Phẫu Thuật Tạo Hình (Tập 6): Bàn Tay Và Chi Trên – Neligan (Ấn bản 4)

637
Polydactyly
In radioulnar synostosis, improved function and decreased
musculoskeletal discomfort in adjacent joints are reported,
with up to 80% having good to excellent results.80 As men­
tioned earlier, even with an interposition graft, re-ankylosis is
possible with increased pronation. Neurologic and vascular
compromise, and Volkmann ischemic contracture have been
reported,85 especially in cases of longitudinal osteotomy at the
fusion site. This should be avoided by monitoring the degree
of rotation and removing a segment at the osteotomy site.5
Secondary procedures
Secondary procedures performed on metacarpal synostosis
and radioulnar synostosis are re-operations because of incom­
plete correction, re-ankylosis, or loss of motion. Because of the
influence of growth, children should be observed until skeletal
maturity, especially in metacarpal synostosis. Less motion in
the metacarpophalangeal joint should also be addressed.
Polydactyly
Introduction
Polydactyly (poly = more; dactylos = finger) refers to the dis­
order in which patients have more than five digits on at least
one extremity. Together with syndactyly and camptodactyly,
it is the most common congenital upper extremity difference.
The disorder is easily detected leading to cosmetic concern of
the parents and, if not treated, cosmetic concern of the affected
child. Additionally, depending on the level of the duplication,
it can cause functional impairment. Polydactyly of the hand
can be divided into radial, central, and ulnar polydactyly,
referring to the region of the extra digit or part of a digit.86
The incidence of polydactyly varies depending on the
studied population. Region, race, and combined numbers of
all polydactylies, or only radial- or ulnar-sided polydactyly,
provide very different incidences in published series. The
incidence can range from 2% to 30% when considering all
types of polydactylies and up to 40% in the Chinese popula­
tion. The prevalence of polydactylies is 10.7 of 1000 live births
in people with an African descent compared with 2.5 of 1000
live births in Chinese people and 1.6 of 1000 live births in
Caucasians. Ulnar polydactyly is 10 times more common in
those of African descent and twice as common in males,
whereas radial polydactyly is more common in Asians. Central
polydactyly is extremely rare in all published studies.
At the authors’ center, out of 200 new patients per year with
congenital hand deformities, about 15% have polydactyly,
including 50% with radial polydactyly, 9% with central poly­
dactyly, and 33% with ulnar polydactyly (some in combina­
tion). In the authors’ series, 42% of polydactylies are bilateral.
Radial polydactyly is mostly isolated, whereas syndromic
association is more common in ulnar polydactyly. Further­
more, ulnar polydactyly is mostly bilateral and can be associ­
ated with syndactyly and polydactyly of the feet. Children
born to parents with nubbins or floating little fingers inherit
the same type of polydactyly. Children born to parents with
more developed extra little fingers can present with all types
of polydactyly.87
Many syndromes have been described in combination
with different kinds of polydactyly. Already, over 97 genetic
Patient selection/treatment/surgical technique
The treatment of symphalangism is mostly conservative.
Because of anomalies of the flexor and extensor tendons, early
surgical intervention is not successful. In well-segmented
joints only, exploration and release of the collateral ligaments
and dorsal capsule may improve function; however, results
are not predictable. Arthrodesis in a more functional posi­
tion of the involved joints at skeletal maturity will improve
function and grip. Distraction lengthening of the stiff fingers
in symbrachydactyly may improve the pinch between the
thumb and a short index finger. It can lead to possible better
aesthetics; however, the treated fingers can become thin
and stiff.
The indications for treatment of a metacarpal synostosis are
both functional and aesthetic, and depend on the type of
fusion. The abducted little finger may get caught in pockets
and therefore be a problem. The goal of treatment will be a
longitudinal, axially oriented ray with the MCP joint in line
with the other joints, preserving its motion and joint surface,
and correction of angulation and rotation. Less functional
digits should be positioned out of the way of the thumb to
little finger prehension.5 Several operative treatments have
been advocated, depending on the type of synostosis5,68
ranging from osteotomy alone68,78 to lengthening with bone
graft or callodistraction5,68,79 and longitudinal splitting com­
bined with an interposition bone graft.5
Carpal synostosis will only need treatment in painful
fibrocartilaginous coalition. If this is the case, it is advised to
fuse the involved carpal bones.
In radioulnar synostosis, only few patients will need
surgery because patients will compensate with wrist and
shoulder movement. Treatment is recommended when a
patient is compromised in daily actions with a fixed pronation
of 60° or more in unilateral and bilateral cases.
To restore movement, longitudinal osteotomy at the fusion
site in combination with a vascularized adipofascial flap and
alignment of the radial head80 has been performed. Several
types of osteotomies have been described. Only resection of
the synostosis is disappointing with rapid re-fusion as a
result. To prevent re-fusion, the anconeus muscle has been
used, as well as pedicled and free vascularized fat flaps.80,81
Derotational osteotomies are performed just distal to the
fusion,82 or at the proximal radius and distal ulna.83,84
Postoperative care
In symphalangism, postoperative treatment consists of
passive movement of the released joints. When an osteotomy
is performed, treatment should be focused on the type of
fixation. If a metacarpophalangeal joint is released, with or
without additional tendon transfers, these should be addressed
accordingly.
Outcomes, prognosis, and complications
In most of the cases, metacarpal synostosis will not need
treatment. If treating metacarpal synostosis, early problems
are the diminished metacarpophalangeal movement, mostly
present preoperatively, and insufficient alignment of the
involved digits in metacarpal synostosis. Late problems may
be a recurrent abduction of the little finger, and a re-ankylosis
at the osteotomy site, despite interposition.

638
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
chromosome lies the regulatory element of the SHH protein.
In isolated ulnar polydactyly an autosomal-dominant inheri­
tance pattern occurs, with a diminished genetic penetrance
and a variable expression. It is genetically heterogeneous,
implying that the cause lies in mutations in various genes.
Diagnosis/patient presentation
Radial polydactyly
In radial (preaxial) polydactyly, the most commonly used
classification is the Wassel classification.
The Wassel classification (Fig. 28.11)89 is based on radiology
and is easy to remember. The levels of duplication of the
different types are: type I at the distal phalanx; type II at the
IPJ; type III at the proximal phalanx; type IV at the MCPJ; type
V at the metacarpal; and type VI at the CMCJ. Thumbs, of
which at least one is triphalangeal, are type VII – this can be
either at the MCPJ or at the CMCJ.
In most series, the three most common Wassel types are,
respectively, Wassel IV (about 50%); Wassel II (about 20%),
and Wassel VII (about 12%). The percentages are variable in
the different reported series.90–92 As 31% in our polydactyly
series could not be well classified, we modified the classifica­
tion system.92 The essence lies in keeping the Wassel classifica­
tion but naming the differences such as deviation and
triphalangism (see below).
At first consultation, following medical history and general
physical examination, both upper limbs are examined. If
the index to little finger is normal, with normal hand and
finger creases, and a normal hypothenar region, the examina­
tion is concentrated on the radial side of the hand. In our
experience, it is worthwhile performing this systematically,
as quite often more anomalies are present than only an
extra thumb.
syndromes have been associated with polydactyly.88 In syn­
dromes, there may be anomalies of the hands and feet.
Moreover, combinations of polydactyly in the hand can occur,
such as radial with ulnar, and central with ulnar. In the Greig
syndrome (cephalopolysyndactyly), polydactyly can occur in
any combination. For common syndromes associated with
polydactyly, see Table 28.4.
A separate entity is the mirror hand or ulnar dimelia. In the
forearm, two ulnae exist without a radius. Because the medial
side is a mirror image of the lateral side, the term mirror hand
became popular. It is extremely rare.75 The classic mirror hand
is unilateral, usually with seven to eight fingers without a
thumb, and is not combined with other anomalies. The etiol­
ogy is unknown. The inheritance pattern has not been found.
Geneticists believe it to be a spontaneous mutation.
History
Polydactylous hand and footprints have been found when
studying rock art and petroglyphs, some dated 1000 AD. The
first time that polydactyly was referred to in literature was in
the Old Testament, where in a battle in Gath, a giant had six
fingers on each hand, and six toes on each foot. The term
polydactyly is first described by Kerckring in 1670. Since then,
many reports have been made on polydactyly.
Basic science/disease process
Genetic aspects
In the patient with isolated radial polydactyly, a diminished
genetic penetrance exists, with a variable expression. Further­
more, in numerous families with inherited isolated dominant
radial polydactyly and triphalangeal thumb, a connection
with chromosome 7q36 is demonstrated. In this particular
Table 28.4  Common syndromes associated with polydactyly
Syndrome
Hand difference
Concurrent symptoms
Radial polydactyly
Holt–Oram
Hypoplastic thumb to bifid thumb
Different anomalies of the upper limb,
unilateral or bilateral
Cardial abnormalities
Fanconi anemia
Variety of radial differences of the hand
Radial dysplasia
Pancytopenia, abnormalities of heart, lungs, kidneys and digestive tract,
deafness, eye/eyelid problems, short stature
Townes–Brocks
Polydactyly
Imperforate anus, abnormally shaped ears, kidney abnormalities, hearing
loss, heart defects, genital malformations
Ulnar polydactyly
Bardet–Biedl
Ulnar polydactyly in majority of cases,
might be in combination with syndactyly
Poor vision, reduced or loss of smell, cardiovascular disease, urogenital
disease, mental and growth retardation, obesity
Smith–Lemli–Opitz
Polydactyly
Syndactyly of second and third toe
Microcephaly, mental retardation, malformations of the heart, kidneys,
gastrointestinal tract and genitalia
Trisomy 13
Polydactyly
Abnormal palm pattern, overlapping
fingers over thumb
Microcephaly, mentally and motor challenged, eye defects,
meningomyelocele, rocker-bottom feet, cutis aplasia, cleft palate,
urogenital defects, heart defects
Combined polydactyly
Greig syndrome
Variety of polydactyly with or without
syndactyly, broad thumbs
Ocular hypertelorism, macrocephaly
Polydactyly of the feet (medial and/or lateral)

639
Polydactyly
Ulnar polydactyly
Ulnar (postaxial) polydactyly classifications include either
two or three types. In the two-stage classification according
to Temtamy and McKusick, type A comprises an extra little
finger at the MCPJ, or more proximal including the CMCJ.
The little finger can be hypoplastic or fully developed. Type
B varies from a nubbin to an extra, non-functional little finger
part on a pedicle. In the three-type classification, type I
includes nubbins or floating little fingers, type II includes
duplications at the MCPJ, and type III includes duplications
of the entire ray.90,93,94
The most common presentation of ulnar polydactyly is a
small appendix on a skin pedicle, in which a neurovascular
bundle is present. The appendix, in most cases attached at the
ulnar border of the proximal phalanx, has mostly a small nail
and thus a distal phalanx, and is non-functional. Sometimes
two bones are present. If the extra little finger is more devel­
oped, the more common site of duplication is at the MCPJ. In
these cases, the fifth metacarpal is broad. Depending on the
extent of development and the attachment at the MCPJ, the
extra little finger flexes and extends well. Full motion of
the MCPJ of the normal fifth finger can be affected. Flexor
tendons and extensor tendons are usually Y-shaped and
asymmetrical, as in the duplicated thumb. The extra finger is
usually abducted at the MCPJ and radially deviated at the
PIPJ. The hypothenar muscles are attached at the ulnar side
of the extra digit and palmarly at the broad MCPJ.
If the extra little finger is at the base of the metacarpal or
at the CMC, the joint of the finger is usually well developed.
Physical examination of ulnar polydactyly depends on the
development of the extra little finger. Nevertheless, the
same rules apply, as described in the examination of radial
polydactyly.
Central polydactyly
In central polydactyly, the second, third and/or fourth
digit can be involved in the duplication. Fully developed
extra independent fingers are rare. In order of appear­
ance, the most frequently affected is the ring finger, fol­
lowed by the long finger, and ultimately the index finger.
Frequently, the duplication of the fourth finger is partial
The examination is performed from proximal to distal,
starting with examination of the thenar muscles. The thenar
musculature varies widely from normal to severely hypoplas­
tic. In the Wassel I and II, the thenar musculature is mostly
normal, in contrast to the Wassel V, VI, and VII. Hypermobile
joints should always be related to the other joints in the hands.
It is important to look for creases both dorsally and palmarly.
If creases are present, then an active movement in that par­
ticular joint can be expected.
The CMCJ in polydactyly can be normal, stiff or hypermo­
bile. If abnormalities in the CMCJ are present, they are mostly
encountered in the more proximal polydactylies. If polydac­
tyly is situated at the CMCJ, the MCPJ in the best thumb can
be near normal. In these cases, the movement is dependent
on the presence of a syndactyly between the duplication.
Depending on the location of the polydactyly, the MCPJ
can be stiff, normal moving, or hypermobile and hypoplastic.
For instance, in a polydactyly involving the MCPJ, quite often
the duplication moves as a block. In most of these cases, the
radial-sided thumb is hypoplastic and stiff, and the ulnar
thumb is the better one, often moving well at the IPJ. Finally,
the IPJ can present with normal movement, stiffness, or
hypermobility. If the duplication is at the IPJ, both parts can
move as a block. The range of motion in those cases is typi­
cally less than in a normal IPJ. In an asymmetric duplication
at the IPJ, the best-developed part usually moves better.
Normal examination includes extrinsic and intrinsic move­
ment. In a newborn, it is often difficult to distinguish between
these movements. However, flexion and extension can be
evaluated, as well as the presence of palmar abduction. In
duplicated thumbs, the flexor pollicis longus is Y-shaped in
the majority of cases, with a less developed tendon to the most
hypoplastic thumb. Therefore, flexion can be seen simultane­
ously in both thumbs. The extensor apparatus is usually less
developed or absent in the more hypoplastic thumb. It can be
Y-shaped as in the flexor and asymmetrically attached, there­
fore, deviating the distal part. The fingertips can be either
normal or asymmetric. The asymmetric side is typically found
on the opposing sides of the two thumbs. The nails are smaller
and asymmetrical in most cases. The first web is nearly always
normal in the distal duplications. In more proximal polydac­
tylies, the first web can be narrower than the normal contra­
lateral side.
Fig. 28.11  Wassel type I–VII.
I
II
III
IV
V
VI
VII

640
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
The surgical technique of Wassel type II and type IV will be
discussed further, as they are the most frequent types.
Radial polydactyly at the IPJ (Wassel type II)
Wassel type II thumbs usually have a broad proximal phalanx
with two (partial) distal phalanges with varying degrees of
fusion of the bones or epiphyses (Fig. 28.12). In the case of an
unequal duplication, it is straightforward to select the smaller
thumb to be excised. The incision can be made dorsally in a
zigzag manner for good exposure. The authors prefer a more
lateral incision because it leaves less conspicuous scars. First
the nail and nail bed of the smaller thumb is excised and then
the skin over the extensor tendon is developed, if present.
At the lateral side distal to the IPJ, the collateral ligament with
periosteum or a small piece of cartilage is dissected proxi­
mally. The IPJ is then opened and inspected. The flexor tendon,
if present, is identified and preserved and released distally. In
excising the extra phalanx, the cartilage can be cut intra-
articularly at the junction, corresponding to the former two
thumbs. This leaves a surplus of skin, a proximally attached
ligament, either (or not) an extensor and flexor tendon, and a
broad proximal phalanx. On the side of the excised distal
phalanx, a longitudinal wedge of bone from the articular
surface of the proximal phalanx is removed, taking care not
to remove the collateral ligament. If necessary, a transverse
wedge is removed distally leaving the IPJ surface intact and
subsequently placing the IPJ perpendicular to the axis of the
proximal phalanx. Then the extensor and flexor of the remain­
ing distal thumb are inspected. Mostly, the smaller part of the
flexor will be excised at the border of the remaining flexor
tendon. The smaller extensor tendon can be used to augment
the extensor tendon of the remaining thumb. This extra exten­
sor tendon is best used as a graft and not left in continuity
with the extensor tendon to the remaining (ulnar) thumb. If
the extensor tendon is used to reinforce the collateral liga­
ment, it could influence extension of the remaining thumb.
The collateral ligament is attached to the remaining distal
phalanx. The remaining skin is carefully inserted on the lateral
side, taking care to augment the deficient pulp on that side,
while bulkiness of skin should be prevented.
In the event of two equal hypoplastic distal thumbs, which
are fortunately less frequent, the two outer halves can be
joined after excising the two inner halves longitudinally,
in the Bilhaut–Cloquet operation.95 Instead of the Bilhaut–
Cloquet operation, the authors prefer a good smaller nail than
a larger nail with a broad ridge, which draws attention.
Therefore, one nail and nail bed is removed in nearly all cases
and the usually insufficient pulp is augmented by the radial
pulp of the excised thumb. In rare cases, a modified Bilhaut
procedure can be used, augmenting a curved nail by elevating
the nail bed from the bone, and supporting the radial side by
bone from the radial (removed) thumb.
Radial polydactyly at the MCPJ (Wassel type IV)
Wassel type IV thumbs often demonstrate a hypoplastic or
smaller extra thumb on the radial side. The operative tech­
nique has great similarities with the operation at the IPJ (Fig.
28.13). The radial thumb is excised preserving the radial col­
lateral ligament with some cartilage, and after dissecting the
extensor and flexor tendons, the joint is inspected. The broad
and hidden by a syndactyly, typically to the third finger.
A number of varieties are possible because the duplication
often is not confined to one finger. In addition to the bony
deformity and aberrant growth plates, anomalies of the
flexor and extensor tendons and neurovascular structures
are present.
In ulnar dimelia (mirror hand), the hand looks very pecu­
liar and is restricted in function due to the lack of a thumb
and the presence of multiple digits. Seven to eight fingers are
present without a thumb. Syndactyly can occur. The wrist is
broad and mostly flexed. The arm is shorter with a broad
elbow, an extension deficit, and restricted forearm rotation
and shoulder movement.
Patient selection
Polydactylies are usually treated surgically. Functional
impairment can vary from slight to severe, depending on
the extent of the deformity. Polydactylies can be a nuisance
in, for example, shaking hands, putting hands in pockets
or narrow spaces, and wearing gloves. However, most
parents visit the outpatient clinic with their child for aes­
thetic and social reasons. In patients with a syndrome with
serious concomitant disease, surgery can be delayed or even
be avoided.
Treatment/surgical technique
The timing of surgery is arbitrary. Surgery is recommended
at the end of the first year of life by many authors, as it is
important to be able to identify the structures properly and
to minimize the anesthetic risks. Floating little fingers are
often removed early in the newborn nursery under local
anesthesia.
Knowledge of patho-embryology and patho-anatomy is
essential to obtain long-term good results. Try to visualize
aberrant anatomy without too much dissection!
Hints and tips
Radial polydactyly
Principles in treatment of radial polydactyly are:
• “Make one thumb from two, do not simply excise one.”
• Decide which thumb should be kept.
• Do not just excise the most hypoplastic thumb but preserve
tendons, ligaments and skin to align, balance and augment
the residual thumb.
• At the first operation as much correction as possible is
performed on both soft tissues and bones.
• Always be aware and search for less obvious anatomical
anomalies, besides the extra digit.
• Align articular surfaces as axial and as congruent as
possible by transverse and longitudinal osteotomies.
• Perform ligament reconstructions or releases.
• Balance tendon insertions.
• Adjust skin cover as accurately as possible.
• Postoperative dressings should be meticulously applied
and resistant to removal by the child.

