Foot Ankle Clin N Am

9 (2004) 105 125

Muller-Weiss disease: clinical and

biomechanical features
Ernesto Maceira, MDa,b,*, Ramon Rochera, MDc
a
Hospital Beata Mara Ana de Jesus, calle Doctor Esquerdo 83, 28007 Madrid, Spain
b
Department of Human Anatomy and Embryology (I), School of Medicine,
Universidad Complutense de Madrid, Ciudad Universitaria s/n, 28040 Madrid, Spain
c
Clnica Teknon, Consultorios Vilana-Desp. 122, c. Vilana 12, 08022 Barcelona, Spain

The so-called Muller-Weiss disease (MWD) was first written about during
the early twentieth century in Europe. In 1927, Walther Muller [1], a surgeon at
Leipzig, reported on a patient who had gross deformation of both tarsal navicular
bones which appeared compressed, condensed, and fragmented. Konrad Weiss [2],
an Austrian radiologist who was working with Robert Kiembock, described similar
findings in two patients. Subsequently, the disease was named after them, although
Schmidt, in 1925, reported on a patient who had pluriglandular endocrine failure
and presented similar deformities at the tarsal navicular and was the first author
who reported on the disease [1]. Muller believed that it was a congenital defect,
whereas Weiss recalled radiographic similarities of the condition with the recently-
described necrosis of the lunate by his master Kienbock. Weiss point of view was
supported by several investigators, particularly radiologists, who assumed that
the deformity was the consequence of an osteonecrotic process suffered by
the navicular (malazie des os naviculare pedis) [2]. From that point, controversy
persisted around the cause and pathogenesis of the condition; several theories, often
overlapping, have been proposed, including primary osteonecrosis [3 13], osteo-
chondritis [14 16], necrosis of traumatic or biomechanical origin [5,17 20],
plastic deformation of an otherwise normal bone as a result of increased burden [3],
congenital malformation [1,21 24], osteoarthritis (OA) on a dysplasic navicular
[25], abnormal evolution of Kohlers disease (KD) [1,18,20,26], trauma [14,27],
and even a normal variant [28] or migration of an accessory cuboid [22,23].

* Corresponding author. Department of Human Anatomy and Embryology (I), School of

Medicine, Universidad Complutense de Madrid, Ciudad Universitaria s/n, 28040 Madrid, Spain.
E-mail address: emaceira@med.ucm.es (E. Maceira).

1083-7515/04/$ see front matter D 2004 Elsevier Inc. All rights reserved.
doi:10.1016/S1083-7515(03)00153-0
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Osteochondrosis of the navicular in childhood was already known for its

spontaneous favorable evolution which led to complete healing without sequelae;
its clinical relevance was the differential diagnosis from other prevalent diseases
that may alter the radiographic appearance of the tarsal navicular, including lues,
tuberculosis, and osteomyelitis [29,30].
Brailsford [14,27] considered trauma to play an important role in the gene-
sis of the disease and termed it listhesis navicularis because of the squeezing-
like displacement that the fragments suffer after splitting. He pointed out the
high number of patients he attended (his series included 5 patients in 1935, 17 in
1945, and 20 in 1953), its predominance among females (88%), and its bilat-
eral, but asymmetric, character. He stated that the disease probably was not
recognized in many occasions, and, therefore, was more prevalent than realized.
While studying a collection of 80 dry tarsal naviculars we found one specimen
that showed marked compression at its lateral half and an incomplete line of

