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11 Cleft Hand or Split Hand Foot Malformation
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129
tions of the normal pattern of hands and feet
remain unclear. Among morphogens involved in
the formation of the autopod, DLX5 and DLX6
seem to be crucial for the persistence of the apical ectodermal ridge (AER) and are upregulated
by TP63. In mice Tp63 Null, the AER fails to
stratify and the expression of the Dlx genes is
strongly reduced [17]. Disorders in the pathways
explain some similarities in the SHFM1 and
SHFM4 phenotypes (EEC and developmental
delay). Duijf etal. [18] have postulated that cleft
hand is primarily due to a defect in the central
part of the apical ectodermal ridge (AER) on the
autopod; this was also suggested by Naruse etal.
[19]. If the disruption of the central AER occurs
precisely on the top of the median ray, one can
observe a central polydactyly. When the disruption to the central portion of the AER becomes
irregular and wider, the consequence is a failure
of induction of nger rays and disorganization of
the formation of the precartilaginous anlagen of
the future central digits. The third or median
anlage can fuse with the fourth or the second
anlage or with both of them. In severe cases,
some rays can be missing.
Another consistent nding in SHFM is the
presence of extrinsic tendons and intrinsic muscles in the hand despite the absence of the bones,
which raises questions about the close relationship of bones and tendons during development.
11.6 The Cleft Hand
intheClassications
oftheCongenital Hand
Anomalies
exposed pregnant rats to an antimitotic agent
(Busulfan) at different stages and observed central polydactyly or central clefts of various severity. His conclusion was that cleft hand and central
polydactyly are derived from the same embryological mechanism, and that the aetiology is primarily not a failure of formation but a failure of
differentiation of parts. Twice we observed, in
child a medial cleft on one hand, bidactylous
form, and an authentic central polydactyly on the
other hand. Flatt [1] in his book shows an example of monozygotic twins with one twin presenting a central polydactyly, and the other twin a
complete absence of the third ray. Indeed, the
radiological analysis of some SHFM distinctly
shows a separation in two of the third ray which
migrates on the second and/or fourth nger,
which evokes more a lack of separation than a
real lack of formation of a ray (Fig.11.4).
Ogino’s publication sparked off controversial
discussions between specialists; some decided to
stay with the former classication whilst others
decided to modify the position of cleft hand
within Swanson’s classication. More recently,
and in light of recent knowledge gained from
developmental biology, a new classication has
been adopted by the IFSSH, namely, the Oberg–
Manske–Tonkin or OMT classication [22].
Based on the three axes of development of the
hand and upper limb, cleft hand is now classied
within the category of ‘Malformation, which is a
failure of formation/differentiation, of unspecied axis and complex’ [23]. With many colleagues, we have proposed to move SHFM to
malformation; handplate; and proximal-distal
axis (IB1iv).
The quarrels regarding classication and treatment of cleft hand since Swanson’s classication
(1968) [20] are in part due to the incredibly low
incidence of this disorder. In this system previously adopted by the International Federation for
Surgery of the Hand (IFSSH), cleft hands were
classied as ‘failure of formation of parts, longitudinal arrest, central ray’. This remained largely
unchanged until Ogino (1990) [21] published the
results of his experiment. In his work, Ogino
11.7 Classication ofClinical
Forms ofSHFM
Concomitantly, many attempts had been made to
classify the different types of ‘typical’ cleft hand.
The most relevant have been those providing
guidelines for surgical treatment, such as the
classication by Glicenstein etal. [24] where it is
divided into three groups: simple, complex and
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severe. The simple type has a more or less marked
cleft on the third ray, usually with the presence of
the third metacarpal. Complex types are associated with membranous or complex syndactylies
and/or with bone anomalies: transverse phalanges (cross-bones) [6], delta phalanges and bone
fusions (Fig. 11.5). The severe types are the
three-ngered or bidactylous or monodactylous
forms (Fig.11.6).
