plastic surgery

1. CONGENITAL HAND ANOMALIES
Dr Subhakanta Mohapatra
IPGME&R,Kolkata.INDIA

2. Embryology
 Upper limb bud

Develops from lateral wall of embryo .
 On 4th wk after fertilization .
 Consists of mesodermal cells covered by ectoderm .
 Under guidance of three signaling centers:
1. AER (apical ectodermal ridge) - proximo-distal.
2. ZPA (zone of polarizing activity) - antero-posterior
growth.
3. NRE (non ridge ectoderm)(wing less type) - dorsoventral growth.

3. Swanson classification

4. SWANSON CLASSIFICATION
 Accepted by IFSSH & ASSH.
 Based on their embryologic origin & morphological
appearance.
 Expanded by Knight & Kay in 2000, & Upton in 2006.
 But recently , adequacy of this classification has been
questioned.

5. Swanson classification
 Failure of formation of parts
 Failure of differentiation or separation of
parts
 Duplication
 Overgrowth
 Undergrowth
 Congenital constriction ring syndrome
 Generalized skeletal abnormalities &
syndromes

6. 1. Failure of formation of parts
 Detected prenatally.
2 types:
A.Transverse arrest
B. Longitudinal arrest:
 radial club hand (pre-axial arrest)
 ulnar club hand (post-axial arrest)
 cleft hand (central arrest)
 phocomelia (intercalary arrest) – an
intervening segment of limb is absent.

7. A. Congenital transverse arrest
 Rare, always U/L.
 Sporadic / environmental.
 Level defined by skeletal absence.
 Commonly at level of proximal forearm.
 Defect in AER signaling
 Lt > Rt
 2 groups:
 defect in limb formation
 Intrauterine amputation after limb formation
 Most will not require surgery, but benefited
from prosthesis if referred early.

8. B. Longitudinal arrest
i) Phocomelia (seal limb)
 Intercalary arrest
 Intervening segment of limb is absent (arm/forearm)
 Thalidomide in 1st trimester
 Type I (complete) - hand directly attached to trunk
Type II (proximal) – short forearm attached to trunk.
Type III (distal) – short humerus attached to hand.
 Surgery – very little role.

9. ii) Radial ray dysplasia (aka: Radial club hand/Preaxial
deficiency/Longitudinal radial deficiency)
 Radially deviated, flexed hand with pronated and
shortened forearm.
 Deficient thumb ray & carpal bones (scaphoid &
trapezium), radial nerve & vessels
• Normal ulnar two digits
• Median nerve subluxed towards concave side.
 Commonly associated with syndromes (e.g. VATER,
TAR, Holt oram)
 U/L , M>F , Rt>Lt .
 I –Short radius
II – Hypoplastic radius
III – Partial absence of radius (replaced by anlage)
IV – Complete absence of radius – most severe &
common.

10. Radial ray dysplasia

11. Bayne & Klug classification of radial
longitudinal deficiency

12. iii) Ulnar ray dysplasia (aka: Ulnar club
hand /Postaxial deficiency)
 Rarest of longitudinal ray deficiency.
 Association with syndromes – uncommon
 Disruption of ZPA signaling.
 Short,bowed radius with a hypoplastic or absent
ulna.
 Elbow severely affected (with a relatively stable
wrist).
 M>F , Lt>Rt , U/L>B/L.

13. Bayne
classification
 I – Ulnar
hypoplasia
 II – Partial ulnar
aplasia
 III- Total ulnar
aplasia
Paley & Herzenberg
classification
 I – Ulnar hypoplasia with intact
distal epiphysis
 II – Partial ulnar aplasia (distal
1/3rd )
 III – Partial ulnar aplasia (distal
2/3rd )
 IV – Total ulnar aplasia
 IV – Radiohumeral
synostosis
 V – Radiohumeral synostosis

14. Bayne & Klug classification of ulnar
longitudinal ray deficiency

15. iv) Central ray deficiency /cleft
hand
 Most common longitudinal deficiency.
 Defect in AER signaling.
 B/L (frequently).
 Structures proximal to wrist – normal.
 Little finger – always present
 Associated syndactyly & narrow web space.
 Complex syndactyly (thumb & index) – in severe
case.
 Hand – “functionally good but aesthetically a
disaster‟‟.
 Association with cleft feet in 1/3rd cases (SHSF)
 Other Syndromic associations: EEC
syndrome(ectrodactyly, ectodermal dysplasia,

16. Atypical cleft
hand
Typical cleft hand
 Deep V shaped central








defect
Bilateral
Inherited (AD)
Cleft feet associated
Hypoplasia of long ray
Thumb involved
Associated cleft lip/palate
No chest wall
involvement
Little finger – only digit
 Shallow U shaped








defect
Unilateral
Sporadic
Not
Rays of central 3 digits
Rarely
No
Seen in Poland
syndrome
Thumb - only digit