Fig. 1. Dry specimen of tarsal navicular bone showing compression at the lateral half and incomplete
fragmentation on the sagittal plane. (A) posterior view, (B) anterior view, (C) plantar oblique view,
(D) plantar view, and (E) plantar oblique view. The arrows indicate the marginal points of
fragmentation. (From the collection of the Department of Human Anatomy and Embryology
(I), School of Medicine, Universidad Complutense de Madrid.)
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fragmentation on the sagittal plane (Fig. 1), but no degenerative changes on the
articular surfaces.
Simons [3], reported on a 40-year-old patient who had unilateral affection
of osteopathia deformans of the tarsal navicular; he included the radiograph of
the sound foot which showed mild dysplasia of the bone. He disagreed with Muller,
who believed that the deformity could be congenital in origin or due to altered
ossification and proposed that it was acquired as a result of increased burden, with
possible primary or secondary necrosis. Frosch [4] understood the disease as the
consequence of a pathologic fracture that took place on a necrotic bone.
Volk [21] believed that the deformity was due to abnormal ossification from two
different centers. One of the patients he described was a 22-year-old locksmith who
underwent orthopedic treatment including casting and the use of insoles, for some
kind of congenital deformity at his feet since he was a child, during World War I.
The rearfoot was fixed in varus.
In 1941, the Danish radiologist Erik de Fine Licht [23] reported on four patients
who had bipartite os naviculare pedis that he studied between 1935 and 1940.
Two patients had bilateral affection, another one had unilateral asymmetrical
deformities and the fourth patient had bilateral asymmetrical deformities. He
pointed out that the lateral fragment of the bone can appear as a diffuse shadow
which results in difficulty in identification. The talar head was widened in some
cases. The navicular on the dorsoplantar view had a comma-shaped or hourglass
appearance with increased density. OA changes were common around the bone,
especially on its talar side. The most severe cases, beside the fragmentation of the
bone into two portions, showed rotation with elevation of the lateral piece and
lowering of the medial one. He was surprised at the different functional impact that
the deformity meant for the patients; it could be a crippling condition or nearly
asymptomatic. Actually, one of his patients complained of pain at her knee, not at
the deformed foot.
To our knowledge, it was Fontaine et al in 1948 [17] who coined the term adult
tarsal scaphoiditis. This is the preferred noneponymic term in western Europe
because the accessory navicular (os tibiale externum) frequently is referred to as
bipartite navicular in those countries. Chambers [31] considered the bipartite
tarsal navicular as a congenital malformation of the bone and stressed the differ-
ences between this condition and the accessory navicular. In Mediterranean
countries the term bipartite navicular is sometimes used to describe the accessory
navicular which is actually a supernumary bone beside the navicular tuberosity:
the os tibiale exterum. In English literature the term bipartite navicular refers
exclusively to a bipartition through the body of the bone.
In 1981, Willey and Brown [24] reported on six patients, the largest North
American series to our knowledge, who were studied at Ottawa. They believed that
the bipartition arose from heterogeneous ossification centers and recognized
osteonecrosis of the navicular as being a different disease.
The foot of the living mammalian developed from the elements of the
primitive tetrapod foot; the navicular is formed by the distal tibial and fibular
centrale of the second and third rays [32]. This is why several investiga-
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tors considered the bipartite navicular to be a normal variant of atavistic nature

and why the variant exists apart from the pathologic bipartite navicular [22,
26,32].

Epidemiology
This article is based on the study of 191 cases (101 patients) that were collected
from six centers in Spain as part of the research for the doctoral thesis of one of us
(EM). When studying the large series at the Hospital de San Rafael at Barcelona,
Spain (73 patients), two outstanding facts were drawn from the filiation data of
the patients: (1) most of them were born outside the province of Barcelona and [2]
there was an uneven distribution when the dates of birth were displayed (Fig. 2).
Only 11 patients were born in Barcelona; 85% of the patients who had MWD had
moved from rural areas of Extremadura, inner Andaluca, and Castilla-la-Mancha,
regions which in the 1950s suffered a depopulation with massive migrations to
Barcelona, Madrid, and the coastal provinces [33]. Seventy percent of the control
group of patients who attended the hospital at the same time but for different
reasons were born in Barcelona. Most of the patients who had MWD were
housekeepers or manual workers who lacked professional qualification; 73% were
women. This means that the vast majority were immigrants whose families had
moved to Barcelona seeking prosperity. Traditional agriculture in Spain disap-
peared in the early second half of the twentieth century; crop failures were
followed by progressive mechanization of agricultural techniques that required
fewer workers [33]. The Spanish Civil War (1936 1939) was followed by a harsh
postwar period of poverty and international isolation; the main social and
nutritional stress cycle is recorded in the patients birth date distribution. A
sudden onset of cases of MWD occurred in the late 1920s with the maximum
annual incidence in 1932. The children were supposed to undergo ossification of
the tarsal navicular under the most stressing of environmental conditions. A
second, smaller burst of MWD took place in the middle of the century, just before
the massive migratory movements.
Several investigators noted this epidemic quality. Muller [1,34] was surprised to
attend a second patient only 1 year after seeing his first; both were born just before
World War I. Brailsford [27] reported on 20 patients and postulated that MWD was
not a well-recognized disease. de Fine Licht [23] studied four patients in 5 years
and collected 10 patients who were reported by several investigators in central
Europe. The rarity of MWD in the United States can be explained by the absence of
environmental predisposing factors, whereas Europe accounts for the vast majority
of the literature on MWD.
Because the final fate of MWD is perinavicular OA several cases will be
considered as a degenerative joint disease unless the eventual splitting or a major
deformity of the navicular bone is noticeable [27].
Apart from the clusters of cases, there are scattered patients in whom no
environmental precipitating factors can be found but who suffered some kind of
 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125
Fig. 2. Distribution of patients who had MWD by date of birth (number of patients born in 5-year periods). The highest peak took place around 1932, four years before
the Spanish Civil War (1936 1939).

