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24 Palliative Surgery inObstetrical Brachial Plexus Palsy
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Fig. 24.3 Coracohumeral ligament is exposed before resection. Ligament resection allows the shoulder joint to achieve an increased external rotation
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Unfortunately,subscapularis muscle releasefails inabout 50% of cases [13].
Although few reports have been published about a comparison of procedures,our impression is that coraco-humeral release seems to be more effective than subscapularis release. According to Gilbert’s observations, the latter procedure seems to be inefcient after 4 years of age. In older chil­dren, subscapularis muscle release might repre­sent one step of a multistage procedure, but we advise against using it as the rst choice treatment at major age. On the other hand, the coraco­humeral release may be performed during the rst years of life to obtain room for the motion of the humeral head that is expected to remodel during the residual growth(Fig. 24.3).
Preventive X-ray exploration to identify humeral head deformation is advisable, while at older ages, MRI (magnetic resonance imaging) and CT (computed tomography) scans are man­datory to conrm joint congruence.
Nerve transfer, as described above in very young children, might be a successful solution in addition to shoulder joint release. At any rate, whichever surgery might be used, a period of 3–6 weeks of immobilization in a brace or cast with the shoulder positioned in the abduction and external rotation is needed to allow the transfer (nervous or tendinous) to be stabilized.
From four to ten years of age, lack of active external rotation can be corrected by latissimus
dorsi/teres major transfer to the rotatory cuff, as a rst choice [8, 14].
In our experience, combined coracohumeral releaseand latissimus dorsi/teres major transfer can be effective, when residual stiffness of the shoulder is detected (Fig.24.3).
When bone growth of proximal humeral phy­sis is about to stop, approximately from ten years on, derotational osteotomy and plating at the upper third ofhumeral shaft in external rotation, performed above pectoralis major insertions,can be helpful tocorrect the internal rotational defect and compensate the anteposition of the shoulder girdle, additionally making up for cosmetic appearance [15, 16]. Muscle transfers, that is, latissimus dorsi muscle, can be added to rein­force motion (Fig.24.4).
It is extremely important to perform a mild derotation of the bone stumps avoiding an exten­sive derotationover25°topreventfrom a loss of motion in internal rotation. Excessive derotation limits the capability to reach the median line of the body with the hand.
During growth, some patients who developed a severe defect of shoulder external rotation show a typical pattern with internal rotation of the shoul­der and progressive exion deformity of the elbow. After humeral derotation procedure, parents must be informed of the risk of losing some degrees of internal rotation because of the achievement of the same amount of degrees in external rotation.
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a
Fig. 24.4 (a, b) Derotational osteotomy of humeral prox- imal metaphysis provides a satisfactory improvement of external rotation motion of the shoulder, as is shown in
preoperative (a) and postoperative images (b). X-ray images conrm the deformation of the humeral proximal epiphysis
24 Palliative Surgery inObstetrical Brachial Plexus Palsy
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b
Fig. 24.4 (continued)
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24.2.1.2 Limited Abduction
Limited abduction of the shoulder is one of the most common features of OBPP. There are dif­ferent degrees of disability, so correction is requiredaccording to the severity of lesion.
Lack of abduction of the shoulder usually stems from different factors, above all insuf­cient deltoid muscle strength, but it can also be due to incongruence of the shoulder joint and unbalance of shoulder girdle muscles, particu­larly for the predominance of internal rotators.
During the rst years of life, latissimus dorsi muscle transfer permits to attain a satisfactory external rotation along with a slight increasing of abduction. Inevitably, a loss of this minimal amount ofabduction occurs during the following years, butthistransfer is helpful for the develop­ment of the scapulo-humeral joint, favoring joint congruence and avoiding posterior dislocation of the humeral epiphysis.
Late nerve surgery throughtransfer of nerve branches of the long head of the triceps muscle to axillary muscle in very young children is a recent proposal which, however, is still debated
on the risk of impair a relatively good triceps muscle [17].
In the event of severe lack of abduction, from six to ten years of age, trapezius muscle trans­ferred onto the deltoid insertion can be a good solution, even though only a partial recovery of function is expected. In this procedure, a perios­teal strip is elevated and sutured onto the deltoid insertion without skeletal anchorage to avoid pro­gressive tightening of the transferred muscle dur­ing further growth. Excessive tension of transferred muscle results in abduction contrac­ture with additional scapulohumeral stiffness. From 10 to 16 years of age, trapezius muscle transfer with olecranon graft xed into humeral diaphysis is more appropriate.
Trapezius muscle transfer is effective for bal­ancing a weak shoulder, particularly when com­bined with other muscle transfers, even though a limited amount of abduction is achieved [1820].
In young adults, shoulder joint fusion is another option to better stabilize the scapulo­humeral girdle.
