Scapulothoracic fusion for fascio-scapulo-humeral dystrophy

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  • #73537
    Scapulothoracic fusion for fascio-scapulo-humeral dystrophy

    Just thought I would post the most up to date article I could find on this procedure. Published Jan 2011.

    http://onlinelibrary.wiley.com/doi/10.1111/j.1758-5740.2010.00103.x/full

    Background Scapulothoracic (ST) fusion has been recommended for suitable patients suffering from FacioScapuloHumeral Dystrophy (FSHD). It helps in creating a fulcrum for the deltoid and prevents winging of the scapula during flexion and abduction of the arm, thus improving activities of daily living. We present here an overview of the techniques used and the results of 9 ST fusion carried out at our department.

    Method The scapula is fixed to the underlying rips using Luque wires around the 2nd to 6th ribs, then passed through the medial border of the scapula, then through an 8 hole semi tubular plate placed over the dorsal aspect of the whole medial border of the scapula. After which the Luque wires are tied firmly, locking the scapula onto the chest wall. Morsellised bone allograft is placed between the scapula and the ribs before tightening the wires.

    Results In total 9 ST fusions were carried out. No Intra-operative complications occurred but 2 patients developed haemothoraces post-operatively, one resolved with chest drain and one needed thoracotomy. Late complications included one scapula fracture (subsequently internally fixed), one frozen shoulder (resolved with physiotherapy) and one protruding plate (subsequently removed). An average of 40 degrees of additional abduction was achieved and overall good patients satisfaction.
    Conflicts of interest

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

    None declared
    INTRODUCTION

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

    Fascio-scapulo-humeral dystrophy (FSHD) is a rare autosomal dominant disease affecting 7 in 1,000,000 [1] of the population, with almost full penetrance by the age of 20 years [2]. It selectively affects the muscles of the face and the shoulder and particularly those that stabilize the scapula onto the chest wall of the scapulothoracic joint. FSHD invariably spares the muscles acting on the glenohumeral joint, such as the deltoid and rotator cuff muscles [3–7]. Contracting the glenohumeral muscles during abduction or flexion of the arm results in a significant rotation and winging of the scapula and thus severely limits abduction of the arm [3–5,8,9]. Therefore, patients who suffer from FSHD face many difficulties when performing activities of daily living such as combing their hair, shaving or reaching up to shelves [3–5,10].

    Stabilizing the scapula onto the chest wall creates a fulcrum for the deltoid and other glenohumeral joint muscles. This significantly enhances the patient’s ability to abduct and flex the arm. This not only helps with activities of daily living, but also assists the patient with their ambulation when they require walking aids as the disease progresses.

    Scapulothoracic (ST) arthrodesis (fusion) has been successfully used to stabilize the scapula in patients with severe winging of the scapula and impaired function [3,4,6–8,10–17]. It has been shown to significantly improve the strength of the deltoid muscle, resulting in almost full return to simple tasks of daily living such as reaching for shelves dressing and coming hair [4,8,10,16]. Many studies report a significant improvement in abduction averaging between 90° and 120°[7,12,13] and a relatively recent study demonstrated that an abduction of almost 150° can be achieved following ST fusion [3]. Long-term follow-up results reported by Copeland et al. [4,10] and Rhee et al. [16] show a satisfactory level of function and an average abduction of 103° and 109°, respectively. Many patients were satisfied with the overall functional and cosmetic outcome [8,10,16]; some would have the same surgery again and would also recommend it to others [8]. With time, the strength of the deltoid can progressively weaken in some patients with the generalized deterioration of the disease resulting in fatigability and a loss of shoulder motion despite the rigidity of the fusion [3,4].

    ST fusion has also been carried out for other conditions such as painful winging of the scapula long thoracic nerve palsy and spinal accessory nerve palsy [8,14,18].

    An alternative surgical procedure to ST fusion is soft tissue scapuloplexy, which involves fixing the scapula to the thoracic wall without fusion using variety of materials such as facia lata bands [19,20] and wires [5]. This has been heralded on occasions with a loosening and deterioration of function over the long term [4,19]. Traditional orthotic devices were also used but these were poorly tolerated and of limited effectiveness.

