ORIGINAL ARTICLE
Reconstruction of Elbow Flexion in Arthrogryposis Multiplex Congenita Type I: Results of Transfer of Pectoralis Major Muscle With Follow-up at Skeletal Maturity Jirˇı´ Chomiak, MD, PhD, Pavel Dungl, MD, PhD, and Josef Vcˇela´k, MD
Background: The purpose of this study was to analyze the results of a pectoralis major transfer to restore active elbow flexion in patients with extension elbow contracture in arthrogryposis. The hypotheses were: (1) this transfer ensures permanent useful elbow flexion; and (2) flexion elbow deformity will not progress during growth and after its cessation. Methods: Unipolar transfer of the 3 distal parts of the pectoralis major muscle was used in 9 extremities of 5 patients (age range, 5 to 9 y; average age, 6.3 y) and the results were prospectively followed in the period of 13 to 16 years. Posterior elbow release was necessary in 5 extremities to achieve passive flexion of 90 degrees before the transfer. The subjective evaluation of daily living activities and data on the physical examination of the range of movement of the elbow, muscle strength, and electrical activity of the transferred muscle were assessed. Two specimens from transferred muscles were histologically examined. Results: All extremities achieved the active elbow flexion. Significant improvement of function for daily living activities was achieved in 5 extremities (55.5%). It includes the following results: 1 very good with flexion of 90 degrees and a deficit of extension of 35 degrees; 2 good with flexion of 92 and 100 degrees and a lack of extension of 42 and 45 degrees; and 2 satisfactory with a limited arc of motion between 20 and 45 degrees. Four extremities remained unsatisfactory with the arc of motion of 5 to 15 degrees. Significant elbow flexion contracture of 70 to 80 degrees developed in 4 extremities. Extremities with a necessity of posterior elbow release achieved a limited range of movement or significant elbow flexion contracture. Electromyography corresponded to a partial denervation of the transferred muscle followed by reinervation. Histologic examinations showed partial atrophy with signs of ongoing regeneration. Conclusions: The hypotheses of the study were not confirmed, because this muscle transfer restores useful elbow flexion without flexion deformity if the passive flexion at children’s age exceeds 90 degrees without a necessity of posterior release. In From the Department of Orthopedic, 1st Faculty of Medicine of Charles University and Institute of Postgraduate Medical Study and Hospital Na Bulovce, Prague, Czech Republic. The authors declare no conflicts of interest. Reprints: Jirˇ ı´ Chomiak, MD, PhD, Orthopedic Department Hospital Na Bulovce Budı´ nova 2, 18081 Prague 8, Czech Republic. E-mail: [email protected]. Copyright r 2014 by Lippincott Williams & Wilkins
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these cases, bilateral pectoralis to biceps transfer is recommended. Level of Evidence: Level II. Key Words: arthrogryposis, elbow, pectoralis major muscle transfer (J Pediatr Orthop 2014;34:799–807)
T
he upper extremities are involved in 70% of patients with arthrogryposis.1–2 Two main patterns have been described by Brown et al.3 Type I is characterized by the extension of the elbow with weak or absent biceps and brachialis muscles and adduction and internal rotation at the shoulder, pronation of the forearm, and flexion deformity of the wrist and digits occurred in 61% to 62% of patients.4 In type II, the elbow is fixed in flexion and deformities of the shoulder and wrist are similar. The goal of treatment in type I is to facilitate functional independence4,5 and to restore the flexion of the elbow as the essential function of the upper limb6 that would enable patients to reach the mouth and head with the hands, as well as maintain active extension of the elbow for hygienic purposes and transportation. Passive flexion of the elbow of over 90 degrees is necessary before any attempt to improve active flexion is made.7 Active flexion of the elbow can be achieved by a variety of muscle transfers.6–12 We modified the method of Clark13 based on the transfer of distal parts of the pectoralis major muscle. The longterm concern of the muscle transfers is the potential progressive flexion contracture of the elbow during growth.14 The aims of this study were to evaluate the outcomes of pectoralis major transfer performed during childhood and followed until skeletal maturity. The hypotheses were: (1) the transfer will restore permanent active flexion of the elbows; (2) the transfer will not lead to progressive flexion contracture of the elbow during growth and after its cessation.
METHODS Between 1996 and 2012, active elbow flexion was reconstructed in 9 patients (17 elbow joints) with classic arthrogryposis (type Ia according to Hall).1 This study www.pedorthopaedics.com |
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TABLE 1. Demographic Data of Patients Before Surgical Treatment ROM of Elbow Study No. 1.1 1.2 2.1 2.2 3.1 3.2 4.1 4.2 5.1
Side
Sex
Extremities Involvement
R L R L R L R L L
F F F F M M M M M
Tetramellic Tetramellic Tetramellic Tetramellic Tetramellic Tetramellic Bimellic Bimellic Bimellic
Age at Surgery
Active
5 5 7 9 8 6 6 5 6
0 0 0 0 0 0 0 0 0
EMG-Activity (Grade)
Passive
Points Objective Assessment
Biceps m. Brachial m.
PMAJ
0-0-100 0-0-100 0-0-50 0-0-30 0-0-40 0-0-90 0-20-20 0-0-20 0-0-110
3.66 3.66 3.66 3.66 3.33 3.66 4 3.66 3.66
0 0 0 0 0 2 0 0 0
5 4 4 4 4.5 4.5 4 4 4.5
Objective assessment (average of points): excellent: 1, very good: 1.1 to 2, good: 2.1 to 2.5, satisfactory: 2.6 to 2.9, unsatisfactory: 3.0 to 5. Electromyographic maximum electric activity was graded: 0—no activity, 1—single potentials, 2—more than single potentials recorded, 3—mixed pattern, baseline visible, 4—submaximal activity, 5—interference pattern. Biceps m. indicates biceps brachii muscle; Brachial m., brachial muscle; EMG, electromyography; PMAJ, pectoralis major muscle; ROM, range of movement.
concerns on 5 patients (9 elbow joints) aged 5 to 9 years (average age, 6.3 y, Table 1) because they met the required criteria, namely: surgery was performed in childhood; and they were followed until skeletal maturity (follow-up, 13 to 16 y). No active flexion was noted and 8 elbows were in full extension, whereas 1 had mild flexion contracture. This elbow (#4.1) had undergone previous surgery; namely pectoralis minor muscle transfer. Four patients underwent bilateral nonsimultaneous unipolar transfers of 3 distal parts of the pectoralis major. Preoperatively, a subjective assessment referred to the ability to feed oneself (reach the mouth with a hand); to comb one’s hair; to attend to toileting needs (using the hand for perineal hygiene); and to dress. For the objective assessment, the range of movement (ROM) and muscle strength of biceps brachii, brachial, and pectoralis major muscles were evaluated. ROM data consisted of goniometric measurements of passive and active antigravity movement of the elbow in the standard position of the shoulder in adduction and neutral rotation. The scapula was not stabilized and it was not allowed to swing the arm. The strength of the muscle was graded with manual muscle testing according to the Medical Research Council grading system.15 A modified evaluation of the results according to Atkins et al10 was used: grade 1— excellent, grade 2—very good, grade 3—good, grade 4— satisfactory, grade 5—unsatisfactory (Table 1). The following criteria were scored: (1) the degree of maximum active elbow flexion; (2) the degree of the extension; and (3) the strength of elbow flexion. Electromyography (EMG) activities of the biceps, brachialis, and pectoralis major muscles were examined using concentric needle electrodes in all patients before surgery, as well as activity of the transferred muscle was evaluated in all patients, 1 to 3 years after surgery for the purposes of the study. Activity at rest was registered and activity at maximum voluntary contracture was graded 0 to 5.16 The surgical procedure includes 1 or 2 steps. In 5 extremities lacking 90 degrees of passive elbow flexion after completing a program of ROM exercises, a posterior elbow capsulotomy with V-Y lengthening of the distal triceps bra-
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chii muscle was performed to achieve passive flexion of over 90 degrees. This procedure was used 4 to 6 weeks before the transfer in 4 elbows or concomitant with the transfer in 1 elbow, respectively. The second step is the transfer of the sternocostal, costal, and abdominal parts of the pectoralis major muscle according to the previously described technique17 and in Figure 1. The transferred muscle was connected: (1) to the remnant of distal tendon of the biceps (1 extremity); (2) to this tendon and to the string of aponeurosis of the common origin of the forearm extensors (3 extremities); (3) to other muscles of the flexor or extensor of the wrist (5 extremities). Their tendons were dissected in the wrist and then pulled subcutaneously anterior from the elbow joint, whereas proximal 3 to 4 cm of muscles remain intact and serve as new insertion for the transmission of power to the forearm. The radial/ulnar flexors were chosen if flexion deformity of the wrist joint predominated in either a radial or ulnar deviation. The extensors were chosen when the extension of the wrist was satisfactory. The same technique was used, when the fixation of the transferred muscle failed. The transferred pectoralis major muscle was fixed at 90 degrees of elbow flexion (Fig. 1B) and tension was classified as appropriate if it allowed a lack of elbow extension of up to 45 degrees. The extremity was fixed in shoulder spica for 4 weeks at 90 degrees of elbow flexion and 20 degrees abduction of the shoulder. The postoperative rehabilitation encompassed the transcutaneous electric stimulation of the transferred muscle and exercises of the elbow and shoulder with isotonic concentric contraction in the first phase (4 to 12 wk). In the second phase, the passive and active exercises of the elbow for a minimum of 15 minutes daily were recommended for the next 3 months. Two specimens (5 10 mm) of the distal medial part of the transferred muscle were harvested during the revision surgery 2 years (#4.2) and 11 years after the primary procedure (#5.1), respectively, and histologically examined. A light microscopy of paraffinmounted sections stained with hematoxylin-eosin was used. r
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FIGURE 1. Pectoralis major transfer in a 5-year-old girl. A, The pectoralis major muscle is bluntly divided between the second and third rib, and then the distal parts of the muscle below the second ribs and below manubrium sterni origin (sternocostal, costal, and abdominal parts) are dissected as a unit from the chest wall and sheet of rectus abdominis muscle in a proximolateral direction (*). Humeral origin of the muscle remains intact (arrow). The subcutaneous tunnel connecting the ventral side of elbow and shoulder is made using the vessel clamp. B, Transferred muscle (*) is passed through the tunnel to the brachium and fixed using fish-mouth technique to the tendon of the flexor carpi ulnaris muscle (thick arrow) in elbow flexion of 90 degrees. Neurovascular bundle of transferred part (thin arrow). M indicates manubrial non-transferred part of the pectoralis major muscle.