17. Typical cleft hand with central deep V
shaped cleft

18. Manske‟s classification of cleft
hand
 Type I – Normal 1st web
• Type II
A – mildly narrowed web
B – severely narrowed
 Type III – Syndactylised web
 Type IV – Merged web
 Type V – Absent web
A – Partial suppression of
radial ray
B – Complete suppression of
radial ray

19. 2. Failure of differentiation or separation of parts
A. Syndactyly
 One of the most common congenital hand
malformations.
 3rd web > 4th web > 2nd web
 Association – Poland, Apert syndrome.
 Complicated syndactyly –
 More than only distal bony fusion
 Abnormal bone structure inside .
(fusion ,missing bone, abnormal joints,
rudimentary bones ,cross bones)
 Seen in Apert syndrome,
Central synpolydactyly
Typical cleft hand

20. Complex & complicated
syndactyly

21. B. Contracture
i) Clinodactyly (inclined finger)
Radio ulnar deviation of digit ( >
10 ) distal to MCPJ.
Most common –
Radial deviation of little finger
at DIP.
(Middle phalanx of little finger
– last
bone to ossify)
2nd most common –
proximal phalanx of thumb
Due to Delta phalanx( a
trapezoid shaped middle phalanx

22. ii) Camptodactyly (arched finger)
 Painless, progressive flexion Contracture of
PIPJ
( antero posteriorly)
 Due to imbalance in flexors
& extensors
 Little finger (>70% cases)
 3 types:
type I . newborn (M=F)
type II. adolescent females
type III. Multiple digits/with
syndromes

23. iii) Congenital trigger thumb
 Stenosing tenosynovitis of FPL tendon at A1 pulley .
 Fixed flexion of IPJ (thumb locked in flexion).
 “Notta node” – palpable nodule over flexor aspect of
MCPJ of thumb proximal to A1 pulley.
 Snapping/popping as the nodule passes beneath A1
pulley
 Compensatory hyperextension at MCPJ.
 Frequently B/L.

24. iv) Congenital clasped thumb
 Deficient thumb extensor mechanism.
 Mild clasped (type I) – deficiency of EPB .
Extension lag at MCPJ.
 Severe clasped (type II) – deficiency of EPB & EPL
Extension lag at both MCPJ & IPJ.
 Type III – clasped associated with arthrogryposis

25. v) Kirner’s deformity
 Progressive palmar radial curvature of the
distal phalanx of little finger.(deviation in 2
planes)
 Distortion & widening of physeal plate along
with curvature of the diaphysis of the distal
phalanx.

26. vi) Arthrogryposis
 Non progressive multiple congenital joint contracture
 Elbow – most commonly with lack of flexion.
 Amyoplasia – classic type
symmetric limbs
shoulder- adducted ,
internally rotated
elbow –extension
forearm- pronation
wrist – flexion
hand – ulnar deviation
thumb – flexed, adducted
fingers - flexed

27. vii) Synostosis
 Union of two or more adjacent bones.
 Associated with other conditions.
 Symphalangism in Apert syndrome.
 Metacarpal, carpal,radio ulnar synostosis are rare.

28. 3. Duplication
A.Polydactyly
 Most common congenital anomaly in upper
extremity .
 Radial (preaxial)/central/ulnar(postaxial).
Radial polydactyly Ulnar polydactyly
1. Asians
2. Isolated
3. U/L
4. Wassel
classification
type I – VII
type IV – most
common(50%)
1. Africans
2. syndromic
3. B/L
4. Temtamy &
McKusick
Type A - well
formed
Type B rudimentary

30. 4.Overgrowth
A. Macrodactyly
• Rare
• Misleading term
• „Digital nerve oriented
neurofibroma‟ – correct
term
• Whole finger
clinodactyly if one digital
nerve involved.
• Syndactyly may
coexist.
• Flatt’s classification Type I –

31. 5. Undergrowth
A. Hypoplastic Thumb
Blauth Classification
 Type I – mild hypoplasia (all structures present)
 Type II – moderate hypoplasia (thenar muscles
absent)
 Type III –severe hypoplasia (skeletal hypoplasia)
A – stable CMC joint B – unstable CMC joint.
 Type IV – floating thumb (pouce flottant) [only soft
tissue bridge].
 Type V – aplasia
Additional 5 categories
 Type VI – central deficiency (cleft hand)
 Type VII – constriction ring syndrome
 Type VIII – five fingered hand
 Type IX – radial polydactyly
 Type X – syndromic short skeletal thumb ray

32. Blauth classification (thumb
hypoplasia)

33. B.Madelung’s deformity
 Radial & palmar angulation of distal radius
 Ulnar & palmar part of distal radial physis –growth
disturbance point.