641
Polydactyly
Fig. 28.12  (A) Wassel type II operative
technique. (B,C) Wassel type II
preoperatively and (D,E) postoperatively 2
years later (same child bilateral).
A
B
C
D
E

642
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
Fig. 28.13  (A) Wassel type IV operative technique. (B,C) Wassel type IV preoperatively and (D) Wassel type IV, at 15 years follow-up.
1
2
3
4
A
B
C
D

643
Polydactyly
distal metacarpal is treated in the same way as the proximal
phalanx in the IPJ in the Wassel type II, by excising a portion.
The proximal phalanx is then aligned after exploring the
flexor and extensor tendons of the remaining thumb. The
flexor tendon can be asymmetrically situated without pulleys.
Asymmetric tendons should be realigned in the “ulnar”
thumb. If deviation at the IPJ is present, it should also be
corrected with a transverse wedge osteotomy at the distal end
of the proximal phalanx, as it will not adjust spontaneously
during growth. In diamond-shaped polydactylies, IPJ correc­
tions are always necessary. In equal-sized thumbs, the ulnar
thumb is usually preserved as the ulnar collateral ligament is
more important. The radial collateral ligament is subsequently
reconstructed, providing stability on the new radial side of
the MCPJ. With the radial collateral ligament, the thenar
musculature is also reattached. Again, skin coverage of the
radial side should be meticulously adjusted to the residual
defect. Adjustment of the first web is rarely necessary in
Wassel type II and IV.
Ulnar polydactyly
In the floating little finger type, the pedicle is excised at its
base and the neurovascular bundle is buried to prevent
painful neuromas in the scar (Fig. 28.14). It can be performed
in the newborn nursery under local anesthesia. Care should
be taken to avoid residual polydactyly at the base as this can
occur when a ligature is used around the pedicle. Revisions
may be necessary later in life To prevent this residue, the use
of vascular clips has been introduced. These can be positioned
flush to the skin of the little finger, only leaving a scar. If a
more developed extra little finger exists, the same principles
apply as for radial polydactyly.
Central polydactyly
The basic problem in central polydactyly, in the majority of
cases, is the often bizarre configuration of the skeleton with
duplication and aberrant bones hidden by syndactyly. As the
variety in presentation is extensive, basic rules should be
followed (alignment of bones and articular surfaces) and, if
possible, balanced by available tendons. However, involved
fingers are insufficient or hypoplastic, and the joints are stiff.
Syndactyly releases should be performed only if the fingers
will obtain sufficient support (Fig. 28.15).
Mirror hand
Reconstruction in the very rare mirror hand can include elbow
correction, osteotomies at the forearm to improve supination,
and correction of flexion deformities at the wrist. The hand
mostly involves resecting two or three fingers on the lateral
side making a thumb of the best finger (usually the extra long
finger) and creating a sufficient first web.90
Postoperative care
The reconstructed hand following polydactyly is immobilized
for 6 weeks, allowing finger movement in the bandage.
Following the initial 6 weeks, and depending on the level
and complexity of the reconstruction, further treatment
consists of uninhibited full motion to a removable splint for
several weeks. Hand exercises under supervision are seldom
necessary. In the simple ulnar polydactyly, a bandage is given
for 2 days, as in regular wound care.
Outcomes, prognosis, and complications
When reconstruction in a congenital hand is performed,
definitive outcome can only be assessed after growth has been
completed. Therefore, it is advisable to check children several
times during growth. At our outpatient clinic, examination is
performed as described (Table 28.5). Specific attention is paid
to nail deformities, movement and stability of joints, active
range of motion, and appearance, broadness and malalign­
ment of the skin. Older children and parents hardly complain
of lack of function even though joints can be stiff. Painful
thumbs are very rare. However, they do complain about
appearance, often masked by an insignificant functional
complaint. Teenagers often visit the outpatient clinic wanting
the aesthetic outcome to be improved.
Outcome can be divided into unavoidable and avoidable
results. In the unavoidable outcome in Wassel type II, the
distal part of the thumb can be from nearly normal to hypo­
plastic, depending on the initial presentation. Insufficient
pulp, smaller nails, and less developed IPJs are inevitable. In
nail bed reconstructions, the nail will never be completely
normal.
Table 28.5  Polydactyly: sequence of examination of the first ray
Feature
To be noted
Aspect
Appearance
Normal/hypoplastic
Alignment
Straight/deviated
Position
Flexed/extended
Skin
Creases
Normal/absent at joint(s)
Fingertip
Normal/asymmetric pulp
Syndactyly
Complete/incomplete
1st-web
Normal/narrow
Nails
Nails
Normal/asymmetric/broad/small
Joints
CMC, MP, IP
Stable/hypermobile/unstable
Normal/impaired movement/stiff
Bones
MC, P1, P2, (P3)
Normal/broad/slender
Muscle/tendon
Thenar
Normal/hypoplastic
Extension
Present/absent/aberrant
Flexion
Present/absent/aberrant
Adduction
Present/absent/aberrant
Palmar abduction
Present/absent/aberrant
Radial abduction
Present/absent/aberrant
CMC, Carpometacarpal joint; MP, metacarpophalangeal joint; IP, interphalangeal
joint; MC, metacarpal; P1, proximal phalanx; P2, middle phalanx; P3, distal
phalanx.

644
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
Fig. 28.14  (A,B) Ulnar polydactyly at metacarpophalangeal joints, preoperatively with X-ray, and (C) at 4 years follow-up.
A
B
C
A
B
Fig. 28.15  Bilateral central polydactyly with syndactyly (synpolydactyly):
(A,B) preoperative right hand and X-ray; and (C) at 6 years follow-up, of both
hands.
C

645
Triphalangeal thumb
■ Opposition transfers (sometimes in the more proximal
Wassel types)
■ Nail deformity correction
■ Osteotomy and alignment of articular surfaces
■ Neurolysis and neuroma treatment in adult patients
■ Arthrodesis of IPJ or MCPJ
■ Scar revision.
Triphalangeal thumb
Introduction
The general incidence is estimated by Lapidus as being 1 in
25 000 live births.99 Of all patients with a triphalangeal thumb
(TPT), two-thirds have a family history of thumb abnormali­
ties. They mostly have bilateral deformities and have
non-opposable thumbs.3 TPT can be inherited as an autosomal-
dominant trait.100 Sporadic cases are mostly unilateral and
supposed to be opposable. In our series, which comprised
many inherited cases, thenar muscle deficiency varied consid­
erably. This muscle deficiency is related to the degree of
opposability of the thumb.101 In the Netherlands, the incidence
of TPT is probably higher than stated in most reports, due to
a number of families with an autosomal-dominant trait.
History
A thumb with three phalanges was first reported by Columbi
in 1559,75 and later by Dubois in 1826.102 Many authors have
proposed treatment ranging from nothing to extensive surgery.
Basic science/disease process
By combining family pedigrees, the inheritance pattern of TPT
is as an autosomal-dominant trait.100 The location of the gene
has been discovered on chromosome 7q36 in 1994.103
Diagnosis/patient presentation
A wide variety of phenotypes can occur with TPT. Anatomical
differences are not only restricted to the extra phalanx, there­
fore a thorough examination should be performed of both
upper extremities.
The extra middle phalanx is a constant finding in TPT. Its
shape can vary from a very small wedge-shaped extra phalanx
resulting in a deviation at the IPJ to a fully developed extra
phalanx resulting in a long thumb. Other features can be a
near normal to very hypoplastic thenar musculature, a near
normal to contracted and less deep first web, and an extra
partial or fully developed ray. The thumb can be in the same
plane as the other fingers with a finger-like appearance, hence
the name of five-fingered hand in some of these cases.
In TPT, both IPJs are mobile. In the small wedge-shaped
middle phalanx, no clinical distinction can be made in move­
ment at the proximal or distal IPJ. When the middle phalanx
is trapezoidal or rectangular, often active movement is pos­
sible at both joints.
The MPJ can be stable to very unstable; hyperextension at
this joint is common. The CMCJ can vary from hypoplastic
to malformed, resulting in variations from a less mobile to
an unstable joint. The CMCJ can also be absent. Also the
Fig. 28.16  Z-deformity following resection of only radial extra ray without further
correction in unilateral Wassel type IV.
Avoidable outcomes in Wassel type II relate to inappropri­
ate alignment, resulting in nail deformities, scarring at the nail
wall, and extra nail development. If the collateral ligament is
constructed with cartilage or a small piece of bone from the
excised phalanx, new formation of bone can be the result. At
the IPJ, incongruent alignment causes deviation, instability, or
stiffness. Too much resection at the base of the distal phalanx
damages growth of the remaining distal phalanx.
In the unavoidable outcome in Wassel type IV, the residual
thumb is always less developed than the normal contralateral
thumb.1 The pulp is often less developed, the nail is usually
smaller, and motion can vary from nearly normal to stiff,
especially at the IPJ.96,97 Goldfarb found only a difference in
nail width; however, motion was not taken into account in
this study. But even though the thumb appears smaller,
patients are satisfied as long as it is well shaped.97,98
Avoidable outcomes in Wassel type IV also relate to inap­
propriate alignment. IPJ and MCPJ angulation decreases the
aesthetic outcome.96,97 Deviations at either the IPJ or MPJ can
mostly be prevented at initial operation by aligning the articu­
lar surfaces and balancing the thumb properly. Too much
resection at the MPJ results in growth disturbances at the
proximal phalanx of the remaining thumb. In late S- and
zigzag deformities (Fig. 28.16), proper initial alignment has
not been accomplished. Residual unstable IPJs or MCPJs can
develop following improper ligamentous reconstruction at
the initial stage.
In ulnar polydactyly, a small painful mass can remain fol­
lowing ligation or inadequate resection of the pedicle of the
extra floating little finger. If the extra finger originates at
the MCPJ, full flexion at the MCPJ might not be possible as
the joint is not normally developed.
Secondary procedures
Growth can make secondary interventions advisable without
really being complications. Possible secondary procedures,
either or not in combination, can include:
■ Ligament reconstruction
■ Tenolysis
■ Tendon rebalancing

646
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
intrinsic and extrinsic muscle abnormalities. If TPT is com­
bined with polydactyly, further classifications are possible.
Wassel classified all possible combinations into Wassel VII.
Our group has developed a classification for TPT combined
with duplication or triplication, based on the Wassel classifi­
cation in which TPT is classified according to the joint
(Fig. 28.17).
Patient selection
In TPT, the patient’s precision grip is impaired as a result of
diminished opposition, joint instability, or additional length,
leading to alternative, compensating maneuvers. If syndactyly
or an extra thumb is also present, proper use of the hand is
further decreased. Children may compensate easily for these
drawbacks. Parents of young patients and older children
with TPT, however, often want the anomaly corrected for
trapezium and scaphoid can be hypoplastic, malformed, or
absent.
In inherited cases, differences are often bilateral. An extra
ray is common in these patients, and triplications may occur.
The ulnar thumb is nearly always the best developed in cases
of two thumbs.
In TPT, other malformations can also occur such as syndac­
tyly, ulnar polydactyly, cleft hand, longitudinal radial defi­
ciency, differences of the lower extremities, and as part of a
syndrome with abnormalities in one or more of the other
systems.104–106
The aforementioned makes classification of TPT difficult.
Wood107,108 classified TPT on the shape of the extra phalanx,
distinguishing three types of an extra middle phalanx in the
thumb: delta type, trapezoid type, and full rectangular type.
Buck-Gramcko created a classification with six types, based
on treatment options, including joint mobility, webspace,
Fig. 28.17  Our radial polydactyly and triphalangeal thumb classification. (Reproduced with permission from Zuidam JM, Selles RW, Ananta M, et al. A classification system
of radial polydactyly: inclusion of triphalangeal thumb and triplication. J Hand Surg Am. 2008;33:373–377.)
I
II
III
IV
V
VI
VII
VIII
T
Triplication
Tph
Triphalangism
H
Hypoplastic/floating
D
Deviation
S
Symphalangism
Triplication on different
levels associated with
triphalangism

647
Triphalangeal thumb
excises the short middle phalanx before the age of 6 years,
because if the child is older, the joint does not remodel
anymore. Wood,108 on the other hand, operates between 6
months and 2 years of age.
Authors’ preferred method of treatment126
Delta middle phalanx
In children < 6 years, resection of the extra phalanx is per­
formed under fluoroscopy control (Figs. 28.18 & 28.19). Sub­
sequently, the radial collateral ligament at the new IPJ is
released and the ulnar collateral ligament tightened. The skin
is released on the radial side with a Z-plasty and adjusted on
the ulnar side by excision of skin. When the child is older, a
corrective osteotomy with arthrodesis at the DIPJ is advised.
This latter technique can be also performed in the trapezoidal
middle phalanx.
Rectangular middle phalanx and five-fingered hand
Via a Y-shaped incision dorsally a reduction osteotomy of the
middle phalanx is made followed by arthrodesis of the distal
interphalangeal joint. In this method, a 1–1.5 cm shortening is
achieved. The skin is then closed as a “V”. As the shortening
is not enough, this procedure is combined with a shortening,
rotation, and palmar abduction corrective osteotomy at the
metacarpal level to correct for position and length of the
thumb (1–1.5 cm extra shortening). Furthermore, the extensor
tendons as well as the intrinsics are shortened. In recent years,
a volar plate tightening at the MCPJ has been added to prevent
hyperextension. Basically, this procedure resembles a pollici­
zation. The original CMC1J, even though it is not optimal, is
better and more stable than a reconstructed CMCJ in a formal
pollicization. For this reason, the author does not use a formal
pollicization in the five-fingered hand.
First web deficiency
Correction can mostly be performed with a simple four- or
five-flap Z-plasty. When more skin is needed, a flap from the
dorsoradial side of the index is used as described above. In
case of polydactyly, the extra skin from the radial discarded
extra thumb can be used to shift to the ulnar thumb, while the
skin of the ulnar thumb is used for the first web (Fig. 28.20).
Postoperative care
For postoperative care, see the section on radial polydactyly,
above.
Outcomes, prognosis, and complications
The authors’ group performed a long-term follow-up of oper­
ated (40 patients, 68 hands) and non-operated patients (15
patients, 29 hands). Patients scored their function and appear­
ance. The improvement in appearance was significant in the
operated group; the improvement in function was higher but
not significant. There was a trend towards a stronger thumb
in the operated group; however, no opposition transfer had
been performed.
Treatment of patients with TPT is not easy and often under­
estimated due to a lack of knowledge of possible deformities
that may occur; therefore, many of these are left uncorrected.
Skin problems, infection, and mal-union or non-union are
appearance and very rarely for functional deficits. Timing of
operation is not always clear in minor defects, such as small
triangular phalanges.
The authors’ series includes a high number of dominant
hereditary triphalangeal thumbs, with or without polydactyly.
The affected parent was often intensively teased at school and
therefore wished their child to be operated before going to
school.
Because of the presence of cortical representation of a
thumb, a hard indication to operate very early is not present,
as a new learning process is not necessary. In only rare cases
is a scissors grip between index and middle finger used. It is
advisable to operate on these patients before the age of 2 years
for early adaptation of the cortex to the newly created thumb.
Treatment/surgical technique
Bunnell109,110 advised not to operate on TPTs. Beatson111 and
Milch,112 among others,113,114 proposed removal of the abnor­
mal phalanx to reduce length and to correct deviation. After
removal of the extra phalanx, the risk of an angulated joint as
end result exists.115,116 Buck-Gramcko117 stated that resection of
the delta type extra phalanx and ligament reconstruction
in the young child gives adequate results in a single
procedure.
For patients with a trapezoidal or rectangular extra middle
phalanx, several types of osteotomies are described such as
resection of the distal joint and part of the distal phalanx
followed by arthrodesis116 to excision of the PIPJ.2 Buck-
Gramcko117 added an osteotomy of the first metacarpal
with reinsertion of the intrinsic muscles and widening of
the first web. If necessary, an opposition transfer was also
performed.
In the “five-fingered” hand, pollicization is advised.118 If
the first web needs only minimal deepening, a four-flap
Z-plasty119 can be used. Foucher120 described a W-transposition
flap in which skin and subcutaneous tissue is used from the
dorsoradial side of the proximal phalanx of the index to
provide skin for the first web. A large rotation flap of the
dorsum of the hand can be used in a non-opposable TPT
where, in addition to deepening, lengthening of the first web
is required.117,121 In larger deficiencies of the first web, where
the ray is placed in palmar abduction (such as in patients with
a five-fingered hand), Upton122 favors a distally based radial
forearm fasciocutaneous flap.
An opposition transfer can be performed if hypothenar
muscle deficiency exists. The most used tendon transfers for
opposition transfer are the abductor digiti minimi muscle
(Huber technique123), the superficial flexor of the ring finger,114
and the flexor carpi ulnaris.124
When TPT is accompanied by radial polydactyly, Wassel89
and Wood115 advised extirpation of the TPT even if the other
biphalangeal thumb is more rudimentary. Wood transposed
the rudimentary biphalangeal thumb to the base of the meta­
carpal of the TPT, creating a shorter thumb. This transposition
can be combined with a metacarpal osteotomy. Upton,125 on
the other hand, excises the rudimentary biphalangeal thumb,
retains the triphalangeal thumb, and corrects the TPT. No
clear evidence exists regarding the best technique as most
published series are small.
Timing of operation differs not only for the several types
of TPT but also among various authors. Buck-Gramcko117

648
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
A
B
Fig. 28.18  (A,B) Operative technique of extirpation of the middle phalanx in the young child (note the skin and ligament correction on both sides).
very rare. In our experience of treating patients with TPTs, one
of the problems is unpredictable growth of the thumb, espe­
cially of the first metacarpal after initial shortening. This
results in longer thumbs than intended (method of measur­
ing: thumb tip is distal to the PIPJ of the index).127 Parents and
older children should, therefore, be informed. Furthermore,
the arthrodesis of the distal IPJ can give a nail deformity. The
V-Y approach can avoid this when performed with caution in
relation to the nail bed.
Secondary procedures
Secondary procedures that are not directly related to compli­
cations include an opposition transfer to improve opposition
strength. Not many patients feel this operation is necessary.
Thumb length corrections can be performed using epiphysio­
desis or shortening osteotomies when growth has stopped.
Occasionally, volar plate-tightening procedures are offered for
hypermobile MCPJs.

649
Triphalangeal thumb
Fig. 28.19  (A,B) Preoperative triphalangeal thumb with delta phalanx and (C) postoperative result following resection of the middle phalanx of the thumb and ligament
reconstruction; note the reduction in length.
A
B
C
Fig. 28.20  (A) Right hand preoperatively in bilateral polydactyly with triphalangeal thumb. (B) X-ray with
one side operated following reduction–arthrodesis at the distal interphalangeal joint and reduction–rotation
and abduction osteotomy at the first metacarpal with shortening of the intrinsic and resection of the radial
ray. (C) Long-term follow-up of correction on right side.
A
B
C

650
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
2. The adolescent, mostly female, with sudden onset or
increasing camptodactyly of mostly only the fifth finger
following growth spurts.
3. Severe camptodactyly associated with other
malformations.138,139 However, it is disputed by others if
this group should be diagnosed as camptodactyly.132
Foucher et al.140 recognized four conditions: (Ia) the early and
stiff; (Ib) the early and correctable camptodactylies; (IIa) the
late and stiff; (IIb) the late and correctable deformities. Tests
to assess degree of severity of camptodactyly are as follows140:
1. Can the PIPJ be actively extended while the wrist is
held in a neutral position?
2. Does skin blanching appear while extending the finger
as much as possible at both the MPJ and PIPJ?
3. Does the PIPJ flex when the wrist and MCPJ are
extended (the superficial flexor tenodesis test)?
4. Can the PIPJ extend passively by tenodesis effect while
the MCPJ and wrist are held in flexion (central band or
Smith test)?
5. Can the PIPJ actively extend while the MCPJ is held in
slight flexion (the Bouvier maneuver)?
6. Is the flexor digitorum superficialis (FDS) of the little
finger independently working from the fourth finger?
Patient selection
The best patient to operate on is the patient who has a supple
PIPJ but cannot actively extend the PIPJ well. Furthermore,
patients should be selected on the basis of expected compli­
ance to hand therapy.
The older patient with a stiff PIPJ is a bad candidate for
release of the PIPJ with the intention to obtain a reasonable
moving PIPJ.
Treatment/surgical technique
A splint is applied for all patients with camptodactyly in
which the PIPJ is not supple (Fig. 28.21). The splint stresses
the PIPJ gradually to as much extension as possible. The MCPJ
is in flexion in a forearm splint. A reduction to < 30° extension
lag is considered good enough to refrain from surgery.
The duration of splinting therapy before further surgical
treatment varies from 3 to 12 months. Some authors consider
an extension lag of 60° as an indication for surgery.132
If the splinting program demonstrates a passively improved
joint without improved active extension or if the joint move­
ment is not improved, surgery can be indicated. In the older
patient, one should think twice before surgery is performed,
especially when joint movement is not improved. In non-
compliant patients or parents, surgery is not recommended.
A variety of operations has been proposed as the etiology
is unclear. Operations range from skin plasties, arthrolysis,
tenotomies, tendon transfers, and osteotomies to arthro­
desis. The authors’ preferred technique is based on the
algorithm and description of the operative technique by
Foucher.132,140
In the correctable PIPJ, mostly skin and fibrous bands
release are performed, with exploration of the lumbrical
muscle, interosseus muscles, FDS and flexor digitorum pro­
fundus (FDP). Very often, the lumbrical is anomalous together
with the FDS (Fig. 28.22). If the FDS is usable, and pulling on
Camptodactyly
Introduction
Camptodactyly is a contracture of the PIPJ in the anteropos­
terior direction (campto = arched; dactylos = finger). It occurs
as consequence of an anatomical imbalance of the extrinsics
and anomalous insertion of intrinsics to the affected finger(s).
Camptodactyly can be sporadic without previous family
history. Familial cases can inherit in an autosomal-dominant
pattern. In about 30% of cases, camptodactyly has a familial
background. This condition can furthermore be present in
many syndromes such as Holt–Oram syndrome and Poland
syndrome. Prevalence can vary considerable from 1–24%.128
Camptodactyly usually stops being progressive with skeletal
maturity.
History
The description of camptodactyly was first introduced by
Tamplin in 1846.129 One of his pupils, Adams, considered the
skin deficiency on the palmar side as a secondary effect.130 The
term “camptodactyly” was first used by Landouzy in 1906.131
Depending on the author, camptodactyly can either be only
an isolated PIPJ flexion contracture or also include those
fingers where the PIPJ cannot be actively extended but have
supple joints.132 All structures surrounding the PIPJ have been
associated with the pathogenesis of camptodactyly. Attention
has been drawn to an imbalance of the flexor and extensor
mechanism.133,134 Subsequently, the role of the anomalous
lumbrical insertion to the superficial flexor was described as
a consistent finding.135–137 Most authors describe the bone
changes of the joint and shortage of the skin as secondary to
the contracture.
Basic science/disease process
As a consequence of the imbalance of flexors and extensors,
flexion at the PIPJ occurs; this can lead to deformation of the
intra-articular bone ends with a stiff joint as a result, and
deficient skin on the palmar side. With increasing age, the
extensor mechanism becomes more insufficient, leading to a
further extension lag. Other mechanisms have also been
described, such as circulatory disturbance, skin shortness,
subcutaneous bands, lumbrical abnormality, short flexor
superficialis, shortness of the flexor profundus, and retraction
of the collateral ligaments and volar plate.
Diagnosis/patient presentation
The functional defect is mostly limited, and therefore appear­
ance is mainly the concern. In severe forms however, func­
tional deficit can be substantial, especially when more fingers
are affected. The fifth finger is the most affected (> 70%), fol­
lowed by the fourth finger (< 20%). The other fingers are
involved in less than 10% of patients with camptodactyly.
Patients with camptodactyly can be divided into three
groups:
1. The newborn male or female patient with
camptodactyly of mostly the fifth and/or fourth finger
and sometimes all fingers.

