In silico analysis of rib force distribution in postscapulothoracic arthrodesis model.

Scapulothoracic arthrodesis (STA) is carried out by fixing the scapula to thoracic ribs which in turn allows the patient suffering from Facioscapulohumeral Muscular Dystrophy to carry out shoulder-joint dependent activities of daily living. A biomechanical analysis of this procedure has not been conducted in the literature and, for the first time, this study investigates the finite element calculated glenohumeral-applied load distributions on ribs by creating a post-STA model. Three loading directions on the glenohumeral joint are designated: anterior-posterior, superior-inferior, and lateral-medial. Reaction forces on the ribs are calculated based on the glenoid force percent. Simulations are repeated by removing a singular rib contact to observe the change in force distributions in the case of missing levels or failed bonding as well as the impact of clavicle osteotomy. Total load distribution is observed highest at T2 followed by T3 and T6. In the T2 missing scenario, total loads on T3 and T4 increase. In the T4 missing case, the most affected level is T3. In the T6 missing scenario, total loads on T5 and T7 increase. In the clavicular osteotomy scenario, all levels' loads increase; the highest is recorded in T7 by 460%, followed by T5, T4, T2, T6, and T3. While all levels contribute to fixation strength, T2 is subjected to the highest loads, and, in the missing level scenarios, the loads are tolerated sufficiently by the remaining levels. Missing T4 scenario has the least effect on the system, which is interpreted as potentially the only skippable level of fixation. Clavicular osteotomy has the highest effect on the arthrodesis site.

Scapulothoracic Fusion Using Multiple Suture Tape Cerclage.

ABSTRACT: Scapulothoracic arthrodesis has been proposed for the treatment of painful scapular winging in patients with facioscapulohumeral muscular dystrophy. It was introduced to improve shoulder function. Several methods of fixation have been proposed to obtain the union of the scapula to the ribs. These include plates, screws, cables, or wires with or without bone grafting. The purpose of this manuscript is to describe the surgical technique of scapulothoracic arthrodesis using plate and cerclage suture tapes. LEVEL OF EVIDENCE: Level IV, treatment study (case series).

Erector Spinae Plane Block for Scapulothoracic Arthrodesis for Facioscapulohumeral Dystrophy Patients: A Case Series.

Facioscapulohumeral dystrophy (FSHD) typically affects the periscapular muscles, resulting in scapular winging. Scapulothoracic arthrodesis (STA) stabilizes the scapula to provide better movement for these patients. Analgesia regimen for FSHD patients who received a single-shot erector spinae plane block (ESPB) and a catheter at the area were retrospectively analyzed in this study. Patients were asked to rate their pain postoperatively and only 5 of 10 patients needed rescue analgesic. No complications occurred. Our experience suggests that continuous ESPB may be helpful for providing analgesia in FSHD patients undergoing STA.

Outcomes of scapulothoracic fusion in patients with facioscapulohumeral dystrophy: a comparison of allograft versus autograft bone grafting.

