2/14/2018 1 Posterior Fossa Syndrome: Rehabilitation Across the Continuum Lindsey Christoffersen, PT, DPT, C/NDT Sherry Lockett, PT, DPT • The speakers have no conflicts of interest to disclose • Any equipment or materials mentioned in the presentation are available to the speakers at their facility • The speakers have no financial gain in mentioning any specific products Conflict of Interest Disclosure At the conclusion of this session, participants will be able to: 1. Discuss the pathophysiology and clinical characteristics of Posterior Fossa Syndrome (PFS). 2. Discuss the role of each member of the interdisciplinary team in the comprehensive management of patients with PFS. 3. Identify and discuss appropriate evaluation tools and treatment strategies to address the impairments and functional limitations associated with PFS across a variety of settings. Learning Objectives Near the base of the skull Contains the brainstem and cerebellum - Brainstem: breathing, heart rate, swallowing, alertness, digestion - Cerebellum: movement, posture, balance, coordination The Posterior Fossa 1,2 Image courtesy of University of Rochester Medical Center Account for 60-70% of all pediatric brain tumors ~40% to 50% of these are medulloblastoma Posterior Fossa Tumors 3 Reported incidence following PF tumor surgery ranges from 2%-50% Older studies often report lower numbers - Failure to recognize the syndrome? Gadgil et al. (2016) knowledge update - Occurs in 8-24% of children following resection of PF masses Robertson et al. (2006) - Reported PFS in 24% of their study population (N=450) Incidence of PFS 4-6
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2/14/2018
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Posterior Fossa Syndrome: Rehabilitation Across the Continuum
- Emphasis on engaging abdominals/upright posture during functional tasks
Strengthening
Will sometimes perform in therapy sessions, but often have family/patient perform as part of HEP
- Ankle dorsiflexion
- Hamstrings
- Trunk rotation, extension
Stretching
Control of dynamic movement
- Weight shifting in sitting � standing
- Activation of glutes and abdominals to perform true weight shift
Movement in multiple planes
Coordination
- Timing, sequencing, amount of force, spatial awareness
- Zip Ball, catching and throwing a ball, weighted therapy bar ball taps, squat <->
stand, jumping (in place, to a target)
Obstacle negotiation
- Small obstacles (hurdles, River Stones, ramps), unstable surfaces (AirEx foam,
DynaDisc), unpredictable surfaces (grass, gravel)
Balance Training & Coordination
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Narrowed base of support
- Walking between balance beams
- Romberg position (eyes open, eyes closed)
- Tandem stance on line � balance beam � elevated balance beam
- Single limb stance
• Trapping soccer ball
• Flamingo freeze
Balance Training & Coordination
Pre-gait activities
- Foot to target
• Make increasingly difficult: heel strike when bringing foot to target, weight shift onto limb once foot is on target, weight shift with step through to next target
- Step up and over therapy bench for stair prep
LiteGait on treadmill or over ground
Ambulation with appropriate assistive device or in || bars
- Fine motor activities to improve coordination and strength
- School based skills
- Handwriting
- Independence in higher level self
care (tying shoes, buttons)
- Functional cognition
X Rigorous RT schedule, often with sedation
X Side effects from chemotherapy
- Nausea/emesis
- Anemia
- Thrombocytopenia
X Infections
X Slow progress due to nature of PFS
X Cancer-related fatigue
X Communication challenges
X Psychosocial considerations
Barriers to Achieving Goals
Myelosuppression is a major side effect of chemotherapy
- Potential for adverse events with stressful conditions (exercise)
• Thrombocytopenia � bleeds
• Severe anemia � cardiac arrhythmias
• Neutropenia � sepsis
Guidelines for Safe Exercise25
Retrospective chart review (Gilchrist and Tanner, 2017)
- Adverse events during or after 37 of 406 PT sessions
• Most common event was tachycardia not requiring medical intervention
- No serious adverse events occurred
Guidelines for Safe Exercise25
Cancer-Related Fatigue26
“A distressing, persistent subjective sense of physical, emotional, and/or
cognitive tiredness or exhaustion related to cancer or cancer treatment that is not proportional to recent activity and interferes with usual functioning.”