651
Clinodactyly
Postoperative care
Postoperative care consists of a splint, the duration of which
depends on the technique used. A K-wire for 2 weeks can be
used for the PIPJ following release of the joint. Compliance to
hand therapy is essential.
Outcomes, prognosis, and complications
In the young child, long-term splinting during the night of the
stiff PIPJ results in a good outcome, with an 80–92% improve­
ment rate after long-term splinting.141,142 In the Caucasian
hand, these good results could not be achieved in the same
way. Young children with stiff joints do react well with splint­
ing, resulting in a mean improvement of 40° following 19
months of splinting.139,140 The variety of operations that has
been proposed inevitably results in various outcomes ranging
from 14% to 35% improvement.132,143 Because of this insuffi­
cient outcome, and even sometimes worsening of the contrac­
ture, some authors prefer to rely only on splinting.
Foucher created an algorithm in which patients presenting
late with a stiff joint are not operated. In all other patients,
splints were applied. If no satisfactory improvement in
extension was present, surgery was performed resulting in
improvement rates from 68% to 88%, depending upon whether
or not the joint was passively correctable.140 Complications
can include: insufficient possibility to extend the PIPJ after
surgical treatment, osteoarthritis, recurrence of contracture,
and stiffness and pain.
Secondary procedures
Secondary procedures include: re-release of capsular contrac­
ture, corrective osteotomy, or formal PIPJ arthrodesis.
Clinodactyly
Introduction
Clinodactyly is reserved for radioulnar deviations in the hand
and lateromedial deviations in the foot. The angle of deviation
defining clinodactyly varies among authors from > 8° to
A
Fig. 28.21  (A) Note the flexed proximal interphalangeal joint of digit 5 and less of
digit 4 before splinting. (B) After long-term night splinting.
B
Fig. 28.22  The lumbrical muscle inserts into the flexor digitorum superficialis at
the A1 pulley.
the lateral band gives extension to the PIPJ, an FDS tendon
transfer to the lateral band will be performed.
In the non-correctable PIPJ, which improves after splinting,
an extensive skin release is performed with a Malek flap
(proximally based palmar homodigital flap). Subsequently,
the flexor sheath just proximal from the PIPJ is opened trans­
versely and the checkreins, accessory ligament, and if neces­
sary the palmar plate is released creating passive extension.
The intrinsics and extrinsics of the operated finger are explored
and released if an anomaly is present. An FDS tendon transfer
is used from the same or otherwise adjacent finger to restore
active extension. The transfer is attached to either the lateral
band, if sufficient, or to the central band, if the lateral band is
not sufficient.132,140

652
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
The thumb is next in line in frequency of occurrence
(Fig. 28.23). The thumb can be an isolated occurrence or as
part of a syndrome. The angle of deviation can amount to 70°.
In Apert syndrome, the thumb can be severely deviated. The
fingers are less involved and, in most series, the least affected
is the long finger. More than one digit can be affected. Over
half of the patients present with a delta phalanx. As a consid­
erable number of patients present with clinodactyly after
epiphyseal closure, the presence of a delta phalanx is difficult
to assess radiologically. X-rays of both hands and affected
fingers in two directions are a prerequisite in making the
diagnosis.
Patient selection
Clinodactyly in the little finger without scissoring is not often
treated operatively because of the risk of impairing joint
motion in a normal functioning finger. In those patients where
function is impaired, an indication to operate can exist. In
a more severe deviation with a longitudinally bracketed
epiphysis, age of surgery depends on the technique. Resection
of the lateral physis with interposition of fat is performed at
a younger age than a formal wedge osteotomy with or without
bone graft. In syndromic clinodactyly, correction of clinodac­
tyly depends very much on the associated abnormalities.
Treatment/surgical technique
Treatment for clinodactyly can be divided into the following
procedures:
■ Opening wedge osteotomy with or without bone graft
■ Closing wedge osteotomy
■ Reversed wedge osteotomy
■ Release of soft tissues on the concave side
> 15°.144,145 Clinodactyly is a symptom and not a disease. It may
be isolated or may be associated with other congenital mal­
formations, such as in Down syndrome or Apert syndrome.
Burke described 25 syndromes associated with clinodactyly.146
The incidence varies from 1–19.5%, depending on the degree
of angulation used to determine clinodactyly.147 Depending on
the inclusion criteria, different groups can be distinguished:
familial clinodactyly usually without associated differences
(brachymesophalangism); clinodactyly with associated mal­
formations; asymmetric injury to the growth plate; and
triphalangeal thumb.
Brachymesophalangism is the most frequently encountered
form of clinodactyly. It is the result of an articular surface that
is not perpendicular to the longitudinal axis due to the abnor­
mally shaped bone. The phalanx looks like a delta phalanx
without the osseous rim as in the delta phalanx with a
C-shaped epiphysis. The middle to distal phalanx ratio is 1 : 1,
compared with 1.3 : 1 in normal fingers.148
Second, an aberrant growth plate surrounding one side of
the delta phalanx in a “C” shape results in deviation at the
involved joint(s). Other names for delta phalanx are triangular
bone and longitudinally bracketed epiphysis.149–151 Reports
suggest that anomalies with a delta phalanx should be clearly
distinguished from the classical clinodactyly.152 As it is not
clear, the authors would reserve clinodactyly for a congenital
difference with or without a C-shaped epiphysis. Angulations
after injury to the growth plate in an otherwise normal finger
or a deviation in a joint following syndactyly release are
secondary conditions.
History
Early reports exist on curvatures in the finger in the dorsopal­
mar plane.153 Clinodactyly, however, refers to an angulation
in the radioulnar plane. Smith described the condition in
mongolism in 1896.146 As clinodactyly is a physical sign, defin­
ing this group is difficult and has been variably described
over the past century.
Basic science/disease process
Clinodactyly is considered to be a congenital deformity at
the phalangeal level. The mode of inheritance of clinodactyly
is as an autosomal-dominant trait with incomplete pen­
etrance.154 The middle phalanx is ossified later in embryonic
development than the other phalanges, and latest in the little
finger. Brachyphalangism therefore appears most in the little
finger.155 In proximal phalanges, however, aberrant growth
plates also occur resulting in clinodactyly and brachyphalan­
gism, and often in selected fingers. The exact etiology is still
unknown.
Diagnosis/patient presentation
The most common form of clinodactyly is seen in the middle
phalanx of the little finger presenting with an inward devia­
tion of more than 10°. A positive family history in these
patients is frequent. These little fingers mostly do not scissor
over or under the ring finger, making this deformity more an
aesthetic than a functional problem. Scissoring can also be
prevented by abducting the little finger in the MCPJ. The
deviation is clinically not only visible at the PIPJ but also at
the DIPJ.
Fig. 28.23  Clinodactyly of the thumb.

653
Future directions
In 1987, Vickers described a new technique by excising the
continuous region of the physis and diaphysis in the isthmic
region of the middle phalanx via a lateral incision. The bone
is further removed in this region with a burr or curette. The
remaining cavity is filled with a fat graft. This operation is
called physiolysis and works well in younger children (under
the age of 6 years), with less severe clinodactyly, and a trap­
ezoidal phalanx.158,159
Postoperative care
In the physiolysis technique, the operated finger can be taped
to the adjacent finger for approximately 2 weeks. In the closing
wedge technique, 3 weeks of immobilization is enough. In the
reverse and open wedge osteotomies, also depending on
the soft-tissue correction, 4–6 weeks immobilization is often
carried out.
Outcomes, prognosis, and complications
Reversed wedge osteotomies normally heal well. Slight angu­
lations in the dorsal–palmar plane following surgery mostly
disappear during growth. In open wedge osteotomies, skin
and ligament release can be insufficient, leading to skin tight­
ness and recurrence of angulation. In physiolysis, results in
children over 5 years are not as good as in younger children.
Furthermore, it works well in less severe clinodactylies.
Complications of the bone can be mal-union or malrota­
tion. Non-union in children is very rare. Even if the X-ray
demonstrates a non-consolidated osteotomy, the fibrous tissue
bridging the two parts can prevent movement, and will have
no clinical implications. Furthermore, extensor tendon adhe­
sions can result in a mallet-like deformity. In some cases,
stiffness of joints can occur. Pain is rarely an issue.
Secondary procedures
Secondary procedures mainly address recurrence of the devia­
tion either by insufficient release or excision of the physis or
by inadequate soft-tissue release. A new osteotomy and soft-
tissue release can be performed.
Future directions
The OMT classification
When classifying congenital hand and upper limb differences,
the Oberg, Manske, Tonkin (OMT) classification is used. This
classification was first introduced in 2010,160 and was approved
by the Scientific Committee for Congenital Conditions of the
International Federation of Societies for Surgery of the Hand
(IFSSH)161 to substitute the Swanson classification.162 Since
2010, the OMT classification has been subject to assessments
with subsequent modifications, the last being published in
2015.163 At this moment, the OMT classification is still a flex­
ible and fluid classification, adapting to new developments
and additional conditions.
The OMT classification is designed to accommodate
the current knowledge of limb embryogenesis, classifying
congenital hand and upper limb differences in one of
three groups: malformations, deformations and dysplasia
(Fig. 28.25). The malformations group comprises the most
■ Tightening of soft tissues on the convex side
■ Physiolysis.
Although a closing wedge osteotomy is advised for moderate
and severe deviations (> 30°),156 the authors are not in favor of
using this technique, as the affected finger will be further
shortened.157 An open wedge osteotomy lengthens the digit
the most and is preferred. In the more severe deviations,
however, bone correction only will not be enough. Release of
soft tissue (collateral ligaments and the skin) on the contracted
side is also necessary. The thenar musculature at its insertion
in the radially deviated thumb should also be released,
otherwise recurrence is inevitable. Tightening of soft-tissue
structures, especially collateral ligaments on the convex side,
can also be useful.
In the less severe deviations, a reverse wedge osteotomy
works well but is technically more demanding (Fig. 28.24).
The advantage of the reverse wedge is that no other donor
site is necessary. In the open wedge technique and in the
reverse wedge technique, the graft is transfixed with a percu­
taneous K-wire either with or without a suture to hold the
bone graft in position.
A
Fig. 28.24  (A) Reversed wedge osteotomy of the ring finger preoperatively and
(B) postoperatively, the wedge is placed at the radial side of the proximal phalanx.
B

654
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
Fig. 28.25  Oberg, Manske, and Tonkin (OMT) classification. (Reproduced with permission from Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and classification of congenital anomalies of the hand
and upper extremity. J Hand Surg Am. 2010;35:2066–76; and Ezaki M, Baek GH, Horii E, et al. IFSSH Scientific Committee on Congenital Conditions. J Hand Surg Eur Vol. 2014;39:676–8.)
Congenital
Upper Limb
Differences
Deformation
A. Constriction ring
sequence
B. Trigger digits
C. Not otherwise
specified
A. Hypertrophy
B. Tumorous
conditions
Dysplasia
Malformation
Abnormal axis
formation/Difference:
Hand Plate
Abnormal axis
formation/Difference:
Entire Upper Limb
1. Proximal‐distal
axis
2. Radial‐ulnar
axis
3. Dorsal‐ventral
axis
4. Unspecified
axis
i. Brachymelia with
brachydactyly
ii. Symbrachydactyly
a) Poland
syndrome
b) Whole limb
excluding Poland
syndrome
iii. Transverse
deficiency
a) Amelia
b) Clavicular/scapular
c) Humeral
d) Forearm
e) Wrist
f) Metacarpal
g) Phalangeal
iv. Intersegmental
deficiency
a) Proximal
b) Distal
c) Total
v. Whole limb
duplication/
triplication
i. Radial
longitudinal
deficiency -
Thumb
hypoplasia
ii. Ulnar
longitudinal
deficiency
iii. Ulnar dimelia
iv. Radioulnar
synostosis
v. Congenital
dislocation of
the radial head
vi. Humeroradial
synostosis -
Elbow ankyloses
vii. Madelung
deformity
i. Ventral dimelia
a) Furhmann/
Al‐Awadi/Raas‐
Rothschild
syndromes
b) Nail Patella
syndrome
ii. Absent/
hypoplastic
extensor/flexor
muscles
i. Shoulder
a) Undescended
(Sprengel)
b) Abnormal
shoulder muscles
c) Not otherwise
specified
ii. Arthrogryposis
1. Proximal‐distal
axis
2. Radial‐ulnar
axis
3. Dorsal‐ventral
axis
4. Unspecified
axis
i. Brachydactyly
ii. Symbrachydactyly
iii. Transverse
deficiency
a) Wrist
b) Metacarpal
c) Phalangeal
i. Radial
deficiency
ii. Ulnar
deficiency
iii. Radial
polydactyly
iv. Triphalangeal
thumb
v. Ulnar dimelia
vi. Ulnar
polydactyly
1. Whole limb
2.Partial limb
i. Hemihypertrophy
ii. Aberrant
flexor/extensor/
intrinsic muscle
i. Macrodactyly
ii. Aberrant intrinsic
muscles of hand
1. Vascular
2. Neurological
i. Hemangioma
ii. Malformation
iii. Others
i.
Neurofibromatosis
ii. Others
3. Connective
tissue
i. Juvenile
aponeurotic
fibroma
ii. Infantile digital
fibroma
iii. Others
i. Dorsal dimelia
(palmar nail)
ii. Ventral (palmar)
dimelia (including
hypoplastic/
aplastic nail)
i. Soft tissue
a) Syndactyly
b) Camptodactyly
c) Thumb in palm
deformity
d) Distal
arthrogryposis
ii. Skeletal
deficiency
a) Clinodactyly
b) Kirner’s
deformity
c) Synostosis/
sympha langism
iii. Complex
a) Complex
syndactyly
b) Synpolydactyly–
central
c) Cleft hand
d) Apert hand
e) Not otherwise
specified

655
Postoperative regime
hand teams with incorporation of this outcome set in daily
practice as an ultimate goal.
Postoperative regime
With regard to changes in insight and the will to improve
healthcare, with minimal stress for the patient and their
parents as a goal, we have introduced a different regime of
postoperative bandage in our clinic. With the phases of wound
healing in our mind, we decided to treat our patients postop­
eratively with a bandage that would stay put for 6 weeks. In
this period, complete wound healing takes place for regular
wounds and skin grafts. Furthermore, the best moment for
taking care to achieve an ultimate position of the fingers and
hand after reconstruction in the bandage is at the time the
patient is under general anesthesia. With respect to the debate
on children and general anesthesia, our goal is to minimize
the number of operations and duration of an operation.
Because of this combination, we decided to have the bandage
removed after approximately 6 weeks and start hand therapy
according to a regular schedule. Parents receive information
about alarm signals regarding the bandage, as is given with
the regular shorter bandage regimes. Preliminary data of the
last 5 years show far fewer infections with the new regime,
with fewer prescriptions of antibiotics. An additional advan­
tage to this regime is the number of bandage changes the
patient is subjected to: instead of daily changes after 2 weeks
until 6 weeks after the operation, this is decreased to only one
after 6 weeks. This decreases the stress for the patient and
parents in a period where bonding between the both is impor­
tant. Because of this outcome, we introduced this regime as a
standard practice, fitting our ideas on wound healing, anes­
thesia, psychology, and logistics of our outpatient clinic.
diagnoses (74–90% of total in several analyses) and is further
divided according the involvement of the whole limb or the
hand plate alone. These two groups are subdivided according
the three axis of limb development (i.e. proximal–distal,
radial–ulnar, dorsal–ventral) and an additional unspecified
axis. The deformations group is subdivided into: constriction
ring sequence, trigger digits, and not otherwise specified; the
dysplasia group is subdivided into hyperplasia and tumorous
conditions, with further subdivision (see Fig. 28.25). Addi­
tionally, if a syndrome is present with a congenital upper limb
difference, this will be cross-referenced according to a list
of syndromes that can be or are accompanied with a
syndrome.
As yet we do not have a full understanding of the basis of
all differences at the level of cell signaling and molecular
pathways, but as this becomes clearer, its incorporation into
the classification is possible, giving a fuller categorization of
congenital hand and upper limb differences. With regard to
this changing knowledge, the IFSSH Scientific Committee for
Congenital Conditions has been advised to review and
improve the OMT classification every 3 years.
With regard to future obligations regarding patient-related
outcome measures, the OMT classification can form the basis
of classifying the subjects researched. It is therefore important
that the most recent OMT classification is used when present­
ing data on outcome. Furthermore, with the Patient Reported
Outcome Measurement (PROM) getting a lead in reporting
quality of care and outcome of healthcare providers for
research purposes or in evaluating healthcare by insurance
companies, outcome measures for congenital hand and upper
limb malformations should be formalized and incorporated
by the teams treating them. At this moment, an outcome set
specifically for congenital hand an upper limb differences is
being formulated and will be evaluated globally by congenital
1. Dobyns JH, Doyle JR, Von Gillern TL, et al. Congenital anomalies
of the upper extremity. Hand Clin. 1989;5:321–342.
9. Ogino T. Teratogenic relationship between polydactyly, syndactyly
and cleft hand. J Hand Surg [Br]. 1990;15:201–209.
13. Tonkin MA. Failure of differentiation part I: Syndactyly. Hand Clin.
2009;25:171–193. This is a review of the classification, incidence,
indication, and review of surgical techniques and complications in
syndactyly in different hand anomalies associated with syndactyly.
16. Niranjan NS, Azad SM, Fleming AN, et al. Long-term results of
primary syndactyly correction by the trilobed flap technique. Br J
Plast Surg. 2005;58:14–21.
51. Upton J. Apert syndrome. Classification and pathologic anatomy
of limb anomalies. Clin Plast Surg. 1991;18:321–355. An excellent
overview of Apert syndrome concerning limb anomalies.
56. Zuker RM, Cleland HJ, Haswell T. Syndactyly correction of the
hand in Apert syndrome. Clin Plast Surg. 1991;18:357–364.
57. Fearon JA. Treatment of the hands and feet in Apert syndrome: an
evolution in management. Plast Reconstr Surg. 2003;112:1–19.
68. Foucher G, Navarro R, Medina J, et al. Metacarpal synostosis: a
simple classification and a new treatment technique. Plast Reconstr
Surg. 2001;108:1225–1234.
89. Wassel HD. The results of surgery for polydactyly of the thumb.
Clin Orthop Relat Res. 1969;64:175–193.
96. Ogino T, Ishii S, Takahata S, et al. Long-term results of surgical
treatment of thumb polydactyly. J Hand Surg Am. 1996;21:478–486.
Some 113 hands with reconstruction of radial polydactyly and an average
follow-up of 4 years were evaluated. According to Tada’s evaluation, 97
hands were classified as good, 12 hands as fair, and four hands as poor.
The type of deformity, type of procedure, and skill of the surgeon
influenced the outcome. Wassel type III, V, and VI and triphalangeal
thumb polydactyly have a higher incidence of unsatisfactory results.
97. Goldfarb CA, Patterson JM, Maender A, et al. Thumb size and
appearance following reconstruction of radial polydactyly. J Hand
Surg Am. 2008;33:1348–1353.
126. Hovius SE, Zuidam JM, de Wit T. Treatment of the triphalangeal
thumb. Tech Hand Up Extrem Surg. 2004;8:247–256. In the
triphalangeal thumb, the extra phalanx can have different shapes, from
wedge to rectangular. Furthermore, the involved joints, ligaments,
muscles, and tendons of the first ray, from distal interphalangeal joint to
radiocarpal joint can be hypoplastic, malformed, or absent with varying
degrees of stiffness or instability. Also, the first web can be insufficient
and radial polydactyly as well as other hand deformities can be present.
In this series depending on the malformation, operations varied from
removal of the delta phalanx with ligament reconstruction to multiple
osteotomies and rebalancing as well as pollicization.
140. Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a
spectrum of congenital deficiencies: a treatment algorithm based
on clinical examination. Plast Reconstr Surg. 2006;117:1897–1905.
Camptodactyly is classified into four groups. Camptodactyly can be
correctible or non-correctible and can be moderate or severe. Careful
assessment with clinical tests can distinguish the possible treatment,
varying from splinting, the lasso procedure with or without lumbrical
repositioning, and tendon transfer of the FDS of the fourth or fifth finger.
150. Wood VE, Flatt AE. Congenital triangular bones in the hand. J
Hand Surg Am. 1977;2:179–193.
Access the complete reference list online at

