POLYDACTYLY

Embryology of Upper
Limb
• The upper and lower limbs begin
development in the 4th week of gestation.
• The limb buds are the precursor structures
of the limbs. Their formation begins in the
4th week.
• The limb buds first appear on the ventrolateral
body wall initially and extend ventrally.
• They consist of a central core of
undifferentiated mesenchyme tipped with a
layer of ectoderm - the apical ectodermal
ridge (AER).
• Elongation occurs through proliferation of the
underlying mesenchyme core
1. AER – Apical Ectodermal ridge
• Acts as signaling center to guide underlying mesoderm to differentiate into
appropriate structure
• It is essential for proximo-distal limb development
• It is responsible for interdigital necrosis ,which separates the webbed
hand.
2. ZPA - The zone of polarizing activity
• Signaling center for antero-posterior( Radio Ulnar) limb development
• Responsible for limb orientation
3. Dorsal Ectoderm – produce morphogenic protein controlled by
wingless-type (Wnt) signaling center which is responsible for
dorsalization of upper limb.(Dorso-ventral axis)
• All 3 function in coordination to ensure proper limb
8 week after fertilization,
embryogenesis is complete &
all limb structures are present.
– majority of congenital
anomaly occurs during this
period.

Primary Ossification center are

present in all long bones by
12th week
Timeline :

Onset of development of arm bud :27 days 4th week

Well developed arm bud: 28-30 days

Elongation of arm bud: 34-36 days 5th week

Formation of hand paddle: 34-38 days

Onset of finger separation 38-40 days

full separation of fingers : 50-52 days

7th week
• Congenital anomalies affect 6 per 10000 of newborns

• Out of which 75% have Upper extremity abnormalities.

Classification of Congenital Anomalies of
UL
• International federation of societies for surgery of the hand (IFSSH-1983)
1) FAILURE OF FORMATION OF PARTS:(Arrest of development)
a) transverse arrest : common levels are upper third of Forearm, wrist, metacarpal, phalangeal
b) Longitudinal arrest : phocomelia , club hand, cleft hand
2) FAILURE OF DIFFERENTIATION OF PARTS:
a) Soft tissue involvement
b) Skeletal involvement
Ex. radio ulnar synostosis ,camptodactyly ,arthrogryposis
3) DUPLICATION: polydactyly, mirror hand
4) OVERGROWTH: macrodactyly
5) UNDERGROWTH: thumb hypoplasia, madelungs deformity
6) CONGENITAL CONSTRICTION BAND SYNDROME
7) GENERALIZED ABNORMALITIES AND SYNDROME
Classification of Congenital Anomalies of
UL
• International federation of societies for surgery of the hand (IFSSH-1983)
1) FAILURE OF FORMATION OF PARTS:(Arrest of development)
a) transverse arrest : common levels are upper third of Forearm, wrist, metacarpal, phalangeal
b) Longitudinal arrest : phocomelia , club hand, cleft hand
Classification of Congenital Anomalies of
UL
• International federation of societies for surgery of the hand (IFSSH-1983)
2) FAILURE OF DIFFERENTIATION OF PARTS:
a) Soft tissue involvement
b) Skeletal involvement
Ex. radio ulnar synostosis ,camptodactyly ,arthrogryposis
Classification of Congenital Anomalies of
UL
• International federation of societies for surgery of the hand (IFSSH-1983)
3) DUPLICATION: polydactyly, mirror hand
4) OVERGROWTH: macrodactyly
Classification of Congenital Anomalies of
UL
• International federation of societies for surgery of the hand (IFSSH-1983)
5) UNDERGROWTH: thumb hypoplasia, madelung’s deformity
6) CONGENITAL CONSTRICTION BAND SYNDROME
7) GENERALIZED ABNORMALITIES AND SYNDROME
Polydactyly - Definition
Polydactyly (poly = more; dactylos = finger) refers to the
disorder in which patients have more than five digits on at
least one extremity
History
Polydactylous hand and footprints have been found when studying rock art and
petroglyphs, some dated 1000 AD.

Polydactyly was first time referred in literature - in the Old Testament, (a battle in
Gath) a giant had six fingers on each hand, and six toes on each foot.

The term polydactyly is first described by Kerckring in 1670.

 Genetics
Inherited isolated dominant radial polydactyly and triphalangeal thumb -
chromosome 7q36 – regulated by SHH protein.

Isolated ulnar polydactyly an autosomal-dominant inheritance pattern with variable

expression.

