Professional Documents
Culture Documents
Hand
Khiem D. Dao, MD, Alexander Y. Shin, MD, Annette Billings, MD, Kerby C. Oberg,
MD and Virchel E. Wood, MD
Dr. Dao is an Orthopaedic Hand Surgeon, Westminster, CA. Dr. Shin is Assistant Professor, Department
of Orthopedic Surgery, Mayo Clinic, Rochester, MN. Dr. Billings is Orthopaedic Resident, Department
of Orthopaedic Surgery, Loma Linda University School of Medicine, Loma Linda, CA. Dr. Oberg is
Professor of Anatomy, Department of Anatomy, Loma Linda University School of Medicine. Dr. Wood
is Chief, Hand Surgery Service, Department of Orthopaedic Surgery, Loma Linda University School of
Medicine.
Reprint requests: Dr. Dao, West Valley Orthopaedics and Sports Medicine, Suite 150, 23101 Sherman
Place, West Hills, CA 91304.
Syndactyly is a congenital anomaly of the hand that is more common in males, is
present bilaterally in 50% of affected patients, and often is associated with other
musculoskeletal malformations or systemic syndromes. The goal of syndactyly release is
to create a functional hand with the fewest surgical procedures while minimizing
complications. For simple syndactyly, surgical reconstruction can begin at
approximately 6 months, although many surgeons prefer to wait until the infant is 18
months old. Special situations, such as complex syndactyly and involvement of border
digits, may warrant surgical intervention earlier than 6 months. Reconstruction of the
web commissure is the most technically challenging part of the operation, followed by
separation of the remaining digits. Full-thickness skin grafting is almost always
required for soft-tissue coverage. Complex syndactyly and syndactyly associated with
other hand anomalies warrant special consideration. After reconstruction, patients
should be examined periodically until they have achieved skeletal maturity because late
complications such as web creep can occur.
Syndactyly is a congenital anomaly that may be the most common1,2 or second most
common3 hand malformation. It may hinder hand function if not corrected appropriately
during infancy. Although the basic principles of surgical release of simple syndactyly
have been well established, each patient requires a thorough assessment of the soft-
tissue and bony components in the syndactylized region. Reconstruction must be
planned carefully when more than two digits are involved or when the syndactyly is a
component of a systemic congenital syndrome.
Embriología
Isolated syndactyly of the hand is estimated to occur in 1 per 2,000 to 2,500 live births.4
It is more common in males than females (56% to 84%)4–7 and occurs with equal
frequency unilaterally or bilaterally. This autosomal dominant trait has variable
expression or reduced penetrance.8 Advances in molecular biology have provided
insight into the embryologic etiology of syndactyly. During the fourth week of
gestation, the hand plate emerges, partially rimmed by a digital plate. During the fifth
week, radial condensations of mesoderm within the digital plate form the fingers, or
digital rays. The digital rays are separated by webs of interdigital tissue. Within the
interdigital space, radial apoptotic zones form, and programmed cell death first indents
the dorsal surface. The distal tips then separate, and programmed cell death carves out
the interdigital space proximally toward the central carpal region. The interdigital space
has formed by the end of the sixth week, but the digits continue to elongate and develop
ventral tactile pads and undergo phalangeal differentiation.
On a molecular level, the formation of separate and independent digits occurs via a
complicated interaction between fibroblast growth factors (FGFs), sonic hedgehog
protein, bone morphogenic proteins (BMPs), and homeobox transcription factors, with
MSX2 the most important.9–12 These factors, in turn, are regulated by the apical
ectodermal ridge (AER), which is known to have a critical role in determining digit
identity and formation of the interdigital space.
Early in limb development, FGF-10 expression within the lateral plate mesoderm
induces FGF-8 and FGF-4 expression in the distal ectoderm destined to become the
AER.13,14 Subsequently, cooperative interaction between AER-generated FGFs and sonic
hedgehog protein secreted from the zone of polarizing activity regulates BMP
expression within the mesoderm, forming a gradient across the cranial-caudal axis
(anterior-posterior axis) that provides positional cues for digit identity.15,16
By mechanisms that are as yet unclear, AER-generated FGFs then orchestrate the
production of BMP-regulating molecules that induce chondrogenesis at digital locations
(ie, BMP antagonists Gremlin17 and activins18). These FGFs also orchestrate the
expression of factors associated with the apoptotic cascade at interdigital spaces (ie,
MSX2,12 Snail11). Thus, within the digital plate, the AER helps establish digital and
interdigital identity.
