Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular hereditary disease after Duchenne muscular dystrophy and myotonic dystrophy.32 First described by Landouzy and Dejerine in 1884 and later studied by others,10,11,15,20,25,32,34 FSHD consists of an atrophic myopathy with predominant involvement of the face, upper arms, and shoulder muscles. It may extend to the trunk and pelvic girdle muscles, producing a lordotic posture that may progress to loss of ambulation later in life.10,20,25,28
Facioscapulohumeral muscular dystrophy produces severe consequences in the function and biomechanics of the shoulder, whose movements are produced by the synergetic work of two joints. The first 90° abduction of the arm occurs prevalently at the glenohumeral joint, and the remaining abduction occurs at the scapulothoracic and glenohumeral joints in a 1:2 ratio.2,17,31 Facioscapulohumeral muscular dystrophy compromises the muscles of the scapulothoracic joint (serratus anterior, rhomboids, trapezius, teres major, teres minor, and elevator scapulae) and usually spares glenohumeral joint muscles (deltoid, supraspinatus, infraspinatus, and subscapularis).15 When patients with FSHD try to abduct or forward flex the shoulder, the contraction of the preserved glenohumeral muscles produces an abnormal rotation of the scapula. The scapula is not held to the rib cage because of insufficient scapulothoracic muscles, resulting in a winged scapula and limited arm motion (Fig 1). This prevents patients from performing activities of daily living (ADL) such as combing hair, shaving, and brushing teeth.7,8
Different nonoperative and surgical techniques have been used to correct winged scapulae secondary to FSHD. Various orthotic devices have been described to fix the scapula externally against the posterior thoracic cage.3 In 1995, a molded spinal jacket for treatment of a 14-year-old patient with FSHD was described.3 The patient had improved shoulder function, but no long-term followup results or cosmetic improvements were reported.3 The surgical correction seems reasonable as patients with FSHD have a long life expectancy.10,20 The first surgical procedures were described in 189836 and 1906.29 The current surgical techniques for patients with FSHD can be classified into: arthrodesis of the scapula to the thorax (scapulodesis)1,4-8,16,18,21,22,33,35 and fixation of the scapula to the thorax without arthrodesis (scapulopexy).9,12,19,30,37 Although scapulodesis is widely used, many complications have been reported.1,4,6-8,18,22,33,35 There are few reports concerning scapulopexy for FSHD, but with few patients and short-term followups12,19,30 (Table 1).
An alternative method of scapulopexy to stabilize the scapula to the ribs using metal wires was described by Giannini et al in 1992.12 We proposed this technique would improve shoulder range of motion (ROM) and muscular strength, and evaluated whether fixing the scapula over the rib cage by metal wires without arthrodesis could achieve a stable and long-lasting scapulothoracic fixation. We also assessed if the procedure would improve cosmetic and functional satisfaction.
MATERIALS AND METHODS
We retrospectively reviewed nine patients (18 procedures) with FSHD who were treated by scapulopexy from 1983-2000. We compared our patients' functional and cosmetic results and complications with those of historical control groups. We included patients with winged scapulae and FSHD. The diagnosis of FSHD was made according to family history, disease onset in facial or shoulder girdle muscles with sparing of the extraocular, pharyngeal, and lingual muscles as confirmed by electromyography (EMG), muscular computed tomography (CT), estimation of creatine kinase activity, and muscular biopsy. All patients had shoulder drop, anteposition, and scapular winging. Genetic analyses confirmed the diagnosis in six patients examined after 1992. We excluded patients who could not walk, patients in poor general condition, and patients with poor deltoid and supraspinatus function. Patients were also excluded if they had a negative Horwitz maneuver.4,14 Instead, these patients were considered for physiotherapy. During the Horwitz maneuver,4,14 the patient tries to abduct or forward flex the shoulder while the examiner presses the scapula against the thoracic wall. If the deltoid and supraspinatus muscles are spared, the patient is able to perform these movements and the maneuver is positive (Fig 2). When the shoulder muscles are involved, the patient is unable to abduct the shoulder even when the physician holds the scapula in the correct position (negative Horwitz maneuver); surgery would not improve shoulder ROM. Two patients were excluded from the study; they had physiotherapy but no surgery. The series included three male patients and six female patients. The mean age of the patients at the time of diagnosis of myopathy was 22 years (range, 13-35 years). The average age at surgery was 25.2 years (range, 15-35 years). All patients were checked at a minimum followup of 3 years.
The preoperative examination consisted of measuring shoulder ROM in abduction and flexion (Table 2). We performed the Medical Research Council (MRC)13,24 test of motor power during the Horwitz maneuver to determine muscle strength by rating isolated muscle groups responsible for abduction and flexion of the shoulder on 0-5 point scale (Table 3).10,13,24 All patients presented with limited activities of daily living (ADL) from shoulder instability or weakness, but none had pain. Radiographs of both scapulae were taken in two views. Preoperative functional pulmonary tests were performed in six patients.
