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Modified transfer of the pectoralis major muscle for restoration of elbow flexion in facioscapulohumeral muscular dystrophy: A case report
∗Corresponding author: Jae-Sung Yoo. osarthro@dkuh.co.kr
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Received: ,
Accepted: ,
This article was originally published by Reed Elsevier India Pvt. Ltd. and was migrated to Scientific Scholar after the change of Publisher.
Abstract
Abstract
Facioscapulohumeral muscular dystrophy (FSHD) is the third most common form of muscular dystrophy. It primarily exhibits slowly progressing symmetric muscle weakness in the facial, scapular, and upper arm muscles. The loss of elbow flexion is the most debilitating impairment for daily activities such as eating, grooming, and work-related tasks. The restoration of elbow flexion is more critical than any other functional recovery.
We present case of a 41-year-old male patient with non-functioning elbow flexion due to FSHD. The authors selected the pectoralis major muscle as the donor muscle through preoperative electromyographic and clinical examinations. The authors opted for the modified pectoralis major transfer method, connecting the distal tendon of the pectoralis major to the proximal tendon of the biceps brachii, to minimize surgical risks through a minimal approach. By the 3 months after the surgery, the patient demonstrated the ability to perform active elbow flexion.
For performing elbow flexoplasty in FSHD patients, it is essential to conduct thorough electromyographic and clinical examinations prior to surgery to accurately select the donor muscle. Additionally, the use of modified pectoralis major transfer for flexoplasty is considered a simple and safe surgical technique, offering the potential for favorable outcomes.
Keywords
Muscular dystrophy
Biceps
Pectoralis
Transfer
FSHD
EMG
MUAP
1 Introduction
Facioscapulohumeral muscular dystrophy (FSHD) ranks as the third most prevalent muscle disorder in the United States, following Duchenne and myotonic muscular dystrophy. Its occurrence in the United States is estimated at one in 15,000–21,000 people, while the overall frequency of this condition stands at 4–12 cases per 100,000 individuals.1,2 The disease's manifestation varies considerably among patients; nonetheless, it frequently commences with uneven muscle engagement in the facial muscles, resulting in noticeable indications such as incomplete eye closure or a flattened smile. Subsequently, the disorder advances to the proximal muscles in the upper trunk, including the rhomboids and the anterior serratus, which may manifest as scapular winging or an inability to raise the arm beyond 90°. Eventually, the distal muscles of the lower extremities and more proximal muscles are impacted. As FSHD progresses, distinct signs such as increased axillary fat and muscle wasting in the proximal arm become more prominent. Notably, the forearm is typically unaffected, giving rise to the characteristic Popeye or Beevor sign associated with this dystrophy.2–5
The authors performed a modified pectoralis major tendon transfer on a 41-year-old FSHD patient who was unable to perform elbow flexion, and we intend to report satisfactory results along with a literature review.
1.1 Case report
A 41-year-old male presented to the outpatient department with a chief complaint of weakness in the right upper limb. He has been experiencing progressive muscle weakness without any specific history of trauma for several years, and he also exhibited facial asymmetry in his expressions, with differences between the left and right sides. On physical examination, severe muscle weakness was observed, with a motor grade of II for right shoulder abduction and a motor grade of I for right elbow flexion. He had a family history of similar symptoms, with both his father and brother displaying similar issues. Genetic testing revealed the presence of shortening of the repeated DNA elements located within the 4q35 region of chromosome 4.
His creatine kinase level was elevated at 433, and on electromyography (EMG), findings in the forearm and below were close to normal. However, there were observations of decreased insertional activity, polyphasic and low amplitude motor unit action potentials (MUAP), early recruitment pattern, and a discrete interference pattern in the muscles of the upper arm and around the shoulder.
He expressed a primary desire for the restoration of elbow flexion, and to determine the appropriate donor muscle, EMG studies were conducted on the latissimus dorsi, pectoralis major, and triceps brachii muscles. The examination results showed decreased insertional activity, polyphasic MUAP, and a discrete interference pattern in both the latissimus dorsi and triceps brachii muscles. Based on these findings, the pectoralis major muscle was selected as the donor muscle.
2 Surgical technique
The authors decided to perform a modified pectoralis tendon transfer, connecting the distal insertion of the pectoralis major tendon to the proximal tendon of the biceps brachii long head tendon (Fig. 1). A regional block was performed, and the surgery was conducted in the beach chair position. An anterior approach was taken, initially detaching the pectoralis major tendon from the humerus insertion site. The detached muscle's degree of contraction was double-checked using an electric stimulator. Following that, the proximal biceps tendon was tenotomized. The entire biceps muscle was found to be atrophied and adhered to surrounding tissues, so the incision was extended to perform adhesiolysis. Subsequently, with the elbow joint flexed at 120°, the Pulvertaft technique was used to connect the distal pectoralis major tendon to the proximal biceps brachii tendon (Fig. 2).
3 Postoperative rehabilitation & clinical outcomes
After the surgery, a long arm cast was applied, maintaining the elbow at a flexion of 100° for three weeks. Subsequently, an elbow hinged brace was used for an additional three weeks to facilitate passive range of motion exercises. Starting from six weeks post-surgery, full range of motion and gentle strengthening were permitted. By the 3 months after the surgery, the patient demonstrated the ability to perform active elbow flexion (Fig. 3). The Mayo Elbow Score improved from 65 points before surgery to 95 points at three months post-surgery.
4 Discussion
In FSHD, motor weakness primarily manifests in the upper limbs. Among these, weakness in elbow flexion is particularly critical for regaining functionality, as it can impair essential self-care activities such as combing hair, washing, brushing teeth, and eating. Various muscle transfer techniques, including those involving the pectoralis major pectoralis minor, latissimus dorsi, triceps brachii, and gracilis muscles have been reported for the restoration of elbow flexion function.6–10 However, selecting a suitable donor muscle can be challenging in FSHD patients due to the accompanying weakness in shoulder and upper limb muscles. Therefore, careful donor muscle selection should be based on thorough preoperative clinical and electromyographic examinations. The authors selected the pectoralis major muscle as the donor muscle through preoperative electromyographic and clinical examinations.
There are different surgical techniques for pectoralis major tendon transfer, including methods that involve connecting the proximal part of the pectoralis major to the distal tendon of the biceps brachii and methods that connect the distal tendon of the pectoralis major to the proximal tendon of the biceps brachii.8,11,12 The authors opted for the modified pectoralis major transfer method, connecting the distal tendon of the pectoralis major to the proximal tendon of the biceps brachii, to minimize surgical risks through a minimal approach. However, in this patient who had FSHD, there was generalized atrophy of the biceps muscle and adhesion to surrounding tissues, necessitating adhesiolysis and requiring additional incisions.
The authors utilized the pectoralis major muscle as the donor muscle, determined through thorough preoperative examinations, to achieve the restoration of elbow flexion function in an FSHD patient with characteristic upper limb motor weakness. Additionally, we performed a relatively simple and safe modified pectoralis major transfer technique, resulting in satisfactory functional recovery within three months post-surgery. We intend to present this case report along with a literature review.
Ethical statement
This study was reviewed and accepted by the Dankook University medical center Institutional Review Board (DKUH-2023-09-022).
Funding
This research was conducted with the support of Dankook University.
Informed consent to participate
Informed consent was obtained, and patient participation in the study was voluntary.
Consent for publication
All of authors agree to publication.
Availability of data and material
It is available when reviewers request.
CRediT authorship contribution statement
Joong-Bae Seo: designed this study. Jae-Wook Jung: took retrospective data. Jae-Sung Yoo: designed this study, took retrospective data.
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