FIBULAR HEMIMELIA
Most Common Long Bone Deficiency | Achterman-Kalamchi Classification | Syme vs Reconstruction | Foot Preservability Key
ACHTERMAN-KALAMCHI CLASSIFICATION
Critical Must-Knows
- Treatment based on foot preservability, NOT fibula length - key principle: assess foot rays (under 3 = Type 2 = amputation usually)
- Achterman-Kalamchi classification: Type IA (mild), IB (moderate), II (severe) - guides treatment approach
- Birch classification: Type 1 (foot preservable, 4-5 rays) vs Type 2 (foot not preservable, under 3 rays)
- Syme amputation: Indicated for predicted LLD over 30-40cm, under 3 foot rays, severe ankle valgus over 30 degrees
- Reconstruction: Multiple lengthenings, foot centralization, ankle stabilization - long treatment course (10-15 years)
Examiner's Pearls
- "Treatment decision based on FOOT (not fibula) - under 3 rays = Type 2 = usually amputation
- "Syme amputation preserves plantar heel pad for end weight-bearing - better function than BKA
- "LLD percentage is constant throughout growth - predict final LLD using multiplier method
- "Reconstruction requires 2-4 lengthenings, each 4-8cm, starting age 6-8 years
Clinical Imaging
Imaging Gallery




Critical Fibular Hemimelia Exam Points
Foot Preservability Determines Treatment
Treatment based on FOOT, not fibula length - assess foot rays: 4-5 rays (Type 1) = reconstruction possible, under 3 rays (Type 2) = usually Syme amputation. This is the most important decision point.
Syme vs Reconstruction Decision
Syme amputation: Predicted LLD over 30-40cm, under 3 foot rays, ankle valgus over 30 degrees, family acceptance. Reconstruction: LLD under 30cm, 4-5 foot rays, moderate valgus (correctable), motivated family. Syme often has better function in severe cases.
LLD is Main Problem
Limb length discrepancy is the primary issue - predict final LLD using multiplier method. LLD percentage is constant throughout growth. Severe LLD (over 30-40cm predicted) favors amputation over reconstruction.
Associated Anomalies Common
Common associations: Anteromedial tibial bowing, tarsal coalition, equinovalgus foot, cruciate deficiency, PFFD (50%), coxa vara, acetabular dysplasia. Always assess entire limb and other systems (cardiac, renal).
Fibular Hemimelia Treatment Decision - Quick Reference
| Foot Rays | Predicted LLD | Ankle Valgus | Treatment |
|---|---|---|---|
| 4-5 rays | Under 30cm | Under 30 degrees | Reconstruction |
| 3-4 rays | 30-40cm | 30-40 degrees | Reconstruction or Syme |
| Under 3 rays | Over 30-40cm | Over 30 degrees | Syme amputation |
FOOTFibular Hemimelia Treatment Decision
Memory Hook:FOOT determines treatment: Foot rays count, Over 30cm LLD, Over 30° valgus, and Treatment decision based on foot!
TALUSFibular Hemimelia Associated Findings
Memory Hook:TALUS findings: Tibial bowing, Ankle valgus, Limb length discrepancy, Ulnar hemimelia, and Syndactyly/tarsal coalition!
SALVAGESyme Amputation Indications
Memory Hook:SALVAGE decision: Severe LLD, Ankle valgus over 30°, Less than 3 rays, Valgus uncorrectable, Acceptance needed, Greater function, End weight-bearing capability!
Overview and Epidemiology
Fibular hemimelia is the most common congenital long bone deficiency, characterized by partial or complete absence of the fibula. It represents a spectrum from mild hypoplasia to complete absence, with associated limb length discrepancy and foot deformities.

Epidemiology:
- Incidence: 1 in 40,000 live births
- Male to female ratio: 1.5:1
- Bilateral involvement: 9-52% of cases
- Right side slightly more common
- Most common long bone deficiency
Pathophysiology: Fibular hemimelia results from failure of normal fibular development during embryogenesis. The exact cause is unknown but may involve:
- Vascular insult during development
- Genetic factors (rare familial cases)
- Teratogenic exposure
- Failure of mesenchymal condensation
The condition represents a spectrum, with Achterman-Kalamchi classification describing severity based on fibular presence and foot status.
