IC11: The Single Event Multi-level Surgery (SEMLS) Went Well

– Now What? An Evidence-Based guide to pain management, orthotics, and rehabilitation in the first year after surgery to improving gait in children with

cerebral palsy

Vedant A. Kulkarni, MD Jon R. Davids, MD Karen Howes, FNP

Suzanne Bratkovich, PT

Shriners Hospitals for Children – Northern California / University of California, Davis Sacramento, CA USA

Full course material can be downloaded from: www.shrinerschildrens.org/aacpdm2017

Course Introduction

I. Four Phases of Single Event Multilevel Surgery (Priorities) a. Acute Inpatient Phase (Pain control, safe transfers, avoidance of complications of

surgery and immobility) b. Early Post-Operative Phase (Pain control, avoidance of complications, gentle

improvement in range of motion, preparation for ambulation) c. Early Intensive Rehabilitation (Weight bearing, Range of Motion, Gait Training) d. Outpatient Rehabilitation (Endurance, Strength, Functional Improvements)

II. SEMLS Journey Board – Appendix 1

Acute Inpatient

Early Postop

Early Intensive Rehab

Outpatient Rehab

Phase 1: Acute Inpatient Phase

I. Principles of Acute Pain Management a. Multi-modal pain pathway b. Get ahead of the pain

II. Structure of Pain Management Team a. Individual Consultants b. Co-management c. Acute Pain Service

III. Modalities for Pain Management a. Neuraxial Anesthesia

i. Epidural Anesthetic ii. Intrathecal Spinal Anesthetic

iii. Regional Anesthetic b. Intravenous Modalities

i. Ketamine ii. Demedetomidine

iii. Naloxone iv. Methadone

c. Oral Modalities i. Opioid

ii. Acetaminophen iii. Non-steroidal anti-inflammatories iv. Clonidine

IV. Neuraxial Anesthesia Protocols – Appendix 2

V. Post-Operative Urinary Retention

a. Frequency (!) of the problem b. Prevention of Urinary Retention c. Management of Urinary Retention

Patient Age

Max Bladder Volume

Formula (under 12 Yrs)

(Age x30)+30 =Max bladder volume in mL

1/3 rd of Max Bladder Volume

2 90 ml 30 ml 3 120 ml 40 ml 4 150 ml 50 ml 5 180 ml 60 ml 6 210 ml 70 ml 7 240 ml 80 ml 8 270 ml 90 ml 9 300 ml 100 ml

10 330 ml 110 ml 11 360 ml 120 ml

12 to adult 390 ml 130 ml

References

1. Brenn BR, Brislin RP, Rose JB (1998) Epidural analgesia in children with cerebral palsy. Can J Anaesth 45: 1156-1161.

2. Chalkiadis GA, Sommerfield D, Low J, Orsini F, Dowden SJ, et al. (2016) Comparison of lumbar epidural bupivacaine with fentanyl or clonidine for postoperative analgesia in children with cerebral palsy after single-event multilevel surgery. Dev Med Child Neurol 58: 402-408.

3. Elsamra SE, Ellsworth P (2012) Effects of Analgesic and Anesthetic Medications on Lower Urinary Tract Function. Urol Nurs 32: 60-7.

4. Karaman MI, Kaya C, Caskurlu T, Guney S, Ergenekon E (2005) Urodynamic findings in children with cerebral palsy. Int J Urol 12: 717-720.

5. Nolan J, Chalkiadis GA, Low J, Olesch CA, Brown TC (2000) Anaesthesia and pain management in cerebral palsy. Anaesthesia 55: 32-41.

Phase 2 – Early Postoperative Phase

Pain Management and Psychosocial Support

I. Preoperative A. Psychosocial Assessment / Patient and Family Education

1. Therapeutic Child Life and Play Intervention 2. Cultural Issues and Communication with team 3. Community Resources 4. Handouts and Contact Information

II. Early Postoperative A. Psychosocial support

1. Optimize distraction 2. Behavioral regression 3. Family encouragement and communication portal 4. Reminder of goals

B. Medical comorbidity management C. Resumption of routines, i.e., bowel, bladder, nutrition, sleep D. Pain management

1. Premedication for clinic visits 2. Guidance on when to wean pain medication 3. Spasticity management 4. Neuropathic pain 5. Wound management

E. Preparation for Early Rehabilitation 1. Range of motion, Intensive Outpatient Therapy (IOTP) schedule 2. Referrals and communication

a. Community rehabilitation facility and primary care 3. Medication optimization 4. After cast care, shoes – Appendix 3 and 4 5. Community and school integration

