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13 Surgical Management oftheBlauth 1 to3A Thumb Hypoplasia
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c
d
e
Fig. 13.5 Huber transfer; abductor digiti minimi (ADM).
(a) division of the ADM from distal to proximal. One can
lengthen the transplant by harvesting a periosteal slip of
the ulnar base of the proximal phalanx. (b) the transfer is
by Royle [24], it is a pure tendon transfer, which
gives a longer transplant and is frequently used to
restore opposition and stabilize the ulnar collateral ligament (UCL) [25]. The two lateral strips
of the FCS can be disinserted distally at the intermediate phalanx but now we prefer avoiding an
incision on the nger. We perform a transverse
incision on the distal fold of the palm, proximal
dragged subcutaneously to the radial side of the MP joint
of the thumb. (c) Same girl right hand; result after
10years. (d) Kapandji’s score is 8. (e) True active antepulsion up to 40°
to the A1 pulley, we isolate the FCS tendon and
by pulling strongly with a silicone loop, with the
fourth nger in exion, it is possible to bring the
chiasma into the palm and cut it. It is then easy to
harvest the tendon. Another transverse proximal
incision is realized to identify the transplant at
the exit of the carpal tunnel (Figs.13.6a–i) and a
buttonhole is made in the middle of the retinacu-
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S. Guéro
Fig. 13.6 Flexus adductus thumb on type 3A.Transfer of
the sublimis of the fourth nger (Royle transfer). (a)
Dorsal view. (b) palmar view showing the lateral passive
hyper-laxity. (c) opening of the rst web with resection of
a supernumerary transverse muscle. (d) Harvesting of the
two slips of the FCS of the fourth nger. We no longer
open the fourth nger since we are able to harvest the FCS
by pulling strongly the tendon in the palm and cutting the
chiasma. (e) Making a pulley by opening a buttonhole in
the exor tendon retinaculum. (f) the transfer is dragged
to the radial edge of the MP joint. One slip is sutured very
proximal to the base of the proximal phalanx, while the
other is dragged through a hole in the metacarpal head,
from radial to ulnar, to reconstruct the UCL. (g–i) Same
child; 2years follow up with a true active antepulsion and
opposition
13 Surgical Management oftheBlauth 1 to3A Thumb Hypoplasia
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g
h
i
Fig. 13.6 (continued)
lum which will serve as reection pulley. The
tendon is then dragged to the lateral face of the
MP joint and of the proximal phalanx of the
thumb to be reinserted on the supposed distal
insertion of the APB.
To stabilize the MP joint, many authors perform a transosseous tunnel in the head of the rst
metacarpal bone and use one of the strips to
reconstruct the ulnar collateral ligament.
The removal of FCS tendon does not compromise the exion of the fourth nger, nor create
any instability of the PIP joint.
Extension Transfer
This transfer is largely underestimated in the literature. In case of EPB aplasia, if children have
the extension through the EPL, it is often inadequate to cause a correct opening of the rst web.
In this case, we can use the extensor indicis proprius (EIP) after checking preoperatively that the
child has an independent extension of the index.
As reported in the Japanese publications, the
interest of this transfer is to improve the thumb
extension and to stabilize the ulnar side of the
MP joint.
Harvesting the EIP is easy. We start distally by
separating the distal insertion of the EIP on the
MP joint of the index. The tendon is then dragged
by a small incision on the dorsal surface of the
wrist or taken directly when opening of the rst
web. It is then slipped on the dorsal and ulnar side
of the rst metacarpal and then inserted on the
base of the proximal phalanx by making numerous mooring points on the ulnar part of the capsule of the MP joint becoming both an active
transfer of extension and of joint stabilization.
Stabilization oftheMP Joint
It should be checked pre-operatively if it is a simple ulnar or multi-directional instability. In this
latter case, it is illusory to attempt to reconstruct
the ligaments. It is better to perform a chondrode-
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S. Guéro
sis between the head of M1 and the base of P1,
but this ‘arthrodesis at pediatric age’ must be carried out with care so as not to damage the growth
cartilage at the base of P1.