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.
Together with Syndactyly and Camptodactyly, it is the most common congenital
upper extremity difference.
The incidence can range from 2% to 30% when considering all types of
Polydactylies.
Polydactyly can occur in combinations / involve both hands (and / or) feet,
Can be associated with syndactyly.
Over 97 genetic syndromes have been associated with polydactyly.
Polydactyl
y
• Duplication of fingers
• It is of 3 types
• Pre axial > duplication of thumb more common in whites.
• Central > duplication of index, middle and ring fingers
• Post axial > duplication of small finger - most common in African
Ulnar polydactyly is 10 times more common in – African - Americans and 2 times
more common in males.
Ulnar polydactyly is mostly bilateral and can be associated with syndactyly and
polydactyly of the feet.
Syndromic association is more common in ulnar polydactyly.

Radial polydactyly is more common in Asians.

Radial polydactyly is mostly unilateral.

Central polydactyly is extremely rare in all published studies.

PREAXIAL POLYDACTYLY : THUMB
DUPLICATION (BIFID THUMB)
Epidemiology
• Most common duplication pattern in Whites and Asians
• 1 in 3,000 births
• Usually unilateral. Cause unknown.
• Sporadic, with systemic problems
Classificatio
n
Wassel classification (most commonly used) – Based on Radiology

Adapted from Wassel HD: The results of surgery for polydactyly of the thumb: a review,
Clin Orthop Relat Res 125:175–193, 1969.
Classificatio
nWassel classification
• Type I : Partial duplication of The distal
phalanx and a common epiphysis
• Type II: Complete duplication including
epiphysis of the distal phalanx.
• Type III : Duplication of distal phalanx and
bifurcation of proximal phalanx.
• Type IV: Complete duplication of distal and
proximal phalanx
Classificatio
nassel classification
W
• Type V : Complete duplication of distal and
proximal phalanx with bifurcation of the
metacarpal
• Type VI : Complete duplication of distal and
proximal phalanx and metacarpal.
• Type VII : Variable degree of duplication
with triphalangeal thumb

Type IV is most common (50%).

Type VII (20%).
Type II (12%).
Clinical Assesment
Detailed Medical history and Family history.
General physical examination – If the index to little finger is normal, with normal
hand and finger creases, and a normal hypothenar region, the examination is
concentrated on the radial side of the hand.
Examination is performed from proximal to distal.
Start with thenar musculature and con
Wassel I and II, the thenar musculature is mostly normal in contrast to wassel type
V, VI, and VII
Hypermobility of involved joints vs Generalized ligamentous laxity.
Initial examination includes
1. Observation of thumb usage,
2. Palpation of the bony elements,
3. Assessment of joint stability.
4. Passive motion,
5. Varus/Valgus stress testing of the joints,
6. Palpation of tendons (especially flexor
tendons),
7. Observation of the skin crease
Look for creases both dorsally and palmarly. If creases are present, then an active
movement in that particular joint can be expected.
In most of these cases, the radial-sided thumb is hypoplastic and stiff, and the ulnar
thumb is the better one.
Little or absent passive motion at the bifurcation site of the minor thumb (most
radial one) suggests an odd-numbered Wassel type I, III, or V.
One of the duplicated elements is usually dominant and preferred for prehension.
If polydactyly is situated at the CMCJ, the MCPJ in the best thumb can be near
normal.
Several investigators have noted that the thumb is not truly duplicated. Neither
component is as robust as a normal thumb. Each thumb is thinner than normal and
has diminished mobility. The term split thumb seems more appropriate.
Range of motion is typically less than in a normal IPJ. In an asymmetric
duplication at the IPJ, the best-developed part usually moves better.
In a newborn - it is often difficult to distinguish between intrinsic and extrinsic
movements.
In duplicated thumbs, the flexor tendon and extensor apparatus is usually less
developed or absent in the more hypoplastic thumb.
The split parts may share bony elements, tendons, ligaments, joints,
neurovascular structures, and nails.
The nails are smaller and asymmetrical in most cases.
Wassel type 1 & 2 – Widening of nail plate.
The first web is nearly always normal in the distal duplications.
In more proximal polydactylies, the first web can be narrower than
the normal contralateral side.
• A Typically there is some degree of hypoplasia of
both duplicates and commonly radial duplicate is
more Hypoplastic.

• There may be convergence or divergence of

duplication

• Ulnar innervated intrinsic muscles to the thumb

(adductor policies, deep head of flexor polices
brevis), typically insert on the ulnar most thumb
duplicate
• Median innervated intrinsic muscles to the thumb (abductor policies, superficial
higad of the flexor pollicis brevis and opponens pollicis) insert on the radial most
thumb duplicate

• Extrinsic flexor and extrinsic tendons may be duplicated and usually are
eccentric.
• Collateral ligaments of the duplicated joints are shared

• Both radial and ulnar neurovascular bundle to the digits may be completely
duplicated or may be shared with small separate branches that supply the
individual digits.
Concerns

• Cosmetic deformity – concern to both parents and child.

