Søren Eiskjær, lektor, specialeansvarlig overlæge ... · cerebral palsy Søren Eiskjær, lektor, specialeansvarlig overlæge Ortopædkir. afd.,Aalborg UniversitetsHospital . Asworth

The spine and cerebral palsy

Søren Eiskjær, lektor, specialeansvarlig overlæge Ortopædkir. afd.,Aalborg UniversitetsHospital

Asworth Scale for grading of spasticity

0 no increase in muscle tone 1 slight increase in muscle tone giving a

catch and release when the limb was moved in flexion and extension

2 more marked increase in muscle tone, but limb moves easily

3 considerable increase in tone, passive movement difficult

4  limb rigid in flexion or extension

Grades the resistance encountered in a specific muscle group by means of passively moving the a limb through its range of motion at a non-specified velocity

Disposition

•  Nogle begreber •  Hvor hyppigt er skoliose når man har cerebral

parese (CP) •  Hvorfor får man skoliose når man har CP •  Hvordan ser skoliosen ud hos CP patienter •  Konservativ behandling – virker det •  Skoliosens naturhistorie ved CP •  Indikationer for skoliosekirurgi ved CP •  Ultrakort om forløb

Disposition

•  Præoperativ vurdering inden skoliosekirurgi ved CP •  Hvad fortæller vi CP patienten inden skoliosekirurgi •  Operationsteknik •  Hvor svært er det at sætte alle de skruer? •  Postoperativt forløb •  Cases •  Død og ødelæggelse •  Hvad med alle de røntgenbilleder – Tjernobyl? •  Livskvalitet efter skoliosekirurgi hos CP patienter •  Kan vi gøre noget bedre •  Etiske betragtninger •  Konklusion

Sitting

Sitting is a fundamental position of function and health for people with CP. The upright position facilitates vision, communication and mobility. It also facilitates feeding and protects pulmonary function by minimizing gastric reflux and aspiration

GMFSC

GMFCS:

CPCHILD

Cobb vinkel = kurvestørrelse:

•  Vinklen mellem øvre endeplade og nedre endeplade på den hvirvel der hælder mest i henholdsvis den ene og den anden retning.

Cobb angle

Incidence of scoliosis in CP:

•  Perfect symmetry: 2% of all •  Idiopathic scoliosis: (Cobb angle > 10°)

1-2% •  Neuromuscular scoliosis: 15%-80% of CP

population •  CP nonambulatory with total body

involvement: 62% incidence of scoliosis •  CP and bedridden: 100% incidence of

scoliosis

Deformity pattern CP patients:

Collapsing spine: Long C shaped curve Most often kyphoscoliotic but can also be lordoscoliotic Association between hip dislocation and scoliosis? Pelvic obliquity

Etiology

•  Muscle weakness and truncal imbalance in CP patients

•  Incidence and severity of scoliosis in CP patients appear to be related to the degree of involvement

•  Directly related to the primary cerebral injury or secondary impairments – muscle weakness, spasticity, poor balance, nonabulatory status

Conservative treatment:

•  Bracing

•  Customized seating arrangements

•  Combinations

Braces and wheelchair inserts:

Conservative treatment

•  Can improve sitting balance

•  Cannot control scoliosis progression

•  Poor patient/caregiver compliance

•  Respiration?

Annual changes in radiographic indices in CP patients:

•  Lee SY et al 2015 •  Annual Cobb increase 3.4° •  Annual thoracic kyphosis angle increase 2.2° •  Annual increase in apical vertebral translation 5.4 mm

Indications for Surgery (GMFCS IV and V):

•  Cobb angle > 40° •  Kyphosis angle > 70°

•  No effect of conservative treatment •  Impaired sitting ability •  Pain •  Pulmonary dysfunction?