F. Preparation for Community Based Rehabilitation 1. Clinic visits - Observational gait, strength, ROM, orthotic modification 2. Physical education and school accommodation 3. Recreational activities

References

1. Cuomo, Anna V., Seth C. Gamradt, Chang O. Kim, Marinis Pirpiris, Philip E. Gates, James J. Mccarthy, and Norman Y. Otsuka. Health-Related Quality of Life Outcomes Improve After Multilevel Surgery in Ambulatory Children With Cerebral Palsy. Journal of Pediatric Orthopaedics 27.6 (2007): 653-57.

2. Ellerton, Mary-Lou, and Craig Merriam. Preparing Children and Families Psychologically for Day Surgery: An Evaluation. Journal of Advanced Nursing 19.6 (1994): 1057-062.

3. Gates, Philip E., Norman Y. Otsuka, James O. Sanders, and Jeanie Mcgee-Brown. Relationship between Parental PODCI Questionnaire and School Function Assessment in Measuring Performance in Children with CP. Developmental Medicine & Child Neurology 50.9 (2008): 690-95.

4. Li, Ho Cheung William, and Violeta Lopez. Effectiveness and Appropriateness of Therapeutic Play Intervention in Preparing Children for Surgery: A Randomized Controlled Trial Study. Journal for Specialists in Pediatric Nursing 13.2 (2008): 63-73.

5. Li, Ho Cheung William. Evaluating the Effectiveness of Preoperative Interventions: The Appropriateness of Using the Children's Emotional Manifestation Scale. Journal of Clinical Nursing 16.10 (2007): 1919-926.

6. Pirpiris, Marinis, Philip E. Gates, James J. Mccarthy, Jacques D'Astous, Chester Tylkowksi, James O. Sanders, Fred J. Dorey, Sheryl Ostendorff, Gilda Robles, Christine Caron, and Norman Y. Otsuka. Function and Well-Being in Ambulatory Children With Cerebral Palsy. Journal of Pediatric Orthopaedics 26.1 (2006): 119-24.

7. Raina, P., M. O'Donnell, P. Rosenbaum, J. Brehaut, S. Walter, D. Russell, M. Swinton, B. Zhu, and E. Wood. The Health and Well-Being of Caregivers of Children With Cerebral Palsy. Pediatrics 115.6 (2005): E626-636.

8. Risto, Olof, Anita Ãkerstedt, Pia Ãdman, and Birgitta Ãberg. Evaluation of Single Event Multilevel Surgery and Rehabilitation in Children and Youth with Cerebral Palsy: A 2-year Follow-up Study. Disability and Rehabilitation 32.7 (2009): 530-39.

9. Thomason, Pamela, Richard Baker, Karen Dodd, Nicholas Taylor, Paulo Selber, Rory Wolfe, and H. Kerr Graham. Single-Event Multilevel Surgery in Children with Spastic Diplegia. The Journal of Bone and Joint Surgery-American Volume 93.5 (2011): 451-60.

Phase 2: Early Post-Operative Phase

Soft Tissue Surgery to Improve Gait in Children with Cerebral Palsy: Technique of “Slow Surgical Lengthening”

I. Muscle Function During Gait A. Muscle Function in Gait 1. Force Generation B. Children with CP 1. Compromised a. Spasticity b. Weakness c. Contracture d. Selective Control C. Rationale for Intervention 1. Surgical Lengthening of Soft Tissue (Muscle Tendon Unit) a. Improve Range of Motion b. Optimize (Don’t Compromise!) Force Generating Capacity II. Muscle Function in Children with Cerebral Palsy A. Weaker Than Age Matched Peers B. Strength to Weight Ratio 1. Less Favorable with Increasing Age C. Pathoanatomy 1. Myostatic Deformity a. MTU: Short b. Tendon: Long c. Muscle Belly: Short d. Myofibrils: Long(!) III. Soft Tissue Surgery to Improve Gait: What is the Dose? A. Dynamic Deformity / Dysfunction 1. Botulinum Toxin 2. Selective Dorsal Rhizotomy 3. Intrathecal Baclofen Therapy 4. Surgical Tendon Transfer B. Myostatic Deformity 1. Release a. Myotomy, b. Tenotomy 2. Lengthen a. Myotendinous Junction (Recession) b. Tendon (Z Lengthening)

