Spina Bifida

SPINA BIFIDA

Dr Kevin DUNNE Developmental Medicine Rehabilitation Service 2010

Spina Bifida Spina Bifida

„ Aetiology/Classification „ Malformation of spinal cord and brain „ Incidence/ Prevalence/Prevention „ 28 days of development of embryo „ „ Initial assessment Failure of fusion of neural folds during neuralation „ Clinical Description „ Cause unknown? „ Management „ ?Genetic ?Metabolic ? Environmental „ Orthopaedic/Neuro/urology „ 75% isolated „ Education/social „ 25% associated with other defects „ Outcome

Spina Bifida Spina Bifida

„ Partial paralysis of lower limbs Neurogenic bladder „ No weakness Urinary incontinence „ Partial paralysis renal and bladder abnormalities „ Wheelchair dependence Neurogenic bowel Faecal incontinence „ Joint deformity Skin „ Spinal abnormalities anaesthetic –pressure sores „ Scoliosis „ Kyphosis/lordosis

1 Spina Bifida Spina Bifida

„ Hydrocephalus „ spina bifida occulta „ 10% adult spines

„ Arnold Chiari Type 2

„ CSF shunt „ menilingocele „ 6% cases „ Learning problems „ 11% survivors

„ Upper spinal cord mild abnormalities „ myelomeningocoele „ 94% cases „ 89%survivors „ Mild upper limb problems „ Encephalocele

„ Lipomeningocele

Spina Bifida -types- types Myelomeningocele

„ N Dysraphisn Meningocele MM

Spina Bifida Spina Bifida Thoracic

2 Spina Bifida Meningocele Severe

Incidence Spina BifidaBifida--incidenceincidence

„ Australia „ 0.95/1000 „ Varies from country to country „ Range 11-- 5 /1000 live births „ Western Europe „ 1.51.5--3.03.0/1000 „ High in Celts (Ireland , Wales)

„ Low in Northern Europe. „ Sex Incidence „ Encephaloceles higher in SE Asia „ Male „ 58%

„ Female „ 42%

Foetal neural tube Spina Bifida

„ Conception -D18- D18 „ 3 germ layers which make the different tissues and the neural plate forms along length of fetus QuickTime™ and a decompressor are needed to see this picture. „ D19 -D28- D28

„ Neural tubes folds and should close along length of foetus

3 Spina BifidaBifida-- Classification Myelomeningocele

„ Neural tube defect „ Cervical 0.5% mild

„ Anencephaly 50% (lethal) „ Thoracic only 0.5% Severe „ Spina Bifida Cystica „ Low thoracic 27% Severe „ Myelomeningcele 45% (2/3 – 90% live) „ „ Meningocele 5% (all live) High Lumbar L1L1--2223% WChair „ Encephalocele „ Low Lumb,Upper sacral 45%Braces

„ Lipomyelomenigocele „ Lower sacral 4% Independ „ Clinically similar but no Hydrocephalus

„ Spina Bifida Occulta

„ ?10 % back pain, constipation

Lipomyelomeningocele Lipomeningocele

„ Lipoma

„ Grows in the spinal canal or outside

„ Press on the spinal cord

„ Nerves traverse and become nonfunctional

„ Partial weakness of lower limbs

„ Neurogenic bladder and bowel

„ Spine abnormality

„ No hydrocephalus – normal cognition

Spina BifidaBifida-- Aetiology Genetics

„ Unknown –multifactorial– multifactorial „ MultiMulti--factorialfactorial „ Abnormal gene interacts with other genetic loci and or environmental factors to modulate the incidence or „ 1 affected child 1:50 Recurrence Risk

severity of the defect. „ 2 affected children 1:10 RR

„ Genetic „ 3 affected children 1:4 RR „ Increased in chromosomal abnormalities i.e. trisomy 13 and 18. Note „ Increased in siblings and other relatives „ Risk of any child born with a major defect 1:30 (3%) „ Abnormal genes „ If an adult with Spina Bifida has a child risk is 1:25 „ Som „ VANGL1 (mouse, 3/166) (NEJM 356,(14)2007,

4 Spina BifidaBifida-- aetiology Incidence

„ Drugs „ Australia 0.95/1000 Valproate (1%) „ Western Europe 1.5- 1.5-33/1000 Methotrexate Clomiphene „ Male 58% Folic acid antagonists Trimethaprim, Anticonvulsants Environment „ Incidence has decreased significantly in Maternal diabetes, Hyperthermia, Victoria, largely due to termination, PaternalPaternal-- Agent orange secondarily due to folate.