    Here, we present some of the different techniques used to carrying out ST fusion and fully describe our adopted technique together with the results achieved.
    TECHNIQUES OF ST FUSION

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

    Different techniques of ST fusion have been described in the literature. The surgical approach and the dissection method are similar, although they mainly differ in the way that the scapula is fixed and fused to the thoracic wall.

    The simplest methods described so far for ST fusion involve using a combination of screws or wires [4,10,12,13,15], fixing the scapula directly to the underlying ribs with bone graft from the iliac crest.

    Bunch et al. used two wires for each rib: one passing through the scapula near the medial border and the other near the lateral border of the scapula [15]. This achieved good fusion and improvement in function. Howard and Copeland used a method of indirectly fixing the scapula to the underlying ribs with segments of tibial and iliac crest bone graft using screws [10]. Subsequently, a different method was used by the authors involved the scapula being directly fixed to the underlying ribs using screws with a morsellized bone graft sandwiched between the scapula and the ribs [4].

    A slightly more complicated and totally different technique was used by Letournel et al. [7] and later on by Berne et al. [11]. This successful fusion technique involved osteomizing and transfixing the upper most rib (underlying the infraspinatus fossa) through a window cut in the medial third of the scapula. The two ends of the rib were then fixed with a plate. Two other inferior ribs were also fixed to the scapula using two two-hole plates for each rib. One plate was positioned on the anterior surface of the rib and the other was positioned parallel to it on the posterior surface of the scapula [7,11].

    The technique of using a plate along the medial border of the scapula has been used by several authors [3,6,8,14,16]. Roland et al. [14] describe a technique using a four- or five-hole 4.5 mm reconstruction plate contoured over the medial border of the scapula in the infraspinatus fossa. This was held by wires threaded around the underlying ribs, through holes made in the scapula and into the corresponding holes in the plates. No plates were used in the suprspinatus fossa; instead, a wire was tied directly around the underlying rib and through a hole in the scapula. Diab et al. [3] and Ziaee et al. [6] used a two plate technique. One plate was placed cephalad and the other distal to the scapula spine.
    Preoperative assessment

    Candidates contemplating ST fusion must have severe symptoms of scapula winging resulting in impaired function. The patient must be assessed to make sure that an improvement in function will be gained from this procedure. This is usually carried out using the Copeland and Howard fatigability test [10]. The patient is asked to raise their arms and hold them abducted until fatigue causes them to drop. The clinician then manually stabilizes and holds the scapula against the chest wall to prevent winging while the patient repeats the same procedure. The patient would be expected to benefit from ST fusion if there was improvement in the height of abduction and the duration of fatigability.

    Other relative indications include cosmoses, especially in females where the severe winging of the scapula results in cosmetic deformity and hinders the wearing of garments with shoulder straps [4].

    It is mainly contraindicated in patients with a nonfunctioning deltoid or axillary nerve palsy [14,19] or in those with severe respiratory disease [14]. The respiratory function might deteriorate especially in some bilateral fusions; however, many authors report this deterioration to be clinically insignificant [7,13,16], with a tendency to be associated with a generalized deterioration in the disease [7,11,15].
    MATERIALS AND METHODS

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

    Patients

    Between 2002 and 2009 a total of nine ST fusions were carried out in eight patients suffering from FSHD. The diagnoses were confirmed genetically in all patients. All patients were mobile but had had various disabilities as a result of their FSHD. The operations were performed by the senior surgeon (W.A.W.).
    Surgical technique

    Recently, a technique for ST fusion was described by this department in which a Rush Pin (Rush Pin, LLC, Meridian, MS, USA) was used to reinforce the medial border of the scapula in the treatment of painful winging of scapula in nondystrophic patients [18]. In that procedure, the Rush Pin was contoured to fit the dorsum surface of the scapula with the hook lying over and above the scapula spine. Here, we propose the use of plate (instead of the Rush Pin), although the principles remains the same (Fig. 1).