As programmed cell death ensues within the interdigital zone, the overlying AER also
begins to regress. However, the AER persists over the chondrogenic zones, and
continued FGF expression is associated with digital growth and extension beyond the
hand plate.19 Because of the complex molecular interactions, disruption of several
factors that relate to AER regression, interdigital identity, or programmed cell death can
lead to syndactyly. Ectopic expression of the BMP-modifying factor Gremlin,17 BMP
overexpression,20 or a reduction in AER-related FGF expression11,20 all have been linked
to the formation of simple syndactyly. Conversely, overproduction of AER-related FGF
also has been associated with syndactyly.21 Presumably, both alterations in FGF
production disrupt the apoptotic cascade.
Clasificación
Syndactyly is classified as simple when only soft-tissue structures are involved in the
webbing and as complex when bone or fingernails of adjacent fingers are involved.
With complete syndactyly, the entire length of adjacent digits is involved in the
webbing; with incomplete syndactyly, the webs do not extend the entire length of the
digit. Complicated syndactyly is the term used to label complex cases that involve a
mixture or collection of synostoses.24 Syndactyly of the hand occurs most commonly
between the ring and long fingers (40% to 50%), with the next most common site being
between the ring and small fingers (25% to 28%).6,7 The least common location is
between the thumb and index finger (7% to 9%).6,7,23
Condiciones asociadas
Tratamiento quirúrgico
The overall goal of syndactyly release is to produce a hand with as many independent
and functional digits as possible with the fewest number of surgical corrections and
complications. Many techniques have been described to accomplish this goal; Upton26
reported 46 different methods used over the past two centuries. The intent with each is
to produce a web space commissure that is both deep and wide27,28 to cover the
remaining sides of the digits with as much local soft tissue as possible and to cover any
remaining skin deficits with skin grafts.
Timing of Surgery
Although surgical correction may be started at age 6 months, some surgeons wait until
18 months. The type of syndactyly required also may influence the timing of surgery.
Operating when the infant is at least 6 months old allows the bone and soft tissue to
grow and minimizes the anesthetic risks inherent when operating on an infant younger
than 6 months. Furthermore, starting at this age allows time for staged reconstruction
while the child is growing. However, if border digits are involved, reconstruction may
be started as early as 3 to 4 months. The differentials in longitudinal growth rates
between the thumb and index finger and between the ring and little fingers are so great
that, inevitably, bone and joint deformity develop if the fingers are not released early
(Fig. 3 ).
Figure 3 Complete syndactyly between the small and ring
fingers and partial webbing between the ring and middle
fingers. (Reprinted with permission from Dao KD, Wood
VE, Billings A: Treatment of syndactyly. Tech Hand
Upper Ext Surg 1998;2:166–177.)
Multiple-digit syndactyly requires staged surgery because releasing both sides of a digit
at the same time risks necrosis to the digit if it is supplied by only a single artery. For
syndactyly that involves all of the digits, the ring and long fingers and the thumb and
index fingers typically are released first, followed by the index and long fingers and the
ring and small fingers in a second procedure 4 to 6 months later. This approach
minimizes the number of surgeries and the anesthetic risk.23 In patients with syndactyly
of all fingers, the first operation may be done before age 6 months.32
Technical Considerations
Surgical techniques to manage syndactyly have not changed radically over the past
century. Dividing webbed digits by separating them with a pair of scissors in the
nursery, as was done in the very early 1800s, is not recommended because of the
inevitable risk of flexion contractures caused by longitudinal scarring. A full-thickness
skin graft (FTSG) usually is recommended to cover areas of the fingers not covered by
local flaps. FTSGs are preferred over split-thickness skin grafts because FTSGs have a
lesser tendency to scar and contract. Pedicle flaps usually are not required for congenital
syndactyly coverage. The surgical plan for syndactyly release includes the following:
establishment of a wide and deep commissure; separation of the digits with zigzag skin
incisions both dorsally and palmarly; separation of the nail, if necessary; division of any
bony interconnections; and application of FTSGs to deficient areas.
Surgical Technique
Surgery should be done under general anesthesia with a tourniquet on the involved
extremity; the opposite groin is prepared for FTSG harvesting.32 The commissure is
designed dorsally to have its medial and lateral base borders at the midsagittal line of the
contiguous digits, starting at the metacarpophalangeal joints (Fig. 4 ). Distally, the
bases of the flap are tapered slightly toward each other to accommodate the width of the
proximal phalanges on either side after the flap is inset. Making the flap length
approximately two thirds the length of the proximal phalanx provides a palmar
commissure edge approximately one third the length of the proximal phalanx, once the
flap is inset.23 The distal border of the flap is shaped like a dart to minimize scarring at
the commissure border between the dorsal and palmar skin. A reciprocal V-shaped
incision is then marked on the palmar surface of the proposed new webspace to
accommodate this flap. It is important that the created commissure slope in the
proximal-dorsal to distal-palmar direction.
