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6 Syndactyly
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Fig. 6.5 Simple syndactyly can be separated with a dorsal Omega ap and triangular interdigital aps. The skin defects
can be covered with full-thickness skin grafts
microvascular clamp and open the tourniquet to
reassure sufcient vascularization of both digits.
Distal bifurcation of digital nerves can be treated
by interfascicular longitudinal dissection and
separation with a scalpel under loup (3.5×) or
microscopic magnication.
6.5.1.5 Simultaneous Separation
ofMultiple Syndactylies
Traditionally, the separation of multiple adjacent
syndactylies was avoided to prevent digital ischemia due to simultaneous dissection on the radial
and ulnar neurovascular bundle. Nevertheless,
simultaneous separation of adjacent syndactylies
may be considered as long as there is a good visualization of the neurovascular bundles during dissection. If all ngers are syndactylized,
simultaneous separation may not be optimal due
to the limited skin and soft tissue coverage. A
two-stage procedure, starting with the rst and
third and followed by the second and fourth web
space in a second stage, should be considered in
these hands.
6.5.1.6 Dressings andDressing
Changes
Dressings protect the wound and secure skin
grafts. Application under tourniquet is acceptable
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D. M. Weber
Fig. 6.6 Skin substitutes such as hyaluronic scaffolds can give similar results to full-thickness skin grafts, however, the
time to primary wound healing may be prolongated
as long as the surgeon feels condent regarding
ap vascularity and if recapillarization of the ngertips can be observed with the dressing. Both
traditional wound dressings with fat gauze and
cotton, as well as more elaborate wound dressings
with synthetic dressings such as Mepilex Silver
Transfer® (Fig. 6.6) give similar results in the
author’s hands. Tie-over dressings are not needed
to secure digital skin grafts in hands. However,
dressings must be well secured and may be stuck
6 Syndactyly
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Fig. 6.7 Asymmetrical nger pulp aps, modied after the Buck-Gramcko technique result in a good nail wall reconstruction and unimpaired nail growth
Fig. 6.8 Complete
syndactylies of digits
with unequal length
should be separated
early to avoid
progression of
clinodactyly
67
directly on the skin. An additional stocking on top
of the rst dressing can be changed by the parents.
The thumb should be left under the stocking and
the arm should be immobilized in a sling to prevent the use of the hand and inadvertent removal
of the rst dressing. Splint or cast immobilization
is reserved for complex cases with osteotomies
and K-wire insertion.
The rst dressing change can be planned after
2 to 3weeks. The author recommends the use of
resorbable sutures such as, for example, VicrylRapid 6–0®, so that no routine anesthesia is
needed for the rst dressing change and removal
of the stitches [7]. At the rst dressing change, a
similar dressing as that during surgery can be
applied together with some ointment, followed
by further dressing changes as needed. Parents
are encouraged to massage the scars with ointment or silicone gel. Prophylactic splinting with
silicone application at night for up to 6months
may be considered if hypertrophic scars are
anticipated.
6.5.1.7 Timing ofSurgery
Syndactylized ngers of unequal length tend to
get a progressive clinodactyly and should be separated early, preferentially around 6months of
age (Fig.6.8). All other syndactylies can be oper-
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D. M. Weber
ated ideally from about the rst birthday until the
age of 18months. Anesthesia at an earlier age is
more demanding and may bear higher risks [8].
The determined will of children between the ages
of 18 months and 4 years and yet the limited
understanding of the goal of surgery makes elective surgery and immobilization difcult in this
age group and may pose a major burden to the
children and their families.
6.5.2 Surgical Techniques
6.5.2.1 The First Web Space
oftheHand
Syndactylies of the rst web space are usually
associated with syndromes, such as Apert’s syndrome, or other anomalies, such as symbrachydactyly, thumb hypoplasia, or amniotic bands.