BACKGROUND: Scapulothoracic (ST) fusion for facioscapulohumeral muscular dystrophy (FSHD) is an established treatment that corrects scapular instability, although it has high reported complication rates. The purpose of our study was to characterize the outcomes of ST fusion for FSHD in a large patient cohort and compare the outcomes based on bone graft type and fixation technique. Our hypothesis was that union rates would not differ by bone graft type during ST fusion. METHODS: A retrospective chart review was undertaken to identify patients who underwent ST fusion at multiple institutions performed by a single surgeon between 2013 and 2019 with minimum 2-year follow-up. Patient demographic characteristics, surgical technique, time to union, complications, and clinical outcomes including patient-reported outcome measures were recorded. Univariate and multivariate statistical analyses including regression analyses were performed to compare preoperative and postoperative outcomes. RESULTS: A total of 50 patients with 54 ST fusions (bilateral in 4 patients) and an average follow-up period of 5.8 years (standard deviation, 1.6 years) were included for analysis. Active forward elevation (77° vs. 124°, P < .00001) and abduction (60° vs. 90°, P < .00001) both improved significantly after fusion. Average internal rotation after fusion was at spinal level L3-L4. The visual analog scale pain score (2.6 vs. 1.2, P < .00001), Subjective Shoulder Value score (33 vs. 76, P < .00001), and American Shoulder and Elbow Surgeons (ASES) score (41.8 vs. 76.1, P < .00001) all improved significantly postoperatively. Of the shoulders, 50% (27 of 54) received treatment with cerclage wires and 50% (27 of 54) received treatment with Luque wires. Femoral head allograft was used in 53.7% of shoulders (29 of 54), whereas iliac crest autograft was used in 46.3% (25 of 54). Average radiographic time to healing was 11.1 weeks (standard deviation, 3.2 weeks), with no incidence of nonunion, and did not significantly differ by bone graft type (P = .26) or technique (P = .20). The complication rate was 24.1%, including seroma (n = 3), superficial infection (n = 2), transient neurologic injury (n = 2), hemothorax (n = 1), rib fracture (n = 1), pneumothorax (n = 1), and shortness of breath (n = 1), although none requiring reoperation. There was no significant difference in the rate of postoperative complications when compared by surgical technique (P = .81) and bone graft type (P = .93). There were no independently predictive factors influencing the rate of postoperative complications by multivariate regression. Regression analysis showed that the postoperative ASES score was independently associated with the preoperative ASES score (P < .0001), use of iliac crest autograft (P < .011), and presence of complications (P < .043). CONCLUSION: Patients receiving ST fusion for FSHD demonstrate globally improved active motion and patient-reported outcome measures. Fusion construct or type of bone graft does not affect time to union or complication rates. Surgeons should be aware of a relatively high complication rate in the early postoperative period.

Management of spine deformity secondary to facioscapulohumeral dystrophy in pediatric patients. A case description and a literature review.

PURPOSE: The aim of this report is to describe the management of a severe spinal deformity in an adolescent with facioscapulohumeral dystrophy (FSHD) and review the available literature on the topic. METHODS: A 14-year-old patient with a genetically confirmed diagnosis of FSHD was evaluated for right thoracolumbar scoliosis (TL) and severe lumbar hyperlordosis. Spinal radiographs showed a right-sided curve of 32° and in the sagittal plane a lordotic curve T10-S1 -143°, TL junction -51.6°, LL -115°, pelvic incidence (PI) 25.5°, pelvic tilt 63.3°, PI-LL mismatch -90°, and a sagittal imbalance of -146 mm. An MRI scan evidenced atrophy of the paraspinal muscles. An instrumental gait analysis revealed significant pelvic anteversion associated with hip flexion and mild equinus. During follow-up, the patient developed a progressive inability to walk and difficulty sitting along with respiratory compromise and pain. RESULTS: At the age of 16 years, a posterior T2-iliac spinal fusion was performed using pedicle screws and four iliac anchors, with a 4-rod system placed at the lumbopelvic level. Significant correction of the hyperlordosis, the PI-LL mismatch, and sagittal imbalance was achieved, and the patient improved her sitting capacity, quality of life (QoL) and self-esteem and reported a decreased perception of disability at 2-year follow-up. CONCLUSIONS: This is the first published case of spinal deformity secondary to FSHD to use gait analysis to supplement the decision of optimal timing for surgery, and the second published case of spine surgery in a pediatric patient. Although spinal fusion surgery is controversial in ambulatory FSHD patients with extensive deformity, when ambulation is impaired, surgery improves function, prevents progression, and restores sagittal balance, increasing patient's QoL.

ScapuloThoracic Arthrodesis for Facio-Scapulo-Humeral Dystrophy: Outcomes at mean 7.3 years [3.5-13] follow-up. CT measurement of the fixation position of the arthrodesis and radioclinical correlations.