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24.2.1.3 Limited Internal Rotation
A typical feature of the defect is the incapability of reaching the median line of the body with the hand. This defect is far more limiting than the lack of external rotation because it has a severe impact on many basic daily functions. It is com­monly observed during growth, but it becomes more limiting when other defects of motion are associated (Fig.24.5).
Generally,the scapulohumeral girdle appears hypoplastic and placedin a forward position with a twisted clavicle. Scapular winging is always present, sometimes extremely evident.
Very often patients present witha lack of both external and internal rotations because of gleno­humeral incongruence. Some patients show a paradoxical pattern in forced external rotation of
Fig. 24.5 During growth, this child developed a singular pattern in shoulder rotation. Despite a good external rotation, there is an evident lack of internal rotation that limits many basic daily functions
the shoulder with no ability to internally rotate the upper arm.
Before ten years of age, coracoid process resection and subscapularis muscle release to reduce scapular winging might be helpful. Sometimes rhomboid muscle plication can reduce shoulder blade axis rotation.
From ten years of age on, internal derotational osteotomy of the humerus below pectoralis major insertion is a more advisable procedure that allows the patient to improve internal rotation and reach the anterior midline of the body with the hand. Similar to external derotational osteotomy, exces­sive internal derotation might be dangerous to the risk of losing the opposite function. Pectoralis muscle insertion laterally xed onto the humeral shaft can help improve the internal rotation.
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Retroposition is a complex combination of abduction and internal rotation of the shoulder. Retropositional defect is a constant feature of upper palsy that is usually treated through physi­cal therapy, but rarely, it can be restored.
24.2.1.4 Posterior Subluxation
It usually occurs in adolescents with an internal rotational pattern, namely, hypoplastic development of the shoulder, modication of acromion, clavicle, coracoid process, and glenoid cavity [21] (Fig.24.6).
The defect is due to incongruence between the anomalous shaped humeral head andthe glenoid cavity. Hyperlaxity of the joint and muscle palsy trigger backward dislocation of the humeral head (Fig.24.7).
Derotational osteotomy of the humeral shaft along with coracohumeral ligament release or coracoid resection and posterior capsuloplasty with or without glenoid osteotomy of re­orientation can help correct the instability [22].
These procedures are inconstantly rewarding because of the possible recurrence of deformity.
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Fig. 24.7 Shoulder clinical evaluation demonstrates a severe posterior dislocation of the humeral head
Fig. 24.6 During growth, a large group of patients pres­ent with severe skeletal deformities of the shoulder joint. Glenoid fossa dysplasia, coracoid process hypertrophy,
and proximal humeral head dislocation signicantly impair shoulder motion
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24.2.2 Elbow
Impairment of elbow joint motion is another basic point that interferes with the outcome. Different from the shoulder which acts on multi­ple planes, the elbow has two axes of motion, namely, exion and extension. Pronation and supination of the forearm are inuenced by elbow position even though they do not take part in elbow motion.
Elbow joint morphology is altered by muscle imbalance, forced position of the elbow that causes the joint deformity, loss of perfect congru­ence, and growth changes due to physeal plate asymmetrical stimulation.
A weak elbowcan present with twodifferent patterns.The main important is due to palsy of the biceps muscle which stems from nerve palsy at birth, sometimes with partial strength recovery.
Triceps muscle palsy represents the opposit patternbut the predominance of the bicepsmus­cleparadoxally causes a exion deformity.
This exion deformity posture is typical of upper-intermediate palsy in which internal rota­tionpositioning of the shoulder and elbow ex­ion due to retraction of reinnervated biceps muscle results in a lack of elbow extension.
Elbow deformities are commonly assessed by the Raimondi and Gilbert’s classication, which scores the severity of elbow limitation in degrees of exion or extension.
24.2.2.1 Lack ofActive Elbow Flexion
Lack of active elbow exion is a typical feature in the majority of children presenting with OBPP in the rst months of life, even though it tends to decrease during the rst year of life. Its persis­tence is a typical feature of severe forms of neo­natal palsy; currently, these patients are usually enrolled as candidates for early nerve surgery. However, older children lacking elbow exion might be observed later in life, or they are excep­tionally suffering sequela of unsuccessful bra­chial plexus surgery.
Palliative surgery can offer options for cor­recting the defect, but the number of procedures is limited.
Recent publications about nerve transfers in adults have shown the options to apply the same procedure to the child. Innervation of the biceps and/or brachialis muscle in the rst years of life can be successful when proper indications are followed [23]. Active elbow exion can be obtained through different nerve trunk transfers depending on the level of injury. In the event of a C5–C6 palsy, fascicles of the ulnar nerve assigned to the exor carpi ulnaris can be transferred onto muscle cutaneous nerve or rather to biceps or brachialis muscle branches to achieve active elbow exion. When there is C7 involvement or in the event of a partially recovered complete neonatal palsy, transfer of intercostal nerves con­nected to muscle cutaneous nerve trunk or its fas­cicles represents a better option to obtain elbow exion [24].
