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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_777_Библиотеки_им_академика_М_И_Перельмана
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S. K. Vilkki
17.5.4 Observations ontheResults
17.5.4.1 The Metatarsal: Distal Ulna
Ratio
The balance [11] in two forks at Y-form ulna is
slowly changing with the growth and age. Due to
overcorrection, there will be some ulnar deviation of the wrist axis after the transfer and it
slowly will turn into mild radial deviation at adolescence. This may sometimes be an indication
for secondary correction at the age of 12–15years.
17.5.4.2 MTP-II Joint Alignment
andStability
There has appeared a tendency for MTP-joint
subluxation in some extremities. That will affect
the result allowing more radial deviation. This
was learnt during long-term follow-up study and
more attention has been paid thereafter to primary operation. It is important to reconstruct all
possible stabilizing forces around the joint. A
slight exion at the joint graft will further provide better stability in the beginning. Possible
reasons for later developing instability are collected in Table17.2.
Fig. 17.10 Good growth balance at Y-fork arms during
the growth. MT-arm length 45.5mm and Ulna-arm length
44.0mm. Total Ulna length 130mm. X-ray taken at about
10years of age and almost 8years postoperatively.
17.5.3 Donor Side Morbidity
The foot development has been good in long term
after second ray removal. Very few complaints
have been reported and most patients consider
their operated foot as normal (Fig. 17.12). It is
important that during primary operation the web
space can be closed without skin grafts. Therefore,
only minimum of skin has been taken with the
graft.
17.5.4.3 Necessity forLong-Term
Follow-Up
Especially after age 11, the continuous observation on development of wrist alignment and
MTP-joint stability is important. During adolescence, the second faster growth spurt may change
the wrist axis to turn slowly into radial deviation.
At that period from 11 to 15years, a keen follow up is mandatory. The tendency for the change to
radial deviation can be eliminated by performing
an epiphyseodesis at distal ulna. Alternatively,
when the ultimate length of forearm is important,
a reoperation to correct the malalignment may be
needed in some cases. That salvage operation can
be accomplished with a joint transport technique (Fig.17.13). It requires an osteotomy at the
base of metatarsal graft and use of secondary dis-
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b
a
d e
c
Fig 17.11 A long-term result: Wrist reconstruction was
done at age 15months. Pollicization at age 3years and
corrective ulna osteotomy at age 14years. (a–c) X-ray
series taken at 1year, 3years, and 26 years. (d, e) The
Patient at age 26. Good wrist mobility from 20-degree
extension to 70-degree exion. Her profession is a nurse
(video 17.1)
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Fig. 17.12 “No problems at the donor foot postoperatively. Second ray removed from the right foot. Also the
scar has remained minimal when a fast Zig-Zag incision
was used. Compare Figure Fig.17.5a”
S. K. Vilkki
Table 17.2 Possible reasons for malalignment and
instability
1. Primary malalignment of the graft
• Too much deviation of MT-branch. Below 40o is
acceptable
• Joint graft in hyperextension
2. Primary lack of external muscle power around the
joint because of difculty to reconstruct normal
dynamic stabilizers of the MTP-joint. Often not
enough tendons to be transferred
3. Secondarily malalignment due to a poor metatarsal
bone growth, leading to wrong balance at Y-fork
during the growth
4. Secondary instability at MTP-II joint
Fig 17.13 (a–c) X-ray series of a salvage procedure to
correct wrist motion axis and alignment. Secondary distraction after detachment of the proximal graft end with
longitudinal osteotomy. Joint transport will happen easily
using double distractors. A small separate xator is
attached between two pins inserted at metatarsal bone and
one distal hand pin together with larger distractor
ab
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217
traction. The metatarsal becomes elongated during distraction and it easily consolidates
spontaneously with new bone formation.
Selection and need for secondary procedures as
well as timing are made individually.
17.6 General Remarks about
Microvascular
Reconstruction ofRadial
Club Hand
17.6.1 Positive Remarks
Microvascular reconstruction with MTP-II joint
transfer has some advantages over conventional
methods. The approach is less traumatic to
epiphysis of distal ulna. It allows a natural growth
of ulna as it would typically happen in radial dysplasia. However, it cannot normalize the forearm
length, which will commonly remain clearly subnormal in Types II–IV of radial dysplasia. In
author’s series, relative ulna length (RUL) has
been in mean 67%.
