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Surgical technique and Up-to-date

management
Common Congenital Hand
anomalies
Christian Dumontier, MD, PhD
FWI- Guadeloupe

Can be downloaded at: www.diuchirurgiemain.org


DECLARATION

I have no conflict of interest or


disclosure in relation to this presentation.

Professor Christian Dumontier, MD, PhD
The aim of surgery

• Provide the child with the best functional and


cosmetic outcome possible.

• Best functional outcome will provide the child with


normal grasp patterns for both single and bimanual
tasks.
Contraindications to surgery ?

• « Absolute » : if surgical
reconstruction will interfere with
function (ex: centralization
procedure in a patient with poor
elbow function or with a primary
postaxial pinch).
Contraindications to surgery ?
• Relative : if reconstruction will only
improve cosmesis and not alter
function (ex: separating functionless
or stiff digits),

• Relative: In adult patients who


function well with minor cosmetic
deformity.

• Relative: Cultural aspects should be considered. A


malformation considered intolerable in one culture
may be tolerable or even desirable in another.
How to handle patients with hand
anomalies (congenital differences)

• Correct diagnosis

• May need the help of a


pediatrician +/- genetician
Apert
• Knowledge of normal
embryology (see
www.diuchirurgiemain.org)

Referral to a specialized hand clinic where psychological, genetic and hand therapy input is available
and should be arranged as soon as possible after diagnosis.
How to handle patients with hand
anomalies (congenital differences)

• Treatment plan

• Evaluation of deficit (may be minor)

• Non-surgical treatment (needs parents’ cooperation)

• Timing of surgery if needed

• Psychological coping (both parents and patient)

Referral to a specialized hand clinic where psychological, genetic and hand therapy input is available
and should be arranged as soon as possible after diagnosis.
Correct diagnosis

• Isolated anomaly ?

• Associated anomalies ? Classification

• Syndromic disease ?
Classification

• IFSSH classification derives


from Swanson (1964)

• Very questionable
(descriptive, difficulty to
classify many presentations,
do not take into account the
genetical basis,…)
Descriptive classification is difficult owing to
our limited knowledge of the pathophysiology

• Bulsulfan in rats

• Evaluation of embryos at days 12


to 21

• Similar abnormal clefts in the


embryos accounted for the
development of central deficiency
(Type I), polydactyly (Type III),
and syndactyly (type II) - should
now be classified as a single
category- « abnormal induction of
digital rays «
➡duplication; ➤ syndactyly; * cleft
Naruse T et al. Busulfan-induced central polydactyly, syndactyly and cleft hand or foot: A common mechanism of disruption
leads to divergent phenotypes . Develop. Growth Differ. (2007) 49, 533–541
Descriptive classification is difficult owing to
our limited knowledge of the pathophysiology

• Radial longitudinal
deficiency (RLD)
represents a spectrum of
abnormalities from
thumb hypoplasia alone
to the complete absence
of the radius.

James MA, Green HD, McCarroll HR Jr, et al. The association of radial deficiency with thumb hypoplasia. J Bone Joint Surg Am
2004;86(10):2196–205.
James MA, McCarroll HR Jr, Manske PR. The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg
[Am] 1999;24(6): 1145–55.
Evaluation of deficit ?
• Most children do very well with their congenital
differences

• A stable, sensate and mobile thumb is essential for a


competent hand function.

• If the thumb is competent, then the focus can turn to the


other digits.

• Other upper limb anomalies should be noticed (elbow


function, …)
Timing of surgery ?
• Urgent: No, except for ring constriction syndrome with
severe distal oedema and neonatal Volkmann’s ischaemic
contracture.

• Very early: if it can be done under local anaesthesia in


neonates (ex: some extra digits, release of minor
acrosyndactyly (the distal joining of finger tips in ring
constriction syndrome).

