and compromised function.

Florid reactive periostitis of the clavicle: A case report and literature review.

RATIONALE: Florid reactive periostitis (FRP), a rare reactive bone lesion, typically presents in the short tubular bones of the extremities, with infrequent occurrences in the long tubular bones. This report discusses a unique case of FRP in the clavicle, managed through comprehensive lesion debridement and bone grafting, yielding positive results over a 3-year duration. PATIENT CONCERN: A 25-year-old male presented with a discernible mass at the left sternal end of the clavicle, discovered incidentally 2 weeks prior. The patient exhibited no clinical signs of inflammation, pain, sinus tract, or suppuration. DIAGNOSIS: Initial pathological examination of the local excision suggested benign lesions, although malignancy could not be ruled out. A definitive diagnosis of clavicular FRP was reached post complete lesion resection, with supporting evidence from postoperative pathology, imaging, and clinical symptoms. INTERVENTION: The left clavicle was reconstructed through an open surgical procedure involving total mass removal and ipsilateral extraction of an iliac bone of suitable dimensions. This was implanted into the clavicular bone defect and internally fixed with a plate. OUTCOMES: Three years of consecutive follow-up revealed no recurrence of hyperplasia, absence of mass or tenderness at the left sternal end of the clavicle, and unimpaired function of adjacent joints. LESSONS: The primary clinical challenge with FRP is its diagnosis. While pathological diagnosis remains crucial, it is also important to incorporate imaging and clinical symptoms for a comprehensive assessment. Complete mass excision may offer specific benefits in distinguishing FRP from its malignant counterparts.

A recurrent homozygous LMNA missense variant p.Thr528Met causes atypical progeroid syndrome characterized by mandibuloacral dysostosis, severe muscular dystrophy, and skeletal deformities.

Atypical progeroid syndromes (APS) are premature aging syndromes caused by pathogenic LMNA missense variants, associated with unaltered expression levels of lamins A and C, without accumulation of wild-type or deleted prelamin A isoforms, as observed in Hutchinson-Gilford progeria syndrome (HGPS) or HGPS-like syndromes. A specific LMNA missense variant, (p.Thr528Met), was previously identified in a compound heterozygous state in patients affected by APS and severe familial partial lipodystrophy, whereas heterozygosity was recently identified in patients affected by Type 2 familial partial lipodystrophy. Here, we report four unrelated boys harboring homozygosity for the p.Thr528Met, variant who presented with strikingly homogeneous APS clinical features, including osteolysis of mandibles, distal clavicles and phalanges, congenital muscular dystrophy with elevated creatine kinase levels, and major skeletal deformities. Immunofluorescence analyses of patient-derived primary fibroblasts showed a high percentage of dysmorphic nuclei with nuclear blebs and typical honeycomb patterns devoid of lamin B1. Interestingly, in some protrusions emerin or LAP2α formed aberrant aggregates, suggesting pathophysiology-associated clues. These four cases further confirm that a specific LMNA variant can lead to the development of strikingly homogeneous clinical phenotypes, in these particular cases a premature aging phenotype with major musculoskeletal involvement linked to the homozygous p.Thr528Met variant.

Clinical and functional outcome after partial or total claviculectomy without reconstruction for oncologic causes.

BACKGROUND: To achieve clear margins in rare malignant clavicular neoplasms, claviculectomy may become necessary. This study aimed to review clinical, functional, and oncologic outcomes following partial or total claviculectomy without reconstruction. METHODS: This study retrospectively included 15 patients from 2 tertiary sarcoma centers (mean age, 42.6 ± 20.3 years; 66.7% male patients). The median length of clinical and oncologic follow-up was 48.0 months (interquartile range [IQR], 24.0-83.5 months). Functional follow-up (Musculoskeletal Tumor Society score and QuickDASH score [short version of the Disabilities of the Arm, Shoulder and Hand questionnaire]) was available in 9 patients at a median of 36.0 months (IQR, 20.0-100.0 months). RESULTS: Of the 15 patients, 7 underwent total claviculectomy; 5, partial lateral claviculectomy; and 3, partial medial claviculectomy. No postoperative complications emerged. The median Musculoskeletal Tumor Society and QuickDASH scores at latest follow-up amounted to 26.0 points (IQR, 24.0-29.0 points) and 18.0 points (IQR, 11.0-22.0 points), respectively. Notably, scores tended to be lower in patients who underwent total claviculectomy (n = 2) in comparison to partial claviculectomy (n = 7). CONCLUSION: Satisfactory clinical and functional results can be achieved following partial or total claviculectomy without reconstruction, with a low complication rate and acceptable mid- to long-term function.

