Author

Type of study

Level of evidence

Patients (feet)

N (female: male)

Age (years)

(min-max)

Follow-up

(min-max)

Procedures

Outcome measure

Results

Karlholm 1968 [37]

Cohort study

C

4 (8) CMT

2:2

5-19

4.5 – 8.5 years

Lengthening of the Achilles tendon; plantar fasciotomy; posterior tibial tendon transfer

No particular outcome measure; description of results (poor / excellent)

The primary results were excellent in all 4 cases. In one case there has been recurrence and detoriation.

Miller 1982 [38]

Cohort study

C

43 (74) only 3 (5) CMT

13:30

0.5-28

2-14 years

Tibial tendon transfer

No particular outcome measure; description of results (poor / excellent)

In CMT, PTT transfer should only be done as part of staged treatment that includes possible future fusion.

Roper 1989[25]

Cohort study

C

10 (18) CMT

7:3

5-36

6-32 years

Elongation of calcaneal tendon; flexor-to-extensor transfer; Robert Jones procedure; calcaneal osteotomy

Classificatie van Levitt 1973: excellent / good / fair / poor

All the patients had satisfactory results (as regards function, appearance and symptoms). However, one patient needed triple arthrodesis and another patient had Steindler release and split tibialis anterior transfer.

The sequence of soft tissue procedures should vary according to the deforming forces in each individual foot. Lengtening of the calcaneal tendon, Steindler release and split tibialis anterior transfers should be performed early and may be repeated as required. Clawing of the toes may be corrected by flexor to extensor tendon transfers and the Robert Jones procedure.

Wetmore 1989 [39]

Cohort study

C

16 (30) CMT

6:10

6-30

6-51 years

Triple arthrodesis

Pain, long-term effects of the triple arthrodesis on the ability to walk, life-style, and vocation.

Criteria of Patterson: excellent / good / fair / poor

Unsatisfactory results:

• 2 patients excellent results
• 5 patients goed results
• 9 patients fair results
• 14 patients poor results

Triple arthrodesis should be reserved as salvage procedure

Santavirta 1993 [29]

Cohort study

C

15 (26) CMT

9:6

5-61

1-33 years

In 21 patients: triple arthrodesis. In six feet: triple arthrodesis complemented by soft tissue procedure

Classificatie: excellent / poor (volgens Levitt)

• 3 patients had a nonunion
• 4 feet had excellent results
• 15 feet had good results
• 4 feet had fair results
• 3 feet had poor results

The number of poor results was not greater in patients followed up for more than 15 years than in those whose fol- low-up was shorter. At the follow-up none of the patients wore braces.

The patients with fair/poor results used irthopaedic shoes, while the one with excellent and good results usually managed with normal or slightly improved shoes.

Except for those with poor results, patients thought they had benefitted from the operation. The walking distance had not significantly improved.

Saltzman 1999 [41]

Cohort study

C

57 (67): 34 (37) poliomyelitis; 5 (7) clubfeet; 5 (6) CMT; 4 (6) tarsal coalition; 3 (4) spinal cord abnormalities; 2 (3) cerebral palsy; 1 (1) Guillain-Barré syndrome

Unclear

age range of CMT group at operation: 15-57 years

Total group: 1^st follow-up 10-49

And 2^nd follow-up 29-68 years

Triple arthrodesis (with a standard anterolateral approach without internal fixation)

Questionnaires at follow-up: pain, use of medication, walking support, max walking distance, additional operative procedures. Physical examination: foot & ankle motion in sagittal plane, assessment of residual deformity while standing, plantar callosities. Radiographic evaluation. Overall assessment following Angus & Cowell: good / fair / poor

CMT group:

• 3 patients with forefoot, midfoot, ankle or knee pain.
• 2 patients needed medication, 1 had shoe insert, 1 needed a cane
• In 4 feet there were callosities present, and in almost all feet were mild to severe degenerative changes in the ankle or foot.

Metaxiotis 2000 [42]

Cohort study

C

21 (26): 12 (15) CMT; 4 (5) myelomeningocele sacral; 2 (3) spastic paraparesis; 1 (1) residual clubfoot; 1 (1) poliomyelitis; 1 (1) tethered cord

12:9

14-52

9-49 months

Cavovarus feet operations: different mixture of osteotomies, STP and triple arthrodesis.

