Management of Disorders of

Separation Syndactyly
 For the pediatric hand surgeon  one of the two most common congenital differences

• Although interdigital webbing usually presents as

an isolated anomaly
 it is also frequently seen in association with other soft tissue and
skeletal anomalies of the hand and malformations of other organ systems
as part of a syndrome

• The main focus of the following discussion is :

 diagnosis
 treatment
CLASSIFICATION

1. LEVEL
• Complete
• Incomplete

2. SOFT TISSUE AND SKELETAL ANATOMY

• Simple
• Complex
3. COMPLEXITY OF SKELETAL STRUCTURES
• A complex syndactyly contains abnormal osseous or cartilaginous skeletal
unions between adjacent fingers plus or minus the thumb.

4. THUMB-INDEX WEB SPACE (FIRST INTERDIGITAL WEB SPACE)

• There are no specific practical classifications for this unique web space
 INCIDENCE
• Varies from one part of the world to another
• Type of interdigital webbing is 1 of every 2000 live births.
• Half of the patients have bilateral defects, and boys are more often affected.
• Familial syndactyly is seen in 15% to 40% of most reported series.
• All different types of genetic inheritance patterns have been documented with syndactyly.
• In families, the dominant genes show a reduced penetrance and variable expression
• The third (long-ring) interdigital web space in the hand and the second interdigital web space
in the foot are the most commonly affected areas.
• The highest degree of penetrance is seen within those families with synpolydactyly
• The first (thumb-index) interdigital web space in the hand is the least frequently affected
• The incidence of ray involvement :
– first interdigital web space, 5%
– second interdigital web space, 15%
– third interdigital web space, 50%
– fourth interdigital web space, 30%.
 ETIOLGY
“Because of the varied presentations of interdigital webbing
with/without osseous Coalitions or other abnormalities, there are no
well-accepted causes of syndactyly formation”

 Between the fifth and sixth weeks of gestation, interactions between the apical ectodermal ridge and
the underlying mesenchymal tissue within the hand plate result in a distal to proximal cleft formation
between each of the rays of the hand.

 By a process of "programmed cell death" or apoptosis, the soft tissue between the longitudinal
cartilaginous rays of the hand disappears as separate digits and a thumb appear.

 In the constriction ring syndrome, the distal tissues of the digit are often rejoined by the constricting
amniotic bands encircling them.

 This results in an inflammatory reaction, and the subsequent common scar often binds adjacent digits
together.

 Just proximal to this common scar are dorsal to palmar epithelium-lined sinuses, which represent the
presence of a commissure that had formed or was forming at the time of the band formation.
 SYNDROMIC ASSOCIATIONS

Syndactyly is part of at least 28 syndromes

Poland

Multiple Most
facial Apert
syndromes common

constriction
ring
 1. POLAND SYNDROME

CLINICAL PRESENTATION

• “Poland syndrome is a common syndactyly-related syndrome”

• It typically consists of the :

 absence of the sternocostal portion of the pectoralis major muscles
 hypoplasia of the hand and, to a lesser degree, of the forearm & upper arm
 simple complete or incomplete syndactyly
 short fingers (brachysymphalangism)

• The cause of the condition is unknown.  there also are no commonly

associated anomalies.

• Because these limb anomalies can be seen with some associated head and neck malformations,
some researchers believe they fall along a disruption sequence involving the brachiocephalic
arterial system.

• Disruption sequences in the distal portion of this system would present as the Poland syndrome.

• On occasion, portions of several syndromes may coexist.

CLINICAL PRESENTATION (...)

• Children with the Poland syndrome present with a wide variety of hand malformations.

• In the most severe form of Poland syndrome, hypoplastic thumb and fifth fingers are present, and
the remaining digits are represented by hypoplastic nubbins.

• The most common presentation involves the absence of the sternocostal portion of the muscle
with or without all degrees of breast hypoplasia.

