British Journal of Rheumatology 1996;35:1019-1021
PAEDIATRIC RHEUMATOLOGY
CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS: SPINAL
INVOLVEMENT AND RADIOLOGICAL APPEARANCES
J. C. MARTIN, R. DESOYSA,* M. M. O'SULLIVAN, E. SDLVERSTONEf and H. WILLIAMS*
Departments of Rheumatology, * Paediatrics and ^Radiology, Wrexham Maelor Hospital, Wrexham, Clwyd
LL13 7TD
SUMMARY
We report a case of chronic recurrent multifocal osteomyelitis which presented as back pain in a 14-yr-old male. The full
distribution of the spinal lesions was determined by MRI, which proved to be more sensitive than other imaging modalities.
After 1 yr, in spite of a good symptomatic response to corticosteroid therapy, new MRI abnormalities had developed.
KEY WORDS: Osteomyelitis, Spine, Magnetic resonance imaging.
surrounding sclerosis extending into the left pedicle (Fig. 1).
An MRI scan of the lower thoracic and lumbosacral spine
was performed to evaluate the lesion further. T2-weighted
images confirmed the lesion in the body and left pedicle of
Til, and showed further unsuspected high-signal lesions in
the vertebral bodies of L5, SI and S2 (Fig. 1). The lesions
showed modest uniform enhancement with IV gadolinium
(as its DTPA compound). The discs and endplates were
normal, and there was no soft tissue mass. An abdominal
mass was excluded by abdominal ultrasound and review of
relevant CT scan and MR scan images. A CT-guided biopsy
of the Til lesion was performed, histology from which
showed necrosis of bone fragments, fibrotic marrow and a
non-specific chronic inflammatory cell infiltrate. Culture of
the biopsy specimen for bacteria, including tubercle, was
negative. The X-rays of his foot from 1 VT previously were
reviewed and considered to show lytic lesions in the second,
third and fourth metartarsal shafts with pathological
fracture, which subsequently healed without clinical sequelae.
On the basis ofrecurrentmultifocal lytic bony lesions with
no evidence of infection or neoplasia, a diagnosis of CRMO
was made. Only partial symptomatic relief was achieved with
ibuprofen and paracetamol. Prednisolone 30 mg/day (0.5 mg/
kg/day) was started with complete resolution of symptoms
within 1 month. The dose was changed to an alternate-day
regime, reduced and discontinued over the following 3
months. Twelve months later, there had been no recurrence
of symptoms, but a follow up MRI of his thoracolumbar
spine (Fig. 2) showed some loss of height of L5 vertebral
body with discal herniation through the superior end plate
with some dehydration of the L4/5 disc. There were persistent
signal changes in the body and left pedicle of Tl 1, with some
loss of height and a small discal herniation through the
superior end plate. There was minor collapse of the T10
vertebral body with discal herniation through the superior
end plate. There were signal changes in the T9 vertebra,
consistent with a new lesion. There was partial collapse of T8
with depression of the superior end plate.
CHRONIC recurrent multifocal osteomyelitis (CRMO) is
a condition of unknown aetiology characterized by
remissions and exacerbations of multiple painful bone
lesions. It has a wide differential diagnosis from which
infection and neoplasia must be excluded. We present
a case of CRMO in a young boy who presented with
back pain, and describe the associated radiology,
highlighting the superiority of MRI to other imaging
modalities in denning the distribution of spinal lesions.
CASE REPORT
A 14-yr-old schoolboy was referred with a 3 month history
of progressive lower back pain, two episodes of night sweats
and an elevated plasma viscosity (PV) of 2.03 mPa [normal
range (NR) 1.55-1.75]. There was no other preceding history
of constitutional upset and he was otherwise asymptomatic,
taking only simple analgesia. At presentation, there were no
peripheral joint or bony symptoms, although 1 yr previously,
pain in his right foot was attributed to stress fractures of the
second, third and fourth metatarsals. There was no family or
other past medical history of note. A thoracolumbar scoliosis
with paraspinal muscle spasm, reduction of spinal movements and tenderness over the first lumbar vertebra (LI) was
apparent on examination. Bilateral straight leg raising was
limited to 45°, but there was no neurological deficit in his
lower limbs. Mild acne without conglobata was noted over
his chest and back. He was apyrexial and examination was
otherwise normal. Investigations were as follows: haemoglobin 12.2 g/dl, white cell count 7.5 x 10'/l, platelets
523 x lO'/l (NR 150-400) with rouleaux on the blood film,
PV 2.01 mPa, C-reactive protein 26.1 mg/1 (NR < 10). Urea,
creatinine, electrolytes, calcium, serum immunoglobulins,
a-fetoprotein, human chorionic gonadotrophin and angiotensin converting enzyme were normal, as was an
echocardiogram. ASO titre, viral and atypical bacterial
serology were negative, as was his HLA B27 status. An X-ray
of the lumbar spine was initially thought to be normal. An
isotope bone scan showed a small increase in radioactivity in
the body and left pedicle of the eleventh thoracic vertebra
(Tl 1) (Fig. 1). A CT scan of this area showed irregular
destructive areas in the vertebral body of Til with
DISCUSSION
CRMO typically presents in children and adolescents, with a female predominance. Although rare,
adult cases have been reported [1, 2]. Bone pain and
tenderness are the usual presenting symptoms,
although fever and constitutional upset have also been
Submitted 22 May 1996;revisedversion accepted 19 June 1996.