Complete excision of giant clavicular hydatid cyst: a case report.

BACKGROUND: Echinococcosis, also known as hydatid disease, is a zoonotic parasitic disease prevalent in pastoral areas, mainly involving the liver and lungs, and less frequently the bones and surrounding soft tissues. Diagnosis and treatment of bone hydatid disease is a challenge, and because of the insidious course of the disease, the lesions are often widely disseminated by the time patients seek medical attention. CASE PRESENTATION: A 29-year-old woman presented with a painless mass that was gradually increasing in size in the cervical thorax. Imaging revealed an enlarged clavicle with multiple bone cortical defects and the existence of cysts in the soft tissues surrounding the clavicle, for which complete excision of the clavicle and the attached cysts was performed. There was no recurrence of the cyst within one year after the operation, and the patient felt well and had normal shoulder joint movement. CONCLUSIONS: Bone hydatid may appear in bones throughout the body, and cysts that leak from the bone into the surrounding soft tissues may spread at a relatively rapid rate. Prompt surgical removal of the affected bone and surrounding cysts is necessary for treatment.

Clinical outcomes of tumors and tumor-like lesions located in the clavicle.

OBJECTIVES: The aim of this study was to examine the characterization of tumors and tumor-like lesions located in the clavicle and to present their clinical results. PATIENTS AND METHODS: Between January 2006 and December 2018, a total of 44 patients (25 males, 19 females; mean age: 36.2±21.8 years; range, 2 to 87 years) who were operated in our clinic for a clavicular lesion were included. Among 4,856 extremity tumors operated in our clinic between these years, 44 cases (0.9%) located in the clavicle were eligible. Demographic data, tumor types, location, surgical method, metastasis, survival analyzes and clinical results of these patients were reviewed retrospectively. According to the clavicle location, the patients were divided into groups according to their involvement in proximal, middle, lateral and more than one region. RESULTS: The most frequently involved site was the lateral edge of the clavicle, and the most common tumor was aneurysmal bone cyst. The most common malignant tumor was Ewing sarcoma and plasmacytoma (13.6%) and the most common surgical method in the clavicle was wide resection with 34.1%. Of the 38 primary clavicle tumors remaining after the metastatic lesions were removed, 21 (55%) were benign, while 17 (45%) were malignant. Aneurysmal bone cyst was most common in the group under 30 years of age, osteochondroma was most common in the 30-50 age group, and plasmacytoma was most common in those over 50 years of age. The mean musculoskeletal tumor society (MSTS) score of 15 patients (34.1%) who underwent clavicle resection was 79.4±6. CONCLUSION: We believe that a wide spectrum should be considered in the differential diagnosis of tumors located in the clavicle. Age is an important predictive factor for malignancy. We believe that resection should be applied without fear considering its effects on function and recurrence.

Clavicular Malignancies: A Borderline Surgical Management.

Nearly 1% of all bone cancers are primary clavicular tumors and because of their rarity, treating clinicians are unfamiliar with their diagnosis, classification, treatment options, and prognosis. In terms of preserving function and avoiding complications, clavicle reconstruction seems logical; however, further studies are needed to support this measure. Reconstruction techniques are difficult taking into account the anatomical structures surrounding the clavicle. When chest wall defects are present, a multidisciplinary team, including an orthopedist and thoracic and plastic surgeons, is of paramount importance for optimal surgical management. Malignant clavicle tumors may include primary and secondary malignancies and neighboring tumors with clavicular invasion. Surgical resection of complex thoracic tumors invading the clavicles can result in larger defects, requiring chest wall reconstruction, which is a substantial challenge for surgeons. Correct diagnosis with proper preoperative planning is essential for limiting complications. Post-resection reconstruction of the partial or total claviculectomy is important for several reasons, including maintaining the biomechanics of the scapular girdle, protecting the vessels and nerves, reducing pain, and maintaining the anatomical appearance of the shoulder. The chest wall resection and reconstruction techniques can involve either partial or full chest wall thickness, influencing the choice of reconstructive technique and materials. In the present paper, we aimed to synthesize the anatomical and physiopathological aspects and the small number of therapeutic surgical options that are currently available for these patients.