• Associated chest wall, skeletal, and soft tissue deformities often require treatment, particularly in
the adolescent girl with a hypoplastic breast

• Although respiratory function is rarely compromised, some of these children may have severe
pectus carinatum or pectus excavatum sternal deformities coupled with multiple rib hypoplasias or
aplasias.
 2. APERT SYNDROME

CLINICAL PRESENTATION

• The Apert syndrome, which affects all races, is the most common of six
acrocephalosyndactyly syndromes seen by the hand surgeon.

• It has an autosomal dominant inheritance pattern and an incidence higher

than 1 in 100,000 live births.

• Geneticists have identified and confirmed two chromosome loci in these

children.

• The author has observed that clinically, the more frequent serine substitution
at the FGFR-2 receptor represents the "good hands and bad head“
malformation; the less frequently observed proline substitution is more
indicative of the "bad hands (type III) and good head" combination.
CLINICAL PRESENTATION (...)
• Symmetric complex and complicated syndactylies of both hands
and feet are present.

• There is usually skeletal dysplasia of the glenohumeral joint and

occasionally of the elbow joint.

• Three basic types of hand patterns are seen:

1. The flat, spade-like hand;
2. The constricted, cupped, mitten hand;
3. The tightly coalesced "hoof" or "rosebud" hand.

• Common to all are a short, radially deviated thumb with an

abnormal, delta-shaped proximal phalanx; a complex side-to-side
fusion involving phalanges of the index, long, and ring fingers;
symphalangism within the four digits; and a simple syndactyly
involving the fourth web space.

• Fewer than half of these patients have syndactyly of the first web
space.
 3. ACROSYNDACTYLY

CLINICAL PRESENTATION

• Acrosyndactyly denotes fused digits and is generally considered part of the

 constriction ring syndrome

• There is always a proximal dorsal to palmar epithelium-lined space or sinus

• some type of disruption sequence initiated by an ischemic insult occurs after the digits have
been separated and that the resulting scar tissue draws adjacent soft tissue and bone
together.

• Associated anomalies, including facial clefts, congenital heart defects, and clubbed feet.

• Frequently, upper and lower limbs are involved.

• It is possible that a combination of malformations, disruption, and deformation sequences

may be involved in this syndrome
CLINICAL PRESENTATION (...)

Walsh and others categorized them as :

 mild
 moderate
 severe
depending on skeletal structure alone developed

• Incomplete simple syndactylies may be a component in all three varieties.

• The two hands in any given patient never have identical mirror image
deformities, commonly seen in congenital differences of genetic origin.
CLINICAL PRESENTATION (...)

• The metacarpals of all five rays are present, and most

of the digits contain two or more phalanges and intact
proximal interphalangeal and distal interphalangeal
joints.
• The syndactyly is usually incomplete.
• The coalesced soft tissue and skeletal parts are joined
by scar tissue.

• The proximal phalanges are all present with small but

present middle phalanges and at least one intact
proximal interphalangeal joint.
• Dorsal to palmar epithelialized sinuses are often
present adjacent to these phalangeal stumps.

• No normal phalanges are present, and metacarpals

may often be deficient.
• The digits are often functionless nubbins with or
without proximal phalangeal remnants
.
4. OTHER SYNDROMIC

The hand of a child with either arthrogryposis or the Freeman

Sheldon syndrome (whistling face syndrome) will often have
simple incomplete interdigital webbing combined with ulnar
drift of all digits and a tight adducted thumb with a deficient
first web space.

- Those patients with two-digit hands often present with a

complete syndactyly.
- The conjoined nail here with longitudinal ridges indicates that
there are at least three distal phalangeal components.
- Two are part of a distal thumb polydactyly hidden within the
syndactyly.
- Simple, simple complete, or complex syndactylies
are often seen in the digits of the typical cleft hand.
- The digits involved are usually adjacent to the
central cleft.

This three-digit hand contains an incomplete

syndactyly between the thumb and index rays and a
complete webbing between the index and long
digits.
ABNORMAL
ABN ANATOMY

• Almost all permutations and combinations of abnormal

soft tissue and skeletal anatomy can be present in
syndactyly.

• A determination of abnormal can be made only after

consideration of normal interdigital relationships

• Often, the radiographic appearance of the skeletal

structures and degree of hypoplasia are good
indicators of associated tendon, nerve, arterial, or
ligament abnormalities.
 • Skin is invariably deficient, especially in the region of the normal commissure.
.
• the circumference of two digits held together and comparing it with the

SKIN sum of the circumferences of the individual digits.