Spina Bifida Spina Bifida - Prevalence Common Birth Defect

„ NTD conceptions in Victoria

„ 40% decline 1995 to 2000

„ Due to folic acid supplementation

„ 1995 2005

„ NTD 19.2 -12.2/10000 -12.2/10000

„ Spina Bifida 8.3 -7.9/10000 -7.9/10000

„ Increase in encephalocele (increased Asian)

„ Reduced births due to TOP

Spina Bifida Number of new cases/year Royal Children’s Hospital

30

25

20

15 number new SB cases 10

5

0 1970 1990 2000 2006 2008

5 Spina Bifida Spina BifidaBifida--PreventionPrevention Incidence

„ So why have the numbers dropped „ 1960’s Laurence in Wales ?Vitamin Deficiency. „ 1970’s Smithells Periconceptual multivitamins „ Antenatal diagnosis with ultrasound RdReduced concepti on rat e if previ ous NTD Methodological flaws „ Termination of pregnancy „ MRC Study 1992 „ Folate – small impact „ Multi centre trial „ Stopped at 1400 (n = 2000) „ 76% reduction recurrence risk for NTD „ Small drop in prevalence „ Folate rather than other Vitamins the cause

Folate SB --FolateFolate Prevention

„ Folate supplementation reduces recurrence by How does it work – unknown up to 72% Lancet 1991 Generalizable.? Berry NEJM 2004 „ RdRecommend - 130000 Chinese women 0.4 mg folate,117000 controls 1 month prepre-- and 3 months postpost--conceptionconception – 41% reduction NTD „ Routine 0.5mg daily Risks –nil reported ? Twins „ High risk pregnancy 5mg oral daily „ Parent with SB How much? „ Parent with a previously affected child „ Mother on AntiAnti--EpilepticEpileptic Drugs 0.4mg –36% „ Mother with Diabetes Mellitus 5mg - 85%

PeriPeri--conceptualconceptual folic acid NTD Recurrence Risk Awareness post 1996,1999

Before 1997 2000 „ 0ne child 2% „ Two Children 10% 1515--2424 yrs 5% 6% 4% „ Parent NTD 4%

„ Blood related 1% 2525--3434 yrs19% 24% 29%

34yrs+ 12% 16% 26% „ Remarry 0.6%

6 Spina Bifida SB – Folate to flour

„ 12.2 NTD per 10000 „ Folate added to flour conceptions „ Average daily consumption „ 85 NTD for 70000 „ Supplement to 0.4 mg conceptions Vic pa

„ 5 new case treated „ USA and 38 other countries

„ 1% blood relative risk „ 36 % reduction in NTD

„ Australia about to start

Spina Bifida MultidisiplinaryTeam Management

„ Allied Health Medical

„ Stomal therapist Urologist

„ Physiotherapist Neurosurgeon Occupational therapist Orthopaedics

„ Orthotist Neonatologist

„

„ Paediatrician

Spina Bifida Multidisciplinary team Antenatal diagnosis

„ Neuropsychologist „ Ultrasound „ Social Worker „ Lesion vertebral column

„ Clinical Psychologist „ Hydrocephalus – lemon sign

„ Education „ Amniocentesis „ General practitioner „ AFP, open lesion only

„ Maternal Serum Screening „ Spina Bifida Association -social-social „ AFP non specific (twins, down etc)

7 Spina Bifida Spina Bifida Antenatal Ultrasound Antenatal Ultrasound

„ Can visualise at 1515--1616 weeks „ Ultrasound –“sac”– “sac” „ Vertebra have 3 ossification centres

Spina Bifida Spina Bifida Antenatal ultrasound Foetal Surgery

„ Lemon and banana sign „ Does foetal surgery improve outcome

„ Randomized trial USA

„ Ongoing at moment

„ No data yet

Spina Bifida Termination Mode of Delivery

„ Prenatal Ultrasound „ Improved outcome with elective LUCS „ 1818--2020 weeks „ Use if leg movement seen on US

„ Detailed foetal anatomy „ Rationale „ FetoFeto--maternalmaternal obstetric outpatients for „ – reduces damage to neural plaque discussions and counselling „ 2 neurosegmental levels better „ Termination at 20-20-2222 weeks should the parents choose „ Bladder and bowel status unchanged „ (LUTHY et al NEJM 1989)

