Long Head of Triceps Tendon Transfer for Agenesis of Biceps and Brachialis: Two Cases

Introduction

Elbow flexion is crucial for self-feeding, dressing, and personal hygiene. Lack of elbow flexion results in significant disability. ¹ Lack of elbow flexion may be seen in obstetric brachial plexus palsy, congenital neuromuscular disorders, congenital myopathies and muscular dystrophies, congenital or obstetric neuropraxias, arthrogryposis, and muscular agenesis. ² Many techniques have been described to restore elbow flexion, including pectoralis major transfer, latissimus dorsi transfer, common wrist flexor or extensor transfer, transfer of whole triceps, and transfer of long head of triceps.^1,2

We present 2 patients with bilateral agenesis of the biceps and brachialis muscles. Both patients underwent anterior ulnar nerve transposition and transfer of the long head of triceps tendon for restoration of elbow flexion.

Cases

Case 1

A 20-month-old female presented with absent elbow flexion. The patient’s pregnancy was uncomplicated. The patient had no developmental delays. Absent antecubital creases with forearms in pronation at rest were noted. The patient had normal hand, wrist, and shoulder function. Passive elbow flexion was 0 to 130 degrees bilaterally with active extension but no active flexion. Magnetic resonance imaging revealed bilateral total absence of brachialis and biceps musculotendons units (Figure 1). A proper electromyography could not be performed due to lack of compliance. At 28 months of age, the patient underwent bilateral ulnar nerve anterior transposition and long head triceps transfer to ulna in the same setting.

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Figure 1.

Magnetic resonance imaging images of case 1 demonstrating absences of biceps and brachialis muscles.

Technique

Both patients underwent similar operative techniques as described by Gogola et al. ³ Both patients were administered general anesthesia with endotracheal tubing. In the supine position, the ulnar nerve was transferred subcutaneously. Then, blunt dissection was used to isolate the distal third of the long head of the triceps. Two centimeters of the intramuscular septum was removed in order to obtain adequate excursion of the triceps along with a portion of the flexor carpi ulnaris (FCU) fascia to add extra length to the triceps tendon. The anterior cortex of the ulna just distal to the coronoid served as the target for the attachment site and was confirmed with mini c-arm. The flexor origin was split with good exposure of the anterior ulna and the tendon was attached (Figures 2a and 2b). The elbow was then examined through a ROM to ensure proper tensioning and adequate ROM.

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Figure 2.

(a) The ulnar nerve was transposed and the triceps was prepped for the transfer. (b) the triceps tendon was transferred to the ulna.

There were some differences between the cases. In case 1, the FCU fascia was not amenable to inclusion in the triceps tendon bilaterally, therefore a 4 cm gracilis tendon allograft was utilized. The graft tendon was secured proximally to triceps tendon with the Pulvertaft technique. The distal attachment to ulna was performed by drilling 2 adjacent 2.5 mm holes and passing the tendon through the holes with #26 surgical wire. The distal stump was then sutured to the proximal limb creating a loop around the cortical holes. In case 2, the space within the flexor origin was limited, making the aforementioned technique impossible, so the tendon was secured to the anterior ulnar cortex with a 2.5 mm suture anchor (Mitek, DePuy-Synthes, Raynham, MA).

Postoperative course

Both patients were kept in long arm casts in 90 degrees of elbow flexion and neutral supination for 4 weeks. The cast was then removed and twice weekly occupational therapy (OT) was initiated for passive ROM with a custom fabricated hinged elbow brace (HEB) for an additional 2 weeks. At 6 weeks, active ROM was initiated. In case 1, the patient was lost to follow-up at from 8 weeks to 1 year postoperatively. In case 2, the patient was consistent with follow-up and OT regimen. Case 1 developed 25 degree elbow flexion contractures bilaterally, and case 2 developed 15 degrees of flexion contracture bilaterally. At 4 years postoperatively after adherence to the therapy regimen, case 1 achieved M3 elbow flexion on the left side and M3+ on the right side (Figure 3a and 3b, Supplemental Video 1). At 2-year follow-up, case 2 obtained M4 strength of elbow flexion (Figure 4a and 4b, Supplemental Video 2). Both patients could feed and clothe themselves. Both patients are still being followed up in clinic.

