Pediatric Orthopaedic Surgery and the HMSNs

Transcription

1 Reviewed and accepted by the Neuromuscular Committee of the American Association of Neuromuscular & Electrodiagnostic Medicine Certified for CME credit 10/ /2020 Reviewed 10/2017 by the Neuromuscular Committee Pediatric Orthopaedic Surgery and the HMSNs Robert M. Bernstein, M.D.

2 Orthopaedic Problems Related to the type of HMSN Weakness/gait abnormalities Vertually Foot deformities/pain all types Hand deformities/problems with ADLs Sensory abnormalities Scoliosis Hip dysplasia

3 CMT Today Early Diagnosis Basic Science Research Function? Clinical Disease Cure!!

4 The Foot

5 Common Foot Deformities Equinus deformities and foot drop Cavovarus (high arch, heel tilted in) Toe Deformities

6 Can we intervene early to improve function and delay or prevent the need for fusion surgery?

7 Problems? Weakness & Imbalance Flexible deformity Time Rigid deformity Pain & Disability Normal Valgus Pathologic Varus

8 Goal A flexible, stable, plantigrade foot!

9 Current Treatment Options Physical therapy Bracing Tendon Lengthening Transfers and osteotomies Triple arthrodesis (fusions)

10 Physical Therapy Improve strength Improve range of motion

11 Bracing Support Fixes joint position Replaces non functional muscle

12 Surgery Treat deformities not treatable with PT or Bracing *Rigid deformities and Cavus cannot be braced

13 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

14 Foot drop PT Strengthening of Dorsiflexors Routine stretching of Achilles Start early!

15 Foot drop Bracing Leafspring AFO Dorsiflex assist AFO Equinus Night time stretching brace

16 Equinus Slight equinus usually not a problem Severe: Tripping Difficulty with balance *True contracture cannot be braced

17 Equinus Tendon lengthening Percutaneous Brace after lengthening Don t overlengthen! Equinus is better then calcaneus

18 Radiographic Evaluation Standing AP, Lateral Locate the apex of deformity!! Cobey Views No! Yes! Apex

19 Heel tilts in (varus) High arch (cavus) Claw toes Calluses Ankle sprains Becomes rigid Cavovarus

20 Coleman Block Test Is the hindfoot varus rigid? Allow the 1 st metatarsal head to drop. Does the varus correct?

21 Transfers/Osteotomy Realign hindfoot Move functioning muscles EHL, TA, PL PB +1 st metatarsal osteotomy May not be permanent (moving target) No bridges burned

22 Osteotomies Heel 1 st Metatarsal

23 Surgery Rigid Transfers alone will not work! Triple arthrodesis (fuse hindfoot) +transfers

24 Fusion Places the foot in corrected position Reduces motion Adds stress to other joints 76% arthrosis of other joints at 21 years f/u

25 Fusions Surgery Rigid

26 Fusion Pro Immediate results Permanent Con Limited motion Accelerates degeneration of surrounding joints

27 Fusion Better plantigrade and stiff then cavovarus and stiff!

28 The Spine

29 Scoliosis 15 30% of patients Kyphosis common Rarely responds to brace treatment Spinal cord monitoring? JBJS 2007;89:

30 Scoliosis

31 The Hip

32 Hip Dysplasia Prevalence unclear?8 20% of CMT patients Natural history not well documented

33 Evaluation Newborn with known HMSN Ultrasound of hips Child with HMSN Standing AP Pelvis every 2 yrs until skeletal maturity

34 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)

35 Summary Orthopaedic manifestations common in HMSNs Gait Foot Spine Hip

36 Summary Early therapy for foot/ankle Bracing Surgery Transfers and Osteotomies Fusion last resort

37 Summary Scoliosis Common in CMT 1a Kyphosis Bracing ineffective Surgery Hip DDH Regular radiographic evaluation Surgical treatment

38 Thank you!