RESULTS Complications There were no intraoperative or perioperative complications. Subsequent surgical procedures were necessary in 7 extremities, mostly (5 extremities) the transferred muscle had to be fixed to the alternative tendon of the forearm in the period of 10 to 26 months after the primary procedure. In 1 patient (#5.1), the hypertrophic scar was corrected 1 year after surgery and the tendon of the transferred muscle was lengthened and ventral elbow capsule was released 11 years later for elbow contracture, respectively. In 1 extremity (#1.1), the tendon pulley was r
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reconstructed for an abundant cutaneous fold (pterygium) on the ventral side of the elbow. Before surgery, all extremities were evaluated as unsatisfactory with a score range of 3.33 to 4 points (Table 1). In the subjective assessment according the patients evaluation, 4 extremities improved significantly, 2 extremities improved partially, and 3 extremities remained unchanged (Table 2). In the objective assessment, all extremities achieved the active elbow flexion. The significant improvement of function in 5 extremities (55.5%) includes very good, good, and satisfactory results. One elbow joint was evaluated as very good result, (#1.2) (Figs. 2A–C), 2 extremities (#1.1, #3.2) were evaluated as good, and 2 extremities (#3.1, #5.1) as satisfactory results, respectively. Four extremities were evaluated as unsatisfactory (#2.1, #2.2, #4.1, #4.2). Flexion deformity of 70 to 80 degrees was noted in 4 patients (#3.1, #4.1, #4.2, and #5.1) and developed early within 2 years after surgery in 2 of them (#4.1, # 4.2), whereas 2 (#3.1, #5.1) were noted late in adolescence. The patients with early flexion deformities underwent posterior elbow capsulotomy and triceps lengthening before the transfer. First patient with late flexion deformity (#3.1) required previous triceps lengthening and posterior elbow capsulotomy, and the flexion deformity progressed slowly in adolescence (Fig. 3A, Table 2). Second patient with late flexion deformity was described in complications (#5.1, Fig. 4). He was able to control and transport the items with his hand (Fig. 4), but he was not satisfied with lack of elbow extension and ROM 0-80-100 degrees. Passive extension increased and active flexion decreased after tendon lengthening of the transferred muscle and elbow capsulotomy and the patient subjectively and objectively improved (Table 2). All but one of 4 elbows without posterior release attained very good or good results with a useful arc of motion ranging from 50 to 55 degrees (Table 2, Figs. 2, 3A, B—left elbow), whereas all of 5 elbows requiring a posterior elbow release attained a limited arc of motion of 5 to 20 degrees and in 3 of them the elbow flexion contracture developed (Table 2—#3.1, 4.1, 4.2). The strength of elbow flexion was graded as M4 and M4+ in very good and good results, and M3 and M3+ in satisfactory and unsatisfactory results, respectively. It means all patients flexed the elbows against gravity, but not in a full passive ROM, because passive flexion was obvious in 7 extremities as being increased. Concerning the preoperative EMG, 8 extremities showed complete atrophy and 1 extremity showed incomplete severe atrophy of the biceps and brachialis muscles, respectively. An activity grade of 4 to 5 was registered in all pectoralis major muscles. Postoperative EMG of the transferred muscle corresponded to a partial denervation of the muscle followed by repair of innervation and activity grade 3 to 4+. Histologic examinations (Fig. 5) are interpreted as a partial atrophy of muscle with signs of ongoing regeneration and also the hypertrophy of some muscle fibers. www.pedorthopaedics.com |
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Subjective assessment: 1 point—significant improvement, 2 points—partial improvement, 3 points—no improvement. Objective assessment (average of points): excellent: 1, very good: 1.1 to 2, good: 2.1 to 2.5, satisfactory: 2.6 to 2.9, unsatisfactory: 3.0 to 5. The degree of maximum active flexion in elbow joint was scored as follows: 1 point—over 120 degrees, 2 points—90 to 120 degrees, 3 points—75 to 89 degrees, 4 points—61 to 74 degrees, 5 points—under 60 degrees. The degree of the deficit of extension was scored: 1 point—0 to 5 degrees, 2 points—6 to 35 degrees, 3 points—36 to 50 degrees, 4 points—51 to 70 degrees, 5 points— > 70 degrees. *Range of movement after lengthening of the distal insertion tendon of the pectoralis major muscle and release of elbow at skeletal maturity. AE indicates aponeurosis of extensors; ECRB, extensor carpi radialis brevis muscle; ECRL, extensor carpi radialis longus muscle; FCRL, flexor carpi radialis longus muscle; FCU, flexor carpi ulnaris muscle; MRC, Medical research council grade; PMAJ, pectoralis major muscle; ROM, range of movement; Yes-TS, table support: means support of the elbow using the table .