C. Classical Techniques 1. Acute, Complete Correction a. Complications (i.) Weakness (Damage to Myofibrils) (i.) Neuropraxia (Nerve Stretch) 2. Recent Recommendations a. Optimize Tone Management b. Prioritize Skeletal Surgery c. Avoid Soft Tissue Surgery IV. Slow Surgical Lengthening of the Medial Hamstring Muscles A. Alternative Technique 1. Current Understanding a. Pathoanatomy / Pathophysiology B. Surgical Technique: Recession a. Myotendinous Junction

b. Minimal Acute Lengthening (i.) Δ Popliteal Angle 30 Degrees c. No Disruption of Muscle Fibers

C. Post-Operative Management: Subsequent Gentle Stretching 1. Knee Immobilizer / Positional

(i.) Slow, Gentle Hamstring Stretch in Wheelchair (ii.) Knee Extended / Hip Flexed (iii.) Minimal Damage to Myofibrils

2. Serial Stretch Casting – Appendix 5

References1-19

1. Altuntas AO, Dagge B, Chin TY, Palamara JE, Eizenberg N, Wolfe R, et al. The effects of

intramuscular tenotomy on the lengthening characteristics of tibialis posterior: high versus low

intramuscular tenotomy. J Child Orthop. 2011;5(3):225-30. Epub 2011/07/23.

2. Barrett RS, Lichtwark GA. Gross muscle morphology and structure in spastic cerebral

palsy: a systematic review. Dev Med Child Neurol. 2010;52(9):794-804. Epub 2010/05/19.

3. Brunner R, Jaspers RT, Pel JJ, Huijing PA. Acute and long-term effects on muscle force

after intramuscular aponeurotic lengthening. Clin Orthop Relat Res. 2000(378):264-73. Epub

2000/09/15.

4. Dagge B, Firth GB, Palamara JE, Eizenberg N, Donath S, Graham HK. Biomechanics of

medial hamstring lengthening. ANZ J Surg. 2012;82(5):355-61. Epub 2013/01/12.

5. Dallmeijer AJ, Baker R, Dodd KJ, Taylor NF. Association between isometric muscle

strength and gait joint kinetics in adolescents and young adults with cerebral palsy. Gait

Posture. 2011;33(3):326-32. Epub 2010/12/28.

6. Damiano DL, Abel MF, Pannunzio M, Romano JP. Interrelationships of strength and gait

before and after hamstrings lengthening. J Pediatr Orthop. 1999;19(3):352-8. Epub 1999/05/27.

7. Davids JR, Rogozinski BM, Hardin JW, Davis RB. Ankle dorsiflexor function after plantar

flexor surgery in children with cerebral palsy. J Bone Joint Surg Am. 2011;93(23):e1381-7. Epub

2011/12/14.

8. Dreher T, Vegvari D, Wolf SI, Geisbusch A, Gantz S, Wenz W, et al. Development of knee

function after hamstring lengthening as a part of multilevel surgery in children with spastic

diplegia: a long-term outcome study. J Bone Joint Surg Am. 2012;94(2):121-30. Epub

2012/01/20.

9. Eek MN, Beckung E. Walking ability is related to muscle strength in children with

cerebral palsy. Gait Posture. 2008;28(3):366-71. Epub 2008/07/04.

10. Eek MN, Tranberg R, Beckung E. Muscle strength and kinetic gait pattern in children

with bilateral spastic CP. Gait Posture. 2011;33(3):333-7. Epub 2010/12/21.

11. Graham HK, Rosenbaum P, Paneth N, Dan B, Lin JP, Damiano DL, et al. Cerebral palsy.

Nat Rev Dis Primers. 2016;2:15082. Epub 2016/05/18.

12. Jaspers RT, Brunner R, Baan GC, Huijing PA. Acute effects of intramuscular

aponeurotomy and tenotomy on multitendoned rat EDL: indications for local adaptation of

intramuscular connective tissue. Anat Rec. 2002;266(2):123-35. Epub 2002/01/15.

13. Jaspers RT, Brunner R, Riede UN, Huijing PA. Healing of the aponeurosis during recovery

from aponeurotomy: morphological and histological adaptation and related changes in

mechanical properties. J Orthop Res. 2005;23(2):266-73. Epub 2005/03/01.

14. Karol LA, Chambers C, Popejoy D, Birch JG. Nerve palsy after hamstring lengthening in

patients with cerebral palsy. J Pediatr Orthop. 2008;28(7):773-6. Epub 2008/09/25.