Once all of the skin incisions are made, the dissection is begun dorsally by raising the
dorsal flaps. The thin natatory ligaments near the metacarpal heads are divided to fully
separate the digits; the transverse inter-metacarpal ligaments are not divided. The
incision is first taken distally between the nails, then palmarly. The interdigital soft
tissue is carefully divided to protect the neurovascular structures.
If the common digital nerves and arteries bifurcate proximal to the new commissure, as
is commonly the case, they will not require separation and will not impede insetting the
dorsal commissure flap. Should they bifurcate distal to this site, the digital nerves must
be carefully separated from distal to proximal. Should the digital arteries bifurcate
distally, the smaller of the two arteries can be ligated if there is only a simple web-space
syndactyly, because both digits still will have an arterial supply based on the opposite
side of the finger. When a central digit has syndactyly on each side (eg, index-middle-
ring finger syndactyly), the digital artery supplying the side digits bordering the middle
finger may be ligated because they also will have one remaining digital arterial supply
ulnarly or radially.
Syndactyly of the central digit is less an issue when both sides of the digit are not
operated on simultaneously. Alternatively, one may clamp a vessel, deflate the
tourniquet, and evaluate the resultant blood flow before ligating the artery in question.
Vascular complications are rare when only one side of a digit is operated on at a time,
and neither preoperative arteriograms nor exploration of the opposite side of a digit is
necessary.
Before securing the flaps, excess subcutaneous fat should be trimmed until only
approximately 1 mm of thickness remains. This decreases postoperative edema and the
risk of a tight closure. The tourniquet is de-flated before skin closure to control potential
bleeding. The dorsal commissure flap is sewn to its reciprocating V-shaped palmar
anchor with 5-0 absorbable sutures. The dorsal flaps along the digits are swung
palmarly, and palmar flaps are swung dorsally and sutured. Tight closure should be
avoided to minimize flap necrosis.
Usually one finger may be closed completely with skin flaps, whereas the other requires
an FTSG. Nearly all cases other than a very simple incomplete syndactyly require
grafting. Harvesting the FTSG lateral to the femoral artery prevents future pubic hair
growth on the flaps. Other donor sites include the antecubital fossa and the medial
aspect (ie, instep) of the foot. Care must be taken to obtain an accurate template of the
total area of skin defect to be covered. This template is transferred to the proposed donor
site before harvesting the FTSG. The skin graft also requires defatting before being
applied to the digits to prevent fat necrosis and graft loss.
Gauze dressings are placed deep between the separated digits because any exposed open
area can heal and resyndactylize the digits. As a final check, the vascularity of the digits
must be evaluated before casting to ensure that the gauze placed between them will not
constrict arterial flow. A long-arm club cast with the elbow flexed past 110° should be
used to protect the fingers from excessive motion while the skin graft is consolidating.
To allow the wound to be examined, the cast can be changed at 10 days and then
reapplied for 1 week or more. Examination of the wounds can be delayed for 3 weeks if
no signs of infection are present. Other than careful attention to the postoperative
dressing and immobilization, most children do not require extensive therapy after the 2
to 4 weeks of immobilization required for wound healing. This is also true for aftercare
of complex syndactyly reconstruction.
Consideraciones especiales
Although the techniques described are adequate for most patients, other conditions, such
as a short, incomplete proximal web, complex syndactyly, cleft hand, central
polydactyly, and acrosyndactyly, require alternative techniques.
Cleft Hand
Cleft hand deformities usually involve a central ray deficiency with webbing between
the two remaining digits of each border component (ie, ring-small finger syndactyly,
thumb–index finger syndactyly). The standard syndactyly release procedure should be
used to release these webbings early (by 6 months), followed by closure of the central
defect and release of the thumb adduction contracture either 6 months later or at age 18
months.39 Release of the thumb adduction contracture and closure of the central defect
may be achieved simultaneously with either the Snow and Littler technique35,40 (Fig. 8 )
or the Miura and Komada35,41 procedure.
Figure 8
Preoperative
photograph of
a typical cleft
hand. B, The
same hand
after
reconstruction
with the Snow
and Littler
technique.
(Reprinted
with
permission
from Dao KD,
Wood VE,
Billings A:
Treatment of
syndactyly.
Tech Hand
Upper Ext
View larger version (150K): Surg
[in this window] 1998;2:166–
[in a new window] 177.)