Yes Yes Yes Yes Yes Yes No No Yes Yes Yes Yes Yes No Yes No No Yes Yes Yes Yes-TS Yes-TS Yes Yes Yes-TS Yes-TS Yes 2.33 2 3.66 3.66 2.66 2.33 3.33 3 2.66 1 1 3 3 2 1 2 2 1 4+ 4+ 3 3 4 4+ 3+ 4 4 0-42-92 0-35-90 0-40-45 0-40-45 0-70-90 0-45-100 0-75-90 0-80-95 0-40-85* Pulley reconstruction 2nd fixation to FCU 2nd fixation to FCU — — 2nd fixation to FCU 2nd fixation to AE 2nd fixation to BRA (1) Scar correction; (2) Elbow-release+PMAJtendon lengthening ECRB Bicep+AE Biceps Biceps+AE ECRL Biceps+AE ECRB FCU FCRL — — During transfer Before transfer Before transfer — Before transfer Before transfer — 16 16 16 14 14 16 15 16 13 1.1 1.2 2.1 2.2 3.1 3.2 4.1 4.2 5.1
Perineal Hygiene Feeding/ Combing Objective Assessment Subjective Assessment Following Procedures
ROM of Elbow Active PMAJ Fixation to Elbow Capsulotomy and Triceps m. Lengthening Study Follow-up No. Period (y)
TABLE 2. Results After the Transfer of Pectoralis Major Muscle at Skeletal Maturity
Flexion Strength MRC (Grade)
Points
Daily Activities
Dressing
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DISCUSSION The hypotheses of this study were not confirmed because: (1) the transfer of pectoralis major muscle permanently improved the elbow flexion of 5 upper extremities (55%) only; and (2) flexion deformity of the elbow developed in 4 extremities (45%). Global improvement in function was noted in 5 extremities (55.5%). The patients with very good and good outcomes (33%) with active flexion 90 degrees were able to use the extremities for independent self-feeding as well as for perineal hygiene. No excellent results were achieved, because a ROM of the elbow or flexion of over 120 degrees was not obtained. According to literature reports,9 the necessary flexion of elbow is 120 degrees for independence in self-feeding, but it is usually not possible to achieve in arthrogryposis. Two extremities were evaluated as satisfactory, and still remained useful for some daily living activities. Four extremities remained unsatisfactory due to the very limited ROM, they were probably caused by the severe joint contractures of the elbow joint and due to the overall weakness of transferred muscles. The mechanical weakness of muscle was not caused due to the denervation or avascular necrosis of the muscle, which was confirmed by EMG and histologic examinations. But these 4 patients did not describe this result as a deterioration. It can be explained due to the achievement of some arc of active motion and the stabilization of the extremity in the space and using a compensatory mechanism for insufficient elbow flexion. It is clearly visible that the patients with initial passive elbow flexion of over 90 degrees achieved very good and good active ROM, whereas the patients with necessary posterior elbow release achieved only a limited arc of motion, including a greater lack of extension. It is in concordance with the findings of Van Heest et al.7 We can speculate that the subsequent repeated joint contracture develops on the basis of predetermined joint stiffness in these extremities with initial limited elbow motion. It means, the children with adequate passive elbow flexion before transfer will benefit from the pectoralis major muscle transfer, whereas the children with limited motion may not achieve useful arc of motion and flexion contracture will probably develop. The pectoralis major to biceps transfer was chosen because the length of the fibers, the cross-sectional area, and muscle force are similar to the biceps brachii muscle and the muscle is appropriate for splitting on the basis of the segmental anatomy and neurovascular supply.17–19 Clark13 described the transfer of the part of the muscle from the origin on the sixth rib, but this part is too weak and cannot substitute the biceps and brachialis function in arthrogryposis.9,17 Therefore, this technique was modified using the transfer of distal 3/5 of the muscle. The functional sequels for the shoulder are minimal, because 2 proximal parts of the muscle remain intact. Pectoralis major muscle transfer can be used as: (1) a partial unipolar as described above; (2) a partial bipolar, when humeral origin is released and fixed to the acromion and sternocostal part of the muscle is transferred8,9; and (3) a r
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FIGURE 2. The girl with tetramellic form of AMC type I (#1.1, 1.2) at the age of 17.5 years, 13 years after bilateral pectoralis major transfers. A, In anterior view, the extension deficit in elbow joints 30 and 35 are obvious. Cosmetic appearance of breast and chest wall development is not disturbed. B, Ninety-two degrees of active flexion of the right elbow allows to the hand to reach the mouth. C, Ninety degrees of active flexion of the left elbow.
complete bipolar when the whole muscle is released from the chest wall and rotated to the brachium.7,9 Our results are comparable to these techniques only. The results of the small series of these procedures vary in literature reports. Lloyd-Roberts and Lettin20 reported permanent improvement and self-feeding in 6 of 8 patients. They modified Clark’s technique with more distal attachment of the transferred muscle to the ulna, which increases mechanical efficiency. We used a similar technique for the same reasons. Carroll and Kleinman9 described excellent r
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results after a complete bipolar transfer in 3 of 4 patients with a useful arc of motion of 60 degrees and elbow flexion of 120 degrees. In 1 patient with arthrogryposis in their series, the method failed due to fibrofatty degeneration of the muscle. Doyle et al8 reported the independence in feeding in 6 of 7 patients after a partial bipolar procedure. Van Heest et al7 reported the results of 5 patients (1 good, 3 fair, and 1 poor) after Carroll’s modification, and they do not recommend this method. All these authors used the technique for unilateral www.pedorthopaedics.com |
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FIGURE 3. A 17-year-old boy with tetramellic form of AMC type I (#3.1, 3.2). Pectoralis major muscles were transferred in age of 6 (left) and 8 years (right). A, Anterior view 9 years (right) and 11 years (left) after surgery. Extension lack of 60 degrees of the right elbow (after previous posterior capsulotomy and triceps lengthening) and 45 degrees of the left elbow (without capsulotomy and lengthening), respectively. Flexion contracture of right wrist joint is also obvious. B, Active flexion of the left elbow to 100 degrees enables to bring the hand to the face. C, Moderate extension deficit of the left elbow of 45 degrees allows to reach the buttock and perineum with the left hand.
reconstruction and they did not report progressive elbow flexion deformity. This fact supports our opinion that the bilateral reconstruction of the active elbow flexion is possible. The lack of extension to 45 degrees after a bilateral pectoralis major transfer does not interfere with care of the perineum or possibilities for transportation. This opinion is also supported by some authors.21,22 Flexion elbow contractures of 70 to 80 degrees were noted in 4 extremities of our group and led to unsatisfactory or satisfactory results. Lahoti and Bell14 reported a pro-
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gressive flexion deformity of over 90 degrees in 8 of 10 elbows after long-term follow-up after a complete transfer of the muscle originally described by Brooks and Seddon.23 These patients were operated on at children’s age and only 1 had surgery at the age of 14 years and attained good elbow function. The cause of flexion deformity is unclear but it is related to the muscle transfer and the power of the transferred muscle was not deteriorated.14 We can speculate that growth also influences the development of flexion deformity in extremities with predetermined r
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FIGURE 4. A 17.5-year-old boy with bimellic form of AMC type I (#5.1), 11 years after pectoralis major muscle transfer on left side. A, Lack of extension of the elbow 80 degrees. B, Active powerful elbow flexion 95 degrees during elevation of the sand pack.
