Unilateral Spastic CP - One Side, Arm Usually Worse Than Leg
- UNILATERAL SPASTIC CEREBRAL PALSY follows a static, unilateral brain lesion. In the Canadian Cerebral Palsy Registry analysis of 662 children with unilateral CP, 299 (45%) had definitive perinatal stroke on expert MRI review - 169 (57%) perinatal arterial ischaemic stroke and 130 (43%) periventricular venous infarction. Acute intrapartum factors (low Apgar score, prolonged rupture of membranes) were associated with arterial stroke, while in-utero factors (small for gestational age, primigravida) were associated with venous infarction. The brain lesion never progresses; the musculoskeletal deformity always can.
- THE WINTERS, GAGE AND HICKS GROUPS, as the original paper defines them in 46 patients, are an ascending ladder of involved levels: Group I - the primary abnormality is a DROP FOOT IN THE SWING PHASE; Group II - a TIGHT HEEL CORD IN THE STANCE PHASE as well as a drop foot in swing; Group III - more PROXIMAL involvement, specifically RESTRICTED MOTION OF THE KNEE, as well as ankle equinus; Group IV - in addition, RESTRICTED MOTION OF THE HIP. Note carefully that Group III in the source is defined by restricted knee motion (stiff knee), not by knee hyperextension.
- THE AFFECTED LIMB IS SMALLER, NOT JUST STIFFER. Hemiatrophy affects length and girth, is usually more marked distally (foot and hand size differ before femoral or tibial length does), and is proportional to the extent of corticospinal tract disruption. Leg-length equalisation is judged against function rather than a numerical zero: because the hemiplegic ankle cannot dorsiflex normally, a residual shortening of 0.5-1.5 cm at maturity is the deliberate target.
- IN THE EQUINOVARUS FOOT THE OPERATION DEPENDS ON WHICH MUSCLE DRIVES THE VARUS. Supination that is worst in SWING, with the foot everting once loaded, indicates tibialis anterior; varus present throughout STANCE, with a callus over the fifth metatarsal base and lateral border loading, indicates tibialis posterior. Split anterior tibial tendon transfer addresses the former; intramuscular tibialis posterior lengthening or split transfer addresses the latter. A fixed hindfoot varus and a very weak tibialis anterior are the two documented predictors of a poor SPLATT result.
- SENSATION SELECTS THE HAND, NOT DEFORMITY. In the defining thumb-in-palm series, the quality of voluntary muscle control and sensibility were the most important factors predicting the success of operation, and rational decisions could be made only by assessing hand and thumb FUNCTION rather than the static position of the thumb. A child with severe stereognostic loss who ignores the limb will not convert a surgically improved posture into use.
- “Winters Group III = restricted KNEE motion with equinus; Group IV = restricted HIP motion in addition. Do not confuse Group III with knee hyperextension.
- “Silfverskiold test: dorsiflexion improving markedly with the knee flexed isolates gastrocnemius; equally limited in both positions implicates the whole gastrocsoleus or a fixed joint.
- “Tardieu R1 (catch at fast stretch) versus R2 (slow, full passive range): a large R2 minus R1 is dynamic spasticity and is toxin- or cast-responsive; a small difference with a low R2 is fixed contracture and needs lengthening.
- “GMFCS and MACS dissociate in hemiplegia - a GMFCS I child may be MACS II-III. Classify hand function separately; never infer it from walking.
- “Botulinum toxin A and serial casting had similar efficacy for dynamic equinus in the randomised comparison; toxin gave more prolonged passive dorsiflexion and fewer side effects, but median time to reintervention was similar.
The original Winters, Gage and Hicks description defines Group III by restricted motion of the KNEE (a stiff knee) added to ankle equinus, and Group IV by restricted motion of the HIP in addition. A widely repeated secondary version labels Group III as "equinus plus knee hyperextension"; that is not how the source defines it. State the groups as an ascending ladder of involved levels, which is what the paper actually demonstrates.
Varus is not one deformity. Swing-phase supination is tibialis anterior; stance-phase varus is tibialis posterior. Transferring the wrong tendon produces either a persistent varus or an overcorrected planovalgus foot. In the combined-procedure series, the poor results were in the two patients with a fixed hindfoot varus and the one with a very weak anterior tibial muscle - both identifiable before the incision is made.
Definition, Epidemiology & Aetiology
Spastic hemiplegia - increasingly and more accurately termed unilateral spastic cerebral palsy - is a disorder of movement and posture attributable to a non-progressive lesion of one cerebral hemisphere acquired in the antenatal, perinatal or early postnatal period. Cerebral palsy as a whole affects about 1 in 500 neonates, with an estimated 17 million people affected worldwide; unilateral spastic CP is one of the two commonest ambulant phenotypes alongside bilateral spastic CP.
The orthopaedic surgeon's position is specific and worth stating plainly: the neurological lesion is fixed, the musculoskeletal consequence is not. Every deformity treated in this condition is a secondary, growth-driven consequence of spasticity, weakness, loss of selective motor control and disuse operating on a growing skeleton. That is why surveillance matters, why deformity recurs after early surgery, and why timing dominates surgical decision-making.
Aetiology: two distinct lesions, two distinct stories
Perinatal stroke underlies almost half of unilateral CP - and arterial and venous lesions have different risk profiles
- Of 2093 cases in the Canadian Cerebral Palsy Registry, 662 had unilateral CP; 299 (45%) had definitive perinatal stroke on expert review of MRI reports.
- Of those, 169 (57%) were perinatal arterial ischaemic stroke (AIS) and 130 (43%) were periventricular venous infarction (PVI).
- Median age at diagnosis was 11.9 months for AIS (IQR 6.2-25.7) and 25.3 months for PVI (IQR 14.5-38) - the venous group presents later.
