Presentation on theme: "Physiotherapy Management of Neuromuscular Scoliosis"— Presentation transcript:
1Physiotherapy Management of Neuromuscular Scoliosis Hannah WaughBleep 9126Specialist Physiotherapist,The Royal Hospital for Sick Children,Edinburgh
2Contents What is Scoliosis? Medical Management Pre Operative Planning Hospital AdmissionChallenges post discharge
3What is Scoliosis?Complex three dimensional deformity where the curve is greater than 10 degrees
4Prevalence of Neuromuscular Scoliosis 20% of children with Cerebral Palsy60% of children with Myelodysplasia90% of children with Duchenne Muscular Dystrophy
5Neuromuscular Scoliosis Development Spinal curvature may begin very early in lifeOften after the patient starts supported sittingCurve may progress rapidly once patient becomes non ambulant (averaging 10 degrees/year)
8Preventing Progression of Scoliosis Prolong mobilitySteroids24 hour postural managementSpinal bracing (not always effective particularly in progressive neuromuscular curves)
9Referral Criteria Consultant to consultant referral only Confirmed scoliosis - requesting specialist assessment for surgical interventionNeurological – usually after the age of 10 as surgery unlikely prior to thisDMD – when patient becomes non ambulant
10Initial Spinal Clinic Assessment In-depth history is takenscoliosis progression, pain, functionpast medical historymedicationsocial historyObjective AssessmentX-rays : standing or sitting to establish severity, bending films to identify flexibility – cobb angle, also check risser gradePhysical assessmentAsymmetry of trunkUneven ShouldersUneven PelvisListing to one sideAdam’s Forward BendingTest Thoracic prominenceLumbar prominenceLeg LengthPlumb lineFrom C7 spinous process to hang below buttocksNeurological examinationPainNumbnessTinglingWeaknessMuscle spasmBowel/bladder changesTo ensure that the patient can tolerate reconstructive spinal surgery, a detailed preoperative history and assessment should include an evaluation of respiratory competency, cardiac status, nutrition, possible feeding difficulties, seizure disorders, urologic status, and metabolic bone disease.Patients capable of cooperating should be evaluated with pulmonary function studies. Patients with vital capacities less than 30% of the predicted reference value may require postoperative ventilatory support. Performing formal pulmonary function testing is difficult in patients with neuromuscular scoliosis because patients are often unable to cooperate.Patients with Duchenne muscular dystrophy and Friedreich ataxia should be evaluated for cardiac involvement.Poor nutritional status is strongly linked to perioperative complications in these patients. Nutritional deficiencies should be corrected preoperatively through a forced nutritional improvement schedule or postoperatively with feeding tubes. Elective placement of gastric feeding tubes 3 months preoperatively dramatically improves nutritional status. The use of total parenteral nutrition (TPN) perioperatively also can be helpful in decreasing problems with wound infections.[22, 23]
11Cobb AngleThis is taken to monitor progression but is only one part of the assessment tool. Not everybody about a certain angle gets PSF.