joint stiffness, whereas after the cessation of growth the flexion deformity will not progress. It is supported by our observation in 2 patients with a bilateral transfer provided at skeletal maturity, where flexion deformity did not increase in the follow-up. Regardless of the surgical modifications, the most critical part of the surgical procedure is the fixation of the transferred muscle to the connective tissue of the forearm, which enables permanent mechanical efficiency. The fixation to the remnant of biceps muscle failed and it was necessary to augment the fixation to the
FIGURE 5. Histologic specimen of transferred pectoralis major muscle harvested 11 years after surgery (#5.1). Moderate increase of fibrofatty tissue (*) and muscle fibers of normal and bigger and smaller diameter are obvious. Muscle fibers with centrally located nucleus represent the regenerating fibers (arrows) (hematoxylin-eosin, magnification: 80). r
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aponeurosis of extensors or tendons of forearm flexors or extensors, respectively. The latissimus dorsi muscle transfer represents a further option.7,21,24 According to our opinion and literature reports,7 its muscle mass can be inadequate or even completely fails in arthrogryposis. Microsurgical transfers of different muscles, primarily gracilis muscle6,25,26 represent very progressive solution. EMG proved useful for providing information on the activity of the biceps and brachialis muscles before surgery and on the contractility of the transferred muscle before and after surgery. It also allowed distinguishing between mechanical failures and atrophying due to denervation or vascular lesions. All transposed muscles revealed changes indicating a minimum denervation followed by reinnervation. Two histologic examinations reveal the sparing of the majority of muscle fibers and very good reinnervation and blood supply of muscle fibers, which were partially damaged. The similar findings in the specimen of the skeletally mature patient confirmed our assumption that the transferred muscle with intact innervation and vascular supply can grow and change according to the functional rights.27 Van Heest et al28 reported the largest series of 23 patients with extension contracture using the posterior elbow capsulotomy with triceps lengthening, which enable the passive flexion of the elbow using the passive assistance. According to our opinion, the patients with arthrogryposis may benefit from pectoralis major to biceps transfer more than from solely the restoration of passive elbow flexion, because the active flexion is useful for daily living activities and for the stabilization of the extremity in the space. It remains a controversial question www.pedorthopaedics.com |
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if any muscle transfer for active elbow flexion should be used in elbows with limited passive flexion, when we can expect the lack of elbow extension and/or limited arc of motion during growth, or in other words, to sacrify the elbow extension. According to our opinion the active flexion of 90 degrees and adequate strength of the transferred muscle allow the hand to reach the head and to elevate the items of adequate weight despite the lack of elbow extension. If the patient is not satisfied with the lack of extension, it is possible to increase the extension due to the lengthening of the tendon of the transferred muscle, but elbow flexion will decrease. Limitation of arc of motion should be prevented due to the regular and intensive rehabilitation. According these findings we changed our treatment strategy. Formerly, we preferred the bilateral restoration of active elbow flexion in all patients irrespective of the necessity of posterior elbow release. Currently, we recommend the bilateral pectoralis major transfer in patients with native passive elbow flexion without the necessity of posterior elbow release. In patients with initial limited passive elbow flexion, we recommend now to restore active elbow flexion on one side (preferably with greater arc of motion), whereas the extremity of the opposite side without transfer can be used for perineal hygiene. The parents should be informed about the probability of the lack of extension on the operated side and possible limitation of the arc of movement during the growth. Another possibility would be to postpone the restoration of active elbow flexion to the age of growth cessation, which was confirmed in our observations and literature reports.14 The rehabilitation represents an important part of the treatment strategy and requires the cooperation of the patient. Therefore, we recommend starting the surgical procedure in the fifth year of life. It is in concordance with Van Heest et al,7 whereas Mennen29 recommended corrective surgery in 3 to 6 months of age. We suggest that the regular passive and active exercises of the elbow joint to the extension are necessary to prevent elbow flexion contracture in any age after this muscle transfer. With regard to the cosmetic appearance after the pectoralis major transfer, we modified 1 incision technique due to 2 shorter incisions technique. Neither modification disturbed cosmetic appearance of breast or chest wall development in any females in our study (Fig. 2). This finding is in accordance with literature reports,7 but we did not study the morphology and function of the breasts particularly. This study has the following strengths. It provides mid-term to long-term follow-up data in the prospective study of patients with arthrogryposis. The number of patients is limited but comparable with those from the literature reports, because of the rare condition. The described technique was based on the anatomic study of the neurovascular supply of the muscle.17 EMG examinations preoperatively and postoperatively and the rehabilitation regimen were unified. The viability of the transferred muscle is supported also due to 2 histologic examinations.
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The study has the following limitations: 2 surgeons in 1 institution treated the patients, so the effects of institutional bias may be possible; the data of shoulder and wrist and fingers were not particularly analyzed; and no data of control group of patients were available. In conclusion, unipolar transfer of 3 distal parts of pectoralis major muscle may establish bilateral permanent effective elbow flexion by retaining sufficient extension for perineal hygiene in patients with arthrogryposis. In patients with the necessity of posterior elbow release, this transfer is only partially effective with possible subsequent elbow flexion deformity. In these cases we recommend to restore active elbow flexion on 1 side. REFERENCES 1. Hall JG. Artrogryposis multiplex congenita: aetiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop Part B. 1997;6:159–166. 2. Sarwark JF, McEwen GD, Scott CI. Current concepts review. Amyoplasia: a common form of arthrogryposis. J Bone Joint Surg Am. 1990;72:465–469. 3. Brown LM, Robson MJ, Sharrard WJW. The pathophysiology of arthrogryposis multiplex congenita neurologica. J Bone Joint Surg Br. 1980;62:291–296. 4. Ezaki M. Treatment of the upper limb in the child with arthrogryposis. Hand Clin. 2000;16:703–711. 5. So¨dergard J, Hakamies-Blomqvist L, Sainio K, et al. Arthrogryposis multiplex congenita: perinatal and electromyographic findings, disability, and psychosocial outcome. J Pediatr Orthop Part B. 1997; 6:167–171. 6. Kay S, Pinder R, Wiper J, et al. Microvascular free functioning gracilis transfer with nerve transfer to establish elbow flexion. J Plast Reconstr Aesthet Surg. 2010;63:1142–1149. 7. Van Heest A, Waters PM, Simmons BP. Surgical treatment of arthrogryposis of the elbow. J Hand Surg Am. 1998;23:1063–1070. 8. Doyle JR, James PM, Larsen LJ, et al. Restoration of elbow flexion in arthrogryposis multiplex congenita. J Hand Surg. 1980;5:149–152. 9. Carroll RE, Kleinman WB. Pectoralis major transplantation to restore elbow flexion to the paralytic limb. J Hand Surg. 1979;4:501–507. 10. Atkins RM, Bell MJ, Sharrard WJW. Pectoralis major transfer for paralysis of elbow flexion in children. J Bone Joint Surg Br. 1985; 67:640–644. 11. Tsai TM, Kalisman M, Burns J, et al. Restoration of elbow flexion by pectoralis major and pectoralis minor transfer. J Hand Surg. 1983;8:186–190. 12. Gogola GR, Ezaki M, Oishi SN, et al. Long head of the triceps muscle transfer for active elbow flexion in arthrogryposis. Tech Hand Up Extrem Surg. 2010;14:121–124. 13. Clark JMP. Reconstruction of biceps brachii by pectoral muscle transplantation. Br J Surg. 1946;34:180–181. 14. Lahoti O, Bell MJ. Transfer of pectoralis major in arthrogryposis to restore elbow flexion. Deteriorating results in the long term. J Bone Joint Surg Br. 2005;87:858–860. 15. Medical Research Council. War Memorandum No.7. II ed. London: Her Majesty’s Stationery Office; 1943. 16. Ludin H-P. Electromyography in Practice. Stuttgart, New York: Thieme; 1980. 17. Chomiak J, Dungl P. Reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. Part I: surgical anatomy and vascular and nerve supply of the pectoralis major muscle as a basis for muscle transfer. J Child Orthop. 2008;2:357–364. 18. Chaffai MA, Mansat M. Anatomic basis for the constructions of a musculotendinous flap derived from the pectoralis major muscle. Surg Radiol Anat. 1988;10:273–282. 19. Nakajima K, Die Y, Abe S, et al. Anatomical study of the pectoral branch of thoracoacromial artery. Bull Tokyo Dent Coll. 1997;38:207–215. r
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20. Lloyd-Roberts GC, Lettin AWF. Arthrogryposis multiplex congenita. J Bone Joint Surg Br. 1970;52:494–508. 21. Maila¨nder P, Machens HG, Rieck B, et al. Bilateral restoration of elbow flexion by bipolar transposition of latissimus dorsi in arthrogryposis multiplex congenita: A Case Report. Handchir Mikrochir Plast Chir. 1996;28:59–63. 22. Martin G, Perrot P, Duteille F. Treatment of congenital deficit of elbow flexion in a case of arthrogryposis. Chirurgie de la main. 2009;28:116–119. 23. Brooks DM, Seddon HJ. Pectoral transplantation for paralysis of the flexors of the elbow. J Bone Joint Surg Br. 1959;41:36–43. 24. Gagnon E, Fogelson N, Seyfer AE. Use of the latissimus dorsi muscle to restore elbow flexion in arthrogryposis. Plast Reconstr Surg. 2000;106:1582–1585. 25. Chuang DCC, Carver N, Wie FC. Results of functioning free muscle transplantation for elbow flexion. J Hand Surg Am. 1996;21: 1071–1077.
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26. Doi K, Muramatsu K, Hattori Y, et al. Restoration of prehension with double free muscle technique following complete avulsion of the brachial plexus: indications and long-term results. J Bone Joint Surg Am. 2000;82:652–666. 27. Grim M, Dittertova´ L, Vejsada R, et al. Experimental and anatomical basis for reconstruction of anal musculature employing gracilis muscle graft with intact vascular supply. In: Freilinger G, Holle J, Carlson BM, eds. Muscle Transplantation. Wien, New York: Springer; 1981:265–274. 28. Van Heest A, James MA, Lewica A, et al. Posterior elbow capsulotomy with triceps lengthening for treatment of elbow extension contracture in children with arthrogryposis. J Bone Joint Surg Am. 2008;90:1517–1523. 29. Mennen U. Early corrective surgery for the wrist and elbow in arthrogryposis multiplex congenita. J Hand Surg Br. 1993;18: 304–307.