Another way of choosing a surgical treatment
is to consider the state of the rst web, as suggested by Manske and Halikis [25]. In type 1
Fig. 11.4 SHFM with fusion of the third and fourth
metacarpal, moderate central cleft and st web
syndactyly
(normal rst web) or 2 (rst narrow rst web), a
simple treatment of the cleft and/or syndactyly of
ab
c
Fig. 11.5 ‘Simple closure’ of a moderate SHFM. . (a)
Pre-operative X-rays showing the migration of the third
ray onto the fourth. (b) Per operative palmar view. Note
the abnormal brous anlagen in the cleft and the remnant
of the exor tendon of the third digit running on the radial
side of the fourth. This extra tendon limits the extension of
the PIP joint. (c) Dorsal view demonstrating a central tendinous loop (blue silicone loop) and a supernumerary
extensor tendon
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Fig. 11.6 Bilateral monodactylous hand. Feet have the
same feature. No surgical solution. The child can write
very nicely with a bimanual grasp
the rst commissure is performed. In some more
severe cases of type 2, a translocation of the second ray on the third metacarpal will be proposed,
or we will simplify the hand resecting the second
ray to achieve a three-ngered hand.
11.8 Principle ofTreatment
As Adrian Flatt [3] said: ‘cleft hand is a functional triumph but a social disaster’. The rst surgical goal is to improve the disturbing appearance
to an almost normal hand. After surgery, one ray
might still be missing but with good closure of
the cleft with a minimal amount of scar and by
achieving parallelism of the long ngers, the
hand anomaly will be barely noticeable. The second principle is to preserve the function by treating the thumb web or associated camptodactyly,
but we should never compromise the child’s former function. The third principle is to combine
the two, cosmesis and function or a ‘cosmetic
function’ by avoiding rotation of the ngers during exion and preserving the normal skeletal
structures (bones, joints, tendons) from the deleterious effects of the additional elements (bone
fusions and supernumerary tendons).
11.8.1 Tendon Anomalies
Before considering the surgical treatment of such
complex hands, it is necessary rst to compre-
131
hend and understand anatomical abnormalities.
As a rule, we must consider that growth can alter
a result that was previously thought to be good.
We must not dissociate aesthetics and function.
The surgeon can make a hand that is aesthetically
pleasing at rest but, looks abnormal during use,
such as the rotation of the ngers during exion.
These are important notions that the surgeon
must keep in mind before proposing a therapeutic
schedule to families.
If bone abnormalities are visible on radiographs or CT scan with 3D reconstructions, tendon abnormalities are often neglected. It is
imperative to explore the cleft and the adjacent
ngers to make the distinction between the normal elements of each ray and the supernumerary
elements which will have to be largely resected.
We previously had the opportunity to perform
a dissection of a human specimen with SHFM
[26]. The patient presented with bilateral SHFM,
and bidactylous hands; little other information
was available. The thumbs were slightly hypoplastic whereas the ulnar digit was broad, probably resulting from the fusion of two rays
(superdigit) [27]. Before dissection, skeletal
radiographs of the upper limbs and computertomographic scans with three-dimensional
reconstruction of the images were performed.
The wrist and forearm bones were normal, but
arthrosis on the proximal-inter-phalangeal joint
of the most ulnar digit was noted. During the dissection, we were able to identify all muscles and
tendons in the forearm, even exor and extensor
tendons for the missing ngers. These tendons
were present in the forearm and travelled to the
hand but ended in the cleft, forming a few loops
in the middle of the cleft or tendon plexuses onto
the lateral side of the two digits.
This observation was of major interest for our
current practice in reconstructive surgery, as we
noted that extrinsic exor and extensor tendons
could be formed even in the absence of some rays
in the hand. During surgery, additional tendons
could join together in the centre of the cleft and
migrate on the lateral side of the adjacent ngers
in the presence or absence of cross-bones. These
extra tendons could be partially responsible for
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camptodactyly and rotation of the adjacent ngers during exion. Careful division of the cleft
and of the lateral side of the ngers with removal
of additional tendons greatly improved the cosmetic and functional outcomes of patients with
split hands.
Bone anomalies: it is necessary to remove the
abnormal elements which are more or less fused
with the adjacent ngers, especially the transverse phalanges or cross-bones which have a
physeal plate often very active and whose growth
can cause a divergence of neighbouring metacarpals, further aggravating the deformity. These
transverse phalanges must be removed carefully,
they often share joints with neighbouring metacarpophalangeal joints (MP joints). It is advisable to keep a limited portion of these phalanges
to avoid an instability of the MP joints. Delta
phalanges can be treated either by osteotomy or
Vicker’s procedure.
