Syndactyly

Christian Dumontier, MD, PhD

Guadeloupe - FWI
 Syndactyly
• From the Greek (syn = united and
daktulos = finger)

• Congenital anomaly characterized

by variable fusion of the soft tissue,
skeletal, or both elements of
adjacent digits (or toes)

• Occurs when the normal processes

of digital separation and web space
formation fail to some degree.

• Represents about 20% of all hand

malformations
 Normal formation of digits
• The development of the hand
starts on day 27 of
intrauterine life. The fingers
are normally separated
between day 44 – 46.

• During normal development,

the digits form as
condensations of mesoderm
within the terminal paddle of
the embryonic upper limb.

SHH: Sonic Hedgehox; PFR: phalanx-forming region;

AER: Apical ectodermal ridge
 Normal formation of digits
• Spaces form between the fingers in a
distal to proximal direction to the level
of the normal web space by a process of
regulated apoptosis.

• This process is dependent on the apical

ectodermal ridge and the molecular
signaling of several cytokines, including
bone morphogenetic proteins,
transforming growth factor-β, fibroblast
growth factors, and retinoic acid

• Most syndactylies can be attributed to

errors in programmed cell death
(apoptosis).
 Epidemiology
• Isolated syndactyly is a common congenital
anomaly of the hand with an incidence of
approximately 1 in 2000.

• 2.8 per 10 000. (Danish registry)

• 4 per 10,000 (England & wales)

• 50% of cases are bilaterally symmetrical

• Caucasians > African Americans

• In isolated syndactyly, the long/ring finger

web space is most commonly affected (57%),
followed by the ring/small finger web space
(27%). Thumb/index finger and index/long
finger web syndactylies are the least common
 Epidemiology

• ~80% of cases are sporadic

• Between 10% and 40% of patients have a positive family

history that is inherited as an autosomal dominant trait.
Variable expressivity and incomplete penetrance account for
the male preponderance (2 : 1) and the variable phenotype
within a family pedigree.
 Epidemiology
Apert Syndrome
• Syndactyly is also seen with other deformities
such as polydactyly, clinodactyly,
brachydactyly, symphalangism, and synostosis
as part of a broader anomaly of the child’s
hand, either as a sporadic or in a syndromic
association (at least 28 described, more
frequent being Apert, Poland, amniotic band
syndrome, multiple craniofacial syndromes…)

• In syndromic cases, the thumb/index finger

and index/long finger web spaces are relatively
more frequently affected.

Constriction band Syndrome

Poland Syndrome
 Pathology
• Insufficient amount of skin

• Insufficient vascular supply

caused by an abnormal number
of digital arteries and digital
nerves.

• Excess fascial interconnections

• Bone anomalies : Joints may be

ankylosed, deviated, deformed,
tethered, stiff or unstable
 Classification

• Complete:

• Incomplete:

• Simple:

• Complex:

• Complex complicated:
 Classification

• Complete: if the web space extends

to include the fingertip

• Incomplete: when the web space

occurs anywhere between the
normal commissure and the
fingertips.
 Classification
• Simple: only skin or soft tissue connections.
Joints are usually normal, tendons are
independent , neurovascular bundles are
normal although the bifurcation may be
distal to normal.

• Complex: Skeletal anomalies (about 16.5 %

of the cases)

• Complex complicated: accessory phalanges

or digits.
 Preoperative evaluation
• Web space(s) involved

• Extent of the syndactyly

• Involvement of nail

• Other anomalies ?

• X-rays are mandatory (US or MRI in complicated

cases may help)
 Abstention ?

• For cultural reasons mainly

• Can lead to good function in

3/4 syndactyly
 Surgery is the treatment

• Recommended for most children

• The purpose of treatment is to enhance the

functional level and the cosmetic aspect.
 Contra-indications ?

• If it could reduce existing

functional level

• Medical conditions

• Mild, incomplete syndactyly

without functional limitations
is only a relative indication
for surgery.