All patients were treated under general anesthesia in the prone position. Both shoulders were treated in the same surgical stage. To place the scapula in the neutral position, the patient's arms were placed with the shoulder 80° abducted, 20° flexed, and externally rotated. The winged scapula usually disappeared in this position. If a residual deformity was observed, it was corrected by applying a spacer between the bed and the shoulder to press the scapula more medially. When the scapula was in the correct position, its medial border formed a 15°-20° angle with the line connecting the spinous processes.
We performed a skin incision on the medial border of the scapula. The trapezius, rhomboid, or their remains, were detached from the medial border of the scapula. The supraspinatus, the infraspinatus, the subscapularis, and the serratus anterior muscles were detached from the medial border of the scapula to expose approximately 2 cm of bone. Four ribs starting from the fourth or the fifth were exposed in the region under the scapula and a doubled metal wire (1 mm diameter) was passed under each rib. The scapula was positioned over the rib cage in its definitive position, and four 2.8-mm holes were drilled approximately 1.5 cm from the medial border of the scapula. Each hole was made just above the four underlying ribs, and the metal wires were threaded through the holes to anchor the scapula to the rib cage (Fig 3). With the scapula held in position, the metal wires were tightened, and bone to bone contact of the scapula and the ribs was obtained. The wound was closed routinely, and radiographs were taken. The mean surgery time was 65 minutes (range, 35-90 minutes).
A figure-of-eight dressing holding the shoulders back was applied immediately after surgery and maintained for 6 weeks. Physiotherapy of the scapulohumeral joint was started as soon as tolerated to regain full glenohumeral movements and strengthen the deltoid muscle. Patients were discharged from the hospital 4-6 days postoperatively.
All patients were followed monthly for 3 months, then yearly. We evaluated shoulder ROM in abduction and flexion. These functional results and any complications were compared with results and complications reported in the literature. We performed the MRC test of motor power (for the same muscle groups examined preoperatively) 1 year postoperatively and at the last followup. Radiographs were taken 1 year postoperatively and at the last followup. Radiographic examination included comparing the position of the scapula on the thoracic wall postoperatively and at the last followup. All patients were asked to state their subjective satisfaction regarding cosmetic and functional results according to a visual analog scale as very satisfied, satisfied, fairly satisfied, or unsatisfied.
We used SPSS software (version 9.0, SPSS Inc., Chicago, IL) for statistical analyses. All continuous data were expressed as mean and standard deviation (SD). The Wilcoxon nonparametric test was used to calculate the differences between the values of abduction and flexion of the arm before surgery, at 1 year followup, and at last followup. The least significant difference non-parametric test was used to calculate the differences between the values of muscle strength before surgery, at 1-year followup, and at last followup. The results were evaluated by the Monte Carlo method for small samples.26 For all tests, p < 0.05 was set as significant.
All patients had improvement in active ROM (Table 2). At 1 year followup, arm abduction increased (p = 0.005) from 68.3° (SD = 19.2) preoperatively to 96.1° (SD = 16.7) postoperatively (Fig 4). Arm flexion increased (p = 0.003) from an average of 57.2° (SD = 16.6) preoperatively to 116.1° (SD = 31.4) postoperatively (Fig 5). At an average followup of 9.9 years (range, 3-16 years), arm abduction decreased from 96.1° (SD = 16.7) to 90° (SD = 16.7), whereas arm flexion decreased from 116.1° (SD = 31.4) to 103° (SD = 30.6). The MRC test showed that at the 1-year followup muscle strength during arm abduction increased (p < 0.01) from 2.6 (SD = 0.5) preoperatively to 4.5 (SD = 0.5) postoperatively. Arm flexion increased (p < 0.01) from an average of 2.4 (SD = 0.5) preoperatively to 4.5 (SD = 0.5) postoperatively. The MRC test performed at an average followup of 9.9 years (range, 3-16 years) showed muscle strength decreased. The mean value of muscle strength was 4.1 (SD = 0.8) during arm abduction and 4 (SD = 0.5) during arm flexion (Table 3).
Fixing the scapula over the rib cage by metal wires without arthrodesis achieved a stable and long-lasting fixation. Radiographs showed the position of the scapula obtained by surgery was maintained at the last available followup (Fig 6). Two patients had one of the eight wires break at 3 years and 7 years, respectively. However, the scapula remained well fixed to the thorax, and the patients maintained shoulder ROM observed in previous controls.