    Figure 1. Schematic drawing showing the position of the plate on the scapula.
    image
    Positioning of the patient

    The patient is placed prone on the operating table under general anaesthesia. The arms are placed symmetrical with the elbows flexed at 90° and the hands at the side of the head to assist in the positioning of the scapula during fixation.
    The incision and dissection technique

    A paramedian incision is made along the medial border of the scapula and the parasaggital muscles are dissected. Usually the parasaggital muscles and other muscles, such as the rhomboid muscles and the levator scapulae, are atrophied and transformed to fat. Some 2 cm to 3 cm of the medial attachments of supraspinatus and infraspinatus to the scapula are cleared and the medial border of the scapula is lifted and retracted laterally to reveal the underlying ribs and the anterior surface of the medial aspect of the scapula (Fig. 2). The attachment of the serratus anterior is detached from the anterior surface of the medial aspect of the scapula, which is then cleared and decorticated with burr. This part of the scapula is thin and care must be taken not to over do the roughening of the scapula. The underlying five ribs, usually from the second to the sixth, are exposed. Approximately 3 cm to 4 cm laterally from the tubercle of the rib, the posterior surface of the ribs is also cleaned and decorticated with burr (Fig. 3). This dissection technique is similar to what has already been reported from our department [18] as well as by other authors [3,8].

    Figure 2. The surgical exposure.
    image

    Figure 3. Burr is used to decorticate the surface of the scapula and ribs.
    image

    A vertical hole through the spine of the scapula is made approximately 1 cm to 2 cm from the medial border of the scapula using a 4.5-mm drill. A semi-tubular plate (usually a seven- or eight-hole plate) is inserted from below, through the hole in the medial scapula spine to lie against the length of the medial scapula border. The medial border of the scapula is positioned at an angle of approximately 15° from the median vertebral line. This has been suggested to be the optimum position to achieve good abduction and avoid traction on the brachial plexus [5,12,13,15,21,22]. Holes are drilled via the plate holes through the scapula (along the medial border of scapula) in line with the underlying ribs. Luque wires are doubled and the bent end is then passed subperiosteally around the ribs, making sure not to puncture the pleura. One end of each double wire is then passed into its corresponding hole in the medial border of the scapula and then through the related plate hole (Figs 1 and 2). This procedure should be repeated with another three to four ribs (Fig. 4). A morsellized allograft, obtained from a femoral head, is then packed between the decorticated anterior surface of the scapula and the ribs. The rest of the bone graft is placed between the medial border of the scapula and the midline. The wires are then tightened using the usual ‘pull, rotate and relax’ technique and the excess is trimmed from the twisted knot (Fig. 5). The knots are then bent and punched towards the chest. A routine wound closure is then carried out without drains.

    Figure 4. Positioning of the plate and wires.
    image

    Figure 5. The final positioning of the plate on the scapula.
    image
    Postoperative care and follow-up

    The vital observations are monitored and a postoperative chest X-ray is obtained to check for any signs of pneumo- or haemothorax (Fig. 6). The neurovascular status of the ibsilateral arm is checked and the patient can be discharged when pain is controlled. The patient is immobilized in a polysling and reviewed in 4 weeks to 6 weeks. Following this, pendulum exercises and active movements are commenced and the patient is reviewed again 4 months to 6 months later. If no concerns and no more treatment is planned at this stage, the patient is usually discharged from the clinic.

    Figure 6. Postoperative X-ray.
    image
    RESULTS

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

    Nine operations were carried out on eight patients (Table 1). One patient had bilateral ST fusions at different times. The average age of the patients at surgery was 35.4 years (range 21 years to 58 years) with an average follow-up of 17 months (range 4 months to 48 months). Because patients were usually discharged after 4 months to 6 months if no new concerns or further treatment was planned, our follow-up period is relatively short. All patients were satisfied with their final results at the last follow-up. There was an average improvement in abduction of almost 40°. One patient did not attend any of his follow-up appointments despite multiple invitations.
    Table 1. Characteristics of patients and the results of operation Patient number/gender Side Age at operation Length of stay (days) Abduction before operation Abduction after operation Patient satisfaction Early complications Late complications Length of follow-up
    1/Male Right 58 3 45 120 Satisfied Nil Fracture of scapula 22 months
    2/Male Left 31 5 Lost to follow-up Lost to follow-up Lost to follow-up Postoperative pain Lost to follow-up —
    3/Male Left 21 5 70 100 Satisfied Nil Nil 4 months
    4/Male Left 48 5 60 90 Satisfied Nil Stiff shoulder 7 months
    5 and 6/Female Right 22 6 60 100 Satisfied Nil Nil 4 years
    Left 23 5 60 90 Satisfied Nil Protruding plate. Plate removed 3 years
    7/Male Left 35 5 — 90 Satisfied Nil Nil 6 months
    8/Female Right 56 31 60 100 Satisfied Heamothorax needing thoracotomy Nil 8 months
    9/Female Right 25 17 60 90 Satisfied Heamothorax and postoperative pain Pain. Plate removed 8 months
    Average 35.4 9.1 59 98 17.4

