This are slides from the talk I gave at the 2019 Singapore Society for Hand Surgery Hand Review Course and Annual Scientific Meeting

1. Congenital Hand Conditions
Alphonsus Chong
Senior Consultant, Department of Hand and Reconstructive Microsurgery
National University Hospital
Associate Professor, Department of Orthopaedic Surgery
Yong Loo Lin School of Medicine, National University of Singapore
Link to slides: https://bit.ly/2Xl5KHl

2. Scope
• Embryology and Development of the Upper Limb
• Classification of Congenital Hand Conditions
– IFSSH
– OMT
– Condition specific classifications
• Polydactyly
• Syndactyly
• Radial Longitudinal deficiency and hypoplastic thumb
• Miscellaneous conditions
– Macrodactyly
– Trigger thumb
– Arthrogryposis & Camptodactyly
– Cleft hand
https://bit.ly/2Xl5KHl

3. Megan Fox:
Thumb brachydactyly
type D
Epidemiology
• 1-2% of children have congenital
anomalies
• 10% of these affect upper
extremity (1)
• Many don’t need surgical
treatment
Little Finger
Clinodactyly
Gemma Atherton:
Post-axial polydactyly
Amy Khor: Bilateral thumb
polydactyly

4. Abbreviated 1983 Swanson/International Federation of
Societies for Surgery of the Hand classification
Main Category Subcategory Diagnosis (Example)
I. Failure of formation (arrest) Transverse longitudinal Radial club
Cleft hand (typical/atypical)
Phocomelia
II. Failure of differentiation
(separation)
Soft tissue
Skeletal
Tumorous
Arthrogryposis
Cutaneous syndactyly
Camptodactyly
Radioulnar synostosis
Osseous syndactyly
Clinodactyly
III. Duplication — Mirror hand
Polydactyly
IV. Overgrowth (gigantism) — Hemihypertrophy
Macrodactyly
V. Undergrowth (hypoplasia) — Brachysyndactyly
Brachydactyly
VI. Constriction band
syndrome
Focal
Amputation
Constriction band
Acrosyndactyly
Intrauterine amputation
VII. Generalized — Achondroplasia
Marfan's syndrome

5. Oberg Manske Tonkin
(OMT) Classification
• Recommended by IFSSH as
classification of choice (2014)
• Hand/UE deformity perspective
– Most useful for comparing case mix between
places/ over time
• Complements other perspectives
• Main groupings (decreasing incidence)
– Malformations – Axes
– Deformation e.g. constriction ring, trigger
– Dysplasias e.g. macrodactyly, extra muscles
– Syndrome e.g. Apert’s

6. Polydactyly - Thumb
Duplication

7. Epidemiology
• ~ 10% bilateral (20/237 or 10.1%)*
• ~ 10% family history (19/237 or 8.0%)*
• ~ 10% associated anomalies (32 or* 13.5%)
* Tada K, JBJS, Duplication of the
thumb, 1983

8. 3/15/2008
Case 1
Level of
duplication Size of each
duplicate and
nail
Joint stability
Position of
thumb
Web space

9. Tada’s modification of Wassel’s classification
Tada K et al, JBJSA, 1983
Grouping
1. “Floating” VII
1. Narrow stalk
2. Asymmetrical
1. Broad based VII
2. Wassel II/III
3. “Typical” type IV
4. IV(D) Convergent type
5. Small symmetrical types
6. Other less common
types
Type IV
subclassification
(Hung 1996, CORR)
• IV(A) Hypoplastic
• IV(B) Ulnar
deviated
• IV(C) Divergent
• IV(D) Convergent
(Tada 1983, JBJS)

10. Treatment
• Accept deformity
–Often functionally fine
• Surgery
–Usually
Reconstruction
–Improve appearance
–Improve function
• Better position
• Less interference
• Stability
Dr Amy Khor,
Senior Minister
of State for
Health
Hrithik
Roshan

