congenital disorders of the hand
TRANSCRIPT
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Congenital Disorders of the Hand
Overview and Classification
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Classification◆ Phocomèle ◆ Hemimèle ◆ Ectromèle
Isidore Geoffrey, Saint-Hilaire, 1837
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Classification◆ Kelikian ◆ Frantz & O’Rahilly ◆ Swanson ◆ IFSSH
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I. Failure of Formation of Parts II. Failure of Separation of Parts III. Duplication IV. Overgrowth
Classification
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V. Undergrowth VI. Congenital Constriction Ring
Syndrome VII. Generalized Skeletal
Abnormalities
Classification
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I. Failure of Formation of Parts
A. Transverse !B. Longitudinal
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Longitudinal Absences
Phocomelia
Radial Club Hand Cleft Hand Ulnar Club
Hand© Louisville Hand Surgery
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Longitudinal Deficiency◆ PHOCOMELIA ◆ RADIAL (Preaxial) ◆ CENTRAL ◆ ULNAR (Postaxial)
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Transverse Absences
Upper Limb
Forearm and Hand
Hand Digits
© Louisville Hand Surgery
PhalangesNormal
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II. Failure of Differentiation
A. Soft Tissue Involvement B. Skeletal Involvement C. Congenital Tumorous
Conditions
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Syndactyly
© Louisville Hand Surgery
SimpleComplex
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A. Soft Tissue 1. Disseminated - Arthrogryposis 2. Shoulder 3. Elbow & Forearm 4. Wrist & Hand
a. Cutaneous syndactyly b. Camptodactyly c. Thumb-in-palm deformity d. Deviated/deformed digits
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B. Skeletal1. Shoulder 2. Elbow - Elbow Synostosis 3. Forearm
a. Proximal radioulnar synostosis b. Distal radioulnar synostosis
4. Wrist & Hand a. Osseous syndactyly b. Synostosis of carpal bones c. Symphalangism d. Clinodactyly
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C. Tumorous Conditions1. Hemangiotic 2. Lymphatic 3. Neorogenic 4. Connective Tissue 5. Skeletal
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III. Duplication1. Whole Limb 2. Humeral Segment
3. Ulnar Segment - Mirror Hand
4. Digit - Polydactyly
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IV. Overgrowth
1. Whole Limb 2. Partial Limb
3. Digit - Macrodactyly
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V. Undergrowth
1. Whole Limb 2. Partial Limb
3. Digit - Brachydactyly
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VI. Congenital Constriction Ring
Syndrome
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VII. Generalized Skeletal Abnormalities
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Diagnosis◆ Whole body/multiple limb ◆ Entire Limb ◆ Too many fingers ◆ Too few fingers ◆ Short Fingers
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Diagnosis◆ Abnormal Thumb ◆ Too Big ◆ Bent Fingers ◆ Rigid Parts ◆ Combined Parts ◆ Neuromuscular Abnormalities
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Whole Body/Multiple Limbs
◆ SYNDROMIC ◆ Apert & Craniofacial Syndromes
◆ Dwarfism
◆ Arthrogryposis
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Entire Limb◆ Proximal Parts - Phocomelia ◆ Unstable/Fused Elbow - Ulnar
Dysplasia ◆ Radially deviated hand - Radial
Club Hand
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Too Many Fingers◆ Ulnar - Ulnar Polydactyly ◆ Thumb - Radial Polydactyly ◆ Central - Central Polydactyly ◆ Many Fingers/No Thumbs -
Mirror Hand
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Too Few Fingers
◆ Single Digit Hand/Ulnar Side - Cleft Hand
◆ Single Digit Hand - Symbrachydactyly
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Too Few Fingers◆ Normal structures proximal/Bands/
Edema/Other Limbs/Scarring - Amniotic Band
◆ Normal/underdeveloped proximal structures.Small/absent fingers/ Nubbins - Symbrachydactyly
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Too Few Fingers
◆ Absent/Small Thumb - Hypoplastic Thumb
◆ Combined/Missing Fingers/Deep Central Cleft - Cleft Hand
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Too Few Fingers
◆ Two/Three Fingered Hand; Thumb Present - Ulnar Dysplasia
◆ Stubby, Empty Soft Tissue Sleeves; Fingers Partially or Totally Absent - Symbrachydactyly
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Short Fingers◆ Brachydactyly ◆ Brachymetacarpia ◆ Brachyphalangia ◆ Brachysyndactyly
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Abnormal Thumb
◆ Small Thumb - Hypoplastic Thumb ◆ Segmented Thumb - Radial
polydactyly ◆ Finger like thumb - Triphalangeal
Thumb
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Abnormal Thumb
◆ Angled Thumb - Delta Phalanx ◆ Thumb in same plane as fingers/too
few fingers - Ulnar Dysplasia ◆ Thumb in same plane/Many fingers -
Mirror Hand
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Abnormal Thumb
◆ Tight Thumb - Trigger Digit, Spastic Hand/Arthrogryposis/ Clasped Thumb
◆ Long Thumb - Triphalangeal Thumb/Radial polydactyly
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Too Big◆ Entire Limb - Hemihypertrophy ◆ Single Finger - Ollier, Klippel-
Trenaunay-Weber/Vascular malformations/Hamartomatas
◆ Combined Digital Parts - Cleft Hands
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Bent Fingers◆ Flexed - Trigger, Camptodactyly,
arthrogryposis ◆ Small Fingertip/Adolescent/
Progressive - Kirner’s Deformity ◆ Angled - Delta Phalanx/Clinodactyly
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Rigid Parts◆ Rigid Forearm/Lack of Motion -
Radioulnar Synostosis ◆ Rigid Elbow - Radiohumeral
Synostosis/Ulnar Hemimelia ◆ Limited Elbow Motion - Congenital
Radial Head Dislocation
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Rigid Parts◆ Digit Stiff in Extension -
Symphalangism !◆ Digit Stiff in Flexion - Contractures,
Camptodactyly, Arthrogryposis
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Combined Parts◆ Webbed Fingers - Syndactyly ◆ Unsegmented Finger Joints -
Symphalangism, Brachysyndactyly ◆ Combined Digital Skeleton -
Cleft Hand ◆ Lack of forearm Motion- RU
Synostosis
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Neuromuscular Abnormalities
◆ Generalized Weakness - Congenital Myopathies, Cerebral Palsy
◆ Spasticity - Encephalopathy, Cerebral Palsy
◆ Intrinsic Muscle Weakness/Tightness
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