In already grown children, elbow exion can be achieved through pedicled muscle transfers (i.e., pectoralis major or minor, triceps, latissi­mus dorsi muscles) as well as using the classical Steindler procedure. However, very often local muscles are weak and unable to restore function [25].
Free muscle transfers (i.e., free vascularized gracilis muscle transfer) are another option, par­ticularly in younger children when the lever arm of the elbow is favorable and body weight is lim­ited [26, 27].
24.2.2.2 Lack ofElbow Extension
Although lack of elbow extension is a severe defect impairing upper limb function, lack of elbow active extension is thought to be partially compensated by gravitational forces (Fig.24.8). Nevertheless, the involvement of the C7 root and posterior cord of the brachial plexus is not negli­gible. When posterior trunk involvement is seen in severely affected children, early nerve surgery is the correct indication. Conversely, indication for surgery might be more complicated when a
24 Palliative Surgery inObstetrical Brachial Plexus Palsy
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Fig. 24.8 Elbow extension limitation is constantly evi­dent in the upper palsy. The deformity increases during growth impairing elbow motion and determining cosmetic problems in the patients
dissociated recovery of OBPP causes an isolated triceps muscle palsy or when the child is observed late.
In young children, nerve transfers can help restore triceps muscle activity, essential for con­trasting biceps muscle exion and avoiding pro­gressive deformity of the joint, as described above. There is no general agreement about late nerve transfer and which nerve might be transferred.
Fascicles of the ulnar nerve assigned to the exor carpi ulnaris can be transferred onto the radial nerve or rather on the branches of the long head of the triceps muscle to achieve active elbow extension. When there is C7 involvement or in the event of a partially recovered neonatal com­plete palsy, intercostal nerves connected to the
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radial nerve trunk or its fascicles can induce rein­nervation of the triceps muscle. In already grown children, elbow extension can be obtained through pedicled muscles (latissimus dorsi mus­cle, deltoid muscle) [28].
24.2.2.3 Flexion Deformity forLimited Extension
A exed attitude of the elbow with limitation of elbow extension is the commonest pattern, usu­ally detected in the majority of patients present­ing with OBPP sequelae. Flexion degree increases during growth, particularly during adolescence. Incidentally, patients particularly complain about this defect for functional and cosmetic reasons.
Progressive exion degree of the elbow joint can reach >90°, particularly when a strong biceps muscle is not opposed by a weak triceps muscle. In this condition, elbow joint geometry is modied by muscle imbalance and loss of articular congruency. As a consequence, there is an alteration of the growing elbow, particu­larly the olecranon process that grows unhin­dered. These modications result in a further decrease in motion due to the forced position of the elbow and physeal plate asymmetrical stim­ulation [29, 30].
Since the surgical correction of the defect is challenging, prevention is always attempted as the rst step. Physical therapy can be effective in preventing worsening deformity. Repetition of exercises to increase the range of motion in ex­ion and extension along with prono-supination of the forearm permits to avoid increased exed deformity of the elbow.
Static or dynamic nocturnal orthoses can be useful in keeping the elbow in extension, relaxing muscles and reducing joint stiffness. These devices are barely toleratedby toddlers. For this reason, progressive application of casts has been advised along with botulin toxin injections into the brachialis or internal rotator muscles of the shoulder to reduce the progression of exion deformity [31].
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At any rate, a correct approach to the shoulder represents in itself prevention against elbow pro­gressive deformation.
Wheneverelbow exion appears severely lim­iting, surgery may be requiredforboth functional and cosmetic purposes.
In the literature, biceps tendon lengthening, brachialis muscle release, and anterior open joint capsulotomy have been proposed during growth, even though recurrence of the deformity have been often reported. Furthermore, biceps muscle lengthening entails the risk of losing muscle strength that had undergone reinnervation during the rst years of life [32].
A posterior wedged osteotomy in the distal humerus could obtain a satisfactory extension of
the elbow, with the risk, however, of losing active exion due to the modied joint angles.
External xators have been proposed; how­ever, they entail the risk of infections, stretching of palsied structures, stiffness, etc. [33].
At the time being, there are no denite proce­dures to completely correct the elbow.
Observing the pitfalls of the other techniques, we published an original procedure including both anteromedial incisions of elbow joint cuff along with brachialis aponeurosis incision (leav­ing biceps tendon untouched) and posteriorly a partial resection of olecranon tip so to increase the passive motion of the elbow. These two steps have been performed to remove mechanical obstacles on opposite sides of the elbow joint (Fig.24.9).
