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〈 Case Report 〉
Bilateral Osteonecrosis of
the Navicular and Medial
Cuneiform in a Patient
With Systemic Lupus
Erythematosus
Robert M. Greenhagen, DPM,
Brandon E. Crim, DPM,
Andrew B. Shinabarger, DPM, and
Patrick R. Burns, DPM
A Case Report
Abstract: Introduction. Atraumatic
avascular necrosis (AVN) is an unusual
pathology to the foot. Risk factors include
the use of corticosteroids, smoking, alcohol, rheumatologic disorders, hematologic disorders, and metabolic disorders.
To the authors’ knowledge, this study
presents the first case of bilateral atraumatic AVN to the navicular and medial
cuneiform in a patient with systemic
lupus erythamatosus (SLE). Case description. A 40-year-old man presented
with a past medical history of SLE in
which he developed AVN of the tarsal navicular and medial cuneiform.
This occurred first on the subject’s right
foot and then while recovering from
surgical intervention, on his left foot.
Talonaviculocuneiform arthrodesis was
performed with the use of distal tibial
autograft on both extremities. The subject’s American Orthopaedic Foot and
Ankle Society midfoot score improved
from 34 to 80 at 21 months on the right
was treated with bilateral talonaviculoand 37 to 90 at 15 months to the left.
Discussion. Patients with SLE carry a sig- cuneiform arthrodesis. The patient
nificant risk of developing AVN. Comorbidities
such as vasculitis, corA number of associated risk factors [for
ticosteroid use, cytoavascular necrosis] have been identified,
toxic medication, and
peripheral neuropathy
including corticosteroids, smoking, alcohol,
are known risk factors
in the development of
rheumatologic disorders, hematologic
AVN. Unusual features
such as multifocal AVN
disorders, and metabolic disorders.”
and unusual anatomic
locations can occur with
demonstrated considerable improvement
SLE. AVN of the foot is generally treated
to both extremities.
with surgical intervention. Treatments
such as core decompression, open reduction and internal fixation, and arthrodLevels of Evidence: Therapeutic,
esis have been recommended based
Level IV
on the symptoms and presentation.
Conclusion. The authors present a very
Keywords: arthritis and joint disrare presentation of bilateral osteonecro- ease; complex foot and ankle condisis of the tarsal navicular and first cune- tions; hereditary/genetic disorders; other;
iform in a patient with SLE. The patient
reconstructive foot and ankle surgery
“
DOI: 10.1177/1938640012439605. From the Foot and Ankle Center of Nebraska, Omaha, Nebraska (RMG) and University of Pittsburgh Medical Center, Pittsburgh,
Pennsylvania (BEC, ABS, PRB). Address correspondence to Robert M. Greenhagen, DPM, Foot and Ankle Center of Nebraska, 7337 Dodge Street, Omaha, NE 68114; e-mail:
[email protected].
For reprints and permissions queries, please visit SAGE’s Web site at http://www.sagepub.com/journalsPermissions.nav.
Copyright © 2012 The Author(s)
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vol. 5 / no. 3
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Introduction
Osteonecrosis, also referred to as avascular necrosis (AVN), is an uncommon
occurrence in the tarsal bones. The most
common sites for AVN to occur in the
foot are the talus, navicular, and first and
second metatarsals.1 Trauma generally
occurs prior to the development of AVN,2
though atraumatic AVN has been estimated to occur in approximately 25% of
talar AVN.3 A number of associated risk
factors have been identified, including
corticosteroids, smoking, alcohol, rheumatologic disorders, hematologic disorders, and metabolic disorders.2 To the
authors’ knowledge, this study presents
the first case of bilateral atraumatic AVN
to the tarsal navicular and medial cuneiform in a patient with systemic lupus
erythamatosus (SLE).
Figure 1.
Figure 3.
Lateral radiograph demonstrates
abnormal osteopenia within
the navicular. This abnormality
correlated with pain on palpation
clinically. Anteroposterior radiograph
demonstrates rectus alignment of the
right foot.
Postoperative anteroposterior
and lateral radiographs of the
right foot show a well-healed
talonaviculocuneiform arthrodesis.
Case Report
A 40-year-old man presented with the
chief concern of pain in his right midfoot for approximately 4 weeks. Patient
denied any recent trauma or change in
activities. The patient had a significant
past medical history, including SLE for 20
years, end-stage renal disease on dialysis
since 2007, rheumatoid arthritis, Sjogren
syndrome, Raynaud’s disease, cerebral
vasculitis, hypertension, and hyperlipidemia. The patient was currently taking CellCept, Plaquinil, and prednisone.
