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54
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C. Parolo et al.
Fig. 5.5 Static splint
splint is used (Fig. 5.5), while for irreducible
ones (type IB) a dynamic splint in extension with
circular base module, wrist included and Levame
type bar as dynamic tractions is custom made
(Fig.5.6). It is very important that the splint is
worn correctly. The splint in PIP extension must
have dorsal closure stabilizing the V metacarpal
very well and positioning the MPJ at about 50° of
exion. In fact, in addition to the bending stiffness of the PIPJ, there is often a compensatory
attitude in hypertension of the MPJ Furthermore,
the dynamic bar must pull the middle phalanx on
the proximal phalanx by means of a velcro ring
with a perfectly perpendicular force.
The difculty of realization is linked to the
complexity of the orthosis and the size of the fth
nger of a child (or a newborn). The splint is
Fig. 5.6 Dynamic splint
often worn from the early months of life and used
during the night and during the afternoon nap.
The initial choice of the type of splint is therefore not a denitive choice. A type IB camptodactyly may become reducible after a few months
of treatment with the use of a dynamic splint and
therefore a static type will be chosen. During
development stiffness may recurr and a dynamic
splint should be applied again.
5.6 Surgical Technique
A volar linear incision is used and converted to
multiple z-plasties, placing the central limbs over
the exion creases of the joints. On reecting the
skin, the shortened retinaculum is visualized; it is
released, including the bony attachment of
Grayson’s ligaments. At this stage, the lateral
5 Camptodactyly
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bands of the intrinsic apparatus and interosseous
muscles are freed from their abnormal and widespread attachment to the sides of the proximal
phalanx. It is this attachment that prevents the
extension of the PIP joint. On its release, proximal pull on the lateral bands will conrm that PIP
extension can now occur. Some attenuation of the
central slip may have occurred and is probably
secondary, as in other forms of prolonged PIP
joint contracture. Its presence can be conrmed
by the central slip tenodesis test. It involves exing the wrist and the MP joints. In the normal
hand, the PIP joint will automatically fully
extend, owing to tension on the central slip insertion. If the central slip is attenuated, there will be
an extensor lag at the PIP joint. In such patients,
the central slip can be treated by appropriate
postoperative splinting. The lumbrical muscle is
abnormally inserted and often adherent to the
proximal phalanx. It may also have an abnormal
origin and occasionally may be inserted into the
FDS tendon proximal to the A1 pulley. The FDS
tendon is tested by a tenodesis test to ascertain
whether it is short. If the PIP joint cannot be fully
extended when the wrist is in extension, the FDS
is short and must be released. Two types of FDS
abnormalities exist: (1) one in which the FDS is
merely short, and (2) the other in which only the
distal portion of the FDS is present, there is proximal aplasia, and the distal part of the FDS acts as
a tenodesis, producing a exion contracture of
the PIP joint. In such cases, complete surgical
release by division is undertaken. A small minority may require release of the exor tendon
sheath, volar plate or even the accessory collateral ligaments. The central slip attenuation
responds to postoperative extension splinting.
From 2002 to 2018, we have treated 54
patients and 88 ngers affected by
camptodactyly.
In total, 59 ngers were affected by the exible form. Twentynine ngers were affected by
the rigid form.
A total of 85 ngers underwent conservative
treatment (59 exible and 26 xed).
55
Fig. 5.7 Pre-op
Fig. 5.8 Post-op
Among this series, 67 ngers concluded the
conservative treatment with success (56 with no
lack of extension—excellent result; 11 ngers
with an extensor lag minor than 20°—good
result) (Figs. 5.7 and 5.8). Conservative treatment failed in 18 ngers (lag of extension major
than 20°). Ten ngers (seven from the conservative group plus three from the xed group never
treated with splinting) underwent surgery with a
lag of extension of more than 40°.
