142

Case Report

Chronic Recurrent
Zehava Sadka Rosenberg,1 Steven

Multifocal
Shankman,1 Michael

Osteomyelitis
Klein,23 and Wallace Lehman4

Chronic recurrent multifocal osteomyelitis (CRMO) was first described in 1 972 under the name of subacute and chronic recurrent osteomyelitis [1 ]. This unusual form of chronic osteomyelitis was distinguished by a benign, self-limited dinical course and symmetric osteolytic lesions in the metaphysis of tubular bones in children. Since then, reports of similar cases have further delineated the clinical, radiologic, and histopathologic characteristics of the disease. It has been linked with pustulosis palmoplantaris [2] and clavicular and sternal abnormalities [3-5]. Also, the lytic bony lesions were not always symmetric [2], nor did the entity exclusively occur in children [2]. A new designation was offered-chronic recurrent multifocal osteomyelitis [6]. In the last year, three cases of CRMO were seen at the Hospital for Joint Diseases, Orthopaedic Institute. In all three cases, there was a 3-month to 1 -year delay in the diagnosis, partly because the diagnosis was made by exclusion and partly because of a lack of awareness of this entity by clinicians and radiologists. We suspect that the disease is more common than is presently realized and hope to familiarize radiologists with this unusual entity.

admitted to our hospital. Multiple sistent with chronic osteomyelitis.

right clavicular biopsies were conMultiple courses of IV antibiotics

were not helpful.

Physical

examination

revealed

a tender,

swollen

right clavicle. Laboratory data were not remarkable, except for an elevated sedimentation rate of 56. All bacteriologic and immunologic studies were negative. Radiographs revealed an expansile, sclerotic lesion of the right clavicle with mottled lucencies (Fig. 1A). Radiographs of the right leg were obtained because of pain. Focal sclerosis

was noted at the proximal right tibia. A Tc-MDP

increased increased uptake uptake

bone scan revealed

in the right clavicle. A gallium-67 scan revealed in both the right clavicle and proximal right tibia.

Open

biopsy

of the right clavicle

was

performed

(Fig. 1B). The

histopathology revealed a polymorphous inflammatory infiltrate dommated by plasma cells and lymphocytes but containing polymorphonuclear leukocytes and a few histiocytes. Focal areas of osteonecrosis were present, but subperiosteal new bone formation was a prominent feature.

Discussion

Case Report
A 7-year-old
right clavicle.

giri presented
One year before

for elective admission,

biopsy of a painful, swollen tender swelling was noted

in both
been

feet, the right shoulder, and the right clavicle. The patient had hospitalized five times at other institutions before she was

CRMO is a benign, self-limited process involving the axial and appendicular skeleton. The cause is unknown. A fastidious, slow-growing bacterial or viral organism has been implicated as the causative agent [3, 7], but blood and bone cultures have almost always been sterile. An impaired host immunologic system has also been postulated as a cause for the diseases [1 ]; however, immunologic studies have been negative [2]. CRMO is more common in females (female:male, 2:1). The age of onset has ranged from 20 months to 55 years [3], but CAMO is most common in children and adoles-

Received October
1

Department

1 9, 1 987; accepted after revision February 1 6, 1988. of Radiology, Hospital for Joint Diseases, Orthopaedic Institute,

301 E. 1 7th St., New York,

NY 1 0003.

Address

reprint

requests

to S. Shankman.

2 3 4

Department of Pathology, Hospital for Joint Diseases, Orthopaedic Institute, New York, NY 10003. Present address: Department of Pathology, Mount Sinai Medical Center, New York, NY 10029. Department of Orthopaedics, Hospital for Joint Diseases, Orthopaedic Institute, New York, NY 10003. July 1988 0361-803X/88/1 51 1-0142 American Roentgen Ray Society

AJR 151:142-144,

AJR:151,July

1988

MULTIFOCAL

OSTEOMYELITIS

143

Fig. 1.-A, Radiograph of right clavicle reveals an expansile, sclerotic lesion with interspersed B, Bone surface reveals extensive subperiosteal new bone formation (H and E, x 15). C, Detail of B reveals inflammatory infiltrate among reactive bone fragments (H and E, x40).

mottled

lucencies.