- Chorioamnionitis, illicit drug exposure, diabetes, gestational age and maternal age were independently associated with both lesions; low Apgar score and prolonged rupture of membranes with AIS alone; small for gestational age and primigravida with PVI alone.
- Term infants more often have an arterial territory infarct, classically middle cerebral artery, producing cortical and subcortical injury with a greater upper-limb than lower-limb deficit and a higher rate of associated epilepsy and cortical sensory loss.
- Preterm or growth-restricted infants more often have asymmetric periventricular white-matter injury (periventricular venous infarction, or asymmetric periventricular leukomalacia), where descending corticospinal fibres to the leg lie medially near the ventricle - which is why some of these children have a leg-predominant picture.
- Other causes include cerebral malformations (schizencephaly, unilateral polymicrogyria), neonatal hypoglycaemia, infection and early postnatal insult. A progressive or fluctuating picture is not unilateral CP - reconsider the diagnosis.
Almost all children with unilateral spastic CP walk independently, and the great majority are GMFCS level I or II. If a child labelled hemiplegic is not walking, or if the pattern is deteriorating, the label is probably wrong. Reconsider hereditary spastic paraplegia (progressive, often familial, symmetrical), dopa-responsive dystonia (diurnal fluctuation, dramatic levodopa response), a spinal cord or posterior fossa lesion, a tumour or a metabolic or neurodegenerative disorder. A first MRI that was normal, or that was never obtained, makes this review mandatory.
Classifying the child, not just the limb
Hand function must be classified separately - MACS is a valid, highly reliable five-level system
- MACS classifies how children with CP use their hands when handling objects in daily activities - typical manual PERFORMANCE, not maximal capacity - and classifies the collaborative use of both hands together.
- Reliability was tested in 168 children aged 4 to 18 years (including 52 with hemiplegia).
- Interrater intraclass correlation between therapists was 0.97 (95% CI 0.96-0.98).
- Agreement between parents and therapists was 0.96 (95% CI 0.89-0.98).
Population data confirm that motor function and impairment load travel together and should be recorded formally. In the western Sweden population-based study of 411 children with CP, GMFCS was distributed level I in 32%, II in 29%, III in 8%, IV in 15% and V in 16%, with accompanying learning disability in 40%, epilepsy in 33% and severe visual impairment in 19%, and more severe GMFCS levels correlating with a larger burden of accompanying impairments (Himmelmann 2006, PMID 16700930). In the clinic this means an orthopaedic plan for a hemiplegic child is also a plan around epilepsy medication, visual field loss and learning support.
Q: What is the advantage of the Surveillance of Cerebral Palsy in Europe terminology?
A: The SCPE system classifies spastic CP as unilateral or bilateral rather than hemiplegia, diplegia and quadriplegia. The older topographic terms are unreliable between observers - the boundary between "diplegia" and "quadriplegia" in particular depends on who is looking. The unilateral/bilateral split is reproducible, maps directly onto the underlying lesion (one hemisphere versus both), and was the classification used in the population-based motor-function studies. In an examination, use the modern term and then show you can still apply the classical gait classifications that were published under the older name.
Natural History & Musculoskeletal Pathomechanics
Nearly all children walk. Independent ambulation is the rule in unilateral spastic CP and is usually achieved within a normal or mildly delayed timeframe, typically between 18 and 24 months. Walking is not the clinical problem; the quality, efficiency, symmetry and durability of walking are.
The characteristic trajectory is:
- Toddler years - asymmetry of reach and hand preference before 12 months is the classic early sign (a true hand preference before one year of age is abnormal and warrants neurological assessment). Early toe-walking on the affected side, fisting, and a hemiplegic posture on running.
- Early childhood (3-7 years) - dynamic equinus, intermittent toe-drag, tripping. Deformity is predominantly dynamic: it disappears under anaesthesia and corrects passively in clinic. This is the window for tone management, orthoses and therapy, not for lengthening.
- Growth spurts - muscle-tendon units fail to elongate at the rate of the bone they span. Spastic muscle has a reduced capacity to add sarcomeres in series, so the dynamic deformity converts to a fixed contracture across each growth spurt. Parents report "sudden" worsening; what has changed is bone length.
- Adolescence - fixed contracture, secondary bony change (talar flattening, loss of calcaneal pitch in a long-standing equinovarus), lever-arm dysfunction from torsional malalignment, and pain from lateral-border loading or brace intolerance. This is when reconstructive surgery is usually indicated.
- Adulthood - deterioration in walking efficiency, degenerative change in the overloaded unaffected limb, and back or knee pain from chronic asymmetry.
Gait Classification: What the Source Actually Says
The original description: four homogeneous sagittal-plane patterns forming an ascending ladder of involvement
- Four homogeneous patterns of gait were defined in 46 patients with spastic hemiplegia secondary to cerebral palsy or other neurological disorders, by analysing sagittal-plane kinematic data and electromyographic data.
- Group I (20 patients): the primary abnormality was a DROP FOOT IN THE SWING PHASE.
- Group II (13 patients): a TIGHT HEEL CORD IN THE STANCE PHASE as well as a drop foot in the swing phase.
- Group III (5 patients): also more proximal involvement - that is, RESTRICTED MOTION OF THE KNEE - as well as an equinus deformity of the ankle. Group IV (8 patients): in addition, RESTRICTED MOTION OF THE HIP.
Secondary sources frequently describe Group III as "equinus plus knee hyperextension" and Group IV as "equinus plus knee and hip flexion and adduction". The original paper defines Group III by restricted motion of the knee added to ankle equinus, and Group IV by restricted motion of the hip added to that. Knee recurvatum does occur in hemiplegic gait - it is a well-recognised consequence of stance-phase equinus driving the tibia backwards - but it is not the defining feature of Group III in the source. When asked, give the source definitions and then note that recurvatum is a common accompaniment of the stance-phase equinus seen from Group II upwards. Being able to say why the versions differ is worth more than either version alone.