Results were excellent in two digits (no lag of
extension), good in three (lag of extension less
than 20° and poor in ve (lag of extension more
than 20°) (Table5.3).
In all of our series, there was an improvement
after treatment and the quality of results depended
upon the severity of the contracture and protocol
compliance.
The conservative approach leads to gains in
function. Hand therapy and custom-made splinting are essential to obtain and maintain improvement of passive extension and to regain active
extension both in conservative and surgical treatment. Continuous monitoring, constant use of
splints and manipulation are important to maintain the achieved results.
56
Results
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Table 5.3 Results of treatment
C. Parolo et al.
-at least 1y follow up-
Flexible form -59 fingers-
Rigid form -29 fingers-
2 fingers
Excellent result
Almost complete AROM
Just conservative treatment
All with good results
26 fingers conservative treatment
8 fingers
Good result
Extension lag < 20*
3 fingers direct
surgical treatment
Extension lag > 40*
All had benefits in gains of extension and correction of
the deformity, in most cases with reduced AROM
Further Reading
Courtemanche AD.Campylodactyly: etiology and man-
agement. Plast Reconstr Surg. 1969;44:451–4.
Dautel G.Camptodactylies. Chir Main. 2003;22:115–24.
De Haas WHD. Camptodactylie Nederlands tijdschr
Geneesk. 1957;101:2121–4.
Dofn G, Lenoble E, Foucher G, etal. Camptodactylie:
classication et résultats thérapeutiques. Ann Chir
Main (Ann Hand Surg). 1994;13(1):20–5.
Engber WD, Flatt AE. Camptodactyly: an analysis of
sixty-six patients and twenty-four operations. J Hand
Surg. 1977;2:216–24.
Gupta A, Burke FD.Correction of camptodactyly. J.Hand
Surg. 1990;15B:168–70.
Hori M, Nakamura R, Inoue G, et al. Nonoperative
treatment of camptodactyly. J Hand Surg.
1987a;12A:1061–5.
Inoue G, Tamura Y. Camptodactyly resulting from para-
doxical action of an anomalous lumbrical muscle.
Scand J Plast Reconstr Hand Surg. 1994;28:309–12.
Koman LA, Toby EB, Poehling GG. Congenital exion
deformities of the proximal interphalangeal joint in
children: a subgroup of camptodactyly. J Hand Surg.
1990;15A:582–6.
Maeda M, Matsui T.Camptodactyly caused by an abnor-
mal lumbrical muscle. J Hand Surg. 1985;10B:95–6.
56 fingers
Excellent result
Extension lag
3 fingers
Good result
Extension lag < 20*
18 fingers
Poor results
Extension lag > 20*
7 fingers Surgery
Extension lag > 40º
3 fingers
Good result
Extension lag < 20*
5 fingers
“Poor” result
Extension lag > 20*
McCash C.Congenital contractures of the hand. In: Stack
HG, Botton H, editors. The proceedings of the Second
Hand Club, British Society for Surgery of the Hand.
London; 1975. p.399–401.
McFarlane RM, Curry GI. Evans HBAnomalies of the
intrinsic muscles in camptodactyly. J Hand Surg.
1983;8:531–44.
McFarlane RM, Classen DA, Porte AM, Botz JS. The
anatomy and treatment of camptodactyly of the small
nger. J Hand Surg. 1992;17A:35–44.
Minami A, Sakai T. Camptodactyly caused by abnormal
insetion and orgin of lumbrical muscle. J Hand Surg.
1993;18B:310–1.
Miura T.Nontraumatic exion deformity of the proximal
interphalangeal joint: its pathogenesis and treatment.
Hand. 1983;15:25–34.
Miura T, Nakamura R, Tamura Y.Long-standing extended
dynamic splintage and release of an abnormal
restraining structure in camptodactyly. J Hand Surg.
1992;17B:665–72.
Miura T, Nackamura R, Tamura Y. Long standing
Extended Dynamic splintage and release of an abnormal restraining structure in camptodactyly. J Hand
Surg Br. 1992;17B:665–72.