Reconstruction of congenital pseudarthrosis of the clavicle with a barrel-shaped mono-cortical iliac crest autograft: A case report.

BACKGROUND: Congenital pseudarthrosis of the clavicle (CPC) is a rare congenital entity with unresolved aetiology and pathogenesis. Nearly 250 cases have been reported to date. CPC is characterized by a definite defect in the mid-clavicle at birth and is usually diagnosed when the deformity becomes evident in late childhood or adolescence. Surgical management is controversial, especially in asymptomatic children, with various techniques reported in the literature. CASE REPORT: We report a case of a 6-year-old boy who was diagnosed with CPC during a medical examination for primary school enrollment. Operative treatment included debridement of pseudoarthrosis, internal fixation with third tube plate, and barrel-shaped mono-cortical iliac crest autograft. RESULTS: A complete bone union was obtained 9 months after the operation, and satisfactory function and cosmetic appearance were observed 4 years and 3 months postoperatively. CONCLUSION: In our opinion, reconstruction with barrel-shaped mono-cortical iliac crest autograft was an effective and reproducible surgical technique to treat CPC.

Clavicula Pro Humero Reconstruction for Malignant Tumor of the Proximal Humerus in Children and Adults.

BACKGROUND/AIM: The effectiveness of clavicula pro humero (CPH) reconstruction for pediatric proximal humerus sarcoma has been reported in a small number of cases. We aimed to investigate the effectiveness of biological CPH reconstruction for malignant bone tumors of the proximal humerus in children and adults. PATIENTS AND METHODS: This was a retrospective cohort study that included eight patients who underwent CPH reconstruction due to a malignant bone tumor around the proximal humerus. Postoperative parameters, including complications, postoperative upper limb function, and the period until bone fusion, were investigated. RESULTS: Three patients had non-union and fracture of the clavicular segment. Among them, one patient underwent revision surgery for internal fixation and bone grafting. Five patients achieved bone fusion, and the overall mean Musculoskeletal Tumor Society score was 70%, which is comparable to previous reports. CONCLUSION: CPH reconstruction is an effective technique for malignant bone tumors of the proximal humerus in all ages.

Leiomiosarcoma primario de hueso en fémur distal metastásico a clavícula: reporte de un caso / Primary leiomyosarcoma of bone in the distal femur with clavicle metastasis: a case report

El leiomiosarcoma (LMS) es un tipo de tumor de células fusiformes de muy baja incidencia, que tiene un comportamiento agresivo, con alta tasa de mortalidad, por lo que el manejo debe ser quirúrgico, con una resección amplia de la lesión. No está claro el papel de la radio ni de la quimioterapia en su manejo. Presentamos a una paciente de 28 años que consultó por dolor de 2 meses en la rodilla derecha. Radiográficamente, se caracterizó como una lesión osteolítica pura en el fémur distal. La resonancia nuclear magnética (RNM) contrastada mostró áreas hipervasculares dentro del tumor. La gammagrafía mostró un marcado aumento en la captación de radiotrazadores. Se tomó una biopsia, con un informe de patología de LMS óseo bien diferenciado. Se trató con 3 ciclos de quimioterapia neoadyuvante preoperatoria con ifosfamida 1.000 mg/m2 en los días 1 a 3, además de doxorrubicina 70 mg/m2 , y resección quirúrgica de la lesión y salvamento de la extremidad con endoprótesis de rodilla. Una vez que se resecó la lesión, la paciente recibió quimioterapia adyuvante con 4 ciclos de gencitabina 1.000 mg/m2 entre los días 1 y 8, y doxetacel 70 mg/m2 el día 1. Durante los dos meses de seguimiento, la paciente presenóa una fractura en el tercio medio de la clavícula, compatible con una lesión patológica en radiografías y tomografía por emisión de positrones (TEP). La biopsia reveló una lesión metastásica de LMS óseo que fue tratada mediante resección quirúrgica de la clavícula. Este es un caso único, dado que, durante el seguimiento, recibió tratamiento adyuvante con quimioterapia y se evaluó con una TEP, con una evolución clínica satisfactoria y sin evidencia de nuevas lesiones