11. Timing of Surgery
• Besides the ligation procedure, all
others need general anesthesia
• Typically from 9 – 18 months of age
• Not too early
– Anesthetic risk
– Structures still small
• Not too late
– Develop abnormal patterns of use
– Patient becomes aware
– Deformity worse
– Surgery more difficult (bone work harder)

12. Treatment Groupings
VII Type with
stalk
Asymmetrical
(VII/II/III)
Type IV
“typical”
Type IV
“convergent”
“Small”
symmetrical
(II/III)
“Others”
Less common
Excision with +/-
reconstruction
Combination procedure (Bilhaut-
Cloquet type)
Suture ligation
Simple excision

13. Suture ligation for VII type with stalk
• Referred early
• Suture / liga-clip ligation
• Advantages
– “Solves problem”
– Outcome good
• Disadvantages
– “nipple” may need
excision

14. Broad Based VII /
asymmetrical II/III
• “Easiest”
• Excision with small local flap
• May need radial collateral
ligament reconstruction
• Large component of
aesthetics:
– Expectations are higher!!

15. Principal goals in reconstruction
“Make a thumb that is:”
• Single -- aesthetics
• Stable
• Straight
• Mobile
How about size?
• Perfect match not needed
• Too small a nail
• Angulation at IPJ bigger problem
Goldfarb 2006, JHSA

16. Typical Wassel IV:
Polydactyly
Reconstruction

17. Polydactyly
Reconstruction

18. Polydactyly
Reconstruction

19. Polydactyly
Reconstruction

21. Type IV variations
IV divergent type
IV deficient skin on radial side

22. Bilhaut Cloquet Operation

24. 6 months post Bilhaut Cloquet reconstruction

25. IVD type
1 year post-
operation

26. Choosing an operation
• Bilhaut-Cloquet is completely disliked by some
surgeons
– Better to have a smaller thumb ?
• Indications:
– Small symmetrical thumbs < 2/3 other side or smaller than
index
– Both IPJ and MPJ instability in relatively symmetrical
duplications

27. Nail issues
Outcomes Residual angular
deformity
Metacarpal head
prominence
Functional
issues

28. Summary
• “split thumb” NOT “doubling”
• “perfect” model of the contralateral normal thumb is
usually impossible
– Goal: reasonable size, which is aesthetically pleasing, and
functionally acceptable to the patient is usually achievable
• Thumb polydactyly is an interesting condition
– Surgical plans are individualized
– Surgeon and parents are usually very satisfied with results

29. Syndactyly

30. Epidemiology
• Very common – either in isolation or with other
anomalies
• 1:2000 live births (Kelikian 1974, Leung 1982)
• > 50% bilateral
• More in Caucasians
• AD in some families

31. Assessment
• , or brachydactyly).
Single digit, isolated
Multiple digit, isolated
Syndactyly in symbrachydactyly
4th web syndactyly with thumb
duplication and MF deformity

32. Classification
• Extent
–Complete
–Incomplete
• Type
–Simple (non-bony)
–Complex (bony fusion)
–Complicated
• With increasing complexity, abnormal
NV/tendons increase
• Special- acrosyndactyly

33. Syndactyly vs Acrosyndactyly
• Paddle shaped hand
• Apoptosis of interdigital space
cells
• Different mechanism
• Refusion of tips
• Often gap in the web is seen
Acrosyndactyly in Amniotic
band syndrome
(Langman's 9th edition 8.14) shows
scanning electron micrographs of a
human hand at 28 days (A), 51 days
(B), and 56 days (C).