If it is stability only on the ulnar side, the two
techniques mentioned above will be carried out:
stabilization transfer of the EIP, transfer of the
FCS and for some Huber transfer (ADM).
Cosmetic improvement: thenar eminence augmentation. As mentioned before, the Huber transfer can correct the thenar muscle amyotrophy but
very partially, to be honest. In order to restore the
thenar relief, I have performed some fat grafts
according to Coleman with satisfactory results.
This should be proposed only at the end of
growth. Upton [26] has published an adipofascial
island ap in pollicization but it could be performed in type 2 or 3A as well.
13.11.2.2 Indications
Whatever the type of hypoplasia, treatment
should be early, started in the rst year and ideally completed before 2years of age [27].
Type 1
For type 1, as we have seen, the function is normal. There is therefore no surgical indication.
Type 2
For type 2, the treatment systematically comprises three components:
– Opening of the rst commissure with skin
aps and musculo-aponeurotic release.
– Thumb opposition transfer and possibly palli-
ative extension.
– Stabilization of the MCP.
The reconstruction of the rst web space is not
the subject of any controversy. The choice of the
type of opposition and the stabilization of the
MCP depend on the habits of the authors. Paul
Smith [28] proposed an algorithm of therapeutic
decisions according to the type of instability of
the MP joint. In case of uni-axial instability, he
proposes the transfer of the FCS of the fourth nger with a transosseous passage of one of the
strips to reconstruct the ulnar collateral ligament.
It is also our method of choice. If the instability is
multi-axial, he recommends a chondrodesis of
the MCP and a Huber-type opposition transfer.
In our experience, the use of chondrodesis is
rarely necessary in type 2, because we have the
habit, in case of multi-axial instability with decit extension of the MCP to practice at rst the
transfer of the ‘EIP to restore the extension [29]
and perform a transfer of the FCS of the fourth
nger while the FPL of the thumb actively stabilize the MCP.
Type 3
We only mention the type 3 A case since the 3 B
and 3 C are treated by index pollicization for the
vast majority of authors.
For type 3A, multi-axis instability of the MP
joint is the rule and chondrodesis is most often
required. The intrinsic tendons are all absent and
the extrinsic tendons are very slender. It is therefore imperative to restore the opposition and the
extension of the thumb as previously described.
The opening of the rst commissure most
often requires a ‘generous’ ap-type Buck
Gramcko.
The closure of the angle between the rst and
second metacarpal bone (M1 and M2) is so
important that we could be tempted to perform
M1-M2 arthrodesis by bicortical trapezoidal
bone graft. However, this technique can only be
performed at adolescence because it would block
the physeal plate and thus the growth of the base
of M1.
Key Points
– Do not confuse a exus adductus thumb
whose orthopaedic treatment with manipula-
tions and splinting is always favourable with a
true thumb hypoplasia that requires release of
the rst web, stabilization of the MP joint and
restoration of the opposition and extension of
the thumb.
– Take time to check if instability of the MP
joint is only passive or both passive and active.
If the child can stabilize actively his joint with
13 Surgical Management oftheBlauth 1 to3A Thumb Hypoplasia
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165
a proper contraction of his intrinsic or extrinsic muscles, there is no need for a ligament
reconstruction.
– Regardless of the type of hypoplasia, even in
type 1, keep in mind that the malformation of
the thumb is rarely isolated, requiring a
complete examination of the child, of his ipsiand contra-lateral upper limb, and of the
parents.
– The choice of the tendon transfer is made
according to the stability of the MCP.
– Any kind of procedure requires a postopera-
tive immobilization by splinting for several
months to avoid a secondary retraction of the
rst web.
– It should not be forgotten that the functional
result depends on the severity of the hypoplasia and especially if associated with a RCH.
References
1. Light TR, Gaffey JL.Reconstruction of the hypoplastic thumb. J Hand Surg Am. 2010;35(3):474–9.
2. Blauth W. The hypoplastic thumb. Arch Orthop
Unfallchir. 1967;62(3):225–46.
3. Dautel G. Thumb hypoplasia. Chir Main.
2006;25(1):1–15.
4. Buck-Gramcko D. State of the art: congenital malformation of the hand and forearm. Part III.Eur Med
Hand Surg. 1993;3:7–31.