INTRODUCTION: Scapulothoracic arthrodesis may be proposed to patients having facio-scapulohumeral dystrophy to achieve gains in shoulder motion and pain relief. This study aimed to assess shoulder motion, pain and functional scores at last follow-up and to present a method of computed tomography measurements of the position of the scapulothoracic arthrodesis and study their correlations with shoulder motion. PATIENTS AND METHODS: Seven patients (11 arthrodesis) were included. Shoulder motion, pain, respiratory function and deltoid strength were compared with preoperative values and Constant, Brooke and Vignos scores were assessed at last follow-up. The elevation/depression and upward/downward position of the scapula were measured by performing postoperative 3D CT reconstruction. The protraction/retraction position was measured with 2D CT reconstructions on axial view. Correlations between these measurements and shoulder flexion and abduction were analysed. All complications were searched. RESULTS: We found a significant improvement in mean VAS (from 3±2 to 1±1, p=0,008) shoulder flexion (64°± 11 to 113°±20, p=0,003) and abduction (from 63°±9 to 92°±13°, p=0,004). Postoperative external rotation wasn't significantly different (from 49°±19 to 43°±10, p=0,112) and on internal rotation, the hand reached on average the 9th thoracic vertebra (S1-T2). Scapulothoracic arthrodesis was mainly positioned in regard to the 1st and the 6th rib. The mean protraction/retraction position was 38,5°±8° and the mean scapular upward/downward rotation position was 92°±15°. No correlations were found between the scapular position and shoulder flexion and abduction. CONCLUSIONS: Scapulothoracic arthrodesis for facioscapulohumeral dystrophy improved pain, shoulder flexion and abduction and provided good functional outcomes at 3,5 to 13 years of follow up. A method of CT assessment of the position of the arthrodesis is presented to analyse precisely the position of the scapula but no correlations with shoulder motions were found. Preoperative evaluation of deltoid function and scapular winging seemed to be the most important predictors of shoulder motions gains after this procedure. LEVEL OF EVIDENCE: III, Retrospective cohort study.

Bilateral Scapulothoracic Fusions Fixed with High-Strength Suture Tapes for Facioscapulohumeral Dystrophy: A Case Report.

CASE: A 32-year-old male right-hand dominant school-bus driver with facioscapulohumeral dystrophy (FSHD) was referred to an upper limb specialist for consideration for scapulothoracic fusion (STF) because of limited upper limb function and cosmetic issues. Staged bilateral STFs were performed with seven high-strength suture tapes tensioned to secure a standard tubular plate in situ. CONCLUSION: STF may be a desirable treatment option for patients with FSHD. The use of high-strength suture tapes in cases of STF allows for secure fixation of the scapulothoracic joint. This potentially confers a reduction in operative time while ensuring adequate tensioned fixation and minimizing the risk of iatrogenic pneumothorax.

A novel shoulder disability staging system for scapulothoracic arthrodesis in patients with facioscapulohumeral dystrophy.

BACKGROUND: Scapulothoracic arthrodesis (STA) is a well-established surgical technique to provide scapular stabilisation in patients with facioscapulohumeral dystrophy (FSHD). There is no staging or scoring systems available to guide surgical decision. The aim of this study was to develop a staging system to evaluate the shoulder disability in patients with FSHD to guide surgical decision-making and assess its reliability among surgeons. METHODS: Fifty-seven shoulders of 29 patients (15 male, 14 female) with an average age of 34.5 years (13-73) were included. Six stages of the disease were defined to create a system consisting of shoulder elevation, deltoid function and scapular winging. Patients were assessed by two independent orthopaedic surgeons who were blind to each other. Statistical analyses included mean and standard deviation for descriptive variables, Pearson's correlation and Cohen's Kappa for inter- and intraobserver agreement. RESULTS: Measurement of elevation showed excellent correlation in both inter- and intraobserver assessment. There was substantial agreement on deltoid function and moderate agreement on scapular winging. Decisions on stage showed excellent agreement on interobserver and substantial agreement on intraobserver assessment. Surgical decision using the stage showed excellent agreement on both inter- and intraobserver assessment. CONCLUSION: This novel staging system has an excellent inter observer agreement on FSHD patients' shoulder disability. This would provide surgeons a beneficial tool to define patient groups that would have negatively or positively affected from STA.

Bilateral scapulothoracic arthrodesis for facioscapulohumeral muscular dystrophy: function, fusion, and respiratory consequences.

