Cleft Hand

Imaging Gallery

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Imaging Gallery

Cleft hand (ectrodactyly) is a central longitudinal deficiency characterized by absence of one or more central rays, creating a V-shaped cleft. Classified as typical (sporadic, U-shaped cleft, good first web, better function) or atypical (syndromic/familial, V-shaped cleft, poor first web, worse function). Manske-Halikis classification (I-V) grades severity based on thumb web status and central defect. Associated with EEC syndrome (ectrodactyly-ectodermal dysplasia-clefting). Treatment focuses on functional reconstruction: cleft closure (Snow-Littler procedure), first web deepening, and syndactyly release at 12-18 months. First web space depth is the key determinant of function.

Cleft hand (also called ectrodactyly, split hand, or lobster claw deformity) is a congenital longitudinal deficiency characterized by the absence of one or more central rays (metacarpals and phalanges), creating a V-shaped or U-shaped cleft in the central portion of the hand.

Epidemiology

• Incidence: Approximately 1 in 90,000 to 1 in 150,000 live births
• Bilateral: 50% of cases involve both hands
• Associated Foot Clefts: 60% of patients also have cleft feet (split hand-split foot malformation - SHFM)
• Gender: Equal distribution male:female
• Typical vs Atypical: Approximately 70% typical, 30% atypical

Genetics and Inheritance

• Typical Cleft: Usually sporadic, no clear inheritance pattern
• Atypical Cleft: Often familial, autosomal dominant with variable expressivity and incomplete penetrance
• Genetic Loci: Multiple loci identified (chromosomes 2, 3, 7, 10, 17)
• EEC Syndrome: Autosomal dominant, TP63 gene mutation (chromosome 3q27)

Associated Syndromes

• EEC Syndrome (Ectrodactyly-Ectodermal dysplasia-Clefting): Most common
• SHFM (Split Hand-Split Foot Malformation): Isolated limb defects
• Other: Cornelia de Lange syndrome, Adams-Oliver syndrome

Embryology

• Develops during limb bud formation (4-8 weeks gestation)
• Failure of formation of central rays (index, middle, ring fingers)
• Zone of Polarizing Activity (ZPA) and Apical Ectodermal Ridge (AER) disruption
• Suppression or absence of central digital rays during differentiation
• First web space development affected in atypical forms

Normal Hand Development

• Digital Rays: 5 rays form from radial (thumb) to ulnar (small finger)
• Central Rays: Rays 2, 3, 4 (index, middle, ring) are central rays
• AER and ZPA: Control proximal-distal and radial-ulnar patterning
• Programmed Cell Death: Normal interdigital tissue apoptosis

Pathologic Anatomy in Cleft Hand

• Central Ray Absence: One or more of rays 2, 3, 4 are completely or partially absent
• Metacarpal Absence: May involve metacarpals, phalanges, or both
• Soft Tissue Cleft: V-shaped or U-shaped defect extends proximally
• Syndactyly: Border digits often joined (index-middle or ring-small)
• First Web Contracture: Variable suppression of thumb-index web space

Typical Cleft Anatomy

• U-Shaped Cleft: Wider, more shallow cleft
• Normal First Web: Good thumb-index web space preserved
• Central Deficiency Only: Isolated to central rays
• Better Function: Opposition and grasp relatively preserved
• Single Ray Absence: Often middle finger (ray 3) only

Atypical Cleft Anatomy

• V-Shaped Cleft: Narrow, deep cleft extending to carpus
• Suppressed First Web: Thumb-index web is tight, narrow, adducted
• Multiple Ray Absence: Often 2 or 3 central rays absent
• Transverse Bones: Abnormal transverse metacarpal elements
• Worse Function: Poor opposition and grasp

Key Anatomical Considerations

• First Web Space Depth: THE critical determinant of hand function
• Neurovascular Bundles: May have abnormal course around cleft borders
• Tendons: Extrinsic tendons may be absent or misdirected
• Thumb Position: May be radially deviated or adducted
• Border Digits: Often stiff, may have joint contractures