He was a previous 8 pack-year smoker
and had discontinued smoking 15 years
prior to the initial presentation. On physical examination, neurovascular status
was intact. Nonpitting edema was noted
on the right foot with pain on palpation over the navicular and medial cuneiform. Radiographs of the right foot
demonstrated rectus alignment of the
right foot with osteopenia noted within
the navicular (Figure 1). Magnetic resonance imaging demonstrated increased
signal intensity within the navicular and
medial and intermediate cuneiform on
the right, consistent with AVN (Figure 2).
The patient was treated nonoperatively
with immobilization in an offloading boot
with crutches for a period of 2 months.
After progression of pain and further
Figure 2.
Sagittal T1 image of the right foot
demonstrates decreased signal intensity
within the navicular and medial
cuneiform bones. Axial STIR (short-tau
inversion recovery) image demonstrates
increased signal intensity within the
navicular and cuneiform bones both
consistent with avascular necrosis.
bony destruction, surgical intervention
was performed. Arthrodesis of the talonavicular and naviculocuneiform joints was
performed with supplemental distal tibial autograft (Figure 3). His postoperative
course consisted of non–weight bearing
in a cast for a period of 3 months, followed by gradual transition to weight
bearing in an offloading boot.
During the postoperative course, the
patient began to experience similar midfoot pain on the left foot. Radiographs
(Figure 4) and magnetic resonance imaging (Figure 5) verified similar changes
in both his navicular and medial cuneiform on the left lower extremity. The
patient was then instructed to remain
non–weight bearing to the left lower
extremity. This conservative trial lasted 2
months at which time the patient elected
to undergo surgical correction of the left
midfoot. At approximately 6 months’ status postsurgical correction of the right
foot, a talonavicular fusion and naviculocuneiform arthrodesis with distal tibial
autograft was performed to the contralateral limb (Figure 6). The patient underwent an identical postoperative course
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Foot & Ankle Specialist
Figure 4.
Figure 6.
Lateral and anteroposterior images of
the left foot demonstrate osteopenic
changes within the navicular. Similar
to the right foot, this abnormality
correlates with pain on palpation on
physical exam. Rectus alignment of the
foot is noted on both images.
Intraoperative images demonstrate a
dorsal approach between the tibialis
anterior and extensor hallucis longus.
The incision was extended proximally
to obtain distal tibial autograft. Images
show joint preparation and the fixation
construct following graft placement.
Figure 7.
Postoperative radiographs
demonstrate a well-healed left foot
talonaviculocuneiform fusion.
Figure 5.
Sagittal STIR (short-tau inversion
recovery) image shows increased signal
intensity within the navicular and medial
cuneiform. Axial T1 image demonstrates
decreased signal intensity within the
navicular and medial cuneiform. These
findings are consistent with osteonecrosis.
15 months to the left foot, his American
Orthopaedic Foot and Ankle Society
midfoot score was 80 and 90, respectively. Figure 7 shows the postoperative
radiographs.
of non–weight bearing followed by transitional weight bearing in an offloading boot. His preoperative American
Orthopaedic Foot and Ankle Society midfoot score was 34 on the right and 37
on the left; at 21 months’ status postsurgical correction of the right foot and
Discussion
Osteonecrosis, or AVN, is defined as
the death of bone that results in collapse
of structural bony architecture, which
leads to pain, destruction, and eventual
loss of function. AVN develops because
of interruption of the vascular supply to
the bone caused by either local trauma
or a nontraumatic systemic condition.
Although the incidence of medial cuneiform and navicular AVN is unreported
in the literature, Assouline-Daylan et al2
found that the most common sites for
AVN listed in order are the hip (femoral
head), knee (femoral condyles), shoulder
(humeral head), and the ankle (talus).
The reported prevalence rate of AVN
in SLE varies widely and ranges from 2%
to 30%.4 Gladman et al5 found that the
prevalence of symptomatic osteonecrosis in SLE has been described to be close
to 12% and often involves multiple joints.