Pathophysiology and Mechanisms
Normal Fibular Anatomy: The fibula is the lateral bone of the lower leg. It provides:
- Lateral stability to the ankle
- Muscle attachments (peroneals, flexor hallucis longus)
- Contributes to ankle mortise
- Minimal weight-bearing (10-15%)
Fibular Hemimelia Pathology: In fibular hemimelia, there is:
- Partial or complete absence of fibula
- Anteromedial tibial bowing (characteristic)
- Limb length discrepancy (main problem)
- Foot deformities (equinovalgus, absent rays, tarsal coalition)
- Ankle valgus (lateral tether absent)
- Knee problems (valgus, cruciate deficiency)
Pathophysiology: The absence of fibula causes:
- Loss of lateral support → ankle valgus
- Anteromedial tibial bowing (compensatory)
- Limb length discrepancy (fibula contributes to length)
- Foot deformities (lateral column affected)
Associated Musculoskeletal Findings:
- Anteromedial tibial bowing (characteristic)
- Tarsal coalition (common)
- Equinovalgus foot (most common)
- Absent lateral rays (common)
- Cruciate ligament deficiency
- Genu valgum
- Lateral femoral condyle hypoplasia
- PFFD (50% association)
- Coxa vara
- Acetabular dysplasia
Understanding the anatomy helps determine treatment - foot status is key, not fibula length.
Classification Systems
Achterman-Kalamchi Classification (1979)
Based on fibular presence and foot status:
Achterman-Kalamchi Classification Summary
| Type | Fibula | Foot | Treatment |
|---|---|---|---|
| Type IA | Present, hypoplastic (under 50% short) | Normal (5 rays) | Reconstruction |
| Type IB | Present (over 50% short) | 3-4 rays | Reconstruction or Syme |
| Type II | Complete absence | 0-3 rays | Syme amputation (usually) |
Type IA: Mildest form. Fibula present but hypoplastic (under 50% of normal length). Foot normal with 5 rays. Treatment: Reconstruction with lengthening, epiphysiodesis if needed.
Type IB: Moderate severity. Fibula present but significantly shortened (over 50% of normal length). Foot has 3-4 rays. Treatment: Reconstruction possible but complex, or Syme amputation depending on LLD and family preference.
Type II: Most severe. Complete absence of fibula. Foot has 0-3 rays. Treatment: Usually Syme amputation (better function than reconstruction in severe cases).
The classification guides treatment approach but foot status is the key decision factor.
Clinical Assessment
History:
- Shortened lower limb noted at birth
- May have foot deformity noted
- Family history (rare but may be present)
- Difficulty with walking or activities
- Previous treatment (if established case)
Physical Examination:
Inspection:
- Shortened lower limb
- Anteromedial tibial bowing (characteristic)
- Foot deformity (equinovalgus most common)
- Assess number of foot rays (toes)
- Assess for bilateral involvement
- Look for associated deformities (hip, knee, upper limb)
Palpation:
- Fibula may be absent or hypoplastic
- Anteromedial tibial bowing palpable
- Assess ankle stability
- Assess foot structure
Range of Motion:
- Ankle: May have limited motion, valgus deformity
- Knee: May have valgus, assess cruciate function
- Hip: Assess for PFFD, coxa vara
- Assess for contractures
Measurements:
- True leg length: ASIS to medial malleolus
- Apparent leg length: umbilicus to medial malleolus
- Tibial length: knee joint line to ankle
- Predict final LLD using multiplier method
Foot Assessment:
- Count functional rays (toes)
- Assess foot position (equinovalgus, equinovarus)
- Assess for tarsal coalition
- Assess ankle valgus angle
Associated Examination:
- Hips: Assess for PFFD (50% association)
- Knees: Assess for valgus, cruciate deficiency
- Upper limbs: Assess for ulnar hemimelia
- Other systems: Cardiac, renal (rare associations)
Investigations
Radiographs:
AP and Lateral Lower Limb:
- Assess fibular presence/absence
- Evaluate anteromedial tibial bowing
- Measure limb length discrepancy
- Assess ankle valgus angle
- Evaluate foot structure (ray count, tarsal coalition)
Full-Length Standing Radiographs (Scanogram):
- Accurate LLD measurement
- Assess alignment
- Evaluate tibia-femur ratio
- Plan treatment
Foot Radiographs:
- Count functional rays
- Assess for tarsal coalition
- Evaluate foot structure
- Assess ankle valgus
CT Scan (if reconstruction considered):
- Detailed foot anatomy
- Assess tarsal coalition
- Plan foot centralization
- Evaluate ankle structure
Other Imaging:
- MRI: May assess soft tissue structures
- Ultrasound: Usually not needed
LLD Prediction:
- Multiplier method: Current LLD × multiplier = predicted final LLD
- Growth remaining method: More complex, accounts for growth
- LLD percentage is constant throughout growth
Management Algorithm

Treatment Philosophy
Key principle: Treatment based on foot preservability, NOT fibula length.