Central Polydactyly
Central polydactyly, or polysyndactyly, involves polydactyly of the index, long, and
ring fingers in association with a complex syndactyly. The ring finger is the most
commonly duplicated digit,42 and most cases involve duplication of a digit that has
normal components (eg, type II polydactyly in the Turek and Stelling
classifications).39,43
Parents should be warned about the large number of surgeries required for correction. In
one series of 22 patients with central polydactyly, there was a total of 160 operations,44
with one patient requiring 15 procedures. Correction by age 6 months is recommended,
especially when both the normal and duplicated digits articulate with the same phalanx.
It may be necessary to create a more functional three-fingered hand rather than risk
impairing circulation by attempting to preserve all of the digits. Creating a three-
fingered hand by sorting out the jumble of bones in a polysyndactyly often is the best
method of treatment, requiring far fewer surgeries with better functional results.
The surgery itself actually may be easier to do than a simple syndactyly release because
more skin is available for closure (Fig. 9 ). The standard syndactyly release is used, and
the extra bony components and soft tissue are resected. Tendons may be transferred
from an accessory digit to a retained digit to maximize function. Furthermore, parts of
two or three digits may be transferred to make one functional finger.
Figure 9 Preoperative photograph (A) and
anteroposterior radiograph (B) of polysyndactyly.
(Reprinted with permission from Wood VE:
Treatment of central polydactyly. Clin Orthop
1971;74:196–205.[Medline])
Acrosyndactyly
Acrosyndactyly is considered to be a form of secondary syndactyly caused not by a
failure of differentiation but from later in utero damage and fusion of the damaged
terminal phalanges. It occurs sporadically, is not hereditary, and may be termed
"amniotic disruption sequence." No webbing exists proximally between the adjacent
digits; rather, they are separated proximally by a fenestration or sinus. When the
interconnection between the digits is only a narrow skin bridge, it can be simply divided
or tied off with silk sutures in the newborn nursery. For more complex forms of
acrosyndactyly, surgical release and reconstruction must include excision of the sinuses
and incorporation of the longitudinal cleft in the reconstruction. Despite the FTSG
obtained from the fatty skin protuberances after their excision and defatting, abundant
FTSGs still will be necessary for complete coverage.
Resultados
Kettelkamp and Flatt6 reported the results of 69 syndactyly releases in 34 patients using
a variety of commissure designs and zigzag incisions with skin grafting. The most
important determinant of outcome was age of the patient at time of surgery, with the best
results in patients older than 18 months. The type of commissure design did not
influence the final outcome.
Posch et al45 reviewed 262 cases, including 340 hands, 558 web spaces, and 1,235 sides
of fingers. The revision surgery rates (for web creep or flexion contractures) after
syndactyly release were higher for border digits (eg, thumb–index finger web, ring-little
finger web) than for long and ring fingers. Revision surgery rates also were higher for
releases done before age 18 months than for older patients.45 However, in their review of
218 web releases in 100 patients, Percival and Sykes46 did not find any statistically
significant differences in the rate of web recurrence related to the age of the patient at
the time of surgery. They reported poorer outcomes in patients with concomitant
ipsilateral congenital hand abnormalities and in those who had split-thickness skin graft
rather than FTSG.
Complicaciones
The most common complication in syndactyly release is scar formation and progression,
either at the web or along the surgical scar (Fig. 10 ). Web creep is eight times more
common with split-thickness skin graft than with FTSG,25 and it is caused by a
combination of abnormal tissue, surgical scar, and increasing growth of the underlying
osseous structures.24 It may be inevitable even under optimal circumstances. If not
corrected, web creep may cause deformity of the growing bones and joints. Parents
should be advised of this possible complication, and the patient should be examined
periodically through adolescence. Correction requires release of the scar, similar to that
of the original procedure, correction with Z-plasty, and possibly an additional skin graft.
Web creep may be corrected with a four-flap or three-flap web Z-plasty.
Sumario
Reconstructing a hand with syndactyly can be complicated and is fraught with potential
pitfalls. Once the anomaly is classified as simple, complex, or complicated, the surgical
procedures and their sequence should be carefully planned. Consideration must be given
to whether border digits should be released early, whether skin grafting is needed, or
whether one finger should be sacrificed to produce a more functional, three-fingered
hand. For syndactyly involving digits of nearly equal lengths, surgery should be delayed
until age 18 months. Associated anomalies and systemic abnormalities should be
addressed by appropriate referrals. Careful planning and meticulous surgical techniques
can minimize potential errors and allow satisfactory separation of syndactylized digits.