• Although infrequently webbed, the thumb-index web space is usually
deficient in the hypoplastic hand, where webbing of the digits is
commonly present.

•Bones and joints may present bizarre and erratic configurations. On occasion,

BONES AND alignment of skeletal elements into digital rays is impossible.

•Joints in patients with syndactyly may be incompletely developed, angulated,
ankylosed, or even fused in a wide range of variations.

JOINTS • Missing or hypoplastic phalanges or metacarpals are as common as duplicated

skeletal structures and are often accompanied by tendon, nerve, and ligament
deficiencies

• Digital nerves and arteries often have a wide variety of branching

NERVES patterns within a web space.
• An artery may loop around a digital nerve to form an arterial loop,

AND ARTERY
• Nerves can easily be teased apart under the microscope, but arterial
loops present a much more complex problem if they represent the
primary or only arterial conduit to a digit or thumb.

• Flexor and extensor tendons may have similar distal

TENDONS branching patterns and interconnections within a
web.
 TREATMENT
Timing
• Timing of surgical correction varies with the complexity of the deformity and the web space involved.
• Complicated and complex webbing involving adjacent digits with different growth potential warrants early release
by 1 year of age
• Patterns of prehensile function are established by 24 months, which should be the upper age limit for correction.
• There has been interest in correction within the first 2 weeks of life in infants with an abundance of mobile skin
and less complex deformities.
• Restoration of maximum functional potential and liberation of all digits should be completed by school age
• Complete webbing involving the important first (thumb-index) and fourth (ring-small) web spaces is best released
by 6 to 12 months of age.
• For progression of skeletal deformities to be avoided in patients with complex syndactylies, which may not be
corrected with growth once they are established, it is often necessary to perform surgery before 1 year of age.
• The urgency for correction increases with greater discrepancy between the webbed digits; thus, one should not
wait as long with the thumb-index web space as with the middle-ring web space.
• In general, the majority of simple and complex syndactylies are corrected between 12 and 24 months of age.
• Whenever possible, bilateral procedures should be performed on patients with deformities in both hands.. This is
best completed in nonambulatory children who are younger than 12 to 14 months because by 2 or 3 years of age,
the toddlers are more active and much more difficult to manage.
• In the child with multiple malformations and a normal neurologic system, maximal early treatment of the upper
limb malformations definitely helps these children reach their developmental milestones at more appropriate
ages.
• In some patients, however, the correction of major skeletal deformities and digital separations are preferably
completed before major craniofacial procedures in those with limb and facial malformations.
Historical Development

• The history of the surgical correction of syndactyly is fascinating,

and refinements made during the past 200 years have established
many important principles.

• The evolution of described techniques has progressed through four

overlapping periods-simple separation and epithelialization, use of
skin grafts, use of interdigitating sidewall flaps, and use of local
island flaps-to the use of distant pedicled and free flaps.

• Obviously, during the past 4 decades, all combinations of local

advancement and rotational flaps were described for release of the
first web space, and many were ultimately incorporated in the
treatment of congenital differences. The single Z-plasty and
combinations of these transposition flaps have been described. One
procedure, the four-flap Z-plasty, has become the "gold standard"
for the release of mild and moderate first web space deformities.
Principles

• Syndactyly separation is a
"simple problem and should not be made more complicated”

• The anatomy of the four normal interdigital web spaces has not
changed with time and has been well described and illustrated.

• It is important that each surgeon learn to execute his or her

preferred technique well for given indications.

• Good surgeons understand that principles and rules are made to be

broken.

