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Polydactyly

Christian Dumontier, MD, PhD
Centre de la Main, Guadeloupe, FWI

Most non-attributed figures belong to Green’s operative hand surgery
3 types of polydactylia
• Refers to the su-ituation where there are more than the
usual number of digits in a hand or foot. Broadly classified
in:

• Post-axial: Black people, inherited, autosomal dominant
pattern but a variable penetrance pattern, ≈ 1/3000.

• Central: Rare, Ring > long > index, isolation or part of a
syndrome

• Pre-axial: White and asiatic people, ≈ 1/7000
Associations

• A large proportion
is isolated

• May be associated
with
Post-axial
polydactyly
Post-axial polydactyly

• More common in Africans
and African Americans.

• Can be bilateral

• Prevalence in African
Americans estimated to be 1
in 143 live births (and 1 in
1339 live births in white)
12 year-old with bilateral polydactyly and
left long-ring finger syndactyly
Post-axial polydactyly

• Postaxial polydactyly in a
white person is often
indicative of an underlying
syndrome (e.g., chondro-
ectodermal dysplasia or Ellis-
van Creveld syndrome)
Classification of post-axial
polydactyly

• Type A: well developed
supernumerary digit

• Type B: rudimentary and
pedunculated

Treatment of rudimentary digit
(type B)
• Ligation at the base will lead to
necrosis and fall of the extra-digit

• Pro: easy to perform, no anesthesia

• Cons: may traumatized the patient,
leaves the child with a scar or a
nubbin (43% of cases-Watson).

• Of 105 ligated digits: unacceptable
cosmetic result/tender digit in 16%;
Bleeding 1%, infection 6%-Rayan
Rayan GM, Frey B. Ulnar polydactyly. Plast Reconstr Surg 2001;107(6):1449–54.
Watson BT, Hennrikus WL. Postaxial type-B polydactyly. Prevalence and treatment. J Bone Joint Surg Am 1997;79(1
65–8.
Treatment of Type A digit
• Excision of the extra-digit
through an elliptic incision

• Nerves and arteries are
coagulated

• Transfer of any important
parts (e.g., ulnar collateral
ligament and abductor digiti
quinti) to the adjacent finger.
Well-developed post-axial polydactyly-type A - From Little KJ. Orthop Clin
North Am 2016
Central polydactyly
Central polydactyly

• Rare

• Ring > Long > Index

• Isolation or part of a
syndrome (e.g, Grebes’
chondro-dysplasia)
Central polydactyly
• May be hidden within a
concomitant syndactyly
(i.e., synpolydactyly)

• Central polydactyly (ring
finger duplication)
combined with
syndactyly has a familial
inheritance pattern and
has been linked to a gene
mutation (HOXD13 gene)
on chromosome 2.
2 year-old girl with familial
synpolydactyly
Synpolydactyly (syndactyly + central polydactyly)
Central polydactyly
Central polydactyly with
syndactyly and post-axial
polydactyly - 7 fingers
(pre-op planning)
Finger motion
Treatment of central polydactyly
• Depends of finger status and concurrent anomalies

• Fully formed digit and normal function does not require removal

• Isolated central polydactyly with limited motion is treated by ray
resection. The span of the hand is maintained by transposition of
the adjacent digits and/or intermetacarpal ligament reconstruction

• Synpolydactyly is treated by separation of the syndactyly and
reduction of the concealed polydactyly through wide surgical
exposure (difficult surgery and unlikely to restore normal motion
and alignment)
Treatment of central polydactyly

• Partial central polydactyly is
treated with principles similar
to those used for
reconstruction of the
duplicated thumb

Partial index polydactyly with thumb duplication
Pre-axial polydactyly
Pre-axial polydactyly
• Mostly white and asiatic people

• Most cases are unilateral,
sporadic, and without systemic
problems

• Positive family history may be
present

• Classification according to
Wassel, type IV representing
almost 50% of cases (type II
15% second most common)
This classification had some
limitations
• Tri-phalangism can involve
the radial, the ulnar or both
split thumb

• The floating thumb is not
included in this classification

• The axis of the split thumbs
can be aligned, unbalanced,
convergent, divergent or both
Floating thumb. From Dautel G, 2008
Associated conditions

• Pollex abductus: abnormal
connection between EPL and
FPL
From LITTLE KJ. OCNA 2016
• ≈ 20% of hypoplastic and
duplicated thumb

• Suggested by abduction of
affected digit + absence of IP
joint crease

From TONKIN M. Indian J Plast Surg 2011
Type VII polydactyly

• Associated with several syndromes including

• Holt-Oram syndrome

• Fanconi’s anemia

• Blackfan-Diamond anemia

• Imperforate anus

• Cleft palate

• Tibial defects
Pre-op workup

• Nail widening ?

• MCP stability ?