BACKGROUND: Scapulothoracic arthrodesis (STA) has been proposed for the treatment of painful scapular winging in patients with facioscapulohumeral muscular dystrophy (FSHD). However, the rate of osseous fusion is variable, and there is a theoretical risk of reduced respiratory function after bilateral STA. METHODS: This was a retrospective study of 10 STAs, performed sequentially, in 5 FSHD patients. The surgical technique involved use of a semitubular plate and wire construct with autograft (iliac crest) interposed between the scapula and rib cage. Osseous fusion, respiratory function, and shoulder function were evaluated. The mean follow-up period was 141 ± 67 months (range, 24-225 months). RESULTS: Early complications included 1 pneumothorax and 1 pleural effusion. No late complications occurred, and no patients underwent reoperation. On postoperative computed tomography images, complete bony union of the scapula to the ribs was observed in 90% of shoulders (9 of 10). Comparison of preoperative and postoperative pulmonary function test results showed no significant difference in vital capacity (from 87% ± 14% to 85% ± 12%) and forced vital capacity (from 86% ± 16% to 77% ± 15%). Patients gained on average 40° of active forward elevation (from 62° ± 20° to 102° ± 4°) and 22° of abduction (from 58° ± 21° to 89° ± 7°) (P < .001). The mean Subjective Shoulder Value increased from 25% ± 8% to 72% ± 18% (P < .001). All patients were pleased with the results and would recommend the procedure to other persons. CONCLUSION: In patients with FSHD, bilateral STA provides satisfactory shoulder function with a high rate of scapulothoracic fusion and few or no significant respiratory repercussions.

Functional Outcomes and Complications Following Scapulothoracic Arthrodesis in Patients with Facioscapulohumeral Dystrophy.

BACKGROUND: Facioscapulohumeral dystrophy (FSHD) is an autosomal-dominant myopathy characterized by facial and shoulder girdle muscle weakness with scapular winging. Scapulothoracic arthrodesis is a successful treatment approach for patients with <90° of shoulder elevation. The purpose of the present study was to assess functional outcomes and complications following scapulothoracic arthrodesis in patients with FSHD. METHODS: We retrospectively reviewed the records of 40 patients (64 shoulders) in whom scapulothoracic arthrodesis was performed. To achieve fusion, multiple multifilament cables were used together with autologous bone and allograft bone. Preoperative and postoperative shoulder elevation and abduction; Disabilities of the Arm, Shoulder and Hand (Quick version, qDASH) scores; and pulmonary function were compared. Recorded complications were classified as pulmonary or scapular. RESULTS: The mean age of the patients at the time of the operation was 25.4 years (range, 15 to 60 years), and the mean duration of follow-up was 71.2 months (range, 12 to 185 months). When the preoperative values were compared with those at the latest follow-up, significant improvement was noted in terms of elevation (from a mean [and standard deviation] of 60.6° ± 17.2° to 123.7° ± 26.7°; p < 0.001), abduction (from 52.7° ± 15.8° to 98.8° ± 20.3°; p < 0.001), and qDASH scores (from 34.7 ± 11.4 to 13.3 ± 13.1; p < 0.001). The overall complication rate was 26.6%. There were 7 pulmonary complications (4 pneumothoraxes, 2 pleural effusions, and 1 major atelectasis), and 5 chest tube placements were required. Ten complications (including 3 rib fractures, 1 brachial plexus palsy, 2 cases of implant irritation, 2 nonunions, 1 delayed union, and 1 scapular fracture) were related to the scapular fixation, and 7 revision procedures were required. Scapulothoracic fusion was achieved in all patients but 1, who had a scapular fracture. Pulmonary function tests were performed for 19 patients, and no difference was observed between preoperative and postoperative results. CONCLUSIONS: Scapulothoracic arthrodesis with use of multifilament cables is a successful surgical technique with high fusion rates and low morbidity. Pulmonary complications are common but resolve with careful attention. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

A multidisciplinary clinical approach to facioscapulohumeral muscular dystrophy.

BACKGROUND AND PURPOSE: Impaired shoulder function is the most disabling problem for daily life of Fascioscapulohumeral muscular dystrophy (FSHD) patients. Scapulothoracic arthrodesis can give a high impact to the functionality of patients. Here we report our experience with scapulothoracic arthrodesis and spinal stenosis surgery in FSHD patients. METHODS: 32 FSHD patients were collected between 2015-2016. Demographical and clinical features were documented. All the patients were neurologically examined. The Medical Research Council (MRC) and the FSHD evaluation scale was used to assess muscle involvement1. Scapulothoracic arthrodesis and spinal stenosis surgeries were performed in eligible patients. RESULTS: There were 16 male and 16 female (mean age 34.4 years; range 12-73) patients. 6 shoulders of 4 patients aged between 2132 years underwent scapulothoracic arthrodesis (two bilateral, one left and one right sided). Only one 63 years old female patient with severe hyperlordosis had spinal fusion surgery. All of the patients undergoing these corrective surgeries have better functionality in daily life, as well as superior shoulder elevation. CONCLUSION: Until the emergence and clinical use of novel therapeutics, surgical interventions are indicated in carefully selected patients with FSHD to improve arm movements, the posture and the quality of life of patients in general. Scapulothorosic arthrodesis is a management with good clinical results and patient satisfaction. In selected cases other corrective orthopedic surgeries like spinal fusion may also be considered.