• These hands require exceptional surgical approaches for the best

results to be achieved.
Technique

THUMB TO INDEX (FIRST) WEB RELEASE

• Correction of the thumb-index deficiency varies with the

severity of the contracture. Although more comprehensive
systems for evaluation have been described, first web space
deficiencies may be evaluated as :
– Mild
– Moderate
– Severe

• This characterization has the most direct impact on surgical

treatment.
 MILD

• For small defects, many variations of Z-plasty transposition

flaps have been described.
• It is preferable to use the four-flap Z-plasty because it
provides excellent length and contour within the depth of
the web space
• In a similar procedure, a complex central V-Y, lateral Z-
plasty approach may also accomplish the same result and
may be more useful within the interdigital web space.
• Techniques for providing dynamic muscle balance for
palmar abduction (opposition) after soft tissue release are
covered in other sections.
 MODERATE
• Tissue must be brought into the released web space, and all varieties of V-Y
flaps, dorsal transposition flaps and dorsal rotation or rotation-advancement
flaps with and without skin grafts have been described.
• Orthopedic surgeons like to use local rotation flaps from the dorsal surface of
the index metacarpal or proximalphalanx.
• Plastic surgeons usually eschew this technique, which leaves a conspicuous
skin graft on a visible surface. Whenever possible, it is preferable to use
variations of the advancement of dorsal skin into the defect.
• The mobility of the dorsal nonglabrous skin of the hand allows repeated
advancement of this tissue.
• The base of this flap is broad, and all proximal soft tissue attachments to the
flap are carefully preserved.
• A zigzag configuration of the incisions within the depth of the commissure will
reduce the chance of secondary contracture.
• Skin grafts are best placed along the lateral border of the thumb or index
finger and not directly across the web space.
• Tissue expansion can be used
• A temporary pin is used to hold the thumb metacarpal in maximal abduction.
 SEVERE
• The release and excision of the fibrous band or bands between the thenar intrinsic muscles
often includes a release of the carpometacarpal joint.
• Distal bifurcations of the common artery to the ulnar side of the thumb and the radial side of
the index finger are often present.
• One must be ligated, usually that to the index if there is a normal vessel to the opposite side
of this digit within the second interdigital web space.
• A combination of local rotation-advancement flaps with or without skin grafts is preferred
primarily because these flaps can be readvanced with subsequent operations.
• Distal tubed pedicled flaps are no longer used because they are too cumbersome.
• Distally based radial forearm flaps are an excellent source of regional tissue based on the
radial artery system or the dorsal interosseous vessels but they have two disadvantages.
• First, they leave a forearm scar, and second, they are difficult to perform in a 6-month to 2-
year-old child because of the large layer of adipose tissue.
• Free flaps from groin, opposite forearm, or lateral arm donor sites constitute another option
for the confident microsurgeon
• A pin or external fixator must be used to hold the thumb in maximal abduction
postoperatively. Skin expansion has the same results in these hands as it does in the less
restricted web spaces.
• Finally, ray resection of the index finger is an excellent option and is preferred when the
epiphysis of the proximal phalanx is abnormal or when three additional functional digits are
present on the same hand.
RELEASE OF COMPLETE, SIMPLE SYNDACTYLY
(SECOND, THIRD, AND FOURTH INTERDIGITAL WEB SPACES)

• A syndactyly release is a simple but meticulous operation that should not be made
more complicated than necessary.

• During the past 10 years, the author has used more straight-line dorsal incisions
for aesthetic reasons and prefers either a large dorsal rectangular flap or a
combination of dorsal and palmar triangular flaps for the commissure.

• General anesthesia is preferred.

ANESTHESIA • After routine preparation and draping, incisions are
planned with the pediatric tourniquet deflated.
MARKING

The clinical appearance and markings of a patient

with bilateral radial dysplasia and a type IIIA hand
in which the carpometacarpal joint is intact.

The first web space is deficient, and there are no

functional median-innervated
thenar intrinsic muscles.

The preoperative angiograms of the forearm and

hand show that there is no radial artery to the
hand, which is supplied primarily by the ulnar
system.

The blockage in the prominent interosseous

system negated possible use of a distally based
forearm flap
A precise amount of full-thickness tissue was
harvested from the opposite forearm, where the
donor site was closed primarily

Two arterial anastomoses were performed: one to

the radial side of the palmar arch and a second to
the index radial digital artery, which required
transection during the web release.

Dorsal veins were used for the venous recipient

vessels.

A C wire to hold the web space apart was placed

before flap inset and left in place for 4 weeks
postoperatively.

