C o n g e n i t a l An o m a l i e s

of the Hand—Principles
of Management
Kevin J. Little, MD*, Roger Cornwall, MD

KEYWORDS
 Congenital hand  Syndactyly  Polydactyly  Thumb hypoplasia  Opponensplasty  Pollicization

KEY POINTS
 Although congenital hand anomalies are relatively rare, pediatric orthopedic surgeons and hand
surgeons will frequently see them during the course of clinical practice.
 The clinician should be aware of the associated malformations and conditions that may, in some
cases, be fatal if not recognized and treated appropriately.
 The goals of surgery are to improve hand function and cosmesis while limiting complications that
could impair function long term.
 The surgeon must balance functional, cosmetic, and cultural goals and align those goals with the
proper surgical techniques in order to maximize patient and parent satisfaction following surgical
intervention.
 This article discusses syndactyly, preaxial polydactyly and postaxial polydactyly, and the
hypoplastic thumb.

INTRODUCTION mothers older than 40 years of age are twice as

likely to have children with congenital hand differ-
Congenital anomalies of the upper extremity, ences as those only 10 years younger.5,6
although less common than congenital heart dis- Malformations of the hand and forearm were
ease, are noted in approximately 2 per 1000 live classified by Swanson7 in 1964 and adopted by
births.1,2 This incidence varies by country due to the International Federation of Societies for Sur-
higher incidence of certain malformations in pa- gery of the Hand (Table 1). Although this classifi-
tients of certain ethnic backgrounds, such as poly- cation has its use, it is generally hard to use in
dactyly in those of African descent or amniotic clinical instances, because patients may be classi-
bands in Japanese. Although many of these mal- fied into several categories at once, and it does not
formations lead to minor functional deficits, they guide treatment or prognosis. Oberg and col-
can pose a concern for the parents and lead to leagues8 proposed a modified classification based
psychological distress in children.3 In addition, on a more recent understanding of the embryology
the 1-year mortality of patients with hand malfor- of congenital hand malformations. Using this clas-
mations is 14% to 16% due to associated malfor- sification, malformations are divided into malfor-
mations, often involving the heart, kidneys, or mations, deformations, and dysplasias, and then
tracheoesophageal complex.4 Boys are affected further subdivided (Table 2).
more commonly than girls by a 3:2 ratio, and
orthopedic.theclinics.com

Division of Pediatric Orthopaedics, Department of Orthopaedic Surgery, Cincinnati Children’s Hospital Medical
Center, University of Cincinnati School of Medicine, 3333 Burnet Avenue, ML 2017, Cincinnati, OH 45229, USA
* Corresponding author.
E-mail address: kevin.little@cchmc.org

Orthop Clin N Am 47 (2016) 153–168

http://dx.doi.org/10.1016/j.ocl.2015.08.015
0030-5898/16/$ – see front matter Ó 2016 Elsevier Inc. All rights reserved.
154 Little & Cornwall

Table 1
Embryology
Swanson classification system for congenital Fetal limb development is initiated with the
hand differences appearance of a limb bud, consisting of undiffer-
entiated mesenchyme, at the lateral body wall 26
Type I Failure of formation
 Transverse deficiency
to 28 days after fertilization. Subsequently, the
 Longitudinal deficiency limb develops rapidly in a proximal-to-distal direc-
 Preaxial: Hypoplasia of thumb tion over the next 4 weeks. Complex interactions
and/or radius between signaling centers orchestrate this embry-
 Central: Typical and atypical onic differentiation.9–12 The first axis to form in the
cleft hand limb bud is the preaxial-postaxial (radial-ulnar)
 Postaxial: Hypoplasia of ulna axis, which is defined by the zone of polarization
and/or hypothenar hand activity. The next axis to form is the dorsal-volar
Type II Failure of differentiation axis defined by the apical ectodermal ridge
 Soft tissue (syndactyly, Poland (AER). Finally, the AER defines the limb proximal-
syndrome, camptodactyly) distal axis and controls interdigital cellular
 Skeletal (synostosis, carpal apoptosis. Signaling pathways critical to limb for-
coalition, complex syndactyly)
mation include sonic hedgehog (Shh), wingless-
Type III Duplication (polydactyly, mirror type, and fibroblast growth factors. Ectopic Shh
hand)
expression is a known source of polydactyly
Type IV Overgrowth (macrodactyly) because of its role in digit number and identity.9–17
Type V Undergrowth (radial hypoplasia,
symbrachydactyly, brachydactyly)
INDICATIONS/CONTRAINDICATIONS
Type VI Congenital constriction ring
syndrome (amniotic band For all congenital hand malformations, the primary
syndrome) surgical indication is to improve hand function and
Type VII Generalized skeletal abnormalities cosmesis. Contraindications include patients with
From Swanson AB. A classification for congenital malfor- surgical reconstruction that interferes with func-
mations of the hand. N J Bull Acad Med 1964;10:166. tion, such as for a centralization procedure in a pa-
tient with poor elbow function or with a primary