109
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condition that affected the growth of the whole individual or the tarsal navicular
alone. Most of the recent articles on MWD are single case reports.

Etiopathogenesis
There is a definite prerequisite for the disease to take placea delay in the
ossification of the tarsal navicular. Its chondral structure will not be sufficient in
the coming years to cope with the large compression stresses that the keystone of
the plantar vault must support. The delay in the ossification may take place as a
generalized or localized developmental disturbance.
Generalized developmental disorders that may temporarily slow or even stop
the ossification process of the child include nutritional defects of extrinsic (low
socioeconomic status, poverty, war) or intrinsic nature, pluriglandular failure endo-
crinopathies, and general diseases that imply consumptive conditions [35 37].
Anthropologists believe that Harris lines, dental enamel hypoplasia, and cribra
orbitalia represent episodes of nutritional stress among children in ancient
populations [38 40]; the first two findings are common in patients who have
MWD. Children who experience nutritional stress also may show reduced
thickness of cortical bone [37,39,41]; a common finding in MWD is hypoplasia
of the metatarsal bones, except the second one which usually shows some degree of
hypertrophy and did not appear shorter than the first one in our series. Dental
development is much less affected by environmental stress than bone growth [39].
Dental pathology has not been looked for in our historical records, but several of the
patients that are currently being followed have enamel hypoplasia and related
common dental diseases, mainly caries, that eventually produced teeth loss around
their third decade. Harris lines represent episodes of bone growth arrest and can be
reproduced experimentally by infection, starvation, and other less severe nutri-
tional stresses [37 40]. There is no one-to-one correspondence between each insult
and line formation. Furthermore, the lines can disappear by bone remodeling and
may appear in normal subjects with no episode of stress. Following a period of
growth arrest, the growth will return to its original trajectory if sufficient resources
are available and resume at an accelerated rate; this phenomenon is referred to as
canalization or spurt of catch-up growth [39]. If recovery from the stress episode
that caused the growth arrest is insufficient to sustain a period of catch-up growth,
the growth will resume at a normal or slowed rate so that the child does not return to
his/her original, genetically-designed, growth trajectory. Final adult size may not
be affected because skeletal maturity often is delayed under such circumstances to
allow for an extended growing period; however, if the chondral anlage of the tarsal
navicular bone has suffered a plastic deformation, its ossification will reproduce the
abnormal shape that was generated during an extended period of time when the
resilience of the navicular was too low to cope with the high compressive stress that
is established at the plantar vault keystone [32].
Localized delayed ossification of the tarsal navicular can take place in meta-
tarsus adductus and clubbed feet [42 44]. In the discussion of Kidner and Muros
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article [30] on KD, Lowman pointed out the high incidence of growth deviations
that he found in the tarsal navicular of club foot. One of us (RR) found delayed
ossification of the navicular in otherwise healthy children who had varying degree
of forefoot adduction [45]. Under these circumstances, the tarsal navicular may
undergo plastic deformation as well, but no stigmata of generalized growth dis-
turbance appears.
Anderson and coworkers [46] stated the consequences of growth inhibition of
the foot are not known, but the series they studied (including healthy children
from Boston) showed larger sizes than those collected by Davenport [47]
(children from Brooklyn Orphanage) and Meredith (Iowa children from rural
areas and European ancestries) [48].
Delayed ossification of the tarsal navicular is mandatory for MWD to take place,
but an abnormal force distribution pattern is necessary as well to produce the
compression of the lateral half of the bone. If the compressive stress is homoge-
neously distributed along the entire navicular, the chondral anlage would probably