The ring flexor digitorum superficialis tendon

transfer to the abductor pollicis aponeurosis was
performed simultaneously to improve palmar
abduction (opposition) of the thumb.
DISSECTION

Principles of interdigital release and donor sites

A. One technique uses dorsal and palmar

markings for zigzag incisions to minimize
straight-line scar contractures with growth.

B. After the flaps have been sutured,

templates of the raw areas are made for
skin grafts, which may be obtained from a
variety of sources. Inset: "Like tissues" from
a similar part. The inguinal crease is most
commonly used, and the lower abdominal
flexion crease is used when large amounts
of skin are required during sequential
release of multiple web spaces in the same
patient. Foreskin preputial grafts are
described at periodic intervals but are not
preferred because of their frequent
infection and dark pigmentation.

C. After tourniquet release, the full-thickness

skin grafts are meticulously applied.

The ensuing dressing and immobilization are

imperative for a complete take of the graft.
Release of interdigital web space
A. The dorsal and palmar markings show a large dorsal flap to
line the commissure; the transverse components of the zigzag
palmar incisions lie in the normal existing interphalangeal
flexion creases.

The length and width are 13 mm and 7 mm in this 11-month-

old child.

B. Thick interdigital fibrous bands may originate both dorsal and

palmar to the neurovascular structures.

C. The new commissure has been formed and the side flap
sutured under no tension. Excessive defatting invites tight
closures.

D. The open areas have been covered with full-thickness skin

grafts that are secured with absorbable sutures.
E. A dressing with interdigital moist cotton is carefully applied
before the extremity is immobilized with a long-arm cast.

F. The flaps and grafts are seen 6 weeks later.

G. For 6 to 8 weeks after cast removal, an interdigital stent

secured with Coban is worn at night to maintain early
correction and to keep the hand out of the baby's mouth.
Nail folds
Paronychial and eponychial folds are one of the most difficult portions of the digit to construct.

A. The lateral skin can be

undermined and simply
advanced with minor
deficiencies.
B. Full-thickness skin grafts may
be used. Glabrous donor skin
is preferred.
C. After release, one side can be
covered with skin and the
other resurfaced with a graft.
In these patients, there is
never enough skin to cover
both sides.
D. Composite skin and pulp grafts
from the lateral side of the
great toe are effective.
E. Distal triangular flap from
either side of the fingertip
provides excellent paronychial
fold coverage in complex
syndactyly
SKELETAL CORRECTION
• The transverse metacarpal ligament is identified and incised if greater metacarpal mobility is
desired, as in the hypoplastic hand or Apert hand.
• However, duplicated skeletal parts should be excised, collateral ligaments reattached, and intrinsic
muscles appropriately reattached or completely excised during correction of complex and
complicated syndactylies, particularly within the central portions of the hand.

FLAP INSET
• The dorsal flap is rotated into the depth of the release, and the palmar flap is interdigitated and
secured with 5-0 or 6-0 absorbable sutures tied under no tension.
• The distal flaps along the sides of the digits are closed with 6-0 chromic sutures.
• Excessive defatting to gain mobilization and to close these flaps is not generally recommended but
may be helpful along the borders.
• Exact templates are made of all areas to be grafted.
• The tourniquet is released, flap circulation is checked, and bleeding is controlled with the bipolar
cautery.

SKIN GRAFTS
• A pressure dressing is applied while full-thickness skin grafts are harvested from the lower
abdominal flexion crease or the inguinal crease, well lateral to the future hair-bearing escutcheon .
• The donor site is closed with buried absorbable sutures and external Steri-Strips or skin adhesives.
• The skin grafts are defatted and sutured into position with 6-0 chromic sutures.
DRESSING

The dressing is all important!

Grafts and incisions are covered with one layer of a medicated gauze, followed by a compressible synthetic
foam or moistened cotton placed as a stent within the interdigital web space.

The fingers are positioned in abduction to avoid any kinking of the commissure flaps.

Next, a bulky fluff dressing is applied and secured with a circumferential Kling wrap.

The extremity is then immobilized with a well-padded long-arm cast or splint extending well above the flexed elbow.