- Defining abnormality (source)
- Drop foot in the SWING phase; ankle behaviour in stance is not the primary problem
- What you see at the bedside
- Toe-drag and tripping in swing; heel contact preserved or near-preserved; passive dorsiflexion usually adequate
- Treatment logic
- Orthosis first - a hinged or posterior leaf-spring AFO to control swing-phase dorsiflexion; strengthening; functional electrical stimulation in selected children. Calf lengthening is not indicated
- Defining abnormality (source)
- Tight heel cord in the STANCE phase as well as drop foot in swing
- What you see at the bedside
- Forefoot or flat initial contact, early heel rise, reduced passive dorsiflexion; recurvatum may accompany it
- Treatment logic
- Tone management and casting while the deformity is dynamic; gastrocnemius or gastrocsoleus lengthening once fixed on a positive Silfverskiold test and adequate ankle power
- Defining abnormality (source)
- More proximal involvement - RESTRICTED MOTION OF THE KNEE - as well as ankle equinus
- What you see at the bedside
- Stiff-knee gait: reduced and delayed peak swing knee flexion, toe drag despite adequate dorsiflexion, circumduction or hip hitch to clear the limb
- Treatment logic
- Address ankle and knee together; rectus femoris transfer considered only when gait analysis confirms inappropriate rectus activity in swing with reduced knee flexion range and timing
- Defining abnormality (source)
- In addition, RESTRICTED MOTION OF THE HIP
- What you see at the bedside
- Reduced hip extension in terminal stance, flexed and internally rotated posture, adduction, compensatory pelvic rotation and lumbar lordosis
- Treatment logic
- Multilevel problem requiring multilevel assessment; consider psoas lengthening over the brim, adductor surgery, femoral derotation, plus the distal work - the classic indication for a unilateral SEMLS
SSKHWinters Groups - Climb the Limb
Hook:The groups ascend the limb one joint at a time: swing ankle, stance ankle, knee, hip. If you can name the highest involved level, you have named the group.
The companion algorithm
Rodda and Graham subsequently presented classifications of gait and postural patterns in spastic hemiplegia and spastic diplegia explicitly built on the work of previous authors, using them as a biomechanical basis linking spasticity, lower-limb musculoskeletal pathology and appropriate intervention strategies - so that the choice of target muscles for spasticity management, the contractures requiring lengthening and the choice of orthosis are all linked to the underlying gait pattern (Rodda and Graham 2001, PMID 11851738). This is the conceptual bridge from "what pattern is this?" to "what am I going to do about it?", and it is the framing examiners reward.
Q: A 10-year-old with unilateral spastic CP has adequate passive dorsiflexion, no fixed contracture, but drags the toe in swing and hitches the hip to clear the limb. Peak knee flexion in swing is 35 degrees (normal approximately 60) and occurs late. What is the pattern, and what is the operation?
A: This is a stiff-knee gait with a preserved ankle - the knee is the involved level, so it sits in Group III territory. The toe drag is a consequence of failed knee flexion, not of ankle equinus, and lengthening the calf would be the classic error: it would weaken push-off and worsen the problem. The question to answer with instrumented gait analysis and dynamic EMG is whether rectus femoris is active inappropriately in swing. If it is, rectus femoris transfer to a posteromedial or posterolateral structure is the targeted procedure. If the reduced range is due to hamstring and hip-flexor co-contraction or to weak hip flexion power, transfer will not help.
Clinical Assessment: The Examination That Changes the Operation
Ask what has changed and why the family has come now. In a static condition, a new complaint means either growth has outstripped a muscle, a brace has stopped fitting, or expectations have shifted.
- Birth and neonatal history - gestation, birth weight, Apgar scores, prolonged rupture of membranes, neonatal seizures or encephalopathy, neonatal intensive care admission, cooling. Was an MRI performed and what did it show?
- Diagnosis and milestones - age of diagnosis, age of independent walking, early hand preference (before 12 months is abnormal).
- Current function - community walking distance, stairs, running, sport, falls (frequency and direction), fatigue, footwear wear pattern, shoe-size difference between feet.
- Hand use - what the affected hand does in real bimanual tasks (holding paper while writing, stabilising a bowl, dressing, fastening). "Does it help, or is it in the way?" is the most useful single question.
- Pain - lateral border of the foot, fifth metatarsal base callus, brace pressure, knee or back pain from asymmetry.
- Previous treatment - botulinum toxin (which muscles, how many cycles, what happened and for how long), serial casting, orthoses tolerated or abandoned, previous surgery and its result.
- Co-morbidity - epilepsy and anticonvulsants, visual field defect (a homonymous hemianopia to the affected side changes falls risk and therapy), learning and communication needs, behavioural issues.
- Goals - what specific change would make an operation worth it? An answer such as "to stop the tripping" is operable; "to make it look normal" needs a conversation before it needs a knife.
- Is the deformity dynamic or fixed? Tardieu R2 and examination under anaesthesia answer it. Dynamic deformity is treated with tone management; fixed deformity is treated with lengthening.
- Which muscle is doing it, and when? Swing versus stance for varus; dynamic EMG where available. This answers which tendon.
- Can the child use what you give back? Selective motor control and strength for the leg; sensation, stereognosis and voluntary control for the hand. This answers whether to operate at all.
Surgical Management of the Lower Limb
Timing: the single most examinable decision
Surgery in unilateral spastic CP is reconstructive, not curative, and its result depends more on when it is done than on how well it is done.