Ogino T, Kato H.Operative ndings in camptodactyly of
the little nger. J Hand Surg. 1992;17B:661–4.
Oldeld MD. Camptodactyly: exor contracture of the
ngers in young girls. Br J Plast Surg. 1956;8:312–7.
5 Camptodactyly
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57
Scott J.Hammer nger with notes of seven cases occuring
in one family. Glasgow Med J. 1903;60:335–44.
Siegert JJ, Cooney WP, Dobyns JH.Management of sim-
ple camptodactyly. J Hand Surg. 1990;15B:181–9.
Siegert JJ, Cooney WP, Dobyns JH.Management of sim-
ple camptodactyly. J Hand Surg Br. 1990;15B:181–9.
Smith PJ.Ross DAThe central slip tenodesis test for early
diagnosis of potential Boutonniere deformities. J
Hand Surg. 1994;19B:88–90.
Smith RJ, Kaplan EB.Camptodactyly and similar atrau-
matic exion deformities of the proximal interphalangeal joints of the ngers: a study of thirty- one cases. J
Bone Joint Surg. 1968;50A:1187–203.
Stoddard EE. Nomenclature of hereditary crooked n-
gers: Streblomicrodatyly and camptodactyly—are
they synonyms? J Hered. 1939;30:511–2.
Todd AH. Hereditary contracture of the little ngers
(Kamptodactyly). Lancet. 1929;2:1088–9.
Syndactyly
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DanielM.Weber
6
Abstract
Syndactyly is a common hand anomaly that
affects 1 in 2000–3000 live births. It has a
spectrum from simple syndactylies with
fusion of the skin and soft tissues only, com-
plex syndactylies with fusion of bones and
nails to complicated syndactylies which are
often associated with syndromes.
This chapter covers the epidemiology, classication, and diagnostic workup of syndactylies. It presents treatment strategies for
surgical corrections of syndactylies, including
techniques with skin grafts, without skin
grafts, and with skin substitutes.
Keywords
Syndactyly · Child · Hand · Surgery · Release
6.1 Introduction
Syndactyly is a congenital limb anomaly that is
characterized by an incomplete separation of digits, caused by abnormal interdigital connections.
The term is derived from the Greek words syn,
meaning together and dactylos, meaning digit.
D. M. Weber (*)
Division of Hand Surgery, Department of Pediatric
Surgery, University Children’s Hospital, Zurich,
Switzerland
e-mail: Daniel.Weber@kispi.uzh.ch
6.2 Epidemiology
Syndactylies are among the most common hand
anomalies, with an estimated incidence of 1 in
2000–3000 live births, affecting both hands in
50%. Ten to 40% have a positive family history
with a clear male predominance.
The basic principles of segmentation of the
hand are fairly well understood. The apical ectodermal ridge controls the proximodistal outgrowth of the limb, whereas the development and
differentiation along the radio-ulnar axis are controlled by the zone of polarizing activity (ZPA).
The ZPA and the AER work in a close feedback
loop, resulting in a hand plate that becomes visible in the fth week of development. During the
elongation process, digits form through condensation, whereas tissues in between are removed
by apoptosis in a distal to proximal orientation.
Only a perfect equilibrium between the Bone
Morphogenic Proteins (BMPs) that suppress
broblast growth factors (FGFs), thereby inducing apoptosis, and the BMP inhibitors enable a
normal development. Interference with or mutations of BMPs, FGFs, and BMP inhibitors suppress controlled apoptosis and can therefore
induce syndactylies, sometimes associated with
polydactylies or synostoses [1, 2].