5. Smith P.Lister’s the hand. Diagnosis and indications.
London: Churchill Livingstone; 2002. p.505–12.
6. Soldado F, Zlotolow DA, Kozin SH.Thumb hypoplasia. J Hand Surg Am. 2013;38(7):1435–44.
7. Tonkin MA.On the classication of congenital thumb
hypoplasia. J Hand Surg Eur Vol. 2014;39(9):948–55.
8. Lister G.Pollex abductus in hypoplasia and duplication
of the thumb. J Hand Surg Am. 1991;16(4):626–33.
9. Dellon AL, Rayan G.Congenital absence of the thenar muscles. Report of two cases. J Bone Joint Surg
Am. 1981;63(6):1014–5.
10. Rayan GM. Congenital thumb hypoplasia. J Okla
State Med Assoc. 1994;87(12):546–50.
11. Manske PR, McCarroll HR Jr, James M. Type
III-A hypoplastic thumb. J Hand Surg [Am].
1995;20(2):246–53.
12. Hovius SE, van Nieuwenhoven C.Commentary on
Tonkin. On the classication of congenital thumb
hypoplasia. J Hand Surg Eur Vol. 2014;39(9):956–7.
13. Smith P. Re: Tonkin, M.A. On the classication of
congenital thumb hypoplasia. J Hand Surg Eur. 2014,
39: 948–55. J Hand Surg Eur Vol. 2015;40(4):427.
14. Tonkin MA. Re: Tonkin, M. A. On the classication of congenital thumb hypoplasia. J Hand Surg
Eur. 2014, 39: 948-55. Reply. J Hand Surg Eur Vol.
2015;40(4):427–8.
15. Guero S. Conduite à tenir devant Une hypoplasie
du pouce de type 1 à 3A [surgical management of
the Blauth 1 to 3A thumb hypoplasia]. Chir Main.
2008;27(Suppl 1):S62–70.
16. James MA, McCarroll HR Jr, Manske
PR. Characteristics of patients with hypoplastic
thumbs. J Hand Surg [Am]. 1996;21(1):104–13.
17. Hall RF Jr, Keuhn D Jr, Prieto J. Congenital hypoplasia of the thumb ray with absent carpal navicular
and hypertrophic styloid process of the radius: a case
report. J Hand Surg Am. 1986;11(1):32–5.
18. James MA, etal. The association of radial deciency
with thumb hypoplasia. J Bone Joint Surg Am.
2004;86(10):2196–205.
19. Glicenstein J, Bonnefous G.La plastie en trident. Ann
Chir Plast. 1975;20:257–60.
20. Foucher G, Gazarian A, Pajardi G. Reconstructive
surgery of Blauth type III hypoplasia of the thumb.
Chir Main. 1999;18(3):191–6.
21. Graham TJ, Louis DS. A comprehensive approach
to surgical management of the type IIIA hypoplastic
thumb. J Hand Surg Am. 1998;23(1):3–13.
22. Gupta A, etal. Treatment of the severely injured upper
extremity. Instr Course Lect. 2000;49:377–96.
23. Takayama S, et al. Modied abductor digiti minimi
opponensplasty in congenital hypoplastic thumb with
laxity of metacarpophalangeal joint. Tech Hand Up
Extrem Surg. 2002;6(4):166–70.
24. Royle ND. The functions of human voluntary muscles. Sydney: Angus & Robertson limited; 1938.
p.42.
25. Kozin SH, Ezaki M. Flexor digitorum supercialis
opponensplasty with ulnar collateral ligament reconstruction for thumb deciency. Tech Hand Up Extrem
Surg. 2010;14(1):46–50.
26. Upton J, Sharma S, Taghinia AH.Vascularized adipofascial Island ap for thenar augmentation in pollicization. Plast Reconstr Surg. 2008;122(4):1089–94.
27. Lister G. Reconstruction of the hypoplastic thumb.
Clin Orthop Relat Res. 1985;195:52–65.
28. Smith P, etal. Blauth II thumb hypoplasia: a management algorithm for the unstable metacarpophalangeal
joint. J Hand Surg Eur Vol. 2012;37(8):745–50.