Dynamic pedobarography

Calcaneal pitch angle. Talometatarsal angle. Gait line (Centre of pressure line), contact areas, peak pressures, pressure time integral.

Postoperatively increase in contact area and decrease in peak pressures and pressure time integral. Clinical results such as plantar callosities and roll over avoidance gait did not always correlate with pedobarographic data.

Sammarco 2001 [44]

Cohort study

C

15 (21): 10 (15) CMT; 2 (2) post polio syndrome; 1 (1) porencephalic cyst left frontal lobe; 1 (1) sacral lipomeningocele; 1 (1) peripheral neuropathy

8:7

15-61

8-101 months

Osteotomy of calcaneus; osteotomy of metatarsals.

8 additional procedures (Jones Tendon transfer,

 hammertoe correction)

Maryland Foot Rating Score. AOFAS (8 feet). Motion of foot and ankle. Complications. Walking aid. Radiographic analyses.

89% toon teen goede tot excellente outcome à allemaal een significante verbetering op de scores.

Radiologisch bij alle voeten de componenten van de cavovarus verbeterd (minder cavovarus).

Maryland Foot Rating Score improved from 72.1 to 89.9. AOFAS improved from 46.3 to 89.1. Ankle or foot motion was maintained or improved in 16 feet. Complications in 5 of 65 osteotomies. All patients were brace free postoperatively (3 used AFO preoperatively).

Decrease in forefoot adduction, reduction in both hindfoot and forefoot cavus leading to an overall 13% reduction in the height of the longitudinal arch.

Chan 2007

Cohort study retrospective

D

9 children CMT (14 feet)

unclear

13.3 (9-17)

unclear

Combination of oste

omies and muscle transfers

Preop and postop pedobarographic measurements

Postoperative measurements showed sign improvement of the cavovarus deformity (statistically sign for lateral talus-first metatarsal, the calcaneus-first metatarsal angle, calcaneal pitch.

Comparing preop and postop pedograph values, good correction of the increased Lateral MidFoot segment pressure was seen.

Ward 2008

Cohort Study

C

25 CMT (41) with flexible hindfoot varus that was passively correctable

11-14

15.5 (8.7-25.1)

plantar fasciotomy, transfer of the peroneus longus to the peroneus brevis tendon, and first metatarsal osteotomy in most patients.

SF36, FFI, gait analysis, radiographic evaluation.

Correction of the cavus deformity was well maintained, although most patients had some recurrence of hindfoot varus as seen on radiographic examination. The patients had a lower mean SF-36 physical component score than age- matched norms, and the women had a lower mean SF-36 physical component score than the men, although this difference was not significant. Smokers had lower mean SF-36 scores and significantly higher mean FFI pain, disability, and activity limitation subscores (p < 0.0001). Seven patients (eight feet) underwent a total of eleven subsequent foot or ankle operations, but no patient required a triple arthrodesis. Moderate-to-severe osteoarthritis was observed in eleven feet. With the numbers studied, the age at surgery, age at the time of follow-up, and body mass index were not noted to have a significant correlation with the SF-36 or FFI scores.

Leeuwenstein 2010

Cohort study

C

33 (52): 52 CMT pes cavovarus

19:14

13-59

57 (13-153)

Dorsiflexion osteotomy of MTP I and tendon transfers. Calcaneal osteotomy in persistent cavovarus feet

Early and late complications. FFI patient’s satisfaction with QUOTE

No major complications.

FFI pain 29.3 -> 14.8

FFI disability 37.8 -> 23.5

77% less pain after surgery90%

Napiontek 2015 [50]

Cohort study

C

26 (43): 17 (30) CMT; 3 (5) Friedreich; 6 (2) peripheral motor and sensory neuropathies

12:14

5-56

0.5-15 years

Arthrodesis; joint preserving procedures

AOFAS, WOMAC, subjective assessment of surgical outcome (0-10)

All patients stated that they would decide to undergo the treatment again. For children, joint preserving surgeries gave better results; however, the results could not be statistically confirmed. The results for adults were inconclusive as to which sur-gical techniques should be preferred, arthrodesis or joint preserving. The results show that none of the surgical techniques used for correction of foot deformities in motor- sensory polyneuropathies seems to be preferable.