    We did not measure the respiratory function of any of the patients either pre-operatively or postoperatively, although none of the patients had any objective respiratory complications or symptoms prior to surgery or at the end of follow-up.
    Complications

    No intra-operative complications occurred but, postoperatively, two patients developed heamothoraces. One was successfully treated with a chest drain but the other needed thoracotomy after failing to resolve with radiologically guided chest drain. Both patients did not have any clinical respiratory compromise at follow-up.

    Two patients complained of severe pain not controlled by simple analgesia post surgery. The pain team were involved and the pain gradually resolved in one patient. The other patient’s (number 9 on the table) symptoms persistent for few months following surgery, compromising shoulder physiotherapy. This necessitated exploration of the fusion to check for failure. The fusion was found to be sound and the plate was removed. Although we were uncertain of the cause of the pain, the patient’s pain and movement improved significantly postoperatively.

    One patient initially had a significant improvement in shoulder abductions 6 weeks post surgery but then suddenly deteriorated after attending their follow-up appointment 5 months later. Radiographs did not reveal any abnormalities and it was initially considered that the fusion had failed. The operation site was re-explored with the intension of revising the fusion but a fracture was found in the scapula just distal to the fusion, which had not been evident on the pre-operative radiographs. The fusion was solid and intact. The fracture was fixed using screws and bone graft onto the fusion plate and the shoulder movement subsequently improved. We consider this to be a stress related fracture as a consequence of over use of the shoulder in the immediate postoperative period.

    The patient who had bilateral fusion had a protruding plate after her second operation, which caused discomfort. At the time of plate removal, the fusion was found to be sound and the removal of the plate solved the problem.

    One patient developed a stiff shoulder 6 weeks following surgery, although this also resolved with physiotherapy.
    DISCUSSION

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

    In this report, we have presented one technique for scapluthoracic fusion. It differs slightly from other plating techniques in two ways; however, the main principles remain the same. First, we used a single plate penetrating the spine of the scapula and therefore reinforcing the whole medial border of the scapula. This distributes tension uniformly over the whole length of the medial border and avoids the risk of wires cheese-wiring through the scapula during tightening or during the recovery period. We consider that, by using the whole medial border of scapula, we achieve a better biomechanical fixation compared to using a short segment [12]. Second, we used femoral head allograft, which avoids a second operation to harvest the bone graft from the iliac crest with the risk of donor site morbidity. The complications encountered were similar to those reported in the literature.

    During the passage of wire around the ribs, there is a risk of penetrating the pleura or dissecting the intercostal vessels leading to pneumo- or heamothoraces. This is why we emphasize on passing the curved end of the Leque wire subperiostally around the anterior surface of the rib to minimize the risk of penetrating the pleura or catching the intercostal vessels. Some authors advocate the use of double lumen tracheal tube to allow deflation of the ibsilateral lung during the passage of wires [8]. When drilling the holes through the scapula, we place a lead plate underneath the anterior surface of the scapula to prevent the drill from overshooting and penetrating the thoracic cavity. From the nine procedures, heamothoraces occurred in the last two patients.

    The fracture of the scapula resulted from over zealous use of the arm after the improvement gained in its function after surgery. In addition, that part of the scapula is known to be very thin [23] and decorticating it during surgery adds to the risk of fracture. Lateralization of the fusion to reduce the risk of scapula fracture is difficult because of the risk of injuring the nerve to the subscabularis [8]. Also fusing the ribs more laterally might reduce the respiratory compliance.

    In most of the studies published, all ST fusion patients were immobilized postoperatively [3,6–8,10–15] for a period of 3 weeks to 12 weeks. This will inherently increase the chances of stiff shoulder, although this improves with physiotherapy. Early active mobilization of shoulders straight after surgery was reported in one study [12]. However, from the six patients described in that study, one had non-union needing revision, one patient had stress rib fractures, and three patients suffered wire breakages.