Fig. 3. Dorsoplantar weight-bearing (w-b) view of four patients (A D). The metatarsals are usually
parallel to each other; their divergence angle is reduced, particularly between the first and second
metatarsals. The first metatarsal did not appear longer than the second metatarsal in our series; M1 is
usually shorter. The whole medial column may be shortened as well because of internal rotation of the
navicular (both feet at C, right foot at D). The second metatarsal is frequently hypertrophied.
112 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125

cope with it and no residual deformity would result except for an eventual harmonic
reduction in its anteroposterior width. This is most likely to occur in KD, which is
well-known as a self-limited benign process. Severing the vascular supply of the
navicular in rats is not enough to produce permanent changes in the bone [49].
Conversely, uneven distribution of compressive stress on the navicular with in-
creased solicitation at its lateral half, can make the lateral half of the bone give
way; shearing forces may appear between the talar head and the lateral cuneiforms
that lead to increased deformity and eventual fragmentation of the cartilaginous
navicular. The lateral displacement of the talar head implies a fixed rearfoot varus
which is a constant feature in MWD. Biomechanical conditions that lead to lateral
displacement of the net compressive forces on the navicular include primary
subtalar joint varus, first ray brachymetatarsia (congenital or acquired), and
compound conditions, such as mild or misdiagnosed forms of clubfoot. A short
first metatarsal may fail to assume compressive stress during the third rocker of the
gait cycle, thus resulting in force transfer through the second ray and increased
strain at the lateral half of the anlage. Owing to Delpechs laws, overgrowth of the
medial half and undergrowth of the lateral half will occur. This, in turn, may
account for secondary lateral shift of the talar head that results in rearfoot varus.
None of our cases exhibited an index plus metatarsal formula and most had a short
first ray, either because of a short first metatarsal itself or as the result of a relative
shortening of the whole medial column derived from internal rotation of the
navicular on the transverse plane and a relative retroposition of the first cuneome-
tatarsal joint with respect to the second one (Fig. 3). Mau [50] and Vilaseca and
Casademunt [18] noticed that the first metatarsal bone was frequently shorter than
the second one; Lafontaine et al [19] described a case that was associated with
hypermobility of the first ray. Both conditions may increase force transfer through
the second ray during the third rocker. de Retana (personal communication, 2003)
studied a 4 year-old-girl who developed MWD in one foot after a shortening of
the first metatarsal that was due to traumatic epiphysiodesis of its base.

Pathologic anatomy
The talar head is received into a deep socket or acetabulum pedis, whose bony
facies lunata, formed by the posterior articular facet of the navicular and the anterior
and middle facets of the calcaneum, is completed with several soft-tissue structures
that include the spring ligament, the superomedial calcaneonavicular ligament
(ligamentum neglectum) carpeted by the so-called fibrocartilago navicularis,
and the navicular component of the bifurcate ligament. The flexibility of the
acetabulum pedis permits the adaptability in form and size of the containing socket
as necessitated by the relative displacements of the talar head, calcaneus, and
navicular [32,51].
Any displacement of the talar head toward the medial side of the coxa pedis
implies subtalar valgus. Conversely, lateralization of the head of the talus defines
subtalar varus. Any incompetence of the acetabulum pedis at its lateral end will
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result in rearfoot varus. MWD is the clinical expression of an acetabular protrusion

at the lateral portion of the acetabulum pedis. Three-dimensional reconstructions
from serial CT-scan images that were performed with Surfdriver software
(www.surfdriver.com) (Fig. 4) show flattening of the lateral half of the posterior
surface of the navicular which allows the talar head to protrude laterally, thus
inducing subtalar varus. A cleft can appear on the sagittal plane that divides the
navicular into a major medial portion and a lateral or dorsolateral smaller fragment;
in the most severe cases the navicular looks like a squeezed plastic material and the
gap between both fragments is large enough to expose the second or third
cuneiforms which then are part of the acetabular wall. Equinization of the rearfoot
takes place if enough space is available for the talar head to plantarflex, despite the
fact that calcaneus remains in an inverted position and the plantar soft-tissues are
not stretched; this produces a paradoxical pes planus varus (Fig. 5).
An immature (chondral) tarsal navicular will cope with compression stress as
long as the stress is distributed evenly along its entire articular surface, even if its
ossification is delayed (navicularis pedis retardatum) [30]. This situation may
account for the genesis of KD. Scaglietti et al [52] found reduced scaphoid space
index (ratio of the distances of the calcaneum-first cuneiform and calcaneus-
astragalus) in children who were affected by KD, mainly in the younger age groups
(3 to 4 years of age). Any mechanical impairment that produces lateral displace-