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Figure 3.

(a and b) Demonstrating elbow flexion of bilateral elbows for case 1.

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Figure 4.

(a and b) Demonstrating elbow flexion of bilateral elbows for case 2.

Discussion

While isolated absence of elbow flexors has been reported, ⁴ it is also seen in conjunction with other abnormalities such as in radial club hand, ⁵ Poland’s syndrome, and Arthrogryposis. ² Case 1 case did not have any other medical problems, but the second case was associated with arthrogryposis.

There are very limited data on the results of restoring functional elbow flexion in pediatric cases. Atkins et al ¹ reported 7 cases with pectoralis major transfer for patients with arthrogryposis. Tsai et al used pectoralis major and minor muscle rotational transfer on 4 patients, although they suggest that this can be a challenging option due to the complexity of the dissection. Three of their patients achieved sufficient elbow flexion, but one required additional Steindler flexorplasty to improve their elbow function. ⁶ We did not choose this option in our female patients in order to avoid a cosmetic chest deformity and any complications of future breast feeding.

Latissimus dorsi transfer is another option to restore elbow flexion. Rivet et al ⁷ treated 8 patients with myocutaenous latissimus dorsi transfers. Although they had good results, 3 patients were unable to reach their mouths. Werthel et al ⁵ reported a patient with radial club hand and lack of elbow flexion treated with latissimus dorsi transfer who achieved 0 to 100 degrees of elbow ROM with M4 power. We believe that this could have been another viable option for our patients.

Another treatment option is transfer of the common wrist extensor origin more proximally on the humerus to increase elbow flexion. Ethans and Leahey report a case where they achieved M3 power with elbow flexion with this technique. ⁸

The long head of the triceps has been described for treatment of both adult and pediatric patients. Rao et al describe a series of adult patients with upper brachial plexus injuries treated with long head of triceps transfer. In general, they reported good results; however, approximately one-third of their patients did not achieve any elbow flexion. ⁹ Carrol and Hill reported 15 patients who were treated with transfer of the entire triceps. They found that this transfer provides predictable benefit in patients who lack elbow flexion due to traumatic injuries, but not in patients with arthrogryposis. ¹⁰

For our patients, we utilized the technique described by Gogola et al. ³ This technique requires transfer the ulnar nerve in order to avoid iatrogenic compression of the nerve and does not require the presence of a biceps tendon for attachment; therefore, it can be performed in cases of total agenesis. Isolated transfer of the long head of the triceps yields a relatively small muscle bulk. We wonder if targeting 2 heads of the triceps would result in more elbow flexion strength. In this scenario, the remaining triceps would have sufficient strength to extend the arm with the assistance of gravity. Gogola et al recommended that patients undergoing this transfer should be mature enough to participate in rehabilitation and suggested that patients should be around 7 years of age before performing the transfer. We believe that this intervention can be performed in children younger than 3-years-old in order to take advantage of the significant neuroplasticity available at this age. We also believe that enabling active elbow flexion at an earlier age has significant benefit for both the patient and caregivers.

Based on our results and the results in the other series presented here, it appears that muscle and tendon transfers have only achieved M3-4 strength at maximum. It is possible that in our patients, case 1 achieved less power than case 2 due to the use of tendon allograft rather than native triceps tendon. Also, the tensioning of the triceps may not be sufficient to provide the desired excursion. Additionally, case 1 was less adherent to her therapy regimen, which also likely contributed to her comparatively decreased strength, highlighting the importance of postoperative therapy.

The main limitations of this study are our sample size of 2 and its lack of comparison to other tendon transfer options, making it problematic to specifically recommend this technique over the other available options. However, the rarity of this condition makes these 2 cases unique, challenging the current dogma of waiting to perform the transfer until age 7. While transferring an elbow extensor to achieve elbow flexion may seem counterintuitive, it offers sufficient flexion strength without sacrificing donor extensor function. We favored this technique over latissimus dorsi largely due to its less morbid dissection. We demonstrate here that this comparatively uncomplicated intervention provides reliable results in restoring elbow function sufficient for activities of daily living.