robert m. bernstein, m.d. - aanem · robert m. bernstein, m.d. reviewed and accepted by the...
TRANSCRIPT
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Pediatric Orthopaedic Surgery and the HMSNs
Robert M. Bernstein, M.D.
Reviewed and accepted by the 2011-2012 Neuromuscular Committee ofthe American Association of Neuromuscular & Electrodiagnostic Medicine Certified for
CME credit 10/2011 – 05/2020Reviewed 10/2017 by the 2017-2018 Neuromuscular Committee
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Orthopaedic Problems
• Related to the type of HMSN• Weakness/gait abnormalities• Foot deformities/pain• Hand deformities/Problems with ADLs• Sensory abnormalities• Scoliosis• Hip dysplasia
Vertuallyall types
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Cure!!
Basic ScienceResearch
CMT Today
Early Diagnosis
Clinical Disease
Function?
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The Foot
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Common Foot Deformities
• Equinus deformities and foot drop• Cavovarus (high arch, heel tilted in)• Toe Deformities
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• Can we intervene early to improve function and delay or prevent the need for fusion
surgery?
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Problems?
Weakness & Imbalance
Rigid deformity
Flexible deformity
Time
Pain & Disability
NormalValgus
PathologicVarus
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Goal
• A flexible, stable, plantigrade foot!
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Current Treatment Options
• Physical therapy• Bracing• Tendon Lengthening• Transfers and osteotomies• Triple arthrodesis (fusions)
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Physical Therapy
• Improve strength• Improve range of motion
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Bracing
• Support• Fixes joint position• Replaces non‐functional muscle
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Surgery
• Treat deformities not treatable with PT or Bracing
• *Rigid deformities and Cavus cannot be braced
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Foot drop
• Steppage gait – Lack of Tib Ant Increased tripping
• Foot slap• May walk on tip‐toes (equinus)• Slight equinus• Not usually a problem• Severe Equinus (contracture)• Decreased balance, stability
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Foot drop
• PT• Strengthening of Dorsiflexors• Routine stretching of Achilles• Start early!
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Foot drop
• Bracing• Leafspring AFO• Dorsiflex assist AFO• Equinus• Night‐time stretching brace
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Equinus
• Slight equinus usually not a problem• Severe:• Tripping• Difficulty with balance
• *True contracture cannot be braced
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Equinus
• Tendon lengthening• Percutaneous• Brace after lengthening• Don’t overlengthen!• Equinus is better then calcaneus
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Radiographic Evaluation
• Standing AP, Lateral• Locate the apex of deformity!!• Cobey Views
No! Yes!
Apex
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Cavovarus
• Heel tilts in (varus)• High arch (cavus)• Claw toes• Calluses• Ankle sprains• Becomes rigid
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Coleman Block Test
• Is the hindfoot varus rigid?
• Allow the 1st metatarsal head to drop.
• Does the varus correct?
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Transfers/Osteotomy
• Realign hindfoot• Move functioning muscles• EHL, TA, PL‐PB• + 1st metatarsal osteotomy• May not be permanent (moving target)• No bridges burned
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Osteotomies
• Heel
• 1st Metatarsal
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Surgery ‐ Rigid
• Transfers alone will not work!• Triple arthrodesis (fuse hindfoot)• + transfers
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Fusion
• Places the foot in corrected position
• Reduces motion• Adds stress to other joints• 76% arthrosis of other joints at 21 years f/u
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Surgery ‐ Rigid
• Fusions
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Fusion• Pro
– Immediate results– “Permanent”
• Con– Limited motion– Accelerates degeneration of surrounding joints
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Fusion
• Better plantigrade and stiff then cavovarus and stiff!
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The Spine
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Scoliosis
• 15‐30% of patients• Kyphosis common• Rarely responds to brace treatment• Spinal cord monitoring?
JBJS 2007;89:1504-1510
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Scoliosis
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The Hip
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Hip Dysplasia
• Prevalence unclear• ?8‐20% of CMT patients• Natural history not well documented
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Evaluation
• Newborn with known HMSN• Ultrasound of hips• Child with HMSN• Standing AP Pelvis every 2 yrs until skeletal maturity
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Treatment
• Bracing likely ineffectual after newborn period• Infant: Pavilk Harness• Child: Surgical• Address acetabular dysplasia• Address femoral malalignment• *Avoid lengthening procedures like Salter Osteotomy
Orthop Clin N Am 37 (2006) 203-209
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Summary
• Orthopaedic manifestations common in HMSNs
• Gait• Foot• Spine• Hip
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Summary
• Early therapy for foot/ankle• Bracing• Surgery• Transfers and Osteotomies• Fusion last resort
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Summary
• Scoliosis• Common in CMT 1a• Kyphosis• Bracing ineffective• Surgery • Hip• DDH• Regular radiographic evaluation• Surgical treatment
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Thank you!