- National Comprehensive Cancer Network, 2017
Cancer-Related Fatigue27,28
Cancer-Related Fatigue
Cancer Treatment
Burden
Disease Burden
Co-Morbid Conditions
Psychosocial Burden
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Cancer-Related Fatigue29
• Feeling tired, weary, exhausted even after a good night’s sleep
• Lack of energy/prolonged fatigue after activity
• Weakness, heaviness in arms/legs
• Listlessness or irritability
• Trouble starting or finishing tasks due to fatigue
• Needing to sleep during the day
• Unable or requiring help to do usual or desired activities
• Being too tired to eat
• Difficulty with concentration and memory
• Limiting social activities due to
fatigue
Consistent therapist when possible
- May have to introduce self and role multiple times
One voice at a time
1-2 step commands
- Consider automatic movements
- This then that statements
Allow increased time for processing
Collaborate with speech and psychology regarding
communication and cognitive abilities
Strategies for Communication
7-year-old male
- GTR medulloblastoma, 4th ventricle
- Post-op: inability to speak, left-sided weakness, irritability
- Transferred to inpatient rehabilitation hospital
• PT, OT, ST; slow progress
• Mute for a few days, then slow speech improvements but remained dysarthricwith “robotic speech”
• Gait unsteady, but able to walk without assistance; unable to negotiate stairs
• Challenging mood/behavior requiring risperidone during RT
Two years after completion of treatment
- Speech fluent, continuing to work with PT to address ongoing balance issues
- Behaviorally and emotionally at baseline
Published Case Studies20
13-year-old male
- GTR medulloblastoma
- Post-op: paucity of speech, left-sided weakness, ataxia, VI CN palsy, emotional outbursts
- Transferred to inpatient rehabilitation hospital x 6 weeks
• Made significant progress with speech and ambulation
• Allowed for safe participation in PT and ST and completion of RT
At 5-year follow-up
- Attending school full-time
- Behavior and speech issues fully resolved
- Ongoing challenges with balance � continuing to work with PT and OT regarding these issues
Published Case Studies20
Harbourne et al. 2014
- 14-year-old female and 6-year-old female with PFS
- Completed inpatient rehabilitation and outpatient programs, discharged due to lack of progress
Neuromodulation devices on the tongue
- Static and dynamic balance activities utilizing devices
- 3 days of intensive training, followed by HEP consisting of activities performed 5 days/week for 8 weeks
Clinical improvement noted in both participants
- Improved balance beyond MCID on BBT-P
- Increase in time standing on dynamic surface
- Increased gait speed and step length
Published Case Studies30
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- PFS occurs in up to 24% of patients who undergo posterior fossa tumor resection
- Symptoms are often severe and last years beyond cancer treatment
- The literature strongly advises participation in rehab, but specific strategies for
management of this syndrome are lacking
- More research is needed to determine the efficacy of specific rehab interventions for this population
Summary
Questions?
1. Rehab for posterior fossa syndrome. St. Jude Children's Research Hospital. Available at: https://www.stjude.org/treatment/patient-resources/caregiver-resources/patient-family-education-sheets/rehabilitation/rehab-for-posterior-fossa-syndrome.html. Accessed October 25, 2017.
2. Adler L, Dozier T, eds. Anatomy of a Child's Brain. Anatomy of a Child's Brain - Health Encyclopedia - University of Rochester Medical Center. https://www.urmc.rochester.edu/encyclopedia/content.aspx?ContentTypeID=90&ContentID=P02588. Accessed October 25, 2017.
3. Kirk E, Howard V, Scott C. Description of posterior fossa syndrome in children after posterior fossa brain tumor surgery. Journal of Pediatric Oncology Nursing. 1995;12(4):181-187.
4. Reed-Berendt R, Phillips B, Picton S, et al. Cause and outcome of cerebellar mutism: evidence from a systematic review. Child’s Nervous System. 2014;30(3):375-385.
5. Robertson P, Muraszko K, Holmes E, et al. Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children’s Oncology Group. Journal of Neurosurgery: Pediatrics. 2006;105:444-451.
6. Kupeli S, Yalcin B, Bilginer B, et al. Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatric Blood and Cancer. 2011;56(2):206-210.
7. Medulloblastoma. St. Jude Children’s Research Hospital. Available at: https://www.stjude.org/disease/medulloblastoma.html. Accessed November 21, 2017.
References
8. Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)–Health Professional Version. 2017. Available at: https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq#link/_307_toc. Accessed November 28, 2017.