- Operate too early (typically before about 7-8 years) and the deformity recurs, because the child has years of differential muscle-versus-bone growth still to come, and the family is drawn into the pattern of repeated annual operations that single-event multilevel surgery was designed to abolish.
- Operate too late and secondary bony change - talar flattening, loss of calcaneal pitch, fixed hindfoot varus, established torsional malalignment - converts a soft-tissue problem into a bony one, with a longer recovery and a worse ceiling.
- The usual window is therefore middle to late childhood and early adolescence, once the deformity is fixed, the pattern is stable across serial reviews, the child is mature enough to participate in rehabilitation, and gait analysis (where available) has defined the full list of levels involved.
- Bony work at the foot (calcaneal osteotomy, arthrodesis) is generally deferred until deformity is genuinely fixed; epiphysiodesis for limb-length discrepancy is timed independently against bone age and a growth-remaining prediction.
Single-event multilevel surgery in the unilateral child
The principle of SEMLS - correcting every contributing deformity at every involved level in one anaesthetic, followed by one rehabilitation programme - was established in bilateral spastic CP. The pilot randomised trial in spastic diplegia (19 children, mean 8 procedures each) produced a 34% improvement in Gait Profile Score and 57% in Gillette Gait Index at 12 months, with gross motor function and quality-of-life gains not appearing until 24 months (Thomason 2011, PMID 21368077), and the five-year prospective follow-up found the improvements stable over time, with GPS improved by 5.29 degrees and GMFM-66 by 3.3% at five years (Thomason 2013, PMID 22818117). Two lessons transfer directly to the unilateral child: the functional gain lags the kinematic gain by about a year, and the gain is durable if the indication was right.
Unilateral SEMLS improves gait in unilateral CP and the improvement is maintained to about ten years
- Fourteen children with unilateral CP (mean age 12.1 years) had gait analysis and clinical data reviewed preoperatively, at 1 year, at 3-5 years and at approximately 10 years after unilateral SEMLS.
- The Gait Profile Score of the affected leg improved by 3.73 degrees - well above the minimal clinically important difference of 1.6 degrees.
- No deterioration of Gait Profile Score occurred throughout the follow-up period, so the postoperative improvement was maintained long term.
- Additional fine-tuning procedures were required during follow-up in 5 of 14 children, and three complications occurred (one grade II and two grade III).
- Problem
- Reduced hip extension in terminal stance from psoas tightness (Winters Group IV)
- Procedure
- Psoas lengthening over the pelvic brim (intramuscular tendon lengthening), preserving iliacus
- Key caveat
- Over-lengthening weakens hip flexion and swing-limb advancement; avoid complete tenotomy in an ambulant child
- Problem
- Persistent femoral anteversion causing internal rotation gait and lever-arm dysfunction
- Procedure
- Femoral derotation osteotomy (proximal or distal), with internal fixation
- Key caveat
- Correct the lever, do not lengthen the muscle. Under-correction is common; measure anteversion formally rather than estimating
- Problem
- Stiff-knee gait: reduced and delayed peak swing knee flexion (Group III)
- Procedure
- Rectus femoris transfer to a posterior structure (semitendinosus, gracilis or iliotibial band)
- Key caveat
- Only when dynamic EMG confirms inappropriate swing-phase rectus activity. Release alone is generally less effective than transfer
- Problem
- Fixed knee flexion contracture with increased popliteal angle
- Procedure
- Medial hamstring lengthening, with lateral lengthening only if needed
- Key caveat
- Uncommon in unilateral CP compared with diplegia; over-lengthening risks anterior pelvic tilt and recurvatum
- Problem
- Fixed equinus with a POSITIVE Silfverskiold test
- Procedure
- Gastrocnemius recession - Strayer, Baumann or Vulpius type
- Key caveat
- The correct operation for isolated gastrocnemius tightness; preserves soleus and therefore push-off
- Problem
- Fixed equinus with a NEGATIVE Silfverskiold test
- Procedure
- Tendo-Achilles lengthening (percutaneous or open Z-lengthening)
- Key caveat
- Highest risk of over-lengthening, calcaneus gait, loss of push-off and crouch. Under-correct rather than over-correct; a residual 5 degrees of equinus is far safer than a calcaneus foot
- Problem
- Tibialis anterior overactivity causing swing-phase supination
- Procedure
- Split anterior tibial tendon transfer (SPLATT) to cuboid, lateral cuneiform or peroneus tertius
- Key caveat
- Requires a STRONG tibialis anterior and a flexible hindfoot
- Problem
- Tibialis posterior overactivity causing stance-phase hindfoot varus and forefoot adduction
- Procedure
- Intramuscular lengthening of tibialis posterior, or split posterior tibial tendon transfer to peroneus brevis
- Key caveat
- Complete transfer or tenotomy of tibialis posterior risks progressive planovalgus; intramuscular lengthening is the more forgiving option
- Problem
- Hindfoot does not correct on the Coleman block test or under anaesthesia
- Procedure
- Lateral closing-wedge or lateralising calcaneal osteotomy (Dwyer-type), with soft-tissue work
- Key caveat
- Soft-tissue surgery alone will fail. Recognise fixity BEFORE the operating list, not during it
- Problem
- Fixed deformity with established degenerative change and pain
- Procedure
- Triple arthrodesis as a salvage
- Key caveat
- Definitive but stiffening; reserved for the skeletally mature painful rigid foot where lesser procedures cannot achieve a plantigrade result
- Problem
- Plantarflexed first ray driving forefoot-based varus
- Procedure
- Dorsiflexion osteotomy of the first metatarsal, with plantar fascia release
- Key caveat
- Identified by a positive Coleman block test showing a flexible hindfoot
- Problem
- Discrepancy distorting gait, typically over 2.5 cm
- Procedure
- Epiphysiodesis of the UNAFFECTED distal femur and/or proximal tibia, timed against bone age
- Key caveat
- Aim for a residual 0.5-1.5 cm of shortening on the affected side, not equality, because the hemiplegic ankle cannot dorsiflex for clearance
The equinovarus foot in detail
SPLATT converts brace-dependent equinovarus feet to unbraced community ambulation at ten years
- Split anterior tibial tendon transfer was performed on 21 patients (27 feet) with cerebral palsy and spastic equinovarus deformity.