Table 2
Oberg classification system for congenital hand differences

I: Malformations A. Abnormal axis formation/differentiation: entire upper limb

i. Proximal-distal axis
ii. Radial-ulnar (anterior-posterior) axis
iii. Dorsal-ventral axis
iv. Unspecified axis
B. Abnormal axis formation/differentiation: Hand plate
i. Proximal-distal axis
ii. Radial-ulnar (anterior-posterior) axis
iii. Dorsal-ventral axis
II: Deformations Constriction ring syndromes, trigger digits
III: Dysplasia A. Hypertrophy
i. Whole limb
ii. Partial limb
B. Tumorous conditions
i. Vascular
ii. Neurologic
iii. Connective tissue
iv. Skeletal
IV: Syndromes A. Specified
B. Others
From Oberg KC, Feenstra JM, Manske PR, et al. Developmental biology and classification of congenital anomalies of the
hand and upper extremity. J Hand Surg Am 2010;35(12):2073; with permission.
 Congenital Anomalies of the Hand 155

postaxial pinch. Relative contraindications include digital fusion at the fingertips along with a variably
separating functionless or stiff digits, whereby patent web space proximally (Fig. 1).
reconstruction will only improve cosmesis and
not alter function, or adult patients who function Surgical Procedure
well with minor cosmetic deformity. In addition, The treatment of syndactyly requires good preop-
the cultural aspects of congenital hand malforma- erative planning and a thorough discussion of the
tions should be considered before surgery is per- risks and benefits with the family. Syndactyly
formed, because a malformation considered release should be performed at 3 to 6 months of
intolerable in one culture may be tolerable or age only in patients with mismatched finger size
even desirable in another.18 where growth is leading to deviation of the fingers.
Otherwise, it can be delayed until 1 to 2 years of
SYNDACTYLY age. Adjacent web spaces should not be released
in the same setting to avoid vascular compromise.
Syndactyly is a narrowed or fused web space be- A simple, longitudinal separation creates signifi-
tween adjacent fingers. Syndactyly occurs in cant scar tissue, which can impede growth and
approximately 2 to 3 patients per 10,000 live create deformity and joint contractures. Thus
births, affecting male patients more commonly zigzag flaps have been advocated to break up lon-
than female patients.19,20 Unilateral presentation gitudinal scar lines. The commissure should be
is equally as common as bilateral presentation.21 created with supple dorsal skin flaps, and grafts
Heritable forms of syndactyly are transmitted in should be avoided in this area to limit scarring
an autosomal-dominant pattern with variable and web creep. Furthermore, thumb-index syn-
penetrance and are often associated with syndac- dactyly separation is more complicated than finger
tyly between the second and third toes.19–21 Syn- syndactyly and should be treated with a 2 or 4 flap
dactyly is often classified by the length of the z-plasty for mild narrowing, or with a dorsal rota-
web, where complete syndactyly extends to the tional advancement flap for isolated complete syn-
tips of the fingers; incomplete syndactyly does dactyly24 (Fig. 2). It is essential to remember that
not include the fingertips. In addition, complex the decision-making process needs to include
syndactyly describes bony fusions, which are typi- input from families as well as the health care
cally identified with a single nail plate (synonychia), team (physician, therapist, and so on), and it often
while simple syndactyly describes only soft tissue can be dominated by the families’ perspective of
connections. Formal classification of syndactyly the condition.
was originally proposed by Temtamy and McKu-
sick,22 breaking it down into 5 distinct subtypes  A nonsterile brachial tourniquet inflated to no
based on phenotype and the underlying genetic more than 100 mm Hg greater than systolic
abnormalities. This classification has been pressure and the use of a hand table and
expanded many times and now includes 9 types loupe magnification is recommended.
and numerous subtypes.23
Syndactyly can be associated with other skel-
etal manifestations, including cleft hand, symbra-
chydactyly, and synpolydactyly, where extra
digits are syndactylized in the central digits. Syn-
dactyly is also associated with other genetic syn-
dromes, including acrocephalosyndactyly (Apert,
Pfeiffer, and Crouzon syndrome), characterized
by craniosynostosis, midface hypoplasia, and
complex syndactyly that can involve all 5 digits,
as well as acrocephalopolysyndactyly (Noack
and Carpenter syndrome), manifested by cranio-
synostosis, syndactyly, and preaxial polydactyly.
Fourth web space syndactyly, between the ring
and small fingers, is commonly associated with
oculodentodigital dysplasia, where patients can
have optic nerve hypoplasia, small teeth with
numerous caries, and a narrow midface. Amniotic
band syndrome or amniotic disruption sequence is Fig. 1. Patient with acrosyndactyly due to amniotic
often associated with multiple constriction bands, band syndrome. Note the distal syndactyly with
digital amputations, and acrosyndactyly, with apparent digital separation proximally.
156 Little & Cornwall

Fig. 2. (A) Patient with thumb-index syndactyly. (B) Same patient treated with a dorsal advancement flap.