29. Vacher C, etal. Congenital thumb hypoplasia. Clinical
study of twenty patients. Ann Chir Main Memb Super.
1997;16(4):316–25.
Thumb Hypoplasia: Genesia,
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Pollicization
GiorgioPajardi, ElisaRosanda, andChiaraParolo
14
Abstract
Congenital malformation of the thumb is one
of the most important problems in congenital
disease. The hypoplastic thumb is characterized by a variable degree of bony and soft tissue inadequacy. It may occur alone or as part
of a multiple congenital anomaly syndrome.
Depending on degree of hypoplasia, the nonsurgical or surgical treatment differs. Every
treatment, usually, starts very early in the
childhood to improve brain plasticity.
In case of surgical management, the primary goal is to improve or restore pincer grip.
In general when the hypoplastic thumb lacks
basilar joint stability or is absent, the hand is
best treated by politicization of the index nger. When hypoplasia is less severe, surgical
strategy includes rst web deepening, MP
ligamentoplasty, opponensplasty, and tendon
transfer.
G. Pajardi
Department of Hand Surgery and Rehabilitation,
S. Giuseppe Hospital IRCCS MultiMedica,
Milan University, Milan, Italy
e-mail: gpajardi@centrostudimano.it
E. Rosanda (*) · C. Parolo
Milan, Italy
e-mail: elisa.rosanda@multimedica.it;
chiara.parolo@multimedica.it
Keywords
Congenital hand deformities · Thumb
hypoplasia · Pollicization · First web deepening · Opponensplasty · Tendon transfer
Congenital malformation of the thumb is one of
the most important problems in congenital disease. The thumb has unique characteristics
despite of long ngers. The CMC joint, the intrinsic and extrinsic muscles, the vascularization,
and innervation make it the most important nger
in the hand with the capability of opposition that
differentiates human from the other species.
The hypoplastic thumb is characterized by a
variable degree of bony and soft tissue inadequacy. There are several denitions of hypoplastic thumb: A thumb is considered to be
underdeveloped if deciency of any one or all
structures is present [1]. Congenital hypoplastic
thumb is dened as a short, underdeveloped
thumb with decient or absent intrinsic muscles
with or without decient extrinsic musculoskeletal structures [2]. The thumb is considered hypoplastic when its tip does not reach the midway
point of the proximal phalanx of the index
nger.
Depending on degree of hypoplasia, the nonsurgical or surgical treatment differs. In general,
when the hypoplastic thumb lacks basilar joint
stability or is absent, the hand is best treated by
politicization of the index nger. When hypopla-
© Springer Nature Switzerland AG 2023
G. Pajardi (ed.), Pediatric Hand Surgery, https://doi.org/10.1007/978-3-031-30984-7_14
167
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G. Pajardi et al.
sia is less severe, the thumb should be retained
and reconstructed.
Treatments, usually, starts very early in the
childhood. The pollicization is performed at
about 1 year of age. This is preferred because by
6 months the infant begins to move the thumb
voluntarily, by 9months the thumb gains its independence and mobility from the palm whereas by
1 year of age, it becomes a crucial portion of
hand function [3]. Than younger surgery takes
advantage of brain plasticity and ease of incorporation into daily activities.
14.1 Epidemiology
The exact incidence of thumb hypoplasia is difcult to determine because of the large number of
upper limb malformations, which contain some
type of thumb deciency. Kozin reported that the
rate of birth anomalies is about 1% to 2%; of
these about 10% occur in the upper extremity [4].
Entin [5] reported a 16% incidence of thumb
hypoplasia among Canadian patients whereas
Flatt [6] published an 11.2% incidence of thumb
abnormalities and a 3.6% incidence of thumb
hypoplasia or aplasia.
14.2 Associated Conditions
Thumb hypoplasia can occur isolated or in the
context of other diseases. The presence of associated congenital anomalies and syndromes should
be investigated with the aid of a geneticist.
More common association include:
• Holt Horam syndrome.
• VACTERL associations: vertebral abnormali-
ties, anal atresia, cardiac abnormalities, tra-
cheo esophageal stula and/or esophageal
atresia, renal agenesis and dysplasia, and limb
defects.