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Reconstruction of Elbow Flexion in Arthrogryposis Multiplex Congenita Type I: Results of Transfer of Pectoralis Major Muscle With Follow-up at Skeletal Maturity Jirˇı´ Chomiak, MD, PhD, Pavel Dungl, MD, PhD, and Josef Vcˇela´k, MD
Background: The purpose of this study was to analyze the results of a pectoralis major transfer to restore active elbow flexion in patients with extension elbow contracture in arthrogryposis. The hypotheses were: (1) this transfer ensures permanent useful elbow flexion; and (2) flexion elbow deformity will not progress during growth and after its cessation. Methods: Unipolar transfer of the 3 distal parts of the pectoralis major muscle was used in 9 extremities of 5 patients (age range, 5 to 9 y; average age, 6.3 y) and the results were prospectively followed in the period of 13 to 16 years. Posterior elbow release was necessary in 5 extremities to achieve passive flexion of 90 degrees before the transfer. The subjective evaluation of daily living activities and data on the physical examination of the range of movement of the elbow, muscle strength, and electrical activity of the transferred muscle were assessed. Two specimens from transferred muscles were histologically examined. Results: All extremities achieved the active elbow flexion. Significant improvement of function for daily living activities was achieved in 5 extremities (55.5%). It includes the following results: 1 very good with flexion of 90 degrees and a deficit of extension of 35 degrees; 2 good with flexion of 92 and 100 degrees and a lack of extension of 42 and 45 degrees; and 2 satisfactory with a limited arc of motion between 20 and 45 degrees. Four extremities remained unsatisfactory with the arc of motion of 5 to 15 degrees. Significant elbow flexion contracture of 70 to 80 degrees developed in 4 extremities. Extremities with a necessity of posterior elbow release achieved a limited range of movement or significant elbow flexion contracture. Electromyography corresponded to a partial denervation of the transferred muscle followed by reinervation. Histologic examinations showed partial atrophy with signs of ongoing regeneration. Conclusions: The hypotheses of the study were not confirmed, because this muscle transfer restores useful elbow flexion without flexion deformity if the passive flexion at children’s age exceeds 90 degrees without a necessity of posterior release. In From the Department of Orthopedic, 1st Faculty of Medicine of Charles University and Institute of Postgraduate Medical Study and Hospital Na Bulovce, Prague, Czech Republic. The authors declare no conflicts of interest. Reprints: Jirˇ ı´ Chomiak, MD, PhD, Orthopedic Department Hospital Na Bulovce Budı´ nova 2, 18081 Prague 8, Czech Republic. E-mail: [email protected]. Copyright r 2014 by Lippincott Williams & Wilkins
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these cases, bilateral pectoralis to biceps transfer is recommended. Level of Evidence: Level II. Key Words: arthrogryposis, elbow, pectoralis major muscle transfer (J Pediatr Orthop 2014;34:799–807)
T
he upper extremities are involved in 70% of patients with arthrogryposis.1–2 Two main patterns have been described by Brown et al.3 Type I is characterized by the extension of the elbow with weak or absent biceps and brachialis muscles and adduction and internal rotation at the shoulder, pronation of the forearm, and flexion deformity of the wrist and digits occurred in 61% to 62% of patients.4 In type II, the elbow is fixed in flexion and deformities of the shoulder and wrist are similar. The goal of treatment in type I is to facilitate functional independence4,5 and to restore the flexion of the elbow as the essential function of the upper limb6 that would enable patients to reach the mouth and head with the hands, as well as maintain active extension of the elbow for hygienic purposes and transportation. Passive flexion of the elbow of over 90 degrees is necessary before any attempt to improve active flexion is made.7 Active flexion of the elbow can be achieved by a variety of muscle transfers.6–12 We modified the method of Clark13 based on the transfer of distal parts of the pectoralis major muscle. The longterm concern of the muscle transfers is the potential progressive flexion contracture of the elbow during growth.14 The aims of this study were to evaluate the outcomes of pectoralis major transfer performed during childhood and followed until skeletal maturity. The hypotheses were: (1) the transfer will restore permanent active flexion of the elbows; (2) the transfer will not lead to progressive flexion contracture of the elbow during growth and after its cessation.
METHODS Between 1996 and 2012, active elbow flexion was reconstructed in 9 patients (17 elbow joints) with classic arthrogryposis (type Ia according to Hall).1 This study www.pedorthopaedics.com |
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TABLE 1. Demographic Data of Patients Before Surgical Treatment ROM of Elbow Study No. 1.1 1.2 2.1 2.2 3.1 3.2 4.1 4.2 5.1
Side
Sex
Extremities Involvement
R L R L R L R L L
F F F F M M M M M
Tetramellic Tetramellic Tetramellic Tetramellic Tetramellic Tetramellic Bimellic Bimellic Bimellic
Age at Surgery
Active
5 5 7 9 8 6 6 5 6
0 0 0 0 0 0 0 0 0
EMG-Activity (Grade)
Passive
Points Objective Assessment
Biceps m. Brachial m.
PMAJ
0-0-100 0-0-100 0-0-50 0-0-30 0-0-40 0-0-90 0-20-20 0-0-20 0-0-110
3.66 3.66 3.66 3.66 3.33 3.66 4 3.66 3.66
0 0 0 0 0 2 0 0 0
5 4 4 4 4.5 4.5 4 4 4.5
Objective assessment (average of points): excellent: 1, very good: 1.1 to 2, good: 2.1 to 2.5, satisfactory: 2.6 to 2.9, unsatisfactory: 3.0 to 5. Electromyographic maximum electric activity was graded: 0—no activity, 1—single potentials, 2—more than single potentials recorded, 3—mixed pattern, baseline visible, 4—submaximal activity, 5—interference pattern. Biceps m. indicates biceps brachii muscle; Brachial m., brachial muscle; EMG, electromyography; PMAJ, pectoralis major muscle; ROM, range of movement.
concerns on 5 patients (9 elbow joints) aged 5 to 9 years (average age, 6.3 y, Table 1) because they met the required criteria, namely: surgery was performed in childhood; and they were followed until skeletal maturity (follow-up, 13 to 16 y). No active flexion was noted and 8 elbows were in full extension, whereas 1 had mild flexion contracture. This elbow (#4.1) had undergone previous surgery; namely pectoralis minor muscle transfer. Four patients underwent bilateral nonsimultaneous unipolar transfers of 3 distal parts of the pectoralis major. Preoperatively, a subjective assessment referred to the ability to feed oneself (reach the mouth with a hand); to comb one’s hair; to attend to toileting needs (using the hand for perineal hygiene); and to dress. For the objective assessment, the range of movement (ROM) and muscle strength of biceps brachii, brachial, and pectoralis major muscles were evaluated. ROM data consisted of goniometric measurements of passive and active antigravity movement of the elbow in the standard position of the shoulder in adduction and neutral rotation. The scapula was not stabilized and it was not allowed to swing the arm. The strength of the muscle was graded with manual muscle testing according to the Medical Research Council grading system.15 A modified evaluation of the results according to Atkins et al10 was used: grade 1— excellent, grade 2—very good, grade 3—good, grade 4— satisfactory, grade 5—unsatisfactory (Table 1). The following criteria were scored: (1) the degree of maximum active elbow flexion; (2) the degree of the extension; and (3) the strength of elbow flexion. Electromyography (EMG) activities of the biceps, brachialis, and pectoralis major muscles were examined using concentric needle electrodes in all patients before surgery, as well as activity of the transferred muscle was evaluated in all patients, 1 to 3 years after surgery for the purposes of the study. Activity at rest was registered and activity at maximum voluntary contracture was graded 0 to 5.16 The surgical procedure includes 1 or 2 steps. In 5 extremities lacking 90 degrees of passive elbow flexion after completing a program of ROM exercises, a posterior elbow capsulotomy with V-Y lengthening of the distal triceps bra-
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chii muscle was performed to achieve passive flexion of over 90 degrees. This procedure was used 4 to 6 weeks before the transfer in 4 elbows or concomitant with the transfer in 1 elbow, respectively. The second step is the transfer of the sternocostal, costal, and abdominal parts of the pectoralis major muscle according to the previously described technique17 and in Figure 1. The transferred muscle was connected: (1) to the remnant of distal tendon of the biceps (1 extremity); (2) to this tendon and to the string of aponeurosis of the common origin of the forearm extensors (3 extremities); (3) to other muscles of the flexor or extensor of the wrist (5 extremities). Their tendons were dissected in the wrist and then pulled subcutaneously anterior from the elbow joint, whereas proximal 3 to 4 cm of muscles remain intact and serve as new insertion for the transmission of power to the forearm. The radial/ulnar flexors were chosen if flexion deformity of the wrist joint predominated in either a radial or ulnar deviation. The extensors were chosen when the extension of the wrist was satisfactory. The same technique was used, when the fixation of the transferred muscle failed. The transferred pectoralis major muscle was fixed at 90 degrees of elbow flexion (Fig. 1B) and tension was classified as appropriate if it allowed a lack of elbow extension of up to 45 degrees. The extremity was fixed in shoulder spica for 4 weeks at 90 degrees of elbow flexion and 20 degrees abduction of the shoulder. The postoperative rehabilitation encompassed the transcutaneous electric stimulation of the transferred muscle and exercises of the elbow and shoulder with isotonic concentric contraction in the first phase (4 to 12 wk). In the second phase, the passive and active exercises of the elbow for a minimum of 15 minutes daily were recommended for the next 3 months. Two specimens (5 10 mm) of the distal medial part of the transferred muscle were harvested during the revision surgery 2 years (#4.2) and 11 years after the primary procedure (#5.1), respectively, and histologically examined. A light microscopy of paraffinmounted sections stained with hematoxylin-eosin was used. r
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FIGURE 1. Pectoralis major transfer in a 5-year-old girl. A, The pectoralis major muscle is bluntly divided between the second and third rib, and then the distal parts of the muscle below the second ribs and below manubrium sterni origin (sternocostal, costal, and abdominal parts) are dissected as a unit from the chest wall and sheet of rectus abdominis muscle in a proximolateral direction (*). Humeral origin of the muscle remains intact (arrow). The subcutaneous tunnel connecting the ventral side of elbow and shoulder is made using the vessel clamp. B, Transferred muscle (*) is passed through the tunnel to the brachium and fixed using fish-mouth technique to the tendon of the flexor carpi ulnaris muscle (thick arrow) in elbow flexion of 90 degrees. Neurovascular bundle of transferred part (thin arrow). M indicates manubrial non-transferred part of the pectoralis major muscle.