Separation of the rst web space must therefore
always incorporate treatment of all aspects of the
anomaly. The associated narrowing of the rst
web space is not only due to the cutaneous syndactyly but also due to contractures of the adductor pollicis and the rst dorsal interosseous
muscle. The fascia of these muscles must be
incised during the exposure of the rst web space
and the insertion of the adductor on the third
metacarpal may need to be released. Fibrous
bands, resembling interglenoid ligaments
between the thumb and index, can be found and
must be released when present. An artery that
runs in the rst web space and bifurcates to the
thumb and index far distally is a common nding.
Before the ligature of one arterial branch, it may
be clamped and the perfusion checked after
release of the tourniquet.
In a relatively proximal partial syndactyly,
local “Z-aps” and a “VY-ap” in the rst web
space may be sufcient. More distal forms and
narrow web spaces need large aps such as the
dorsal rotation-advancement ap (Fig. 6.9) and
eventually a full-thickness skin graft. A microsurgical ap may be considered in very tight rst
web spaces with complete syndactyly.
6.5.2.2 The Second toFourth Web
Spaces oftheHand
Dozens of techniques and ap designs to separate
syndactylies have been published [9]. The author
recommends surgeons to limit themselves to a
few techniques and to become familiar with them
before eventually trying other ap designs. The
standard armamentarium should comprise a technique for complete syndactylies with fullthickness skin grafting or skin substitutes
(Figs.6.5 and 6.6) [10] and graftless techniques
for partial syndactylies until the PIP joint
(Fig.6.10) or slight distalization of the web space
(Fig.6.11) [3]. Irrespective of the ap design, it is
important to make a deep, i.e., proximal web
space since it always tends to migrate distally
again. Short ngers, such as in symbrachydactyly, look longer if the web space is slightly too
proximal.
6.5.2.3 Syndactyly oftheFeet
The incidence of simple, partial syndactylies of
the second web space of the feet is x per 10,000
live births. Most concerns consider appearance of
the foot, since functional problems or pain is
exceptional. Although surgical separation of
syndactylies of the feet follows the same principles as that of the hands, indications should be
kept restrictive: The complication rate after syndactyly repair, particularly for infections, is
higher on the feet than on the hands. Scars can be
very cumbersome at sports, particularly with athletic shoe wear. The authors therefore recommend the separation of syndactylies between the
second and third toes only at the patient’s wish at
an older age and not upon parental desire only.
Surgical separation is recommended for the
rst web space, since syndactyly fuses the biphalangeal rst toe the biphalangeal second toe and
because web space is functional for wearing ipop sandals. Furthermore, the separation of toes
of unequal length, mostly in the third web space,
should be considered, because fusion of unequal
toes results in progressive clinodactyly and may
be associated with pain or clavus formation.
6 Syndactyly
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Fig. 6.9 Wide, dorsal
rotational advancement
ap to liberate the rst
web space in a patient
with symbrachydactyly
69
Associated anomalies, such as postaxial polysyndactyly, should be excluded with an X-ray before
surgery. Interdisciplinary evaluations together
with a pediatric orthopedic surgeon should be
considered in complex syndactylies of the feet,
particularly in children with syndromes, such as,
for example, Apert syndrome. We recommend
early syndactyly repair at the age of 9 to
12 months, before children start walking, since
this makes care much easier for the families.
Surgical principals are the same as those for
hands, with an emphasis of a good ap coverage
in the web space. Small full-thickness skin grafts
may be taken below the lateral malleolus, if
needed (Fig.6.12).
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Fig. 6.10 Repair of partial syndactylies up to the PIP joint with primary skin closure without skin grafts
D. M. Weber
Fig. 6.11 Web deepening for proximal forms of partial
syndactylies with multiple Z-plasties (trident aps) without skin grafts
6 Syndactyly
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Fig. 6.12 Syndactyly repair of the feet is warranted for the rst web space. It follows similar principles as that of the
hands with wide commissural aps and full-thickness skin transplants
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6.6 Complications
Injury to digital arteries or tension of skin aps
may delay wound healing, promote infections,
and result in the loss of skin grafts. Severe scarring and web creep can be the consequences
and lead to functional as well as esthetic decits that require reoperations [11]. Scars may
not inhibit hand function initially. However,
tight scars do not grow with the child and may
result in contractures, clinodactyly, and even
luxation of joints years after the initial surgery
(Fig.6.13) Therefore, splinting at night is rec-
ommended in children with difcult scars after
syndactyly release. Keloid formation is an
unusual complication after syndactyly release.