- All patients required orthoses preoperatively.
- All but two patients were community ambulators at follow-up with improved gait and WITHOUT need for orthoses.
- There was one recurrence of deformity.
Combining SPLATT with intramuscular tibialis posterior lengthening works for flexible varus - and the failures are predictable
- Twenty patients had 22 combined split anterior tibial tendon transfer plus intramuscular lengthening of the posterior tibial tendon, with or without concomitant Achilles lengthening.
- All had preoperative DYNAMIC hindfoot varus and forefoot adduction in BOTH the stance and the swing phase of gait.
- At mean 6.2 years (range 2.3 to 8.8), results were 14 excellent, 4 good and 4 poor.
- Poor results occurred in the two patients who had a FIXED hindfoot varus and the one who had a VERY WEAK anterior tibial muscle; the combined procedure was concluded to be effective for FLEXIBLE varus.
Step 1 - Is the hindfoot flexible? Coleman block test, passive correction, and examination under anaesthesia if uncertain. Fixed → a bony procedure (lateralising or closing-wedge calcaneal osteotomy) is required in addition to any soft-tissue work; soft-tissue surgery alone will fail.
Step 2 - If flexible, which muscle, and in which phase?
- Supination maximal in SWING, foot everts on loading → tibialis anterior → SPLATT.
- Varus throughout STANCE, lateral-border callus → tibialis posterior → intramuscular lengthening (or split posterior transfer).
- Varus in BOTH phases → both muscles → combined SPLATT plus intramuscular tibialis posterior lengthening.
Step 3 - Is tibialis anterior strong enough for a split transfer? Grade it against resistance. A very weak tibialis anterior is a documented cause of a poor result - consider tibialis posterior transfer through the interosseous membrane or an orthotic solution instead.
Step 4 - Deal with the equinus in the same sitting. Silfverskiold positive → gastrocnemius recession; negative → tendo-Achilles lengthening, deliberately under-corrected.
Step 5 - Confirm the end point on the table: a plantigrade foot that sits in an AFO, with the heel reaching the ground and no residual forefoot adduction.
Over-lengthening the Achilles tendon in a spastic hemiplegic limb removes the plantarflexion-knee-extension couple. The result is a calcaneus gait with excessive dorsiflexion in stance, absent push-off, a crouched knee and a marked increase in the energy cost of walking - and it is far harder to salvage than the equinus it replaced. Three rules: perform a gastrocnemius recession rather than a tendo-Achilles lengthening whenever the Silfverskiold test is positive; never lengthen a calf in a child who cannot perform a single-limb heel raise; and where lengthening is genuinely indicated, aim to leave a few degrees of residual equinus rather than to achieve neutral on the table.
The Upper Limb: Selection Before Technique
Voluntary control and sensibility - not the static posture of the thumb - predict success in thumb-in-palm surgery
- One hundred and sixty-five different surgical procedures were performed for correction of thumb deformities in 56 patients with spastic cerebral palsy between 1967 and 1975.
- The quality of VOLUNTARY MUSCLE CONTROL and SENSIBILITY were the most important factors in predicting the success of operation.
- Thumb deformities had previously been classified on the basis of the STATIC position of the thumb, but rational treatment decisions can be made only by careful assessment of the patient's hand and thumb FUNCTION.
- Using combinations of releases, tendon transfers and joint stabilisations, measurable and predictable improvement in function was achieved in all 56 patients whose records were analysed.
- Deformity
- Metacarpal adduction only; thumb web contracture without joint deformity
- Principal contracted structure
- Adductor pollicis and first dorsal interosseous
- Surgical implication
- First web release (adductor release) with skin lengthening; stabilisation usually unnecessary
- Deformity
- Metacarpal adduction with metacarpophalangeal joint FLEXION
- Principal contracted structure
- Adductor pollicis plus flexor pollicis brevis
- Surgical implication
- Release plus augmentation of extension (for example extensor pollicis longus rerouting or a transfer to abductor pollicis longus)
- Deformity
- Metacarpal adduction with metacarpophalangeal HYPEREXTENSION or instability
- Principal contracted structure
- Adductor contracture with an incompetent MCP joint
- Surgical implication
- Release plus MCP joint stabilisation (capsulodesis, sesamoid arthrodesis or fusion) - correcting the web alone will worsen the hyperextension
- Deformity
- Metacarpal adduction with BOTH metacarpophalangeal and interphalangeal flexion
- Principal contracted structure
- Adductor, flexor pollicis brevis and flexor pollicis longus
- Surgical implication
- The most extensive: release, flexor pollicis longus lengthening or transfer, extension augmentation and often joint stabilisation
Selecting a hand for surgery
Favourable features:
- Preserved sensation, and in particular intact or near-intact stereognosis. A child who can identify objects in the hand without vision will incorporate an improved hand into bimanual tasks.
- Voluntary wrist extension, even if weak, and demonstrable selective finger extension with the wrist supported.
- Active use of the hand as an assisting hand already - documented on an Assisting Hand Assessment or equivalent - so that surgery improves an existing behaviour rather than trying to create one.
- Adequate cognition and motivation for a demanding post-operative therapy programme, and family capacity to deliver it.
- Skeletal maturity approaching, with a deformity that is stable and fixed rather than still evolving.