8 Spina Bifida Spina Bifida Initial Assessment Initial Assessment

„ Refer specialist centre „ Clinical exam –chromosomal/other „ Neurosurgical assessment „ Extent of lesion –can– can it be closed „ Paediatric assessment „ Motor level „ Discussion with family „ Hydrocephalus cranial US „ Facts not biases „ Musculoskeletal Hip US „ If severe - non treatment option „ Joint deformity „ Family’s wishes respected „ Spine deformity „ Palliative care –10% survive „ Renal Renal US

Spina Bifida Spina Bifida Initial Assessment

„ “to treat or not to treat” „ Treatment considerations „ Prior 1955 most died

„ Hydrocephalus, Meningitis , Renal „ Severe paralysis „ 1955 CSF shunt (VA), Ileal conduits „ Severe hydrocephalus „ 19551955--7070 many with severe abnormalities „ Kyphosis „ Treatment Criteria „ Other congenital „ Lorber 1971 abnormalities „ Smith and Smith 1973

Spina Bifida SB - Neurosurgical Initial Treatment

„ Back closure within 24 hours (infection) „ Back Closure „ Prophylactic antibiotics „ Within 24 hours

„ Delay closure for 72 hour „ Preserve nerves „ Monitor for hydrocephalus „ Motor deterioration post surgery

„ Back closure head circumference increases „ „ Shunt insertion about 1 week later Investigations US, CT MRI

„ Hospital 11--33 weeks „ Back closure - hydrocephalus

9 Spina Bifida SB - Hydrocephalus Hydrocephalus

„ 8080--90%90% develop significant hydrocephalus „ Increased with higher level

„ 26% present at birth and by 1 month of age in 77% „ Rarely develops after 6 months „ Frequently progress after back closure/ „ Most shunts in 1st month.

SB - Hydrocephalus CSF Shunt

„ Arnold chiari type 2 „ VP shunt

„ Complications

„ Infection, obstruction, disconnection

„ Low pressure

Arnold Chiari Type 2 Tethered Cord Causes

„ Myelomeningocele

„ Post operative

„ Tight filum

„ Lipoma/lipomeningocele

„ Split Cord (diastematomyelia) „ Dermal sinus

„ Tight or fatty filum terminale

10 Tethered Cord Symptoms Spina Bifida Tethering

„ Pain „ WeaknessWeakness-- evolving „ Sensory Loss „ Incontinence „ Scoliosis „ Cutaneous markers

„ Investigate Muscle chart, MRI

Lipoma -tethering-tethering Diastematomyelia

Neurosurgical Clinical Filum terminale

„ Epilepsy

„ 10 –15%

„ Increased with shunt obstruction, Infection, ventriculitis

„ Arachnoid Cysts

„ Sphyrinx

11 Spina Bifida SB Orthopedic Aims

„ Orthopaedic „ Pattern of motor development near normal

„ Maximize mobility

„ Stable posture if standing

„ Centre of gravity over feet

„ Prevent pelvic obliquity „ Correct spinal deformity

„ “stability of skin”

SB Orthopaedics SB - Orthopaedic Principals Hip

„ Developmental knowledge „ Hip Flexion contracture „ Natural history of condition „ Anterior hip release

„ Monitor progress „ Femoral or pelvic osteotomies „ „ Time interventions Abduction External Rotation Deformity „ „ Investigations Limitation of Abduction

„ Imaging „ Hip surveillance prevent dislocation

„ Muscle Charts

„ Gait analysis

SB Orthopaedic SB Orthopaedics Hip dislocation Knee

„ Thoracic L1, L2, L3 53% „ Valgus deformity „ L4 33% „ Flail undeformed knee „ L5 20% „ Undeformed knee with „ Bilateral reduced quadricep „ Operative benefit ?marginal „ Fixed flexion „ Pain „ Prevent hip flexion contracture – or fit an orthosis (RGO) „ Treatment „ Cast „ Unilateral „ Braces „ Leg length discrepancy, pelvic obliquity „ Muscle transfers „ Ducubiti „ Ostetomies „ Operate on low lesion

12 Deformities in Ankle Feet Clubbed Feet

„ The deformities include; Equinus deformity Clubfoot or talippqes equino varus deformity . Calcaneal deformity. Cavus or cavocavo--varusvarus deformity. PlanoPlano--valgusvalgus deformity.