• TAR SYNDROME (thrombocytopenia-absent
radius): does not result in thumb hypoplasia
but instead leads to a at broad thumb.
• Fanconi anemia.
• CHARGE syndrome (coloboma of the eye,
heart defects, atresia of the nasal choanae,
retardation of growth and/or development,
genital and/or urinary abnormalities, and ear
abnormalities and deafness).
14.3 Classication
Muller described the rst classication of thumb
hypoplasia in 1937, subsequent modication
done by Blauth, Buck-Gramcko and Manske has
improved the classication.
Nowadays, the accepted classication is the
modied Blauth classication that is categorized
into ve general types or grades.
This classication is used not only to describe
the degree of hypoplasia but also to predict physical ndings and guide treatment.
A type I hypoplastic thumb is stable with good
overall function but slightly smaller than a typical thumb. Both intrinsic and extrinsic muscles
are present.
In type II and type III, there is a narrowing of
the thumb index web space, aplasia/hypoplasia of
thenar muscles, and instability of the thumb
metacarpophalangeal (MCP) joint. In type II,
thumbs have intrinsic muscle aplasia/hypoplasia
whereas type III thumbs have intrinsic and extrinsic muscle aplasia/hypoplasia.
Manske etal. [7] sub-classied type III thumbs
into A or B based on the condition of the carpometacarpal (CMC) joint. The distinction is that the
type IIIA thumb has a stable CMC joint. Type IIIB
has severely underdeveloped CMC joint unstable.
Type IV deciency is a oating thumb, in
which a rudimentary digit is connected to the
hand by only skin and a neurovascular bundle.
Type V deciency is a complete absence of the
thumb (Fig.14.1).
14.4 Indications
In severe grade of hypoplasia, from type IV to
type V, the indication is the pollicization of the
index nger.
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Fig. 14.1 Grades of thumb hypoplasia from grade II onwards
169
Although in the last years there are some controversial for the indication in Blauth IIIB, index
nger pollicization remains the ideal reconstruction also in this grade of hypoplasia.
Pollicization is also indicated in other congenital diseases as mirror hand, macrodactyly, multingered hand and some traumatic cases.
The patient has to be healthy and able to tolerate general anesthesia. Must be investigated for
any associated syndrome and disease. The surgery is not appropriate for children with several
central nervous system deciencies.
Must be discussed with parents the expected
functional results: a stiff index nger will make a
stiff neo thumb [8].
14.5 Techniques
The current technique of pollicization represents
a consolidation of contributions from surgeons
over the last 100years [9–11].
The procedure was rst described by Littler in
1952, modied by Buck-Gramcko in 1971 and
then many surgeons rened the technique to
improve appearance and function.
In the literature, the most discussed items
include skin incision, positioning and xation of
the metacarpal head, rebalancing of intrinsic
muscles and extensor tendons.
There isn’t a perfect technique but every surgeon prefers the one in his hands is better.
The goals are to provide ample access to the
index for pollicization, to reconstruct the rst
web space, recreate a functional new thumb with
a good opponent position.
The pollicization requires a stepwise approach.
Some of the critical points are:
1. Adequate design of the skin incision to allow
easy index nger transposition and creation of
an adequate thumb-index web space. This
allows avoiding scar in the web space and preventing a contracture.
2. Preservation and reinsertion of intrinsic muscles in order to ensure thumb adduction and
abduction,
3. Epiphysiodesis of the grow plate of metacarpal to prevent excessive growth of the base of
the pollicized index nger,
4. extension of the MCP joint to avoid future
thumb hyperextension,
5. xation of the new thumb in opposition [12].
Our personal technique based on Buck-
Gramcko technique modied by Foucher [13].
In this procedure, there’ s a modication of
the skin incision, rebalancing of extensor tendons, and bone xation (Fig.14.2).
The surgery starts with the children placed in
a supine position under general anesthesia. A brachial plexus axillar block is performed to avoid
pain during operation and in the early postoperatory period. A pediatric tourniquet is placed on
the upper arm. Preoperative antibiotic prophylaxis is administered routinely.