655.e1
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655.e2
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
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operatoire. Cong Fran de Chir. 1889;4:576–580.
96. Ogino T, Ishii S, Takahata S, et al. Long-term results of surgical
treatment of thumb polydactyly. J Hand Surg Am. 1996;21:478–486.
Some 113 hands with reconstruction of radial polydactyly and an average
follow-up of 4 years were evaluated. According to Tada’s evaluation, 97
hands were classified as good, 12 hands as fair, and four hands as poor.
The type of deformity, type of procedure, and skill of the surgeon
influenced the outcome. Wassel type III, V, and VI and triphalangeal
thumb polydactyly have a higher incidence of unsatisfactory results.
97. Goldfarb CA, Patterson JM, Maender A, et al. Thumb size and
appearance following reconstruction of radial polydactyly. J Hand
Surg Am. 2008;33:1348–1353.
98. Tada K, Kurisaki E, Yonenobu K, et al. Central polydactyly – a
review of 12 cases and their surgical treatment. J Hand Surg Am.
1982;7:460–465.
99. Lapidus PW, Guidotti FP, Coletti CJ. Triphalangeal thumb. Report
of six cases. Surg Gynecol Obstet. 1943;77:178–186.
100. Zguricas J, Snijders PJ, Hovius SE, et al. Phenotypic analysis of
triphalangeal thumb and associated hand malformations. J Med
Genet. 1994;31:462–467.
101. Zguricas J, de Raeymaecker DM, Snijders PJ, et al. Psychomotor
development in children with triphalangeal thumbs. A preliminary
study. J Hand Surg Br. 1998;23:526–529.
102. Dubois P. Sexdigitaire (pouce à de trois phalanges). Arch Gén Méd.
1826;4:148.
103. Heutink P, Zguricas J, van Oosterhout L, et al. The gene for
triphalangeal thumb maps to the subtelomeric region of
chromosome 7q. Nat Genet. 1994;6:287–292.
104. Haas SL. Three phalangeal thumbs. AJR Am J Roentgenol.
1939;42:677–682.
105. Phillips RS. Congenital split foot (lobster claw) and triphalangeal
thumb. J Bone Joint Surg. 1971;53:247–257.
106. Miura T, Nakamura R, Horii E, et al. Three cases of syndactyly,
polydactyly, and hypoplastic triphalangeal thumb (Haas’s
malformation). J Hand Surg Am. 1990;15:445–449.
107. Wood VE. Treatment of the triphalangeal thumb. Clin Orthop Relat
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110. Smith H, Campbell WD, Speed JS. Campbell’s Operative Orthopedics.
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111. Beatson GT. Congenital deformity of both thumbs. Scott Med Surg
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112. Milch H. Triphalangeal thumb. J Bone Joint Surg Am. 1951;33:
692–697.
113. Cotta H, Jager M. [Familial triphalangism of the thumb and its
surgical treatment]. Arch Orthop Unfallchir. 1965;58:282–290.
114. Miura T. Triphalangeal thumb. Plast Reconstr Surg. 1976;58:
587–594.
115. Wood VE. Polydactyly and the triphalangeal thumb. J Hand Surg
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116. Peimer CA. Combined reduction osteotomy for triphalangeal
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118. Buck-Gramcko D. Pollicization of the index finger. Method and
results in aplasia and hypoplasia of the thumb. J Bone Joint Surg
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119. Woolf RM, Broadbent TR. The four-flap Z-plasty. Plast Reconstr
Surg. 1972;49:48–51.
120. Foucher G, Medina J, Navarro R, et al. Correction of first web
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121. Tajima T, Watanabe Y, Uchiyama J. [Treatment and study of the
hypoplastic thumb]. Keisei Geka. 1967;10:227–234.
122. Upton J, Havlik RJ, Coombs CJ. Use of forearm flaps for the
severely contracted first web space in children with congenital
malformations. J Hand Surg Am. 1996;21:470–477.
123. Huber E. Relief operation in the case of paralysis of the median
nerve. 1921. J Hand Surg [Br]. 2004;29:35–37.
124. Scharizer E. Dreigliedrige daumen. Fortschr Rontgenstr.
1929;40:693–694.
125. Upton J, Schoen S. Triphalangeal thumb. In: Gupta A, Kay SP,
Scheker LR, eds. The Growing Hand. 1st ed. London: Mosby;
2000:255–268.
126. Hovius SE, Zuidam JM, de Wit T. Treatment of the triphalangeal
thumb. Tech Hand Up Extrem Surg. 2004;8:247–256. In the
triphalangeal thumb, the extra phalanx can have different shapes, from
wedge to rectangular. Furthermore, the involved joints, ligaments,
muscles, and tendons of the first ray, from distal interphalangeal joint to
radiocarpal joint can be hypoplastic, malformed, or absent with varying
degrees of stiffness or instability. Also, the first web can be insufficient
and radial polydactyly as well as other hand deformities can be present.
In this series depending on the malformation, operations varied from
removal of the delta phalanx with ligament reconstruction to multiple
osteotomies and rebalancing as well as pollicization.
127. Zuidam JM, Dees EE, Lequin MH, et al. The effect of the
epiphyseal growth plate on the length of the first metacarpal in
triphalangeal thumb. J Hand Surg Am. 2006;31:1183–1188.
128. De Haas WHD. Camptodactylie. Ned Tijdschr Geneeskd.
1957;101:2121–2124.
129. Tamplin R. Lecture on the Nature and Treatment of Deficiencies.
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130. Adams W. On congenital contraction of the fingers and its
association with “hammer toe”; its pathology and treatment.
Lancet. 1891;138:111–114.
131. Oldfield MC. Campylodactyly: flexor contracture of the fingers in
young girls. Br J Plast Surg. 1956;8:312–317.
132. Smith PJ, Grobbelaar AO. Camptodactyly: a unifying theory and
approach to surgical treatment. J Hand Surg Am. 1998;23:14–19.
133. Millesi H. Camptodactyly. In: Littler J, Cramer L, Smith J, eds.
Symposium on Reconstructive Hand Surgery. St. Louis, MO: Mosby;
1974:175–177.
134. Smith RJ, Kaplan EB. Camptodactyly and similar atraumatic
flexion deformities of the proximal interphalangeal joints of the
fingers. A study of thirty-one cases. J Bone Joint Surg Am.
1968;50:1187–1203.
135. Courtemanche AD. Campylodactyly: etiology and management.
Plast Reconstr Surg. 1969;44:451–454.
136. McFarlane RM, Classen DA, Porte AM, et al. The anatomy and
treatment of camptodactyly of the small finger. J Hand Surg Am.
1992;17:35–44.
137. McFarlane RM, Curry GI, Evans HB. Anomalies of the intrinsic
muscles in camptodactyly. J Hand Surg Am. 1983;8:531–544.
138. Gupta A, Burke FD. Correction of camptodactyly. Preliminary
results of extensor indicis transfer. J Hand Surg [Br].
1990;15:168–170.
139. Benson LS, Waters PM, Kamil NI, et al. Camptodactyly:
Classification and results of nonoperative treatment. J Pediatr
Orthop. 1984;14:814.
140. Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a
spectrum of congenital deficiencies: a treatment algorithm
based on clinical examination. Plast Reconstr Surg. 2006;117:1897–
1905. Camptodactyly is classified into four groups. Camptodactyly can
be correctible or non-correctible and can be moderate or severe. Careful
assessment with clinical tests can distinguish the possible treatment,
varying from splinting, the lasso procedure with or without lumbrical
repositioning, and tendon transfer of the FDS of the fourth or fifth
finger.
141. Hori M, Nakamura R, Inoue G, et al. Nonoperative treatment of
camptodactyly. J Hand Surg Am. 1987;12:1061–1065.
142. Miura T, Nakamura R, Tamura Y. Long-standing extended
dynamic splintage and release of an abnormal restraining structure
in camptodactyly. J Hand Surg [Br]. 1992;17:665–672.
143. Engber WD, Flatt AE. Camptodactyly: an analysis of sixty-six
patients and twenty-four operations. J Hand Surg Am.
1977;2:216–224.
144. Smith DW. Recognizable patterns of human malformation: genetic,
embryologic, and clinical aspects. Major Probl Clin Pediatr.
1970;7:1–368.
145. de Marinis FM, de Marinis R. Frequency of clinodactyly in
children between the age of 5 and 12. Acta Genet Med Gemellol
(Roma). 1955;4:192–204.
146. Burke F, Flatt A. Clinodactyly. A review of a series of cases. Hand.
1979;11:269–280.
147. Flatt A. The Care of Congenital Hand Anomalies. 1st ed. St. Louis,
MO: Quality Medical; 1977:154–163.
148. Schmid F, Moll H. Atlas der Normalen und Pathologischen
Handskeletentwicklung. Berlin: Springer Verlag; 1960.
149. Jaeger M, Refior HJ. The congenital triangular deformity of the
tubular bones of hand and foot. Clin Orthop Relat Res.
1971;81:139–150.
150. Wood VE, Flatt AE. Congenital triangular bones in the hand.
J Hand Surg Am. 1977;2:179–193.
151. Theander G, Carstam N. [Longitudinally bracketed diaphysis].
Ann Radiol (Paris). 1974;17:355–360.
152. Jones GB. Delta phalanx. J Bone Joint Surg Br. 1964;46:226–
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153. Tamplin RW. Lectures on the Nature and Treatment of Deformities
Delivered at the Royal Orthopaedic Hospital. London: Longman,
Brown, Green; 1896:256.
154. Hersch AH, de Marinis FM, Stecher RM. On the inheritance and
development of clinodactyly. Am J Hum Genet. 1953;5:257–268.
155. Fürst J. Ein Fall von Verkürzten un Zweigliedrigen Fingern
Begleitet von Brustmuskeldefecten un anderen Missbildungen.
Zeitschr Morphol Antropol. 1900;2:56–76.
156. Ali M, Jackson T, Rayan GM. Closing wedge osteotomy of
abnormal middle phalanx for clinodactyly. J Hand Surg Am.
2009;34:914–918.
157. Al-Qattan MM. Congenital sporadic clinodactyly of the index
finger. Ann Plast Surg. 2007;59:682–687.

655.e4
SECTION IV
CHAPTER 28 • Congenital hand IV: Syndactyly, synostosis, polydactyly, camptodactyly, and clinodactyly
158. Vickers D. Clinodactyly of the little finger: a simple operative
technique for reversal of the growth abnormality. J Hand Surg [Br].
1987;12:335–342.
159. Caouette-Laberge L, Laberge C, Egerszegi EP, et al. Physiolysis for
correction of clinodactyly in children. J Hand Surg Am. 2002;27:
659–665.
160. Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental
biology and classification of congenital anomalies of the hand and
upper extremity. J Hand Surg Am. 2010;35:2066–2076.
161. IFSSH Scientific Committee on Congenital Conditions. J Hand Surg
Eur Vol. 2014;39:676–678.
162. Swanson AB, Barsky AJ, Entin MA. Classification of limb
malformations on the basis of embryological failures. Surg Clin
North Am. 1968;48:1169–1179.
163. Tonkin MA, Oberg KC. The OMT classification of congenital
anomalies of the hand and upper limb. Hand Surg.
2015;20:336–342.

SECTION IV • Congenital Disorders
29 
Congenital hand V: Overgrowth,
undergrowth, and generalized
skeletal deformities
Julie Colantoni Woodside, Shelley Noland, and Terry Light
SYNOPSIS
■ Contemporary understanding of limb embryology and morphologic
characteristics has shaped the Oberg–Manske–Tonkin (OMT)
classification of upper extremity anomalies. The OMT classification
categorizes congenital upper extremity anomalies as malformation,
deformation, or dysplasia.
■ Undergrowth of the upper extremity affects a portion of or the entire
extremity. Variable presentations include hypoplasia or aplasia of bone
and soft tissue. These may be associated with a syndrome.
■ Overgrowth includes syndromes as well as isolated conditions of
enlargement such as macrodactyly. The presentation and natural
history of macrodactyly in the upper limb varies greatly. Treatment by
debulking or amputation may require sequential procedures to balance
growth, appearance, and function.
■ Generalized skeletal anomalies of the hand include conditions such as
multiple hereditary osteochondromas, Maffucci syndrome, and Ollier
disease. Treatment of skeletal abnormalities of the hand and forearm
requires accurate diagnosis and careful monitoring during years of
rapid skeletal growth. Surgical intervention is indicated to preserve
joint motion.
Classification
Historically, the Swanson classification, accepted by the Inter­
national Federation of Societies for Surgery of the Hand
(IFSSH) in 1974 has been the most widely used classification
for congenital abnormalities of the upper limb.1 A new clas­
sification system, developed by Oberg, Manske, and Tonkin
(OMT)2 is based on a more contemporary understanding of
developmental biology. The OMT system uses dysmorpho­
logic descriptions and the three axes of the limb development
to classify conditions based on their effect on the hand plate
and/or the entire limb. The IFSSH adopted this classification
with some modifications in 20143 (see Chapter 25 and Table
25.2 for most recent update).
The OMT classification categorizes abnormalities as mal­
formations, deformations, or dysplasias. Malformations are
further divided into the developmental axis (proximal–distal,
anteroposterior, and dorsal–ventral) and the extent of involve­
ment as either affecting the entire limb or simply the hand.
Deformations include any disruption to a portion of the upper
limb that has already been formed. Dysplasias are abnormali­
ties in size, shape, and organization of cells including
conditions that are associated with cellular atypia or
tumor formation.2 The intra-observer reliability of the clas­
sification system is 85–99% with perfect agreement in inter-
observer reliability of 64%, moderate of 19%, fair of 3%, and
slights of 13%, and poor of 1%. Undergrowth conditions and
brachydactyly show poor agreement, while arthrogryposis,
Madelung deformity, and symbrachydactyly show slight
agreement.4
Overgrowth
Introduction
Congenital limb enlargement and overgrowth is a rare condi­
tion affecting 1/100 000 live births.5 The natural history and
presentation of overgrowth conditions such as macrodactyly
varies greatly. Upper extremity overgrowth can be associated
with several syndromes including Beckwith–Wiedemann,
Proteus, and CLOVES.6 The diagnosis and understanding of
these conditions has advanced in recent years with genetic
sequencing of involved tissues. Mutations in the PIK3CA gene
are associated with overgrowth of fibroadipose tissue. Seg­
mental and progressive growth of the subcutaneous, muscular,
neural, and skeletal tissues can be found in tissues with the
PIK3CA gene mutation.7
Keppler-Noreuil et al.7 studied 35 patients with overgrowth
and found 25 had phenotypes consistent with fibroadipose
overgrowth, hemihyperplasia, multiple lipomatosis, or
macrodactyly. Thirty-three of 35 demonstrated one of two
genotype mutations. All of these patients had progressive,

657
Overgrowth
in the PI3K gene. The rate of growth, location, and the severity
should be evaluated on an individual patient basis.11 Four
types of macrodactyly have been defined by Upton (Table
29.1).12
The clinical presentation varies with each patient. The hand
may show overgrowth with excessive adipose formation.
Affected digits often deviate away from each other due to
asymmetric over growth in the adjacent sides of adjacent
digits (Fig. 29.1). Overgrowth in the palm and forearm is often
apparent as well as fat deposits extending up to the axilla and
chest wall.8 Excessive adipose tissue, commonly seen along
the median nerve in the hand (nerve territory-oriented mac­
rodactyly)9 usually requires multiple debulking surgical
procedures, epiphysiodesis of the phalanges, osteotomy,
arthrodesis, and/or amputation of hypertrophic digits. Neural
elements are often enlarged along the palmar aspect of the
hand.9 In contrast, the vessels are usually of normal or smaller
size.10
Macrodactyly can be static or progressive. The static type
shows enlargement at birth with proportional growth as the
child grows. Macrodystrophia lipomatosa refers to the progres­
sive form of macrodactyly with associated osseous and
asymmetric overgrowth. Six had upper extremity manifesta­
tions. The distal aspect of the limb was often the first to area
to manifest overgrowth; proximal progression of enlargement
could develop with further growth. Sixty percent (21/35) of
cases were unilateral, 42% had renal abnormalities, and all
patients showed adipose dysregulation. In the patients with
hand manifestations, all had surgery, none had ovarian/
testicular manifestations, one had an enlarged kidney (this
patient had global, bilateral presentation), one had a congeni­
tal heart defect, and four had vascular malformations.7 Overall
weight and height were normal except in three patients whose
height was less than fifth percentile.7
Neurofibromatosis may also present with overgrowth in
which the skin and subcutaneous tissue planes are enlarged
by soft-tissue neurofibromas, schwannomas, and other nerve
sheath tumors.8
Proteus syndrome has a variety of soft-tissue and skeletal
abnormalities in addition to overgrowth of the hands or feet.
These patients have cerebriform, hyperkeratotic skin on their
palms and soles. They may develop cartilaginous masses,
joint stiffness (flexion contracture), angulation, and rotational
deformities in the hands. This is a progressive overgrowth in
which all tissues are larger than normal without adipose
infiltration.8
Patients with hemihypertrophy present after birth with
enlargement of one extremity, or one side of the body. The
hands usually have a flexed wrist with ulnar deviation of the
digits and a flexed, adducted thumb. The muscle–tendon
groups do not have normal excursion and progressive con­
tractures are seen with growth of the limb.8
Klippel–Trénaunay syndrome is associated with a slow-
flow vascular lesion that contains capillaries, veins, and/or
lymphatic components, with enlarged hands or extremities
due to skeletal, adipose, and vascular growth.8
CLOVE syndrome includes capillary malformations, lipo­
matous overgrowth, vascular malformations, epidermal
nevi, and skeletal distortions with central nervous system
components.
Other syndromes including Maffucci syndrome and Ollier
disease show enlargements due to vascular malformations
and enchondromas.8
To delineate these overlapping overgrowth disorders asso­
ciated with similar genetic mutations, Keppler-Noreuil et al.
reported the collaboration of several clinicians that outlined
specific characterization of disease for management and
prognosis.6 All cases of PIK3CA-related overgrowth spectrum
(PROS) are sporadic, with mosaic distribution of involved
tissues. The condition may be evident at birth or may be
detected in early childhood presentation. Children may either
have isolated findings or a spectrum of features. Most, but not
all, are progressive. The authors stress that although these are
distinct clinical disorders, there is still much overlap in PROS.6
Macrodactyly
Macrodactyly describes the overgrowth of bony and fibroadi­
pose tissue, often in a pattern that mimics a local nerve.
Muscular overgrowth may be associated with macrodactyly.9,10
Macrodactyly is sporadic unilateral 90% of the time, and
not associated with malignant transformation.10 Rios et al.10
genetically sequenced and histologically examined specimens
from macrodactylous tissue (n = 6) and confirmed mutations
Table 29.1  Types of macrodactyly
Type
Description
I
Macrodactyly with lipofibromatosis of a nerve, static
or progressive
II
Macrodactyly associated with neurofibromatosis
III
Macrodactyly associated hyperostosis
IV
Macrodactyly associated hemihypertrophy
(Reproduced with permission from Upton J. Mathes SJ, Hentz VR (eds). Failure
of Differentiation and Overgrowth. In: Plastic Surgery, 2nd edn. Philadelphia, PA:
Saunders; 2005.)
Fig. 29.1  This pediatric patient with macrodactyly shows an enlarged middle and
slightly enlarged index finger with middle finger deviation away from the index
finger. (Courtesy of OrthoCarolina Hand Fellowship Teaching File.)