 A dorsal rectangular or hourglass commissure deflate the tourniquet to ensure immediate re-
flap is marked out two-thirds of the length of perfusion to that digit.
the proximal phalanx, the width equal to the  Distal nerve bifurcation can be addressed with
center of each adjacent metacarpophalangeal intrafascicular dissection of the nerve
joint (MCP) joint (Fig. 3). branches until sufficiently proximal separation
 Zigzag flaps are marked out between the is achieved.
midsagittal axis of each digit dorsally and vol-  The commissure flap is inset first, using a fast-
arly such that the apex of each dorsal flap will absorbable 5-0 or 6-0 suture followed by
interdigitate with the axilla of each volar flap closure of triangular flaps without tension.
and vice versa (Fig. 4).  Areas that cannot be closed, typically along
 For complete syndactyly, double opposing the lateral commissure, should be covered
fingertip skin flaps are created for the lateral by defatted, full-thickness skin graft. The tour-
nail folds via the Buck-Gramcko technique niquet should be deflated before placing
(Fig. 5). dressings to ensure quick reperfusion to the
 The distal bone connection is transected, and digit.
the neurovascular bundle is dissected proxi-
mally to its bifurcation. If a distal bifurcation Postoperative Care
of the artery is present, place a microvascular
Patients are typically discharged the day of sur-
clamp on the artery branch to be ligated and
gery and return in 3 weeks for cast and dressing
removal. The wounds are inspected for signs of
infection, flap necrosis, or excessive scarring. Pa-
tients are encouraged to begin active range of mo-
tion (ROM) after cast removal and begin scar and
web space massage once wounds have healed.
The use of a silicone scar pad has been advocated
to improve scar suppleness and appearance.
Occupational therapy can be used to help with
scar appearance, to help with ROM exercises in
young patients, and to alleviate minor postopera-
tive scar contracture.

Complications and Management

The most common immediate complication is
vascular compromise to one of the digits. The
best management is prevention, including
Fig. 3. Patient with syndactyly showing a standard avoiding operating on both sides of the digit in
rectangular dorsal flap. the same setting, and careful choice of digital
 Congenital Anomalies of the Hand 157

Fig. 4. Patient with syndactyly demonstrating the (A) dorsal and (B) volar alternating triangular flaps.

artery ligation for distal bifurcation. In cases of a Outcomes

white finger after skin closure, warm the finger
There are more than 40 different descriptions of
with warm saline gauze and reassess after 5 to
flaps used to release digital syndactyly, and
10 minutes. If reperfusion is delayed, inspect
most have reported good outcomes in the most
the fingers for tight flaps and release tight su-
patients. Barabás and Pickford25 recently re-
tures as needed. It is always better to add skin
ported on 144 patients with an average of 5 years
grafts to an open wound than to have too
of follow-up following syndactyly release via a
much tension on a skin flap. Infection can be
traditional Flatt technique. They reported 7 cases
seen, especially with loss of skin flaps or grafts,
of graft failure and a 4.2% rate of web creep
and should be treated appropriately with antibi-
requiring revision surgery, although they noted
otics and skin grafts should be repeated as
that most of the cases with graft failure did not
needed. The most common late complication
result in significant web creep. Vekris and
is web creep due to scar contracture at the
colleagues26 reported on the follow-up of 131 pa-
commissure (Fig. 6). Occasionally this necessi-
tients with an average of 11.5 years of follow-up.
tates revision surgery. In addition, bony defor-
They noted worse results in complex syndactyly,
mity and nail abnormalities are more common
in adjacent digits of dissimilar length, in cases of
following complex syndactyly release and can
delayed presentation, and with the use of dorsal
be treated with digital osteotomy or lateral nail
and palmar triangular flaps rather than a large
fold reconstruction.

Fig. 5. Patient with syndactyly demonstrating the

Buck-Gramcko Flaps used to restore the lateral nail Fig. 6. Patient with syndactyly demonstrating web
folds. creep 2 years following syndactyly release.
158 Little & Cornwall