The wrist-like active extension-exion mobil-
ity has regularly been superior compared to cen-
tralization or radialization series. A common
range of total active motion (TAM), which is easily achieved, has been between 75 and 100° and
in long-term study was in mean 83%.
Cosmetic appearance is greatly improved with
corrected wrist stability (Fig.17.14).
The donor site morbidity remains commonly
very low after second toe ray removal. It is comparable with typical toe-to-hand transfer.
17.6.2 Negative Remarks
There is a slow tendency to partial recurrence of
radial deviation at adolescence. This is because
metatarsal bone growth is slower than distal ulna
growth during the second growth spurt after age
11years.
Secondary subluxation at transferred MTPjoint due to inadequate stabilizing muscle force
can in some lower quality hands deteriorate the
result. Therefore, this reconstruction is not recommended for low-quality extremities with very
high severity index.
Fig. 17.14 (a) An example of a severely deviating radial club hand that was operated at age 2years with vascularized
MTP-II joint transfer. Severity Index was mild or 6 p. (b) The treated forearm at age 6years
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S. K. Vilkki
17.7 Conclusion
The microvascular option using MTP-II ray for
reconstruction of radial dysplasia
types III and IV is providing a stabilization of
radial club hand with growing bone and movable
joint unit. It is giving good results during the rst
decade of life, but it may need further treatment
when second growth spurt in adolescence is
actual. However, the demand for secondary corrections has remained low in author’s series.
Alternatively, an epiphyseodesis of distal ulna at
age 11–12years or a salvage procedure with joint
transport more distally at a later stage can be used
for further correction. Good motion at the wrist is
appreciated by patients [12]. Donor site morbidity is commonly considered minimal. The treatment cannot overcome commonly appearing
limb length discrepancy in unilateral cases
(Table17.3).
Table 17.3 Main principles for successful microvascular
reconstruction
1. Treat from the birth with continuous stretching and
splinting
2. Before any operation check the quality of involved
extremity using severity grading.
3. Distract slowly to full correction to allow some
overcorrection
4. Microsurgical skill and treatment culture is the
prerequisite for success
5. Perfect alignment of the graft is mandatory
6. Healing of the graft at both ends must be complete
before disassembly of distractor
7. Protect the graft during rst 6months
8. Pollicization, when feasible, is done 1year after
joint transfer
9. Follow-up is necessary through the growth period
and especially important before adolescence
References
1. Vilkki SK. Severity grading in radial dysplasia. J
Hand Surg Eur Vol. 2014;39(9):977–83.
2. Morsy M, Parry JA, Moran SL. Vascularized second metatarsophalangeal joint transfer for salvage of
failed centralization in radial longitudinal deciency:
case report. Ann Plast Surg. 2017;78:195–7.
3. Nettelblad H, Randolph MA, Weiland AJ. Free
microvascular epiphyseal-plate transplantation. An
experimental study in dogs. J Bone Joint Surg Am.
1984;66(9):1421–30.
4. Vilkki SK. Distraction and microvascular epiphysis transfer for radial club hand. J Hand Surg Br.
1998;23:445–52.
5. Inberg P, Kassila M, Vilkki S, Neuvonen P.Anaesthesia
for microvascular surgery in children: a combination
of general anaesthesia and axillary plexus block. Acta
Anaesthesiol Scand. 1995;39:518–22.
6. Vilkki SK. Advances in microsurgical reconstruction of the congenitally adactylous hand. Clin Orthop
Relat Res. 1995;314:45–58.
7. Vilkki SK.Vascularized joint transfer for radial club
hand. Tech Hand Up Extrem Surg. 1998;2:126–37.
8. Vilkki SK.Radial Club hand: wrist distraction and
joint transplantation. In: EFORT textbook: surgical
techniques in Orthopaedics and traumatology 55.370C- 10. Amsterdam: Elsevier; 2001. p.5p.
9. Vilkki SK. Vascularized metatarsophalangeal joint
transfer for radial hypoplasia. Semin Plast Surg.
2008;22:195–212.