658
SECTION IV
CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
and accelerated growth. Premature osteoarthritis is common
in the affected by the third decade.8 Standard radiographs of
macrodystrophia lipomatosa show osseous and soft-tissue
overgrowth with splayed, broad, elongated phalanges and
secondary degenerative joint disease.9,13,14
Magnetic resonance imaging of the affected area may
show deposits of fat within the nerve sheath, bone marrow,
periosteum, muscles, and subcutaneous tissues, with fatty
tissue masses and proportional enlargement of mesenchymal
tissues.13,15 The fatty masses are hyperintense on T1 images
and have a lower signal on T2 images.
Though reports vary, there appears to be a relationship
between fibrolipomatous hamartoma of nerve and macrodys­
trophia lipomatosa.16,17 Histological analysis shows abnormal
fatty tissue interspersed with nerve bundles with endoneural
and perineural fibrosis.16,18 The perineurium in macrodactyly
is thickened and the number of myelinated axons is reduced.10
Swollen, dissociated Schwann cells, interstitial fat, and dense
endoneural connective tissue are frequent findings.10 Because
the nerves in the palm are enlarged compressive neuropathies
are common. If this compressive neuropathy is unrecognized
the patient may develop altered sensory function.8
Treatment
Treatment of patients with overgrowth conditions is patient
specific and attempts to address both function and appear­
ance. Surgical planning should consider patient age, clinical
syndrome, severity, progression, deformity, function, range
of motion, and the expectations of the patient and family
adipose overgrowth. Occasionally, the overgrowth appears
static in the distal aspect of the extremity but progresses
proximally. This progression may follow surgical debulking
or partial amputation.9 Timing of surgery differs between the
two types. Reduction surgeries and epiphysiodesis are per­
formed later in the static type and outcomes are more predict­
able. Patients with the progressive form may require earlier
and repeat surgeries, while patients with unrestricted, pro­
gressive growth may require amputations of large bulky parts
(Figs. 29.2 & 29.3A–E).8
Skeletal components can demonstrate increase in length,
width, and girth. Involved epiphyses can show asymmetry
Fig. 29.2  A 14-month-old male patient with congenital, progressive macrodactyly.
A
B
C
D
E
Fig. 29.3  (A–E) This patient underwent index ray
amputation. (Courtesy of OrthoCarolina Hand
Fellowship Teaching File.)

659
Overgrowth
nerves were seen intraoperatively in all nerve-oriented and
lipomatous patients. The most common finger involved
was the middle finger (67%) followed by the index finger
(47%). Those with progressive overgrowth underwent more
surgeries: 4.1 versus 1.8 per patient. Nearly all patients
(19/21) underwent soft-tissue debulking, with other pro­
cedures including closing wedge osteotomies (11 patients),
partial amputation/ostectomy (9 patients), epiphysiodesis (7
patients), and ray amputations (6 patients). Other associated
procedures included neuroplasties of median, ulnar, and radial
nerves and syndactyly release. Closing wedge osteotomy was
employed for angulation greater than 30° or for correction
members. Discussions with the family should include arthritis
and stiffness, recurrence, and the possibility of multiple pro­
cedures. Serial radiographs demonstrate the extent of defor­
mity and progression while magnetic resonance imaging
(MRI) scans demonstrate other abnormalities that may be
present. Timing for procedures is patient specific. Epiphysio­
lysis should be considered when the digit or hand reaches the
size of one of the adult parents. Other surgeries include
debulking, osteotomies, and neuroplasty. Amputation is
considered early in very aggressive cases or when reconstruc­
tion may not provide optimal outcomes.8
Surgical incision placement is important, as volar scars can
lead to hypertrophy and contracture. Midaxial incisions are
preferable as they can be used at revision surgeries. Palmar
incisions distal to the carpal tunnel should be in zig-zag
configuration. Only one side of a digit should be worked on
at a time for the prevention of vascular compromise. Arterial
segments can be reconstructed with vein grafts as needed to
preserve blood flow. Care should be taken to preserve sensa­
tion, especially on the ulnar thumb and radial index finger for
pinch.8
Digit salvage may be possible through soft-tissue and
skeletal reduction surgeries. Growth arrest will halt longitu­
dinal growth of the digit and can be accomplished through
use of a drill, an osteotome, or a burr to destroy the physis.
Physeal stapling is potentially reversible and in selected
instances may allow gradual correction of angular deformity.
Soft-tissue debulking should be thorough with preservation
of digital arteries and at least two dorsal veins per digit.
Precise surgical techniques are important for salvage of
vincular branches to the flexor tendons and to perform
neuroplasty and pulp reduction. The remaining skin will
be reinnervated from the remaining digital nerve8 (Figs.
29.4–29.10).
Historically, a number of strategies have been advocated
for the surgical management of macrodactyly. Barsky reduced
the longitudinal axis through resection of the distal portion of
the middle phalanx, distal interphalangeal joint (DIP) and
distal phalangeal physis with sacrifice of the often relatively
stiff DIP joint motion.19 The author also described stapling or
wiring of the epiphyseal plate and concluded that results from
surgery are not entirely satisfactory. Tsuge described resection
of all three physes of the phalanges combined with tip-plasty.20
Hoshi shortened the digit through resection of the DIP joint
with fusion and unilateral longitudinal soft-tissue wedge
resection. Millesi described a resection of the distal phalanx
of the thumb with shortening of the proximal phalanx through
an oblique shortening osteotomy.21 Ogino described a resec­
tion osteotomy of the proximal phalanx, osteotomy with
fusion of the DIP joint and tip and unilateral soft-tissue resec­
tion.22 Bertelli performed longitudinal resection osteotomies
of the distal, middle, and proximal phalanges with tip and
unilateral soft-tissue resection.23
Outcomes
Cerrato et al.11 evaluated 21 patients with isolated macrodac­
tyly of one or more digits. Right hand involvement was seen
in 67%, progressive overgrowth in 67%, and nerve-territory
orientation in 57% (the others with lipomatous forms). The
territory affected in 10 out of the 12 nerve-territory orienta­
tion patients was in the median nerve distribution. Enlarged
Fig. 29.4  (A,B) A male patient with macrodactyly of the small finger with
overgrowth on the ulnar aspect and radial curvature of the digit.
A
B

660
SECTION IV
CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
Fig. 29.5  (A,B) Same patient as in Fig. 29.4: a coronal and axial cut of an MRI
through the macrodactyly digit showing increased signal with fatty tissue
proliferation along the volar and ulnar aspect of the digit.
A
B
Fig. 29.6  Same patient as in Fig. 29.4: anterior–posterior X-ray of the hand
showing the radial curvature of the digit.
Fig. 29.7  Same patient as in Fig. 29.4:
(A,B) surgical planning for debulking
procedure.
A
B

661
Overgrowth
Fig. 29.10  Same patient as in Fig. 29.4: (A) digit before closure and excised
tissue and (B) finger after tissue debulking and closure.
A
B
Fig. 29.8  Same patient as in Fig. 29.4: enlarged digital nerve lying on top of
hemostat supplying the overgrown tissue.
Fig. 29.9  Same patient as in Fig. 29.4: nerve entering into tissue being excised.
found the middle ray affected most frequently with an average
of 2.5 digits affected, usually adjacent. Six of 20 cases were
progressive; 16 of the 20 cases were treated surgically. Patients
underwent an average of 2.5 surgeries with static disease
requiring fewer surgeries.
Overview
Overgrowth conditions can be associated with syndromes or
can be isolated, as in macrodactyly. Patients have varied
presentations and should have an individual approach for
treatment options. Progressive overgrowth requires a more
aggressive approach and will require more surgeries than
static conditions.
of digital scissoring. The authors recommend epiphysiodesis
when the affected digits reached the adult length of their
parents’ hands.
Hardwicke et al.24 reviewed 32 patients with macrodactyly,
20 affecting the upper limb. There was a slight male predomi­
nance in their series (male : female = 1 : 0.66). The authors

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CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
intervention usually focuses upon aesthetic concerns.27 In
brachymetacarpia, the patient will often present with one
knuckle that is not apparent when the patient makes a fist.
This abnormality usually does not limit the patient’s function,
unless the thumb or index fingers are involved.30 The meta­
carpal physis usually closes prematurely and the resultant
metacarpophalangeal joint demonstrates limited flexion
(Fig. 29.11A,B). To address the loss of length, one may consider
distraction lengthening of the affected bone. In cases of severe
ray shortening or in cases in which multiple digits are affected,
hand function can be compromised. Options for treatment of
such cases include webspace deepening, osteotomy and bone
grafting, non-vascularized toe phalanx transfer, distraction
lengthening, and microsurgical toe to hand transfers.31
Although brachydactyly, symbrachydactyly, and transverse
deficiency primarily affect the hand plate, others can also
affect the entire upper limb.3
Table 29.2  Groups of brachydactyly
Type
Description
A
Shortened middle phalanges
B
Short or absent middle or distal phalanges with
deformed thumbs and great toes
C
Deformity of the middle and proximal phalanges of
the index and middle fingers with shortening of the
metacarpals
D
Short and broad distal phalanx of the thumb and
great toes
E
Shortening of one or more of the metacarpals
A
Fig. 29.11  (A,B) Anterior–
posterior (AP) and
comparison AP X-rays
of a skeletally immature
patient with middle finger
brachymetacarpia with
premature closure of
metacarpal head growth
plates. (Courtesy of
OrthoCarolina Hand
Fellowship Teaching File.)
B
Clinical tips
Brachydactyly
• Brachydactyly can affect the entire finger or individual
bones of the finger.
• Treatment is usually observation, but surgical options
include: phalangeal and metacarpal lengthening,
osteotomy, and web deepening.
Clinical tips
Overgrowth
• Macrodactyly may be associated with an overgrowth
syndrome.
• Can be static or progressive – each patient needs an
individual treatment approach.
• Progressive types require a more aggressive approach and
more surgeries than static types.
Undergrowth
Introduction
Congenital upper limb anomalies occur in approximately 5.25
per 10 000 live births. Among these anomalies, undergrowth
anomalies occur approximately 1.19 per 10 000 live births.25
Undergrowth conditions are classified as malformations in
the OMT classification and are subclassified as either under
the entire limb or hand plate only along the proximal–distal
axis.3
Brachydactyly
Brachydactyly, Greek for “short finger”, is a condition isolated
to the hand plate that presents with shortened digits. Some
types show autosomal-dominant inheritance patterns.26
Brachydactyly may occur in isolation but more commonly it
is associated with other hand malformations.27 The index and
small fingers are the most commonly involved digits while
the middle phalanx is the most commonly affected bone.
Brachymetacarpia refers to brachydactyly isolated to the
metacarpals.5
Bell28 defined subgroup types of brachydactyly (Table 29.2).
Brachydactyly has also been associated with hereditary mul­
tiple exostosis.29
Treatment
Treatment of brachydactyly depends on the severity of
shortening. Typically, function is preserved and surgical

663
Undergrowth
genetic etiology. In contrast to symbrachydactyly without PA,
PA is seen more commonly on the right side.37 It has been
suggested that the cause is disruption of the subclavian artery
during growth and development causing underdevelopment
of the limb.38 Catena et al.37 demonstrated that 57.7% of PA
patients had associated upper limb anomalies including
hypoplastic hand without functional or morphologic anoma­
lies (8%), symbrachydactyly with five functioning fingers
(20.6%), symbrachydactyly and some functioning fingers
(14.9%), and symbrachydactyly with absent or non-functioning
fingers (4.6%).
Treatment
The individual presentation of each hand provides a guide
for treatment. Treatment should aim to increase the child’s
functional capability. An untreated aplastic hand without
digits can serve as a helping hand and can be fitted with a
prosthesis. A monodactylous hand usually has a functioning
thumb and a palm width with hypoplastic or normal meta­
carpals. Bimanual tasks with thumb and palm grip/pinch
and pressing or hooking with one finger are possible. With
a bidactylous hand, usually a thumb and an ulnar digit,
two-finger pinch is possible but grip is usually weak.35,39 These
children have difficulty with cylindrical pinch, or wrapping
their finger around a larger object with the thumb underneath.
If the child has three functioning digits, then tip, palm, and
cylindrical grip are possible, although they will still lack
full-power grip.5 With four or five digits, power grip is
increased, although the fingers may have unstable interpha­
langeal joints; these children may be capable of single-hand
prehension.2
Treatment of these hands may include excision of nubbins,
syndactyly release, webspace deepening, joint stabilization,
bone transfer, and microvascular second toe transfer.35 Since
many manual skills are developed by the age of 3 years,40,41
early reconstruction can help in the development of grasp and
pinch.42
Hypoplastic nubbins, which may have rudimentary, diffi­
cult to care for nails, may occasionally block the prehensile
function of adjacent digits. These small digits can be excised
if the family wishes and if they have extrinsic tendons, these
can be used for toe-to-hand transfer or free phalanx toe
transfer.35
Often the interphalangeal joints that are present in
hypoplastic digits are unstable to pinch. In older skeletally
immature children, these joints may benefit from physis-
preserving interphalangeal arthrodesis. The phalangeal
condyle is denuded of articular cartilage and is fused to the
secondary ossification center of the more distal phalanx.35
Distraction lengthening of the digit can be performed and
combined with other procedures.43 Digits with distal webs or
syndactyly should undergo web deepening or syndactyly
release with or without full-thickness skin grafting.35
Non-vascularized toe phalanx transfer can add osseous
stability to digits if an adequate soft-tissue sleeve is present.
The proximal phalanx of the third or fourth toe is harvested
extraperiosteally with its proximal collateral ligaments and
plantar plate. The soft tissues are sutured to the metacarpal
head creating a stable articulation. Harvesting the graft
extraperiosteally and preserving the distal articular surface
prevents resorption.31 Goldberg and Watson44 found 91%
Symbrachydactyly
Symbrachydactyly, is a rare upper limb anomaly seen in
approximately 0.6 per 10 000 live births. It most commonly
affects males (73%) and is typically unilateral (left side 67%,
right side 27%). Between 1% and 7% of patients have bilateral
presentation. Only 7% have associated anomalies or a positive
family history.32
Surgeon classification of the different manifestations of
symbrachydactyly is inconsistent. The previous IFSSH clas­
sification system categorized symbrachydactyly under catego­
ries I, II, and V; or failure of formation, differentiation, and
undergrowth, respectively.33 The OMT classifies the condition
as a malformation in the failure of axis formation/
differentiation of the entire limb and/or hand plate.2,4 Blauth
and Gekler subdivided symbrachydactyly into four types
(Table 29.3).34
Symbrachydactyly is divided into four types: (1) short
finger type; (2) oligodactylic, atypical cleft hand; (3) monodac­
tylic; and (4) peromelic. Symbrachydactyly presents with a
hand or entire limb that is shorter and smaller than the unin­
volved upper limb. The digits are short, underdeveloped and
often have incomplete syndactyly.35 Since the mesoderm is
primarily affected, the fingernails and distal phalangeal tufts
(ectodermal elements) are often preserved.36 The central digits
and middle phalanges are usually more severely affected.36
Patients may present with small nubbins that have nails
present. Digital amputations due to constriction bands are
distinguishable by the presence of other bands elsewhere on
the body and absent distal finger and nail. Anatomic struc­
tures proximal to the hand are often hypoplastic in sym­
brachydactyly, while proximal structures are usually normal
in hands affected by constriction ring syndrome.35,36
Poland association (PA) is the association of symbrachy­
dactyly with unilateral absence or hypoplasia of the sterno­
costal head of the pectoralis major muscle. There is no known
Table 29.3  Subdivisions of symbrachydactyly
Symbrachydactyly type
Description
Short finger
(brachymesophalangia)
Αll four fingers are short and
incompletely formed with a
relatively normal thumb; middle
phalanges may be missing with
incomplete syndactyly
Oligodactylic (atypical cleft
hand):
Τhe central portion of the hand is
aplastic, whereas the borders
digits are less severely affected
Monodactylic
Τhe fingers are absent or aplastic,
whereas the thumb is present
Peromelic
Ρesembles a transverse
amputation at the
metacarpophalangeal joint level,
may have nubbins with or
without nail remnants
(Reproduced with permission from Julie C. Woodside, MD,* Terry R. Light, MD*
Symbrachydactyly — Diagnosis, Function, and Treatment. J Hand Surg Am. Vol.
41, January 2016).

664
SECTION IV
CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
require one mobile digit and at least a stable post or another
mobile digit to achieve prehension. Survival rates are gener­
ally greater than 95%.50 The procedure was first described by
Cobbett51 and Buncke52 as a single-stage second toe transfer
to the hand. When transfer of two toes is recommended, it
may be accomplished in one or two stages, harvesting the
second toe from each foot. Surgery is usually delayed until
ages 2–5 years to allow the vessels the be of adequate size.50
The symbrachydactyly hand presents challenges different
from those following traumatic amputation or in a child with
constriction ring syndrome, since the hand with symbrachy­
dactyly often has hypoplastic or absent musculotendinous
units, nerves, and arteries.53,54 Often, the common extensor
and flexor mass are used as motors for the transfer. The
median nerve is occasionally absent, requiring that other
nerves such as the dorsal sensory branches of the radial nerve
are used to achieve volar reinnervation.55
Jones et al.50 reported a microvascular success rate of greater
than 97% with micro-anastomosis re-exploration required in
8.5% of 82 digits. Vilkki39 reported that 14 of 17 patients
undergoing toe transfer achieved the ability to actively pinch.
Although most re-operations are performed because of limited
digital motion, tenolysis has not been shown to improve
motion. Transferred digits function primarily as a post.53,56 In
a series of 28 second toe transfers in 14 patients, Van Holder
et al.57 found a mean extension deficit of 20° and total active
flexion of 80°. In contrast, in a series of 65 second toe transfers
in 58 patients, Foucher et al.58 reported an average extensor
lag of 25° but total active motion of only 38°.
The long-term outcomes of microsurgical toe transfers have
been reported by Kaplan and Jones42 using the Pediatric
Outcomes Data Collection Instrument (PODCI). In 12 patients
with an average follow-up of 7.6 years post-surgery, two
re-operations were performed: a tenolysis with arthrodesis
and a sural nerve grafting with a rotational osteotomy. The
parents reported lower scores for upper extremity function,
sports and physical function, and global function, and levels
of happiness similar to general population while the patients
reported lower than normal scores in upper extremity func­
tion and transfer and mobility but levels of happiness higher
than the normal population.
Kay et al.56 reported the psychological outcomes after toe-
to-hand transfers in 17 patients with symbrachydactyly. Both
the parents and patients had a high level of satisfaction for
appearance, function, reaction of others, well-being, and
donor site. The authors also found lengths of up to 100% of
the contralateral toe with normal growth (Figs. 29.12–29.16).
radiolucent physes and 83–100% growth of the contralateral
phalanx if the patient was between 6 and 18 months old at the
time of surgery. As the patient’s age at surgery increased, the
percentage of open physes decreased (67% if between 18
months and 5 years; 50% if older than 5 years), but the per­
centage of growth remained the same. Over time, patients
have reported donor site morbidity in the residual foot. Con­
cerns have been related to the appearance of the foot and
footwear issues related to abnormal toe position. Donor toes
may be short, floppy and unstable. Deformity is sometimes
noted in adjacent toes.45 Donor toe deformity may be mini­
mized by iliac crest bone graft placed at the donor site.46
Distraction lengthening of a short phalanx or metacarpal
can be accomplished with or without intercalary bone graft­
ing. The technique expands the osseous and soft-tissue length
of the digit and does not require neurovascular reconstruc­
tion.47 Kessler et al.47 in 1977 applied osteotomy and distraction
lengthening of one or more metacarpals in 11 children between
5 and 11 years of age, achieving 2.5–4 cm of length. Each
patient underwent a second procedure for interposition of
bone graft to encourage earlier healing. Arslan30 reviewed six
patients who underwent distraction osteogenesis without
grafting for brachydactyly. Indications were decreased pinch
and cosmesis. A proximal metaphyseal osteotomy and exter­
nal fixator were used in all but one patient who had a diaphy­
seal osteotomy. After a 6-day resting period, lengthening was
accomplished at 0.25 mm/day until desired length and the
fixator was then left in place until consolidation (average
12.25 weeks). Pinch improved in the patients with thumb
(n = 2) and index finger (n = 2) lengthening, but those per­
formed for cosmesis of ring finger (n = 2) brachymetacarpia
were dissatisfied with the scar tissue appearance from the
pin sites.
Thumb and first webspace abnormalities are common.
The supinated thumb can lie in the plane of the hand, with
limited grasp and pinch.48 The thumb may be adducted and
retropulsed. Metacarpal rotational osteotomy can better
position the thumb for pinch. Deepening the first web with
release of tight fascia and contracted muscle can be done
at the same surgery. A dorsal rotational flap, dorsal and
palmar triangular flaps, or a four-flap Z-plasty can be used
to widen the first webspace.49 Langer et  al.48 combined first
webspace deepening with an abduction–rotational osteotomy
of the thumb metacarpal in 14 patients. The average palmar
abduction of the metacarpal was 73° with pronation of 60–90°
for pulp-to-pulp pinch. In some patients, Huber transfer for
opposition, metacarpal lengthening, interphalangeal joint
arthrodesis, long finger metacarpal lengthening, and syn­
dactyly release were performed in conjunction with the
thumb reconstruction. Patients underwent an average of four
surgeries per hand. Because of recurrent deformity, three of
14 patients underwent revision osteotomy, while eight of
14 underwent repeat first web deepening. All patients were
ultimately able to perform key pinch, lateral pinch, and
experienced subjective improvement in function and appear­
ance. Five patients were unable to oppose the thumb to
any digit.
Adding sensate digits to a hand can increase a child’s
prehension, pinch, grasp, and function. Microsurgical toe
transfers are useful for a child with an absent thumb distal to
the carpometacarpal joint, absent digits except an ulnar finger,
and for an adactylous hand.42 The adactylous hand will
Clinical tips
Symbrachydactyly
• Symbrachydactyly patients can be evaluated based on
number of functioning digits.
• This can be used to strategize a treatment plan to advance
their function.
• Conservative observation as well as surgical procedures
from nubbin removal, web deepening, to toe transfer are
available treatment options.