History

• Birth Presentation: Cleft visible at birth - obvious deformity
• Family History: Important to ask - atypical forms are familial
• Syndromic Features: Ask about skin/hair (ectodermal dysplasia), cleft palate
• Bilateral: Examine both hands AND both feet
• Developmental Milestones: Usually normal motor development

Physical Examination

Inspection

• Cleft Configuration: V-shaped vs U-shaped
• Number of Digits: Count present digits and identify missing rays
• First Web Space: Assess depth and width (CRITICAL for function)
• Syndactyly: Check for fusion of border digits
• Thumb Position: Assess for radial deviation or adduction contracture

Palpation

• Metacarpals: Palpate to determine which are present/absent
• Transverse Bones: Feel for abnormal transverse metacarpal elements (atypical)
• Soft Tissue: Assess tightness of first web space skin
• Neurovascular: Identify digital arteries/nerves (may be anomalous)

Functional Assessment (MOST IMPORTANT)

• Opposition: Can thumb oppose to remaining fingers?
• Pinch Strength: Assess tip pinch, key pinch, three-point pinch
• Grasp: Power grip capability with remaining digits
• First Web Span: Measure distance between thumb-finger when maximally abducted
• Activities: Can child manipulate objects, feed self, grasp toys?

Red Flags Requiring Syndrome Workup

• Sparse Hair/Teeth: Suggests ectodermal dysplasia (EEC syndrome)
• Cleft Lip/Palate: EEC syndrome
• Cleft Feet: Split hand-split foot malformation (SHFM)
• Multiple Limb Defects: Consider broader genetic syndrome
• Developmental Delay: Suggests syndromic etiology

Associated Findings

• Cleft Feet: 60% have split foot deformity
• Syndactyly: Common in border digits
• Polydactyly: May coexist with central ray absence
• Thumb Hypoplasia: May have underdeveloped thumb
• Stiffness: Border digits often have joint contractures

Radiographic Assessment

Plain Radiographs (Essential)

• AP and Lateral Views: Document bony anatomy
• Identify Present Rays: Determine which metacarpals/phalanges present
• Carpal Bones: May have carpal anomalies in atypical forms
• Transverse Bones: Look for abnormal transverse metacarpal elements
• Syndactyly: Assess bony vs soft tissue fusion

Findings on X-ray

• Typical: Usually absence of middle finger ray (ray 3) only
• Atypical: Multiple ray absence, transverse bones, carpal anomalies
• First Metacarpal: May be short or deviated radially
• Phalanges: May have hypoplastic or absent phalanges

Genetic Testing

• Indicated if: Familial pattern, syndromic features, bilateral involvement
• TP63 Gene: EEC syndrome mutation
• Chromosomal Microarray: Identify deletions/duplications
• Genetic Counseling: Essential for familial cases

Syndromic Workup (If Indicated)

• Skin/Hair/Teeth Assessment: Ectodermal dysplasia features
• Craniofacial Evaluation: Cleft lip/palate assessment
• Foot Examination: Look for cleft feet
• Ophthalmology: Eye anomalies in some syndromes
• Audiology: Hearing assessment if syndromic

Functional Assessment Tools

• Hand Function Tests: Age-appropriate functional testing
• Grip/Pinch Strength: Objective measurements (if child cooperative)
• Video Recording: Document baseline function before surgery

📊 Management Algorithm

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Treatment Goals (In Order of Priority)

1. Function First

• Maximize opposition capability (thumb-finger pinch)
• Improve grasp and release
• Deepen first web space if suppressed

2. Appearance Second

• Close cleft for cosmetic improvement
• Create more normal hand contour
• Improve social acceptance

3. Growth Preservation

• Maintain growth plates during surgery
• Avoid damage to viable digits

Conservative Management

Observation (Acceptable For)

• Manske Type I: Good function, normal first web - surgery optional
• Manske Type II: Mild impairment - may not require surgery
• Unilateral with Good Bilateral Hand Function: Non-dominant hand may not need surgery

Occupational Therapy

• Adaptive Strategies: Teach compensatory techniques
• Strengthening: Maximize function of existing digits
• First Web Stretching: If mild contracture present
• ADL Training: Feeding, dressing, writing adaptations