15. Seniorou M, Thompson N, Harrington M, Theologis T. Recovery of muscle strength

following multi-level orthopaedic surgery in diplegic cerebral palsy. Gait Posture.

2007;26(4):475-81. Epub 2007/09/15.

16. Silver RL, de la Garza J, Rang M. The myth of muscle balance. A study of relative

strengths and excursions of normal muscles about the foot and ankle. J Bone Joint Surg Br.

1985;67(3):432-7. Epub 1985/05/01.

17. Thompson N, Stebbins J, Seniorou M, Newham D. Muscle strength and walking ability in

diplegic cerebral palsy: implications for assessment and management. Gait Posture.

2011;33(3):321-5. Epub 2010/12/21.

18. Yucesoy CA, Koopman BH, Grootenboer HJ, Huijing PA. Finite element modeling of

aponeurotomy: altered intramuscular myofascial force transmission yields complex sarcomere

length distributions determining acute effects. Biomech Model Mechanobiol. 2007;6(4):227-43.

Epub 2006/08/10.

19. Yucesoy CA, Koopman BH, Grootenboer HJ, Huijing PA. Extramuscular myofascial force

transmission alters substantially the acute effects of surgical aponeurotomy: assessment by

finite element modeling. Biomech Model Mechanobiol. 2008;7(3):175-89. Epub 2007/05/09.

Phase 2: Early Post-Operative Phase

Early Orthotic Management Following Single Event Multilevel Surgery (SEMLS) to Improve Gait in Children with Cerebral Palsy

1. Overview

a. Disruption of Foot Function in Cerebral Palsy b. Orthotic Design / Indications / Cost c. Clinical Decision Making

2. Foot Function in Cerebral Palsy 1-7

a. Imbalance b. Spasticity c. Motor Control d. Balance e. 3 Common Malalignment Patterns

i. Equinus ii. Equinoplanovalgus

iii. Equinocavovarus f. Stance Phase Function

i. Shock Absorption (Loading Response) ii. Stability (Mid Stance)

iii. Lever Arm (terminal Stance) g. Swing Phase Function

i. Clearance (Mid Swing) ii. Pre Position for Initial contact (Terminal Swing)

3. Orthotic Indications / Design 2, 3, 7-12

a. Use of Orthoses in Cerebral Palsy i. Protect Surgery (Promote Healing)

1. Variable Time, Intuitive, Limited Scientific Evidence ii. Prevent Deformity With Growth

1. Little Evidence to Support iii. Improve Gait

1. Specific Indications, Based Upon Biomechanics b. Biomechanics

i. Ankle Plantarflexion / Knee Extension Couple ii. Orthosis Provides

1. Direct Control of Foot / Ankle Alignment 2. Indirect Control of Knee Alignment 3. ? Control of Hip Alignment

c. Materials i. Thermoplastics

1. Polypropylene 2. Polyethylene 3. Copolymer

ii. Carbon Fiber / Fiberglass Strips iii. Foam Padding

1. Plastizote iv. Straps / Buckles

1. Elastic / Velcro 2. Plastic / Metal

4. Clinical Decision Making 2, 9-11

a. Diagnostic Matrix i. Physical Examination

ii. Radiographic Examination iii. Gait Assessment iv. Functional Deficits

1. Stance / Swing / Both b. Common Orthotic Designs

i. Posterior Leaf Spring Orthosis (PLSO) / Articulated Ankle Foot Orthosis (AAFO)

1. Primarily Swing Phase Control Orthoses ii. Solid Ankle Foot Orthotic (SAFO) / Floor Reaction Ankle Foot Orthosis

(FRAFO) / Ground Reaction Ankle Foot Orthosis (GRAFO) 1. Stance And Swing Phase Control Orthoses

iii. FRAFO / GRAFO 1. Most Restrictive Orthotic Design 2. Used For Subjects With Crouch Gait

iv. Most Common Orthotic Mistake 1. Use of a PLSO or AAFO for Crouch Gait

C. Post SEMLS Orthotic Design 1. SAFO Convertible to PLSO: SAFO 4-6 Weeks to 4-6 Months Post-op

SAFO with Posterior Keel 2. SAFO Convertible to PLSO: PLSO at 4-6 Months Post-op

C. Indications for Common Orthotic Designs

ORTHOTIC PHYSICAL EXAMINATION GAIT DEVIATION COMMENTS HIP KNEE ANKLE FOOT IC MST TST SW

UCBL NL NL NL Mild, Correctible

NL NL NL NL No Effect On Gait

SMO NL NL NL Mild, Correctible

NL NL NL NL No Effect On Gait

PLSO NL NL DF to 5 degrees

Mild, Correctible

- HS NL NL + PF SW Control

AAFO NL NL DF to 5 degrees

Mild, Correctible

- HS + PF; + KE

(mild)