dorsal rectangular flap. In addition, Lumenta and as 1 in 143 black infants and 1 in 1334 white
colleagues27 reported on 26 patients with average infants.
11.5-year follow-up and noted 7.7% web creep Preaxial and postaxial polydactyly possess indi-
with an additional 42% showing signs of web vidual classification systems aimed to provide a
thickening without creep. An additional 7.7% unified language between surgeons. Preaxial poly-
demonstrated loss of the lateral nail fold without dactyly is described using the Wassel-Flatt classi-
nail abnormality, and 70% demonstrated hair fication, which defines the anatomic level of
growth from the groin full-thickness skin graft. duplication from distal to proximal.37 The
Short-term follow-up studies are available for Wassel-Flatt classification consists of 7 categories
several graftless syndactyly releases with similar and uses even numbers to represent complete du-
results in terms of web creep and revision sur- plications and odd numbers to represent incom-
gery,28–30 but long-term results greater than plete duplications. An easy way to remember this
5 years have not yet been published. classification is to start at the most distal normal
bone and count the number of separate bones
POLYDACTYLY (split bones count as one bone) distal to that. An
exception is the final category, type VII, which is
Polydactyly is categorized into preaxial (radial), the triphalangeal thumb9 (Fig. 7).
central, and postaxial (ulnar) types. In addition, Another difficulty with preaxial polydactyly
rare variants, including ulnar dimelia and mirror nomenclature is that neither of the duplicated
hand, may allow for up to 9-digit duplication. Pre- thumbs is normal. A better term for this condition
axial polydactyly incidence varies greatly and is would be a split thumb, implying that a normal
most commonly reported as 1 per 3000 live thumb was split into 2 or more parts, where the
births.31–34 Overall, preaxial polydactyly affects polydactylous digits are typically asymmetrically
male patients more commonly than female pa- split. This nomenclature has more implications
tients, and whites are more commonly affected for treatment, suggesting that treatment to restore
than blacks. Postaxial polydactyly has a racial pre- normal anatomy is not possible, because normal
dilection that is opposite from preaxial polydac- anatomy does not exist in either duplicated digit.
tyly, affecting blacks more commonly than whites Temtamy and McKusik33 classified postaxial
at a ratio of 10:1. Overall, the incidence in blacks polydactyl into a simple 2-part scheme of pheno-
is estimated to be as high as 1 in 300 live births, typic expression. Type A postaxial polydactyly
whereas whites are estimated to be affected every (Fig. 8) is defined as a fully developed extra digit,
1 in 3000 live births.31–34 With further subclassifi- whereas type B is a rudimentary digit. In whites,
cation of postaxial polydactyly, this racial disparity the occurrence of type A and type B are equiva-
has been isolated to type B postaxial polydactyly, lent; however, in blacks, type B is more
or the rudimentary digit. Postaxial type A has been common.31
reported to have similar racial characteristics.35 Despite several classification systems in the
However, Watson and Hennrikus36 reported an current literature, many surgeons prefer to use a
incidence of postaxial polydactyly type B as high focused descriptive approach to the duplicated

Fig. 7. (A) Clinical photograph and (B) posteroanterior radiograph of a patient with a triphalangeal thumb dupli-
cation (Wassel-Flatt VII).
 Congenital Anomalies of the Hand 159

level of duplication, musculoskeletal components

involved, stability, balanced motor function, devel-
opmental stage (pinch grasp in children), and
cosmetic outcomes all must be incorporated into
the surgical plan of care. Patients with duplications
at the joint level will require joint narrowing and
collateral ligament preservation, whereas duplica-
tions at the bony level often result in deformity that
needs to be addressed with osteotomy. The goal
is to produce a digit that is aligned along the me-
chanical axis with a central pull of the extensor
and flexor tendons.

Preoperative Planning
 Preaxial polydactyly patients should be as-
sessed to determine which thumb is better
suited for reconstruction. Typically, the ulnar
thumb is more functional and less angulated,
Fig. 8. Patient with a well-developed postaxial poly- resulting in the need for ablation of the radial
dactyly (type A).
thumb (Fig. 9).
 Postaxial type B digits with narrow stalks can
digits instead of using the aforementioned sys- be ligated at birth or treated with simple
tems. Surgeons describe the duplicated digit amputation at the bedside under local anes-
anatomically (location, size, degree of individual thesia with pressure hemostasis; however,
component development) and functionally this may result in neuroma formation and
(mobility, stability), which is often thought to be a persistent scarring.
stronger basis for surgical planning.  Alternatively, many surgeons recommend that
Preaxial polydactyly is most often due to spo- postaxial type B digits be treated with elliptical
radic mutations resulting in a unilateral presenta- excision, proximal amputation of the neuro-
tion of the malformation.33 However, an vascular bundle, and primary closure.
exception exists, because triphalangeal thumb
duplications are often inherited in an autosomal-
dominant pattern.38 Triphalangeal thumb duplica-
tion is associated with Blackfan-Diamond anemia,
Holt-Oram syndrome, Bloom syndrome, Carpen-
ter syndrome, and Ullrich-Feichtiger syndrome
and should prompt a referral to a geneticist.31,39
Postaxial polydactyly is also inherited in an
autosomal-dominant pattern with variable pene-
trance particularly in blacks. When postaxial poly-
dactyly is present in whites, it can be associated
with Trisomy 13, Ellis-van Creveld syndrome,
Laurence-Moon-Bardet-Biedle syndrome, and
Meckel syndrome, which can manifest with car-
diac and renal malformations. Therefore, these
children may require a referral to a geneticist for
an echocardiogram or renal ultrasound. In general,
polydactyly of the hand is more commonly associ-
ated with other musculoskeletal abnormalities (ie,
polydactyly of toes) than with other organ system
abnormalities.12,33–36

Surgical Procedure
Fig. 9. Patient demonstrating that the radial thumb
Surgical treatment of polydactyly is inherently duplication is less developed and less functional
complex. Careful consideration of the anatomic than the ulnar thumb.
160 Little & Cornwall

 In patients with preaxial and type A postaxial

polydactyly, preoperative radiographs should
be assessed before reconstruction to identify
bone and joint duplication level in order to
decide on the best treatment strategy.