Surgical Management Principles

Timing: 12-18 months optimal

• Early enough to integrate before fine motor development
• Late enough for safe anesthesia and tissue handling
• Before social awareness (2-3 years)

Goals of Surgery:

• Close the cleft (improve appearance, prevent trauma to cleft margins)
• Deepen first web space (improve opposition and grasp - MOST IMPORTANT)
• Release syndactyly (if present in border digits)
• Maintain viability (preserve neurovascular supply to digits)

Intraoperative Complications

• Neurovascular Injury: Anomalous NV bundle course increases risk (1-2%)
• Excessive Bleeding: Difficult hemostasis in vascular anomalies
• Inadequate Release: Incomplete first web deepening
• Skin Flap Necrosis: Tension or poor design

Early Postoperative Complications (Less than 6 weeks)

• Flap Necrosis: 2-5% incidence - poor flap design or tension
• Infection: 1-3% - treat with antibiotics, may need debridement
• Hematoma: Rare - evacuate if compressing neurovascular structures
• Wound Dehiscence: More common if excessive tension during closure
• Skin Graft Loss: 5-10% of grafts may fail (syndactyly release)

Late Complications (More than 6 weeks)

• Web Space Contracture Recurrence: 10-20% - most common complication
• Scar Contracture: Linear scars contract - use Z-plasties to prevent
• Stiffness: Border digits may become stiff (5-10%)
• Growth Disturbance: If physis injured during surgery (rare with careful technique)
• Cold Intolerance: Vascular insufficiency in border digits
• Neuroma: If nerve transected or entrapped in scar

Functional Outcomes

Good Outcomes (Typical Cleft)

• Near-normal hand function achieved in 70-80%
• Good cosmetic result
• Improved opposition and grasp

Fair Outcomes (Atypical Cleft)

• Function improved but not normal in 50-60%
• First web space may re-contract requiring revision
• Multiple surgeries often needed

Poor Outcomes

• Severe first web contracture recurrence: 10-20% (especially atypical)
• Stiffness limiting function: 5-10%
• Neurovascular compromise: 1-2%

Revision Surgery

• Indications: Web space re-contracture, inadequate initial correction, scar contracture
• Timing: Wait at least 6-12 months for scar maturation
• Techniques: Repeat Z-plasty, skin grafting, free flap coverage
• Success: Lower success rate than primary surgery

Immediate Postoperative Period (0-2 weeks)

• Immobilization: Well-padded splint in position of function
• Thumb Position: Abducted if first web deepened
• Elevation: Keep hand elevated to reduce swelling
• Pain Management: Acetaminophen, avoid NSAIDs (may increase bleeding)
• Wound Care: Keep dressing clean and dry
• First Dressing Change: 7-10 days (or earlier if concerns)

Early Rehabilitation (2-6 weeks)

• Splint Removal: 3-4 weeks post-op
• Gentle ROM: Start passive range of motion exercises
• No Forceful Stretching: Allow tissues to heal before aggressive therapy
• Scar Massage: Begin gentle massage once incisions healed
• Occupational Therapy: Initiate therapy for hand function training

Late Rehabilitation (6 weeks - 6 months)

• Active ROM: Progress to active exercises and strengthening
• First Web Maintenance: Aggressive stretching to prevent re-contracture
• Functional Activities: Encourage use in age-appropriate play and ADLs
• Night Splinting: May use abduction splint at night for 3-6 months
• Scar Management: Silicone gel sheets, massage, moisturization

Long-Term Follow-Up

• Growth Monitoring: Annual follow-up through skeletal maturity
• Functional Assessment: Assess hand function at each visit
• Revision Planning: Identify need for further surgery as child grows
• Psychosocial Support: Address appearance concerns, school integration

Occupational Therapy Protocol

Phase 1 (Weeks 3-6)

• Gentle passive ROM
• Edema control
• Scar management initiation

Phase 2 (Weeks 6-12)

• Active ROM exercises
• Strengthening with therapy putty
• Fine motor skill development

Phase 3 (Months 3-12)

• Advanced strengthening
• Bilateral hand coordination activities
• Writing/drawing skills (age-appropriate)
• Adaptive equipment trial if needed

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