Surgery:

•  Duration of surgery 4-6h

•  ICU 1-2 days

•  Mandatory mobilisation to wheelchair the day after surgery

•  Discharge after 7-10 days – habitual lung function, pain control, defecation, no wound complications and CRP decrease

Preoperative preparation:

•  Often several consultations with participation of caregivers and parents

•  Sitting radiographs AP & Lat. •  Traction radiographs in supine position •  Lateral radiographs in supine position with

bolster under apex of thoracic kyphosis •  Evaluation of respiratory function:

Saturation/blood gases, number of respiratory infections/time interval

•  Nutritional evaluation: weigth/weight increasing/stable/decreasing – G tube

Preoperative preparation:

•  Patient contact evaluation •  Epilepsy •  Baclofen pump •  Wheel chair evaluation •  Brace evaluation •  Evaluation by anaesthesiologist •  SEP measurements •  MRI/CT in some cases •  Blood samples

Information before surgery:

•  The purpose is to improve sitting comfort and decrease pain and improve pulmonary and abdominal function

•  Respiratory complication are the most common and may result in prolonged need for mechanical ventillation and ICU care.

•  The worst case scenario is death •  Surgical complications are not infrequent

but are manageable.

Information before surgery:

•  The scoliosis curve will not progress after surgery

•  Bracing is not needed after surgery •  Parents and caregivers usually observes

improvements in QL after surgery •  Mobilisation to wheelchair the day after

surgery is mandatory •  The wheelchair will at least need

modifications after surgery

Moderne operationsteknik:

•  Segmentel instrumentering – 2 skruer på hvert niveau.

•  Release af kurve – kurven frigøres.

•  Stive stave.

•  Derotation af stav.

•  Derotation af kurvens toppunkt.

•  In situ bøjning af stav.

•  Distraktion og kompression




•  Stive stave.








•  Stive stave. (cobolt chrome, vitallium)








•  Stive stave.

•  Derotation af stav (90° eller mere).







•  Stive stave.








•  Stive stave.








•  Stive stave.




•  Distraktion og kompression.

Cantilever technique: Chang et al, Spine 2009




•  Stive stave.




•  Distraktion og kompression.


•  Sidste stav påsættes.

•  Knogletransplantat.

•  Smertekateter.

•  Dræn.

•  Lukning af sår

Hvad er O-armen: •  Kan både rtg. gennemlyse og lave

scanninger i 3D (CT-scannning). •  Motoriseret. •  Kan huske 5 positioner og gemme disse I

hukommelsen så de kan genkaldes på et hvert tidspunkt under operationen.

•  3D scanningerne anvendes til navigation sammen med et navigationssystem (Stealth Station).

•  Vejer ca. 800kg •  Koster ca. 5 mill. kr

Hvad er navigation: At fastslå hvor man er og finde ud af hvordan man kommer frem til sit bestemmelsessted.

Er det navigation?

Hvor er vi? Sæt ”lanterner” på. (LED’s). Brug faste objekter.

Hvor er vi? Registrer position. (Stealth Station)

Hvor er vi? Tegn kort over stederne. (3D scan med O-arm)

Computer

Spine coordinates

O-arm coordinates

Navigation med O-arm:

Kurvekorrektion:

•  Minimum 50 % (50° til 25°).

•  Gennemsnitlig 70 % (50° til 15°).

Postop:

•  Drain, urinary catheter, epidural catheter, central venous catheter, direct measurement of arterial pressure

•  Mobilisation to wheelchair the day after surgery

•  Antibiotics for 3 days (iv) •  Nutrition starting the day after surgery •  Epidural analgesics for 1-3 days •  CPAP •  Physiotherapy day 1.

Postop:

•  Baclofen pump regulation if needed •  Epilepsy medication •  Laxatives •  Peroral analgesics: Tramadol or morphine

day 3. •  No NSAID’s - bone healing! •  Control radiographs day 4 •  Wound inspection, blood samples •  Discharge day 7-10.