If a divergence of the metacarpal remains, the
surgeon should not hesitate to perform a closing
wedge osteotomy at the base to achieve a complete parallelism, which will grant a harmonious
growth. On the contrary, in the rst commissure,
it will sometimes be necessary to release the
intrinsic muscles to increase the divergence
between the rst and second metacarpals, or perform a metacarpal osteotomy or, in some cases,
remove the second metacarpal and perform an
index translocation on to the third ray.
Interventions should be started early in
infancy. Whenever possible, the rst web must be
corrected between 12 and 18months. For complex translocations, it is not illogical to wait until
2 to 3years old. Usually, we operate only on one
hand to leave the child using the contralateral
hand. Commonly we perform surgery on one
hand and on the ipsilateral foot in the same stage.
11.9 Surgical Procedures
11.9.1 Simple Forms
In the simple type of Glicenstein or the type 1 of
Manske, we perform a simple closure of the cleft.
We have seen that this ‘simple closure’ is in
appearance: it will require a parallelism of the
second and fourth metacarpal, resect the third
metacarpal if present, and rebuild an intermetacarpal ligament. This is facilitated by a complete
exploration of the ngers to identify the exor
tendon sheeths. Their fascial expansions at the
neck of the metacarpals must be preserved to be
sutured at the end of the procedure.
We try to preserve the periosteum around the
third metacarpal, even if there is a risk of reossication in the following years, this ossication
never deforms the hand. The periosteum is a very
resistant fabric for a ‘paletot’ suture, from the
base of the metacarpals to the neck [28].
The procedure begins with a dorsal approach.
The skin incision itself does not require any
sophisticated ap for closure. Our drawing tends
to leave only enough skin taken from the lateral
side of the adjacent ngers to achieve a longitudinal scar. The nal level of the web will be decided
following comparisons with the other webs. We
found that attempts of local aps ended with contractures, imposing a revision surgery to ‘redig
the cleft’!
Neurovascular pedicles are identied, and
careful division of the cleft is a very important
step for the identication of any additional tendons. A central loop between exor and extensor tendons might be found and should be fully
removed. We examine the normal tendons of
digits 2 and 4 and then resect any abnormal
additional tendons. In some cases, we would
observe an improvement of the exion and rotation of the PIP joints. If there remains any
abnormal exion or rotation, we would completely remove the third metacarpal bone. Our
goal is to achieve a good parallelism of the
metacarpal bones (Fig.11.7a–d). If digits 2 and
4 remain divergent, we would consider performing a closing wedge osteotomy of the base of
one of the metacarpal bones. We carefully divide
the exor tendon sheets at the pulley A1 level to
reconstruct the deep transverse metacarpal ligament by using two ligamentous aps made out
of the exor tendon sheaths of the index and
ring ngers to prevent future migration of the
ngers. Although the exor sheaths are strong
structures, they are insufcient for a stable clo-
cd
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133
Fig. 11.7 Two years follow up after closure of a ‘simple
cleft’. (a) Plain radiograph showing the good parallelism
of the second and fourth metatarsal. (b) Hand in full
abduction. Good level of the central web. No ap has been
sure of the inter- metacarpal space. We used to
partially preserve the periosteum of the former
third metacarpal bone to reconstruct two periosteal aps [28]. The dorsal aspect is closed with
‘paletot’ sutures, using an absorbable suture
such as Monocryl® or PDS®, Ethicon, Cincinnati,
USA. After which a passive exion force is
applied to check if the suture limits the range of
motion of the MP joints. Fatty pads in the palm
of the hand are removed to avoid any unaesthetic folds. The skin incisions are then sutured
with absorbable stitches. At the end of the procedure, passive exion of ngers is used to verify that there is no more rotation and that all
performed but a straight longitudinal line of suture. (c)
Dorsal view. Scar on the social face of the hand. (d)
Fingers in exion. No rotation thanks to the removal of
abnormal brous anlagen and tendons
ngertips converge in exion towards the tubercle of the scaphoid (Fig. 11.7d). An occlusive
bandage is left for 2 or 3weeks in cases in which
we have done an osteotomy. K-wires on the base
of the metacarpal bones can be removed if
radiographs conrm bone healing. After release
of the rst bandage, we leave the ngers free to
move but strongly recommend a transverse bandage, with an elastic tape or a self-cohesive bandage, at the metacarpal heads level for another
3 weeks to protect the closure of the intermetacarpal space. For most cases, physiotherapy is not necessary as we have allowed motion
from the second or third week onwards.