A study by Muzaffar etal. demonstrated, that
primary digital enlargement is a highly predictive risk factor for keloid formation and that
standard treatment with pressure, topical or
intralesional corticosteroids may not be sufcient to control keloids [12].
Reoperations for scar contractures or hypertrophic scars should be delayed until the maturation of the scars, which does not occur until
6months postoperative.
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D. M. Weber
Fig. 6.13 Severe late complication with clinodactyly and subluxation of the DIP due to scar contractures
References
1. Ahmed H, et al. Genetic overview of syndactyly
and polydactyly. Plast Reconstr Surg Glob Open.
2017;5(11):e1549.
2. Oberg KC, etal. Developmental biology and classication of congenital anomalies of the hand and upper
extremity. J Hand Surg Am. 2010;35(12):2066–76.
3. Tonkin MA.Failure of differentiation part I: syndactyly. Hand Clin. 2009;25(2):171–93.
4. Sulser PS, Kalisch M, Weber DM.Retroauricular fullthickness skin grafts in syndactyly repair: outcome
and comparison with inguinal full-thickness skin
grafts: retrospective (cross-sectional) study. J Plast
Surg Hand Surg. 2016;50(5):281–5.
5. Landi A, et al. Hyaluronic acid scaffold for skin
defects in congenital syndactyly release surgery: a
novel technique based on the regenerative model. J
Hand Surg Europ. 2014;39(9):994–1000.
6. Errol G.Syndactyly. In: Dieter B-G, editor. Congenital
malformations of the hand and forearm. London:
Churchill Livingstone; 1998. p.131–40.
7. Weber DM, Schiestl CM. Absorbable sutures help
minimise patient discomfort and reduce cost in syndactyly release. Eur J Pediatr Surg. 2004;14(3):151–4.
8. Davidson A, Vutskits L. The new FDA drug safety
communication on the use of general anesthetics in
young children: what should we make of it? Paediatr
Anaesth. 2017;27(4):336–7.
9. Samson P, Salazard B. Syndactyly. Chir Main.
2008;27(Suppl 1):S100–14.
10. D’Arcangelo M, Gilbert A, Pirrello R.Correction of
syndactyly using a dorsal omega ap and two lateral
and volar aps. A long-term review. J Hand Surg Br.
1996;21(3):320–4.
11. Canizares MF, etal. Complications and cost of syndactyly reconstruction in the United States: analysis
of the pediatric health information system. Hand (N
Y). 2017;12(4):327–34.
12. Muzaffar AR, etal. Keloid formation after syndactyly
reconstruction: associated conditions, prevalence, and
preliminary report of a treatment method. J Hand Surg
Am. 2004;29(2):201–8.
Symbrachydactyly
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ElisaRosanda, ChiaraParolo, andGiorgioPajardi
7
Abstract
Symbrachydactyly is a congenital hand defect
where there is both syndactyly and brachydactyly. The clinical manifestations have many
variations, from a hand with hypoplastic ngers to a severe form of adactylous hand.
Symbrachydactyly is typically unilateral,
characterized by failure of the formation of
ngers and presence of rudimentary nubbins
that include elements of nail plate, bone, and
cartilage.
The etiology is still unknown, but vascular
dysgenesis during fetal development is a leading hypothesis.
The treatments vary based on the degree of
malformation and family needs. When surgical treatment is needed, syndactyly release is
the most frequent procedure. In monodactyly
type or adactyly type pinch function can be
created with non-vascularized free phalangeal
transfer procedure or microsurgical toe-tohand transfers.