Surgical Procedure: Preaxial Polydactyly

Reconstruction
 In the case of symmetric components, the ul-
nar thumb is typically preserved to maintain
the ulnar collateral ligament for pinch, unless
the Bilhaut-Cloquet procedure is performed
(see later discussion).
 A large, radial-sided flap with zigzag incisions
is drawn along the radial thumb that will be ab-
lated. This flap should be drawn larger than
expected, and can be trimmed later if not
needed.
 Care is taken to preserve the radial collateral Fig. 10. Patient with preaxial polydactyly demon-
ligament attachment to a small piece of artic- strating (via suture) an anomalous connection be-
ular cartilage at the base of the proximal pha- tween the extensor and flexor pollicis longus tendons.
lanx. This piece of articular cartilage is sutured
or pinned to the proximal phalanx base of ul-
divided and combined with the distal, ulnar
nar thumb to reconstruct the deficient collat-
aspect of the radial thumb.
eral ligament.
 In addition, the radial collateral ligament of the
 Further extra-articular malalignment requires
interphalangeal (IP) joint is kept with the radial
corrective osteotomy, with either a closing- or
thumb distal phalanx piece to provide thumb
an opening-wedge osteotomy parallel to the
stability.
joint surface, and stabilization with a K-wire.
 In some cases, an anomalous connection be-
tween the flexor and extensor tendons is pre- Surgical Procedure: Postaxial Polydactyly
sent, known as a pollex abductus, which must Reconstruction Type A
be released before centralizing the terminal
 Well-developed, postaxial, supranumerary
flexor and extensor tendons (Fig. 10).
digits require excision of the hypoplastic digit
and reconstruction of a single, stable,
Surgical Procedure: Bilhaut-Cloquet Procedure
 The Bilhaut-Cloquet procedure, which was
designed to decrease thumb size mismatch,
can be performed for Wassel types I, II, and
III, when there is minimal size mismatch be-
tween the 2 thumbs (Fig. 11).
 A central wedge of tissue including pulp tissue,
nail bed, and distal phalanx up to the articular
surface of the distal phalanx is excised.
 The round convex surface of the nail bed
should be reapproximated via rotation of the
separated distal phalanges.
 The nail bed is reapproximated with 6-
0 absorbable suture, and the skin is closed
with 5-0 absorbable suture.
 Alternatively, Baek and colleagues40,41
described an extra-articular and extraphyseal
modification to the Bilhaut-Cloquet procedure.
 In this modification, the articular surface and
physis of the ulnar thumb is kept intact, while Fig. 11. Patient with Wassel-Flatt type I polydactyly
the distal, radial aspect of the distal phalanx is with near symmetric bifurcation of the distal phalanx.
 Congenital Anomalies of the Hand 161

functional digit, abiding by similar principles results from residual deformity or imbalanced
as in preaxial digit reconstruction. growth of the thumb. Prevention of this deformity
 A large, ulnar-sided flap with zigzag incisions is difficult in some cases with significant preoper-
is drawn along the supranumerary ulnar digit; ative deformity, but is best ameliorated via strict
this should be drawn larger than expected and adherence to the above-mentioned reconstruction
can be trimmed later. principles including the creating of parallel joint
 The ulnar collateral ligament to the excised surfaces, centralizing the pull of all tendons, and
digit is spared, and the articular surface of limiting longitudinal scar formation. The Bilhaut-
the metacarpal may need to be trimmed Cloquet procedure is complicated by joint stiff-
similar to treatment of a duplicated thumb ness, growth arrest, asymmetric growth, and
(see earlier discussion). longitudinal nail-bed deformities.47 Using the
 In cases of coronal plane deformity, corrective Baek modification, joint stiffness and growth arrest
osteotomy should be performed via opening- can be limited by staying extraphyseal and extra-
or closing-wedge osteotomy and stabilized articular with the osteotomy, although nail-bed ab-
with a K-wire. normalities are still possible, but were not noted in
their series.40,41
Complications and Management
Postoperative Care
Suture ligation for type B polydactyly can be
associated with complications, such as bleeding, In patients without corrective osteotomy or joint
scarring, skin cracking, residual growth (Fig. 12), realignment, dressings are maintained for 10 to
neuroma, infection, and incomplete excision.35,42 14 days and then removed to allow for ROM exer-
Rayan and Frey43 sutured ligated digits on 105 in- cises. Patients who undergo ligament reconstruc-
fants and found that the most significant compli- tion or corrective osteotomy with temporary pin
cation was unacceptable cosmetic result/tender fixation will benefit from cast or splint treatment
digit occurring in 16% of patients, whereas to allow for full healing. After 4 weeks, if bony
bleeding was reported to occur in 1% and infec- union is achieved, ROM exercises are begun un-
tion in 6% of patients. Several authors have re- der the supervision of an occupational therapist.
ported small series of patients treated with Patients should be monitored yearly to biannually
excision of the stalk or revision surgery following until skeletal maturity to evaluate for worsening
previous suture ligation with overall good deformity.
outcomes.44–46
The most common complication following type Outcomes
A reconstruction is a zigzag deformity, which Watson and Hennrikus35 performed suture ligation
on 21 infants with type B postaxial polydactyly.
They reported no complications in regard to infec-
tion or bleeding; however, one child required oper-
ative intervention to remove a digit that failed to
auto-amputate. Forty-three percent had a notice-
able bump from 1 to 6 mm in size. Katz and Lin-
der44 performed excision of 15 type B digits
using topical anesthetic, scalpel excision at base
of the stalk, and subsequent pressure to control
bleeding. Good cosmetic appearance was re-
ported in all patients with no complications noted
at follow-up. Leber and Gosain46 performed 12 pri-
mary elliptical excisions of supernumerary digits
without residual deformity and re-excision of 5
traumatic amputation neuromas resultant from
treatment in the nursery. Mullick and Borschel48
presented 13 hands that underwent previous
string ligation, producing either an unsightly
incomplete amputation or a tender neuroma, or
both. In the series, patients were all successfully
Fig. 12. Patient with recurrent growth of postaxial treated with completion amputation of the residual
polydactyly type B after suture ligation in the stump combined with proximal ligation of the su-
newborn nursery. pernumerary digital nerves.
162 Little & Cornwall