Case EDL

•  14 y, CP, GMFCS level V •  Repeat pneumonia •  CO2 controlled ventillation •  Epilepsy •  G tube •  Baclofen pump •  Pediatricians advocates no surgery •  Family wants surgery – sister improved

after scoliosis surgery

Case EDL

Case EDL

•  Thoracic Cobb angle 84°

•  Kyphosis angle 95°

•  Some pelvic obliquity

•  Supine traction radiography: Rigid curve

Case EDL

Case EDL

•  Prolonged time in the ICU

•  Pneumonia 2-3 times postop.

•  Epilepsy

•  No wound infections, skin perforations, loosenings or implant breakages so far

Case FE

•  15 y, CP, GMFSC V

•  G tube

•  No Baclofen pump

•  Epilepsy

•  Rapidly progressing curve

Case FE

Case FE

•  Cobb angle 100°

•  Lordoscoliotic

•  Supine traction radiographs: Relatively flexible curve

•  Considerable obliquity of pelvis

Case FE

Case FE

Case FE

•  After 1.5 y; skin defect, fistula, deep infection

•  Several reoperations

•  Partial removal of implants

•  Infection controlled

Case SS

•  12 y, w 20kg, GMFCS V

•  Epilepsy

•  G tube

•  Baclofen pump

•  Repeat pneumonia

Case SS

Case SS

•  Cobb angle thoracolumbar; 67°

•  Kyphosis angle; 72°

•  Some pelvic obliquity

•  Flexible curve

Growt guiding: Shilla

Shilla Growing Screws Avoid repeated lengthenings

Polyaxial screw

Shilla cap

Fixed at apex

4.5 rod

Polyaxial screw with Shilla cap

Monoaxial screw without Shilla cap

Subfascial screws (Navigation, Fluoroscopy, O-arm)

Subperiostal screws, rod rotation, apical derotation, fusion

Case SS

Case SS

•  ICU prolonged

•  After 9 months perforation of skin – cranial rod end.

•  Infection

•  Removal of implants after 2 y

•  Final surgery after 3 y – Cobb increase to 71°

Case SS

Case SS

Case MI

•  4 y, CP, GMFCS 4/5

•  Well nourished

•  No G tube

•  No Baclofen pump

•  Epilepsy

Case MI

Case MI

•  Thoracolumbar Cobb angle 43°

•  Slightly kyphoscoliotic curve

•  Slight pelvic obliquity

•  Flexible curve

Magec rod: Magnetically controlled growing rods

Case MI

Case MI

Case MI

•  Magec rod

•  Uncomplicated operation and post-operative course

•  Curve correction satisfactory

•  Adequate distraction so far

Complications: Legg et al 2013

•  Large variation in the overall risk of complications (range 10.9%-70.9%)

•  Large variation in mortality (range 2.8%-19%)

•  Large variation in respiratory complications (range 2.5%-57.1%)

•  Large variation in infection (range 2.5%-56.8%)

•  Worse outcome: significant degree of thoracic kyphosis, days in ICU, poor nutrtional status

Complications: Legg et al

Mortality: Legg et al.

Etiology of death: Tsirikos et al

Factors affecting survival: Tsirikos et al

Pneumonia: Keskinen et al 2015

•  Lifetime risk of pneumonia in neuromuscular scoliosis: the role of spinal deformity surgery

•  N=42 •  Pneumonia needing hospital admission •  CP and non-CP •  15 thoracotomies •  Incidence of pneumonia 2/100 before and 0.7/100

after surgery in CP patients •  Risk factors epilepsy, non-CP, preop main curve >

70° •  Conclusion: Surgery before curves progress to major

curves.

EOS:

•  Fransk udviklet (Nobelpristager – Charpak).

•  Skolioseopt. i 2 planer.

•  Reduktion af stråledosis med en faktor 9-10.

•  Bedre billedkvalitet.

•  Undersøgelsen udføres hurtigere end med normal teknik.

•  Koster 5-6 mill. kr.

