· Formation
· Separation
· Duplication
· Macrodactyly
· Hypoplasia
· Constriction
· General
Transverse failure
· absence of parts
Axial failure
· phocomelia
· radial club hand
· cleft hand
· ulnar club hand
· syndactyly
· Apert's syndrome
· polydactaly
· macrodactaly
· hypoplastic thumb
· hypoplastic hand and digits
· congenital trigger digits
· camptodactylty
· Kirner's deformity
· delta plalanx
· Madelung’s deformity
· Franz and O’Rahilly
· amelia - complete absence of limb
· phocomelia - terminal portion of limb attached to trunk
· hemimelia - absence of major portion of limb
· complete - entire distal half of limb absent
· incomplete - greater portion of distal half of limb absent
· paraxial - preaxial or postaxial portion of distal half of limb absent
· terminal - no unaffected parts distal
· intercalary - proximal and distal segments intact
· no unaffected parts distal to deficient portion
Transverse
· defect extends transversely
Hemimelia
· incomplete
· complete
Amelia
Paraxial
· only preaxial or postaxial portion of limb absent
Hemimelia
· radial
· ulnar
· tibial
· fibular
· middle portion of limb deficient
· proximal and distal portions present
Transverse
· entire central portion of limb absent
Phocomelia
· incomplete
· complete
Paraxial
· segmental absence of prexial or postaxial limb segments
· intact proximal and distal
Hemimelia
· radial
· ulnar
· tibial
· fibular
· limb buds develop at 4 weeks
· differentiate over next 3 weeks
· limb segments develop
· develop in proximal-to-distal sequence
· skeletal elements formed as cartilagenous models
· models ossify
· clefts form and develop joints
· embryonic skeleton well formed at 7 weeks
· numerous theories
· intrinsic factors
· environmental factors
· may cause
· arrest of development of embryonic limb
· destruction of structures already formed
· must occur between 4th and 7th week
· complete absence of some part distal to some point on the upper extremity.
· common levels
· fingers
· upper third forearm
· midcarpal
· 98% unilateral
· aetiology unknown
· except for thalidomide
· seal or flipper limb (Gr)
· seen with thalidomide
· often bilateral
· loss of intercalated segment
· hand suspended from trunk
· loss of elbow joint
· deformed hand with only 3-4 digits
· syn. radial hemimelia
· longitudinal failure of formation of parts along radial border
· short distal radius
· distal radial epiphysis present but delayed in appearance
· hypoplastic radius
· both proximal & distal radial epiphyses delayed in appearance
· moderate shortening of the radius and thickening of the ulna
· partial absence of radius
· usually distal third absent
· carpus unsupported and radially deviated
· total absence of radius
· most common type
· partial or complete radial absence as above
· scaphoid & trapezium absent in 50% cases
· thumb absent in 80%
· marked radial deviation of wrist
· forearm short
· 25% have associated abnormalities
· usually cardiac, haemopoetic, GIT
· often have congenital scoliosis
· splinting from birth
· soft tissue release at age 2 yrs
· reconstruction of severe deformity
· centralisation of carpus over ulna
· pollicization of index finger
· syn. ectrodactaly, crab claw, lobster claw
· longitudinal failure of formation of 2nd,3rd and 4th rays
· function often good
· surgery often not required
· webbed fingers
· due to a failure of separation during embryonic development
· common
· family history in 40%
· simple or complex
· only skin or bony stroctures involved
· complete or incomplete
· extent of finger involved
· acrosyndactaly
· fusion of fingers distally with proximal fenestrations
· brachysyndactaly
· shortening of syndactal digit
· involves
· 60% ring & middle
· 25% little & ring
· 15% index & middle
· bilateral in 50%
· frequently anomolous sharing of
· musculotendinous structures
· nerves and vessels
· fix before school age
· only release one side at a time
· may jepordize vascularity to finger
· preaxial
· duplication of the thumb (bifid thumb)
· most common
· central
· duplication of the index, middle or ring fingers
· rare
· postaxial
· duplication of the small finger
· common
· amputation of extra finger
· rare congenital anomaly with enlargement of the finger
· index finger most commonly affected
· aetiology uncertain
· static type
· grows with child at normal rate
· progressive type
· progressive enlargement out of proportion to normal growth
· surgery often unsuccessful
Types
· debulking procedures
· may need multiple debulks
· epiphysiodesis
· when digit achieved adult length
· digital shortening
· digital nerve stripping
· unsuccessful
· due to amniotic adhesions formed after haemorrhage in the distal rays
· simple ring
· transversely around limb
· deep ring
· associated lymphedema of distal part
· acrosyndactaly
· lateral fusion of digits
· intrauterine amputations
· digit shows persistent flexion deformity rather than triggering
· commonly in the thunb
· bilateral in 25% cases
· due to narrowed & thickened flexor sheath
· spontaneous resolution in 30%
· must be released before 3 years of age
· flexion deformity of the PIP joint
· usually of the little finger
· not related to trauma, systemic or neurological disorder
· different from clinodactaly (radial or ulnar deviation)
· strong hereditary predisposition
· bilateral in 50%
· congenital
· autosomal dominant trait
· may be due to
· short P1
· underdeveloped head of P1
· tight FDS
· abberant insertion of the lumbrical into the FDS tendon.
· initially is correctable
· secondary joint and skin contractures occur
· deformity present at birth
· may not become apparent until age 10 yrs.
· progresses with the growth spurt of puberty.
· does not progress after age 20 yrs.
· with time, PIP jt rotates into supination and volar skin bowstrings
Nonoperative
· usually preferred
Operative
· rarely required
· if deformity severe or cosmetically unacceptable
· deformity passively correctable
· release FDS insertion and reroute into the radial extensors
· deformity not passively correctable
· release insertion of the volar plate and accessory collateral ligament
· osteotomy
· delta phalanx
· abnormal trapezoidal shaped phalanx
· clinodactyly
· medial or lateral deviation of finger at middle phalanx
· most commonly due to delta phalanx
· strong family history
· most common in
· proximal phalanx of thumb
· middle phalanx of little finger
· abnormal C or J shaped epiphysis brackets one side of phalanx
· results in angular deformity
· Vickers
· resection of isthmus of the continuous epiphysis
· insertion of interpositional fat graft
· defective growth and premature fusion of of palmar ulnar part of distal radial epiphysis
· congenital disorder
· autosomal dominant transmission
· more common in females
· idiopathic (most common)
· trauma to radial physis
· skeletal dysplasias
· multiple exostoses
· dyschondroplasia
· other
· Turner’s syndrome
· mucopolysaccharidosis
Deformity
· development of progressive ulnar and volar tilt at distal radial articular surface
· resultant dorsal subluxation of the distal ulna
· lunate lies deeply between lower end of radius and ulna
Appearance
· distal end of ulna unduly prominent dorsally
· radial deviation of wrist
Restriction
· limitation of dorsiflexion and pronation
· lower end of ulna
· enlarged
· dorsally subluxed
· abnormal distal radial physis
· sloping
· medial fusion
· proximal carpal row distorted
· V-shape
· lunate in apex between radius and ulna
· initially nonoperative
· usually pain free with good function
· may require operative intervention
· medial opening wedge distal radial osteotomy
· shortening osteotomy of distal ulna