10. Murphy GRF, etal. Correction of “wrist” deformity
in radial dysplasia: a systematic review and metaanalysis. J Bone Joint Surg Am. 2017;99:2120–6.
11. Vilkki SK, Paavilainen P, et al. Vascularized second
metatarsophalangeal joint transfer for radial deciency—an update. J Hand Surg. 2018;43(9):907–18.
12. Ekblom, etal. Hand function in children with radial
longitudinal deciency. BMC Musculoskel Disord.
2013;14:116–29.
Metacarpal Synostosis
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AnnaM.Acosta andTerryR.Light
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Abstract
Metacarpal synostosis is an uncommon hand
anomaly that may occur in isolation (ringsmall metacarpal) or in connection with a congenital syndrome (Apert, Ellis–van Creveld).
It has been hypothesized to be sporadically
inherited via x-linked recessive or autosomal
dominant traits and is possibly linked to a
genetic abnormality in the FGF16 gene. This
hand difference likely forms during the rst
4–8weeks of gestation during rapid development of the upper limb.
Children are most often evaluated due to
parental concerns regarding a deviated nger,
most commonly the small nger that is
abducted from the hand. Radiographic examination can conrm the diagnosis of metacarpal synostosis. Treatment is guided by
symptoms and digital function. Operative
treatments involving osteotomy and bone graft
interposition. Though multiple techniques
address deformity and length discrepancy,
affected digits may remain hypoplastic and
stiff and may benet from amputation.
A. M. Acosta
MemorialCare Miller Children’s & Women’s
Hospital Long Beach, Long Beach, CA, USA
T. R. Light (*)
Loyola University Stritch School of Medicine,
Maywood, IL, USA
e-mail: tlight@lumc.edu
Keywords
Metacarpal synostosis · Apert syndrome ·
Ellis–van Creveld · Bone Graft · Osteotomy
18.1 Introduction
Metacarpal synostosis is an uncommon congenital anomaly of the hand characterized by a partial
or complete fusion of the metacarpals [1]. The
incidence of metacarpal synostosis has been variously estimated to be 0.02%, 0.07% [2], 0.002
[3], and 0.007 [4]. The rst recorded documentation of metacarpal synostosis was an 1827 drawing depicting fusion of metacarpals in a German
publication [5, 6]. Metacarpal synostosis has
been referred to as absent fth metacarpal [3],
syndactyly type V [7], bilateral ulnar thumbs [8],
congenital metacarpal malformation [9], and fth
ray anomaly [10].
Though metacarpal synostosis may occur
between any adjacent two rays, it is most commonly observed as fusion between the ring and
small nger metacarpals [5, 6, 11–14]. Metacarpal
synostosis may occur in isolation or in association with other anomalies including central polydactyly, radial deciency, ulnar deciency, cleft
hand, and Apert syndrome [5–6, 11–12, 15].
Hands with metacarpal synostosis have one
of two common appearances. The rst is a seemingly normal hand with ngers that are deviated
in the coronal plane from the normal resting
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G. Pajardi (ed.), Pediatric Hand Surgery, https://doi.org/10.1007/978-3-031-30984-7_18
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A. M. Acosta and T. R. Light
alignment of the hand. The digits cannot be
straightened out with manipulation. Abutment
of the proximal phalanx bases at the metacarpophalangeal joints (MCPs) of the neighboring
ngers causes the splaying deformity. The second, less common appearance, is an overly
broad or wide palm due to metacarpal shaft
deviation with associated adduction of the
involved nger. The clinical and radiographic
appearance of the hands guides classication
and treatment strategies. Surgical correction of
metacarpal synostosis aims to alter bony structure to allow for soft tissue realignment across
the metacarpophalangeal joints. Correction of
metacarpal synostosis can be challenging, with
early technically success in some patients
eroded by recurrence of deformity
18.2 Genetics
Metacarpal synostosis may occur in isolation or
in association with other hand anomalies. Early
German literature suggested a familial inheritance pattern for cases of metacarpal synostosis
[5]. More recent literature has suggested a sporadic inheritance patterns, x-linked recessive, or
autosomal dominant, for most cases of isolated
metacarpal synostosis [1, 2, 5, 16]. The most
common presentation of isolated metacarpal synostosis involves the ring and small nger metacarpals. Jamsheer et al. [1] reported on two
unrelated patients with a sporadic presentation of
ring-small metacarpal synostosis. Through
exome DNA sequencing, they found a genetic
nonsense mutation on chromosome Xq21.1 in
exome 3 of the FGF16 gene associated with
X-linked recessive mutations. Thus, they hypothesized that X-linked recessive FGF16 mutations
may be a novel cause of isolated metacarpal synostosis [1]. Other authors have noted this anomaly to be more prevalent in males supporting the
likelihood of an x-linked recessive inheritance
pattern. Females are more likely to be genetic
carriers [5]. Robinow etal. [7] published a case
report on a family with 4 members diagnosed
with syndactyly type V, syndactyly with metacarpal and metatarsal synostosis. His study of this
family suggested a sporadic mutation in the rst
individual affected (mother), proceeding into the
following generation as an autosomal dominant
trait to three of her four children.