RESULTS Complications There were no intraoperative or perioperative complications. Subsequent surgical procedures were necessary in 7 extremities, mostly (5 extremities) the transferred muscle had to be fixed to the alternative tendon of the forearm in the period of 10 to 26 months after the primary procedure. In 1 patient (#5.1), the hypertrophic scar was corrected 1 year after surgery and the tendon of the transferred muscle was lengthened and ventral elbow capsule was released 11 years later for elbow contracture, respectively. In 1 extremity (#1.1), the tendon pulley was r
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reconstructed for an abundant cutaneous fold (pterygium) on the ventral side of the elbow. Before surgery, all extremities were evaluated as unsatisfactory with a score range of 3.33 to 4 points (Table 1). In the subjective assessment according the patients evaluation, 4 extremities improved significantly, 2 extremities improved partially, and 3 extremities remained unchanged (Table 2). In the objective assessment, all extremities achieved the active elbow flexion. The significant improvement of function in 5 extremities (55.5%) includes very good, good, and satisfactory results. One elbow joint was evaluated as very good result, (#1.2) (Figs. 2A–C), 2 extremities (#1.1, #3.2) were evaluated as good, and 2 extremities (#3.1, #5.1) as satisfactory results, respectively. Four extremities were evaluated as unsatisfactory (#2.1, #2.2, #4.1, #4.2). Flexion deformity of 70 to 80 degrees was noted in 4 patients (#3.1, #4.1, #4.2, and #5.1) and developed early within 2 years after surgery in 2 of them (#4.1, # 4.2), whereas 2 (#3.1, #5.1) were noted late in adolescence. The patients with early flexion deformities underwent posterior elbow capsulotomy and triceps lengthening before the transfer. First patient with late flexion deformity (#3.1) required previous triceps lengthening and posterior elbow capsulotomy, and the flexion deformity progressed slowly in adolescence (Fig. 3A, Table 2). Second patient with late flexion deformity was described in complications (#5.1, Fig. 4). He was able to control and transport the items with his hand (Fig. 4), but he was not satisfied with lack of elbow extension and ROM 0-80-100 degrees. Passive extension increased and active flexion decreased after tendon lengthening of the transferred muscle and elbow capsulotomy and the patient subjectively and objectively improved (Table 2). All but one of 4 elbows without posterior release attained very good or good results with a useful arc of motion ranging from 50 to 55 degrees (Table 2, Figs. 2, 3A, B—left elbow), whereas all of 5 elbows requiring a posterior elbow release attained a limited arc of motion of 5 to 20 degrees and in 3 of them the elbow flexion contracture developed (Table 2—#3.1, 4.1, 4.2). The strength of elbow flexion was graded as M4 and M4+ in very good and good results, and M3 and M3+ in satisfactory and unsatisfactory results, respectively. It means all patients flexed the elbows against gravity, but not in a full passive ROM, because passive flexion was obvious in 7 extremities as being increased. Concerning the preoperative EMG, 8 extremities showed complete atrophy and 1 extremity showed incomplete severe atrophy of the biceps and brachialis muscles, respectively. An activity grade of 4 to 5 was registered in all pectoralis major muscles. Postoperative EMG of the transferred muscle corresponded to a partial denervation of the muscle followed by repair of innervation and activity grade 3 to 4+. Histologic examinations (Fig. 5) are interpreted as a partial atrophy of muscle with signs of ongoing regeneration and also the hypertrophy of some muscle fibers. www.pedorthopaedics.com |
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Subjective assessment: 1 point—significant improvement, 2 points—partial improvement, 3 points—no improvement. Objective assessment (average of points): excellent: 1, very good: 1.1 to 2, good: 2.1 to 2.5, satisfactory: 2.6 to 2.9, unsatisfactory: 3.0 to 5. The degree of maximum active flexion in elbow joint was scored as follows: 1 point—over 120 degrees, 2 points—90 to 120 degrees, 3 points—75 to 89 degrees, 4 points—61 to 74 degrees, 5 points—under 60 degrees. The degree of the deficit of extension was scored: 1 point—0 to 5 degrees, 2 points—6 to 35 degrees, 3 points—36 to 50 degrees, 4 points—51 to 70 degrees, 5 points— > 70 degrees. *Range of movement after lengthening of the distal insertion tendon of the pectoralis major muscle and release of elbow at skeletal maturity. AE indicates aponeurosis of extensors; ECRB, extensor carpi radialis brevis muscle; ECRL, extensor carpi radialis longus muscle; FCRL, flexor carpi radialis longus muscle; FCU, flexor carpi ulnaris muscle; MRC, Medical research council grade; PMAJ, pectoralis major muscle; ROM, range of movement; Yes-TS, table support: means support of the elbow using the table .