    Removal of symptomatic hard ware is well known post ST fusion [4,7,8]. In one patient, the protrusion of the plate could have been avoided by choosing a smaller plate. In the other patient, pain and movement resolved after the removal of the plate but we were uncertain whether the plate was the true cause for the pain.

    We did not measure the respiratory function of patients before hand but, clinically, we did not have any objective or symptomatic deterioration in patient breathing following recovery from surgery. As stated previously [18], we consider that ST fusion in patients with normal lung function can be safely carried out. However, patients with preoperative respiratory deterioration will need objective respiratory assessment before the operation.

    The limiting factor in all of the studies addressing this topic is the small number of patients as a result of the rarity of the disease and the small number of patients undergoing ST fusion.
    REFERENCES

    1. Top of page
    2. Abstract
    3. Conflicts of interest
    4. INTRODUCTION
    5. TECHNIQUES OF ST FUSION
    6. MATERIALS AND METHODS
    7. RESULTS
    8. DISCUSSION
    9. REFERENCES

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    wingedwarrior
    Participant
    Posts: 67
    Joined: 10/01/2011
    #79329
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    Thanks for that wingedwarrior, very interesting. Its good to have the information available on the potential complications and what the benefits are so an informed choice can be made.

    kelvidge
    Participant
    Posts: 113
    Joined: 07/09/2010
    #79330
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    thanx very much for this information it has come in very usefull, it is very nerve racking reading about the facts of fshd is there ne local campains that you know about in leicester? ….

    larissa
    Participant
    Posts: 24
    Joined: 12/10/2011
    #79331
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    @larissa wrote:

    thanx very much for this information it has come in very usefull, it is very nerve racking reading about the facts of fshd is there ne local campains that you know about in leicester? ….

    The whole subject will be rather overwhelming at times.

    There are a series of Regional Care Advisors up and down the country, who are
    specialists in all aspects of MD. Myself and many others have greatly benefitted from
    contact with them especially during the early stages. Thet can solve problems
    you do not even know you have.

    The one who covers Leicestershire is based in Nottingham. Jane Cassell :-

    http://www.muscular-dystrophy.org/how_we_help_you/care_and_support/care_advisors/1221_nottingham

    Self referral is OK, you do not need to go through your GP.

    "Even if you are not paranoid, it does not mean they are not out to get you!".

    taungfox
    Participant
    Posts: 4,630
    Joined: 27/09/2010
    #79332
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    i also wanted to know how the scapular fusion will effect day 2 day livin if you were to have children?……..

    larissa
    Participant
    Posts: 24
    Joined: 12/10/2011
    #79333
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    @larissa wrote:

    i also wanted to know how the scapular fusion will effect day 2 day livin if you were to have children?……..

    Hi Larissa
    I also want to know the same things you type above
    it is really important to understand evertying before we consume it especially for our children

    charlie01
    Participant
    Posts: 3
    Joined: 03/02/2012
    #79334
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    hi charlie…do u suffer wiv fshd? i got diagnose about 3 ys ago and now i am on the waitin list for the scapular fusion i have had some positive feed bck onit and i am now tryin to findout everything i cn b4 the big day comes…have u heard of the operation or had it done…i have tryed to get into contact with people tht have had it one buh it proves to be just takin too long nd i need to know sooner rather thn l8r lol…douno of anybody hu has had the operation done tht has kept a week too week diary of what hapends nd how they delt with it…cuz i wud like to know bthe ins and outs of it nd all i cn find is what they are goin to do buh not wat hapens througout recovery? thanks for replyin to my post :D

    larissa
    Participant
    Posts: 24
    Joined: 12/10/2011
    #79335
    Re: Scapulothoracic fusion for fascio-scapulo-humeral dystro

    have you ever heard of anybody having it dione with tape instead of wires?

    larissa
    Participant
    Posts: 24
    Joined: 12/10/2011
    #192116
    Reply To: Scapulothoracic fusion for fascio-scapulo-humeral dystrophy

    Hello I have had both my arms done if you would like to no anything I can try and help
    I have Fshd 😊

    Lizzylilly
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