Spine deformity Scoliosis Surgery

„ Scoliosis „ Kyphosis

„ Lordosis

„ Monitored by Xray

„ Worsen with age „ Adolescence

„ Pain , decubiti sitting „ Brace

„ Surgery

SB Orthopaedics Techniques AFO

„ Casting

„ Orthosis

„ Soft tissue releases

„ Muscle transfers

„ Bony procedures „ Osteotomies

„ Arthodesis

„ Ilizarov frames

13 RGO KAFO Reciprocating gait orthosis

„ Knee and ankle stability

„ Mobilize with crutches

„ Lock to enable sitting

Spina Bifida Muscles and movement 3 Urology

„ Physiotherapist „ Bladder „ In community provide regular therapy „ Bowel „ Eqqpuipment assessment and funding „ Skin „ Independent skills - transfers/muscle strength „ RCH service 6-6-12mly12mly muscle mapping

„ Orthopedic surgeries „ Major reason for admission

SB SB Urology Neurogenic Bladder Treatment

„ 90% plus affected „ Principles „ Protect the kidney’s from damage „ Inervation S2S2--44 „ Social continence „ Associations „ Management

„ VU reflux „ Evaluate renal tract (imaging, urodynamics) „ Regular imaging ( deterioration silent) „ Renal Anomalies (horseshoe kidney) „ Prevent infection (Bactrim, keflex, macrodantin, „ Bladder wall trabeculation cranberry)

„ UTI/pyelonephritis „ Treat infection „ Investigate deterioration „ Surgery

14 SB Neuropathic Bladder 2 Neurogenic Bladder Bladder type Contractile Intermediate Acontractile „ Types *most common Detrusor Strongly Continuous weak Doesn’t contract contraction „ Areflexic (acontractile) Sphincter tone High Weak/ ineffective Weak „ Enlar ged „ Urine dribbles Outflow Obstructed Obstructed Mild resistance „ Sphincter incompetent (incomplete relaxation) „ common Effects High residual volume, Small capacity and Continuous dribbling small bladder capacity continuous dribbling

„ Contractile (Spastic) Upper tracts At riskAt riskRarely at risk „ Small Volume „ Thick wall Options C.I.C C.I.C Anticholinergic „ Sphincterotomy Sphincterotomy Botox AND C.I.C Augmentation Augmentation

SB Neurogenic Bladder Neuropathic bladder 4 Investigations 1. Preserve renal function (ensure emptying and prevent infection) „ Renal Ultrasounds Yearly 2. Achieve continence „ MCU prn

„ Medical „ Surgical „ Nuclear Scans Baseline prn „ Clean intermittent „ Botulinum toxin catheterisation „ Sphincterotomy „ Urodynamics Prn „ Anticholinergics (reduce „ Bladder augmentation detrusor hyperreflexia) „ Cystoscopy prn „ Artificial urinary sphincter „ Manage infections „ Vesicostomy, urinary diversion/ undiversion „ Mitroffanoff procedure

SB Urology SB Urodynamics Surgery

„ Reflux surgery „ Vesicostomies „ high pressure, infection „ Bladder Augmentation „ Enlarge the bladder (mucus, rupture, cancer) „ Flap from Ureter, stomach, bowel, cultured cells „ Mitrofanoff - appendix for anterior access „ Artificial Sphincters „ Select candidates „ Ileal Conduits „ Bleed, Stenosis, stones, Cancer, Last 10 years

15 SB Augmentation cystoplasty SB Artificial Sphincter

SB Urology SB Urology Recent Developments Continence

„ Botox „ Initially nappies

„ High pressure bladder „ Prophalactic antibiotics

„ Detrusor instability „ Clean intermittent catheterization 3 -5/day „ Pads „ Nerve Transplants „ Bokka Pants

„ Chinese research „ Condom drainage „ Improved function „ Medication eg oxybutinin „ Trials underway in USA „ Surgery

Spina Bifida Neurogenic Bowel Neuropathic bowel 1

„ 90% + have faecal incontinence „ 25% of adults with Spina Bifida are bowel continent „ Sensory nerve to colon, rectum, anus „ Most who are incontinent have poor sensation „ S2-4 AND either „ Autonomic nerves

„ Major social problem i. Increased bowel Constipation and outlet resistance overflow diarrhoea ii. Decreased bowel Frequent stools outlet resistance throughout the day

16 SB Neurogenic bowel SB Neurogenic Bowel Types Principles

„ Patulous anus and constipation „ Aim for social continence „ Diarrhoa „ No single program that works

„ Diet sensitive „ Bowel management starts from birth

„ 1010-- 20% „ Try to have a program by school „ Loperamide „ Teenagers and adults work it out „ Patulous anus and constant leakage

„ Seen in mobile (abdominal compression)