NL + PF Primarily SW Control

SAFO NL NL DF to neutral

Moderate, Partially

Correctible

- HS + PF; + KE or +KF

(mild)

+ PF + PF ST and SW Control

FRAFO EXT > -15 Degrees

EXT > -15 Degrees

DF to neutral; TFA

< 30 Degrees

EXT

Moderate, Partially

Correctible

- HS + DF; + KF; + HF

+ DF + DF ST Control for Crouch Gait Pattern

IC = Initial Contact; MST = Mid Stance; TST = Terminal Stance; Sw = Swing; NL = Normal; DF = dorsiflexion; HS = Heel Strike; PF = Plantar Flexion; KE = Knee Extension; KF = Knee Flexion; EXT = Extension; TFA = Thigh Foot Angle; HF = Hip Flexion

REFERENCES

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Phase 3 – Early Intensive Rehabilitation

I. Introduction to the Intensive Outpatient Therapy Program (IOTP) – Appendix 6 - 7 a. Definition and Focus b. Participant characteristics c. Timing, frequency and duration of IOTP and ongoing outpatient Physical Therapy

Services II. Assessment before IOTP

a. Skin Integrity and Swelling b. Range of Motion, Strength, and Selective Motor Control c. Mat Mobility d. Transfers e. Standing Posture and Weight-bearing Ability f. Balance g. Gait h. Orthotic fit, function, and comfort i. Pain j. Engagement in Therapy k. Patient/caregiver Concerns

III. Setting Goals for IOTP a. Focus on functional mobility and increasing tolerance to activity and mobility upon

return to home and school settings. b. Goals must be clearly defined and agreed upon by client/family caregiver and

therapist to maximize motivation and participation. c. Functional Goals for IOTP

i. Transfers: bed, bathroom/toilet, car, home ii. Ambulation : household or short community distances with assistive device

as needed iii. Home Exercise Program for Active ROM, functional strengthening, standing iv. Optimization of orthotic/footwear fit and function

d. IOTP Interventions i. ROM and strengthening

ii. Mat mobility iii. Standing and transfer training iv. Gait training (parallel bars, overground with AD, treadmill, partial weight

support) v. Consultation with orthotist as needed to ensure optimal fit and function of

AFOs vi. Adaptive or stationary cycle

vii. Skin care/scar management – Appendix 8 viii. Patient/caregiver education

e. Surgery-specific PT guidelines for SEMLS – Appendix 9 - 10 f. Coordination required for transition to Outpatient Therapy – Appendix 11 - 15

i. Patient/caregivers with thorough understanding of precautions, brace wear schedules, and next phase of post-SEMLS rehabilitation

1. Written summary of patient/caregiver education g. All needed equipment for early rehabilitation coordinated

i. Outpatient PT services scheduled and lines of communication established 1. Provide written documentation of patient status at discharge to

patient/caregiver as well as outpatient /school-based therapist 2. Written discharge summary in medical record and initiate form for

future/follow-up clinic tracking

Phase 4 – Outpatient Based Rehabilitation

I. Goals a. Review of pre-operative goals and long-term goals b. Emphasis on activity and participation level c. Participation in community-based recreation and lifelong wellness opportunities

II. Interventions III. Frequency and duration

a. Typically 2-3 times per week initially, decreasing as determined appropriate and progress towards goals assessed.

IV. Clinic Follow-up – Communication between hospital and community based care therapy providers essential!

a. 4 weeks post IOTP completion – critical follow-up visit for therapist assessment b. Additional follow up at 6, 9 and 12 months post-op or according to MD direction c. One year post-op, gait study in Motion Analysis Center

i. Measuring Success with Gait Analysis after SEMLS – Appendix 16 V. Common Challenges in the First Year After SEMLS (Panel)

a. Casting Decubitus Ulcers b. Acquired (and temporary!) gait deviations after SEMLS c. Foot sensitivity and pain d. Functional and Anatomic Leg Length Difference e. Recurrent Knee Flexion Contractures f. Difficulty with Bracing