• Early: (<1 year) is for the separation of syndactylized


border digits (the first web space).
Surgery means general anesthesia
• « …The actual risk of a fatal event under anesthesia (for an
otherwise healthy child) is about 1 in 300,000. To put that number
into perspective, the risk of death from an unexpected reaction to
penicillin is about 1 in 80,000. »

• « …Children exposed to general anesthesia before 4 years had 17%,


34%, and 23% increased odds of being developmentally high risk;
or scoring below the national minimum standard in numeracy and
reading, respectively. Although the risk for being developmentally
high risk and poor reading attenuated for children with only 1
hospitalization and exposure to general anesthesia, the association
with poor numeracy results remained… »

Schneuer FJ, Bentley JP, Davidson AJ, Holland AJ, Badawi N, Martin AJ, Skowno J, Lain SJ, Nassar N. The impact
of general anesthesia on child development and school performance: a population-based study. Paediatr Anaesth. 2018
Jun;28(6):528-536.
Timing of surgery ?
• As a rule, surgery is possible when he following criteria are
satisfied:

• The risk of general anaesthesia is minimal or as low as


possible given any other organ anomalies;

• Knowledge of the severity of other anomalies is apparent and


they have been treated where possible;

• Size of the hand structures is such that surgery is possible;

• Sufficient time has elapsed so that the benefit of splinting has


had time to be effective.

Timing of surgery ?
• In the majority of children surgery is performed
between the ages of 1 and 2 years.

• Surgery performed before school age makes recovery


easier and faster.

• May be postponed if co-operation and compliance are


essential.

• Some anomalies can be treated late (trigger thumb,


camptodactyly,…)
Psychological issue
• Even if many malformations lead to minor functional
deficits, they can pose a concern for the parents and
lead to psychological distress in children.

• The attitude of the child’s parents affects their long-


term outcome.

• Parents often have difficulty making decisions about


surgery on behalf of their new baby. This can lead to
parental disharmony and feelings of guilt.
Common hand anomalies ?
• Second to congenital heart disease,

• Congenital anomalies of the upper extremity are


noted in approximately 2-3 per 1000 live births.

• Incidence varies by country due to higher incidence


of certain malformations in patients of certain
ethnic backgrounds, such as polydactyly in those of
African descent or amniotic bands in Japanese.
Real incidence in population studies ?
Prevalence looks at
existing cases, while
incidence looks at new
• 5-year Edinburgh birth registry cases.

• Prevalence of babies born with any limb anomalies


to be 30 cases per 10,000 live births and the
incidence of upper limb anomalies to be 22.5 cases
per 10,000 live births.

• Of those with upper limb anomalies, 35% had


another non–upper limb anomaly.

Rogala EJ, Wynne-Davies R, Littlejohn A, Gormley J. Congenital limb anomalies: frequency and aetiological factors.
Data from the Edinburgh Register of the Newborn (1964-68). J Med Genet. 1974 Sep. 11(3):221-33
Real incidence in population studies ?
• An 11-year total population study of Western Australia

• Prevalence of babies born with upper limb anomalies was 19.76 cases
per 10,000 live births.

• Forty-six percent of those affected had another non-hand congenital


anomaly.

• Fifty-two percent had bilateral hand anomalies,

• 17% had multiple different hand anomalies.

• No significant differences in prevalence or frequency of anomalies


were found between whites and people of color, left and right sides,
and in babies who survived and those who died shortly after birth.

Giele H, Giele C, Bower C, Allison M. The incidence and epidemiology of congenital upper limb anomalies: a total
population study. J Hand Surg Am. 2001 Jul. 26(4):628-34
Real incidence in population studies ?
• An 11-year total population study of the Stockholm region of Sweden
found an incidence of congenital anomalies of the upper limb of 21.5
cases per 10,000 live births.

• 54 % were boys

• Right side only in 30%, the left side only in 33%, and both sides in
37%.

• Non-hand anomalies were recorded in 23% of the children with


congenital anomalies of the upper limb, most commonly in the lower
limbs.

• In 17% of the effected children, there was a known occurrence among


relatives.

Ekblom AG, Laurell T, Arner M. Epidemiology of congenital upper limb anomalies in 562 children born in 1997 to 2007:
a total population study from Stockholm, Sweden. J Hand Surg Am. 2010 Nov. 35(11):1742-54.
Real incidence in population studies ?
• 1993-2005 period

• 419 cases (234 male, 185 female) of upper limb deficiencies

• Incidence was 5.56 per 10,000 births and 5.25 per 10,000 live births.