Long-term results of scapulothoracic arthrodesis with multiple cable method for facioscapulohumeral dystrophy: do the results deteriorate over time?

Aims: The present study aimed to investigate the long-term functional results of scapulothoracic fusion using multifilament cables in patients with facioscapulohumeral dystrophy (FSHD) to identify if the early improvement from this intervention is maintained. Patients and Methods: We retrospectively investigated the long-term outcomes of 13 patients with FSHD (18 shoulders) in whom scapulothoracic fusion using multifilament cables was performed between 2004 and 2007. These patients have previously been reported at a mean of 35.5 months (24 to 87). There were eight men and five women with a mean age of 26 years. Their mean length of follow-up of our current study was 128 months (94 to 185). To evaluate long-term functional results, the range of shoulder flexion and abduction, Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH) scores were analyzed with a comparison of preoperatively, interim and at the final outcomes. The fusion was examined radiographically in all. Results: The complication rate was 33% (six of 18 scapulothoracic fusions) in 13 patients, which comprised failure of fusion in four shoulders (four patients) all occurring within the first year postoperatively. In two shoulders (one patient) wound problems arose due to attribution from the cables which required shortening but the fusion developed satisfactorily. At the final examination, the mean QuickDASH score and range of movement significantly improved in all but one patient (p < 0.001, p < 0.001 and p < 0.001). In the comparison of 13 patients' mid- and long-term results, the mean QuickDASH score decreased from 9.8 (sd 6.7; 3 to 26) in the third year to 9.1 (sd 5.6; 3 to 22) in the tenth year (p = 0.7); the mean range of shoulder flexion and abduction decreased from 129° (sd 22°; 90° to 160°) and 124° (sd 12; 100° to 150°) at the mid-term to 103° (sd 12°; 80° to 120°) and 101° (sd 8°; 80° to 120°) at the long-term, respectively (p = 0.78 and p = 0.65). Conclusion: Scapulothoracic fusion using a multiple cabling method can confer a considerable improvement in clinical and functional outcomes for most patients with FSHD after a long follow-up period. The technique requires careful execution to avoid complications. Cite this article: Bone Joint J 2018;100-B:953-6.

Surgical correction of hyperlordosis in facioscapulohumeral muscular dystrophy: A case report.

BACKGROUND: Hyperlordosis is common in facioscapulohumeral muscular dystrophy (FSHD), which cannot be controlled by bracing. While the surgical treatment is neither reported nor recommended in previous studies, we report the first corrective surgery for hyperlordosis in one wheelchair-dependent FSHD patient. CASE PRESENTATION: A 15-year-old, wheelchair-dependent girl complaining of hyperlordosis and lower extremity weakness was diagnosed as FSHD. Preoperative examination showed hyperlordosis of 116° with scoliosis of 44°. Posterior correction and bone graft fusion was performed. Postoperatively, the hyperlordosis was corrected to 72° and better sitting posture was gotten. 12 months' follow-up showed favorable correction with improvement in SF-36 and ODI score. CONCLUSIONS: The correction for hyperlordosis in FSHD is controversial. We report the first successful case of operative treatment by corrective spine surgery in these rare and demanding patient collective. Corrective surgery for spinal deformity, such as hyperlordosis and scoliosis, could do some help in posture and improve the quality of life especially in wheelchair-dependent patients.

Long-term results of Letournel scapulothoracic fusion in facioscapulohumeral muscular dystrophy: A retrospective study of eight cases.

