CLASSIFICATION OF T H E M I R R O R H A N D - M U L T I P L E HAND

SPECTRUM

M. M. AL-QATTAN, A. AL-THUNAYAN, M. DE CORDIER, N. NANDAGOPAL and J. PITKANEN

From the Divisions of Plastic Surgery, King Fahad National Guard Hospital and King Saud University, Riyadh, Saudi Arabia

A rare variant of mirror hand is described. The hand had eight fingers and the forearm contained an
ulna and a hypoplastic radius. A classification of the mirror hand-multiple hand spectrum is offered
and its embryology discussed.
Journal of Hand Surgery (British and European Volume, 1998) 23B." 4." 534-536

Mirror hand is a rare congenital deformity which is char-
acterized by duplication of the ulna, absence of the
radius and thumb, and excessive number of fingers with
symmetry about the midline. Since the forearm contains
two ulnae, the deformity is often referred to as ulnar
dimelia (Wood, 1988).
The multiple hand deformity is another exceedingly
rare form of duplication. Yang et al (1996) reviewed the
literature and found six reported cases. In this deformity
there is complete duplication of the hand, including the
thumb, with a distinct radius and ulna in the forearm.
Barton et al (1986) described a patient with eight
digits and three bones in the forearm: an ulna and a
radius, as well as a second vestigial ulna. They suggested
that this case might be intermediate between ulnar
dimelia and the multiple hand deformity.
In this paper we describe another rare variant of
mirror hand in which there are multiple fingers with
the forearm containing an ulna and a hypoplastic
radius. A classification o f the mirror hand-multiple
hand spectrum is also offered and its embryology
discussed.
of the carpometacarpal joints. The child has been using
the hand well for grasping and other activities.
DISCUSSION
A classification of the mirror hand-multiple hand
spectrum is shown in Table 1.
It is important to realize that Type 1A ulnar dimelia is
much less common than Type lB. Wood (1988) has
shown that most cases of ulnar dimelia have a short
preaxial ulna and hence the hand is radially deviated.
Furthermore, the distal end of the preaxial ulna will
adapt and broaden over the years, simulating the distal
end of the radius. An extreme example of this delayed
differentiation of the distal end of the preaxial ulna into
a radius which led the authors to believe that this pre-
axial bone was not a true ulna was reported by
Chinegwundoh et al (1997).
The remaining types of mirror hand are very rare.
After reviewing the English literature, we could only find
a single case of Type 2 (Barton et al, 1986), two cases of
Type 3A (King and Hoyes, 1982; Yang et al, 1996), one

CASE REPORT

A newborn boy was seen for the assessment of a congen-

ital right upper limb anomaly. He was born following a
full term pregnancy. Neither parent had medical prob-
lems and the family history was negative for any congen-
ital anomalies. Except for the right upper limb deformity,
there were no other anomalies and growth and develop-
ment were normal. Examination of the shoulder
revealed no abnormalities. There was limitation of elbow
flexion and the wrist was radially deviated. The hand had
eight digits, each with good active flexion and extension.
The ulnar four digits appeared relatively normal while
the radial four digits were smaller in size. The most ulnar
of this group of radial digits had clinodactyly and the
most radial two digits showed syndactyly. An accentu-
ated web space was seen between the two groups of four
digits (Fig 1).
X-ray examination (Fig 2) revealed an ulna and a
hypoplastic radius in the forearm. The hand had seven
metacarpals and eight fingers. The phalanges of the
radial four digits were hypoplastic and the fourth digit
(counting from the radial side) had a small delta middle
phalanx. The radial three digits were removed at the level Fig 1 Clinical appearance of the hand.

534
MIRROR HAND-MULTIPLE HAND SPECTRUM 535

Fig 2 Radiographic examination of the hand (a) and (b) the forearm.

Table l--Classification of mirror hand-multiple hand spectrum

Type Name Clinicalfeatures

1 Ulnar dimelia Multiple fingers with two ulnae:

2 Intermediate type
3 Intermediate type
4 Syndromat mirror hand
5 Multiple hand
Type A: Each ulna is well-formed.
Type B: The preaxial ulna lacks the styloid process or is
hypoplastic.
Multiple fingers with two ulnae (one of the ulnae is vestigial) and a radius.
Multiple fingers with one ulna and a radius:
Type A: The radius is well-formed.
Type B: The radius is hypoplastic.
Bilateral multiple fingers in complex syndactyly.
Mirror feet and nasal defects are also characteristic.
Type A: Sandrow syndrome = The forearm contains two ulnae.
Type B: Martin syndrome = The forearm contains an ulna and a radius.
Complete duplication of the hand including the thumb with a normal
forearm.

case of Type 3B (the current case report), one case of
Type 4A (Sandrow et al, 1970), and three cases of Type
4B (Hersh et al, 1995; Kogekar et al, 1993; Martin et al,
1993). Yang et al (1996) reviewed the literature and
found only six cases of the multiple hand anomaly
(Type 5).
The embryology of the mirror hand-multiple hand
spectrum of abnormalities has a great deal of clinical
significance. The distal end of the limb bud in the
embryo has an apical ectodermal ridge (AER) which
covers a proliferating mesenchyme known as the
progress zone. The posterior border of this progress
zone is known as the zone of polarizing activity (ZPA).
Fibroblast growth factor type 4 (FGF 4) regulates the
AER which in turn controls limb growth in a proximal
to distal fashion. However, the Sonic hedgehog (Shh)
gene regulates the ZPA which is responsible for limb
patterning along the anterior to posterior axes. Both
536
FGF4 and the Shh gene act through feedback loops
and further integrate limb growth by initiating expres-
sion of secondary signalling molecules such as homeo-
box genes and bone morphogenic proteins (Erlebacher
et al, 1995; Laufer et al, 1994). Transplantation of the
ZPA to the anterior margin of a limb bud in the chick
results in mirror image duplication of limb elements
(Tabin, 1991). Similarly, mirror image duplication in
the forelimb of the mouse has been observed with
ectopic expression of Hox b-8 (Charit6 et al, 1994).
Types 1 to 3 of the mirror hand anomaly in humans
may be due to either aberrant positioning of the ZPA or
altered inductive interactions of the Shh gene resulting in
impairment of the mechanism responsible for the
organization and differentiation of the limb bud, rather
than a primary duplication event. In other words, the
primary event results in failure of the radial ray to
develop. The ulnar area will then induce the differentia-
tion of two groups of ulnar rays. Timing of this insult
could play a critical role in determining the type of this
rare from of duplication (Types 1 to 3, Table 1).
Syndromal mirror hand (Type 4) is probably due to a
gene mutation of the Shh gene (Hersh et al, 1995).
Finally, the multiple hand deformity (Type 5) may be
explained by true duplication of the ZPA.
Acknowledgement
The authors would like to thank Professor A. Gilbert for his comments when this
case was presented to him.
T H E J O U R N A L OF H A N D SURGERY VOL. 23B No. 4 A U G U S T 1998
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Received: 17 December 1997
Accepted after revision:18 February 1998
M. M. A1-Qattan FRCSC, PO Box 18097,Riyadh 11415,Saudi Arabia.
© 1998The British Society for Surgery of the Hand