Correspondence to: J. C. Martin, Department of Rheumatology,
University Hospital of Wales, Cardiff CF4 4XW.
© 1996 British Society for Rheumatology
1019
1020
BRITISH JOURNAL OF RHEUMATOLOGY VOL. 35 NO. 10
described [1,3,4]. Bony lesions usually occur in the
metaphyseal, less commonly in the epiphyseal region of
long bones [3-5], and are characteristically lytic in
nature with or without areas of surrounding sclerosis
[1,4,6]. The diagnosis is one of exclusion. Bacterial
osteomyelitis, neoplasia (leukaemia, metastatic neuroblastoma, Ewing's sarcoma, osteoid osteoma and
osteoblastoma), trauma and eosinophilic granuloma
are conditions which have to be excluded. According to
King et al. [1], the following criteria should be met for
the diagnosis to be made: (i) multifocal (two or more)
bone lesions, clinically or radiographically diagnosed;
(ii) a prolonged course (over 6 months) characterized
by varying activity of disease, and with most patients
being healthy between recurrent episodes of pain,
swelling and tenderness; (iii) lack of response to
antimicrobial therapy given for at least 1 month.
CRMO has been reported at many skeletal sites,
most commonly the distal tibia and femur, and the
clavicle. Involvement of the spine occurs less common
with only a handful of cases reported previously
[4, 6-11]. Spinal involvement usually manifests radiologically with complete or partial collapse of the
affected vertebral body, or as in our case with lytic
lesions surrounded by areas of sclerosis. It is worth
noting that in our case the X-ray changes were initially
missed as they were very subtle and even in retrospect
only the L5 lesion was visible (Fig. 1). Bone
scintigraphy failed to demonstrate the full distribution
of spinal lesions, although it has been cited previously
as a useful investigation for detecting those lesions
which are clinically silent [4]. In our case, it was MRI
which revealed the full distribution of spinal lesions,
supporting the fact that MRI is more specific and
sensitive than plain radiography or isotope bone
scanning in defining the extent of spinal lesions in
CRMO [11], and diagnosing conventional vertebral
osteomyelitis [12].
A biopsy was carried out to exclude other pathology,
particularly neoplasia. Histological changes reported
previously in early lesions show an acute and chronic
inflammatory infiltrate with a predominance of polymorphonuclear cells and occasional areas of necrosis.
Later lesions show predominance of lymphocytes and
plasma cells with multinucleated giant cells and
occasional non-caseating granulomata in the bone
marrow. Older lesions show areas of necrosis and
fibrosis with new bone formation [1, 13]. Bearing in
mind the duration of symptoms (3-4 months) with
sclerosis on the CT scan, the histological appearances
of marrow fibrosis, bone necrosis and a non-specific
chronic inflammatory cell infiltrate in our case were in
FIG. I.—Montage of the initial radiological investigations. Top left: X-ray of the lumbar spine with subtle loss of definition of the left side of
the superior end plate of L5. Top right: Isotope bone scan showing increased uptake in the vertebral body and pedicle of Tl 1. Bottom left:
C T s c a n showing lytic lesions surrounded by areas of sclerosis in the vertebral body of T i l . Bottom right: T2-weighted image of the MRI scan
of the lower thoracic and lumbosacral spine showing increased signal in T i l vertebral body and pedicle, and L5 and SI vertebral bodies.
MARTIN ET AL.: CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS
1021
many years [1, 2, 7, 8]. In rare cases, bony sclerosis can
persist, premature epiphyseal fusion can occur with
resultant long bone deformity and growth arrest, and
progressive kyphosis has been reported [1, 4, 6].
CRMO is a diagnosis of exclusion which is
important to make in order to provide appropriate
treatment and support for the individual and family.