BACKGROUND: The aim of this study was to evaluate long-term radio-clinical outcome in scapulothoracic fusion using the Letournel technique (where the fourth rib is passed through the wing of the scapula and cerclage wires are tightened to the two ribs below) for patients suffering from facioscapulohumeral muscular dystrophy (FSHMD), a degenerative pathology affecting scapula-stabilizing muscles, leading to scapular winging, shoulder pain and restricted shoulder abduction and flexion, despite a functional deltoid muscle. METHODS: This retrospective study, with an average follow-up of 14 years (168 months; σ=73 months), included four patients who underwent a bilateral procedure, one side after the other, with shoulder abduction and flexion gain as primary endpoints. RESULTS: Mean improvement in range of motion was 36° (σ=32°) in abduction and 40° (σ=44°) in flexion. Postoperatively, active abduction averaged 99° (σ=28°) and active flexion 110° (σ=45°). In one case, a 58 year-old patient, gain in motion was 50° for abduction and 60° for anterior elevation. In one patient, range of motion showed bilateral decrease due to the natural course of the FSHMD. No intraoperative complications were reported. DISCUSSION: The Letournel technique seems to be an effective long-term solution in FSHMD, without age limit. Improvements appear to be steady over time, the only long-term limitations being the natural course of the disease itself. LEVEL OF EVIDENCE: IV - retrospective case series.

Correction of the Lower Lip With a Cartilage Graft and Lip Resection in Patients With Facioscapulohumeral Muscular Dystrophy.

Facioscapulohumeral muscular dystrophy is an autosomal-dominant, slowly progressive myopathy that involves facial, shoulder, and upper arm muscles. Facial muscle atrophy and weakness leads to drooping of the lower lip, which has unfavorable functional and aesthetic outcomes. While there are several methods to correct drooping of the lower lip, including lip resection, free vascularized muscle transfer, and fascia suspension, there are no reports describing the use of an auricular cartilage graft to elevate the lip. The use of an auricular cartilage graft has several advantages, including a less invasive procedure and a longer-lasting effect. In this study, 3 patients with facioscapulohumeral muscular dystrophy underwent surgery involving an auricular cartilage graft with lip resection to sustain the lower lip, and satisfactory results were obtained. This procedure is simple and minimally invasive; thus, the authors believe that this is an appropriate technique to correct similar patients of lip deformity.

Bimaxillary Osteotomy for Jaw Deformity With Facioscapulohumeral Muscular Dystrophy.

Facioscapulohumeral muscular dystrophy (FSHD) is a subtype of muscular dystrophies which reduces the muscle strength, especially the regions of scapular, shoulder, and upper arms, progressively. According to progressive muscle weakness in FSHD, postoperative stability of patient with FSHD after orthognathic surgery is not reliably acquired same as healthy subjects. A 32-year-old woman with FSHD underwent orthodontic and orthognathic surgical treatment due to jaw deformity. She has been followed up more than 3 years after surgery and acquired skeletal stability. This patient is the first report that showed long-term skeletal stability after orthognathic surgery in patient with FSHD. This patient report suggests that it is possible to apply orthognathic surgical treatment to patients with FSHD.

Human skeletal muscle xenograft as a new preclinical model for muscle disorders.

Development of novel therapeutics requires good animal models of disease. Disorders for which good animal models do not exist have very few drugs in development or clinical trial. Even where there are accepted, albeit imperfect models, the leap from promising preclinical drug results to positive clinical trials commonly fails, including in disorders of skeletal muscle. The main alternative model for early drug development, tissue culture, lacks both the architecture and, usually, the metabolic fidelity of the normal tissue in vivo. Herein, we demonstrate the feasibility and validity of human to mouse xenografts as a preclinical model of myopathy. Human skeletal muscle biopsies transplanted into the anterior tibial compartment of the hindlimbs of NOD-Rag1(null) IL2rγ(null) immunodeficient host mice regenerate new vascularized and innervated myofibers from human myogenic precursor cells. The grafts exhibit contractile and calcium release behavior, characteristic of functional muscle tissue. The validity of the human graft as a model of facioscapulohumeral muscular dystrophy is demonstrated in disease biomarker studies, showing that gene expression profiles of xenografts mirror those of the fresh donor biopsies. These findings illustrate the value of a new experimental model of muscle disease, the human muscle xenograft in mice, as a feasible and valid preclinical tool to better investigate the pathogenesis of human genetic myopathies and to more accurately predict their response to novel therapeutics.