Leiomyosarcoma (LMS) is a type of spindle-cell tumor of very low incidence that tumor has an aggressive behavior, with high mortality rates; therefore, its management must be surgical, with a wide resection of the lesion. The role of radio and chemotherapy in its management is not clear. We present the case of a 28-year-old female patient who consulted for pain lasting 2 months in the right knee. Radiographically, it was characterized as a pure osteolytic lesion in the distal femur. Contrast magnetic resonance imaging (MRI) showed hypervascular areas within the tumor. The scintigraphy showed a marked increase in radiotracer uptake. A biopsy was taken, with a pathology report of well-differentiated osseous LMS. It was treated with 3 cycles of preoperative neoadjuvant chemotherapy with ifosfamide 1,000 mg/m2 in the first 3 days, as well as doxorubicin 70 mg/m2 , and surgical resection of the lesion and limb salvage with knee endoprosthesis. Once the lesion was resected, the patient underwent adjuvant chemotherapy, with 4 cycles of gencitabine 1,000 mg/m2 between days 1 and 8, and doxetacel 70 mg/m2 on day 1. During the 2-month follow-up, the patient presented a fracture in the middle third of the clavicle, which was compatible with a pathological lesion on radiographs and positron-emission tomography (PET) scans. The biopsy showed a metastatic lesion of bone LMS, which was treated by surgical resection of the clavicle. This is a unique case, given that, during the follow-up, the patient underwent adjuvant treatment with chemotherapy, and was evaluated with a PET scan, with a satisfactory clinical evolution and no evidence of new lesions.

Personalized three-dimensional printed polyether-ether-ketone prosthesis for reconstruction after subtotal removal of chronic clavicle osteomyelitis: A case report.

RATIONALE: Three-dimensional (3D) printing has attracted wide attention for its potential and abilities in the assistance of surgical planning and the development of personalized prostheses. We herewith report a unique case of chronic clavicle osteomyelitis treated with a two-stage subtotal clavicle reconstruction using a 3D printed polyether-ether-ketone (PEEK) prosthesis. PATIENT CONCERNS: A 23-year-old Chinese female presented to our clinic complaining about a progressive pain of her right clavicle for about 1 year. DIAGNOSES: Chronic clavicle osteomyelitis confirmed by percutaneous biopsy and lesion biopsy. INTERVENTIONS: This patient accepted a long-term conservative treatment, which did not gain satisfactory outcomes. Thus, a subtotal removal and two-stage reconstruction of the right clavicle with a 3D-printed polyether-ether-ketone prosthesis stabilized by screw fixation system was performed. OUTCOMES: At 2-year follow-up, complete pain relief and satisfactory functional recovery of her right shoulder were observed. LESSONS: Personalized 3D printed prosthesis is an effective and feasible method for reconstruction of complex bone defects.

Homozygous deletion of MYADML2 in cranial asymmetry, reduced bone maturation, multiple dislocations, lumbar lordosis, and prominent clavicles.

A null mutation in a patient can facilitate phenotype assignment and uncovers the function of that specific gene. We present five sibs of a consanguineous Pakistani family afflicted with a new syndrome with an unusual combination of skeletal anomalies including cranial asymmetry, fused sagittal sutures deviating from the medial axis, mandibular prognathia, maxillary hypoplasia, misaligned and crowded teeth, delayed bone age, multiple dislocations, hypoplastic and malpositioned patellae, humeral intracondylar fissures, scapular dyskinesis, long limbs, lumbar lordosis, protruding chest, prominent clavicles, short 5th digital rays, and ventral transverse digital creases plus features of cutis laxa. We mapped the disease gene locus to a 3.62-Mb region at 17q25.3 and identified a homozygous deletion of maximal 7.3 kb deduced to totally inactivate MYADML2 and downstream gene PYCR1, biallelic variants in which cause autosomal recessive cutis laxa (ARCL). All five affected sibs had the most common features of ARCL but not many of the less common ones. We attributed the anomalies not typical for ARCL to MYADML2 deficit, because no other genetic defect possibly a candidate to underlie the skeletal phenotype was found. MYADML2 is a gene of unknown function, has not been studied, and has not been associated with disease. Our findings present a possible phenotype for MYADML2 deficit that includes impaired bone patterning and maturation, definitely show that the gene is not essential for survival, and provide a start point for future studies on the function of MYADML2 protein. Detection of new patients is needed to confirm and delineate MYADML2-deficiency phenotype.