Example of a contra-indication for surgery

(Green’s operative surgery)
 Timing for surgery
• Do all releases before school age

• Better results are seen if simple syndactyly are separated by 18 months of age (Flatt)

• Surgery on a larger hand reduces the risk of scar contracture and development of
web creep, where the webbing creeps distally as the hand grows.

• For syndactylies of the first and fourth interdigital

webs and syndactylies with fusions of several
fingers, surgery is recommended at the age of 4 – 9
months

• The ability to locate the hand in space and

perform grip and pinch manoeuvres are cortical
functions that develop before the age of one year.

• Length discrepancy between fingers increase the

risk of finger deviation
 Timing for surgery
• If multiple digits are involved perform procedure in two stages
(do 1 side of a finger at a time) to avoid compromising vasculature

• Release digits with significant length differences first to avoid

growth disturbances

• Bilateral hand releases

• Perform simultaneously if child is <18 mths (less active)

• Perform staged if child is >18 mths (more active, hard to

immobilize bilateral limbs simultaneously)
 Normal web anatomy
• The normal web space slopes 45
degrees in a dorsal to palmar direction
from the metacarpal heads to the mid-
proximal phalanx.

• The second and fourth webs are

wider than the third web, which From Ni, JHS 2015
allows greater abduction of the index
and small fingers.

• The first web space is a broader

diamond-shaped expanse of skin
composed of the glabrous skin of the
palm and thinner mobile skin dorsally.
 Two main features to remember
when performing surgery

• The incidence of tendon and neurovascular

abnormalities increases with the complexity of the
syndactyly

• The skin envelope of the fused digits is inadequate

to resurface the circumference of each independent
digit
 Commissure reconstruction

• Many designs have been

proposed

• A dorsal, proximally based,

rectangular flap

• Zig-zag incisions

Cronin T. Results of zigzag incision to prevent postoperative contracture. Plast Reconstr Surg. 1956;18(6):460-8.
 Incomplete
syndactyly

• Deepening of the existing

web can be done with various
Z-plasty type flaps

• Remenber than you will lack

some skin in the proximal
web whatever the method
used

Double opposing Z-plasty, from Green

From Deune EG, Medscape 2018
 Complete syndactyly
• Dorsal skin flap is about 2/3 of
the length of the proximal
phalanx

• Zig-zag incisions are fashioned

between the midlines of the
syndactylized digits
 Complete syndactyly

• The tip of the dorsal zig-zag

are opposite to the volar +++

D’Arcangelo M, Gilbert A, Pirello R. Correction of syndactyly using

a dorsal omega flap and two lateral and volar flaps. A long-term
review. J Hand Surg Am. 1996;21(3):320-4.
 Complete syndactyly

• Division or excision of the From Ni

fascial interconnections
between the digits

• Lateral spreading of the digits

facilitates digital separation From Green’s

• Fat defatting improves

closure
 Skin management
• Uncovered skin surfaces can
be managed

• By secondary healing
(increase the risk of
hypertrophic scar)

• Skin grafts (full-thickness

to lessen scar contracture)
usually from the groin

• Skin substitute
 Direct closure

• Ekerit
• Hsu et al.
• Many authors have published • Shaw et al.
• Sherriff
• Suppose some defatting of the • Tadiparthi et al
finger • Yao et al.
• Greuse and Coessens
• Sahin et al.
• Suppose skin tension • Teoh et Lee
• …
 Example of a flap designed to
match the hourglass shape of
the web

Ni F et al. The Use of an Hourglass Dorsal Advancement Flap Without Skin Graft for Congenital Syndactyly. J Hand
Surg Am. 2015;40(9):1748-1754
 Other technical difficulties
• Distal division of neurovascular bundles

• A interfascicular dissection and

proximal separation can be done

• One artery has to be sacrificed

provided that the other side of the
digit is not operated on or the
contralateral digital artery is known
to be intact. Otherwise, the web
release is limited to the level of the
arterial bifurcation,

• When multiple digits are released,

each digit must have at least one
digital artery remaining

From Green
 Other technical difficulties

• Nail reconstruction includes a nail fold

reconstruction

• Buck-Gramcko described the use of laterally

based, long narrow triangular flaps raised
from the hyponychium and folded around to
form the lateral nail fold