In patients with type A polydactyly, the decision deformity by Bayne and Klug,54 and this classifica-
to perform surgery is the first priority. If ablation tion has been modified by James and Bednar55 to
and reconstruction are indicated or requested, include thumb hypoplasia and abnormalities of the
care must be taken to maintain joint stability and radial carpus (Table 3). Blauth56 classified thumb
motion of the remaining small finger. Complica- hypoplasia by the severity of clinical deformity
tions are relatively low when treated surgically, from type I, mild decrease in thumb size with intact
Rayan and Frey43 reported 1 complication (infec- intrinsic and extrinsic function, to type V, complete
tion) in 27 patients treated surgically for type A thumb absence. This classification was modified
postaxial polydactyly. by Manske57 to include a differentiation in type
Lee and colleagues49 reviewed 139 cases of III, which included the stability of the trapeziometa-
Wassel type IV duplications and found 44 cases carpal joint (type IIIA) or lack thereof (type IIIB)
of postsurgical Z deformity, defined as joint angu- (Table 4). In patients with trapeziometacarpal joint
lation greater than 20 at MCP and IP. Yen and col- instability (type IIIB or more severe), reconstruction
leagues45 reported joint instability in 25 of 36 and of a stable, useful thumb is exceedingly difficult,
joint deformity in 10 of 36 surgically treated and most surgeons would consider these patients
thumbs. Stutz and colleagues50 evaluated 41 pa- appropriate for thumb ablation and index finger
tients with 43 surgically reconstructed thumbs at pollicization.
greater than 10 years (mean 17 years) and noted Patients with radial and thumb deficiency
that 10 revision surgeries were required, most commonly have other musculoskeletal and sys-
commonly for IP joint deformity and pain. The temic abnormalities. Scoliosis is the most com-
rate of revision surgery increased over time, indi- mon musculoskeletal abnormality noted with
cating that long-term follow-up is recommended radial deficiency. The most common systemic as-
for patients with these deformities. sociations are Holt-Oram syndrome, thrombocy-
There are few isolated reports of outcomes for topenia absent radius (TAR) syndrome, Fanconi
the Bilhaut-Cloquet procedure, often intermixed anemia, and VACTERL (Vertebral defects, Anal
with other techniques for thumb polydactyly atresia, Cardiac malformations, Tracheoesopha-
reconstruction. Most demonstrate good results, geal fistula, Esophageal atresia, Renal anomalies,
and outcomes scores are equivalent among and Limb anomalies) association.53,58 The treating
most groups.45,47,51 In a small sample size of 7 pa- surgeon may be the first person to recognize these
tients, Baek and colleagues40 demonstrated satis- associations and should initiate further diagnostic
factory functional and cosmetic results with steps. A referral to a pediatric geneticist may be
preserved preoperative ROM in type III, improved of benefit, but if one is not available, the hand sur-
ROM in type II thumbs, and no cases of nail defor- geon should order spine radiographs to screen for
mities or growth arrest. scoliosis, renal ultrasound to evaluate for kidney
malformations, echocardiogram to evaluate for
THUMB HYPOPLASIA cardiac malformations such as atrial or ventral
septal defect commonly seen in Holt-Oram syn-
Hypoplasia of the thumb occurs within a spectrum drome, and a complete blood count and chro-
of hypoplasia along the radial side of the entire up- mosomal breakage test to evaluate for
per extremity. This radial longitudinal deficiency thrombocytopenia and Fanconi anemia, respec-
can be mild, involving only mild size discrepancy tively. Patients with TAR syndrome have complete
to the thumb, or severe and include global shoul-
der hypoplasia and instability along with elbow
deficiency, and complete thumb and radius
absence. The estimated incidence of radial longi- Table 3
Modified Bayne and Klug classification of
tudinal deficiency is approximately 1:30,000 live
radial longitudinal deficiency
births52; however, this frequency may be underes-
timated because mild deformity may be missed Type N Isolated thumb hypoplasia/absence
due to excellent function and cosmesis. Thumb
Type 0 Type N with anomaly of radial carpus
hypoplasia can occur in isolation but is almost al-
Type 1 Shortened distal radius
ways noted with underlying radial longitudinal defi-
ciency. Male patients and female patients are Type 2 Hypoplastic radius
equally affected, and the incidence of bilaterality Type 3 Partial absence of radius
is approximately 50%, although asymmetrical pre- Type 4 Complete absence of radius
sentation is commonly seen.53 From James MA, McCarroll HR Jr, Manske PR. The spec-
Radial longitudinal deficiency was initially classi- trum of radial longitudinal deficiency: a modified classifi-
fied radiographically by the severity of radial cation. J Hand Surg Am 1999;24(6):1147.
 Congenital Anomalies of the Hand 163