There are a variety of surgical techniques which can be freely combined: from lengthening/release of contractures, tendon transposition and corrective osteotomies to arthrodesis. The last two procedures are performed both to correct deformity and stabilize joints. The choice of the technique depends on the patient’s age and the degree of deformity as well as, to a considerable extent, on the experience of a surgeon who is to decide on the best treatment. Whenever possible, all elements of deformity should be corrected during one surgery

Dreher 2014 [28]

Cohort study

C

14 (23) CMT

Unclear

17-50

28.8 ± 12.8 months

Tibialis posterior tendon transfer to the dorsum of the foot (total split posterior tibial tendon transfer, T-SPOTT)

Passive ankle range of motion. AOFAS. 3d Gait analysis.

The three-dimensional gait analysis revealed significant increases in tibiotalar and foot-tibia dorsiflexion during the swing phase after surgery. These increases were accompanied by a significant reduction in maximum plantar flexion at the stance-swing transition but without a reduction in active range of motion. Passive ankle dorsiflexion measured in knee flexion and extension increased significantly without any relevant decrease in passive plantar flexion. The AOFAS (American Orthopaedic Foot & Ankle Society) score improved significantly. Tibialis posterior tendon transfer was effective at correcting the foot drop component of cavovarus foot deformity in patients with Charcot-Marie-Tooth disease, with the transfer apparently working as an active substitution. Although passive plantar flexion was not limited after surgery, active plantar flexion at push-off was significantly reduced and it is unknown whether this reduction was the result of a tenodesis effect or calf muscle weakness.

Level

Faldini & Gianini 2015

Cohort study

C

12 CMT (24) with a flexible deformity

2:10

14-28

6 years (2-13)

plantar fasciotomy, midtarsal osteotomy, extensor hallucis longus tendon transfer to the first metatarsal (Jones procedure), and dorsiflexion osteotomy of the first metatarsal. Mean

Maryland Foot Score. Increased plantar arch, plantar flexion of the first ray and secondary heel varus, associated claw toe deformity, and callosities. Alignment of the heel. Activity & strength of each muscle group, range of motion of the foot and ankle, gait abnormalities.

The mean Maryland Foot Score was 72 preoperatively and 86 postoperatively. The post- operative result was rated as excellent in twelve feet (50%), good in ten (42%), and fair in two (8%). Mean calcaneal pitch was 34⁰ preoperatively and 24⁰ at the time of the latest follow-up, the mean Hibb angle was 121⁰ preoperatively and 136⁰ postoperatively, and the mean Meary angle was 25⁰preoperatively and 2⁰ postoperatively. Plantar fasciotomy, midtarsal osteotomy, the Jones procedure, and dorsiflexion osteotomy of the first metatarsal yielded adequate correction of flexible cavus feet in patients with Charcot-Marie-Tooth disease in the absence of fixed hindfoot deformity. The fact that the improvement in the outcome score was only modest may be attributable to the lack of motor balance

Author

Type of study

Level of evidence

Patients (feet)

N (female:male)

Age (years)

(min-max)

Follow-up

(min-max)

Procedures

Outcome measure

Results

Oganesya 1996 [40]

Cohort study

C

65 (83): 22 (22) neurovascular injury; 14 (14) poliomyelitis; 12 (22) CMT; 8 (12) clubfeet; 4 (5) myelodysplasia; 3 (4) cerebral palsy; 1 (2) spondyloepiphyseal; 1 (2) myopathy

26:39

16-56

3-19 years

Volkov-Oganesyan-Povarov hinged distraction apparatus

Good / satisfactory / unsatisfactory (deformity, gait, walking distance, shoes)

Good (59 feet), satisfactory (20), unsatisfactory (4)

Kucukkaya 2002 [45]

Cohort study

C

8 (9): 6 (6) polio; 1 (2) CMT; 1 (1) traumatic injury

2:6

9-26

26-48 months

V-osteotomy; Ilizarov method

All patients were satisfied with their gait.