34. The normal webspace

35. Principles of
Reconstruction
• Separate fingers with:
– Normally formed web
– Ideally in one surgery
• Avoid
– Function loss
– Scar contracture
– Web creep
Different techniques, but same
few ideas:
• Skin flap for commissure
reconstruction
• Digital incisions – zig zag
interdigitating flaps
• How to overcome shortage of
skin

36. One Technique
Braun, T. L., Trost, J. G., & Pederson, W. C. (2016). Syndactyly
Release. Seminars in plastic surgery, 30(4), 162-170.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5115922/

37. Syndactyly Separation

40. RADIAL CLUB HAND/
THUMB HYPOPLASIA

41. 41
Unoperated older child with RLD

42. Bayne and Klug Classification
II II
III IV

43. Treatment
• Correct any elbow stiffness
• Stretching of wrist
–Cast vs Ex-fix
• Repositioning the wrist
–Centralization – release, reposition, transfers
• Treatment of thumb and hand problems
• Contra-indications to surgery
–Multiple severe anomalies
• Remember to assess TAR, Holt-Oram, VACTERL
–Older patients with established patterns

45. Normal
Type I Type II Type IIIa
Type IIIb Type IV Type V
Modified Blauth
Classification of
Hypoplastic Thumb
(Buck-Gramcko and
Manske)

46. Normal
No treatment
Widen web, MPJ stabilization,
Opposition transfer
Pollicization
Treatment Options
in Hypoplastic
Thumb
Type I
Type II Type IIIa
Type IIIb Type IV Type V

47. Opposition Transfers
• Huber Transfer
MPJ Stabilization
Huber Transfer (ADQM)
FDS MF/RF Transfer

48. First web widening
Four-flap Z-plasty
Free Lateral arm flap

49. Pollicization
• Indication
• Principles
• Outcome
• Problems
Pollicization
Type
IIIb
Type
IV
Type
V

50. A miscellany of
congenital hand
conditions

51. Trigger Finger in Children
• Different entity from adult trigger
– Steroid injection not accepted
treatment
• Thumb much more common than
fingers
• In thumb usually no “trigger”
– Dynamic IPJ flexion contracture
– A1 pulley and FPL size mismatch
– Notta’s nodule
– Mistaken for
• Dislocation/ fracture
– May be bilateral (25%)
– Likely acquired rather than congenital
• Treatment
– Non-surgical
– Release of A1 pulley Baek JBJSA 2008

52. Distal arthrogryposis, Camptodactyly
and Clasped thumb
• Definition of
arthrogryposis
– Multiple congenital
contractures
– 2 or more different areas
• Distal arthrogryposis (AD)
– Camptodactyly
– Clasped thumb
– Overriding digits
– Hypoplastic fingers – absent
creases
– LL
Amyoplasia
Clasped thumb
Camptodactyly
https://wiki.nus.edu.sg/display/
HS/Camptodactyly

53. Macrodactyly
• Disproportionate enlargement of
part or whole of hand
• All tissues involved (bone, soft
tissue etc.)
• Presents at birth or soon after
• Rare
• Sporadic
• Differentiate “true” macrodactyly
from other causes of enlargment
See also:
https://wiki.nus.edu.sg/display/HS/Macrodactyly

54. Radioulnar Synostosis
• Rare
• Soft tissue or bony connection
• Isolated vs Associated anomalies
• More bilateral (60%)
• Mostly sporadic
• Position is important – extreme
supination/ pronation is bad
• Compensation by shoulder, and wrist
• Treatment
– No treatment (most cases)
– Corrective osteotomy ? Position
– Restoring motion – difficult – Kanaya
• Indication for surgery
– Bilateral
– Younger

55. Summary
• Big range of congenital hand conditions, a few are
much more common
• Variations in morphology also vary in many of the
conditions – treat patient first e.g. RLD
• For the exams: most of the questions are standard –
you should prepare for it
Resources
Link to slides: https://bit.ly/2Xl5KHl
Webpages: https://nus.edu/2SjDOjx
SMJ article – a gentle introduction:
http://www.smj.org.sg/article/common-congenital-hand-conditions

56. Thank You
Link to slides: https://bit.ly/2Xl5KHl
Webpages: https://nus.edu/2SjDOjx
SMJ article – a gentle introduction:
http://www.smj.org.sg/article/common-congenital-hand-conditions