12Medical Management Dependent on: Severity of scoliosis Pelvic obliquityAge/Skeletal maturity – risser gradeRib deformity/ Impingement/ PainComplexity of past medical history
14Medical Management - DMD Respiratory FunctionFunctional AbilitySymptomsQuality of Life questionnaireReduction in surgeryLots of contradictory information out there – Does PSF improve respiratory function?, Does PSF stop respiratory function getting worse?Unsure???Functional Ability – use of ULs and flexion of the spine to feed/ play etc if flexion of spine is prevented - ?function is reduced- can get mobile arm supports to overcome this problem.Symptoms – PAIN – very justified to undertake PSF due to pain, particularly if some children have been down the pain management route.- positioing, not as big a problem compared to the children with CP due to their size etcQuality of Questionnaire – not scoring wellReduction in Surgery – Due to the above surgery has decreased from about 5-6/yr to 1/yr
15Medical Management - CP Respiratory FunctionFunctional AbilitySymptomsQuality of Life questionnaireSurgeryRespiratory FunctionFunctional Ability – probably more often than not will improve their function due to the position changeSymptoms – Pain, Positioning, hygiene- a lot of children with hip dislocation with a severe pelvic obliquity, PSF is required first to rectify the pelvic obliquity before pelvic and femoral osteotomies can be undertaken
16Medical Management - mylominingecele Respiratory FunctionFunctional AbilitySymptomsSurgeryRespiratory FunctionFunctional Ability – floor work, often spinal surgeons request an OT and PT assessment to gauge how much function they have on the floorSymptoms – No pain
17Medical Management Every case is very individual Function Medical StabilityMDT decisionNot just the size of the curve is considered.This is a major operation and at stages different things can go wrong
18Medical Management Continue to monitor curve Use of conservative treatmentPSFIntensivistCardiac ConsultantNeurologist
19Physiotherapy Service Aims To ensure smooth pathway from pre admission to dischargeTo be available for contact to reduce any anxieties throughout the patient journeyTo be a resource for local therapists / services for Scotland
20Spinal Surgery Pathway Contact made with local services & familyPre-op assessment completedTheatre list to Physio & OTEquipment requirements identified & commencedLocal services reviewDischargeAdmissionPost-op
21Physiotherapy RoleTo ensure that optimal functional abilities are achieved post operativelyThose functional abilties include:respiratory functionmuscle strengthtransfers/ mobilitypostural managementOverall aim is to maximise independence following surgery in activities of daily livingPostural management is vital and should be considered through out all stages of spinal surgery
22Physio Pre op PlanningCommenced as soon as the patient is listed for theatre (approx 6 weeks)Facilitate smooth admission and discharge from hospitalEarly contact with local services is essential
23Pre-operative Planning Unfortunately due to geographic location of clinics, unable to attendContact will usually be made with the family and local therapists initially by telephoneIf patients admitted for respiratory tests, trial of NIV or attend for anaesthetic assessment we will meet and assess on ward if possible
24Initial Pre-Op Assessment Physio /OT Establish current abilities ofSeating (wheelchairs,other seating systems school, home)Transfers (independent, assisted, hoist)Mobility- use of walking aidsPersonal Hygiene (toileting, bathing/showering, level of assistance ,specific equipment)Respiratory functionOther ADL activities (feeding, self dressing)SchoolEnvironmental issues (access to and within house)- child may need to live downstairs
25SeatingWheelchairsShould be in suitable corrective seating system pre op- consider lateral supports, harness & head supportTilt & recline facilities recommended pre-op for any patient with scoliosis (Bushby et al, 2005)Tilt & recline vital post op if fused to pelvisMoulded wheelchairs are not appropriate post opLocal services to review post op to ensure corrective seating systemSpinal service relies on local therapists/ nursing teams and services to arrange provision of equipment locally
26SeatingIf fused to pelvis other seating systems can be used if have reclineLocal therapists to review postural support from seating systems post opPost op head rests, lateral supports, harnesses will still be required to maintain optimal postural alignmentSofas, beanbags are not acceptable seating systems!
27TransfersHoistingChildren that are lifted pre-op may require to be hoistedHoisting is dependent on age, size, weight and complexityHigh backed slings with head support recommendedBones in slings not necessaryThinner sling ideal- will be left in situ initiallyRemember to consider that child may require increased sling length post opResponsibility of local services to provide hoist training if new/ different equipment has been suppliedManual Lifts are not recommended for a year post surgery.
