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.