Physiotherapy Management of Neuromuscular Scoliosis Hannah Waugh 0131 536 0000 Bleep 9126 Specialist Physiotherapist, The Royal Hospital for Sick Children, Edinburgh

Contents What is Scoliosis? Medical Management Pre Operative Planning Hospital Admission Challenges post discharge

What is Scoliosis? Complex three dimensional deformity where the curve is greater than 10 degrees

Prevalence of Neuromuscular Scoliosis 20% of children with Cerebral Palsy 60% of children with Myelodysplasia 90% of children with Duchenne Muscular Dystrophy

Neuromuscular Scoliosis Development Spinal curvature may begin very early in life Often after the patient starts supported sitting Curve may progress rapidly once patient becomes non ambulant (averaging 10 degrees/year)

Initial Assessment 06.02.2007 14yrs 6mth 66 o 108 o Pelvis ? o S.G., ♂

Progression of curve – 4 months 06.02.2007 14yrs 6mth 26.06.2007 14yrs 10mth 66 o 58 o 108 o 122 o Pelvis ? o Pelvis 34 o S.G., ♂

Preventing Progression of Scoliosis Prolong mobility Steroids 24 hour postural management Spinal bracing (not always effective particularly in progressive neuromuscular curves)

Referral Criteria Consultant to consultant referral only Confirmed scoliosis - requesting specialist assessment for surgical intervention Neurological – usually after the age of 10 as surgery unlikely prior to this DMD – when patient becomes non ambulant

Initial Spinal Clinic Assessment In-depth history is taken scoliosis progression, pain, function past medical history medication social history Objective Assessment X-rays : standing or sitting to establish severity, bending films to identify flexibility – cobb angle, also check risser grade Physical assessment Asymmetry of trunk Uneven Shoulders Uneven Pelvis Listing to one side Adam’s Forward Bending Test Thoracic prominence Lumbar prominence Leg Length Plumb line From C7 spinous process to hang below buttocks Neurological examination Pain Numbness Tingling Weakness Muscle spasm Bowel/bladder changes To 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.[19] 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.[20] Patients with Duchenne muscular dystrophy and Friedreich ataxia should be evaluated for cardiac involvement.[21] 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]

Cobb Angle This is taken to monitor progression but is only one part of the assessment tool. Not everybody about a certain angle gets PSF.

Medical Management Dependent on: Severity of scoliosis Pelvic obliquity Age/Skeletal maturity – risser grade Rib deformity/ Impingement/ Pain Complexity of past medical history

Medical Management Cardiac Respiratory Anaesthetics Neurology/ Neurosurgery Endocrinology GI

Medical Management - DMD Respiratory Function Functional Ability Symptoms Quality of Life questionnaire Reduction in surgery Lots 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 etc Quality of Questionnaire – not scoring well Reduction in Surgery – Due to the above surgery has decreased from about 5-6/yr to 1/yr

Medical Management - CP Respiratory Function Functional Ability Symptoms Quality of Life questionnaire Surgery Respiratory Function Functional Ability – probably more often than not will improve their function due to the position change Symptoms – 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

Medical Management - mylominingecele Respiratory Function Functional Ability Symptoms Surgery Respiratory Function Functional Ability – floor work, often spinal surgeons request an OT and PT assessment to gauge how much function they have on the floor Symptoms – No pain

Medical Management Every case is very individual Function Medical Stability MDT decision Not just the size of the curve is considered. This is a major operation and at stages different things can go wrong

Medical Management Continue to monitor curve Use of conservative treatment PSF Intensivist Cardiac Consultant Neurologist

Physiotherapy Service Aims To ensure smooth pathway from pre admission to discharge To be available for contact to reduce any anxieties throughout the patient journey To be a resource for local therapists / services for Scotland

Spinal Surgery Pathway Contact made with local services & family Pre-op assessment completed Theatre list to Physio & OT Equipment requirements identified & commenced Local services review Discharge Admission Post-op

Physiotherapy Role To ensure that optimal functional abilities are achieved post operatively Those functional abilties include: respiratory function muscle strength transfers/ mobility postural management Overall aim is to maximise independence following surgery in activities of daily living Postural management is vital and should be considered through out all stages of spinal surgery

Physio Pre op Planning Commenced as soon as the patient is listed for theatre (approx 6 weeks) Facilitate smooth admission and discharge from hospital Early contact with local services is essential

Pre-operative Planning Unfortunately due to geographic location of clinics, unable to attend Contact will usually be made with the family and local therapists initially by telephone If patients admitted for respiratory tests, trial of NIV or attend for anaesthetic assessment we will meet and assess on ward if possible

