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Fig. 18.5 (a, b) (a) Partial metacarpal synostosis of the middle and ring nger. (b) Complete metacarpal synostosis (expanded metacarpal head)
Synostosis, when present, is invariably present proximally at the base of the metacarpals. Fusion between the metacarpals may be partial or may extend the entire length of the involved rays in a complete synostosis. The metacarpal head may take the form of a single expanded head or as two distinct heads (Fig.18.5). The metacarpal shafts may deviate, converge, or run
parallel to one another (Fig. 18.6). The con­verging metacarpals may appear hypoplastic and the physis abnormal secondary to compres­sion of the neighboring metacarpal cortex. The proximal phalanx of the adjacent ngers will often abut at the base causing deviation of the phalanges at the metacarpophalangeal joint (Fig.18.7).
bc
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Fig. 18.6 (a–c): Ring-small nger metacarpal synostosis with deviating (a), converging (b), and close parallel (c) metacarpal shafts. Resultant adduction (a) or abduction (b, c) of the small nger at the MCP
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Table 18.1 Buck-Gramcko and Wood [5] classication of metacarpal synostosis
Synostosis at the base of the metacarpals with
Type I
minimal growth disturbance
Type IISynostosis involving <50% of the metacarpal
shafts
Type
Synostosis involving >50% of the metacarpal
III
shafts
IIIa Separate metacarpophalangeal joint for each
digit
IIIb Common metacarpophalangeal joint for both
digits, “super digit”
Fig. 18.7 Radiograph of bilateral hand middle-ring n­ger metacarpal synostosis. Phalangeal abutment at the base of proximal phalanx’ of middle and ring ngers causing splaying deformity
18.6 Classication
In 1993, Buck-Gramcko and Wood [5] proposed a simple classication system for metacarpal synostosis after evaluating 109 patients with 152 involved hands. They described three different anatomic types of metacarpal synostosis (Table18.1). Type I involves coalition at the base of the metacarpal only with minimal deformity or growth disturbance. Type II hands have a synos­tosis that extends up to half of the length of the metacarpal shaft. The small nger is often small, short, and ulnarly deviated. Type III hands include a synostosis that extends along more than half of the length of the metacarpal. Type III hands were subdivided into two groups. Type IIIA hands have a synostosis of greater than 50% of the metacarpal shaft length but possess a sepa­rate metacarpophalangeal joint for each digit. Type IIIB hands have a metacarpal synostosis of more than 50% of the metacarpal length and the involved digits share a common metacarpopha­langeal joint, creating a digit termed a “super
digit” by Wood [22]. In 2001, Foucher etal. [6] presented a new classication system for meta­carpal synostosis (Fig. 18.8). They argued that though the Buck-Gramcko and Wood classica­tion system was simple and easy to use, it failed to address the characteristics that would deter­mine surgical treatment. They observed 36 patients with metacarpal synostosis over a 24-year period and proposed an “easy-to­remember” classication system based on the shape of the synostosis, the direction of the epiphysis growth, the nger deformity, webbing and hypoplasia of the metacarpal bone (Table18.2).
In 2014, Liu etal [23] proposed a third clas­sication system for metacarpal synostosis. Liu reported difculty classifying every patient with ring-small metacarpal synostosis using either the Buck-Gramcko/Wood Classication or the Foucher classications. Liu’s aim was to create a treatment-oriented system that focused on the most common metacarpal synostosis, ring-small metacarpal synostosis. They evaluated 20 hands in 13 patients with ring-small synostosis treated over a 20-year period. Their classication system was dened by the inter-metacarpal angle (IMA) and the degree of shortening of the small nger ray. They proposed treatments based on their classication (Table18.3).
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Fig. 18.8 Foucher etal. [6] classication picture diagram. Permissions pending
a
“I”
a
Table 18.2 Foucher etal. [6] classication of metacarpal synostosis
Shape Deformity Description I-shaped Single enlarged metacarpal
Id Tw o distinct
metacarpophalangeal joints
If Single (fused)
metacarpophalangeal joint for two ngers
U-shaped Parallel epiphysis and
synostosis at metacarpal base Us Symmetric metacarpal lengths Ua Asymmetric metacarpal lengths Ut Tightly fused metacarpals
Y-shaped Divergent epiphysis
Ys Symmetric metacarpal lengths Ya Asymmetric metacarpal lengths
K-shaped Converging metacarpals and
short fth metacarpal, ngers
diverge, or shaped like
parentheses with or without
webbing
b
b
a
“U”
b
“y” “k”
Table 18.3 Liu etal. [23] classication for fourth-fth metacarpal synostosis
Type Description of Deformity Type A1Narrow IMA without severe shortening of the
fth ray, no/mild deformity
A2 Narrow IMA, with severe shortening of the fth
ray, no/mild deformity
Type B1Wide IMA, without severe shortening of the
fth ray, wide palm; wide fourth web; bony prominence on ulnar palm; poor abduction of the small nger
B2 Wide IMA, with severe shortening of fth ray,
wide palm; wide fourth web; bony prominence on ulnar palm; poor abduction of the small nger
Type C1Reverse IMA, without severe shortening of fth
ray, narrow palm; abduction deformity of the little nger
C2
Reverse IMA, with severe shortening of fth ray, narrow palm; abduction deformity of the little nger
c
18.7 Treatment
Treatment of metacarpal synostosis is guided by patient function and family preferences. The severity of deformity is not the sole consideration when making treatment decisions. Treatments
options range from observation to reconstruction to amputation. Many patients with metacarpal synostosis have satisfactory hand function with minimal or no digital malalignment. Buck­Gramcko observed that many deformities stabi­lize around 4years of age [5].