Decision factors:
- Foot rays: Under 3 rays (Type 2) = usually amputation, 4-5 rays (Type 1) = reconstruction possible
- Predicted LLD: Over 30-40cm = favors amputation, under 30cm = reconstruction possible
- Ankle valgus: Over 30 degrees = favors amputation, under 30 degrees = may be correctable
- Family preference: After counseling about both options
Treatment options:
- Syme amputation: For severe cases (Type 2, LLD over 30-40cm, severe valgus)
- Reconstruction: For milder cases (Type 1, LLD under 30cm, correctable valgus)
No "wrong" choice - shared decision making essential.
Surgical Techniques
Syme Amputation
Indication: Type 2 (under 3 foot rays), predicted LLD over 30-40cm, severe ankle valgus.
Technique:
- Incision:
- Transverse across anterior ankle (just above joint line)
- Plantar extension in racquet fashion
- Encompass plantar heel pad
- Dissection:
- Divide nerves high (prevent neuroma): sural, saphenous, deep peroneal, tibial
- Ligate vessels: posterior tibial, anterior tibial
- Divide tendons
- Disarticulation:
- Disarticulate ankle joint
- Remove foot
- Resect medial malleolus flush
- Smooth tibial end
- Heel pad preservation:
- Preserve heel pad with posterior tibial artery branches
- Fix centrally to tibia with sutures through drill holes
- Closure:
- Close without tension
- End weight-bearing stump
Postoperative: Cast 2-3 weeks, then prosthetic fitting.
Key point: Preserves plantar heel pad for end weight-bearing (better than BKA).
Complications
Reconstruction Complications:
Early:
- Infection (20-30% with external fixators)
- Wound healing problems
- Neurovascular injury
- Inadequate correction
Late:
- Persistent LLD (may need additional lengthenings)
- Ankle problems (instability, stiffness, arthritis)
- Knee problems (valgus, cruciate deficiency)
- Foot problems (deformity recurrence, tarsal coalition)
- Contractures (ankle, knee)
- Hardware problems
Lengthening Complications:
- Pin site infection (common)
- Stiffness (ankle, knee)
- Contractures
- Delayed union
- Premature consolidation
- Nerve injury (peroneal most common)
- Refracture
Syme Amputation Complications:
Early:
- Wound healing problems (5-10%)
- Heel pad migration (if not properly fixed)
- Infection (rare)
Late:
- Heel pad migration (may need revision)
- Bony overgrowth (may need revision)
- Prosthetic fitting problems (rare)
Prevention:
- Careful patient selection
- Meticulous surgical technique
- Aggressive physical therapy (reconstruction)
- Long-term bracing (reconstruction)
- Realistic expectations
Postoperative Care
Syme Amputation:
Immediate:
- Pain management
- Wound care
- Cast 2-3 weeks
After Healing:
- Prosthetic fitting (6-8 weeks)
- Gait training
- Return to activities
Long-term:
- Prosthetic adjustments as child grows
- Monitor for heel pad migration
- Monitor for bony overgrowth
Reconstruction:
Immediate:
- Pain management
- Wound care
- Cast or external fixator care
Lengthening Phase:
- Pin site care (daily cleaning)
- Distraction protocol (1mm/day)
- Physical therapy (critical)
- Regular radiographs
After Union:
- Protected weight-bearing
- AFO long-term
- Physical therapy
- Gradual return to activities
Long-term Follow-up:
- Annual assessment until skeletal maturity
- Monitor LLD progression
- Assess function
- Address complications
Outcomes and Prognosis
Functional Outcomes:
Syme Amputation:
- Excellent function in 80-90%
- Running, sports possible with prosthesis
- Minimal restrictions
- High patient satisfaction
- Single surgery, short treatment