SB Neurogenic Bowel SB Neurogenic Bowel Treatment Treatments

„ Nappies initially „ Suppositories „ Diet „ Microlax enema’s „ Give 4-4-55 hour clean - good for school „ Laxatives often worsen but used. „ BlBowel wash outs „ Movicol „ 2 or 3 per week „ Timed Toileting „ Malone antegrade enema „ Pressure „ Anal Plugs „ Digital stimulation „ Work well in some 30% „ , expensive, last 6 hrs „ Combinations „ Buttock strapping eg for swimming

SB neurogenic Bowel SB Neurogenic Bowel Malone procedure Malone/ Anal plugs

17 Malone Antegrade Bowel Washout Washout Peristeen System

SB - Bowel Management Experimental SB Pressure sores

„ Gracilis slings „ Skin Level

„ dermatomes

„ Electrical stimulation „ Mapped using pin prick „ Can be different to motor level

„ Problem anaesthetic skin „ Cuff similar to Artifical Sphincter. „ Pressure areas sitting braces

„ Burns

„ friction

SB Decubitus Decubitus

18 Spina Bifida SB Pressure sores Pressure Sores

„ Prevalence 20 -25%-25% „ Prevention „ Pressure care training, regular lifts „ 80 –90% will have at some stage „ Correct fitting orthosis „ Cause „ Pressure cushions „ Anaesthetic skin

„ Excessive pressure 42% „ Site „ Orthosis 23% „ Low Lumbar, Sacral „ Feet, buttocks „ Urine, faecal soiling 23% „ Thoracic, high lumbar „ Friction 10% „ Spine, buttocks, feet

Spina Bifida Spina Bifida Other Medical Issues Latex Allergy

„ Recognized since late 1980’s „ Obesity „ ? Due to intra abdominal surgery „ Especially in adolescence „ Most Sensitivity „ Rash, lip swelling „ Stop walking - wheelchair „ Rubber gloves, catheters, balloons „ Endocrine

„ Some Anaphylaxis „ Precocious puberty, osteoporosis „ Risk during surgery „ „ Prevalence 33% on RAST Ophthalmology „ Treatment „ Psychological „ Test „ Latex free theatre „ Adults suicidal ideation 10%

Spina Bifida Cognitive function Cognitive

„ Intellectual disability „ General intelligence “normal range”

strongly associated „ Skewed to the lower end with hydrocephalus „ Sppgecific Learning difficulties

„ Verbal IQ > Performance IQ

„ Relative Deficit Increases with age

„ Primary school 5 points down --overestimatesoverestimates „ Secondary 10- 10-1515 points down

„ Factors

„ Hydrocephalus, shunt blockage, infection

19 Spina Bifida Spina Bifida Cognitive profile Cognitive profile

„ Executive Functions „ Visuo motor Integration „ Difficulty completing task „ Difficulty organizing task „ Delayed laterality „ Overwhelmed byyp complex new information „ Poor motor planning „ Impulsive „ Poor hand control „ Difficulty applying new knowledge „ Higher order Language „ Slow mastery of writing „ Information Processing (memory) „ Untidy and disorganized presentation or „ Attention Memory, New learning written work „ Poor sequential and working memory „ Sequencing

Spina Bifida SB School Educational Problems

„ Most attend normal school „ Lack of understanding about cognitive „ Special on cognitive grounds (Yooralla ) problems „ parent should visit schools „ Toileting expectations unrealistic „ UlliUsually require an i ntegrati on aid e „ Continence „ Academically problems around Grade 4 „ Cognitive „ Teacher zealous –disillusioned „ Safety „ School modifications ( access, toilets) „ Child withdrawn, depression „ Program Support group „ Socialization issues „ School therapy

Spina Bifida Spina Bifida Secondary School Therapy

„ Cognitive Difficulties more apparent „ Developmental program

„ Mobility/incontinence affect relationships „ Mainly physiotherapy

„ Peer group issue – isolation „ OT many have upper limb/ cognitive issue „ Poor body image „ Speech „ Sexuality issues „ RCH initially or

„ Impotence in males „ Specialist Children Services (home) „ Misery , low self esteem „ Centre Based from 3y „ Depression „ School based therapy

20 Spina Bifida Therapy Continence

„ Continence nurses „ Motor development depends on level „ Advise: management of the „ L3 and quads for walking bladder and bowels „ Training (CIC, washouts): „ Motor developmental program parents, carers , aides and patients „ Standing frames „ Applications for CAAS funding „ Walking frames „ Sourcing equipment (catheters, pads, nappies, „ Braces specialised underwear) „ Crutches „ Also Stomal Therapists „ Wheelchair training and Wound Care!