Spinal involvement has been thought to be an
uncommon manifestation of this condition, but as it
may be difficult to recognize, the true incidence is
unknown. MRI is the most sensitive and specific
modality for establishing the full distribution of spinal
lesions. Additionally, it does not involve radiation,
which makes it the ideal method of detecting and
monitoring spinal lesions in CRMO.
REFERENCES
FIG. 2.—Tl-weighted images of the repeat MRI scan after 12 months
showing the original lesions (see text) with a new lesion in the T9
vertebra (arrow).
keeping with the findings of older lesions as described
previously [1, 13].
The treatment of this condition is poorly defined.
The use of analgesics and non-steroidal antiinflammatory agents is recommended for symptomatic
relief. Antimicrobial agents are generally considered to
be ineffective [2, 8, 13], and this has been used as a
criterion for diagnosis. As there was no clinical
evidence of treatable infection, our patient did not
receive a trial of antimicrobials. Oral corticosteroids
have been found to be beneficial [1, 2, 10, 13], as in our
case. The lack of response to antibiotics suggests that
the aetiology of CRMO is not due to bacterial infection
per se. Culture of the biopsy tissue in our case was
sterile, as it has been in the majority of cases previously
[1, 13]. Coagulase-negative Staphylococcus, which was
thought to be a contaminant [1], and Mycoplasma
hominis [14] have been isolated in other cases. CRMO
is associated with psoriasis and pustulosis palmoplantaris [1, 10, 13], both of which are also associated with
synovitis-acne-pustulosis-hyperostosis-osteomyelitissyndrome (SAPHO) and the seronegative spondyloarthropathies. It has been postulated that CRMO is an
extension of the spectrum of spondyloarthropathies
with an inflammatory rather than infective aetiology
[1], although, as in our case, affected individuals are
usually HLA B27 negative [1, 13]. The prognosis is
generally good, although symptoms can relapse over
1. King SM, Laxer RM, Manson D, Gold R. Chronic
recurrent multifocal osteomyelitis: a non-infectious
inflammatory process. Pediatr Infect Dis J 1987;6:
907-11.
2. Bjorksten B, Boquist L. Histopathological aspects of
chronic recurrent multifocal osteomyelitis. J Bone Joint
Surg 1980;62B:376-80.
3. Cuende E, Gutierrez MA, Paniagua G, Feito C,
Gonzalez M, Sanchez J. Chronic recurrent multifocal
osteomyelitis: report of a case with epiphyseal and
metaphyseal involvement. Clin Exp Rheumatol 1995;
13:251-3.
4. Carr AJ, Cole WG, Roberton DM, Chow CW. Chronic
multifocal osteomyelitis. J Bone Joint Surg 1993;75B:
582-91.
5. Manson D, Wilmot DM, King S, Laxer RM. Physeal
involvement in chronic recurrent multifocal osteomyelitis. Pediatr Radiol 1989;20:76-9.
6. Brown T, Wilkinson RH. Chronic recurrent multifocal
osteomyelitis. Radiology 1988;166:493-6.
7. Cyrlak D, Pais MJ. Chronic recurrent multifocal
osteomyelitis. Skeletal Radiol 1986;15:32-9.
8. Gamble JG, Rinsky LA. Chronic recurrent multifocal
osteomyelitis: a distinct clinical entity. J Pediatr Orthop
1986;6:579-84.
9. Probst FP, Bjorksten B, Gustavson KH. Radiological
aspects of chronic recurrent multifocal osteomyelitis. Ann
Radiol 1978;21:115-25.
10. Paller AS, Pachman L, Rich K, Esterly NB,
Gonzalez-Crussi F. Pustulosis palmaris et plantaris: its
association with chronic recurrent multifocal osteomyelitis. J Am Acad Dermatol 1985;12:927-30.
11. Dawson JS, Webb JK, Preston BJ. Chronic recurrent
multifocal osteomyelitis with magnetic resonance imaging. Clin Radiol 1994;4fc 133-6.
12. Modie MT, Feiglin DH, Piraino DW et al. Vertebral
osteomyelitis: assessment using MR. Radiology 1985;
157:157-66.
13. Bjorksten B, Gustavson KH, Eriksson B, Lindholm A,
Nordstrom S. Chronic recurrent multifocal osteomyelitis
and pustulosis palmoplantaris. J Pediatr 1978;93:
227-31.
14. Hummel DS, Anderson SJ, Wright PF, Casell GH,
Waites KB. Chronic recurrent multifocal osteomyelitis:
Are the mycoplasma involved. N Engl J Med 1987;
317:511.