• Functional impairment - depending on the level of the duplication

(shaking hands, putting hands in pockets or narrow spaces, and
wearing gloves).
PRE-AXIAL Treatment - goals
• Goals Of Treatment
• To construct a thumb that is 80% of the size of the contralateral thumb
• Resect smaller thumb (usually radial)

• Preserve / reconstruct medial collateral structures in order to preserve pinch

function
• Reconstruction of all components typically done in one procedure

• Timing of surgery 12 -18 months (prior to devolopment of Thumb

index finger pinch)
Surgical exploration may reveal a cartilaginous connection that is not visualized by x-ray.
This operative finding reclassifies a type II thumb to a type I and a type IV thumb to a type
III.
The surgeon needs to be aware of this possible surgical finding, which may alter the
operative plan.
The ideal reconstruction - aligns the thumb along a longitudinal axis, stabilizes the
joints, balances the motor functions, provides an adequate nail plate without deformity,
and restores sufficient thumb size.
Reconstruction or preservation of the collateral ligaments is critical for duplications that
arise from the joint surface (Wassel II, IV, and VI).
CORRECTION OF TYPES I AND II BIFID
THUMBS
• Common nail

• Asymmetric type I or II duplication with distinct components is treated by

ablation of the smaller thumb, with transfer of the collateral ligament and
centralization of the extensor tendon.

• The collateral ligament from the deleted component is elevated with an

osteoperiosteal sleeve and attached via periosteal suture to the reconstructed
thumb.

• A separate facet for the deleted component is removed with an osteotome,

rongeur, or bone-biter to narrow the articular surface and properly align the joint
with the retained component.
TYPES II
 CORRECTION OF TYPES I AND II BIFID
THUMBS
• Symmetric (Wassel) 1 & 2 -Bilhaut-Cloquet Technique

• technique

• involves removing central tissue and combining both

digits into one
• outcomes
• approximately 20% have late deformity

• problems include stiffness, angular and size

deformity, growth arrest, and nail deformities
 CORRECTION OF TYPES I AND II BIFID
•THUMBS
Bilhaut-Cloquet Technique
Modified Bilhaut – cloquet procedure (Baek and collegues)
 CORRECTION OF TYPES III To VI BIFID
THUMBS
• LAMB, MARKS, AND BAYNE

• Indications
• usually favored approach for type III and IV

• type V and VI usually require more complex

transfer of intrinsics and collateral ligaments
• Technique

• preserve skeleton and nail of one component

and augment with soft tissue from other digit
and ablation of lesser digit (radial digit most
commonly)
 CORRECTION OF TYPES III
 In a type III thumb, the entire physis and collateral ligament maybe preserved to
maintain motion, prevent any chances of MCP joint instability, and avoid damage to the
physis .
The osteotomy is designed to preserve the entire proximal phalanx base, and an
osteoperiosteal sleeve is not raised.
 CORRECTION OF TYPES III To VI BIFID
THUMBS
Extraarticular malalignment may require corrective osteotomy to ensure longitudinal
alignment of the thumb (closing wedge osteotomy) to align the joint surfaces in a
parallel manner.
Tendon realignment is also necessary to centralize the tendons along the retained
thumb.
Any thenar intrinsic muscles that are attached to the deleted thumb are transferred
to the retained thumb.
Typically, the abductor pollicis brevis inserts into the radial thumb and must be
detached with an osteoperiosteal sleeve along with the radial collateral ligament
TYPES IV
TYPES IV
TYPES IV
TYPES IV
Type 4
CORRECTION OF TYPE V & VI BIFID
THUMBS
•Treatment of type V or VI duplication uses similar principles, with
the added complexity of additional intrinsic reconstruction.
•In addition, the thumb/index finger web space may be narrowed. A
concomitant “Z”-plasty or dorsal advancement transposition flap
may be required.
 CORRECTION OF TYPE VII BIFID
THUMBS
• In a Wassell type VII duplication (complete duplication), the radial thumb
should be removed. The remaining thumb may require web
reconstruction and metacarpal osteotomy to complete the pollicization.
• If the retained component is triphalangeal - A joint reduction procedure
with chondrodesis across the joint with the least motion will reduce the
number of joints.
• This procedure can be done at the time of thumb reconstruction or as a
second-stage procedure.
Triphalangeal Thumb with Radial polydactyly
Postoperative care
•The a long-arm thumb spica cast applied. The cast and Kirschner wire are removed
4 to 5 weeks after surgery.
•A shortarm thumb spica splint is fabricated, which is removed for therapy and
gentle activities.
•The splint is discontinued 8 to 12 weeks after surgery.
Complications
Small Reconstructed Thumb,
Angulation,
Joint Instability,
Limited Motion,
Scar Contracture
Z- deformity of thumb
Outcomes
The results vary with the degree and complexity of the thumb duplication.
 Satisfactory results are readily obtainable in type I, II, and IV duplications.
Less satisfactory results are prevalent in types III, V, and VI and in triphalangeal
thumbs.
Nail deformities and IPJ stiffness are less common with Modified Bilhaut-cloquet
method.
 Secondary Procedures
Possible secondary procedures, either or not in combination, can include:
■ Ligament reconstruction
■ Tenolysis
■ Tendon rebalancing
■ Opposition transfers (sometimes in the more proximal Wassel types)
■ Nail deformity correction
■ Osteotomy and alignment of articular surfaces
■ Neurolysis and neuroma treatment in adult patients
■ Arthrodesis of IPJ or MCPJ
■ Scar revision.
POSTAXIAL (ULNAR)
POLYDACTYLY:
Autosomal Dominant – variable penetrance pattern