Yes Yes Yes Yes Yes Yes No No Yes Yes Yes Yes Yes No Yes No No Yes Yes Yes Yes-TS Yes-TS Yes Yes Yes-TS Yes-TS Yes 2.33 2 3.66 3.66 2.66 2.33 3.33 3 2.66 1 1 3 3 2 1 2 2 1 4+ 4+ 3 3 4 4+ 3+ 4 4 0-42-92 0-35-90 0-40-45 0-40-45 0-70-90 0-45-100 0-75-90 0-80-95 0-40-85* Pulley reconstruction 2nd fixation to FCU 2nd fixation to FCU — — 2nd fixation to FCU 2nd fixation to AE 2nd fixation to BRA (1) Scar correction; (2) Elbow-release+PMAJtendon lengthening ECRB Bicep+AE Biceps Biceps+AE ECRL Biceps+AE ECRB FCU FCRL — — During transfer Before transfer Before transfer — Before transfer Before transfer — 16 16 16 14 14 16 15 16 13 1.1 1.2 2.1 2.2 3.1 3.2 4.1 4.2 5.1
Perineal Hygiene Feeding/ Combing Objective Assessment Subjective Assessment Following Procedures
ROM of Elbow Active PMAJ Fixation to Elbow Capsulotomy and Triceps m. Lengthening Study Follow-up No. Period (y)
TABLE 2. Results After the Transfer of Pectoralis Major Muscle at Skeletal Maturity
Flexion Strength MRC (Grade)
Points
Daily Activities
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DISCUSSION The hypotheses of this study were not confirmed because: (1) the transfer of pectoralis major muscle permanently improved the elbow flexion of 5 upper extremities (55%) only; and (2) flexion deformity of the elbow developed in 4 extremities (45%). Global improvement in function was noted in 5 extremities (55.5%). The patients with very good and good outcomes (33%) with active flexion 90 degrees were able to use the extremities for independent self-feeding as well as for perineal hygiene. No excellent results were achieved, because a ROM of the elbow or flexion of over 120 degrees was not obtained. According to literature reports,9 the necessary flexion of elbow is 120 degrees for independence in self-feeding, but it is usually not possible to achieve in arthrogryposis. Two extremities were evaluated as satisfactory, and still remained useful for some daily living activities. Four extremities remained unsatisfactory due to the very limited ROM, they were probably caused by the severe joint contractures of the elbow joint and due to the overall weakness of transferred muscles. The mechanical weakness of muscle was not caused due to the denervation or avascular necrosis of the muscle, which was confirmed by EMG and histologic examinations. But these 4 patients did not describe this result as a deterioration. It can be explained due to the achievement of some arc of active motion and the stabilization of the extremity in the space and using a compensatory mechanism for insufficient elbow flexion. It is clearly visible that the patients with initial passive elbow flexion of over 90 degrees achieved very good and good active ROM, whereas the patients with necessary posterior elbow release achieved only a limited arc of motion, including a greater lack of extension. It is in concordance with the findings of Van Heest et al.7 We can speculate that the subsequent repeated joint contracture develops on the basis of predetermined joint stiffness in these extremities with initial limited elbow motion. It means, the children with adequate passive elbow flexion before transfer will benefit from the pectoralis major muscle transfer, whereas the children with limited motion may not achieve useful arc of motion and flexion contracture will probably develop. The pectoralis major to biceps transfer was chosen because the length of the fibers, the cross-sectional area, and muscle force are similar to the biceps brachii muscle and the muscle is appropriate for splitting on the basis of the segmental anatomy and neurovascular supply.17–19 Clark13 described the transfer of the part of the muscle from the origin on the sixth rib, but this part is too weak and cannot substitute the biceps and brachialis function in arthrogryposis.9,17 Therefore, this technique was modified using the transfer of distal 3/5 of the muscle. The functional sequels for the shoulder are minimal, because 2 proximal parts of the muscle remain intact. Pectoralis major muscle transfer can be used as: (1) a partial unipolar as described above; (2) a partial bipolar, when humeral origin is released and fixed to the acromion and sternocostal part of the muscle is transferred8,9; and (3) a r
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FIGURE 2. The girl with tetramellic form of AMC type I (#1.1, 1.2) at the age of 17.5 years, 13 years after bilateral pectoralis major transfers. A, In anterior view, the extension deficit in elbow joints 30 and 35 are obvious. Cosmetic appearance of breast and chest wall development is not disturbed. B, Ninety-two degrees of active flexion of the right elbow allows to the hand to reach the mouth. C, Ninety degrees of active flexion of the left elbow.
complete bipolar when the whole muscle is released from the chest wall and rotated to the brachium.7,9 Our results are comparable to these techniques only. The results of the small series of these procedures vary in literature reports. Lloyd-Roberts and Lettin20 reported permanent improvement and self-feeding in 6 of 8 patients. They modified Clark’s technique with more distal attachment of the transferred muscle to the ulna, which increases mechanical efficiency. We used a similar technique for the same reasons. Carroll and Kleinman9 described excellent r
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results after a complete bipolar transfer in 3 of 4 patients with a useful arc of motion of 60 degrees and elbow flexion of 120 degrees. In 1 patient with arthrogryposis in their series, the method failed due to fibrofatty degeneration of the muscle. Doyle et al8 reported the independence in feeding in 6 of 7 patients after a partial bipolar procedure. Van Heest et al7 reported the results of 5 patients (1 good, 3 fair, and 1 poor) after Carroll’s modification, and they do not recommend this method. All these authors used the technique for unilateral www.pedorthopaedics.com |
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FIGURE 3. A 17-year-old boy with tetramellic form of AMC type I (#3.1, 3.2). Pectoralis major muscles were transferred in age of 6 (left) and 8 years (right). A, Anterior view 9 years (right) and 11 years (left) after surgery. Extension lack of 60 degrees of the right elbow (after previous posterior capsulotomy and triceps lengthening) and 45 degrees of the left elbow (without capsulotomy and lengthening), respectively. Flexion contracture of right wrist joint is also obvious. B, Active flexion of the left elbow to 100 degrees enables to bring the hand to the face. C, Moderate extension deficit of the left elbow of 45 degrees allows to reach the buttock and perineum with the left hand.
reconstruction and they did not report progressive elbow flexion deformity. This fact supports our opinion that the bilateral reconstruction of the active elbow flexion is possible. The lack of extension to 45 degrees after a bilateral pectoralis major transfer does not interfere with care of the perineum or possibilities for transportation. This opinion is also supported by some authors.21,22 Flexion elbow contractures of 70 to 80 degrees were noted in 4 extremities of our group and led to unsatisfactory or satisfactory results. Lahoti and Bell14 reported a pro-
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gressive flexion deformity of over 90 degrees in 8 of 10 elbows after long-term follow-up after a complete transfer of the muscle originally described by Brooks and Seddon.23 These patients were operated on at children’s age and only 1 had surgery at the age of 14 years and attained good elbow function. The cause of flexion deformity is unclear but it is related to the muscle transfer and the power of the transferred muscle was not deteriorated.14 We can speculate that growth also influences the development of flexion deformity in extremities with predetermined r
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FIGURE 4. A 17.5-year-old boy with bimellic form of AMC type I (#5.1), 11 years after pectoralis major muscle transfer on left side. A, Lack of extension of the elbow 80 degrees. B, Active powerful elbow flexion 95 degrees during elevation of the sand pack.