Metacarpal synostosis may also present as
part of a syndrome. Gottschalk etal. [2] detailed
syndromes involving carpal coalitions and metacarpal synostosis. Ellis–van Creveld syndrome is
a rare disorder of chondro- ectodermal dysplasia
that presents as a short- limbed dwarsm. Hands
of individuals with Ellis–van Creveld syndrome
may demonstrate metacarpal synostosis, clinodactyly, capito- hamate coalition, or postaxial
polydactyly [15]. It is an autosomal recessive disorder effecting the EVC1 and EVC2 genes on
chromosome 4p16 [2]. Half of Ellis–van Crevald
syndrome patients also show abnormalities in the
cardiac system, skin, nails, hair, and teeth.
Although uncommon, the most frequent syndrome associated with metacarpal synostosis is
Apert Syndrome. Acrocephalosyndactyly, or
Apert syndrome, is an autosomal dominant disorder characterized by craniofacial malformations
and complex complicated syndactyly of the
hands [17]. Metacarpal synostosis in patients
with Apert syndrome is different from synostosis
noted in isolated cases. The Apert hand synostosis involves the proximal portion of the ring and
small nger metacarpals, however, the small nger is not held in an abducted position as it is in
isolated cases [17] (Fig. 18.1). Dao et al. [17]
determined that approximately 77% of their
Apert syndrome patients demonstrated ringsmall metacarpal synostosis. They noted that
because not all synostoses were ossied at birth
the synostosis may not always be visible initially
on radiographic examination. Because the synostosis is only evident later in childhood, one can
infer that a synchondrosis or synbrosis presents
at birth later ossies. The authors also observed
that the small nger in patients with Apert syndrome tended to be the most “normal” digit in
these hands. They suggested that it was benecial
to resect the synostosis bridge between the ringsmall metacarpal synostosis to increase the
mobility of the small nger carpometacarpal join
thereby enhancing the ability of the small nger
to reach the thumb [17].
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a b
Fig. 18.1 (a, b) (a) Clinical photograph of a hand in a patient with Apert syndrome. (b) Radiograph of a hand in a
patient with Apert syndrome demonstrating complex complicated syndactyly with metacarpal synostosis
18.3 Embryology
Upper limb embryologic development occurs
between the fourth and eighth week of gestation.
At week 4, the upper limb bud begins to develop,
at week 5, the hand plate is present, at week 7,
interdigital apoptosis and chondrication of middle phalanges occur, and at week 8, wrist and carpal inter-zones are created. Most congenital hand
anomalies occur during weeks 4 and 8 of gestation during the rapid development of the upper
limb [18]. Most authors have felt that metacarpal
synostosis was secondary to a failure of differentiation during limb bud development. Other
authors have challenged this theory citing similarities between the hands of metacarpal synostosis and limb longitudinal deciencies as evidence
that this condition is due to a failure of
formation.
Dermatoglyphics is the study of hand and foot
skin ridge patterns. Temtamy and McKusick [16]
studied the dermatoglyphics in the hands of
patients with syndactyly and in the hands of
patients with metacarpal synostosis. The authors
found abnormalities in the patterns of digital tri-
radii c and d in patients with metacarpal synostosis similar to the abnormalities in patients with
syndactyly. Miura [12] reviewed 14 patient cases
with metacarpal synostosis using hand pattern
proles and dermatoglyphics. Miura also concluding that the differences in patients with metacarpal synostosis were similar to those of patients
with syndactyly of the ring and small ngers.