Fig. 4. (A to H) Three-dimensional reconstructions (Surfdriver software) that show increasing

deformity at the navicular. The tibia and astragalus have been removed to view the acetabulum pedis
from a dorsoposterior position. In the most severe cases, the wall of the acetabulum is formed by the
lateral cuneiforms. (I) Compression and bipartition of the lateral portion of the bone rendered as a
wire-frame (contour).
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Fig. 5. (A) Three-dimensional reconstruction of the right foot showing the navicular prominence; the
talus has been removed. (B ,C, D) Clinical appearance. At a glance, both feet may appear as planovalgus
but the medial prominence (n) result from the navicular alone, the legs are externally rotated, and the talar
head lies on the middle of the foot, pointing laterally. (B) The heels (h) are visible on the medial side of
the feet when viewed from the front. (C) Rearfoot varus. (D) Podoscopic examination. (From Maceira E.
Aspectos clnicos y biomecanicos de la enfermedad de Muller-Weiss. Revista de Medicina y Ciruga del
Pie 1996;10(1). p. 57; with permission.)

ment of the net compression stress on an immature navicular during a prolonged

period of time will result in plastic deformation of the chondral anlage because
shearing forces appear between the lateral cuneiforms and the talar head. Primary
subtalar varus and lack of force transmission through the first ray during the third
rocker of the gait cycle that is caused by brachymetatarsia can produce lateral
transfer of the net compressive stress through the acetabulum pedis.
Microscopic examination reveals normal bone except for degenerative OA
changes. Several investigators performed histopathologic studies of the bone but
failed to find evidence of osteonecrosis [20,22,26,34,50].

Clinical findings
Most of the patients who have MWD seek attention for long-standing mechan-
ical pain on the dorsum of both feet, usually in an asymmetrical fashion [23,53].
Minor trauma (10% of the patients sufferred some kind of minor trauma) can
precipitate the onset of symptomatic worsening, although this can occur sponta-
neously around the fifth decade. The deformity can be well-tolerated for years or
remain mostly asymptomatic; in others it is a crippling condition by the second or
third decades [23]. Women represent 73% of the patients in our series. The mean
age at diagnosis was 47.6 years (maximum 91 years, minimum 13 years, SD 14.4
years) but long period of ill-defined mechanical pain is frequently recalled.
One of the patients who was studied by de Fine Licht [23] had pain at her knee;
the foot deformity was a secondary complaint. Knee pain is common in these
patients, although our retrospective research did not allow us to estimate its inci-
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dence. Several patients underwent some kind of surgery at their knees for degen-
erative OA, including total replacement. We believe knee osteoarthritis is second-
ary to impaired mechanics of the whole limb rather than a primary dysplasia at the
knee [54,55].
Clinical examination can show a normal, high-, or low-arched foot with rearfoot
varus. Heel varus can be subtle and only appreciable by palpation. Furthermore,
because the navicular tuberosity is prominent at the medial side of the midfoot,
simple inspection may give a false impression of rearfoot valgus. The talar head is
not at the medial side of the foot (as in subtalar valgus) but is at the midline of the
dorsum of the midfoot and pointing laterally (see Fig. 5). The tibia is externally
rotated and the lateral malleolus is in increased retroposition. Subtalar motion is
reduced and may reproduce the crackling sensation that some patients feel when
walking. The patients who show flatfoot still have fixed rearfoot varus (the
paradoxical pes planus varus) [56]. The heel seems to be prominent posteriorly
because the relative position of the tibiotarsal joint is advanced because of rearfoot
equinization. Another prominence at the plantar aspect of the middle of the foreheel
can be palpated in correspondence with the calcaneocuboid joint.
The toes are usually parallel to each other and there is a strikingly low incidence
of hallux valgus; prominent bunions with no metatarsophalangeal deformity have
seldom appeared and real abduction deformities at the first metatarsophalangeal
joint (MPJ) are rare (see Fig. 3).
Dental pathology should be studied because it may present in the form of caries
and enamel hypoplasia with eventually severe dental loss in the third decade.
Careful anamnesis should be performed for possible environmental (socio-
economic, orphans) or personal factors (endocrinopathies, systemic diseases, post-
natal foot deformities) that impair normal growth and development during infancy.