• Duplication of the small finger

• It is the most common pattern of duplication in the black population

• sometimes indicative of an underlying syndrome (e.g.,

chondroectodermal dysplasia or Ellis–van Creveld syndrome)
 Classification
Two-type classification - Temtamy and McKusick.

Type A (Well devoloped) comprises an extra little finger at the MCPJ, or more
proximal including the CMCJ. The little finger can be hypoplastic or fully developed.

Type B (Rudimentary / Pedunculated) varies from a nubbin to an extra, non-

functional little finger part on a pedicle.
B . Three types classification
• Type-1: Duplication of soft parts only

• Type -2: Partial duplication of the digit, including the osseous

structures
• Type-3: Duplication of the ray including the metacarpal
The most common presentation is a small appendix on a skin pedicle with intact
neurovascular bundle, attached at the ulnar border of the proximal phalanx, has
mostly a small nail and thus a distal phalanx, and is non-functional.
Sometimes two bones are present. If the extra little finger is more developed, the
more common site of duplication is at the MCPJ.
In these cases, the fifth metacarpal is broad. Depending on the extent of
development and the attachment at the MCPJ, the extra little finger flexes and extends
well.
If the extra finger originates at the MCPJ, full flexion at the MCPJ might not be
possible as the joint is not normally developed.
Full motion of the MCPJ of the normal fifth finger can be affected.

Flexor tendons and extensor tendons are usually Y-shaped and asymmetrical, as in the
duplicated thumb.

The extra finger is usually abducted at the MCPJ and radially deviated at the PIPJ.

The hypothenar muscles are attached at the ulnar side of the extra digit and palmarly at
the broad MCPJ.
If the extra little finger is at the base of the metacarpal or at the CMC, the joint of the
finger is usually well developed.
Physical examination of ulnar polydactyly depends on the development of the extra little
finger.
POST-AXIAL Treatment - Surgical
TYPE 1 : tie off in nursery or amputate before 1 year of age
TYPE 2 & 3
• operative

Excision of the extra digit

• technique
• preserve radial digit
• preserve or reconstruct collateral ligaments from ulnar digit
remnant
Outcomes
•In ulnar polydactyly, a small painful mass can remain following ligation or
inadequate resection of the pedicle of the extra floating little finger.
CENTRAL
POLYDACTYLY
Usually associated with syndactyly.

In central polydactyly, the second, third and/or fourth digit can be involved.

Fully developed extra independent fingers are rare.

In order of appearance, the most frequently affected is the ring finger, followed
by the long finger, and ultimately the index finger.

Frequently, the duplication of the fourth finger is partial and hidden by a

syndactyly, typically to the third finger.
A number of varieties are possible because the duplication often is not confined
to one finger.

In addition to the bony deformity and aberrant growth plates, anomalies of the
flexor and extensor tendons and neurovascular structures.
CENTRAL POLYDACTYLY
CENTRAL POLYDACTYLY
CENTRAL
POLYDACTYLY
• Treatment –
• Don’t treat - if digit is fully formed with full motion and function.
• An isolated central polydactyly with limited motion is treated by resection of
the ray.
• Synpolydactyly – Seperation of syndactyly and reduction of concealed
polydactyly (Combination of Resection and osteotomy + ligament
reconstruction )
• Perform early (1 yr ) to prevent angular growth deformities and improve
motion
CENTRAL
POLYDACTYLY
• Treatment –
•Central synpolydactyly with complicated connections may better be left untreated
than separated into individual digits with limited motion and instability.
Type VII Wassel - Type 1 Mirror Hand (Ulnar Dimelia)
Thank
you