• Many techniques proposed as results are

usually not very satisfactory
Golash A, Watson J. Nail fold creation in complete syndactyly using Buck-Gramcko pulp flaps. J Hand Surg Br.
2000;25(B):11-4.
 Postop regimen
• Bulky dressing

• Use above-the-elbow plaster to avoid inadvertent

dressing removal

• Dressings are removed at 3 weeks

• No physiotherapy

• Scar management
 Outcomes
• Acceptable outcome with independent
digits that are freely mobile is usually
achieved with separation of simple
syndactyly.

• Need for secondary procedures is

reported in approximately 10 % of
cases

• Outcome for complex syndactyly is often

limited by associated anomalies and
severe skin shortage, increase scarring
and diminished mobility.
 Outcomes
• 116 simple complete syndactylies in 96 patients

• FU 2 to 11 years (average, 4.2 y).

• Average operation time was approximately 1.5 hours.

• No early complications

• 98 good, 16 fair and 2 patients according to the criterion

of D’Arcangelo.

Ni F et al. The Use of an Hourglass Dorsal Advancement Flap Without Skin Graft for Congenital Syndactyly. J Hand
Surg Am. 2015;40(9):1748-1754
D’Arcangelo M, Gilbert A, Pirrello R. Correction of syndactyly using a dorsal omega flap and two lateral and volar flaps:
a long-term re- view. J Hand Surg Br. 1996;21(3):320-324.
10 years FU in a patient operated at age 20
 Early Complications

• Vascular compromise,

• Infection,

• Wound dehiscence,

• Graft loss
 Early Complications
• 1656 cases, 30 days post, overall incidence of
complications (simple syndactyly, complex syndactyly,
and polydactyly) was 2.2% (McQuillan);

• Superficial surgical site infection (1.7%),

• Readmission (0.3%).

• Incidence was higher in patients undergoing complex

syndactyly repair (5.2% vs 2.3% for all others).
McQuillan TJ, Hawkins JE, Ladd AL. Incidence of Acute Complications Following Surgery for Syndactyly and
Polydactyly: An Analysis of the National Surgical Quality Improvement Program Database from 2012 to 2014. J Hand
Surg Am. 2017 Sep. 42 (9):749.
 Early Complications
• 2047 cases, postoperative complication rate was 1.9%
(Canisares),

• Postop infection rate of 1.6%

• Surgical complication rate of 0.3%

• Median adjusted standardized cost was $4112.50.

• Patients with more than one diagnosis had a 19 times higher

risk of complications than those with a single diagnosis of
syndactyly, and their hospital costs were 13% higher.

Canizares MF, Feldman L, Miller PE, Waters PM, Bae DS. Complications and Cost of Syndactyly Reconstruction in the
United States: Analysis of the Pediatric Health Information System. Hand (N Y). 2017 Jul. 12 (4):327-334.
 Late Complications

• Web creep = distal migration

of the web (8-60% cases)

• Joint contractures

• Unaesthetic nail
reconstruction

• Unaesthetic grafts
 Web Creep
• Most frequent complications

• Scar contraction

• Poor flap design

• Skin graft loss and healing by secondary

intention

• Discrepancy of growing between scar/skin

graft and surrounding tissue during the growth
spurt

• Usually increase with age

• Withey classification can be used

Withey SJ, Kangesu T, Carver N, Sommerlad BC. The open technique for the release of syndactyly. J Hand Surg Br.
2001;26(1) :4-7
 Skin graft problems

• Discoloration

• Hypertrophic scars

• Sometimes hairy at
adolescence
 Skin substitute

• Landi et al reported good

results using Hyalomatrix in
23 children with 24 months
FU

Landi A, Garagnani L, Leti Acciaro A, Lando M, Ozben H, Gagliano M. Hyaluronic acid scaffold for skin defects in
congenital syndactyly release surgery: a novel technique based on the regenerative model. J Hand Surg Eur 2014;39(9):
994-1000