The thumb or at least one other finger is left exposed distally to act as a monitor of hand position within the
cast.

A stockinette-sling is then passed around the cast and tied behind the child's back.

The cast is left in place for 2 to 3 weeks

Cast removal is usually most efficiently accomplished in the ambulatory surgery room under sedation or light
general anesthesia

When healing has been incomplete and small areas of scar are present at the base of the commissure, small
splints and molded inserts are made for the child to wear at night
FINGERTIPS AND CONJOINED NAILS

• In simple or complex complete syndactylies, the soft

tissue deficiencies include the pulp at the distal
phalangeal level, and a variety of techniques can be
useful.

• Nail matrices and underlying bone should be trimmed

to normal proportions.

• Local fat and fascial tissue should then be advanced

over the exposed distal interphalangeal joint or distal
phalangeal bone after the proper amount of skeletal
and nail trimming has been completed.
SKIN GRAFT DONOR SITES

• The ideal is to replace the exact "tissue in kind“ from an amputated extra part.
• Glabrous skin is available along the hypothenar eminence of the hand or the instep of foot
• Preputial grafts obtained from simultaneous circumcision have had periodic enthusiasts but
are no longer used by us because of initial infections or long-term hyperpigmentation.
• Full-thickness grafts from the inguinal flexion crease must be harvested lateral to the future
escutcheon and will result in a distracted scar.
• A Pfannenstiel incision in the lower abdominal flexion crease provides a large amount of
tissue in those with much larger graft requirements, as in the correction of bilateral mitten
hands.
• When this incision is reused for subsequent graft harvests, the grafts should all be taken from
the superior (cephalad) flap.
• Full-thickness grafts harvested with several millimeters of fat and areolar tissue will contract
less and provide better contour for a missing pulp surface
• In some conditions, small areas after web release are left open to epithelialize spontaneously.
• The ulnar side of the hand, hypothenar eminence, wrist, antecubital flexion crease, and
plantar surface of the foot have also been used.
• Donor sites are closed in layers with absorbable suture material.
• The last layer is a running intradermal or subcuticular stitch.
• The epidermal closure is reinforced with Steri-Strips placed along the same axis of the
incision.
• Those placed perpendicular to the incision often cause blistering when they are applied with
some tension.
TWO- AND THREE-DIGIT HAND

• It is recommended that one use the same technique as outlined for

simple syndactyly release but always line the depth of the
commissure with a large full-thickness flap.

• Multiple interdigitating flaps and skin grafts are avoided.

• It is often necessary later to rotate and recess one of these digits

(usually the radial) with an osteotomy to gain a more effective
pinching mechanism.

• These maneuvers are best accomplished by a Y-V principle with

incisions through unscarred tissue.

• Use of a temporary threaded pin or a permanent bone block is

recommended to secure the position of the metacarpals.
Outcomes
RELEASE OF FIRST INTERDIGITAL WEB SPACE

A. The clinical appearance and radiograph of a

unilateral left hand malformation are
shown.

B. A large dorsal flap and zigzag incisions

were used for soft tissue separation.

C. Six months later, a rotation-recession Osteo

tomy of the thumb ray was performed at
the metacarpal level through a Y-V incision.
D. The original dorsal flap could be re
advanced To further widen the web space
between the thumb and index finger.

E. Five years later, the broad web space

seen here from the dorsal view is
maintained on the left hand.
RELEASE OF SECOND THROUGH FOURTH INTERDIGITAL WEB SPACES

A. Scar tissue and residual bands along the sides of

these digits must be excised.

The dorsal flap has been raised and readvanced.

The bifurcation of the common digital artery is the

normal level of the commissure base.

Note the secondary paronychial fold revisions

B. The scar contractures in this child have caused

a combined deviation and rotational deformity
that will affect joint configuration with growth.
 TREATMENT OF SPECIFIC
SYNDACTYLY SYNDROMES
 APERT SYNDROME

CLASSIFICATION
• These hands have been broadly classified into three basic types,
defined by varying degrees of soft tissue and skeletal abnormalities.
• Although several refinements to this system have been proposed,
treatment decisions correlate well with the three-type system
• Three-dimensional imaging is often helpful in the evaluation of
complex type II and type III hands.