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When all these steps are fullled, it is advisable that the result remains stable during growth.
This harmonious four-ngered hand is hardly
noticed by relatives and friends. This agreeable
reconstruction signicantly reduces the psychological impact of the congenital morphological
condition on the child in the difcult time of
childhood and adolescence.
11.9.2 Complex Forms
For complex cases with syndactyly of the rst
web, there are two options according to the severity of the narrowing of the web. If the syndactyly
is proximal and loose, it can be released with any
appropriate ap (Z plasty or combined Z plasties
or a rotation-transposition ap) in association
with closure of the cleft, as described above. For
severe narrowing, the rule is to transpose the second ray onto the third metacarpal bone (if present). It is necessary to resect the distal two-thirds
of the third metacarpal and to transfer the second
metacarpal and the index on the base of the third
metacarpal (as an on-top plasty) after performing
a microsurgical dissection of the pedicles of the
index. Osteosynthesis is usually performed with
two oblique K-wires at the base and sometimes
by transverse pins joining the second and fourth
metacarpals. We no longer use a Snow-Littler
procedure [29] owing to the poor viability of the
palmar ap [30]. We have routinely performed
the Miura and Komada technique [31], with satisfactory results (Fig.11.8a, b), and our preference is now for the technique as described by
Upton [32], through a distal approach to allow a
limited dorsal scar. We usually try to avoid any
dorsal incision since the dorsum of the hand is
the part of the hand most often seen by others and
by the patient himself (Fig.11.9).
With this technique, we obtain both functional
and natural hands and results which are stable
with growth.
The exploration of the cleft is the same as in
the simple closure and, before the translocation,
it is necessary to remove all the supernumerary
elements to keep only the normal elements and
ensure a natural function to the future hand. In
the case of a transverse bone, we would remove it
partially, leaving parts of it in continuity with the
metacarpal joints, in order to avoid damage to the
collateral ligaments, instability or stiffness.
It is necessary, in certain cases, to correct the
clinodactylies of the ngers and in particular to
identify ‘delta’ phalanges which are frequent in
the SHFM. This is often difcult on X-rays of
very young children. If the delta phalanx is conrmed radiologically or, better, by ultrasound, we
can propose, before the age of 7years, Vickers
procedure. This procedure consists in removing
part of the C-shaped bracketed epiphysis, after
resecting an epiphyseal triangle and lateral
Fig. 11.8 Complex SHFM type 7. Miura’s procedure. (a)
Pre-operative view. Deep central cleft and syndactyly of
the rst web. (b) Per-operative view after index transposi-
tion. Good opening of the rst web and well-balanced
cosmetic appearance of the hand
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Fig. 11.9 Upton’s approach for index transposition and
closure of the web. This distal incision gives an excellent
view on the cleft, the rst web and avoid the dorsal scar
growth cartilage to the diaphyseal cancellous
bone, a small amount of fatty tissue is interposed.
This pearl prevents a re-ossication of the bracketed epiphysis. The locker is then released on the
proximal and distal physeal plate. A spontaneous
correction of the deviation is observed after a few
years [33]. If the child is too old, a reverse osteotomy can be proposed by taking a bone triangle
on the base of the triangular phalanx. The graft is
returned and placed in the apex of the phalanx
delta. This technique avoids the shortening created by a closing wedge osteotomy.
11.9.3 Severe Types
For severe forms with three, two or a nger, surgical indications are discussed with the parents
and, if possible, with the child. It is sometimes
useful to do nothing to avoid compromising the
function. In particular, closing cleft at all costs
could, on the contrary, limit the possibilities of
135
grip. Sometimes we just ‘simplify’ some rays to
reduce the monstrous nature of the hand and possibly facilitate prosthetic equipment for social
life. A toe transfer would be a good option, but
feet are usually abnormal. We used this procedure in two children with a monodactylous hand
and bidactylous feet. Harvesting the hallux was
much more difcult than in normal feet. The vascular network was difcult to divide despite the
pre-operative angio-CT scan. One child is still
painful on the donor site. It is wise to discuss
complex malformations on a case-by-case basis
with other specialists in congenital hand surgery.