665
Undergrowth
Fig. 29.12  (A) A comparison bilateral anterior–posterior (AP) X-ray of a 9-month-old male with bilateral symbrachydactyly and (B) an AP X-ray of just the right hand.
These show shortened digits with absent middle phalanges and retained proximal and distal phalanges.
A
B
Fig. 29.13  (A,B) Left hand dorsum and palm of the patient from Fig. 29.12 with
shortened digits and syndactylized small and ring fingers.
A
B
Fig. 29.14  (A,B) The patient from Figs. 29.12 and 29.13 with hands side by side
and just the dorsum of the right hand. This shows distal webs beyond the
metacarpal phalangeal joints
A
B

666
SECTION IV
CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
Fig. 29.15  (A,B) The patient from Figs.
29.12–29.14: postoperative photographs of
the palmer left and right hands, respectively.
The left hand underwent separation with
full-thickness skin grafting of the ring and
small fingers. The right hand underwent
webspace deepening of the ring–small and
index–middle fingers.
A
B
Fig. 29.16  The patient from Figs. 29.12–29.15: dorsal view of the right hand
following webspace deepening.
Skeletal abnormalities
Introduction
Generalized skeletal abnormalities include multiple heredi­
tary osteochondromas, Maffucci syndrome, and Ollier disease.
Multiple hereditary osteochondromatosis
Multiple hereditary osteochondromatosis (MHO) is a skeletal
dysplasia that affects bones that develop through endochondral
ossification. It is inherited through an autosomal-dominant
pattern and is also referred to as multiple hereditary exosto­
ses and multiple exostoses.59 Osteochondromas are common,
benign bone tumors that usually form in the metaphysis of
long tubular bones. Upper limb osteochondromas are com­
monly noted in the proximal humerus, distal radius and
ulna, and metacarpals and phalanges.60–62 They are evident in
Fig. 29.17  X-ray of a 16-year-old male with familial multiple hereditary
osteochondromas (MHO) with a large osteochondroma of the volar aspect of the
radial.
skeletally immature children, growing over the first decade
of life and usually decrease in growth and stop growing at
skeletal maturity.62 They are cartilage-capped exostoses that
can be solitary or associated with a skeletal dysplasia as in
MHO (Figs. 29.17 and 29.18).
MHO usually shows full penetrance and is usually associ­
ated with a defect in either the EXT1 (chromosome 8) or EXT2
(chromosome 11). These genes have a role in regulating chon­
drocyte maturation and differentiation, which, in turn, affects
endochondral ossification.63 Reports suggest that between
30% and 60%59,61,64 have forearm involvement while between
30% and 79%62,63,65–67 demonstrate hand involvement.
Osteochondromas in the hand are usually asymptomatic
but can cause bone shortening and angulation. If a distal
phalangeal osteochondroma is subungual, the lesion may
distort nail growth. In patients with multiple lesions, opera­
tive intervention may be necessary for large lesions or to
address deformity.63 Woodside et al.62 reviewed 83 hands of
patients with an average age of 11 years at initial presentation

667
Skeletal abnormalities
over time was seen in children age 2–6 years at initial presen­
tation, likely reflecting rapid skeletal growth. As the patients
reached skeletal maturity, the tumors stabilized and little
change was seen. Cates et al.29 and Wood et al.68 also found
increased involvement of the small finger and metacarpopha­
langeal joints of the ring and small finger, with sessile lesions
the most common.
MHO of the forearm can cause bowing of the radius,
shortening of the ulna with disruption of the radioulnar joint,
ulnar deviation of the wrist, and radial head dislocation.59,61,69,70
Solomon theorized that the radius either bowed or dislocated
at the radiocapitellar joint in response to relative shortening
of the ulna.67 Burgess and Cates60 reviewed 35 patients
and 65 forearms (average: 11-year follow-up) using defined
radiographic measurements and using normal values from
Gindhart71 (Table 29.4). The results did not show a correlation
of the radial articular deformity with the degree of negative
ulnar variance, which does not support a theory of ulnar
tethering causing the distal radial deformity. There was a
strong correlation between radial and ulnar growth deficits,
with the ulna occurring at a more rapid rate. Wood72 and
Cates60 observed isolated distal radius metaphyseal exostosis
did not affect forearm length. Ulnar shortening of more than
8% was significantly correlated with radial head dislocation.
The authors also found that carpal slip was not related to
any other measurements or to the severity of the disease
process.60
Gottschalk et al.59 reviewed 48 patients with 76 affected
forearms, initial average age of 12 years, with 7-year follow-up
to determine the natural history of the forearm deformity
seen in MHO patients. Forearms are classified according to
osteochondroma location: (1) in distal ulna only; (2) distal
radius and ulna; (3) distal radius only; (4) diaphysis of radius
or ulna; and (5) proximal radius or ulna. The authors mea­
sured radial articular angle, percent ulnar variance, radial
bow, radial length, and ulnar length on radiographs as
Table 29.4  Measurements for forearm multiple hereditary osteochondromatosis
Measurement
Definition
Ulnar variance (UV)
Shortening of the ulnar relative to the radius
Percent ulnar variance (PUV)
Ulnar variance/length of the linear axis of the forearm
Radial articular angle (RAA)
Angle of inclination of the distal articular surface of the radius from the linear axis of the forearm
Radial bow (RB)
Greatest distance the radial diaphysis deviates from the linear axis of the forearm divided by the length
of the linear axis
Percent carpal slip (PCS)
Percentage of the lunate unsupported by the radius
Radial length (RL)
Center of proximal physis to center of distal physis
Radial shortening (RS)
Mean radial length of controls* minus the radial length of the patient
Radial z-score (R-z)
Radial shortening corrected for the standard deviation of the radial length of controls* [patient’s length
(mm) − normal length]/one standard deviation = ratio is the deviation of the length of the radius from
that controls*
Ulnar shortening (US)
Predicted length of the linear axis minus ulnar length
Ulnar z-score (U-z)
Ulnar shortening/standard deviation
*Based on Gindhart PS. Growth standards for the tibia and radius in children aged one month through eighteen years. Am J Phys Anthropol. 1973;39:41-8.
(Measurements from radiographs as described by Burgess RC, Cates H. Deformities of the forearm in patients who have multiple cartilaginous exostosis.
J Bone Joint Surg Am. 1993;75:13-8.)
Fig. 29.18  A clinic photograph of the prominence, the patient hand median
neuropathy with a Tinel’s sign over the mass.
and found the small finger was the most often affected and
the thumb the least often affected. Most of the lesions were
sessile, smaller tumors with < 50% of the bone affected.
Patients had an average of 13.1 tumors per hand and the
involvement of five bones per hand. An average of two bones
per hand had angulation and shortening. The metacarpals of
the small and index finger were the most commonly affected
bone and the metacarpals on the ulnar side of the hand tended
to have the most shortening and angulation. At follow-up
(average 4.6 years), an increase of 2.7 lesions per hand in the
number of detected tumors was evident. Spontaneous regres­
sion of eight tumors was noted in one hand while an increase
of 16 tumors was noted in another hand. Patients of 7–10 years
of age demonstrated the most tumors. The largest increase

668
SECTION IV
CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
Ollier disease and Maffucci syndrome
Ollier disease is a rare (estimated 1/100 000) congenital, non-
hereditary condition that affects cartilage ossification.
Involved patients form enchondromas at the metaphyseal
ends of bones.75 Enchondromas are intraosseous cartilaginous
tumors. They are usually benign and can be solitary lesions
or multiple. Multiple enchondromas in a region are termed
Ollier disease.75 When multiple regional enchondromas are
associated with soft-tissue and visceral hemangioma malfor­
mations, the disease is termed Maffucci syndrome.76 The
metacarpals and phalanges are the common sites of hand
involvement.75,77
Patients present with palpable bony masses on the finger
or hand and usually unilateral asymmetry of the extremity.
The lesions in the metacarpals or phalanges expand the cortex
and cause palpable lesions. The affected bones are often short
and deformed. Radiographs can demonstrate calcifications
with stippled appearance and metaphyseal widening. Initially
enchondromas are adjacent to the growth plate cartilage;
with growth, enchondromas migrate away from the physis.75
Tomlinson et al.77 reviewed 22 hands in 11 patients at an
average age of 17 years. Tomlinson found an average of 8.1
bones affected per hand, with the most common bones being
the proximal phalanges of the middle, ring, and small fingers
on the left, and proximal and middle phalanges of the middle
finger and middle phalanx of the ring finger on the right. The
index finger and thumb were the least commonly affected
bones (Fig. 29.19A–C).
Malignant transformation is possible within enchondro­
mas. The malignant lesions show cortical erosions, extension
of tumor into soft tissues, and irregularity of the surface of the
tumor, while enchondromas demonstrate uniform mineral­
ization.75 Chondrosarcomas are less well circumscribed and
show poor demarcation. Maffucci syndrome is associated
described by Burgess.60 The authors did not find any signifi­
cant differences in radiographic measurements with regards
to the forearm classification. The smallest radial z-scores (R-z)
and ulnar z-scores (U-z), indicating more severe shortening,
were found in type 1 forearms that presented with a dislo­
cated radial head. Type 4 had the largest R-z and U-z scores,
or the least radial and ulnar shortening. Twelve of the 13
forearms with radial head dislocations were type 1. When
type 1 forearms retained a reduced radiocapitellar joint,
significant differences were seen in radial shortening (−2.2 cm
vs. −3.8 cm), ulnar shortening (−3.2 cm vs. −5.2 cm), and
percent ulnar variance (7% vs. 10%). The authors conclude
that the results support the ulnar tether theory for radial head
dislocation.73 When followed longitudinally, patients with an
osteochondroma of the distal ulna have a normal-appearing
radius that gradually deforms with increased shortening of
the ulna that can lead to eventual dislocation of the radial
head. This suggests a tether around the distal radioulnar joint
that impedes longitudinal radial growth, and with more
severe ulnar shortening, increased radial shortening and
deformity (bowing, articular surface tilting, and radial head
dislocation) can be seen.59
Treatment
Treatment remains controversial and varies from conservative
observation to aggressive surgical intervention. Peterson69
recommends intervention to prevent radial head dislocation
for at-risk forearms. Surgical treatment includes excision of
osteochondroma when it interferes with growth or function
or causes pain, ulnar lengthening with osteotomy to prevent
further radial deformity, ulnar tether release, hemiepiphyseal
stapling of the radius, and osteotomy of the radius.59,69,72,74
Diligent radiographic monitoring of these patients is
necessary.
Fig. 29.19  (A–C) Anterior–posterior , oblique, and lateral X-rays of a skeletally mature patient with Ollier disease and multiple enchondromas in the ring and small fingers.
A
B
C

669
Future directions
Future directions
Research involving the PIK3CA-related overgrowth spectrum
and advances in genetic testing and gene therapy will greatly
advance our understanding of the disease and help to possibly
provide different treatment options. Current ongoing research
in functional capabilities in children with symbrachydactyly
will help advance the physician’s understanding and allow
better treatment selections to help improve that function.
Registries that track these more rare conditions can allow
cooperative studies of larger populations of patients with
congenital hand anomalies and, with the addition of a new
classification system, future research will be more streamlined
from physician to physician.
with a high risk of malignant transformation, generally quoted
as being between 17% and 50%. One study has suggested that
there is up to 100% chance of malignancy with very long-term
follow-up. Ollier disease has a smaller risk (25–30%) of malig­
nancy than does Maffucci syndrome.76,78,79
Most multiple enchondroma patients do not have func­
tional limitations from hand tumors, some may experience
shortening, limitations from tumor size, and problems with
hand appearance.80 Kim et al.80 reviewed three patients on
whom six procedures were performed on 30 bones for cosme­
sis. All enchondromas showed thinned and bulging cortices.
A flap of this expanded cortex was raised, the lesion curetted
and some of the bone flaps were trimmed to reduce the
volume of the bulging cortex. Some lesions were filled with
bone substitute. The authors noted bony union in all patients
with improvement in hand appearance without compromis­
ing finger range of motion. Seventy-four percent had at least
a 30% reduction in bulging on radiographs. Thirty-seven
percent had greater than 40% reduction.
Sassoon et al.81 reviewed 80 patients surgically treated with
solitary and multiple enchondromas of the hand and found
that the ring and small fingers were the most commonly
affected and the thumb was the least frequently affected. The
majority of lesions were monocentric with cortical expansion,
and centralized within the bone. Patients in Sassoon’s series
with multiple enchondromas showed full healing after
surgery with new bone formation in 61%, restoration of full
motion in 86%, recurrence in 21%, and one case of malignant
transformation. The malignant transformation occurred in a
55-year-old female with Maffucci syndrome who underwent
amputation through the small finger metacarpophalangeal
joint.
Clinical tips
Osteochondromas
• MHO shows multiple osteochondromas in the skeleton and
can affect the hand and upper extremity.
• These osteochondromas can be associated with bone
shortening, angulation, and blocking motion of joints.
• Treatment of multiple osteochondromas involves
observation or surgical excision.
• Ollier disease is a condition of multiple enchondromas,
while Maffucci syndrome has these and hemangiomas.
Maffucci syndrome has a higher malignant transformation
rate.
4. Tonkin MA, Tolerton SK, Quick TJ, et al. Classification of congenital
anomalies of the hand and upper limb: development and
assessment of a new system. J Hand Surg Am. 2013;38:1845–1853.
The new OMT classification is changing how the congenital upper limb
anomaly community discusses and performs research. This classification
takes into account what research has developed on limb embryology and
used the morphologic characteristics to allow for a very straightforward
usable system.
6. Keppler-Noreuil KM, Rios JJ, Parker VE, et al. PIK3CA-related
overgrowth spectrum (PROS): diagnostic and testing eligibility
criteria, differential diagnosis, and evaluation. Am J Med Genet A.
2015;167:287–295. This article and discovery of the PIK3CA gene related
to overgrowth syndromes advances testing and will possibly allow for
genetic-based therapies for treatment. Recognition of genetic causes for
syndromes and diseases helps to advance our understanding and guide
research to develop testing and treatments.
11. Cerrato F, Eberlin KR, Waters P, et al. Presentation and treatment of
macrodactyly in children. J Hand Surg Am. 2013;38:2112–2123. This is
a comprehensive article that addresses presentation, disease characteristics,
treatment, and outcome of macrodactyly in children. It also addresses
lipomatous versus nerve-territory macrodactyly.
35. Woodside JC, Light TR. Symbrachydactyly – diagnosis, function,
treatment. J Hand Surg Am. 2016;41:135–143. This is a comprehensive
review of symbrachydactyly discussing diagnosis, the function of patients
with the syndrome, and treatment options. It is a comprehensive review of
current research and ideas of approach to the patient.
39. Vilkki SK. Advances in microsurgical reconstruction of the
congenitally adactylous hand. Clin Orthop Relat Res. 1995;45–58.
42. Kaplan JD, Jones NF. Outcome measures of microsurgical toe
transfers for reconstruction of congenital and traumatic hand
anomalies. J Pediatr Orthop. 2014;34:362–368.
43. Buck-Gramcko D. Symbrachydactyly: a clinical entity. Tech Hand Up
Extrem Surg. 1999;3:242–258.
50. Jones NF, Hansen SL, Bates SJ. Toe-to-hand transfers for congenital
anomalies of the hand. Hand Clin. 2007;23:129–136. This is an
excellent resource for indications, timing for surgery, and the surgical
technique of toe-to-hand transfers in the pediatric population. It contains
high-quality images and outcomes for patients postoperatively.
60. Burgess RC, Cates H. Deformities of the forearm in patients who
have multiple cartilaginous exostosis. J Bone Joint Surg Am.
1993;75:13–18.
64. Stanton RP, Hansen MO. Function of the upper extremities in
hereditary multiple exostoses. J Bone Joint Surg Am. 1996;78:568–573.
Access the complete reference list online at

669.e1
References
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The new OMT classification is changing how the congenital upper limb
anomaly community discusses and performs research. This classification
takes into account what research has developed on limb embryology and
used the morphologic characteristics to allow for a very straightforward
usable system.
5. Flatt AE. The Care of Congenital Hand Anomalies. 2nd ed. St. Louis,
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2015;167:287–295. This article and discovery of the PIK3CA gene related
to overgrowth syndromes advances testing and will possibly allow for
genetic-based therapies for treatment. Recognition of genetic causes for
syndromes and diseases helps to advance our understanding and guide
research to develop testing and treatments.
7. Keppler-Noreuil KM, Sapp JC, Lindhurst MJ, et al. Clinical
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23. Bertelli JA, Pigozzi L, Pereima M. Hemidigital resection with
collateral ligament transplantation in the treatment of macrodactyly:
a case report. J Hand Surg Am. 2001;26:623–627.
24. Hardwicke J, Khan MA, Richards H, et al. Macrodactyly – options
and outcomes. J Hand Surg Eur Vol. 2013;38:297–303.
25. Koskimies E, Lindfors N, Gissler M, et al. Congenital upper limb
deficiencies and associated malformations in Finland: a population-
based study. J Hand Surg Am. 2011;36:1058–1065.
26. Burgess RC. Brachydactyly type C. J Hand Surg Am. 2001;26:
31–39.
27. Temtamy SA, Aglan MS. Brachydactyly. Orphanet J Rare Dis.
2008;3:15.
28. Bell J. The Treasury of Human Inheritance: On Hereditary Digital
Anomalies. London: Cambridge University Press; 1951.
29. Cates HE, Burgess RC. Incidence of brachydactyly and hand
exostosis in hereditary multiple exostosis. J Hand Surg Am.
1991;16:127–132.
30. Arslan H. Metacarpal lengthening by distraction osteogenesis in
childhood brachydactyly. Acta Orthop Belg. 2001;67:242–247.
31. Nguyen ML, Jones NF. Undergrowth: brachydactyly. Hand Clin.
2009;25:247–255.
32. Ekblom AG, Laurell T, Arner M. Epidemiology of congenital upper
limb anomalies in Stockholm, Sweden, 1997 to 2007: application of
the Oberg, Manske, and Tonkin classification. J Hand Surg Am.
2014;39:237–248.
33. Swanson AB, Swanson GD, Tada K. A classification for congenital
limb malformation. J Hand Surg Am. 1983;8:693–702.
34. Blauth W, Gekeler J. [Morphology and classification of
symbrachydactylia]. Handchirurgie. 1971;3:123–128.
35. Woodside JC, Light TR. Symbrachydactyly – diagnosis, function,
treatment. J Hand Surg Am. 2016;41:135–143. This is a comprehensive
review of symbrachydactyly discussing diagnosis, the function of patients
with the syndrome, and treatment options. It is a comprehensive review of
current research and ideas of approach to the patient.
36. Knight JB, Pritsch T, Ezaki M, et al. Unilateral congenital terminal
finger absences: a condition that differs from symbrachydactyly.
J Hand Surg Am. 2012;37:124–129.
37. Catena N, Divizia MT, Calevo MG, et al. Hand and upper limb
anomalies in Poland syndrome: a new proposal of classification.
J Pediatr Orthop. 2012;32:727–731.
38. Bavinck JN, Weaver DD. Subclavian artery supply disruption
sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil,
and Möbius anomalies. Am J Med Genet. 1986;23:903–918.
39. Vilkki SK. Advances in microsurgical reconstruction of the
congenitally adactylous hand. Clin Orthop Relat Res. 1995;
45–58.
40. Case-Smith J. Clinical interpretation of “development of in-hand
manipulation and relationship with activities”. Am J Occup Ther.
1995;49:772–774.
41. Gordon A, Forssberg H. Development of neural mechanisms
underlying grasping in children. In: Connolly K, Forssberg H, eds.
Neurophysiology and Neuropsychology of Motor Development. London:
MacKeith Press; 1997:214–231.
42. Kaplan JD, Jones NF. Outcome measures of microsurgical toe
transfers for reconstruction of congenital and traumatic hand
anomalies. J Pediatr Orthop. 2014;34:362–368.
43. Buck-Gramcko D. Symbrachydactyly: a clinical entity. Tech Hand Up
Extrem Surg. 1999;3:242–258.
44. Goldberg NH, Watson HK. Composite toe (phalanx and epiphysis)
transfers in the reconstruction of the aphalangic hand. J Hand Surg
Am. 1982;7:454–459.
45. Garagnani L, Gibson M, Smith PJ, et al. Long-term donor site
morbidity after free nonvascularized toe phalangeal transfer. J Hand
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46. Bourke G, Kay SP. Free phalangeal transfer: donor-site outcome.
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47. Kessler I, Baruch A, Hecht O. Experience with distraction
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48. Langer JS, Manske PR, Steffen JA, et al. Thumb in the plane of the
hand: characterization and results of surgical treatment. J Hand Surg
Am. 2009;34:1795–1801.