Fig. 24.9 Partial olecranon resection and anterior capsu­lotomy allow the exed elbow to achieve a larger range of motion without losing exion degrees. CT scan shows an
increased length of the acromion, while X-ray pictures give details of the level of olecranon resection
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Fig. 24.9 (continued)
A mean increase of approximately 25° in elbow extension has been constantly reached. The results allow a good combination of func­tional and cosmetic advantages with the satisfac­tion of patients. The appropriate timing of the procedure is during adolescence when growth changes of the elbow joint do not lead to a recur­rence of the deformity [34].
24.2.3 Forearm
The forearm is consistently involved in OBPP, both upper-intermediate and complete, showing different features according to palsy recovery.
Upper palsy presents with a pronation atti­tude, whereas severe intermediate and complete palsy usually shows a supinated posture.
24.2.3.1 Defect ofSupination or Pronator Deformity
Pronator deformity is a typical upper trunk palsy sequela. The deformity stems from the imbalance of forearm pronator muscles which are active, whereas supinator muscles are palsied.
Apart from cases in which severe stiffness of the shoulder and elbow worsens forearm pronator deformity, a mild lack of supination is usually well tolerated. A good range of motion of the shoulder makes up adequately for the defect. Conversely, as the shoulder does not take part in forearmpronation movements, a lack of prona­tion cannot be compensated.
Physical therapy, taping, and orthosis in the intermediate position are conventional methods of treatment.
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Inconstantly, skeletal deformities and interos­seous membrane retraction cause more severe pronation of the forearm. A deformity exceeding two-thirds of xed pronation requires surgical correction.
Uncommonly, subluxation of radial epiphysis in a backward position generally occurs in the oldest patients in whom uncontrolled growth of the radial epiphysis impinges on the distal part of humeral meta-epiphysis, further reducing fore­arm motion.
Someliterature reports haveproposed tendon releases and transfers to achieve better supination [35]. Some of them can be effective in the young­est patients; however, forced pronation in a much grownchildren and teensrequires a derotational osteotomy. According to the age, Kirschner wire xation and molded cast are adequate methods of xation, whereas in older children, xation by plate and screws is needed.
Currently, osteoclasis in very young children is an exceptional indication.
Radial head resection is appropriate for achieving a passive motion of the forearm and is commonly performed in late adolescence for severe limitation of pronation movement.
Although ten years of age has been identied as a hypothetical limit for performing soft tissue or skeletal procedures, in our opinion, timing of surgery should be established on a case-by-case basis.
24.2.3.2 Defect ofPronation or
Supination Deformity
Supination deformity is a common feature of complete or intermediate palsy with severe impairment of the C7 root.
The typical appearance of the upper arm with a exed elbow, supinated forearm, and hyper­extended ail wrist springs from the dynamic imbalance among active supinator muscles, that is, biceps brachialis muscle and palsied pronator muscles. Since opposite forces are stemming from contracted muscles and gravitational forces of the elbow joint are not controlled by palsied muscles, further joint instability, namely radial
head subluxation, consistently arises.Soft tissue defects, such ascontracture and shortening of the interosseous membrane, biceps brachialis, supi­nator teres, and dorsal ligament of the distal radioulnar joint are worsened byskeletal defor­mities of bone and joints, namely, anterior sub­luxation of the proximal radial epiphysis and distal ulnar epiphysis subluxation.
Forearm xed deformity, radial dislocation, and hand functionempowering are the main indi­cators for surgery.
Surgical correction of forearm xed supina­tion is achieved using a tight interosseous mem­brane and joint capsule release to obtain a reduction of capital radial head subluxation. When the forearm can easily reach pronation, a Z lengthening of the biceps muscle insertional ten­don at the radius is performed. The distal stump of the tendon is then rerouted around the radial metaphysis and sutured in adequate tension to obtain pronation motion when the elbow exes [36, 37].
In the event of sufcient mobility of the two bones of the forearm, the rst step can be skipped proceeding directly to biceps rerouting.
Surgical treatment is required in the event of progressive dislocation of the radial epiphysis at 4–6 years of age.
Hand impairment reduces chances for recov­ery, even though a correction of xed supination provides chances for empowering some muscles of the wrist or the hand.
Many authors have proposed forearm osteot­omy, reporting good cosmetically and function­ally results in the oldest children [3840].
When severe instability further impairs the chances of using the palsied forearm, radioulnar proximal fusion gives stability to the forearm, which, however, maintains poor function [41].
24.2.4 Wrist andHand
Wrist and hand involvements are frequent in chil­dren suffering from OBPP sequelae, particularly in extensive paralysis.