• Which finger is the best ? None
are normal +++

• X-rays (beware cartilaginous
connection may be present)

• Surgery is delayed until 1 year
(development of thumb-index
finger pinch)

Subtle, type 1 thumb polydactyly, Wassel 1,
with only nail widening
Note that the radial distal phalanx has no epiphysis -
Clinical presentation of a Wassel type II deformity
different patient
Treatment Wassel 1 & 2

• If subtle, no treatment is an option
Treatment Wassel 1 & 2

• If asymmetric, ablation of the
smaller thumb, transfer of the
collateral ligament, and cen-
tralization of the extensor and
flexor tendons
From Bourke, 2011

Treatment Wassel 1 & 2
• If symmetric, a Bilhaut-
Cloquet intervention is
proposed (difficult to deal with
the nail and avoid stiffness)

• Some modifications have been
proposed to improve both
mobility and appearance
(Baeck)

• Preserve one nail if size > 70%
normal nail-Tonkin
Baek GH, Gong HS, Chung MS, et al. Modified Bilhaut-Cloquet procedure for Wassel type II and III polydactyly of the thumb. J
Bone Joint Surg Am 2007;89(3):534–41.
Tonkin MA, Bulstrode NW. The Bilhaut-Cloquet procedure for Wassel types III, IV and VII thumb duplication. J Hand Surg Eur
Vol 2007;32(6): 684–93.
Treatment Wassel 3 & 4

• Remove the smaller thumb
(usually the radial, maintain
the UCL of the thumb)

The ulnar thumb is the smallest (unusual) and has
been removed- From Bourke G. 2011
Treatment Wassel 3 & 4

• Abductor pollicis brevis inserts
into the radial thumb and must be
detached with an osteoperiosteal
sleeve along with the radial
collateral ligament and reattached
to the retained ulnar thumb with
periosteal suture to restore joint
stability and abductor function

• Soft tissues from the ablated
thumb are used to augment the
retained thumb (Dautel
technique)

Flap augmentation technique using an axial radial flap.
From Dautel G, 2008
6 month results of a Wassel type IV correction using a radial axial flap to enlarge the thumb
Treatment Wassel 3 & 4

• Inspect MP joint to look for a
facet that should be removed
Results Wassel IV
• Of 106 Wassel type IV thumbs with more than 3
years follow-up

• Results were fair or poor when the excised digit
was more than 75% of the size of the preserved
digit.

• Cartilaginous interconnections (without bony
connection) had a worse outcome.
Horii E, Nakamura R, Sakuma M, et al. Duplicated thumb bifurcation at the metacarpophalangeal
joint level: factors affecting surgical outcome. J Hand Surg [Am] 1997;22(4):671–9.
Results Wassel IV
• Of 139 Wassel type IV thumbs, 44 had a post
surgical Z deformity (>20° angulation at MP or IP
joint)- Lee

• Of 36 thumbs, 25 had joint instability and 10 a
deformity - Yen

• At > 10 years FU, 10 revision surgeries were
needed out of 43 thumbs - Stutz
Yen CH, Chan WL, Leung HB, et al. Thumb polydactyly: clinical outcome after reconstruction. J Orthop Surg (Hong Kong)
2006;14(3):295–302.

Lee CC, Park HY, Yoon JO, et al. Correction of Wassel type IV thumb duplication with zigzag deformity: results of a new method of
flexor pollicis longus tendon relocation. J Hand Surg Eur Vol 2013;38(3): 272–80.
Stutz C, Mills J, Wheeler L, et al. Long-term out- comes following radial polydactyly reconstruction. J Hand Surg Am 2014;39(8):
1549–52.
Treatment Wassel 5 & 6

• Same principles - usually the
radial thumb is rudimentary

• Intrinsic reconstruction.

• Z-plasty or dorsal flap may be
required to enlarge the 1st
web +++
Treatment Wassel 7

• Same principles

• Bony malalignment requires
osteotomy

• Tendons must be centralized
over the reconstructed thumb
Wassell Stage VII
• Two series recommended:

• A two-stage procedure. In the first stage, the
proximal interphalangeal joint is excised and the
metacarpal had corrective osteotomy. In the second
stage, an opposition transfer is performed-El Karef.

• Early (1–2 years of age) excision of the accessory
phalanx and collateral ligament repair or partial
epiphysis resection (rather than osteotomy)-Horii
El-Karef E. The non-opposable triphalangeal thumb: a new technique of management. J Hand Surg [Br] 2004;29(6):
544–51.
Horii E, Nakamura R, Makino H. Triphalangeal thumb without associated abnormalities: clinical characteristics and
surgical outcomes. Plast Reconstr Surg 2001;108(4):902–7.
Global results and complications
of thumb polydactyl
• Usually satisfactory in type I, II, and IV duplications.

• Less satisfactory with types III, V, and VI and triphalangeal
thumbs.

• The girth of the reconstructed thumb is 25% to 33% less than
the normal thumb.

• Angulation, joint instability, limited motion, MP joint
enlargement and scar contracture

• Reoperation rates between 20% and 25%
Results of pre-axial polydactyly

• If overall function is considered good, functional
assessment revealed 14/19 thumbs had only fair or
poor results

Larsen M, Nicolai JP. Long-term follow-up of surgical treatment for thumb duplication. J Hand
Surg [Br] 2005;30(3):276–81.