Unfavourable features - consider hygiene and appearance goals instead of function:
- Severe stereognostic loss and dense sensory impairment, with a limb that the child ignores.
- Athetosis or dystonia dominating the movement disorder, where tendon transfers behave unpredictably.
- Absent voluntary control - a hand that cannot be opened voluntarily will not open because a tendon has been moved.
- Poor motivation or inability to engage with therapy, which converts a technically successful operation into a stiffer hand.
Upper-limb surgery in unilateral CP has three legitimate but quite different goals - function, hygiene and appearance - and they demand different operations and different consent conversations. A wrist arthrodesis may transform appearance and ease of dressing while adding nothing to function; a superficialis-to-profundus transfer may make a severely contracted hand cleanable and comfortable without making it useful. Agree which goal is being pursued, in writing, before the operation. The commonest source of dissatisfaction in this field is a family who expected function from an operation that was planned for posture.
Q: In unilateral spastic CP following a middle cerebral artery territory perinatal arterial ischaemic stroke, why is the upper limb typically more affected than the lower limb?
A: The motor homunculus. The hand and arm representation occupies the lateral convexity of the precentral gyrus, which lies squarely within the middle cerebral artery territory, whereas the leg representation lies medially on the paracentral lobule, supplied largely by the anterior cerebral artery. An MCA-territory infarct therefore damages arm and hand fibres preferentially. The converse holds for periventricular white-matter injury: descending corticospinal fibres to the leg run closest to the lateral ventricle, so periventricular venous infarction or asymmetric periventricular leukomalacia can produce a leg-predominant hemiparesis. Knowing the lesion therefore predicts the distribution - another reason to ask for the MRI.
Guidelines, Registries & Global Practice
There is no single disease-specific international guideline for unilateral spastic cerebral palsy; practice is built from the general cerebral palsy evidence base, population surveillance registries and the gait-laboratory literature.
Registries and surveillance
- Population CP registries - the Surveillance of Cerebral Palsy in Europe network, the Australian Cerebral Palsy Register, the Canadian Cerebral Palsy Registry and the Nordic registers - underpin the epidemiology in this field. The registry-based case-control study of perinatal stroke in unilateral CP was only possible because of such a register, and it is registries that established the modern unilateral/bilateral classification in place of the less reliable topographic terms.
- Hip surveillance programmes are stratified by GMFCS level. Children with unilateral CP are almost all GMFCS I-II and therefore fall into the lowest-intensity surveillance stream; hip displacement risk rises steeply with GMFCS level and is concentrated in bilateral GMFCS IV-V. Routine intensive hip radiography is not indicated in unilateral CP, but clinical vigilance for asymmetry, pain or loss of abduction remains.
- Gait-laboratory networks and shared summary measures - Gait Profile Score, Gait Deviation Index, Gillette Gait Index, Edinburgh Visual Gait Score - allow outcomes to be compared across centres, and they are how the durability of SEMLS was demonstrated.
- Multidisciplinary team care - paediatric neurology, orthopaedics, rehabilitation medicine, physiotherapy, occupational therapy, orthotics and, where relevant, ophthalmology - is the consistent model worldwide, alongside formal transition planning to adult services.
Global variation
Cerebral palsy is the most common cause of childhood-onset lifelong physical disability in most countries, and meeting the needs of people with cerebral palsy in resource-poor settings is particularly challenging (Graham 2016, PMID 27188686). Several asymmetries follow:
- Instrumented gait analysis is concentrated in a minority of centres worldwide. Most surgeons deciding between SPLATT and tibialis posterior surgery will do so on clinical phase-of-deformity assessment, the confusion test and callus distribution. That is a legitimate basis for a conservative plan, but it is a reason to stage surgery cautiously rather than attempt an ambitious multilevel correction blind.
- Botulinum toxin is expensive and often unavailable. Where it is, serial casting is a genuinely evidence-supported alternative for dynamic equinus - of similar efficacy in the randomised head-to-head comparison, at a fraction of the cost, requiring only plaster and weekly attendance. This is one of the few places in paediatric orthopaedics where the low-cost option is directly supported by a comparative trial.
- Orthotic supply and review is the rate-limiting step in many systems. A brace that is not refitted after a growth spurt stops working; where orthotic review is annual or absent, surgical thresholds shift.
- Late presentation is common where diagnosis is delayed - and note that even within a high-income registry the median age at diagnosis of periventricular venous infarction was over two years. Children presenting late with established fixed deformity and secondary bony change need more reconstructive surgery than the algorithms written for early-identified cohorts assume.
MCQ Practice Points
- In the original Winters, Gage and Hicks description, Group I is a drop foot in the swing phase; Group II adds a tight heel cord in the stance phase; Group III adds restricted motion of the knee; Group IV adds restricted motion of the hip. The groups ascend the limb by level.
- In the Canadian Cerebral Palsy Registry analysis, 45% of 662 children with unilateral CP had definitive perinatal stroke - 57% arterial ischaemic, 43% periventricular venous infarction.
- MACS classifies typical manual performance (not maximal capacity) and the collaborative use of both hands; interrater ICC was 0.97 and parent-therapist ICC 0.96.
- Of 13 clinical spasticity instruments reviewed, only the Tardieu Scale complies with the definition of spasticity as a velocity-dependent increase in tone.
- SCALE correlated inversely with GMFCS (Spearman r = -0.83) with interrater ICC 0.88 to 0.91.
- Botulinum toxin A improved gait function in a placebo-controlled trial of 114 children with dynamic equinus; against serial casting, efficacy was similar and median time to reintervention was the same, although toxin gave more prolonged passive dorsiflexion and fewer side effects.
- After unilateral SEMLS, Gait Profile Score improved by 3.73 degrees (MCID 1.6 degrees) and was maintained to about ten years, but 5 of 14 children needed fine-tuning procedures.