Initial Pre-Op Assessment Physio /OT Establish current abilities of Seating (wheelchairs,other seating systems school, home) Transfers (independent, assisted, hoist) Mobility- use of walking aids Personal Hygiene (toileting, bathing/showering, level of assistance ,specific equipment) Respiratory function Other ADL activities (feeding, self dressing) School Environmental issues (access to and within house)- child may need to live downstairs

Seating Wheelchairs Should be in suitable corrective seating system pre op- consider lateral supports, harness & head support Tilt & recline facilities recommended pre-op for any patient with scoliosis (Bushby et al, 2005) Tilt & recline vital post op if fused to pelvis Moulded wheelchairs are not appropriate post op Local services to review post op to ensure corrective seating system Spinal service relies on local therapists/ nursing teams and services to arrange provision of equipment locally

Seating If fused to pelvis other seating systems can be used if have recline Local therapists to review postural support from seating systems post op Post op head rests, lateral supports, harnesses will still be required to maintain optimal postural alignment Sofas, beanbags are not acceptable seating systems!

Transfers Hoisting Children that are lifted pre-op may require to be hoisted Hoisting is dependent on age, size, weight and complexity High backed slings with head support recommended Bones in slings not necessary Thinner sling ideal- will be left in situ initially Remember to consider that child may require increased sling length post op Responsibility of local services to provide hoist training if new/ different equipment has been supplied Manual Lifts are not recommended for a year post surgery.

Personal Care Toileting Ideal is recline & tilt- limited resources may result in tilt only Showering Recommended in acute post op period Alternative shower chair may be required for postural support Bathing Long term extra postural support in bath may be required

Pre-operative Respiratory Function Extremely beneficial if families have been taught lung volume recruitment techniques and chest clearance techniques prior to admission British Thoracic Society (www.brit-thoracic.org.uk Scottish 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 secs 1.60l/min can clear secs with breath stacking and assisted cough Less than 1.6l/min – may benefit from cough assist

Hospital Admission Usually admitted the day prior to surgery Introduction/assessment by inter-disciplinary team Discussion of post operative management If bowels need prepped then admitted a couple of days prior to surgery.

Operation – Posterior Spinal Fusion 20.09.2007 15yrs 1mth 40 o Combined 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 pelvis 62 o Pelvis 6 o S.G., ♂

Posterior Spinal Fusion +/- pelvic fixation Performed via a large midline incision Spinous processes, interspinous ligaments and facet joints excised Pedicle Screws or hooks attached to spine If fusing to the pelvis wires or pelvic screws are placed Rods applied down either side of the spine and attached to screws and hooks as spinal deformity derotated Bone grafts placed around rods – usually femoral heads from bone bank or bone substitutes Wound is closed with redivac drain insitu

Anterior Release +/- posterior spinal fusion Performed via a thoracotomy – on the convexity of scoliosis A rib is excised for most of its length to access spine (and kept) – rib resection Rib heads may be removed around the apex of the scoliosis to improve cosmetic result – internal costoplasty Pleura is excised Discs are excised and growth plates, cartilage removed Wound closed with intercostal chest drain insitu Indicated 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.

In patient Physiotherapy Reviewed day one post op Chest physiotherapy commenced Passive/active assisted movements Bed mobility – log rolling Mobility/ hoisting once medically stable Liaison with local therapists Ongoing until discharge from hospital

Acute Post Op Challenges Surgical considerations – e.g. pelvic fixation- reclining seating positions Medical stability – e.g. respiratory distress Comfort – pain control Tone Psychosocial – anxiety Nutrition Ileus: 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

Discharge Advice Advise parents to cont passive/active assisted movements To increase mobility or duration sitting in wheelchair If wheelchair reclined- to reduce recline as tolerated To ensure postural alignment maintained – avoid forced flexion/ extension or rotation of spine Ongoing respiratory management – as required

Discharge Advice Unable to use standing frame and some walking aids Unable to swim/ hydrotherapy/ participate in sports Discretion of Consultant on reviewing patient and x-rays at clinic

School ASL Profile provided Return to School – graded School seating Desk height/ position Hand function – writing skills Manual handling/hoisting Toileting Feeding

Challenges after Discharge Home Environment Mobility Self propelling wheelchairs Change to Physiotherapy Program – Hippotherapy, Rebound etc Feeding Family Support Transport Holidays Anxieties Feeding 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.

Conclusion There is variability with each child and we aim to make the pathway as smooth as possible for the patient / carers and local therapists