• Most common: radial ray deficiency (138), undergrowth (91), upper limb defects due
to constriction band syndrome (51), central ray deficiency (41), and ulnar ray
deficiency (33).

• Additional birth defects were found in 60% of these children.

• Prevalence of upper limb defects in relatives of the census population was 2% (11 of
419).

• Perinatal mortality was 14%.

• Infant mortality among children with upper limb deficiencies was 137 per 1,000 live
births, compared with an overall infant mortality of 3.7 per 1,000 live births in Finland.

Koskimies E, Lindfors N, Gissler M, Peltonen J, Nietosvaara Y. Congenital upper limb deficiencies and associated
malformations in Finland: a population-based study. J Hand Surg Am. 2011; 36(6): 1058–1065.
Real incidence in population studies ?

• Midwestern USA : Of 480 extremities with a


malformation, 62% had anomalies of the hand plate
alone, with radial polydactyly (15%),
symbrachydactyly (13%), and cleft hand (11%)
being the most common of these.

• New York registry: 27,2 cases / 10,000 live births


(upper extremity). Polydactyly, syndactyly and
reduction defects were the most frequent
Goldfarb CA, Wall LB, Bohn DC, Moen P, Van Heest AE. Epidemiology of congenital upper limb anomalies in a midwest
United States population: an assessment using the Oberg, Manske, and Tonkin classification. J Hand Surg Am. 2015 Jan.
40 (1):127-32.e1-2.
Goldfarb CA, Shaw N, Steffen JA, Wall LB. The Prevalence of Congenital Hand and Upper Extremity Anomalies based
upon the New York Congenital Malformations Registry . J Pediatr Orthop. 2017 March ; 37(2): 144–148
As a summary

Incidence 5,5-30 / 10,000 births

Associated upper limb anomalies 17 %

Sex 54% ⚦

Bilateral 37-52 %

Relatives up to 17 %

Associated congenital anomalies 23- 46 %

The 1-year mortality of patients with hand malformations is 14% to 18% due to associated
malformations involving the heart, kidneys, or tracheoesophageal complex
Radial club hand
4,6 %
Brachydactyly Hypoplastic thumb
5,2 % 3,5 %
Clinodactyly Central defects
5,5 % Polydactyly
3,9 %
Amputation 14,6 %
6,8 %
Camptodactyly
6,9 %

Syndactyly
18,2 %
Other
30,8 %

% of live births with congenital defects


Some frequent congenital anomalies

• Polydactyly: The prevalence of postaxial polydactyly in


African Americans is estimated to be 1 in 143 live births.
In comparison, the prevalence of postaxial polydactyly in
white individuals is estimated to be 1 in 1339 live births.

• Syndactyly: 1/ 2000 live births

• Camptodactyly: < 1%

• Clinodactyly: from 1% to 19.5%


Polydactyly
Where there are more than the usual number of digits
in a hand or foot.
Classified in Post-axial; Central; Pre-axial.
Mostly isolated but may be part of a syndrome

A more complete presentation on polydactyly is available


at www.diuchirurgiemain.org
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.
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 with
syndactyly and post-axial
polydactyly - 7 fingers
(pre-op planning)
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
• 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 outcomes 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 polydactyly
• 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.
Syndactyly
Christian Dumontier, MD, PhD
Guadeloupe - FWI

A more complete presentation on syndactyly is available


at www.diuchirurgiemain.org
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
• Incidence of isolated syndactyly is 1
out of 2000.
Epidemiology
• 2.8 per 10 000. (Danish registry)

• 4 per 10,000 (England & wales)

• 50% of cases are bilaterally


symmetrical

• Caucasians > African Americans.


10-40% of patients have a positive
family history (autosomal dominant
trait with variable expressivity and
incomplete penetrance)

• 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
• Syndactyly is also seen with other Apert Syndrome
deformities such as polydactyly,
clinodactyly, brachydactyly,
symphalangism, and synostosis, 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.
Timing for surgery
• 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
(questionable in simple syndactyly)

• Release digits with significant length differences first to avoid


growth disturbances

• Bilateral hand releases

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

• Perform staged if child is >18 months (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


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