Plate fixation versus intramedullary nail or Knowles pin fixation for displaced midshaft clavicle fractures: A meta-analysis of randomized controlled trials.

BACKGROUND: Plate fixation and intramedullary nail/Knowles pin fixation methods are commonly used to treat displaced midshaft clavicle fractures. However, the differences between these 2 methods are unclear. OBJECTIVE: This meta-analysis aimed to compare plate fixation and intramedullary nail/Knowles pin fixation for displaced midshaft clavicle fractures. METHODS: We searched PubMed, EBM reviews, and Ovid Medline online for studies related to comparison of plate fixation versus intramedullary nail/Knowles pin fixation for displaced midshaft clavicle fracture from inception to June 30, 2019. Relevant literature search, data extraction, and quality assessment will be performed by 2 researchers independently. The methodological quality of all included studies was appraised using the Cochrane system for randomized trials. The RevMan 5.2 software was used for heterogeneity assessment, generating funnel-plots, data synthesis, sensitivity analysis, and determining publication bias. The fixed-effects or random-effects model was used to calculate mean difference (MD)/relative risks (RRs) and 95% confidence intervals (CIs). RESULTS: This meta-analysis included 839 patients from 12 randomized controlled trials. We found that compared to plate fixation, intramedullary nail/Knowles pin fixation yielded a higher shoulder constant score [MD = -2.43, 95% CI (-3.46 to -1.41), P < .00001] and lower disabilities of the arm, shoulder and hand (DASH) score [MD = 2.98, 95% CI (0.16-5.81), P = .04], and lower infection rates [RR = 2.05, 95% CI (1.36-3.09), P = .003], operation time [MD = 20.20, 95% CI (10.80-29.60), P < .0001], incision size [MD = 6.09, 95% CI (4.54-7.65), P < .00001], and hospital stay [MD = 1.10, 95% CI (0.56-1.64), P < .00001] but with a higher removal rate [RR = 0.52, 95% CI (0.41-0.65), P < .00001] compared to plate fixation. There were no significant differences in nonunion, reintervention, or revision and refracture between these two methods. The limitation is that many studies did not demonstrate the random generated details, and only English articles were enrolled in this meta-analysis. CONCLUSIONS: Intramedullary nail/Knowles pin fixation might be an optimum choice for treating displaced midshaft clavicle fractures, with similar performance in terms of the nonunion, reintervention, or revision and refracture, and better shoulder constant and DASH scores, infection rates, and operative parameters.

Management of clavicle shaft fractures with intramedullary devices: a narrative review.

INTRODUCTION: Displaced and shortened clavicle fractures are frequently treated surgically. Although extramedullary fixation using a plate and screws is most commonly used, intramedullary nailing has become increasingly popular over the last decade. Traditional intramedullary nailing is usually associated with a high risk of hardware migration as well as hardware irritation at its entry point. Newer devices, however, seem to mitigate these problems. The aim of this narrative review is to provide an overview of clavicle shaft fractures and treatment with intramedullary nails, in particular the newer, locked devices. AREAS COVERED: In general, this review covers current literature related to clavicle shaft fractures with a specific focus on the treatment of displaced and shortened fractures with intramedullary nails. EXPERT OPINION: Clavicle shaft fractures can be effectively treated with an intramedullary nail. The risk of hardware migration when employing the newer, improved designs appears to be minimal. The advantages of using intramedullary fixation include smaller incision sizes as well as the avoidance of routine procedures generally associated with the removal of prominent subcutaneous hardware as per extramedullary fixation.

Clavicle fractures.

Clavicle fractures account for approximately 2-5% of all fractures in adults and 10-15% in children. There is a bimodal distribution, with two peaks occurring in patients <25 years of age as a result of direct trauma and in those >55 years of age secondary to a fall onto an outstretched arm. Approximately two-thirds of all clavicle fractures occur in men. This article provides an overview of the presentation, assessment and management of clavicle fractures for both core surgical trainees and acute care common stem/emergency medicine trainees.