- SPLATT left all but two of 21 patients as community ambulators without orthoses at ten years; in the combined SPLATT plus intramuscular tibialis posterior lengthening series the only poor results were in fixed hindfoot varus and a very weak tibialis anterior.
- In thumb-in-palm surgery, voluntary muscle control and sensibility were the most important predictors of success - not the static position of the thumb.
- CIMT beat a low-dose comparison by 5.44 AHA units but showed no advantage over a dose-matched bimanual programme - intensity, not constraint, is the active ingredient.
- Aim for a residual 0.5 to 1.5 cm of shortening at maturity after equalisation, because the hemiplegic ankle cannot dorsiflex for clearance.
Viva Scenarios
Practise clinical reasoning and management decisions out loud
“A 9-year-old boy with unilateral spastic cerebral palsy, GMFCS I, walks on the lateral border of his right foot. There is a thick callus over the base of the fifth metatarsal. The hindfoot varus corrects fully on the Coleman block test and passively to neutral. Ankle dorsiflexion is minus 5 degrees with the knee extended and plus 10 degrees with the knee flexed. He is in a solid AFO which he dislikes. How do you assess and manage him?”
“A 12-year-old girl with unilateral spastic cerebral palsy has had six cycles of botulinum toxin A to the gastrocsoleus since the age of four. The family report that the last two cycles made little difference. Passive dorsiflexion is minus 10 degrees with the knee extended and minus 8 degrees with the knee flexed. She cannot perform a single-limb heel raise on the affected side. They ask for another injection. What do you say and what do you do?”
“A 14-year-old boy with unilateral spastic cerebral palsy has a fixed thumb-in-palm deformity with metacarpal adduction and metacarpophalangeal hyperextension, a wrist held in 40 degrees of flexion and a pronated forearm. His mother asks whether an operation will let him use the hand. He has a dense sensory deficit and cannot identify a coin in the hand with his eyes closed. How do you counsel and plan?”
“An 11-year-old girl with unilateral spastic CP, GMFCS I, has a 3 cm leg-length discrepancy with the hemiplegic side short. She circumducts and vaults on the sound side. Her ankle dorsiflexes actively to 5 degrees. The family ask for the short leg to be lengthened. How do you approach this?”
“A 7-year-old with unilateral spastic CP has a toe-drag in swing on the affected side. There is heel contact at initial contact, passive dorsiflexion to 15 degrees with the knee extended, normal knee kinematics and normal hip extension in terminal stance. Classify the gait and state your management.”
Lesion, pattern and classification
- Unilateral spastic CP: static unilateral brain lesion. 45% of registry unilateral CP had definitive perinatal stroke - 57% arterial ischaemic, 43% periventricular venous infarction.
- MCA-territory infarct = arm worse than leg (hand fibres on the lateral convexity); periventricular injury = leg may predominate (leg fibres lie medially near the ventricle).
- Winters Group I = drop foot in SWING. Group II = tight heel cord in STANCE plus the swing drop foot. Group III = restricted KNEE motion plus ankle equinus. Group IV = restricted HIP motion in addition.
- Nearly all walk - GMFCS I-II. A hemiplegic child who is not walking needs the diagnosis reconsidered (hereditary spastic paraplegia, dopa-responsive dystonia, cord or posterior fossa lesion, tumour).
- Classify hand function separately with MACS - GMFCS and MACS dissociate in unilateral CP.
Examination that changes the operation
- Tardieu: R2 = slow full passive range (true length); R1 = angle of catch at fast stretch; R2 minus R1 = dynamic spasticity.
- Silfverskiold positive (better with knee flexed) = gastrocnemius only = gastrocnemius recession. Negative = whole gastrocsoleus = tendo-Achilles lengthening, deliberately under-corrected.
- Varus in SWING = tibialis anterior = SPLATT. Varus in STANCE with a fifth metatarsal base callus = tibialis posterior = intramuscular lengthening. Both phases = combined procedure.
- Coleman block test: hindfoot corrects = flexible, forefoot-driven. Hindfoot does not correct = fixed, and a calcaneal osteotomy is required.
- Poor SPLATT results are predicted by FIXED hindfoot varus and a VERY WEAK tibialis anterior - both detectable before surgery.
Non-operative management
- Botulinum toxin treats DYNAMIC deformity only; equivalent to serial casting with the same time to reintervention; repeated injection causes denervation atrophy with incomplete recovery at six months in controlled animal work.
- Hinged AFO is the evidence-supported orthosis in hemiplegic CP - improves speed, stride length, single-limb support, symmetry and energy cost.
- CIMT beats low-dose care but not a dose-matched bimanual programme - intensity is the active ingredient, and the evidence quality is low to very low.
Surgery and the traps
- Never lengthen a calf in a child who cannot perform a single-limb heel raise. Calcaneus gait is iatrogenic and hard to salvage.
- Unilateral SEMLS: GPS improved 3.73 degrees (MCID 1.6), maintained to about 10 years, but 5 of 14 needed fine-tuning and complications occur. Function lags kinematics by about a year.
- Leg-length equalisation is by epiphysiodesis of the SOUND side, timed against bone age, targeting a residual 0.5-1.5 cm of shortening - not equality.
- Thumb-in-palm: voluntary control and sensibility predict success, not static thumb position. House types I-IV determine release versus release-plus-augmentation versus release-plus-stabilisation.
Non-Operative Management: What the Evidence Supports and Where It Stops
Therapy and orthoses
Physiotherapy targets strength, range and gait efficiency; occupational therapy targets bimanual performance and participation. Neither abolishes the tendency of spastic muscle to fall behind bone growth.