28Personal Care Toileting Ideal is recline & tilt- limited resources may result in tilt onlyShoweringRecommended in acute post op periodAlternative shower chair may be requiredfor postural supportBathingLong term extra postural support in bathmay be required
29Pre-operative Respiratory Function Extremely beneficial if families have been taught lung volume recruitment techniques and chest clearance techniques prior to admissionBritish Thoracic Society (www.brit-thoracic.org.ukScottish Muscle Network DMD Profile (www.smn.scot.nhs.uk)Peak cough flow can be assessed by using a mask and a peak flow meter,Comprehensive information on suitable respiratory techniques for neuromuscular patients can be found on the British Thoracic Society Website (http://www.brit-thoracic.org.uk) under Guidelines for the Physiotherapy Management of the Adult, Medical, Spontaneously breathing patient. The Scottish Muscle Network has also published a Paediatric Management of Duchenne Muscular Dystrophy Guideline (http://www.smn.scot.nhs.uk) where additional respiratory management guidelines can be obtained.It is extremely beneficial if families have been taught how to perform lung volume recruitment techniques and chest clearance techniques appropriate to the patient’s stage of their condition prior to their admission for spinal fusion.Patients will not be discharged home following spinal surgery until their chest condition is stable and families are able to manage any respiratory problems with some support from local care teams. The family will be more confident in managing any post-op short-term deterioration in respiratory function with support from the local physio if they have prior respiratory experience.Familiarisation with face mask in preparation for potential use of Bipap/ Cough Assist.Require 2.7l/min to clear secs1.60l/min can clear secs with breath stacking and assisted coughLess than 1.6l/min – may benefit from cough assist
30Hospital Admission Usually admitted the day prior to surgery Introduction/assessment by inter-disciplinary teamDiscussion of post operative managementIf bowels need prepped then admitted a couple of days prior to surgery.
31Operation – Posterior Spinal Fusion 15yrs 1mth40 oCombined anterior and posterior fusion is common in the treatment of patients with neuromuscular scoliosis, either because posterior elements are absent, as in myelodysplasia, or because it is necessary to gain correction in a rigid lumbar or thoracolumbar curve and achieve a spine fused in balance over a level pelvis62 oPelvis 6 oS.G., ♂
32Posterior Spinal Fusion +/- pelvic fixation Performed via a large midline incisionSpinous processes, interspinous ligaments and facet joints excisedPedicle Screws or hooks attached to spineIf fusing to the pelvis wires or pelvic screws are placedRods applied down either side of the spine and attached to screws and hooks as spinal deformity derotatedBone grafts placed around rods – usually femoral heads from bone bank or bone substitutesWound is closed with redivac drain insitu
33Anterior Release +/- posterior spinal fusion Performed via a thoracotomy – on the convexity of scoliosisA rib is excised for most of its length to access spine (and kept) – rib resectionRib heads may be removed around the apex of the scoliosis to improve cosmetic result – internal costoplastyPleura is excisedDiscs are excised and growth plates, cartilage removedWound closed with intercostal chest drain insituIndicated when scoliosis is very large, rib hump very pronounced.Skeletally very immature patients who may develop a “crankshaft deformity”When the posterior spine is fused and the anterior spine is left to grow.
34In patient Physiotherapy Reviewed day one post opChest physiotherapy commencedPassive/active assisted movementsBed mobility – log rollingMobility/ hoisting once medically stableLiaison with local therapistsOngoing until discharge from hospital
35Acute Post Op Challenges Surgical considerations – e.g. pelvic fixation- reclining seating positionsMedical stability – e.g. respiratory distressComfort – pain controlTonePsychosocial – anxietyNutritionIleus: Intestinal hypomotility may persist, necessitating prolonged parenteral support.Nutritional problems: When intestinal motility returns postoperatively but the child cannot tolerate oral feedings, a feeding tube can be passed into the stomach or duodenum to allow nutritional support until oral feeding is tolerated
36Discharge AdviceAdvise parents to cont passive/active assisted movementsTo increase mobility or duration sitting in wheelchairIf wheelchair reclined- to reduce recline as toleratedTo ensure postural alignment maintained – avoid forced flexion/ extension or rotation of spineOngoing respiratory management – as required
37Discharge Advice Unable to use standing frame and some walking aids Unable to swim/ hydrotherapy/ participate in sportsDiscretion of Consultant on reviewing patient and x-rays at clinic
38School ASL Profile provided Return to School – graded School seating Desk height/ positionHand function – writing skillsManual handling/hoistingToiletingFeeding
39Challenges after Discharge Home EnvironmentMobilitySelf propelling wheelchairsChange to Physiotherapy Program – Hippotherapy, Rebound etcFeedingFamily SupportTransportHolidaysAnxietiesFeeding crucial to promote good healing, however complications often occur such as SMA.Mobility may not be as good as before initially.Hydrotherapy is not allowed.Some kids are not allowed to self propel post op.
40ConclusionThere is variability with each child and we aim to make the pathway as smooth as possible for the patient / carers and local therapists