2x toe contractures, 1x skin necrosis at the distraction site of the foot.

Minor grade 1 and 2 pin tract problems were observed.

Segev 2008 [27]

Cohort study

C

13 (14): 7 (8) idiopathic clubfeet; 2 (2) CMT; 2 (2) myelomeningocele; 1 (1) neurofibromatosis; 1 (1) distal arthrogryposis

Unclear

8-18

1.8-8.9 years

V-osteotomy; Ilizarov method

Author

Review / overview

AMSTAR max 11

PRISMA max 27

Results / recommendation

Roy 2001

Overview Complications of surgery of the foot & ankle in hereditary neurologic disorders

0

0

A careful examination should be done to determine individual muscle strength and to evaluate the deformity and its components for rigidity. Examination of the foot in the weight bearing position and in the non weightbearing position will give valuable information.

Radiographic assessment should include anterioposterior (AP) and lateral views of the feet and ankles obtained with the patient standing

and an axial or Harris view of the calcaneus.

Several treatment suggestions are made and corresponding complications are mentioned.

Beals 2008

Overview CMT and the cavovarus foot

0

0

A recent MRI study of a small sample of patients who had CMT type 1A demonstrated consistent abnormalities of the intrinsic muscles of the foot and a notable variability in the MRI findings of fatty infiltration of leg muscles. The investigators felt their results help explain the development of the cavus deformity but, more importantly, demonstrated abnormalities in what otherwise appeared to be clinically nor- mal muscles and variability among patients.

Clinical evaluation:

Documentation of the strength of all the muscle groups of the lower extremities is important because it allows an assessment of the progression of the disease over time and helps the surgeon best understand the options for surgical reconstruction, if the symptoms warrant intervention.

Physical examination of the patient who has CMT includes observation of standing posture and assessment of the ability to walk on the heels and toes.

An assessment of the range of motion of the ankle is important, with particular attention to determining if the ankle rolls normally or whether it functions more as a hinge.

The shape of the foot must be characterized, noting the presence or absence of clawing of the toes and the presence or absence of a varus hindfoot. The so-called ‘‘peek-a-boo’’ heel sign is readily observed when viewing the patient from the front.

The degree of flexibility of the forefoot and hindfoot must be assessed and a determination made of the presence or absence of an equinus contracture.

Surgical options:

The combination of both bony and soft tissue procedures is typically necessary for optimal outcome.

Therefore, it is important that surgical reconstruction be done in a stepwise manner with reassessment throughout a procedure, rather than applying a given ‘‘recipe’’ of procedures to each foot with a cavovarus deformity.

Sackley 2009

Systematic Review Rehabilitation interventions for foot drop in neuromuscular disease

5

18

Training for foot drop; no surgical intervention

Krause 2012

Overview

Hindfoot Varus & neurologic disorders

0

0

Physical examination:

A complete gait analysis, observation of standing posture, and of the ability to walk on the heels and toes, both in and out of their shoe gear, are required. The standing hindfoot alignment as viewed from the back reveals the extent of the hindfoot varus, and in cavovarus feet the so-called “peek-a-boo” sign indicating a forefoot adductus as well as an elevated medial longitudinal arch.

Recommendations are given for several tests.

Treatment

Because of the variety of the etiologies and the differences in presentation, treatment decisions must be individualized.

the goal of operative treatment is to prevent or halt anteromedial ankle arthritis. The patient should be informed early about these goals, expected outcome, and recovery time after surgery. In the majority of patients with neurogenic cavovarus feet, an appropriate correction of the foot is achieved with soft-tissue release, tendon transfers, and osteotomies.

In this article, indications / recommendations are offered for: soft tissue release and tendon lengthening, tendon transfer, osteotomy, calcaneal osteotomies, lateral column shortening, dorsiflexion osteotomy, midfoot osteotomy, hindfoot arthrodesis, clawtoe reconstruction

Wicart 2012

Overview Cavus foot, from neonates to adolescents

0

0

Direct Pes Cavus

The various forms of direct pes cavus can be distinguished by radiological analysis.