For some patients, the best option might be to
accept the situation.
11.9.4 Central Clefts oftheFeet
Foot treatment deserves attention as well, and
much earlier correction than it was traditionally
thought. Children are very quickly hampered by
the width of the forefoot and painful calluses on
the lateral faces of the toes which are found in the
plantar position. It is therefore necessary, in the
rst years, to close the cleft by using staged osteotomies on the metatarsals and phalanges. The
principle of closing the cleft is the same as at the
level of the hand.
11.10 Conclusions
Median cleft hand is a complex but rare malformation requiring a multidisciplinary management. Genetics input has recently allowed to
identify seven SHFM subgroups and provide better insight about associated malformations and
inheritance for each type. If an infant is primarily
referred to a hand surgeon, a thorough clinical
examination should be carried out in order to
identify additional malformations and/or a syndromic association. Referrals for genetic counselling and to various specialists might be
necessary, such as paediatricians, ophthalmologists, ENT, dermatologists, plastic and orthopaedic surgeons. Function in the cleft hand is
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impaired because of the anatomical changes but
not necessarily compromised, as children adapt
very well to most congenital malformations.
However, the unusual cosmetic appearance can
have a marked psychological impact on patients.
Surgical techniques have now allowed to improve
the hand aspects and to become ‘socially’ acceptable. It is also now possible to correct any remaining stiffness by removing additional skeletal
structures and to avoid rotation when digits are
exed by modifying the abnormal tendon anatomy. Indeed, even when rays are missing, it is
important to bear in mind that the tendons are
still formed and present in the cleft as a loop or
on the adjacent digits and would need to be
addressed for a good functional result. It is therefore important to have a good knowledge of the
anatomical particularities for optimal treatment
and growth stability.
The very severe malformations will be the
subject of discussions between the specialized
surgeons and in certain cases the therapeutic
abstention will be the most reasonable option.
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Brachydactyly Types D andE
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ZavarukhinV.Ivanovich
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Abstract
The J.Bell’s classication describes two types
of hand ray shortening, namely, brachydactyly
types D and E, which have a lot in common.
When a child suffers either of these diseases,
he/she is born with ahand that appears to be
absolutely healthy, but when he/she is between
four and 7years of age, his/her parents notice
the rst signs of progressive shortening of one
or several ngers. Although these pathologies
are differentiated as two brachydactyly types,
they are just different manifestations of the
same disease with common etiology and
pathogenesis, with similar complaints, and
with similar surgical-treatment approaches.
Brachydactyly type E is shortening of one
or several metacarpals and is also known as
brachymetacarpia.
Brachydactyly type D is shortening of distal phalanx in the thumb where the nail plate
looks extremely short but wide.
Indications for surgical treatment are
mainly cosmetic for brachydactyly type E and
are only cosmetic for brachydactyly type D.
The preferred treatment for both types of
brachydactyly is distraction lengthening.
Keywords
Brachydactyly · Brachymetacarpia
Lengthening · Distraction · Shortening · Stub
thumb
12.1 Introduction
The J. Bell’s classication [1, 2] describes two
types of hand ray shortening, namely, brachydactyly types D and E, which have a lot in common.
When a child suffers either of these diseases, he/
she is born with a hand that appears to be absolutely healthy, but when he/she is between 4 and
7 years of age [3–6], his/her parents notice the
rst signs of progressive shortening of one or
several ngers. Although these pathologies are
differentiated as two brachydactyly types, they
are just different manifestations of the same disease with common etiology and pathogenesis,
with similar complaints, and with similar
surgical- treatment approaches.
Brachydactyly type E is shortening of one or
several metacarpals and is also known as
brachymetacarpia.
Brachydactyly type D is shortening of distal
phalanx in the thumb where the nail plate looks
extremely short but wide [7].
Z. V. Ivanovich (*)
Candidate of Medical Sciences, Head of the
Department of Traumatology N3, St. Petersburg State
University Hospital, St Petersburg, Russia
© Springer Nature Switzerland AG 2023
G. Pajardi (ed.), Pediatric Hand Surgery, https://doi.org/10.1007/978-3-031-30984-7_12
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