Radiology
Arandes and Viladot [51] stated for KD, that clinical findings are nothing,
radiology is everything. Regarding MWD, clinical findings may be helpful but
plain weight-bearing radiographs of the feet are the basic tool for diagnosis. Other
imaging procedures may be useful for differential diagnosis including nuclear
magnetic resonance (NMR), three-dimensional reconstruction (CT scan), and low-
grade deformities (techneticum (Tc) scan), but are not usually essential.
The most striking features take place at the navicular which draws attention to
the bone (Fig. 6). This is misleading finding for two reasons: (1) the radiologic
appearance of the navicular may mimic osteonecrosis, stress fractures, and OA; and
(2) there is plenty of information in the rest of the foot skeleton that is arranged in a
peculiar fashion; the observer may not pay attention to it unless several cases are
studied. Radiology provides us with valuable information to understand the
pathogenetic mechanisms and altered biomechanics of the disease. For didactic
reasons, the findings are grouped into alterations that take place in the rearfoot and
ankle, midfoot, and forefoot.
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Fig. 6. Graphic representation of increasing grades of deformity at the navicular. Detailed w-b radio-
graphs. The talar head is always moved laterally to a certain degree, laying over, but not beside, the
anterior process of the calcaneus. It may appear widened as well.

Alterations about the ankle and hindfoot

All patients who have MWD show rearfoot varus to some degree. We consider
rearfoot varus a prerequisite for MWD diagnosis. Many of the radiologic features
that we are describe are classic markers for subtalar joint inversion (Fig. 7).
On the lateral view there is a decreased talocalcaneal divergence angle; the
astragalus and calcaneus tend to parallel each other. The orthogonal projection of
the tarsal channel makes it visible as a black hole between both bones which gives
the impression of a wide open sinus tarsi. The cyma line represents an elongated
italic S because there is little, if any, overlapping between the talar head and the
anterior process of the calcaneus; the former is over and not next to the latter. There
is an increased retroposition of the fibula with respect to the tibia; this is the
hallmark of external rotation at the ankle. The soft-tissue shadow at the rear part of
the heel may protrude posteriorly when there is marked equinization of the rearfoot
with relative advancement of the transverse axis of the ankle with respect to the rest
of the foot. This view is appropriate to look for Harris lines.
The dorsoplantar projection shows decreased talocalcaneal divergence as well
(Kyte). The talar head is moved laterally and is more or less located over the
anterior process of the calcaneus instead of being at its medial side. As a
consequence, the cyma line appears in this view as a short S with an abrupt
middle inflexion. Several patients seem to have a wide talar head because of
degenerative changes and altered rotation on the coronal plane (see Figs. 6, 7).
 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125 117

Fig. 7. Typical appearance of a severe case of MWD. Positive cuboid sign (arrow).

Alterations about the midfoot

The lateral view shows reduction in the posteroanterior width of the navicular in
variable degreesfrom hardly perceptible to severe compression and extrusion
with direct talocuneiform joint (Figs. 8, 9). The talar head may make contact with
the lateral cuneiforms in the worse cases, when the navicular is completely
squeezed. A real or apparent splitting of the bone may appear that follows a
dorsodistal to plantarproximal direction. In our series, splitting was present in one
half of the cases. The navicular may be condensed, although sometimes it is an
impression because of the superposition of the navicular and the talar head. De-
generative OA at the perinavicular joints is a common finding; it is probably the
cause of pain and seems to be the final fate of the disease in most instances.
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Fig. 8. Schematic representation of the five stages of increasing deformity on the sagittal plane. Stage 1
shows minimal changes, whereas stage 5 is defined by talocuneiform articulation. Stages 2 to 4 show
dorsal, neutral, or plantar intersection of Meary-Tomeno lines. Orthogonal projection of the subtalar
joint; the talar head always lies over the anterior process of the calcaneus. (From Maceira E. Aspectos
clnicos y biomecanicos de la enfermedad de Muller-Weiss. Revista de Medicina y Ciruga del Pie
1996;10(1). p. 58; with permission.)

The dorsoplantar view shows the navicular deformity at its best (see Fig. 6). The
bone is compressed at its lateral half in a comma or hourglass shape with eventual
fragmentation on the sagittal plane at the level of the second and third cuneiforms.
When complete fragmentation takes place, the smaller, outer portion may be clearly
seen or appear as a subtle shadow that is difficult to distinguish from the underlying
cuboid. There is internal rotation of the medial fragment of the navicular that
produces relative shortening of the medial column of the foot (see Fig. 3). At the
 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125 119

Fig. 9. Lateral w-b radiographs of feet at increasing stages. Most stage 1 cases are the less affected
foot of patients who have asymmetrical deformity (positive Tc scan). The arrow points to a bright-
ened area including Harris lines. The tarsal channel is represented by a round shadow and the subtalar
joint is clearly depicted. Even in stage 5, with complete extrusion of the navicular bone and marked
equinization of the rearfoot, the talar head is not superimposed on the calcaneus: the paradoxical pes
planus varus. The lines in 2, 3 and 4 represent the talar inclination axis.
120 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125

lateral margin of Choparts joint, several cases present with medial subluxation of
the cuboid with respect to the calcaneus that resembles the cuboid sign that was
described for club foot (see Fig. 7) [57].