34. 6. Constriction band syndrome
(streeter’s dysplasia)
 Quite common
 Etiology- constricting amniotic band &
intrinsic causes have been proposed
 Patterson clasificationa. simple constrictions (partial/circumferential)
b. Constrictions with distal deformity (lymphedema
+/-)
c. Constrictions with acrosyndactyly characeristic.
(Fenestrated syndactyly)
a. Intrauterine amputation

36. 7. Generalized skeletal
abnormalities
 Most common – multiple exostoses.
 Others –

Poland syndrome(symbrachydactyly)
 Apert syndrome(complex syndactyly)
 Haas syndrome
 Freeman Sheldon syndrome (wind blown
hand)
 Mohr Wriedt syndrome (radial clinodactyly of
index finger)
 Pierre – Robin syndrome (clasped thumb)

37. A. Apert
syndrome(acrocephalosyndactyly)
 Characterised by
1. Craniosynostosis .
2. Acrosyndactyly .
3. Symphalangism
(2nd , 3rd , 4th finger)
4. Radial clinodactyly of
thumb.
5. Simple syndactyly of 5th
finger (4th web)
 Upton classification –
Type I – Spade / obstetrician hand
(thumb & little fingers are free)
Type II – Mitten / spoon hand
(only thumb is free)
Type III – Rosebud / hoof hand
( all fused)

38. B. Haas syndrome
• Presence of 6 metacarpals is
characteristic
• >5 digits
• all having 3 phalanges

39. C. “Wind blown hand” in Freeman
sheldon syndrome
• Severe hyperflexion of fingers at
MCPJ with ulnar deviation .
• Due to metacarpal bone
shortening.
• “Whistling face” syndrome

40. Modified Classification

41. Modified classification of congenital
anomalies of hand & upper limb
I. Malformation
A.Failure in axis formation & differentiation – entire
upper limb
B.Failure in axis formation & differentiation – hand
plate
C.Failure in hand plate formation & differentiation –
II. Deformations –
unspecified axis Constriction ring syndrome
1.
III. Dysplasias
1.Macrodactyly
2.Limb hypertrophy
3.Tumorous conditions

42. A. Failure in axis formation & differentiation – entire
upper limb
Radio – ulnar axis
Dorso
Proximo – distal
ventral axis
axis
1.Symbrachydacty 1.Radial longitudinal 1.Nail
deficiency
ly
patella
2.Ulnar longitudinal
2.Transverse
syndrome
deficiency
deficiency
3.Intersegmental 3.Ulnar dimelia
4.Radio - ulnar
deficiency
synostosis
5.Humero - radial
synostosis

43. B. Failure in axis formation &
differentiation – hand plate
Radio-ulnar(AP)
axis
1.Radial polydactyly
2.Ulnar polydactyly
3.Triphalangeal
thumb
Dorsal vental axis
1.Dorsal dimelia
(palmar nail)
2.hypoplastic/aplasti
c nail

44. C. Failure in hand plate formation &
differentiation –unspecified axis
Soft
Skeletal
tissue
1.Syndacty 1.Brachydactyly
ly
2.Clinodactyly
2.Campto- 3.Kirner‟s
dactyly
deformity
3.Trigger 4.Metacarpal &
digits
carpal
synostoses
Complex
1.Cleft
hand
2.Synpolydactyly
3.Apert
hand

45. Symbrachydactyly
 Type 1- triphalangeal type
 Type 2 – diphalangeal type
 Type 3 - monophalangeal type
 Type 4 – aphalangeal type
 Type 5 – ametacarpia type
 Type 6 – acarpia type
 Type 7 – forearm amputation type

47. Types of symbrachydactyly
Short finger
peromely
atypical
cleft hand
monodactyly

48. Dorsal dimelia of little finger
 Failure in axis formation & differentiation in hand
plate
 Involves dorso ventral axis.
 Non ridge ectoderm – signaling centre.
 Palmar nail.

49. Triphalangeal thumb
 AD
 Extra phalanx of variable size, variable
shape(triangular/trapezoid/rectangular)
normal appearing thumb
 Fully developed extra phalanx lying in the finger
plane
- considered as five fingered hand
- absent thumb with index polydactyly

50. Brachydactyly
Bell’s classificationA. Brachymesophalangy
B. Apical dystrophy
C. Drinkwater type
D. Brachymegalodactyly
( stub thumb)
E. Brachymetacarpia
• Mohr – Wriedt syndrome –
Radial deviation (clinodactyly) of index
finger due to brachydactyly(middle
phalanx of index)
•

51. Bell‟s classification

52. Mirror hand / ulnar dimelia
 Rare
 Symmetric duplication of the limb in
midline
 A central digit with 3 digits (long, ring,
little) on either side
 Total 7 digits , but thumb is absent.
 2 ulna, no radius (ulnar dimelia)
 Due to transplatation/replication of ZPA

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