Table 4
Modified Blauth classification of thumb hypoplasia

Type I Slight decrease in thumb size

Type II Narrowed first web space, hypoplastic thenar musculature, unstable MCP joint
Type III Features of type II hypoplasia, including
IIIA: Extrinsic muscle abnormalities, metacarpal hypoplasia
IIIB: Extrinsic muscle abnormalities, partial aplasia of metacarpal, unstable
carpometacarpal joint
Type IV Pouce flottant with only soft tissue connection to hand (floating thumb)
Type V Complete absence of thumb
From Manske PR, McCarroll HR Jr, James M. Type III-A hypoplastic thumb. J Hand Surg Am 1995;20(2):246.

radius aplasia, but always have thumbs present patients with Blauth type IIIB, IV, and V thumbs,
with varying degrees of hypoplasia. pollicization with ablation of the thumb remnant
is the procedure of choice (Fig. 13).
Surgical Procedure In patients with thumb hypoplasia and underly-
ing severe radial longitudinal deficiency, the
Patients with thumb hypoplasia should be evalu- treatment is somewhat controversial. Most of
ated for concomitant finger abnormalities and for these patients have significant thumb hypoplasia
radial longitudinal deficiency. The treatment for and stiffness of the radial digits, with relatively
these patients is based on the severity of the hypo- good function to the small finger. These patients
plasia. In patients with mild hypoplasia, including may have functional postaxial pinch patterns
type I, and mild variations of type II with some op- present and may lose function with correction
position function, no surgical treatment is indi- of the wrist deformity and subsequent index pol-
cated. Patients with type II and IIIA hypoplasia licization, especially when the index finger is hy-
often benefit from opponensplasty, stabilization poplastic or syndactylized to the middle finger. In
of the radial and ulnar collateral ligaments of the addition, in patients with severe radial deficiency
thumb MCP, and web space deepening. This and limited elbow motion, correction of wrist
treatment is best done with a single tendon trans- radial deviation will limit useful hand-to-mouth
fer from the ring or middle finger flexor digitorum function.
superficialis (FDS) tendon; however, this will not
restore thenar bulk. Opponensplasty using the
Preoperative Planning
abductor digiti minimi (ADM) via a Huber transfer
helps to restore the thenar contour and function,  Careful clinical and radiographic examination
but does not have sufficient length to restore the should be performed in order to establish
collateral ligaments of the thumb MCP joint. In the exact degree of thumb hypoplasia.

Fig. 13. Patient with (A) Blauth type IIIB thumb hypoplasia and (B) following thumb ablation and index finger
pollicization.
164 Little & Cornwall

 In general, a patient will bypass a thumb with In patients with unstable MCP joints following
an unstable trapeziometacarpal joint and pre- Huber transfer, collateral ligament reconstruc-
fer a scissor pinch between the index and tion is performed with free tendon grafts or the
middle fingers. joint can be stabilized with chondrodesis be-
tween the metacarpal head and epiphysis of
Surgical Procedure: Opponensplasty, the proximal phalanx.
Ligament Reconstruction, Web Space
Deepening Surgical Procedure: Pollicization
 A transverse incision is made over the prox-  Drawing appropriate skin incisions is crucial
imal interphalangeal joint crease of the ring to successfully performing a pollicization.
finger along with a longitudinal volar inci- There are 2 main variations described.58–60
sion over the flexor carpi ulnaris (FCU)  The volar neurovascular bundle is isolated
tendon. and, along with the periadventitial fat,
 Both slips of the FDS tendon are divided at the dissected free from the underlying flexor
chiasm, passed through a loop created from tendon sheath to allow for mobilization to the
the FCU tendon and then passed through a thumb position without kinking the vessels.
subcutaneous tunnel to a longitudinal incision  The dorsal skin incision is made making sure
along the radial border of the thumb MCP joint to preserve as many dorsal veins as possible.
(Fig. 14). These veins are dissected proximally and
 The thumb web space is deepened with a distally to allow for easy transposition to the
4-flap Z-plasty, which is left open until collat- thumb position without kinking.
eral ligament reconstruction is performed  The metacarpal is dissected extraperiosteally
(Fig. 15). from the origin of interosseous muscles and
 In cases of MCP joint instability, a drill hole is surrounding tissues and removed from the
made through the metacarpal head at the metaphyseal flare to the epiphysis.
level of the collateral ligament origin. One  The shortened index metacarpal is rotated
slip of the FDS is passed through the tunnel 100 to 120 and placed into 45 of palmar
to the 4-flap Z-plasty and then secured to abduction and 15 of radial abduction to re-
the ulnar volar aspect of the proximal phalanx create the normal thumb position.
base, whereas the other slip is used to recon-  The skin incisions are inset with absorbable
struct the radial collateral ligament (see sutures. The volar skin flap is advanced into
Fig. 14). the new thumb/long finger web space, taking
 Alternatively, in the Huber transfer, the ADM care not to place any suture line through the
muscle can be harvested and transferred on web space to minimize later scar contracture.
its neurovascular pedicle to the thumb APB  The patient is observed for 23 hours postoper-
tendon insertion via a wide subcutaneous tun- atively to ensure that vascularity remains
nel. The ADM muscle can be taken with a skin intact and pain is well controlled before
paddle to provide additional skin contouring. discharge home.