All patients were very satisfied with their cosmetic outcomes. The winged scapula was resolved in all patients, and the shoulder regained a normal shape. Eight patients stated they were very satisfied with the functional results and one patient was satisfied. There were no major surgical complications except for one patient with unilateral pneumothorax 1 day postoperatively which resolved spontaneously in 48 hours. The six patients who had lung function testing preoperatively repeated it at 1 year followup and showed no significant difference of vital capacity.
Surgical treatment of winged scapula secondary to FSHD by scapulopexy to stabilize the scapula to the ribs using metal wires improved ROM and muscular strength in all patients. The correction was maintained with considerable cosmetic and functional satisfaction.
A winged scapula secondary to FSHD is relatively rare,10,11,20,34 therefore the number of patients included in the study was small and there was no control group. We compared our data with that in the literature (Table 1). Surgery was performed in patients with a wide range of ages and different neurologic statuses. Facioscapulohumeral muscular dystrophy is an autosomal-dominant disease with a complete penetrance but variable expressivity so there may be a spectrum of mild to severe cases.28 The course of FSHD is slowly progressive, although many patients have a long period of relatively stable function.10,20,28 For these reasons, clinical evolution of the FSHD after surgery varies, and analysis of the functional results with time may be influenced by involvement of the deltoid muscle by FSHD.
Surgical fixation of the scapula to the thorax in patients with FSHD may be accomplished by scapulodesis or scapulopexy. Both are useful techniques for correcting winged scapulae and improving shoulder ROM.1,4-9,12,16,18,19,21,22,27,30,33,35,37
Some authors1,4-8,16,18,21,22,33,35 reported scapulodesis as an effective but demanding procedure because the scapula and ribs are flat bones. This technique resulted in satisfactory clinical results, with considerable improvements in ROM and patient satisfaction.1,4-8,16,18,21,22,33,35 However, complications have been described in some patients with scapulodesis such as hemothorax, pneumothorax, scapular or rib fractures, and decreased lung function.1,4-8,16,18,21,22,33,35 Some authors also have reported nonunions.4,7,8,22,35 (Table 1).
Whitman37 described a scapulopexy in which the vertebral border of the scapula was fixed to the spinous processes of the thoracic vertebrae for correction of a congenital elevation of the scapula. Rinaldi30 used scapulopexy, as described by Whitman, for correction of FSHD. Later, Ketenjian19 described a scapulopexy using fascia lata grafts, Mersilene, or Dacron strips tied around the ribs through holes drilled in the medial border of the scapula. Ketenjian also compared preoperative and postoperative ROM in a small series; there were no major postoperative complications such as rib fractures, pneumothorax, hemothorax, or nerve injuries (Table 1). Mummery et al, in a literature review, stated that scapulopexy with fascial slings deteriorates with time.27
The scapulopexy we used by tightening the scapula to the rib cage was described by Jakab and Gledhill18 to perform scapulodesis. This kind of fixation allowed extensive bone-to-bone contact between the ribs and the scapula obtained by firm tightening of four metal wires. All patients had considerable improvement of shoulder ROM in flexion and abduction. The muscle strength in flexion and abduction improved 1 year postoperatively because the stable scapulothoracic joint made the muscles of the glenohumeral joint work more efficiently. The scapulopexy seemed stable with no signs of recurrence at a mean of 9.9 years, but slight decreases of muscle strength in flexion and abduction were observed in two patients, presumably from progression of the FSHD. Surgery was still beneficial for these patients because they achieved greater postoperative functional results. No patient had additional surgery. This technique does not require grafts and reduces postoperative complications such as pneumothorax or hemothorax. We suspect fibrotic scar tissue stabilizes the correction and micromotion (responsible for fatigue breakage of the metal wires) between the scapula and ribs.
The correct position for affixing the scapula to the thoracic wall to obtain the most effective benefit is still controversial. If the scapula is fixed in an adducted position, the function of the glenohumeral joint is limited and abduction of the shoulder cannot improve with surgery.19 If the scapula is positioned in an abducted position, it allows the patient to move the glenohumeral joint through more functional ROM. The rotation of the scapula improves the mechanical advantage of the supraspinatus and deltoid.19 However, the shoulder appears abnormally high if the scapula is excessively abducted or rotated. If the scapula is positioned too far laterally, its relation with the ribs will be incongruous to ensure stable fixation. If the scapula is positioned too caudally, it can stretch the nerves of the brachial plexus.1,4-8,18,19,22,23,35,37
We obtained good functional results by fixing the scapula 15°-20° between the medial aspect of the scapula and the interspinous line without any neurovascular complications. All patients achieved stable scapulothoracic fixation with metal wires without arthrodesis. Surgery corrected the shoulder drop and scapular winging, which resulted in a better appearance and functional improvement in abduction and flexion.
We thank Federica Guerra for help in preparing the manuscript and Olivia Faldini for helping with the revision.
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