Ankle-foot orthoses improve gait parameters in CP, with the hinged AFO specifically supported in hemiplegia
- Seventeen studies including 1139 children with CP compared hinged (HAFO), solid (SAFO), floor-reaction (FRO), posterior leaf-spring (PLS) and dynamic (DAFO) orthoses.
- In general, AFO use improved walking speed and stride length, and reduced energy expenditure in children with spastic CP.
- The HAFO was effective for improving gait parameters and decreasing energy expenditure in HEMIPLEGIC CP compared with barefoot, and improved stride length, walking speed, single-limb support and gait symmetry.
- The solid AFO and floor-reaction orthosis were the types effective in reducing energy expenditure in diplegic CP.
Practical prescribing in unilateral CP:
- Hinged AFO (free dorsiflexion, plantarflexion stop) - the default for Group I and mild Group II with adequate passive dorsiflexion to neutral and no significant crouch. Preserves the push-off the child has.
- Posterior leaf-spring AFO - a light option for pure swing-phase drop foot with good stance stability.
- Solid AFO - for stance-phase instability, significant equinus that cannot be controlled by a hinge, or post-operative protection. Accept the loss of ankle power as the price.
- Ground/floor-reaction AFO - has a role where a knee-extension moment is needed, but is the wrong choice if there is fixed equinus, because it will simply drive the knee into recurvatum.
- Fit is a clinical act, not a delegation. Check for skin marking over the navicular and fifth metatarsal base, check the hindfoot sits in the heel cup, and review after every growth spurt. The commonest reason a brace "doesn't work" is that it no longer fits.
Botulinum toxin A: real, reversible and limited
Botulinum toxin A improves gait function in dynamic equinus in a placebo-controlled randomised trial
- One hundred and fourteen children with cerebral palsy and dynamic equinus foot deformity were randomised in a prospective 3-month double-blind trial.
- Outcome was assessed by observational gait analysis, ankle range-of-motion measurement and quantification of muscle denervation by nerve conduction.
- Patients in the botulinum toxin group demonstrated improved gait function and partial denervation of the injected muscle.
- No serious adverse events were reported.
Against serial casting, botulinum toxin was of similar efficacy - with a different trade-off profile
- Twenty children with a dynamic component to calf equinus were randomised to intramuscular botulinum toxin A or serial casting, assessed by clinical examination, video gait analysis and three-dimensional gait analysis.
- Botulinum toxin A was of efficacy SIMILAR to that of serial casting.
- Tone reduction in the toxin group allowed a more prolonged improvement in passive dorsiflexion; gait analysis showed an improved mean ankle kinematic pattern in a subsection of both groups, maintained at 12 weeks in the toxin group whereas the cast group relapsed.
- There were fewer side effects in the toxin group, but MEDIAN TIME TO REINTERVENTION WAS SIMILAR.
Where the toxin evidence stops. A systematic review of lower-limb botulinum toxin A in addition to physiotherapy versus physiotherapy alone, restricted to four Level II trials in 153 children, concluded that the combination seems to have a positive effect on spasticity and range of movement, but that whether it produces greater improvement in functional capacity such as gross motor function or gait parameters was inconclusive (Yana 2019, PMID 30856126). Spasticity is not the same outcome as function, and the literature reflects the gap honestly.
Botulinum toxin acts by blocking acetylcholine release at cholinergic nerve endings, producing temporary denervation and atrophy of the injected muscle; its role is inherently limited by the temporary mode of action and by restrictions on the total dose deliverable per visit, so repeated injections are required (Satila 2020, PMID 32640636). In a controlled animal study, rabbit quadriceps assessed six months after one, two or three injections showed significantly reduced muscle strength and contractile material compared with saline-injected controls, and strength and contractile material did not fully recover within six months (Fortuna 2015, PMID 26087882). In a spastic hemiplegic limb that is already weak and already hemiatrophic, indefinite repeat injection of the gastrocsoleus is a decision with a cost. Inject with a defined goal and a defined end point, reassess strength - single-limb heel raises - between cycles, and stop when the deformity has become fixed, because at that point toxin cannot work and only lengthening can.
The upper limb non-operatively
Constraint-induced movement therapy beats low-dose care but not dose-matched bimanual therapy
- Thirty-six trials published 2004 to 2018 were included; mean age 5.96 years; mean total CIMT dose 137 hours (range 20 to 504 hours) over an average four-week programme.
- Versus a LOW-DOSE comparison, CIMT improved bimanual performance by a mean difference of 5.44 Assisting Hand Assessment units (95% CI 2.37 to 8.51) and improved unimanual capacity across QUEST domains.
- Versus a HIGH-DOSE comparison, CIMT was NOT more effective for bimanual performance (MD -0.39 AHA units, 95% CI -3.14 to 2.36).
- Versus a DOSE-MATCHED comparison such as Hand Arm Bimanual Intensive Therapy, there was no evidence of a difference in bimanual performance (MD 0.80 AHA units, 95% CI -0.78 to 2.38) or manual ability; CIMT appears safe, with reported adverse events limited to frustration, constraint refusal and reversible skin irritation from casting.
Boyd, Morris and Graham's systematic review of upper-limb management in CP identified 60 papers, of which only four were randomised controlled trials and 44 were prospective studies with objective outcome measures, spanning therapies, splinting and casting, focal and generalised pharmacotherapy, and surgery either to improve function or to correct deformity (PMID 11851744). The message for the orthopaedic clinic is that the upper-limb evidence base is thinner than the lower-limb one, and that surgical claims should be framed accordingly when consenting a family.
Splinting has a defined and modest role: a thumb abduction (neoprene or thermoplastic) splint may improve grasp positioning during task practice; a resting wrist-hand splint may slow contracture progression. Neither substitutes for active use, and a splint that a child removes is a splint that does nothing.
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