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Amputation may be considered in patients with a stiff or nonfunctioning digit that inhibits hand function. Yuan et al. [24] published a case report of a 4-year-old with a ring-small metacar­pal synostosis and Y-shaped deformity. Though the authors suggested amputation based on the ngers’ interference with hand function, the fam­ily declined this treatment.
Surgical treatment of metacarpal synostosis is guided by the severity and shape of the deformity as well as the specic metacarpals involved. All techniques involve osteotomy of the conjoined
a
metacarpals and interposition of a spacer fash­ioned to realign the metacarpal heads into a more normal anatomic relationship. Bony realignment may reposition the metacarpal epiphysis into an uninhibited position and improve the balance of intrinsic and extrinsic muscles across the meta­carpal heads. Repositioning the metacarpal head improves the mechanical axis of the musculoten­dinous units crossing the joint and allows the digit to rest in a more anatomic orientation with­out abutment against the adjacent digit [11] (Fig. 18.9). Ueba and Seto [15] recommended
b
Fig. 18.9 (a, b) Pre (a) and post (b) operative clinical photographs demonstrating “splaying deformity” and correction after metacarpal synostosis widening osteotomy with bone graft interposition
cd
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Fig. 18.10 Metacarpal synostosis osteotomy with graft interposition. (a) Metacarpal synostosis with abduction of small nger. (b) Longitudinal osteotomy of synostosis does not penetrate carpo-metaarapl joint. (c) Lamina
surgical correction of metacarpal synostosis before the age of 2years. In patients with splay­ing deformity, they stressed that if the metacarpal is not osteotomized early, the growth would be permanently impaired secondary to compression of the epiphysis by the adjacent metacarpal. Early surgical correction may improve orientation and facilitate opposition when the small nger meta­carpal is involved. In contrast, Ueba and Seto warned that surgical correction of these small bones in young children is technically challeng­ing and increases the risk of physeal injury [15].
Several surgical techniques have been described that address either middle-ring or ring­small metacarpal synostosis. In 1981, Hikosaka and Yabe [25] described an osteotomy of the syn­ostosis with interposition of an iliac crest bone autograft spacer. In 1988, Iwaswa et al. [26] described an osteotomy with interposition of a costal cartilage autograft spacer. Both of these techniques demonstrated the positive effect of widening the space between the metacarpals and repositioning the metacarpal heads in synostoses causing splaying deformity of the ngers (Fig.18.10). Both of these procedures, however, required a remote donor site to obtain the spacer.
spreader increases space between metacarpal diaphyses and gradually realigns small nger small metacarpal. (d) Metacarpal spread is secured with bone graft substitute block. Reproduced from [11]
In 1988, Muira [12] described the correction of metacarpal synostosis with an osteotomy and interposition of a silicone spacer. He hypothe­sized that the silicone would permit independent carpometacarpal motion and could obviate the need to harvest bone graft from the iliac crest of a skeletally immature child. Although the tech­nique corrected the deformity and avoided the donor site morbidity of bone block harvest, fol­low-up demonstrated re-synostosis of the meta­carpal bases [12, 15, 22].
Gottschalk etal. [11] advocated a longitudinal osteotomy of metacarpal synostoses with inter­position of a synthetic bone block. They fash­ioned a spacer from a bone substitute, coralline hydroxyapatite, which mimics the porosity of cancellous bone (Fig. 18.11). They achieved deformity correction and realignment of the metacarpal shafts while avoiding donor site morbidity.
Although these techniques corrected digital splaying deformity, none addressed digital length discrepancy. Hooper and Lamb [20] attempted to address this issue when they described a tech­nique of an oblique osteotomy in the small nger metacarpal. The osteotomy was based from prox-
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b
Fig. 18.11 (a, b) Ring-small (a) and middle-ring (b) nger metacarpal widening osteotomy with interposition of bone substitute graft spacer [11]
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imal radial to distal ulnar in the small nger metacarpal shaft. The metacarpal was then wedged open radially while maintaining ulnar bony contact, resulting in the straightening of the small metacarpal shaft and realignment in paral­lel the ring metacarpal. This effectively addressed the digital splaying deformity but nal results failed to substantially lengthen the metacarpal as they had intended.
In 2004, Jianmongkol et al. [14] proposed a single-stage technique for both deformity correc­tion and lengthening of the metacarpal. They described a double osteotomy coupling a longitu­dinal osteotomy through the metacarpal synosto­sis with an oblique-transverse osteotomy through the metaphyseal base of the shortened metacar­pal. Two bone graft spacers were inserted, one between the metacarpals for correction of angu­lar deformity and one at the base of the shortened metacarpal providing length.