Fig. 14. (A) Ring FDS opponensplasty demonstrating line of pull after passing the FDS tendon through a pulley
created by a slip of the FCU tendon. (B) Reconstruction of the ulnar collateral ligament of the MCP joint with a
slip of the FDS tendon passed through the metacarpal head into the web space z-plasty incision. In this case, the
proximal phalanx physis was closed, allowing drill holes in the proximal phalanx base; however, in younger chil-
dren, the FDS tendon can be sutured to the periosteum at the proximal phalanx base.
 Congenital Anomalies of the Hand 165

Fig. 15. Patient with thumb-index web space contracture demonstrating a 4-flap Z Plasty (A) before separation
and (B) after closure. Note how the letters corresponding to each flap are reorganized from A-B-C-D to C-A-D-B.

Postoperative Care vascularity does not return in 5 minutes, the ar-

teries should be explored for damage and freed
Postoperatively, the patient is casted for 4 weeks,
of any soft tissue tether. Venous congestion is
and the stabilizing pin is removed as needed.
more frequently encountered, which can be
Occupational therapy is then begun to retrain the
ameliorated by loosening dressings or removing
patient for use of the reconstructed thumb. This
tight sutures that may be compressing the dorsal
therapy often lasts for 2 to 3 months postopera-
veins.
tively and can be discontinued when the patient
Long-term complications, such as malrotation,
has maximized function and the family is suffi-
excessive length, or web contracture of the new
ciently versed in a home exercise program to
thumb, are due to technical failures and are best
continue at home. The patients are followed
prevented by meticulous attention to the steps
monthly for the first 3 to 4 months and then yearly
involved in surgical correction. Lack of opposition
to ensure that function has been maintained and to
following pollicization is seen in approximately
evaluate for long-term complications.
10% to 20% of patients and is likely due to an un-
derlying deficiency in intrinsic muscles; this can be
Complications and Management successfully treated by subsequent opponens-
plasty as described previously.61
Complications are infrequent during reconstruc-
tion of a hypoplastic thumb as long as the correct
Outcomes
surgical indications are met and the surgical tech-
niques are carefully followed. In general, if a child In most cases, improved outcomes are achieved
ignores a thumb that does not have a stable following reconstruction in patients with hypoplas-
base, they will likely continue to ignore that thumb tic thumbs as long as the surgical plan addresses
if it is reconstructed. Conversely, a patient who the deficient components for each patient. Abdel-
tries to use a hypoplastic thumb but struggles Ghani and Amro62 reported on 9 patients who un-
due to opposition weakness and MCP joint insta- derwent FDS opponensplasty and web space
bility will likely benefit greatly from opponensplasty deepening. All patients had improved function,
and ligament reconstruction. Therefore, preopera- and 8 of 9 achieved thumb opposition to the small
tive assessment and patient selection are critical finger, whereas only 71% achieved full stabiliza-
to a functional thumb reconstruction. Occupa- tion of the MCP joint. Graham and Louis63 re-
tional therapy evaluation preoperatively and post- ported on the functional results of 14 patients
operatively is very helpful in determining the with hypoplastic thumbs with all achieving func-
surgical goals and assisting the patient in func- tional improvement and only 2 of 14 noting mild
tional recovery. instability of the thumb MP joint. Upton and Taghi-
The most significant complication following pol- nia64 reported on 14 patients who underwent ADM
licization is poor vascular inflow or outflow, which opponensplasty for type II and IIIA thumb hypopla-
can result in loss of the digit if not treated immedi- sia and noted improved function and no loss of
ately. If the new thumb does not turn pink immedi- function during growth.
ately, vasospasm may be the cause, which should Manske and colleagues65 evaluated index
resolve with observation and/or warming. If finger pollicization at an average of 8 years
166 Little & Cornwall

postoperatively and demonstrated that the new malformations in Finland: a population-based study.
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Miric and colleagues66 reported on 8 patients at 7. Swanson AB. A classification for congenital malforma-
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14. Bouldin CM, Gritli-Linde A, Ahn S, et al. Shh pathway
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