hand differences in fanconi anemia · hand differences in fanconi anemia scott h. kozin, md ......
TRANSCRIPT
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Hand Differences in Fanconi Anemia
Scott H. Kozin, MD
Shriners Hospital for Children
Philadelphia, PA
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Physical Anomalies with FA
• Abnormal pigmentation 75%
• Skeletal (Alter 1993) 60- 80%
• Growth retardation 56%
• Microcephaly 43%
• Renal anomalies 28%
• Strabismus 26%
• Hypogonadism 22%
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Skeletal Anomalies
• Upper Extremity 70%
– 1° radial deficiency
• Lower Extremity 4%
• Spine 2%
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Radial Deficiencies
• Total or partial absence of preaxial border
• Variable phenotype
• May be clue to early diagnosis
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Radial Deficiencies
• Bilateral 50%
• Incidence within the same family is small
(5-10% of reported cases)
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Associated Syndromes
• Holt-Oram syndrome (cardiac defects)
• TAR (thrombocytopenia, absent radius)
• Fanconi anemia (aplastic anemia)
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Associated Syndromes
• VACTERRL (vertebral, anal atresia,
cardiac, tracheoesophageal fistula,
esophageal atresia, renal defects, radial
dysplasia, lower limb abnormalities)
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Anomalies
• Bone
– Scapula is often reduced in size
– Deficient capitellum and trochlea
common
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Anomalies
• Bone
– Ulna is approximately 60% normal length
– Ulna thickened and frequently
bowed toward the absent radius
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Anomalies
• Nerve
– Ulnar nerve is normal
– Radial nerve usually terminates elbow
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Anomalies
• Nerve
– Enlarged median nerve substitutes
sensation to the radial aspect of the hand
– Positioned in the fold between the wrist
and forearm
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Anomalies
• Tendon
– Muscles that originate or attach to the radius abnormal or absent
– FCU, ECU, interossei, and hypothenar muscles are often normal
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Anomalies
• Artery
– Normal brachial and ulnar artery
– Radial artery is often absent
– Interosseous arteries well developed
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Type
X-ray Findings
Clinical Features
I
Short radius
Distal radial epiphysis
delayed in appearance
Normal proximal radial
epiphysis
Mild shortening of radius
without bowing
Minor radial deviation of
the hand
Thumb hypoplasia is the
prominent clinical feature
requiring treatment
II
Hypoplastic
Distal & proximal
epiphysis present
Abnormal growth in both
epiphyses
Ulna thickened, shortened,
& bowed
Miniature radius
Moderate radial deviation
of the hand
III
Partial
absence
Partial absence (distal,
middle, proximal) of
radius
Distal 1/3-2/3 absence
most common
Ulna thickened, shortened,
& bowed
Severe radial deviation of
the hand
IV
Total absence
No radius present
Ulna thickened, shortened,
& bowed
Most common type
Severe radial deviation of
the hand
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Radial Deficiency
Treatment Goals
• Maximize length
• Improve
appearance
• Improve function
• Create stable wrist
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Natural History
• 90 to 100 radial deviation
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Treatment Paradigm
• Stretching
• Splinting
• Surgery
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Radial Deficiency - Stretching
• Start early & frequent splint changes
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Surgery
• Centralization indications/ contraindications
– Elbow motion
– Systemic conditions
– Age
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Centralization Principles
• Release tight radial structures to allow
centralization
• Carpal realignment over ulna
• Tendon balance
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Centralization
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Centralization
• ulnar osteotomy (greater than 30 degrees)
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Results & Complications
• Correction
• Recurrence
• Stiffness
• Functional impact
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Results & Complications
Damore E, Kozin SH, Thoder JJ, Porter S:
J Hand Surg 2000;25A:745-751.
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0
10
20
30
40
50
60
70
80
90
Preop Post op Follow up
Totalangulation
Damore, Kozin, Thoder, Porter. J Hand Surg 2000
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Does it Help Function?
• Effect on function unclear
• Individualized per patient
• Self-esteem?
• ??
Goldfarb, Manske, and colleagues. Functional outcome after
centralization for radius dysplasia
Hand Surg 2002;27A:118-124
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Treatment Advances
• Soft tissue lengthening
• Bone lengthening
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Case Example
• MJ- 9 year-old previous centralization
at age 2 with recurrence
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Assemble rings
Tighten wires
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Corticotomy
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Latency period
Lengthening
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Angular correction
& lengthening
Regenerate
bone
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4 years later
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Now What?
• Considerations
–Form
–Function
–??
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Questions
• Recurrence is common, if not
universal
• Centralization improves
appearance, but may not
significantly enhance function
• What is the role of centralization
in FA kids??
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Hypoplastic thumb
• Subgroup of radial deficiency
• Classification of thumb deficiency
– Important for treatment paradigm
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Findings
Treatment
I
Minor generalized hypoplasia
Augmentation
II
Absence of intrinsic thenar muscles
First web space narrowing
UCL insufficiency
Opponensplasty
1st-web release
UCL
reconstruction
III
Similar findings as type II plus:
Extrinsic abnormalities
Skeletal deficiency
A: Stable carpometacarpal joint
B: Unstable carpometacarpal joint
A: Reconstruction
B: Pollicization
IV
Pouce flottant or floating thumb
Pollicization
V
Absence
Pollicization
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Treatment Paradigm
– Type I No treatment
– Type II Reconstruction
– Type IIIA Reconstruction
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Treatment Paradigm
– Type IIIB Pollicization
– Type IV Pollicization
– Type V Pollicization
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Mild Hypoplasia (Blauth I) No Treatment Needed
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Thenar Hypolasia (Blauth II)
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Opponensplasty (Blauth II)
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Pollicization
• Timing
• Usually 1 year
–Developmental
–Ease
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Skin Incision
• Multiple options
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Skin Incision
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Bone & Joint Reorganization
–DIP joint IP joint
–PIP joint MP joint
–MP joint CMC joint
–Metacarpal Trapezium
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Outcome
• Related to degree of index mobility
Kozin SH et al. Functional Results After Index Finger
Pollicization for Congenital Aplasia or Hypoplasia of the
Thumb. J Hand Surg 17A:880-884, 1992.
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Results
• Enhance function
• Grasp > pinch
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Complications
• Stiffness
• Decreased motors tendon transfer
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9 year-old with
recurrent deformity
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Flexion Extension
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Formal centralization
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Ulnar approach
K-wire fixation
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Extension
Flexion
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Results
• Related to degree of index mobility
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Additional half-pins
as needed
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Hand Differences in Fanconi
Anemia
• Outcome
• Measurement tool
–DASH
– Jebsen-Taylor
• Reliable assessment
Goldfarb et al. Functional Outcome after Centralization
for Radial Dysplasia. J Hand Surg 2002;27A:118-124.
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Canadian Occupational
Performance Measure • Client-identified goals
• Client rated scores on performance of and satisfaction with FES
• Likert-type scale between 1-10
–1 cannot perform & extremely dissatisfied
–10 performs very well & extremely satisfied
Law et al. The Canadian Occupational Performance Measure.
CJOT 57: 1990;87.
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Hand Differences in Fanconi
Anemia
• Failure of formation- hypoplastic thumb
• Complications after pollicization
– Malposition