669.e2
SECTION IV
CHAPTER 29 • Congenital hand V: Overgrowth, undergrowth, and generalized skeletal deformities
49. Li WJ, Zhao JH, Tian W, et al. Congenital symbrachydactyly:
outcomes of surgical treatment in 120 webs. Chin Med J.
2013;126:2871–2875.
50. Jones NF, Hansen SL, Bates SJ. Toe-to-hand transfers for congenital
anomalies of the hand. Hand Clin. 2007;23:129–136. This is an
excellent resource for indications, timing for surgery, and the surgical
technique of toe-to-hand transfers in the pediatric population. It contains
high-quality images and outcomes for patients postoperatively.
51. Cobbett JR. Free digital transfer. Report of a case of transfer of a
great toe to replace an amputated thumb. J Bone Joint Surg Br.
1969;51:677–679.
52. Buncke HJ, Buncke CM, Schulz WP. Immediate Nicoladoni
procedure in the rhesus monkey, or hallux-to-hand transplantation,
utilising microminiature vascular anastomoses. Br J Plast Surg.
1966;19:332–337.
53. Gilbert A. Toe transfers for congenital hand defects. J Hand Surg
Am. 1982;7:118–124.
54. Eaton CJ, Lister GD. Toe transfer for congenital hand defects.
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55. Richardson PW, Johnstone BR, Coombs CJ. Toe-to-hand transfer in
symbrachydactyly. Hand Surg. 2004;9:11–18.
56. Kay SP, Wiberg M, Bellew M, et al. Toe to hand transfer in children.
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57. Van Holder C, Giele H, Gilbert A. Double second toe transfer in
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58. Foucher G, Medina J, Navarro R, et al. Toe transfer in congenital
hand malformations. J Reconstr Microsurg. 2001;17:1–7.
59. Gottschalk HP, Kanauchi Y, Bednar MS, et al. Effect of
osteochondroma location on forearm deformity in patients with
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60. Burgess RC, Cates H. Deformities of the forearm in patients who
have multiple cartilaginous exostosis. J Bone Joint Surg Am.
1993;75:13–18.
61. Solomon L. Bone growth in diaphysial aclasis. J Bone Joint Surg Br.
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62. Woodside J, Ganey T, Gaston RG. Multiple osteochondroma
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63. Stieber JR, Dormans JP. Manifestations of hereditary multiple
exostoses. J Am Acad Orthop Surg. 2005;13:110–120.
64. Stanton RP, Hansen MO. Function of the upper extremities in
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65. Black B, Dooley J, Pyper A, et al. Multiple hereditary exostoses. An
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68. Wood VE, Molitor C, Mudge MK. Hand involvement in multiple
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74. Pritchett JW. Lengthening the ulna in patients with hereditary
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81. Sassoon AA, Fitz-Gibbon PD, Harmsen WS, et al. Enchondromas of
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malignant transformation. J Hand Surg Am. 2012;37:1229–1234.

SECTION IV • Congenital Disorders
30 
Growth considerations in pediatric upper
extremity trauma and reconstruction
Marco Innocenti and Carla Baldrighi
SYNOPSIS
■ Skeletal growth is possible because of the presence of an active
physis.
■ The physis is a temporary anatomical structure physiologically
regulated by several factors. It can be hindered by congenital
conditions (chondrodysplasias), direct damage, or interruption of its
blood supply.
■ Regardless of its etiology, when the physis and or the epiphysis are
damaged in a skeletally immature individual, three main issues must
be addressed: restoration of longitudinal growth, restoration of joint
congruency and function, and replacement of the osseous defect.
■ Therapeutic options include completion of epiphyseal arrest, resection
of physeal bar, and epiphyseal distraction.
■ Where there is involvement of the whole epiphysis, autologous
epiphyseal transfer may achieve the dual goals of restoring joint
function and growth potential. The longer the period of time between
the epiphyseal insult and the expected end of growth, the stronger the
indication for surgical treatment.
Introduction
■ The growth plate is a temporary anatomical entity, which
allows axial growth in long bones. Once skeletal maturity
is approached, the function of the growth plate gradually
decreases and eventually stops.
■ Trauma, infection, irradiation, thermal injury, tumors, and
congenital disorders may affect the growth plate and
interfere with the growth process.
■ Any damage to the growth plate in a skeletally immature
individual leads to growth disturbance with deformity
and/or length discrepancy.
■ Goals of the surgical treatment should be restoration of
the physiological growth, prevention of angular
deformities, and correction of established deviation of
bones and joints.
Basic science/disease process
Anatomy and physiology of the epiphyseal
growth plate
The physis (also known as growth plate, epiphyseal plate,
epiphyseal growth plate, epiphyseal cartilage) is a highly
specialized and organized cartilaginous structure derived
from the mesoderm. It develops in the bone bud, secondary
to the primary ossification centers (metaphysis) and is respon­
sible for longitudinal and circumferential bone growth.1 The
physis must be distinguished from the epiphysis, or second­
ary ossification center (Fig. 30.1).
The physis consists of proliferating chondrocytes sur­
rounded by synthesized extracellular matrix. The extracellular
matrix is composed of water, collagen fibrils (mainly types II,
IX, X, and XI) and proteoglycans (aggrecan, decorin, annexin
II, V, and VI) arranged to form a sort of sponge with very
small pores.2 This arrangement confers peculiar mechanical
properties that permit the physis to be “hard” when an axial
load is applied rapidly (a jumping child) or “soft” when
deformed slowly (when chondrocytes secrete new extracel­
lular matrix).3
The physis is traditionally divided into horizontal zones of
chondrocytes at different stages of maturation (see Fig. 30.1).
The resting zone (reserve zone or germinal matrix), immedi­
ately adjacent to the epiphysis, contains small, uniform,
irregularly scattered chondrocytes, also referred to as stem
cells, with low rates of proliferation but rich in storage materi­
als (lipids and cytoplasmic vacuoles) for later growth.4,5 The
resting zone is responsible for protein synthesis and for
maintaining a germinal structure. Injury to this layer results
in cessation of growth.
When chondrocytes enter into the proliferative zone they
undergo rapid duplication, increase the synthesis of collagen,
in particular types II and XI,4 and become flat and well orga­
nized into longitudinal columns. Mitotic activity is present
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671
Basic science/disease process
Vascular anatomy of the growth plate
The epiphyseal and physeal cartilage vascular anatomy in
newborn and early postnatal life is integral to long-bone
development and differs from later postnatal periods. In late
fetal and early postnatal life, epiphyseal cartilage canals (or
transphyseal vessels) are seen passing through the physeal
cartilage and communicating with the metaphyseal marrow.
Such vessels play an active role in forming the secondary
ossification centers18–20 but their primary earlier function is to
provide nutrition by diffusion to epiphyseal chondrocytes.
The transphyseal vessels are constantly obliterated several
months after birth. Once the obliteration has occurred, the
physeal cartilage becomes an avascular structure supplied by
the epiphyseal vessels by diffusion, and by the metaphyseal
vessels invading the lowermost regions of the hypertrophic
zone.21,22
The main vascular supply to the germinal zone is from the
epiphyseal vessels. Two types of epiphyseal vascularization
are described.23 Type A epiphyses are almost entirely covered
by articular cartilage, and the epiphyseal vessels enter the
epiphysis after traversing the perichondrium. With this ana­
tomical configuration, the blood supply to the epiphysis, and
consequently to the germinal zone, is susceptible to damage
if the epiphysis is separated from metaphysis. Type B epiphy­
ses are only partially covered by articular cartilage. Their
blood supply enters from the epiphyseal side and is protected
from vascular injury during separation. The proximal femur
and proximal radius are the only two examples of type A
epiphyses.
Growth plate closure and skeletal age
assessment during puberty
It is generally assumed that long bone physes close at 14 years
of skeletal age in females and at 16 years in males while the
axial skeleton completes its development later.24–26 In 50% of
normal children and adolescents, skeletal age does not differ
from chronological age.27 Prediction of limb length discrep­
ancy and final standing height, as well as decisions regarding
when to perform an epiphysiodesis, are first subjected to
determination of skeletal age.
A complication unique to physeal injuries is growth distur­
bance. Once damage to the physis has occurred, the predicted
amount of growth remaining from that specific physis must
be determined to plan the treatment. This can be accomplished
by determining the skeletal age of the patient and then using
information on physeal growth rates and patterns assembled
by Green and Anderson.28–32
Treatment decisions are often made during puberty. Puberty
is the period of time in the growing individual characterized
by a significant increase in growth rate.33,34 In girls, puberty
starts at 11 years and ends at 13 years of skeletal age; in boys,
it starts and ends 2 years later (13 years and 15 years of
skeletal age).27 Peak height growth and Tanner stage 235 signal
the beginning of puberty. The 2 years of pubertal growth spurt
are known as acceleration phase followed by a deceleration
phase that continues until skeletal maturity.
The radiographic markers currently used to assess skeletal
age are all based on the appearance of ossification centers
in specific skeletal segments at specific times. Several age
assessment methods are based on the use of anteroposterior
only at the base of these columns. This layer is responsible for
longitudinal growth of the bone via active cell division. These
first two zones have an abundant extracellular matrix that
confers a great deal of mechanical strength, in particular in
response to shear forces.
Further morphological changes – maturation, degenera­
tion, and provisional calcification – take place in the transfor­
mation (or hypertrophic) zone, divided into upper and lower
hypertrophic zones.
The presence of provisional calcification4–6 confers shear
resistance to the lower hypertrophic zone. In contrast, the
upper hypertrophic zone containing scant extracellular matrix
is the weakest portion of the physis and it is here that most
injury or alteration to the physis occurs.7–9
In continuity with the metaphysis is the zone of endochon­
dral calcification where the mineralization process of the
matrix becomes more intensive.
The periphysis is a fibrochondroosseous structure that
surrounds the physis of tubular bones and plays an important
role in skeletal development10 (see Fig. 30.1): (1) it allows
gradual circumferential growth of the physis; (2) it continues
to maintain its transverse diameter11; and (3) it is critical to the
overall stability of the growth plate and to support for the
physis bone–cartilage junction.11–13
The part of the periphysis adjacent to the epiphysis is a
wedge-shaped group of germinal cells known as the zone of
Ranvier, while the part adjacent to the metaphysis is known
as the ring of LaCroix. Histologically, the Ranvier and LaCroix
zones are a single structure (see Fig. 30.1).
Chondrocyte proliferation and differentiation in the physis,
and therefore longitudinal skeletal growth, are regulated at
both a systemic and local level.14 A number of endocrine,
paracrine, and autocrine factors and their respective receptors
are involved in this process. Amongst these, the parathyroid
hormone-related protein (PTHrP) and the Indian hedgehog
(Ihh) play a prominent role in skeletal maturation.1,15–18 There
is also mechanical control of growth, which is important
in pediatric orthopedic surgery because it is the basis of
the widely used epiphysiodesis and epiphyseal distraction
procedures.3
Fig. 30.1  Cross-sectional anatomy of the physis.
Secondary ossification
center/epiphysis
Germinative zone
Proliferative zone
Hypertrophic zone
Zone of enchondral
ossification
Periosteal sleeve
Primary ossification
center
Metaphyseal
artery
Zone of
Ranvier
Ring of
LaCroix
Epiphyseal
artery
Physis

672
SECTION IV
CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
(Figs. 30.2 & 30.3). Elbow ossification centers always appear
in a determined sequence (the mnemonic is CRITOE: capitel­
lum, radial head, internal (medial) epicondyle, trochlea,
olecranon, external (lateral) epicondyle). The ages of appear­
ance, as a general guide, are 1–3–5–7–9–11 years.
During the acceleration phase, the skeletal age is best
assessed with X-rays of the left hand and left elbow.42–45 In the
deceleration phase, the growth rate decreases significantly.
The remaining growth in both boys and girls is around 6 cm,
of which 4.5 cm belong to the trunk and skull. During this
phase, the most useful tools for skeletal age assessment are
again the left hand and the Risser stage.
Regardless of the assessment method used, skeletal age
alone is not enough and it should be related to other clinical
and radiological findings such as standing and sitting height,
Tanner stage, and annual growth rate.
radiographs of the left hand and wrist.36–39 Other methods
use left elbow radiographs.27,40 The axial skeleton can also
be used to determine the skeletal age: Risser sign41 is
based on the appearance of the left iliac apophysis of the
pelvis. However, other radiographs can be used to assess
skeletal age.27
Different assessment methods become helpful at different
ages. The Greulich and Pyle atlas36 is probably the most
common and widely used method to assess skeletal age. This
technique, however, has some limitations, especially during
puberty because it does not make it possible to assess skeletal
age at 6-month intervals during the 2 years of peak growth
rate.27 On the other hand, during the pubertal acceleration
phase, the elbow, at the level of the distal epiphysis ossifica­
tion centers and the olecranon apophysis, undergoes peculiar
and identifiable morphological changes every 6 months27,40
Fig. 30.2  Olecranon maturation
according to Dimeglio et al.,35 with
significant changes occurring every
6 months. (A) two ossification
centers (girls 11 years; boys 13
years); (B) half-moon shape (girls
11.5 years; boys 13.5 years);
(C) rectangular shape (girls 12
years; boys 14 years);
(D) beginning of fusion (girls
12.5 years; boys 14.5 years); and
(E) complete fusion (girls 13 years;
boys 15 years).
A
B
C
D
E
Humerus
Ulna
Olecranon
Fig. 30.3  Pubertal diagram. Acceleration phase, between
skeletal age of 11 and 13 years in girls and 13 and 15 years
in boys; Risser sign is 0. End of the acceleration phase,
complete elbow and distal phalanx of the thumb closure;
Risser sign is still 0.
Beginning of puberty
Girls: Age 11
Boys: Age 13
'Acceleration' phase
'Deceleration' phase
Risser I: Distal phalanx fusion
Girls: Age 13.5 (Menarche) Boys: Age 15.5
Triradiate cartilage closure
Girls: Age 12 Boys: Age 14
Risser II: Proximal phalanx fusion
Risser III: Greater trochanter fusion
Intermediate phalanx fusion
Risser IV:
Ulnar epiphysis fusion
Risser V:
Radial epiphysis fusion
END OF GROWTH
ELBOW CLOSURE
Fusion distal phalanx of the thumb
Girls: Age 13
Boys: Age 15
Risser still 0 prior to elbow closure

673
Diagnosis/patient presentation
older children. The epiphyseal fragment contains the entire
germinal layer as well as a metaphyseal fragment, known as
Thurston Holland’s sign. The periosteum on the side of the
metaphyseal fragment is usually intact and provides stability
after reduction. Growth disturbance is uncommon because
the germinal layer remains intact.
In Salter–Harris type III injury (see Fig. 30.4), the fracture
starts through the hypertrophic zone and exits through the
epiphysis. These injuries also tend to affect older children. By
definition, type III fractures cross the germinal layer and are
usually intra-articular. Consequently, they raise concerns
about growth and, when displaced, they require anatomic,
and often open, reduction.
In Salter–Harris type IV injury (see Fig. 30.4), the fracture
starts from the metaphysis and then extends through the
physis and into the epiphysis. By definition these fractures
disturb the germinal layer and are usually intra-articular.
Consequently, these injuries may impair normal growth and
affect articular congruency. In type IV injuries, an anatomic
reduction is mandatory in order to prevent osseous bridging
across the physis and to restore the articular surface.
A Salter–Harris type V injury (see Fig. 30.4) occurs when
the physis is crushed from a pure compression force. It is so
rare that some authors question whether such an injury
exists.66 For those authors who have reported on this injury,65,67
it carries the most concerning prognosis as the growth distur­
bance is almost a rule. It often requires later surgical treatment
to restore limb length and alignment.
Despite being widely used, the Salter–Harris classification
excludes a few physeal injuries: the Rang’s type VI epiphyseal
injury,62,63 which represents an injury to the perichondral ring,
and two additional injuries described in the Peterson classifi­
cation system (Fig. 30.5),56 which is very similar to the Salter–
Harris scheme. The Peterson type I fracture is a transverse
fracture of the metaphysis that extends longitudinally into the
physis. Clinically, this type of fracture is commonly seen in
the distal radius. The Peterson VI fracture is an open injury
associated with loss of the physis.
Treatment of physeal fractures
Growth plate injuries that are less severe, and occur closer to
the time of closure of the growth plate, carry the best progno­
sis. More severe injuries occurring in younger patients require
observation and possibly treatment to prevent problems. The
principles of treatment of physeal fractures are the same as
those involved in the treatment of all fractures, with a few
important considerations.
Diagnosis/patient presentation
Conditions affecting the growth plate
A complication unique to physeal injuries is growth distur­
bance. Trauma is the most common cause of physeal injury
and growth disturbance, but physeal growth arrest may as
well occur after infection, tumor, irradiation, thermal injury,
laser beam exposure, and sequelae of Blount’s disease (growth
disorder with bowing of the tibia).46–49
Trauma
Incidence and distribution in the upper extremity
Physeal injuries represent 15–30% of all fractures in children50–54
and frequently involve the upper extremities.53,55 The inci­
dence varies with age, with a peak in adolescence.53,55,56 The
upper hypertrophic zone, just above the area of provisional
calcification, is the weakest layer of the physis and where
most injuries to the physis occur.7–9 This observation implies
that, after most injuries, the germinal layer of the physis
remains intact and attached to the epiphysis. Consequent
normal growth should resume unless insult to the blood
supply of the germinal layer or development of a “bony
bridge” across the injured physis occurs.57
Classification of physeal fractures
Several classification systems for physeal injuries have been
described.58–64 The Salter and Harris classification (Fig. 30.4)65
is by far the most widely used system. This classification helps
to distinguish different types of fractures and provides prog­
nostic information as well. In Salter–Harris type I (see Fig.
30.4), the injury is a separation of the epiphysis from the
metaphysis. It occurs entirely through the physis and there­
fore the surrounding bone is not involved. It is rare and tends
to be seen in infants or pathologic fractures, such those sec­
ondary to rickets or scurvy. Because the germinal layer
remains with the epiphysis, growth is not hindered unless
blood supply is interrupted (traumatic separation of the
proximal femoral epiphysis). Often, X-rays of a child with a
type I Salter–Harris fracture will appear normal. Healing of
type I fractures tends to be rapid, and complications are rare.
In Salter–Harris type II injury (see Fig. 30.4), the fracture
extends along the hypertrophic zone of the physis but then it
continues and exits through the metaphysis. This is the most
common type of growth plate fracture, and tends to occur in
Fig. 30.4  Salter–Harris
classification of physeal
fractures. (Redrawn after Salter
RB, Harris R. Injuries involving
the epiphyseal plate. J Bone Joint
Surg Am. 1963;45:587–621.)
Type I
Type II
Type III
Type IV
Type V