CP chair for use in EOS:

Quality of life after scoliosis surgery in CP patients:

•  Botz et al. 2011: N=50 •  CPCHILD. Statistically significant improvement

from preop to postop: Domains; comfort and emotion, health, overall quality of life. Item pain significant improvement.

•  91.7% of patients were highly satisfied or satisfied with the outcome of operation and would definitely undergo the operation again

•  Limitations: Retrospective; Questionnaires administered at the same time


•  Difazio et al 2015: N=44 (hip30/spine14) •  Significant increase in mean CPCHILD score from

preop to postop (1y) •  Significant improvements in domains; personal

care/activities of daily living, positioning and transferring and mobility, comfort and emotion, communication, health

•  Caregivers burden (ACEND) was unchanged •  Limitations: No control group


•  Sewell et al 2015: N=18, Controls 15 •  Prospective study, though non-consecutive •  Children who underwent surgery had a significant

improvement in the overall CPCHILD score from 45 to 58 (improved sitting balance and pain reduction)

•  Small decrease in CPCHILD score in control group (no surgery) 50 to 48.

•  4 wound infections, 3 chest infections, 2 haematomas/seromas (50%)

•  Limitations: Selection bias

Ethics – four topic model: Whitaker et al 2015

•  Medical indications

•  Patient preference

•  Quality of life

•  Contextual features

Medical Indications

•  Progressive curve of >40-45°; surgery arrests progression and corrects deformity +

•  Cardiopulmonary compromise; may improve subjective pulmonary function +

•  Pelvic obliquity Surgery improves pelvic obliquity + Surgery does not decrease decubiti ulcers ÷ Surgical morbidity ÷ Surgery associated with decreased life expectancy ÷/+

Patient Preferences

•  Most patients with GMFCS level IV or V are incapable of participating actively or directly in decision-making to a meaningfull extent

•  In this clinical setting the preferences of the patients are unknown and assessment of outcome shift to secondary parties.

Quality of Life

•  Surgery has a high rate of complications ÷ •  Surgery may reduce pain due to severe

spinal deformity + •  Surgery may require revision ÷ •  Surgery increases hospitalization ÷ •  Surgey improves posture + •  Surgery may enhance interaction with

surroundings+

Contextual features

•  Altruism including concern about denial of care +

•  Decreased caregiver fatigue + •  Cognitive disonance ÷ •  Influence of industry ÷ •  Cost to health care system ÷ •  Physician incentive in fee for service

reimbursement (reward for activity) ÷ •  Parents and caregivers wait until it is

evident that surgery is needed which perhaps is too late ÷

Anything we can do better:

•  Patient selection? •  Patient information: Complications, realistic expectations •  Timing of surgery? •  Preparation for surgery: Respiratory training?

Hypernutrition? Epilepsy medication? •  Surgical techniques: Segmental

instrumentation,Posterior release with Ponte osteotomies, VCR, Haemostasis, Navigation, MEP

•  Infection prophylaxis: Pulse lavage? Urinary cultures before surgery, Vancomycin powder applied in the wound perop, broader spectrum iv antibiotics.

Anything we can do better:

•  In hospital: Shorter ICU stay, shorter intubation time, better cooperation with parents and caregivers

•  Outpatient clinic: Focus on complications •  Future: Less invasive surgery; Early Magec

rod?

Conclusions:

•  Detailed and timely selection of candidates for scoliosis surgery in CP patients is a prerequisit for a satisfactory result.

•  Information about realistic expectations regarding surgery and complications is absolutely necessary.

•  Complications are common. •  Mortality slightly increased in newer studies •  QL improves after scoliosis surgery in CP patients. •  Ethical analysis justifies scoliosis surgery in CP

patients

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Søren Eiskjær, lektor, specialeansvarlig overlæge ... · cerebral palsy Søren Eiskjær, lektor, specialeansvarlig overlæge Ortopædkir. afd.,Aalborg UniversitetsHospital . Asworth