Treatment

• posterior pes cavus: surgery before growth completion can stop the progression of the deformity. The first step consists in transferring one or more dorsal flexors (tib-ialis anterior, extensor digitorum longus, peroneus tertius, peroneus brevis) to the calcaneal tuberosity to compen-sate for the triceps surae weakness. Then, an osteotomy is performed to displace the calcaneal tuberosity upwards and posteriorly, thereby increasing the moments of the transferred muscles
• anterior pes cavus: plantar fascia release with or without dorsal tarsectomy is indicated;
• mixed pes cavus: a combination of the above-described techniques

Pes Cavovarus

Plantar fascia release, Tendon lengthening, tendon transfers, Osteotomies, triple atrhrodesis (this procedure is not desirable). Specific age related treatment is mentioned: (children, adolescents and end of the growth period (page 826)

Yagerman 2012

Literature review pediatric orthopedic conditions in CMT

0

0

 Examination

Surgical decision making should start with a careful examination that includes assessment of foot alignment including the Coleman block test. Inspection of plantar callus patterns is also useful to help characterize the deformities and the areas of abnormal weight bearing, such as specific metatarsal heads and the base of the fifth metatarsal. In addition, a careful motor examination is essential to help decide upon muscle transfers. Lastly, radio-graphs play a critical role in decision making. These should be weight-bearing views and include, at a minimum, anteroposterior, lateral, oblique and Harris axial or Saltzman alignment views.

Treatment

 Correction of the cavus can be achieved by plantar fascia release alone in young children. In older children, full correction requires some combi-nation of osteotomies and soft tissue procedures. Osteotomy options for the forefoot/mid-foot include first metatarsal to address the plantar-flexed first ray, medial cuneiform, first tarsometatarsalarthrodesis with realignment, multiple metatarsal osteotomies or combinations of all of these procedures.

Muscle balancing is accomplished through various tendon transfers.

Triple arthrodesis should only be considered as a salvage procedure for a fixed deformity due to the high incidence of associated postoperative degenerative joint disease

Barton 2013

Overview Joint sparing correction of cavovarus feet in CMT

0

0

Each patient must be examined to assess precisely the involvement of each individual muscle, which is of paramount importance if the deformity is to be understood and correctly treated.

The consensus among surgeons is that there is little role for nonsurgical management of the progressive foot deformity in patients with CMT disease. If left untreated, the deformity worsens due to both disease progression and persisting muscle imbalances.

In the young patient, the aim must be to preserve joint motion, balance the foot, and prevent the progression to a fixed and symptomatic deformity

The surgical approach to addressing the cavovarus foot must be on an individualized basis, and the most critical component of evaluating the foot deformity in a patient with CMT for surgery is the clinical examination. Further imaging is certainly a useful adjunct, but the exact nature of the surgery required is based on the physical examination.

Gould stated that the aim of surgery in the cavovarus foot in patients with CMT disease is to achieve a foot that is

Plantigrade, Mobile, Pain free

In this article, indications / recommendations are offered for: calcaneal osteotomies, forefoot/midfoot osteotomies, plantar fascia release, tendoachilles lengthening, tendon transfers, toe deformities,

Rosenbaum 2014

Overview Cavus foot

0

0

Physical Examination

The physical examination should involve assessment with the patient sitting, standing, and walking. While seated, the shape and symmetry of the foot and toes should be evaluated; clawing may be present (extension at metatarsophalangeal and flexion at interphalangeal joints). The skin and soft tissues should be evaluated for lesions and callosities, because longstanding deformity can adversely affect tissue quality. With hindfoot varus, pressure is focused on the lateral border of the foot. This condition can cause pain and callusing, and occasionally fifth metatarsal stress fractures. A full motor examination should be performed, documenting the strength of

each muscle group against active resistance in both legs. Strength can be graded with the Medical Research Council scale or with hand-held dynamometry, which has been found to be an objective instrument for determining muscle strength in the setting of CMT disease.13 A full sensory and vascular examination must also be performed, and the flexibility of the hindfoot, ankle, and toes is important to assess.