There are over 300 known syndromic anomalies causing syndactylies with etiologies that
remain poorly understood, mainly due to their
phenotypic and genetic diversity. Within fami-
© Springer Nature Switzerland AG 2023
G. Pajardi (ed.), Pediatric Hand Surgery, https://doi.org/10.1007/978-3-031-30984-7_6
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D. M. Weber
lies and even individuals, phenotypes may be
severe or mild, unilateral or bilateral, and
affect both or either hands and feet [1].
Geneticists classify syndactylies on Temtamy
and McKusick’s system which has been
extended to nine non- syndromic forms of syndactylies, seven of them with autosomal dominant inheritance, variable expressivity and
incomplete penetrance and two autosomal
recessive forms. Syndactyly type I is the most
common non-syndromic syndactyly, usually
affecting the third web space on the hands and
the second web space on the feet. The most
common subtype 1 has been associated with
the locus 3p21.31 but no disease-causing genes
have been identied [1].
6.3 Classication
Simple clinical classications help surgical planning. The differentiation into complete syndac-
tyly includes the ngertips and partial syndactyly
with a web space that can reach anywhere
between a normal web space and the ngertips
can describe all forms of syndactyly. Furthermore,
syndactylies can be classied into simple syndactylies, complex syndactylies, and complex complicated syndactylies (Fig. 6.1). Simple
syndactylies are characterized by only cutaneous
and soft tissue fusions of the ngers and welldeveloped, separated ngernails. Usually, the
segmentation of the digits, tendons, and pulleys
is normal with occasional distalisation of the
bifurcation of the neurovascular bundles.
Complex syndactylies involve the nail and osseous phalanges and may be associated with segmentation anomalies of tendons and a distalisation
of the bifurcation of the neurovascular bundles.
Complex complicated syndactylies have a
severely disturbed anatomy not only of the osseous elements but also of the neurovascular bundles and musculotendinous structures. They are
often associated with syndromes, such as in
Apert’s syndrome.
As mentioned above, some geneticists and
pediatricians adhere to the classication which is
based on Temtamy and McKusick.
ab
cd
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61
Simple, incomplete
Simple, complete
Complex Complicated
Fig. 6.1 Classication of syndactylies: (a) Simple,
incomplete; (b) Simple, complete; (c) Complex; (d)
Complicated. (From Upton J: Management of disorders of
separation-syndactyly. In: Hentz VR, editor. The hand and
upper lim (Part2) in: Mathes SJ, editor. Plastic surgery
vol. 8. Philadelphia: Saunders Elsevier; 2006. P. 140)
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D. M. Weber
6.4 Diagnostic Workup
Simple syndactylies of the ngers with a full
mobility of the IP joints don’t need a further
workup, since a regular anatomy of tendons and
neurovascular bundles can be anticipated.
Fig. 6.2 The X-ray
reveals the complexity
of this complete,
complex, complicated
syndactyly in a child
with a synpolydactyly
However, if in doubt, an X-ray is recommended,
because the complexity can be underestimated as
illustrated in Fig.6.2 with a patient with a familial synpolydactyly. A pediatric or genetic workup
is recommended for complicated syndactylies
and can be considered in complex syndactylies.
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6.5 Treatment
The aim of surgical separation of web spaces is
improving or maintaining function while optimizing the appearance of the hand by separating
all digits.
6.5.1 General Principles
Irrespective of the localization and type of the
syndactyly, some principles of reconstruction
must be respected.
6.5.1.1 Flap Coverage oftheWeb
The proximal denition of the web should be
covered with a wide, well-vascularized ap to
avoid scar formation and “web creep”, a secondary syndactyly that narrows or distalizes the new
commissure. The width of the ap should ensure
a round conguration and avoid a V-shaped web
space. Dorsally based aps can be mobilized easily since they have hardly any fascio-cutaneous
adherence and have the advantage of bringing
pigmented skin to the dorsal and well-visible
aspect of the web space. Ideally, they mimic the
natural proximal to distal and dorsal to palmar
inclination of the web.