Reconstruction:
- Variable function (60-70% good outcomes)
- Often residual LLD (2-5cm)
- Ankle problems common (instability, stiffness)
- May need AFO long-term
- Multiple surgeries (2-4 lengthenings)
- Long treatment course (10-15 years)
Quality of Life:
- Syme: Excellent quality of life, high satisfaction
- Reconstruction: Variable, depends on outcome
- Both groups function well overall
- Psychosocial support important
Predictors of Success:
- Appropriate patient selection
- Syme: Proper technique, heel pad preservation
- Reconstruction: Motivated family, adequate rays, manageable LLD
Long-term:
- Syme: Prosthetic adjustments as child grows, otherwise stable
- Reconstruction: May need additional procedures, ankle problems may worsen
- Both: Most function independently
Evidence Base
Achterman-Kalamchi Classification
- Classification based on fibular presence and foot status
- Type IA: Mild, reconstruction possible
- Type IB: Moderate, reconstruction or amputation
- Type II: Severe, usually amputation
Birch Classification and Treatment
- Treatment based on foot preservability, NOT fibula length
- Type 1 (4-5 rays): Reconstruction possible
- Type 2 (under 3 rays): Usually amputation
- LLD percentage constant throughout growth
Syme vs Reconstruction Outcomes
- Syme amputation: Better function in severe cases
- Single surgery vs multiple for reconstruction
- Lower complication rate with Syme
- Higher patient satisfaction with Syme in severe cases
Reconstruction Outcomes in Fibular Hemimelia
- Reconstruction requires 2-4 lengthenings
- Each lengthening: 4-8cm
- Treatment course: 10-15 years
- 60-70% good outcomes, but complications common
Associated Anomalies in Fibular Hemimelia
- 50% associated with PFFD
- Tarsal coalition common
- Anteromedial tibial bowing characteristic
- Equinovalgus foot most common
- Cardiac and renal associations rare
Exam Viva Scenarios
Practice these scenarios to excel in your viva examination
Scenario 1: Initial Assessment
"A 1-year-old child presents with fibular hemimelia. On examination, the fibula is completely absent, there is anteromedial tibial bowing, the foot has 2 functional rays, and there is severe ankle valgus (40 degrees). How would you assess and manage this child?"
Scenario 2: Type 1 Reconstruction
"A 2-year-old child with fibular hemimelia has a hypoplastic fibula (Type IA), foot with 4 functional rays (Type 1), predicted LLD of 12cm, and moderate ankle valgus (20 degrees). The family prefers reconstruction over amputation. What is your treatment plan?"
Scenario 3: Syme Amputation Technique
"You have decided to perform a Syme amputation for a 2-year-old with Type 2 fibular hemimelia. Describe the key technical steps and how you preserve the heel pad for end weight-bearing."
MCQ Practice Points
Treatment Decision Question
Q: What is the most important factor in determining treatment for fibular hemimelia - amputation vs reconstruction? A: Foot preservability (number of functional rays), NOT fibula length - this is the key principle. Under 3 foot rays (Type 2) = usually Syme amputation. 4-5 foot rays (Type 1) = reconstruction possible. The decision is based on the foot, not the fibula.
Classification Question
Q: What is the Achterman-Kalamchi Type II fibular hemimelia? A: Complete absence of fibula with foot having 0-3 rays - this is the most severe form. Treatment is usually Syme amputation, as reconstruction is complex and often has inferior outcomes compared to amputation in these severe cases.