9. Gudrunardottir T, De Smet H, Bartha-Doering L et al. Posterior Fossa Syndrome (PFS) and Cerebellar Mutism. In: Marien P, Manto M, ed. 1st ed. London: Elsevier;2015:257-281. Available at: https://books.google.com/books?hl=en&lr=&id=aQGdBAAAQBAJ&oi=fnd&pg=PA257&dq=posterior+fossa+syndrome+children&ots=yfPkzyVgI8&sig=z5I8lDm4Km4BoWa63htcnc2GVdk#v=onepage&q&f=false. Accessed November 14, 2017.
10. Law N, Greenberg M, Bouffet E, et al. Clinical and neuroanatomical predictors of cerebellar mutism syndrome. Neuro-oncology. 2012;14(10):1294-1303.
11. Steinbok P, Cochrane D, Perrin R, et al. Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatric Neurosurgery. 2003;39(4):179-183.
12. Gadgil N, Hansen D, Barry J, et al. Posterior fossa syndrome in children following tumor resection: knowledge update. Surgical Neurology International. 2016;7(Suppl 6):S179-S183.
13. Catsman-Berrevoets C. Cerebellar mutism syndrome: cause and rehabilitation. Current Opinion in Neurology. 2017;30(00).
14. Avula S, Mallucci C, Kumar R, Pizer B. Posterior fossa syndrome following brain tumor resection: review of pathophysiology and a new hypothesis on its pathogenesis. Child’s Nervous System. 2015;31:1859-1867.
15. Morris E, Phillips N, Laningham F, et al. Proximal dentatothalamocortical tract involvement in posterior fossa syndrome. Brain. 2009;132:3087-3095.
References
16. Korah MP, Esiashvili N, Mazewski CM, et al. Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. International Journal of Radiation Oncology* Biology* Physics. 2010;77(1):106-112.
17. Palmer S, Hassall T, Evankovich K, et al. Neurocognitive outcome 12 months following cerebellar mutism syndrome in pediatric patients with medulloblastoma. Neuro-oncology. 2010;12(12):1311-1317.
18. Steinbok P, Cochrane D, Perrin R et al. Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatric Neurosurgery. 2003;39:179-183.
19. Brinkman T, Li Z, Neglia J, et al. Restricted access to the environment and quality of life in adult survivors of childhood brain tumors. Journal of Neurooncology. 2013;111(2):195-203.
20. Lanier J, Abrams A. Posterior fossa syndrome: review of the behavioral and emotional aspects in pediatric cancer patients.
Cancer. 2017;123:551-559.
21. Franjoine M, Darr N, Held S, et al. The performance of children developing typically on the pediatric balance scale. Pediatric Physical Therapy. 2010;22(4):350-359.
22. Pediatric Balance Scale. Shirley Ryan Ability Lab. Available at: https://www.sralab.org/rehabilitation-measures/pediatric-balance-scale. Accessed January 31, 2018.
23. Pearlman L, McVittie A, Hunter K. Discharge management of an adolescent female with posterior fossa syndrome: A case report. Canadian Journal of Neuroscience Nursing. 2008;30(3).
25. Gilchrist L, Tanner L. Safety of symptom-based modification of physical therapy interventions in pediatric oncology patients with and without low blood counts. Rehabilitation Oncology. 2017;35:3-8.
26. “NCCN Clinical Practice Guidelines in Oncology: Cancer-Related Fatigue.” National Comprehensive Cancer Network. Version 2.2017 ed. Published April 10, 2017. Accessed July 11, 2017.
27. Cleveland Clinic. “Fatigue & Cancer Fatigue.” Available at: https://my.clevelandclinic.org/health/articles/cancer-related-fatigue. Accessed July 11, 2017.
28. Hill A, Hughes J. Empowering Cancer Survivors to Become the CEO (Chief Energy Officer) of Everyday Life. 2017.
29. Calvet M, Curran J, Yamada K. Cancer-Related Fatigue. Oncology Section, APTA; 2015:1-3. Available at:
http://oncologypt.org/pdfs/fact-sheets/Cancer-related-Fatigue-rev-4-10-16.pdf. Accessed July 13, 2017
30. Harbourne R, Becker K, Arpin D et al. Improving the motor skill of children with posterior fossa syndrome: a case series. Pediatric Physical Therapy. 2014;26:462-468.