PRINCIPLES FOR TREATMENT

• The Apert hand is unique, often falling well outside the general
rules for treatment of other malformations. Therefore, there are
many additional principles that may be applied in the treatment of
specific patients.
Classification of the Apert hand

A. Type I Apert hand ("obstetrician's hand"), the

plane of the digits is flat, the thumb is
free, the index-long-ring digits are joined in a
complex syndactyly, and the fifth digit is joined in a
simple syndactyly. The distal joint of thefifth digit is
mobile, and there may or may not be an ulnar
duplication. The thumb has a radial clinodactyly
(hitchhiker thumb).

B. Type II Apert hand ("cup hand"), the thumb is

joined in a simple syndactyly, the transverse arch
of the hand is cupped, and there is splaying apart
Of the digits at metacarpophalangeal joint level.

C. Type III Apert hand ("rosebud hand" or "hoof

hand"), there is a tight skeletal union of all digits.
Nails often become ingrown and infected. The
thumb phalanges are often small and can underlap
the central three digits. Thumb radial clinodactyly
may not be present.
 OUTCOMES

Apert metacarpal
Apert hand secondary surgery
synostosis correction
 POLAND SYNDROME

TREATMENT
• Because these hands fall along a broad teratologic spectrum, treatment must be
carefully individualized to both the hand and the patient.
• Those patients with well-formed digits and thumb are best treated with digital
separations and the formation of as large a first web space as possible.
• Those with digital nubbins in the central rays are best treated with augmentation
of the border rays with nonvascularized phalangeal transfers.
• Finally, those with no digits or thumbs are good candidates for toe to thumb
transfers.
• Parents often seek multiple consultations from a large number of experienced
surgeons, all of whom may recommend a different course of reconstruction.

SYNDACTYLY RELEASE
• There are no significant differences in the technique of syndactyly release for
brachysyndactyly.
• The same principles come into play without significant alteration and are often
coordinated with standard constriction ring correction
Poland symbrachydactyly hand
OUTCOMES
• Because these deformities are unilateral, function and
appearance can always be contrasted with the contralateral
normal hand.
• Many have emphasized that the functional loss is much
greater than in those nonsyndromic hands with syndactyly
releases alone, indicating that there are additional soft
tissue and skeletal anomalies contributing to the functional
loss
• Grip, pinch, and precision intrinsic muscle function are
related to the degree of hypoplasia of the thumb and digits
in these hands.
• There is no reason to think there is a difference in the
outcomes after syndactyly release between these patients
and other patients with simple syndactyly.
 ACROSYNDACTYLY

CLASSIFICATION
• No system specifically described for the constriction ring
syndrome
• Terms such as the standard mild, moderate, and severe
may be used as designations for the first web and
incomplete and complete for the other interdigital web
spaces.
• There are no mirror image deformities in this syndrome
because each hand (and foot) is unique.
• There are no skeletal coalitions, but the tapered skeletal
parts at the site of acrosyndactylies are joined only by a
commonscar.
SYNDACTYLY RELEASE

• The same techniques and principles are used in these hands as in those discussed
previously.
• In those with multiple transverse amputations, there is a tendency to overcorrect
and deepen the web to the level of the metacarpophalangeal joint.
• It is important to have all the webs at the same level.
• The epidermal lining of the dorsal to palmar sinuses can often be used for
coverage of raw surfaces but does not have much usefulness because of its
tenuous blood supply.
• A fresh full-thickness skin graft is often preferred.
• Because many of these fingers are short, the surgeon and parents often want to
preserve as much length as possible. It is often better to form a shorter, well-
padded fingertip instead of a longer one that ends in a sharp point with an
overlying callus.
• For mild or moderate deficiencies, local flaps are used.
• For severe deficiencies, tissue must be added before any required thumb
reconstruction
• Under ideal conditions, it is preferable to use toe transfers for thumbs with no
phalangeal components and full-thickness tissue within the first web.
 OUTCOMES

Acrosyndactyly (constriction ring syndrome) toe transfer.