Syme Indication Question
Q: What are the indications for Syme amputation in fibular hemimelia? A: Predicted LLD over 30-40cm, foot with under 3 functional rays (Type 2), severe ankle valgus over 30 degrees, and family acceptance - Syme amputation provides better function than complex reconstruction in severe cases and is the standard treatment for Type 2 fibular hemimelia.
Reconstruction Question
Q: How many lengthening procedures are typically needed for fibular hemimelia reconstruction? A: 2-4 lengthenings, each achieving 4-8cm - reconstruction requires multiple staged lengthenings starting at age 6-8 years. Each lengthening takes 2-3 months per cm for consolidation. Total treatment course is 10-15 years with significant complication rates.
Associated Anomaly Question
Q: What percentage of fibular hemimelia cases are associated with proximal femoral focal deficiency (PFFD)? A: 50% - fibular hemimelia and PFFD are commonly associated. Always assess the hip when evaluating fibular hemimelia. Other common associations include anteromedial tibial bowing, tarsal coalition, equinovalgus foot, and cruciate ligament deficiency.
Australian Context and Medicolegal Considerations
Healthcare System:
- Fibular hemimelia management requires specialized pediatric orthopedic centers
- Syme amputation and reconstruction both available
- Public hospital system provides comprehensive care
- Multidisciplinary teams available (orthopedics, prosthetics, psychology)
Multidisciplinary Care:
- Pediatric orthopedic surgeon (primary)
- Prosthetist (for Syme amputation cases)
- Physiotherapist (critical for reconstruction rehabilitation)
- Occupational therapist (activities of daily living)
- Psychologist (support for child and family)
- Social worker (financial and social support)
Medicolegal Considerations:
- Informed consent critical - major decision (amputation vs reconstruction)
- Shared decision making essential - no "wrong" choice
- Realistic expectations about outcomes and complications
- Documentation of foot assessment (ray count)
- Family counseling about both options
- Long-term follow-up until skeletal maturity
Prosthetic Services:
- Available through public and private providers
- Regular adjustments needed as child grows
- Functional prostheses for activities and sports
- High-quality prostheses available
Research and Outcomes:
- Australian centers contribute to international research
- Registry data helps track long-term outcomes
- Quality of life studies important for treatment decisions
FIBULAR HEMIMELIA
High-Yield Exam Summary
Key Facts
- •Incidence: 1 in 40,000 live births (most common long bone deficiency)
- •Treatment based on FOOT (not fibula) - key principle
- •Achterman-Kalamchi: Type IA (mild), IB (moderate), II (severe)
- •Birch: Type 1 (4-5 rays, preservable) vs Type 2 (under 3 rays, not preservable)
Achterman-Kalamchi Classification
- •Type IA: Fibula present, hypoplastic (under 50% short), foot normal = Reconstruction
- •Type IB: Fibula present (over 50% short), foot 3-4 rays = Reconstruction or Syme
- •Type II: Complete fibular absence, foot 0-3 rays = Syme amputation (usually)
- •Severity correlates with LLD and foot deformity
- •Most common presentation is Type II (complete absence)
Treatment Decision (Birch)
- •Type 1 (4-5 rays): Reconstruction possible - foot centralization, lengthenings
- •Type 2 (under 3 rays): Usually Syme amputation - better function in severe cases
- •Key: Foot rays determine treatment, NOT fibula length
- •LLD over 30-40cm predicted also favors amputation
Surgical Pearls
- •Syme: Preserve plantar heel pad with posterior tibial artery branches
- •Fix heel pad centrally to tibia (drill holes, sutures) - prevents migration
- •Reconstruction: Foot centralization first, then staged lengthenings (2-4 total)
- •Lengthening: 1mm/day distraction, aggressive PT critical, 2-3 months per cm
Complications
- •Reconstruction: Infection (20-30%), stiffness, contractures, delayed union
- •Syme: Heel pad migration (if not properly fixed), wound healing (5-10%)
- •Reconstruction: Residual LLD (2-5cm common), ankle problems (instability, stiffness)
- •Prevention: Careful patient selection, meticulous technique, aggressive PT