REGIONAL SURVEY

Upper limb
Upper limb deformities are seen most typically in the child with spastic hemiplegia or total body involvement and consist of exion of the elbow, pronation of the forearm, exion of the wrist, clenched ngers and adduction of the thumb. In the mildest cases, spastic postures emerge only during exacting activities. Proprioception is often disturbed and this may preclude any marked improvement of function, whatever the kind of treatment. Operative treatment is usually delayed until after the age of 8 years and is aimed at improving the resting position of the limb and restoring grasp. Provided the elbow can extend to a right angle, no treatment is needed. Occasionally it may be necessary to treat a more marked exion contracture by fractional lengthening of the biceps and brachialis tendons with release of the brachialis origin.
Elbow exion deformity Forearm pronation deformity This is fairly common and may give rise to subluxation or dislocation of the radial head. Simple release of pronator teres may improve the position, or the tendon can be rerouted round the back of the forearm in the hope that it may act as a supinator. Wrist exion deformity

thenar muscles, followed by tendon transfers to reinforce abduction and extension. Here again the operations should be performed by a specialist in this eld.

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Lower limb
The functional effects of lower limb spasticity differ considerably, depending on whether the patient has hemiplegia, diplegia or whole-body involvement; this will obviously inuence the lines of surgical treatment. SPASTIC HEMIPLEGIA Four subtypes of hemiplegia have been identied and the most common lower limb problem is with foot deformity.
Foot/ankle Tibialis anterior is invariably weak and the

Neuromuscular disorders

Wrist exion is usually in an ulnar direction; it can be improved by lengthening or releasing exor carpi ulnaris. If extension is weak, the released exor tendon is transferred into one of the wrist extensors. In severe cases wrist arthrodesis with excision of the proximal carpal row may be of cosmetic rather than functional benet. N.B. Before operating on the wrist it is essential to consider what effect this will have on nger movements.

patient develops an equinovarus foot deformity. Active plantar exion is required to assist knee extension during the stance phase of gait so care must be taken when considering a lengthening of the gastrocnemius/soleus complex. The trend is to perform a muscle recession rather than a tendon lengthening procedure. A dynamic varus deformity can be treated by a split tibialis anterior tendon transfer to the outer side of the foot (only half the tendon is transferred so as to avoid the risk of overcorrection into valgus). In older children with xed deformity, formal muscle lengthening with or without a calcaneal osteotomy may be required. Pes valgus (pronated foot deformity) may require subtalar arthrodesis.
Hip/knee Surgery is not usually required but if it is it

follows the principles outlined below for the walking diplegic patient.
Leg length discrepancy Due to discrepancies in growth, the hemiplegic limb is often short irrespective of any joint contractures. An epiphyseodesis of the contralateral distal femoral and/or proximal tibial physes may be considered. This can improve some aspects of the gait pattern.

Flexion deformity of the ngers Spasticity of the long

exor muscles may give rise to clawing. The exor tendons can be lengthened individually, but if the deformity is severe a forearm muscle slide may be more appropriate. Ideally these operations should be undertaken by a specialist in hand surgery. If the ngers can be unclenched only by simultaneously exing the wrist, it is obviously important not to extend the wrist by tendon transfer or fusion.
Thumb-in-palm deformity This is due to spasticity of the thumb adductors or exors (or both), but later there is also contracture of exor pollicis longus. In mild cases, function can be improved by splinting the thumb away from the palm, or by operative release of the adductor pollicis and rst dorsal interosseus muscles. Resistant deformity may need combined lengthening of exor pollicis longus and release of the

SPASTIC DIPLEGIA Most patients with cerebral palsy have a spastic diplegia and treatment is concentrated on the lower limbs. In the very young child, this consists of physiotherapy and splintage to prevent xed contractures. Surgery is indicated either to correct structural defects (e.g. a xed contracture or hip subluxation) or to improve gait. By 34 years of age the sitting and walking patterns can be observed, and particular attention should be paid to the interrelationship between the various postural defects, especially lumbar lordosis/hip exion and knee exion/ankle equinus. Most children will walk but they are delayed in learning to master this a child who is not walking by

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GENERAL ORTHOPAEDICS

the age of 6 or 7 is unlikely to do so. Non-ambulant children often have orthopaedic problems similar to those with total body involvement (see below). In walking diplegics, observational gait analysis is important and computerized gait analysis may have a role in guiding treatment. Affected children are often relatively symmetrical in their gait pattern but in some asymmetry is very marked with one limb maintaining a hemiplegic posture and one more consistent with a diplegic gait. Each limb must be assessed independently.
Hip adduction deformity The child walks with the

thighs together and sometimes even with the knees crossing (scissors gait). This may be combined with spastic internal rotation. Adductor release is indicated if passive abduction is less than 20 degrees on each side. If medial hamstring lengthening is planned (see below) it should be done rst because this alone may restore some hip abduction. For most patients open tenotomy of adductor longus and division of gracilis will sufce. Only if this fails to restore passive abduction (a rare occurrence) should the other adductors be released. Anterior branch obturator neurectomy should not be performed.
Hip exion deformity This is often associated with xed

10.13 Spastic hips X-ray of a boy with spastic adducted hips showing acetabular dysplasia and coxa valga, worse on the left side.