6.5.1.2 Skin Grafts andSubstitutes
There is a shortage of interdigital skin when separating syndactylies. This becomes evident on a
hand drawn sketch and can be explained and
demonstrated to parents easily (Fig.6.3). Various
ap designs have been recommended to separate
partial and complete syndactylies without skin
grafts. Partial syndactylies up to the PIP joint can
be separated reliably without using skin grafts or
substitutes [3] (technique see below), whereas
separation of complete syndactylies without skin
grafts may be associated with higher risks of
complications and a less favorable outcome.
Large skin defects on the hand result in scars
that retract and may distort the neighboring skin
aps. Exceptionally, skin defects may be tolerated in stiff ngers, when contractures are not
an issue, such as in Apert’s hands with symphalangism. Traditionally, skin defects are covered
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Fig. 6.3 A simple hand-drawn sketch illustrates to parents the shortage of skin
with autologous skin grafts. Full-thickness skin
grafts are preferred over split skin grafts,
because they hardly shrink. The pigmentation,
texture, and hair bearing of the donor skin must
match the recipient site on the hand and the
donor site must be inconspicuous. Traditionally,
full-thickness skin grafts are taken from the
ulnar volar aspect of the wrist, the cubital fossa,
or the groin. It is important to remember that the
skin from the groin may grow pubic hair after
puberty, particularly when being taken
medially.
An excellent donor site that is not used commonly is retroauricular skin (Fig.6.4). It is hardly
hair bearing and has a superior color match when
compared with groin skin (Fig. 6.5) [4]. The
ellipsoid incisions should be marked with a pen
so that 1/3 of the skin is taken from the ear.
Injection of a local anesthetic with a vasoconstrictor (such as bupivacaine+adrenaline) facilitates harvesting of the skin graft. After a two-layer
wound closure with resorbable subcutaneous and
intracutaneous sutures, a light dressing may be
applied for a day or two. Experienced surgeons
may harvest the retroauricular skin graft before
operating on the hand to minimize tourniquet
time and avoid leaving the draped hand to raise a
skin graft.
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D. M. Weber
Fig. 6.4 Full-thickness retroauricular skin graft
Dermal substitutes or a hyaluronic scaffold
can be used as an alternative to skin grafts. It
shortens operating time and avoids donor site
scars. Application by suture xation is easy and
spontaneous resorption of the hyaluronic acid is
followed by spontaneous epithelialization with a
good color match (Fig.6.6). Good results were
published [5]; however, no studies have compared the results of skin substitutes with skin
grafts.
6.5.1.3 Fingertip andNail Wall
Reconstruction
Complete complex and complete complicated
syndactylies often have fused nails and therefore
lack a lateral nail fold. Spontaneous scarication
of the nail fold or scarication after application
of full-thickness skin grafts after separation of
the nails results in nail growth disturbance. Flap
coverage of the defect protects nail growth and
creates a natural-looking nail fold. BuckGramcko published symmetrical aps from the
neighboring ngertips [6]. They provide excellent results in children with well-developed n-
gertips. However, quite often, there is not enough
volume and the author prefers doing asymmetrical aps with a transverse Buck-Gramcko ap
from one ngertip, asymmetrical longitudinal
incisions between the pulps and a simple translation of the pulp on the donor ngertip (Fig.6.7).
It is important not to mobilize the fasciocutaneous adherence extensively to avoid wobbly ngertips. Sutures on the nail fold should never be
tight and small defects that heal secondarily are
acceptable.
6.5.1.4 Exposure andSeparation
ofNeurovascular Bundles
Dissection in a bloodless eld with a tourniquet
allows a good visualization of the neurovascular
bundles. In simple syndactylies, the bifurcation
of nerves and vessels is usually deep in the web
space and does not limit the opening of the web
space. In complex syndactylies, such as, for
example, in Apert syndrome, one may nd just
one interdigital artery with a distal bifurcation
that necessitates ligation of one digital artery.
Before ligating one branch, one may apply a
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