674
SECTION IV
CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
located at the metaphyseal level, the growth plate and the
physis are always to be considered as a possible target of
sarcoma invasion. From a functional standpoint, it is critical
to know whether the surgical resection may spare the epiphy­
sis or not.
In the past, the growth plate was thought to be a biologic
barrier to bone tumor invasion71–73; unfortunately this theory
has not proven to be valid74–76 (Fig. 30.6). The current view is
that invasion of the physis must always be suspected when
The goal in the treatment of physeal injuries is to obtain
and maintain an acceptable reduction while avoiding any
further damage to the germinal layer of the physis during
reduction. Thus the most important goal, and the most subjec­
tive one, is to establish the limits of acceptable reduction.
The age of the patient, the location and displacement of the
fracture, and the time elapsed since the injury must be all
taken into consideration when assessing a non-anatomic
reduction. Although animal studies have not shown that
delay in reduction produces a growth disturbance,68 the rec­
ommendation is to accept any displacement in type I and II
injuries after 7–10 days9,69 and rather plan an osteotomy later.
As a general rule, the remodeling potential is inversely pro­
portionate to the age of the patient and is also related to the
location and type of injury. Consequently, a greater deformity
can be accepted in younger children.
Because type III and IV injuries are by definition intra-
articular, the focus must be on achieving an anatomic reduc­
tion regardless of the age of the patient and the time that has
elapsed since the injury. Once the physeal fracture has been
reduced, the reduction can be maintained with cast, pins,
internal fixation, or combinations of these three.
Tumor
Bone sarcoma involving the epiphysis
Most of the common primary malignant bone tumors occur
in patients aged under 30 years.70 Typically, osteosarcoma and
Ewing sarcoma are found in adolescents and young adults,
but they may also be diagnosed during infancy. The current
approach to systemic therapy70 includes preoperative
(neoadjuvant) chemotherapy and postoperative (adjuvant)
chemotherapy for both osteosarcoma and Ewing sarcoma.
Radiotherapy is indicated only in Ewing sarcoma and it may
be the only local treatment in cases of tumor in unfavorable
locations, such as spine and pelvis, and in all cases where
radical surgery is unlikely to be successful. Postoperative
radiotherapy is indicated in all cases of intralesional resec­
tions with the aim of reducing the risk of local recurrence.
With advances in bone reconstruction, the role of surgery
has become predominant in the treatment of primary bone
tumors, and the percentage of limb salvage versus amputa­
tion has significantly increased. In the upper limb, bone sar­
comas are more frequently located in the distal radius and
proximal humerus. Although the tumor is usually initially
Fig. 30.5  Peterson classification of physeal fractures. Type VI injuries are open and associated with loss of the physis. (Redrawn after Peterson HA. Physeal fractures: part
3. Classification. J Pediatr. Orthop. 1994;14:439.)
Type I
Type II
Type III
Type IV
Type V
Type VI
Fig. 30.6  Osteosarcoma involving the proximal epiphysis and the adjoining
diaphysis in a 6-year-old child.

675
Treatment/surgical technique
The extent and location of the bar, the skeletal age of the
patient, and the amount of growth remaining from the physis
must all be determined to plan the treatment of a physeal
bar appropriately. Plain radiography, tomography, computed
tomography (CT), or MRI46,78–81 may all be used to assess the
anatomy of a physeal bar. MRI is by far the preferred method
currently used to investigate physeal anatomy.82–85 In particu­
lar, fat-suppressed, 3D, gradient-recalled echo sequences can
provide an accurate 3D reconstruction of the physis and
estimate the percentage of physeal arrest.85 The classification
of partial physeal arrests is based on their location within the
physis (Fig. 30.7): peripheral (type A) or central (type B or C).
In type B, the bar develops in the center of the physis and is
surrounded by a perimeter of healthy physis. This may create
a tethering effect that “tents” the epiphysis, leading to joint
deformity. In type C (central) the bar traverses the entire
physis (front to back or side to side) while the physis on both
sides of the bar is normal.
Treatment/surgical technique
Treatment of physeal arrest
Different treatment options are available for the management
of physeal arrests. These options include observation, comple­
tion of a partial physeal arrest, epiphysiodesis, physeal bar
resection, or physeal distraction.
Observation
Observation may be the best option when the physeal bar
involves the entire physis with complete growth arrest if
existing limb length inequality and/or angular deformity are
acceptable, or if the individual is close to skeletal maturity
with little longitudinal growth remaining.
Completion of a partial physeal
arrest and epiphysiodesis
Completion of a partial physeal arrest may be indicated if
there is an acceptable existing angular deformity that may
become clinically unacceptable if left untreated. To avoid
significant limb shortening of the upper extremity, the surgeon
must evaluate the predicted remaining growth and foresee a
lengthening procedure if necessary. In the upper extremity
limb, length discrepancy is a relative issue but the asynchro­
nous rate of longitudinal growth between two bones in an
anatomic region where the two bones are paired in close
longitudinal relationship, as in the forearm, or asynchronous
growth in an anatomic region where the harmonic activity of
the tumor is located in the proximity of the growth plate and
all patients should be investigated with magnetic resonance
imaging (MRI). In the event of epiphyseal involvement, the
oncological resection must include the epiphysis and the only
possible reconstructive option for such a defect in the pediatric
age group is autologous epiphyseal transplant.
Congenital chondrodysplasia
Chondrocyte proliferation and differentiation in the physis,
and therefore skeletal growth, are regulated by various endo­
crine, paracrine, and autocrine factors. Any disturbance of the
epiphyseal growth plate physiology and development results
in various skeletal abnormalities known as dysplasia or
chondrodysplasia. These cases may also require reconstruc­
tion to maintain growth.
Patient selection
When the physis is damaged and deformity results or is
foreseen, several treatment options are available. The best
treatment for a growth plate injury depends on the individual
situation. Although physeal injuries are common, problems
arising are rare, occurring in only 1–10% of all physeal frac­
tures.9,53,56 Comminuted fractures, high-energy injuries, and
physeal injuries that cross the germinal layer are more prone
to result in physeal arrest with subsequent growth distur­
bance. Physeal injuries are most common in adolescents close
to skeletal maturity. In these individuals, the remaining
growth is limited.9,53,56 Consequently, even when it occurs,
physeal arrest will produce minimal or no length discrepancy
or angular deviation and will seldom require treatment.
Growth disturbance resulting from a physeal fracture is
usually evident 2–6 months after the injury, but it may take
up to a year to manifest.9 This information is important both
to warn the parents about possible complications and to
anticipate close long-term follow-up. In fact, the management
of a post-traumatic growth disturbance is easier when directed
exclusively towards treating the arrest rather than tackling
both the arrest and the acquired deformity.
Growth disturbance is usually a consequence of the devel­
opment of a bony bridge, or bar, across the physis.57 However,
when the injury only reduces rather than stops the growth
rate of a portion of the physis, it may still produce asymmetric
growth and angular deformity with no development of bony
bridge.77 If the bony bar involves a large portion of the physis,
it may stop the physeal growth completely. However, in most
cases, the bony bar is confined to a rather small portion of the
physis and the growth stops only at that point. The remaining
healthy physis continues to grow, creating a tethering effect,
which may produce either a shortening or a progressive sig­
nificant angular deformity, or both.
Fig. 30.7  Classification of physeal bars. Type A,
peripheral; type B, central, surrounded by normal physis;
type C, central, traversing the physis.
Type A
Type B
Type C

676
SECTION IV
CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
distraction techniques are mostly reported for the treatment
of lower limb deformities and little is known about their
application in the upper limb.
Bar resection
If a portion of the physis has prematurely closed, but the
reminder of the physis is healthy and there is substantial
growth remaining, resection of the physeal bar and insertion
of interposition materials are indicated. This technique in fact
preserves longitudinal bone growth ability.96,97 The procedure
was first introduced by Langenskiöld and has been docu­
mented in both human and animal models.47,79,96,98–101
The surgical technique of bar resection consists in remov­
ing the bone bridge along with the neighboring portions of
metaphysis and epiphysis and filling the cavity with an inert
material that will prevent recurrence of physeal bar formation
(Fig. 30.8).
Type A peripheral bars can be removed under direct vision,
taking care to resect a wide cuff of periosteum. Type B and C
central bars need to be approached through a window in the
metaphysis or through an osteotomy (see Fig. 30.8A–C). Their
resection may be facilitated by the use of fluoroscopy, fiber
optic lighting, and dental mirrors, as well as magnifying
loupes (see Fig. 30.8D).
Physeal bar resection is recommended when all the follow­
ing conditions are present: the remaining physis must be
undamaged and must be large enough to permit growth to
continue; and there should be a significant amount of growth
remaining in that physis before physiologic physeal closure.
Bars involving more than 50% of the physeal surface are
unlikely to respond to surgical treatment.48,62,79,96–98,100 Despite
the acceptance that younger patients, with higher growth
potential, will benefit from physeal bar resection, there is still
no agreement in quantifying the amount of this growth
potential.79,98,101
According to Bright,102 indications for a bone bridge resec­
tion are as follows: there must be more than 50% of remaining
healthy physis; the expected physeal growth must last for 2
years or more; there must be good soft-tissue coverage of the
lesion; and, in the case of growth arrest due to infection, the
infection must have been absent for more than 1 year.
Once the bar is completely resected, various interpositional
materials can be used to fill the void and prevent transphyseal
bone bridge formation.62,79,98 Of the interposition materials
described, fat is the most commonly used.96,103 It has the
advantage of being autologous and immediately available.
several physes contributes to the final size and shape of the
bone, such as the distal humerus, might lead to a great risk of
anatomic distortion. If the likelihood of length inequality is
high, epiphysiodesis of a non-injured physis should be per­
formed at the time of completion of the physeal arrest.
Epiphysiodesis is an established method of limb length
equalization or angular deformity correction in children
and adolescents with projected limb length discrepancies at
maturity as great as 5 or 6  cm. It consists of temporarily
stopping or permanently destroying the activity of the
whole or just a part (hemiepiphysiodesis) of the growth plate.
First introduced by Phemister in 1933,86 epiphysiodesis has
evolved into various reversible or irreversible, open or
percutaneous techniques, with and without instrumentation.
The ideal tool should be minimally invasive, have minimal
morbidity, and be reliably reversible. Pitfalls include errors
in the prediction of growth and planning of the surgery. In
particular, with non-reversible techniques, timing must be
precise. Overcompensation for limb length discrepancy or
creation of an opposite angular deformity can be very dis­
tressing for the patient, family, and surgeon. Although many
different techniques have proven their efficacy, percutaneous
epiphysiodesis using transphyseal screws combines the
minimal invasiveness of a percutaneous technique with
reversibility.87
Physeal distraction
Physeal distraction is an alternative treatment for partial
physeal arrest. Physeal distraction uses the growth plate as a
zone of least resistance. It involves the damaged physis and
therefore acts at the site of the deformity. It requires a force to
be applied longitudinally across the physis, permitting both
lengthening and angular correction on multiple planes, with
external control of the correction until consolidation. Prior
resection of the bony bar is not necessary.
A distinction must be made between distractional epi­
physiolysis and chondrodiastasis,88,89 chondrodiatasis,90 and
hemichondrodiatasis.91
The chondrodiastasis technique employs large forces or
rapid rate of distraction (> 1 mm/day) or both.92 This provokes
a distraction and opening of the physis that provides a rapid
in situ correction from the bony bar without prior bar resec­
tion, but almost invariably it produces a premature physeal
fusion.93,94 This notion limits the indications to patients nearing
skeletal maturity and preventive lengthening according to
predicted limb length discrepancy.94,95 In the literature, physeal
Fig. 30.8  (A) Central bar. (B) Bar resection through
metaphyseal approach. (C) Assessing the resection.
A
B
C

677
Treatment/surgical technique
any angular deformity that is judged to be “clinically
unacceptable”.79,98,97,106
It is well known that the physeal growth rate responds to
the force applied on the physis itself: growth is stimulated by
both mild tension and mild compression.115 However, accord­
ing to the Hueter–Volkmann principle,116 when compression
on the growth plate exceeds a certain level, growth is indeed
suppressed.115 If the compression is applied only on one
side of the physis, worsening of the deformity may ensue.
Improved alignment may therefore facilitate more normal
growth.
Bone lengthening involves the principle of distraction-
induced osteogenesis and the use of an external fixator.
Because of the non-weight-bearing status of the upper extrem­
ity, limb length discrepancy of the upper limbs is better toler­
ated and of less functional and cosmetic significance than
their counterparts in the lower extremity. For these reasons,
along with the incumbent risks associated with the surgery,
indications for bone-lengthening procedures in the upper
extremities are confined to selected cases.104,117–128
Reported complications of upper limb lengthening include
pin tract infections, complications related to callus stability
and formation (callus deformities after fixator removal,
re-fractures, and malunions), diminished range of motion,
elbow flexion contractures, pin-related nerve injuries, tempo­
rary radial nerve paresis as a complication of humeral
lengthening, and sympathetic dystrophy.129–133 Considerable
morbidity results from nerve injury secondary to the transfix­
ing wires.132,133 In addition, in distraction lengthening of the
upper limb, the bone formation takes longer compared to the
lower limb because of the lack of weight-bearing. Therefore
the external fixator must be kept on for several months. The
length of treatment further decreases patient compliance and
can be a major issue in young children and adolescents.
Epiphyseal transfer of the
proximal fibular epiphysis
Access the Historical Perspective section online at

Indications
The main indications for epiphyseal reconstruction are loss of
an epiphysis following trauma, tumor resection, or infection
in children. Vascularized epiphyseal transfer is a procedure
that can simultaneously achieve the double goal of recon­
structing a lost joint and maintaining growth potential.
Because of its biological and morphological characteristics,
the proximal fibula is by far the best donor site for reconstruc­
tion of large bone defects of the upper extremity. In fact,
unlike other bone segments suggested for vascularized
epiphyseal reconstruction,158,159 the proximal end of the fibula
contains a true epiphysis with a growth plate that, if properly
revascularized, maintains its growth potential at the recipient
site. In addition, the fibula is a tubular bone with a long
expendable diaphysis that is perfectly suited for upper limb
reconstruction, allowing safe and stable bone fixation.
The two most common locations of bone sarcomas of the
upper extremity in the pediatric age group are the distal
Failure to prevent re-formation of the physeal bridge104,105 may
be due to the fact that fat may not provide adequate hemosta­
sis of the cavity and may migrate.97,106
Silicone (Silastic) has been experimentally used in both
human and animal studies with good results,102,107 but Silastic
has been withdrawn from the market.99 Methylmethacrylate,
commercially known as Cranioplastic, is a material used to
fill partial defects of the skull, is radiolucent, and is thermally
non-conductive. The advantage as interposition material97 is
that its solid structure may help to support an epiphysis if a
large metaphyseal defect has been created.106 It may, however,
damage the healthy physis because of heat produced by the
exothermic reaction during the solidifying phase.108 It may
also be problematic to remove if further reconstructive proce­
dures are required. Rarely, it can migrate from the physis into
the diaphysis, and cause a pathological fracture.109
Expanded polytetrafluoroethylene (EPTFE) membrane
(Gore-Tex dura substitute; WL Gore, Flagstaff, AZ, USA) is
generally used as an artificial dura substitute. The advantages
of EPTFE membrane are that it is inert, non-reactive, unaf­
fected by long-term exposure within the body,110 easy to
handle, and provides good hemostasis. The disadvantage is
that, being a soft material, it does not provide sufficient
mechanical support and it is therefore only recommended
when the resected area is at most 30% of the cross-section of
the physis.111
Bone wax is readily available and commonly used in
medical applications to control bleeding. It has been success­
fully used as interposition material with the advantages of
being inexpensive and permitting good hemostasis. It is not
associated with excessive complications.112 The disadvantage
is that it does not offer a good mechanical support to the
physis after bar resection.
Regardless of which interposition material is selected, the
purpose is to fill the cavity created in the physis with the
material so that bar formation is prevented. After bar resec­
tion, radiographic markers should be placed on each side of
the physis to assess growth resumption.
Results after bar resection are variable. Even with appropri­
ate patient selection and standardized operative technique,
failure may result. Graft dislocation out of the cavity is one of
the causes of failure.113 It is therefore recommended that the
interposition material be anchored so that it will not dislodge,
allowing bleeding into the cavity.97 It is important that, even
when growth resumes, premature closure of the physis is to
be expected.56,79,98,106,113
Physeal bar resection with interposition of certain materials
plays a role in the treatment of partial physeal arrest; however,
its results are relatively modest.
Corrective osteotomies, lengthening or shortening
Epiphysiodesis and resection of the bone bridge with the
insertion of interposition materials96,97 are the two main
methods to treat partial physeal arrest that also prevent
further progression of angular deformity. In addition, for mild
deformities of less than 20°, we can expect spontaneous
remodeling after bar resection,96,97,114 although this has not
been universally reported.77 When an angular deformity is
already evident, corrective osteotomy is indicated because
remodeling sufficient to correct the deformity cannot be
expected. Corrective osteotomy should be considered for

677.e1
Treatment/surgical technique
Historical perspective
The first reports of free non-vascularized epiphyseal trans­
plant date back to the end of the 19th century.134–136 All attempts
in the pre-microsurgical era had discouraging results in terms
of graft survival and growth. Early revascularization of the
growth plate was identified as the crucial condition in deter­
mining success.137 In the past 30 years, advancements in
microvascular surgery led to experimental research with
several studies proving the feasibility of vascularized epiphy­
seal transfer in animal models.138–146
In the 1980s, this procedure was successfully introduced
into clinical practice.147–149 These first encouraging results
convinced the scientific community to refine further the
technique and expand its indications. During the past 15
years, an increasing number of papers have successfully
reported on limited series of reconstruction of either the
proximal humerus or the distal radius and ulna with this
technique.150–157

678
SECTION IV
CHAPTER 30 • Growth considerations in pediatric upper extremity trauma and reconstruction
metaepiphysis of the radius and the proximal humerus, with
a slightly higher incidence for the latter. Due to morphological
and dimensional similarities, the proximal fibula is ideal for
distal radius reconstruction.154 In addition, under the influ­
ence of a new biomechanical environment, the plasticity of the
immature bone allows remarkable remodeling of the epiphy­
sis after its transfer into the new anatomic location. Since the
forearm is a two-bone segment and severe wrist deformity
may result from asymmetrical growth of radius and ulna, the
younger the patient, the greater is the indication for epiphy­
seal transfer in this location.
Case study 
Resection of the entire radius for sarcoma
Sometimes the extension of the sarcoma is so wide that
resection of the entire radius is required (Fig. 30.9). In such
case, the reconstruction with proximal fibular epiphysis is
particularly demanding. It is actually impossible to restore
the entire length of the radius and, in addition, the absence
of interosseous membrane would lead to unacceptable
instability and proximal migration of the graft.
In the two cases of our series, we performed a bone
fixation of the fibula to the midshaft of the ulna in a neutral
pronation/supination (Fig. 30.10A). This is a sort of
radioulnar synostosis, which provides a stable neoradius
with the only drawback of abolishing pronation and
supination.
The only conceptual concern that may be related to this
choice is the risk to have a divergent growth of the two
bones, because the ulna is straight and the new radius has
an angle of about 45° with the longitudinal axis of the
forearm.
However, in our experience, a longitudinal remodeling
occurred and over the years the distal portion of the new
radius migrated medially toward the ulna (see Fig. 30.10B),
thus improving the stability of the joint and its function
(Fig. 30.11).
Fig. 30.9  Sarcoma involving extensively the radius. Resection of the entire bone is
required.
Despite the anatomic mismatch between the fibular head and
the glenoid fossa and the difference in transverse diameter of
the shafts between the humerus and the fibula,151 autologous
proximal fibular transplant remains the best option for
Fig. 30.10  (A) Bone fixation of the fibula to the midshaft of the remaining ulna.
Radioulnar synostosis in neutral pronation/supination position is created, providing
a stable neoradius but eliminating pronation and supination. (B) Longitudinal
remodeling of the physis occurred over the years.
A
B