bined with acetabular reconstruction. Longstanding dislocation in a non-walker may be impossible to reconstruct; if discomfort makes operation imperative, the proximal end of the femur can be excised. In the adult walking diplegic patient, total hip replacement can be considered in selected cases where painful degenerative change is affecting function.
Knee exion deformity This is one of the commonest

knee exion (the child walks with a sitting posture) or else hyperextension of the lumbar spine. Operative correction is indicated if the hip deformity is more than 30 degrees. In the walking child, it is important not to weaken hip exion too much and thus intramuscular lengthening of the psoas tendon at the pelvic brim is advocated. (In the non-walking child, psoas release at the level of the lesser trochanter is allowed). An associated xed exion deformity of the knee may require medial hamstring lengthening as well.
Hip internal rotation deformity

deformities; it is usually due to functional hamstring tightness but is often aggravated by hip exion or weakness of ankle plantar exion. Spastic exion deformity may be revealed only when the hip is exed to 90 degrees so that the hamstrings are tightened.

Internal rotation is usually associated with exion and adduction. If so, adductor release and psoas lengthening will be helpful. If, after a few years, rotation is still excessive, a derotation osteotomy of the femur (subtrochanteric or supracondylar) may be considered; however, be warned that this may have to be followed by compensatory rotation osteotomy of the tibia.

Hip subluxation Subluxation of the hip occurs in

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about 30 per cent of children with cerebral palsy. A persistent exion-adduction deformity leads to femoral neck anteversion. If the abductors are weak and the child is not fully weightbearing, there is a risk of acetabular dysplasia and subluxation of the joint; in non-walkers there may be complete dislocation. Correction of exion and adduction deformities (see above) before the age of 6 years may have a role in preventing subluxation. Older children may need varusderotation osteotomy of the femur, perhaps com-

(a)

(b)

10.14 Spastic knee exion deformity (a) This boy has spastic exion of the knees due to tight hamstrings. (b) Here he is after hamstring release.

Capsular contracture of the knee joint is uncommon. Gait analysis can be helpful in deciding whether the hamstrings are truly short or only functionally short. Fractional lengthening of the hamstrings (medial more often than medial and lateral combined) reliably improves gait mechanics but risks weakening hip extension and exacerbating hip exion/lumbar lordosis; this is because the hamstrings normally assist with hip extension. Fractional lengthening of semimembranosus can be combined with detachment and transfer of semitendinosus to the adductor tubercle at the distal end of the femur. Good results have been reported by Ma et al. (2006) in children with bilateral spastic exion deformities of more than 15 degrees combined with a exed-knee posture when standing or walking and ability to stand and walk only with support. Severe exion deformities (more than 25 or 30 degrees) have also been treated by extension osteotomy of the distal femur or by physeal plating anteriorly. Remember that knee extension is aided by plantarexion of the foot in walking, so it is important not to weaken the triceps surae by overzealous lengthening of the Achilles tendon (see below).
Spastic knee extension This can usually be corrected by simple tenotomy of the proximal end of rectus femoris. External tibial torsion This is easily corrected by supramalleolar osteotomy, but before doing this rst ensure that the deformity is not actually advantageous in compensating for an ankle/hindfoot deformity (see below). Equinus of the foot

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Neuromuscular disorders

(a)

(b)

10.15 Spastic equinus (a) Standing posture of a young girl with bilateral spastic equinus deformities. (b) Tendo Achillis lengthening resulted in complete correction and a balanced posture.

lengthening and plication of the medial structures when appropriate. External tibial torsion may be corrected by a supramalleolar osteotomy but remember that an externally rotated gait pattern may be compensating for an inability of the foot to clear the ground when walking because of weak muscles/stiff joints.
Single event multi-level surgery (SEMLS) The diplegic patient usually has problems at all levels and often the most appropriate way to improve gait and overall function is to enhance the mechanical efciency of gait by combining changes at hip, knee and ankle. Soft-tissue and bony surgery to both limbs can be performed at one sitting or staged over a few weeks. Postoperative rehabilitation is complex and time-consuming but the results can be very rewarding. A good review of management of lower limb deformities in children with cerebral palsy is presented by Karol (2004).

The child with spastic diplegia usually toe-walks. This triggers an excessive plantarexionknee extension couple that may be manifested as knee hyperextension. In children with limited dorsiexion, the gastrocnemius is often more affected than the soleus. Selective fractional lengthening of the fascia/muscle is gaining favour but judicious percutaneous lengthening of the Achilles tendon is still popular. Relative overlengthening is a problem, particularly when associated knee exion contractures exist. If a varus deformity is present, treatment is as for the hemiplegic patient described above. The more common deformity is, however, one of equinovalgus and a rocker-bottom foot. It makes the use of splints difcult and disrupts the plantarexionknee extension couple, exacerbating a knee exion posture. It is important to note whether the hindfoot deformity is reducible or not. Correction can be achieved by either a calcaneal lengthening or displacement osteotomy but often a subtalar fusion is required. Such surgery must be combined with a release of tight structures (such as the Achilles tendon) and possibly peroneal

Total body involvement

All parts of the body are affected; function is generally poor and the aims of surgical intervention differ signicantly from those for the hemiplegic or walking diplegic patient. HIP Hip subluxation progressing to dislocation is common. The adduction and exion contractures outlined above are more frequent and more severe in this group of patients, leaving the hip at risk of developing subluxation with acetabular dysplasia. Hips are often windswept (one hip lying adducted, exed and

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