Spina Bifida SB Social problems Post School

„ Friendship difficulties „ Unrealistic expectations re careers „ Realization that they are different 77--8y8y „ Tertiary few

„ Exclusion by peers „ University a few .

„ Unable to keep up in playground „ TAFE „ „ Reduced self esteem Employment

„ Body image problems „ Independent Living

Spina Bifida Transition

„ CLINIC STAFF „ When complete high school „ Sometimes ongoing orthopaedics or „ Monitoring urology „ Medical 6 monthly „ Orthopaedic varies „ MECRS „ Physio annually „ Neurosurgery - annually „ Monash „ Urology 6 -12-12 monthly „ Bendigo, Ballarat, Geelong „ Ultrasounds 1-1-22 years

21 Spina Bifida Spina Bifida Adults Outcome Medical

„ Medical „ Urological

„ Psychological „ Recurrent UTI 38%

„ Emppyloyment „ Hypertension 11%

„ Housing „ Stomal Problems 58%

„ Review recommended 36%

„ Orthopaedic

Spina Bifida Adults Spina Bifida Adults Medical Medical

„ Orthopaedic „ Neurosurgical

„ Symptoms of cord tethering 14%

„ Backache 25% „ Shunt review 4%

„ Knee Instability 15% „ Seizures 2%

„ Hip pain 14%

„ Shoulder Pain 10%

„ Arthritis 6%

Spina Bifida Adults Spina Bifida Adults Education Psychological

„ Depression 5% „ 114 Seen in 1992 „ Anxiety 3% „ Tertiary 8%

„ Psychosis 2% „ Completed High School 16%

„ Suicide attempts 7% „ Special school 28%

„ Substance Abuse 7%

„ England 42% completed high school compared to 75% Controls (Tew 1984)

22 Spina Bifida Adults Spina Bifida Adults Pregnancy Employment -Home- Home

„ Competitive Employment 33% „ 17 women 23 pregnancies „ Not Working54% „ Less complications if Vaginal delivery

„ Living with parent 62% „ Recurrent UTI

„ Pyelonephritis

„ Back pain „ Pressure sores

The End

i. Which tissues are involved? ii. Where is the lesion?

„ Spina bifida occulta (bone „ 1% Cervical only (Level 1) only) „ 1% Thoracic only (Level 1) 10% adult spines

„ „ 6% Lower thoracic (Level 2) Meningocoele (bone and QuickTime™ and a QuickTime™ and a decompressor decompressor are needed to seeand this picture. Upper lumbar (Level 3) meninges) are needed to see this picture. 6% SB, 11% survivors „ 92% occur at L3 and below „ Myelomeningocoele [42% Lumbosacral jct (Level (bone, meninges, nerves) 4)] Lower sacral (Level 5) 94% SB, 89% survivors

23 Myelomeningocele Neuropathic Bowel 2 Functional Ambulation

„ Diet, fluids Non Ambulator Thoracic „ Regular sit, cough, push on toilet

„ Laxatives, enemas, washouts Household Thoracic –L3– L3

„ AllAnal plugs QuickTime™ and a Indoors only, braces decompressor are needed to see this picture. „ Biofeedback Wheelchair

„ Malone Procedure Community L3 –Sacral–Sacral (exclude other causes diarrhoea) Indoor/outdoor Braces Crutches Wheelchair long distances

SB –Prevention Sensation PeriPeri--conceptualconceptual folic acid „ Sensory loss below level of lesion „ Occupational „ Patchy therapists „ Folic acid 0.5mg „ Dense „ Seating advise „ „ High risk of pressure areas/burns (includes car) 2 month pre conception „ Slow healing (poor blood supply) „ Pressure care „ Family history 5mg . „ Historically, common cause of death „ Tools for function/ home modification (hoists, rails, handles, grips) „ Campaigns 1996,1999 „ Upper limb function QuickTime™ and a decompressor are needed to see this picture. „ Driver’s Licence

Roho cushion

SB Orthopaedic Spina Bifida Ilizarov frame Initial Assessment

„ Treatment considerations

„ Severe paralysis

„ Severe hydrocephalus

„ Kyphosis

„ Other congenital abnormalities

24 Spina BifidaBifida--NumbersNumbers

„ Treated at the RCH

„ 1970 27

„ 1980 23

„ 1990 15

„ 1995 5

„ 2000 4

„ 2005 4

Spina Bifida Embryology

25