Corticosteroid therapy remains the strongest variable associated with the development of AVN in SLE patients.6 However,
the prevalence of AVN in SLE is much
higher than that seen in other clinical conditions requiring chronic corticosteroid
therapy, suggesting that SLE-related features contribute to the development of
osteonecrosis.6,7 A number of risks factors have been identified and most likely
development of AVN is a multifactoral
process.7 Sayarlioglu et al4 investigated
the predictive factors for AVN. AVN was
more prevalent in males (20.4% vs 9%, P =
.046) and subjects with and earlier onset
or diagnosis of SLE (30.4 ± 12.2 vs 25.7 ±
11.2 years, P = .016 and 31.7 ± 12.1 vs
26.4 ± 11.4 years, P = .008, respectively).4
The presence of peripheral neuropathy
(14% vs 1%, P = .001), cutaneous vasculitis (22% vs 10%, P = .027), Raynaud’s
phenomenon (51% vs 33%, P = .04), oral
ulcers (47% vs 26%, P = .01), and Sjogren’s
syndrome (12% vs 4%, P = .04) all were
found to be significant predictors of AVN.4
The use of cytoxic medications (47% vs
32%, P = 0.03) and higher doses of prednisone (29.1 ± 19.1 g vs 18.5 ± 15.1 g, P =
.001) also increased the risk of developing AVN.4 Mok et al8 reported that higher
prevalence of renal (68% vs 49%, P = .03)
and central nervous system disease (39%
vs 14%, P = .001) in patients with AVN.
All of these risk factors were present in
our patient, making him a very high-risk
patient for the development of AVN.
The association of AVN and oral corticosteroids was first established by Arfi
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Foot & Ankle Specialist
Table 1.
Stage one shows minimal radiographic
changes, whereas stage five is
defined by talocuneiform articulation.
Stages two through four have notable
radiographic navicular damage
therefore differentiation determined
by evaluation of Meary’s angle. A
dorsal, neutral, or plantar orientation
represents a stage two, three and four
respectively. (Reprinted from Maceira E.
Aspectos clınicos y biomecánicos de la
enfermedad de Müller-Weiss. Revista de
Medicina y Cirugıa del Pie 1996;10(1).
p. 58. with permission)
et al9 in 1975. Overall steroid use is considered to be the second most common
cause of osteonecrosis with a prevalence
ranging from 3% to 38%.2 Patients treated
with prolonged high doses of corticosteroid appear to be at the greatest risk for
developing osteonecrosis, although these
patients frequently have multiple risk factors. Corticosteroids cause osteoporosis
by reducing bone formation and increasing bone resorption. Although this bone
weakening does not directly cause AVN,
it does place bone in a compromised
state when trauma or vascular insult is
encountered. The mechanism of
corticosteroid-induced AVN appears to be
3-fold: first, occlusion of small vessels by
fatty emboli; second, impedance of sinusoidal blood flow within the bone by
increasing intraosseous pressure resulting from an increase in fatty mass in a
confined space of the bone marrow; and
third, direct local cytotoxic effects inhibiting angiogenesis and osteogenesis. These
factors combined with the underlying disease process for which steroids are being
used create a disparity between oxygen
supply and demand leading to AVN.10,11
Multifocal osteonecrosis is defined as
disease involving 3 or more separate anatomical sites, the most common being
the femoral head, distal femur, proximal
humerus, and talus.12 Indeed, approximately 3% of the population with AVN
have multifocal disease, and most of
these cases are related to steroids.13
Laporte et al13 found that 30 of the 32
patients (94%) with multifocal AVN had
a history of corticosteroid therapy. They
reported that primary involvement of
the foot or ankle in only 1 of 32 subjects. Zhang et al14 investigated multifocal
AVN in subjects using high-dose steroids
for the treatment of severe acute respiratory syndrome. They reported 4 cases (7
lesions) of AVN in foot and ankle in 43
patients. Bilateral involvement was present in 3 of the 4 patients.14 SLE appears
to be a significant risk for developing
multifocal AVN. Zizic et al15 found that
28 of the 54 SLE subjects (52%) of the
patients developed AVN. Further evaluation demonstrated that 26 of 28 patients
had bilateral osteonecrosis.15 The authors
found a statistically significant correlation
between higher mean prednisone dose
and increased number of bony sites.15
Avascular necrosis of the navicular is
termed Kohler’s disease in children and
Mueller–Weiss or Brailsford’s disease in
adults. Kohler’s disease is generally selflimiting without long-term sequelae.
Mueller–Weiss however is not so benign.