The outcome of scapulothoracic arthrodesis using cerclage wires, plates, and allograft for facioscapulohumeral dystrophy.

BACKGROUND: Scapulothoracic arthrodesis is a recognized treatment for impaired shoulder function in patients with facioscapulohumeral dystrophy (FSHD) and is traditionally performed with autograft. The purpose of the study was to report our experience with scapulothoracic arthrodesis in patients with FSHD using allograft, rather than autograft, with particular respect to the effect of fusion on preoperative and postoperative Disabilities of the Arm, Shoulder and Hand (DASH) scores; forced vital capacity (FVC); and complications. MATERIALS AND METHODS: The early results of 14 consecutive scapulothoracic arthrodeses in FSHD patients with cerclage wires, plates, and allograft (fresh-frozen femoral heads) are reported. DASH scores were recorded preoperatively and 6 months postoperatively. Preoperative and 6-month FVCs were compared. The surgical technique is described. RESULTS: Eleven patients underwent 14 fusions. The mean follow-up period was 29 months (range, 6-50 months). Forward flexion improved from 70° to 115° (P = .001) and abduction from 68° to 109° (P = .007). The DASH score improved from 48 points to 34 points (P = .005). FVC decreased from 98% to 92% of predicted (P = .021), although this was not clinically significant. One patient required revision for nonunion, and metalwork was removed in 5 scapulae. A postoperative chest infection developed in 1 patient and a pleural effusion in another. One brachial plexus palsy occurred, which had almost completely resolved by 27 months postoperatively. CONCLUSION: Scapulothoracic arthrodesis can be performed successfully with allograft. The nonunion and complication rates are similar to those in the existing literature. A small decrease in FVC does occur but not to a clinically significant level.

Medium to long-term outcome of thoracoscapular arthrodesis with screw fixation for facioscapulohumeral muscular dystrophy.

BACKGROUND: Shoulder girdle muscle weakness is the most constant feature of facioscapulohumeral muscular dystrophy and leads to scapular winging. Mechanical fixation of the scapula to the thoracic wall provides a stable fulcrum on which the deltoid muscle can exert its action on the humerus. The aim of this study was to evaluate the medium to long-term outcome of thoracoscapular arthrodesis with screw fixation (the modified Howard-Copeland technique). METHODS: All patients with facioscapulohumeral dystrophy who underwent thoracoscapular arthrodesis with screw fixation and bone-grafting from July 1997 to July 2010 were retrospectively reviewed. Preoperative and postoperative clinical assessment included active shoulder elevation, the Constant score, a patient satisfaction score, and cosmetic satisfaction. Union was determined both clinically and radiographically. RESULTS: Thoracoscapular arthrodesis was performed in thirty-five shoulders in twenty-four patients; eleven patients underwent bilateral procedures. The principal study group consisted of thirty-two shoulders in twenty-one patients with a minimum follow-up of twenty-four months (mean, eighty-eight months; range, twenty-four to 174 months). The mean Constant score increased from 30 (range, 17 to 41) preoperatively to 61 (range, 30 to 90) postoperatively. The mean satisfaction score increased from 1 (range, 0 to 4) to 8.4 (range, 4 to 10). Early complications consisted of one pneumothorax, one superficial wound infection, and four early failures, two of which were associated with noncompliance with the postoperative regimen. Late complications consisted of one posttraumatic fracture resulting in loosening and one painful nonunion; both were treated successfully with revision. CONCLUSIONS: Thoracoscapular arthrodesis with screw fixation prevented scapular winging and improved short-term and long-term shoulder function in patients with facioscapulohumeral dystrophy. LEVEL OF EVIDENCE: Therapeutic level IV. See instructions for authors for a complete description of levels of evidence.

Scapular winging: an update.

Scapular winging is a rare disorder often caused by neuromuscular imbalance in the scapulothoracic stabilizer muscles. Lesions of the long thoracic nerve and spinal accessory nerves are the most common cause. Numerous underlying etiologies have been described. Patients report diffuse neck, shoulder girdle, and upper back pain, which may be debilitating, associated with abduction and overhead activities. Accurate diagnosis and detection depend on appreciation of the scapulothoracic anatomy and a comprehensive physical examination. Although most cases resolve nonsurgically, surgical treatment of scapular winging has been met with success.