The limb is gently exsanguinated to allow better visualization of the digital vessels.
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Fig. 14.2 Buck-Gramcko technique modied by Foucher: skin incision
G. Pajardi et al.
In children with a type IV or IIIB thumb hypoplasia, the incision wraps around the base of nonfunctional thumb. The extra digit is then removed
with bone, tendon, nail, and neurovascular bundle are cauterized. In case of presence of hypoplastic thenar muscle, this is detached and
preserve to restore thenar eminence.
The palmar skin is incised rst and the ap is
raised distally. Neurovascular bundles are identied and isolated from common digital vessels
and nerves to the index-long web space and radial
side of index nger. Additional dissection could
be necessary in case of arterial variations. The
distal arterial bifurcation is identied and its
contribution to the long nger is ligated. Proximal
microdissection is necessary to further separate
the proper digital nerves to easier translation of
the nger. To prevent inadvertent vessel injury
could be helpful the use of surgical loop.
The rst annular pulley of the index nger is
identied and incised to allow mobilization of
index nger and prevent buckling of the exor
tendons after the digit is shortened. The intermetacarpal ligament is identied and divided.
The dorsal incision extends transversely
across the PIP joint. The dorsal ap is sharply
elevated with the conservation of as many dorsal
veins as possible. The veins are then followed
proximally to allow good exposure of extensor
tendons until MCP joint. The index extensor tendons are isolated and cut at the MCPJ.Then are
splitted proximally until PIP joint into two bundle. This procedure creates new tendons for the
reinsertion of intrinsic muscle.
The pollicization proceeds with the identication, isolation, and mobilization of the rst dorsal
and palmar interossei muscles. They are released
distally with a portion of aponeurosis in preparation for suture xation and reinsertion. Beware to
isolate carefully the tendons from collateral ligaments to avoid damage of the metacarpophalangeal joint.
Once all of the soft tissues are adequately dissected and prepared, the entire metacarpal bone
is exposed.
Removing of the diaphysis metacarpal bone
shortens the index nger. With the soft tissues
retracted, two osteotomies through metaphyseal
portion are performed in a perpendicular direction. The distal cut is directly through the physis
using a ne blade. In this step, it is important to
preserve the periosteal because it gives stability
to the new CMC joint. Then it is performed the
epiphysiodesis through physeal ablation to prevent unwanted growth of the new thumb.
The base and the head of the metacarpal bone
are xed into hyperextension position with a mini
or micro mitek anchor. This position is made to
rectify the discrepancy between index metacarpophalangeal joint that hyperextend and normal
thumb carpometacarpal joint that does not
hyperextend.
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Many authors prefer to give the right position
and x the new joint with a Kirschner wire.
Usually, we prefer to avoid that to allow early
rehabilitation program. The correct position is
reached by suturing the intrinsic muscle, tendons,
and skin. Just in older children, the xation with
k wire could give more stability at the joint.
Next step is the reinsertion of intrinsic muscles and extensor tendons. The palmar and dorsal interossei muscles are sutured at the ulnar
and radial bands, respectively. In this way, the
palmar interossei muscles become the new
adductor pollicis and the dorsal one the new
abductor of the thumb. The proximal portion of
extensor tendon is reattached to the central part
of extensor complex to be new extensor pollicis
longus.
This rebalancing gives a stable and good position to the thumb: about 45 degrees of abduction
and 135 degrees of pronation.
Once the index nger has been secured in the
new position is important to ensure hemostasis
control to avoid bleeding and hematoma
formation.
The nal closure of the skin is performed with
rotation of the volar part of the ap to cover the
rst web space. This allows avoiding scar in the
rst web space and giving more stability to the
thumb. Any redundant skin is excised (Fig.14.3).
The procedure is completed. The tourniquet is
deated. Any persistent bleeding, ischemia or
venous congestion must be investigated before
making the dressing. The upper extremity is
immobilized with a well-padded long arm cast
with the thumb in an opposition.
The child is admitted overnight and the arm is
elevated to promote venous drainage. Ten days
later, the cast and the dressing are removed under
sedation and replaced with removable split. From
now start the rehabilitation program.
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