Abstract
Introduction: Facioscapulohumeral muscular dystrophy (FSHD) is the third most common hereditary disease of the muscle after Duchenne and myotonic dystrophy. FSHD consists in an atrophic myopathy with predominant involvement of the face, upper arms, and shoulder muscles. FSHD compromises the muscles of the scapu-lothoracic joint, and usually spares muscles of the scapu-lohumeral joint. Consequently, when the patient tries to abduct or foreword flex the shoulder, the contraction of the relatively preserved scapulo-humeral muscles produces an abnormal rotation of the scapula, that is not hold to the chest wall because of the insufficiency of the scapulo-thoracic muscles, resulting in a winged scapula and in a limited arm motion. The aim of this study is to describe an original scapulopexy and report the long term results obtained in 9 patients affected by FSHD.
Material and methods: Nine patients (4 male and 5 female) affected by winged scapula in FSHD were observed and selected. Average age at surgery was 25 years (range 13–39). Surgery consisted in scapulopexy of both sides in the same surgical time. Through a skin incision on its medial border the scapula was exposed and positioned over the chest. The 5th to the 8th ribs were exposed in the part below the scapula. The position of the scapula over the chest was fixed by 4 doubled metal wires to the 5th, 6th, 7th and 8th rib. Each wire was passed anteriorly to the rib, and into a hole performed 1.5 cm from the medial border of the scapula. By tightening the wires, the scapula was fixed firmly over the chest. A figure of eight dressing that holds the shoulders back was applied immediately after surgery and maintained for six weeks.
Results: The average surgical time was 65 minute for each single procedure. One patient experienced an unilateral pneumothorax the day after operation, which resolved spontaneously in 48 hours. Average follow up was 11 years (range 3–15). All patients experienced a complete resolution of the winged scapula and an improvement of range of motion of the shoulder compared to the pre operative conditions. Average pre-operative abduction was 68 and post-operative was 85. Average pre-operative flexion was 78 and post-operative 112.
Discussion: This technique is easy and quick to perform, does not require grafts, reduces post surgical complications such as pneumothorax or haemothorax, and ensures good results even at considerable follow-up.
Theses abstracts were prepared by Professor Roger Lemaire. Correspondence should be addressed to EFORT Central Office, Freihofstrasse 22, CH-8700 Küsnacht, Switzerland.