They concluded that metacarpal synostosis
should be classied as failure of separation/differentiation (as syndactyly is classied) rather
than as a failure of formation or longitudinal
deciency.
Ogino and Kato [4] reviewed nine cases of
metacarpal synostosis. They found characteristics common to the hands of patients with ringsmall metacarpal synostosis and shared with the
hands of patients with ulnar longitudinal deciency. Patients with ring-small metacarpal synostosis tended to have a relatively small, small
nger with hypoplastic hypothenar musculature
and generalized hypoplasia of the hand. Citing
prior studies of longitudinal deciency, Ogino
and Kato hypothesized that this limb hypoplasia
seen in metacarpal synostosis may also be
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A. M. Acosta and T. R. Light
related to a decit in mesenchymal cells in the
developing limb bud. However, in their study,
they had one patient without hypoplasia of the
ulnar side of the hand, leading them to consider
an alternate explanation of the failure of induction of the digital rays in the hand plate, as previously hypothesized by Miura [12] and
Temtamy and McKusick [16].
18.4 Clinical Exam
Metacarpal synostosis may not be apparent at
birth. Parents often seek evaluation because the
small nger is “growing crooked”. Most will
have detected a deviation of the small nger from
the rest of the hand over time. They may observe
the child having difculty with the nger becoming caught or what they perceive as an awkward
grasping pattern, as the nger remains deviated
or scissors over the neighboring nger as the
hand is closed. Parents often comment that there
seems to be an “absent knuckle” when the short
small nger is compared to the contralateral side
[19] (Fig.18.2)
Synostosis involving the ring and small metacarpals is the most common pattern of involvement. Sixty to eighty percent of these children
have bilateral hand involvement [5]. Synostosis
of the middle and ring metacarpals is the second
most common pattern. These children present
with the divergence of the two ngers, a radially
deviated middle nger and an ulnarly deviated
ring nger. The third most common pattern is
synostosis of the thumb and index metacarpals
(Fig.18.3).
On clinical exam, the affected hand may lack
digital exion or extension creases [5]. Active
exion of the ngers involved may be restricted
in addition to splaying of ngers apart from one
another [1, 5, 12, 20]. The affected child is unable
to fully adduct the involved ngers either passively or actively. When the ngers are exed
into a clenched st, they may scissor over the
adjacent nger [21].
In the ring-small metacarpal synostosis, the
small nger may be hypoplastic and abducted
from the ring nger. Muira [12] attributed the nger abduction phenomenon to be secondary to
both bony and soft tissue developmental differ-
Fig. 18.2 (a, b) Clinical photograph of a hand with isolated ring-small nger metacarpal synostosis. (a) dorsal (b)
volar
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Fig. 18.3 Thumb-index metacarpal synostosis
ences. He found that intrinsic muscles were displaced palmarward to the synostosis. When the
metacarpal heads converge distally, the proximal
phalangeal bases on the adjacent ngers abut
resulting in static deviation. The normally central
tracking extrinsic exors and extensor tendons
are then forced to track eccentrically as the nger
deviates and the space between the metacarpal
heads is narrowed. This creates a secondary
dynamic abduction force across the metacarpophalangeal joints, further deviating the nger.
As the metacarpal heads converge distally,
the physeal orientation in the distal metacarpal
becomes convergent with the adjacent metacarpal metaphysis/shaft leading to altered bone
development. Buck-Gramcko hypothesized
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Fig. 18.4 Radiograph of hand with isolated ring-small
nger metacarpal synostosis with compression of head of
small nger metacarpal
that associated small nger hypoplasia may be
secondary to the pressure applied by the adjacent nger metacarpal [5, 14, 20] (Fig.18.4).
In line with the Hueter-Volkman principles,
compression across the metacarpal epiphysis
may cause retardation of longitudinal growth
of the bone. Buck-Gramko described hypoplastic changes to progress over the rst few years
of life but reported minimal change after the
age of 4years [5].
18.5 Radiographic Exam
Radiographic examination of the hand will conrm the clinical examination and will establish
the diagnosis of metacarpal synostosis.
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