joint stiffness, whereas after the cessation of growth the flexion deformity will not progress. It is supported by our observation in 2 patients with a bilateral transfer provided at skeletal maturity, where flexion deformity did not increase in the follow-up. Regardless of the surgical modifications, the most critical part of the surgical procedure is the fixation of the transferred muscle to the connective tissue of the forearm, which enables permanent mechanical efficiency. The fixation to the remnant of biceps muscle failed and it was necessary to augment the fixation to the
FIGURE 5. Histologic specimen of transferred pectoralis major muscle harvested 11 years after surgery (#5.1). Moderate increase of fibrofatty tissue (*) and muscle fibers of normal and bigger and smaller diameter are obvious. Muscle fibers with centrally located nucleus represent the regenerating fibers (arrows) (hematoxylin-eosin, magnification: 80). r
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aponeurosis of extensors or tendons of forearm flexors or extensors, respectively. The latissimus dorsi muscle transfer represents a further option.7,21,24 According to our opinion and literature reports,7 its muscle mass can be inadequate or even completely fails in arthrogryposis. Microsurgical transfers of different muscles, primarily gracilis muscle6,25,26 represent very progressive solution. EMG proved useful for providing information on the activity of the biceps and brachialis muscles before surgery and on the contractility of the transferred muscle before and after surgery. It also allowed distinguishing between mechanical failures and atrophying due to denervation or vascular lesions. All transposed muscles revealed changes indicating a minimum denervation followed by reinnervation. Two histologic examinations reveal the sparing of the majority of muscle fibers and very good reinnervation and blood supply of muscle fibers, which were partially damaged. The similar findings in the specimen of the skeletally mature patient confirmed our assumption that the transferred muscle with intact innervation and vascular supply can grow and change according to the functional rights.27 Van Heest et al28 reported the largest series of 23 patients with extension contracture using the posterior elbow capsulotomy with triceps lengthening, which enable the passive flexion of the elbow using the passive assistance. According to our opinion, the patients with arthrogryposis may benefit from pectoralis major to biceps transfer more than from solely the restoration of passive elbow flexion, because the active flexion is useful for daily living activities and for the stabilization of the extremity in the space. It remains a controversial question www.pedorthopaedics.com |
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if any muscle transfer for active elbow flexion should be used in elbows with limited passive flexion, when we can expect the lack of elbow extension and/or limited arc of motion during growth, or in other words, to sacrify the elbow extension. According to our opinion the active flexion of 90 degrees and adequate strength of the transferred muscle allow the hand to reach the head and to elevate the items of adequate weight despite the lack of elbow extension. If the patient is not satisfied with the lack of extension, it is possible to increase the extension due to the lengthening of the tendon of the transferred muscle, but elbow flexion will decrease. Limitation of arc of motion should be prevented due to the regular and intensive rehabilitation. According these findings we changed our treatment strategy. Formerly, we preferred the bilateral restoration of active elbow flexion in all patients irrespective of the necessity of posterior elbow release. Currently, we recommend the bilateral pectoralis major transfer in patients with native passive elbow flexion without the necessity of posterior elbow release. In patients with initial limited passive elbow flexion, we recommend now to restore active elbow flexion on one side (preferably with greater arc of motion), whereas the extremity of the opposite side without transfer can be used for perineal hygiene. The parents should be informed about the probability of the lack of extension on the operated side and possible limitation of the arc of movement during the growth. Another possibility would be to postpone the restoration of active elbow flexion to the age of growth cessation, which was confirmed in our observations and literature reports.14 The rehabilitation represents an important part of the treatment strategy and requires the cooperation of the patient. Therefore, we recommend starting the surgical procedure in the fifth year of life. It is in concordance with Van Heest et al,7 whereas Mennen29 recommended corrective surgery in 3 to 6 months of age. We suggest that the regular passive and active exercises of the elbow joint to the extension are necessary to prevent elbow flexion contracture in any age after this muscle transfer. With regard to the cosmetic appearance after the pectoralis major transfer, we modified 1 incision technique due to 2 shorter incisions technique. Neither modification disturbed cosmetic appearance of breast or chest wall development in any females in our study (Fig. 2). This finding is in accordance with literature reports,7 but we did not study the morphology and function of the breasts particularly. This study has the following strengths. It provides mid-term to long-term follow-up data in the prospective study of patients with arthrogryposis. The number of patients is limited but comparable with those from the literature reports, because of the rare condition. The described technique was based on the anatomic study of the neurovascular supply of the muscle.17 EMG examinations preoperatively and postoperatively and the rehabilitation regimen were unified. The viability of the transferred muscle is supported also due to 2 histologic examinations.
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The study has the following limitations: 2 surgeons in 1 institution treated the patients, so the effects of institutional bias may be possible; the data of shoulder and wrist and fingers were not particularly analyzed; and no data of control group of patients were available. In conclusion, unipolar transfer of 3 distal parts of pectoralis major muscle may establish bilateral permanent effective elbow flexion by retaining sufficient extension for perineal hygiene in patients with arthrogryposis. In patients with the necessity of posterior elbow release, this transfer is only partially effective with possible subsequent elbow flexion deformity. In these cases we recommend to restore active elbow flexion on 1 side. REFERENCES 1. Hall JG. Artrogryposis multiplex congenita: aetiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop Part B. 1997;6:159–166. 2. Sarwark JF, McEwen GD, Scott CI. Current concepts review. Amyoplasia: a common form of arthrogryposis. J Bone Joint Surg Am. 1990;72:465–469. 3. Brown LM, Robson MJ, Sharrard WJW. The pathophysiology of arthrogryposis multiplex congenita neurologica. J Bone Joint Surg Br. 1980;62:291–296. 4. Ezaki M. Treatment of the upper limb in the child with arthrogryposis. Hand Clin. 2000;16:703–711. 5. So¨dergard J, Hakamies-Blomqvist L, Sainio K, et al. Arthrogryposis multiplex congenita: perinatal and electromyographic findings, disability, and psychosocial outcome. J Pediatr Orthop Part B. 1997; 6:167–171. 6. Kay S, Pinder R, Wiper J, et al. Microvascular free functioning gracilis transfer with nerve transfer to establish elbow flexion. J Plast Reconstr Aesthet Surg. 2010;63:1142–1149. 7. Van Heest A, Waters PM, Simmons BP. Surgical treatment of arthrogryposis of the elbow. J Hand Surg Am. 1998;23:1063–1070. 8. Doyle JR, James PM, Larsen LJ, et al. Restoration of elbow flexion in arthrogryposis multiplex congenita. J Hand Surg. 1980;5:149–152. 9. Carroll RE, Kleinman WB. Pectoralis major transplantation to restore elbow flexion to the paralytic limb. J Hand Surg. 1979;4:501–507. 10. Atkins RM, Bell MJ, Sharrard WJW. Pectoralis major transfer for paralysis of elbow flexion in children. J Bone Joint Surg Br. 1985; 67:640–644. 11. Tsai TM, Kalisman M, Burns J, et al. Restoration of elbow flexion by pectoralis major and pectoralis minor transfer. J Hand Surg. 1983;8:186–190. 12. Gogola GR, Ezaki M, Oishi SN, et al. Long head of the triceps muscle transfer for active elbow flexion in arthrogryposis. Tech Hand Up Extrem Surg. 2010;14:121–124. 13. Clark JMP. Reconstruction of biceps brachii by pectoral muscle transplantation. Br J Surg. 1946;34:180–181. 14. Lahoti O, Bell MJ. Transfer of pectoralis major in arthrogryposis to restore elbow flexion. Deteriorating results in the long term. J Bone Joint Surg Br. 2005;87:858–860. 15. Medical Research Council. War Memorandum No.7. II ed. London: Her Majesty’s Stationery Office; 1943. 16. Ludin H-P. Electromyography in Practice. Stuttgart, New York: Thieme; 1980. 17. Chomiak J, Dungl P. Reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. Part I: surgical anatomy and vascular and nerve supply of the pectoralis major muscle as a basis for muscle transfer. J Child Orthop. 2008;2:357–364. 18. Chaffai MA, Mansat M. Anatomic basis for the constructions of a musculotendinous flap derived from the pectoralis major muscle. Surg Radiol Anat. 1988;10:273–282. 19. Nakajima K, Die Y, Abe S, et al. Anatomical study of the pectoral branch of thoracoacromial artery. Bull Tokyo Dent Coll. 1997;38:207–215. r
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Volume 34, Number 8, December 2014
20. Lloyd-Roberts GC, Lettin AWF. Arthrogryposis multiplex congenita. J Bone Joint Surg Br. 1970;52:494–508. 21. Maila¨nder P, Machens HG, Rieck B, et al. Bilateral restoration of elbow flexion by bipolar transposition of latissimus dorsi in arthrogryposis multiplex congenita: A Case Report. Handchir Mikrochir Plast Chir. 1996;28:59–63. 22. Martin G, Perrot P, Duteille F. Treatment of congenital deficit of elbow flexion in a case of arthrogryposis. Chirurgie de la main. 2009;28:116–119. 23. Brooks DM, Seddon HJ. Pectoral transplantation for paralysis of the flexors of the elbow. J Bone Joint Surg Br. 1959;41:36–43. 24. Gagnon E, Fogelson N, Seyfer AE. Use of the latissimus dorsi muscle to restore elbow flexion in arthrogryposis. Plast Reconstr Surg. 2000;106:1582–1585. 25. Chuang DCC, Carver N, Wie FC. Results of functioning free muscle transplantation for elbow flexion. J Hand Surg Am. 1996;21: 1071–1077.
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26. Doi K, Muramatsu K, Hattori Y, et al. Restoration of prehension with double free muscle technique following complete avulsion of the brachial plexus: indications and long-term results. J Bone Joint Surg Am. 2000;82:652–666. 27. Grim M, Dittertova´ L, Vejsada R, et al. Experimental and anatomical basis for reconstruction of anal musculature employing gracilis muscle graft with intact vascular supply. In: Freilinger G, Holle J, Carlson BM, eds. Muscle Transplantation. Wien, New York: Springer; 1981:265–274. 28. Van Heest A, James MA, Lewica A, et al. Posterior elbow capsulotomy with triceps lengthening for treatment of elbow extension contracture in children with arthrogryposis. J Bone Joint Surg Am. 2008;90:1517–1523. 29. Mennen U. Early corrective surgery for the wrist and elbow in arthrogryposis multiplex congenita. J Hand Surg Br. 1993;18: 304–307.
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