E. Rosanda (*) · C. Parolo
University Department of Hand Surgery and
Rehabilitation, San Giuseppe Hospital MultiMedica
IRCCS, Milan University, Milan, Italy
e-mail: elisa.rosanda@multimedica.it;
chiara.parolo@multimedica.it
G. Pajardi
Milan, Italy
e-mail: gpajardi@centrostudimano.it
Keywords
Symbrachydactyly · Congenital hand · Poland
syndrome · Toe-to-hand transfer · Nonvascularized free phalangeal transfer
7.1 Introduction
Symbrachydactyly is a congenital hand defect
where in which there is both syndactyly and
brachydactyly.
The clinical manifestations have many variations, from a hand with hypoplastic ngers to
severe form of adactylous hand.
Symbrachydactyly is typically unilateral,
characterized by failure of formation of ngers,
and presence of rudimentary nubbins that include
elements of nail plate, bone, and cartilage. In the
past was called also atypical cleft hand for the
absence of the central digits and presence of the
digit of the border [1].
Symbrachydactyly was rst described by
Poland in 1841. He described a syndrome in
which there was a combination of absence or
hypoplasia of long nger, syndactyly, and hypoplasia of pectoralis major (Fig. 7.1) [2]. Pol in
1921 was the rst who used the terms
Symbrachydactyly and described two different
forms: with or without the association of hypoplasia or aplasia of pectoralis major [3].
© Springer Nature Switzerland AG 2023
G. Pajardi (ed.), Pediatric Hand Surgery, https://doi.org/10.1007/978-3-031-30984-7_7
73
74
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Fig. 7.1 Poland syndrome: (a) hypoplasia of pectoralis major and (b) symbrachydactyly
E. Rosanda et al.
Today, the symbrachydactyly is classied in
the Oberg, Manske, and Tonkin (OMT) classication as an undergrowth or failure of axis
formation.
7.2 Epidemiology
Associated syndrome [71]
Cohen syndrome
Aglossia-adactyly syndrome
Cofn–Siris syndrome
Dyggve–Melchior–Clausen syndrome
Grebe syndrome
CHILD syndrome
Duplication 9p syndrome
The diverse morphologic characteristics of symbrachydactyly make accurate incidence difcult.
A study from Brazil reported a 0.054% incidence
of symbrachydactyly in Caucasians and 0.043%
in African Americans [4]. Two studies from Japan
reported an incidence of 1 in every 20,000 to
30,000 births [5] and 1in 10,000 birth [6]. Other
survey estimates an incidence of approximately
0.6 per 10,000 in live births. In total, 73% of
cases are males. The condition is usually unilateral with the left upper limb alone involved in
67% of cases, the right in 27%, and 1% to 7%
bilateral [7]. A total of 7% of cases have associated anomalies such as Poland syndrome, in
which hypoplasia or absence of the pectoralis
major occurs with additional variable abnormalities [2]. In 7% of cases, there is a positive family
history of symbracydactyly [7–9].
Associated syndrome [71]
Poland syndrome
Moebius syndrome
Langer-Giedion syndrome
Trisomy 9p syndrome
Deletion 5p syndrome
7.3 Etiology
The etiology of symbrachydactyly is still
unknown, but vascular dysgenesis during fetal
development (“subclavian artery supply disruption sequence”) is a leading hypothesis [10].
In support of this theory, a study of eight
patients with Poland syndrome showed decreased
blood ow velocity in affected limbs [11]. Other
studies suggest different etiologies: one study
showed that giving 5-uorouracil early in pregnancy produced symbrachydactyly in rats [12].
Another study showed that injection of busulfan
produced rats with central clefts, osseous syndactyly, and symbrachydactyly [13, 14].
Based on the current understanding of upper
limb development, symbrachydactyly likely
arises through disruption of the apical ectodermal
ridge (AER) of the developing limb bud. The
AER, a thickening of ectodermal cells at the distal end of the limb bud, directs proximal-distal
limb development through a complex cascade of
growth factors and genetic signaling, while con-
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