Alterations about the forefoot

These can be appreciated on the dorsoplantar view. The metatarsals are usually
strikingly parallel to each other (particularly the first and second bones) with
reduced divergence of the first and fifth ones (see Fig. 3). There is a low incidence
of hallux valgus and the sesamoids tend to display a too pretty position relative
to the metatarsal head. A few patients complain of prominent bunions that did not
show articular malalignment, but increased abduction at the phalanx itself
(increased distal articular set angle (DASA)). Patients who underwent first ray
surgery had poor results (overcorrection). In most instances, the second metatarsal
bone is hypertrophied and is not shorter than the first one [14,20,24,50]; radio-
graphs reveal there is some kind of force bypass that makes the first ray inefficient
and the second cuneometatarsal beam the main force transmitter. The first
metatarsal bone sometimes is short, but eventually may have a relative shortening
with respect to the second one as a result of retropositioning of the first
cuneometatarsal joint because of internal rotation of the main medial portion of
the navicular. Although the alignment of Lisfrancs joints on the transverse plane
may depend on the radiological projection, a common pattern in their arrangement
can be recognized in many patients (see Fig. 7). The first cuneometatarsal joint
usually lies on a plane perpendicular to the axis of the foot, as defined by the second
metatarsal shaft. Most of the normal feet have it oriented medially. Conversely, the
second cuneometatarsal joint is frequently externally rotated, whereas in normal
feet the first cuneometatarsal joint usually lays on the perpendicular to the
longitudinal axis of the foot. Another common finding is the presence of an
arthrodial joint between the base of the first and second metatarsals. The wide base
of the first metatarsal and the perpendicular disposition of its proximal articular
surface with respect to its long axis resemble an elephants foot (see Fig. 7). The
fourth metatarsal may appear atrophic; sometimes the first, third, and fifth are
atrophic as well (Fig. 10). The trophic status of the metatarsals is often noticeable
because the second one is usually hypertrophied (see Figs. 3, 10) [18,27].
The overall arrangement of the forefoot bones and joints leads one to consider
what could have happened to an adducted forefoot if there was some kind of force
acting from its inner part at the level of the first metatarsal head toward the lateral
side that coexisted with a weak navicular (see Fig. 7). If the relative lengths of the
metatarsals followed an index plus-minus formula in a metatarsus adductus foot, an
abducting force that acted on the first metatarsal bone would produce: (1) abduction
of the whole metatarsal palette, making the bones parallel to each other; (2) friction
at the base of the first metatarsal against the second one, with the development of an
arthrodial joint; (3) reorientation (external rotation) of the cuneometatarsal joints;
and (4) a relative shortening of the first metatarsal with respect to the second one. A
plastic navicular will fail between the second cuneiform and the talar head and
 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125 121

Fig. 10. Stage 3 MWD (same patient as in Fig. 8.1). Short first metatarsal with hypertrophic second
metatarsal and relative hypotrophy of the rest. Compression of the lateral half of the navicular with lateral
protrusion of the talar head which lies over the calcaneus. Positive cuboid sign. Retroposition of the
fibula with respect to the tibia indicates external rotation at the ankle. Wide open sinus tarsi. The arrow
points to a brightened area showing Harris lines.

allow for the talar head to protrude laterally and distally to a variable extent. The
cuboid may be subluxed medially with respect to the calcaneus.
Conversely, it seems plausible that primary subtalar varus will increase load
transfer along the outer part of the navicular which, if not resilient, would allow for
the talar head to produce an acetabular protrusion through the lateral portion of the
coxa pedis and transfer the load at the forefoot away from the first metatarsal to the
neighboring metatarsals.
Untreated metatarsus adductus may evolve to serpentine or skew foot if
rearfoot valgus is maintained. Metatarsus adductus that coexists with subtalar
inversion is, at least, a mild form of club foot. The condition can be overlooked if it
is mild. The mechanical disadvantages of this type of feet, together with their
tendency to show retardation in the ossification of the navicular, may account for
the pathogenetic mechanisms of certain patients who have MWD (mainly the
nonepidemic cases).