While some authors devoted their work to address metacarpal synostoses with splaying deformity, others focused on metacarpal synosto­ses with nger adduction deformity. In 2000, Yamamoto et al. [27] described an osteotomy in metacarpal synostosis with diverging metacarpals and nger adduction. Their technique involved an osteotomy of the synostosis which included the harvest of a wedge- shaped bone block from the synostosis bifurcation. An opening wedge osteot­omy was then performed ulnarly on the small n­ger metacarpal shaft, leaving the radial cortex intact to maintain the stability of the osteotomy. The harvested wedge was then repositioned into the opening wedge osteotomy of the small nger metacarpal, realigning the small and ring nger metacarpal shafts. The repositioning of the meta­carpals into a more parallel position allowed for realignment of the adducted digit and a slight increase in the length of the shortened metacarpal shaft. This technique allowed deformity correction without the addition of surgical site morbidity from autograft bone block harvest.
Kawabata etal. [28] described a technique of hemi-callotasis designed to correct both nger adduction deformity and the digital length dis­crepancy. This technique was based upon the treatments proposed by Paneva-Holevick and
Yankov in 1980 and De Bastiani in 1987, which demonstrated the ability to lengthen callous 1mm/day using rigid external xation. Kawabata based the osteotomy of the small nger metacar­pal on Fowler’s technique for tibial valgus cor­rection. In Kawabata’s technique, a transverse osteotomy of the small nger metacarpal shaft was performed preserving an intact radial cortex to maintain the osteotomy site stability. The metacarpal was lengthened 1 mm/day using a mini monolateral external xator on the ulnar side of the bone in an opening wedge technique. Kawabata achieved both deformity correction and lengthening of the small nger metacarpal, permitting rebalancing of the abductor digiti minimi force and resulting correction of the n­ger adduction deformity.
In 1993, Buck-Gramcko and Wood [5] detailed their technique for the treatment of middle-ring and ring-small metacarpal synostoses. Like oth­ers, their technique employed the use of synosto­sis osteotomies and bone graft interposition. Their goal was to convert a Type II or III synosto­sis into a Type I (refer to Table18.1 for Buck­Gramcko and Wood classication). In addition, they suggested the possible need for correction of the soft tissues after altering bony alignment. They recommended soft tissue reconstruction to include the possible release or reconstruction of the inter-metacarpal ligament and/or reconstruc­tion of the collateral ligaments of the metacarpo­phalangeal joint.
Buck-Gramcko and Wood [5] also discussed treatment for complicated deformities such as thumb-index metacarpal synostosis. They hypothesized that if treated early, realignment and independent thumb motion may be possible. The surgical procedure included the shifting or transferring of tendons and reconstruction of the collateral ligaments to achieve appropriate thumb function and opposition. Deepening of the rst web space with local skin aps or a large dorsal rotational ap was identied as an essential step. The authors recognized that in cases in which the division of the thumb-index metacarpal synosto­sis would result in a hypoplastic, functionless thumb; that thumb ablation and index polliciza­tion might be a preferable treatment.
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18.8 Complications
Many hands with metacarpal synostosis have metacarpophalangeal joint stiffness, metacarpal deformity, and in some instances a hypoplastic nonfunctioning digit. Surgical treatment of the metacarpal deformity will not correct the hypo­plastic nature of the digit nor resolve metacarpo­phalangeal joint stiffness (Fig. 18.12). Surgical treatment may result in physeal arrest or osteone­crosis of the metacarpal head. This risk might be greater in small hands.
18.9 Summary
Metacarpal synostosis is an uncommon hand anomaly that may occur in isolation (ring-small 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 4–8weeks of gestation dur­ing rapid development of the upper limb.
Recurrence of the metacarpal deformity and renewed splaying of digits has also been reported with long-term follow-up. Re-synostosis of the metacarpal bases is expected after widening osteotomy but has not shown to adversely affect hand function. Re-convergence of metacarpals is more likely after middle-ring synostosis osteotomy than after ring-small synostosis osteotomy.
parental concerns regarding a deviated nger, most commonly the small nger that is abducted from the hand. Radiographic examination can conrm the diagnosis of metacarpal synostosis. Treatment is guided by symptoms and digital function. Operative treatments involving osteot­omy and bone graft interposition have been described in multiple variations. Though multiple techniques address deformity and length discrep­ancy, the affected digits may remain hypoplastic and stiff. Patients with nonfunctioning, stiff, or painful digits may benet from amputation. Complications with surgical correction of meta­carpal synostosis can include physeal arrest, osteonecrosis of the metacarpal head, and recur­rence of the deformity. Patients with metacarpal synostosis are able to lead full and active lives regardless of their deformity.
Children are most often evaluated due to
Fig. 18.12 Follow-up radiograph status post-ring-small nger metacarpal synostosis osteotomy with graft interposition demonstrating continued shortening of the small nger metacarpal and hypoplasia
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