This condition was first described by
Mueller16 in 1927. He theorized that the
condition was due to chronic compression of adjacent structures. Mueller then
described a second case in 1928 and
revised his theory that the disease was
not traumatic but congenital.17 In 1929,
Weiss described a similar condition and
was the first person to attribute this disease to osteonecrosis.18 The typical
Mueller–Weiss patient is middle aged in
the fourth to fifth decade of life.19 Women
are more frequently affected than men
6:4.19-21 As the disease progresses, pes
planus occurs despite displacement of
the talar head laterally and a varus hindfoot.22 Maceria et al described 5 radiographic stages of the disease according to
increases in the sagittal plane deformity
of the talonaviculo-first ray (Table 1).22
As the staging and deformity increase,
plantar pressure significantly increases
in the midfoot while decreasing in the
forefoot.23
Osteonecrosis of the first cuneiform is
extremely rare. Bushcke disease, osteochrondritis of the first cuneiform, is the
most reported form in the literature. This
occurs in children similar to Kohler’s disease. Although Franz Bushcke is credited
with the disease eponym, 3 others had
described the disease prior to his publication in 1934 (Lessere in 1930, Buchman24
in 1933, and Haboush25 in 1933). Many of
the reports include pathology within the
first cuneiform and navicular. As for adult
onset of nontraumatic AVN, the authors
are unaware of any description in the
English language.
Various surgical treatments have been
described to address Mueller–Weiss syndrome, such as core decompression,26
open reduction and internal fixation,1 talonaviculocuneiform fusion,19 talonavicular arthrodesis,21 and triple arthrodesis.27
Fernandez de Retana reported that arthrodesis provided better outcomes compared
with more conservative options such as
open reduction and internal fixation.19
They also did not recommend the use of
an isolated talonavicular or triple arthrodesis since neither procedure address the
navicular–cuneiform joints.19 Their assessment was that the talonavicular cuneiform fusion offered the best outcomes to
address both deformity and pain.19 We
agree with this assessment and because
of the presence of both the navicular and
cuneiform AVN, we performed a talonaviculocuneiform fusion. We were unable
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to find short- or long-term outcomes of
talonaviculocuneiform fusions in the literature for either the treatment of AVN or
other pathologies. This lack of data limits the conclusions that can be drawn
on both the treatment of medial column
AVN and the long-term outcomes from
extended medial column arthrodesis.
Conclusion
The authors present a very rare presentation of bilateral osteonecrosis of the
tarsal navicular and first cuneiform in a
patient with SLE. The patient was treated
with bilateral talonaviculocuneiform
arthrodesis, which considerably improved
the pain and function of both extremities.
The authors feel that our results also support the use of the talonaviculocuneiform
fusion osteonecrosis of the tarsal navicular,
also known as Mueller–Weiss syndrome.
Acknowledgment
The authors would like to thank Jeffrey Manway,
DPM, for providing the intraoperative photography.
5. Gladman DD, Chaudhry-Ahluwalia V,
Ibanez D, Bogoch E, Urowitz MB.
Outcomes of symptomatic osteonecrosis in
95 patients with systemic lupus erythematosus. J Rheumatol. 2001;28:2226-2229.
6. Abu-Shakra M, Buskila D, Shoenfeld Y.
Osteonecrosis in patients with SLE. Clin Rev
Allergy Immunol. 2003;25:13-24.
7. Rascu A, Manger K, Kraetsch HG, Kalden JR,
Manger B. Osteonecrosis in systemic
lupus erythematosus, steroid-induced or
a lupus-dependent manifestation? Lupus.
1996;5:323-327.
8. Mok CC, Lau CS, Wong RW. Risk factors for avascular bone necrosis in systemic lupus erythematosus. Br J Rheumatol.
1998;37:895-900.
9. Arfi S, Moreau F, Heuclin C, Kreis H,
Paolaggi JB, Auguier L. Aseptic osteonecrosis in renal transplantation; apropos of 29
cases [in French]. Rev Rhum Mal Osteoartic.
1975;42:162-176.
10. Canalis E. Clinical review 83: Mechanisms of
glucocorticoid action in bone: implications to
glucocorticoid-induced osteoporosis. J Clin
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11. Mirzai R, Chang C, Greenspan A, Gershwin ME.
The pathogenesis of osteonecrosis and the
relationships to corticosteroids. J Asthma.
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