Degree of deformity
Depending on the appearance of the navicular on the lateral weight-bearing
radiograph and the orientation of Meary-Tomenos (M-T) angle (intersection of the
122 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125

talar and first metatarsal axes), the patients can be grouped according to the degree
of the deformity into five stages (see Figs. 8, 9).
Stage 1 (19%). Normal radiographs with no or minimal changes; positive
technetium scan, CT scan, and MRI (intraosseous edema) [58]. Most of these cases
are the less affected sides in asymmetric bilateral affection. Stigmata of subtalar
varus are subtle but present.
Stage 2 (9%). Subtalar varus that is due to lateral displacement of the talar head
which lies over the anterior process of the calcaneus and not beside it. Initial
cavovarus results, with dorsal angulation of M-T lines. The talar head appears to be
dorsally subluxed. All subsequent stages will also show the stigmata of subtalar
inversion: orthogonal projection of the whole subtalar joint, holelike appearance of
the tarsal channel and sinus tarsi, and reduced area of superposition of the talar head
and the anterior process of the calcaneus.
Stage 3 (46%). As compression or splitting proceed, lowering of the longi-
tudinal arch occurs. The space between the talar head and the cuneiforms is reduced
markedly. Despite persistent rearfoot varus, the arch structure begins to fail
although M-T alignment is neutral.
Stage 4 (19%). With further navicular compression, rearfoot equinization takes
place. Unlike plano valgus foot, in which the main failure takes place at the truss
mechanism, the paradoxical plano varus foot is produced by failure in the beam
component [32]. M-T lines intersect plantarly and reveal longitudinal arch
lowering. The presence of rearfoot varus stigmata is noticeable within the low-
arched foot (see Fig. 7).
Stage 5 (7%). The existence of a talocuneiform joint with complete extrusion of
the navicular. Brailsford [27] termed the disease listhesis navicularis which
described the displacement of the two fragments of the bone. The talonavicular
joint often is seen on osteoarthritic flat feet but also may appear in cases of
surprisingly well-preserved arches.
The transition among increasing degrees of deformity is not seen in patients
during long-term follow-up. Osteoarthritic changes, and, eventually, compression
may seem to progress but we have seen no case in which a nonsplit navicular
developed splitting. We postulate that the five stages may develop progressively
during infancy as long as the navicular bone is still chondral; because it lacks
innervation no pain will appear, as in Perthes disease. After ossification is
reassumed and the mechanical properties of the keystone are appropriate, the
deformity will stop its progression, although the morphologic and mechanical im-
pairment remain forever. Finally, the staging does not correspond necessarily with
increased crippling situations (eg, a stage 1 foot may be painful whereas stage 4 or 5
cases can be well-tolerated) but it is valuable tool for descriptive purposes.

Differential diagnosis
Diseases of the navicular that can mimic MWD include osteonecrosis, osteo-
chondritis dissecans, OA of different origin, neuropathic arthropathy, stress
 E. Maceira, R. Rochera / Foot Ankle Clin N Am 9 (2004) 105125 123

fractures, evolved fractures, and miscellaneous conditions (eg, Pagets dis-

ease, osteomyelitis).
Osteonecrosis should be confirmed microscopically by the finding of empty
lacunae in specimens that can be obtained with puncture biopsy. The procedure
may function as core decompression as well. Osteochondritis dissecans does not
reproduce the deformity and affects only the talar side of the bone [11].
Viladot et al [25] estimated that 2.5% of the normal population shows dyspla-
sic naviculars (mild compression, indentation at the dorsal distal margin of the
bone). When treating those patients for different pathologies at their lower limb,
the possibility of impaired mechanics at the peritalar complex must be taken
into account.

Acknowledgments
This work would not have been possible without the teaching and assistance
of Antonio Viladot and Javier Puerta. The authors wish to thank Ramon Viladot,
Director of the Department of Orthopaedic Surgery (B) of the Hospital de San
Rafael at Barcelona, for his scientific contribution and facilitation of the research.
The following people contributed with their cases: J.C. Gonzalez-Casanova, MD;
V. Leal, MD; A. Viladot Jr, MD; F. Alvarez-Goenaga; M. Pons, MD; P. Fernandez
de Retana, MD; M. Gonzalez-Rodrguez, MD; A. Borras, MD; M. Fernandez-
Berrendero, MD; A. Orejana, DP; J.M. Ropa, DP; Javier Puerta MD, PhD; and
J. Pascual, DP.
Our thanks to the Hermanas Hospitalarias del Sagrado Corazon de Jesus
Congregation, for their co-operation at both Hospitals: San Rafael at Barcelona and
Beata Mara Ana de Jesus in Madrid.

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