cents. The clinical course is benign. General symptoms such as fever or malaise are usually mild or absent [8]. Occasionally, there is a history of throat infection [2]. Patients usually present with an insidious onset of swelling, pain, and tenderness in the affected bones. Association with pustulosis palmoplantaris has been described, particularly when clavicular involvement is present [2]. In these cases, exacerbation of the pustular eruptions appeared to be closely linked to exacerbations of bony lesions. Symptoms may persist for a few years, with intermittent periods of exacerbation and remission at multiple sites. Laboratory findings are nonspecific. Mild elevation of the erythrocyte sedimentation rate is commonly present. The WBC count is usually normal or slightly raised. Antistreptolysin 0 titers are elevated in about one-quarter of the patients described [3]. Bone biopsies show a nonspecific subacute or chronic osteomyelitis, despite negative blood and bone cultures. Radiographically, lytic lesions are found in the metaphyses of long bones. The sites of predilection are similar to those of acute hematogenous osteomyelitis in infants and children. Diaphyseal involvement is less common but may occur, as seen in one of our patients. The lesions are often but not always symmetric, are surrounded by thin sclerotic borders, and may be associated with periosteal new bone formation. Sclerosis and complete regression of the lesions with healing may occur [1 5]. Multifocal destructive bony changes in different stages of development and healing [7] may be present. The most commonly affected sites are the tibia followed by the femur, clavicle, and fibula. Other skeletal sites have been described including the mandible, ribs, humerus, radius, ulna, metacarpals and metatarsals, talus, spine, pelvis, and sternum [3]. Radionuclide bone scans show increased tracer uptake in the affected areas and may detect clinically silent sites of involvement [5]. The radiologic findings are characteristic but not pathognomonic, and other causes of multifocal
,

osteolytic lesions (such as histiocytosis X, viral and bacterial osteomyelitis, leukemia, and neuroblastoma) should be considered. The histopathology of chronic multifocal osteomyelitis resembles that of a nonspecific subacute or chronic osteomyelitis. Usually, an admixture of inflammatory cells, dominated by plasma cells and lymphocytes, contains varying numbers of neutrophils and histiocytes. The composition of the inflammatory infiltrates may vary to such degree that histiocytoses or metastatic round cell neoplasms are suspected. A case has been reported in which necrotizing epithelioid granulomata with giant cells mimicked tuberculosis histopathologically, but no organisms could be either stained or cultured [7]. In addition to the index case, two similar cases were seen at the Hospital for Joint Diseases, Orthopaedic Institute, this past year. Both patients had multiple courses of antibiotic therapy and an open biopsy elsewhere. On admission, extensive laboratory analysis, including immunologic and bacteriologic studies, were negative. The erythrocyte sedimentation rate was consistently elevated. One patient had multiple, illdefined lucencies at several upper extremity phalanges, a lytic lesion with cortical destruction at the left second metatarsal, and a similar lesion with faint periosteal reaction of the distal right ulna. The second patient showed an indistinctness of the medial cortex of the right clavicle (Fig. 2A), which was better defined with CT (Fig. 2B). Open biopsies were performed in both patients, and the histopathology was similar to that of the index case. The treatment for chronic recurrent multifocal osteomyelitis consists of supportive measures. Symptomatic relief may be provided with steroid therapy [7]. Most cases reported in the literature have been treated with antibiotics at one time. The efficacy of this treatment is questionable because no bacterial organism was ever grown from a bone biopsy. Also, antibiotic treatment has not been shown to alter the course of the

144

ROSENBERG

ET AL.

AJR:151

July 1988

Fig. 2.-A, Radiograph of right clavicle reveals an indistinct medial cortex. B, CT scan of right clavicle reveals a lytic lesion, proximally, destroying the cortex.

disease. Familiarity with the diseases and early diagnosis are important to obviate an extensive workup, prolonged antibiotic therapy, and repeated surgical biopsies. Reassurance of the patients of the relative benignity and favorable prognosis of the illness, despite its protracted nature, can also serve to alleviate excessive apprehension and anxiety.

1978;93(2):227-231
3. Cyrlak D, Pais MJ. Chronic recurrent multifocal osteomyelitis. Ske!eta! Radio! 1986;15:32-39 4. Junk AG, Moller BN. Inflammatory hyperostosis and sclerosis of the clavicle. Ske!eta! Radio! 1986;1 5:284-290 5. Gamble JG, Rinsky LA. Chronic recurrent multifocal osteomyelitis: a distinct clinical entity. J Pediatr Orthop 1986;6:579-584 6. Probst FP, Bjorksten B, Gustavson KH. Radiological aspects of chronic recurrent multifocal osteomyelitis. Ann Radio! 1978;21 : 117-1 25 7. Kozlowski K, Masel J, Harbison 5, Yu J. Multifocal chronic osteomyelitis of unknown etiology. Report of five cases. Pediatr Radio! 1983;13: 130-1 36 8. Solheim FL, Paus B, Liverud K, Stoen E. Chronic recurrent multifocal osteomyelitis. A new clinical-radiological syndrome. Acta Orthop Scand

REFERENCES
1 . Giedion A, Holthusen W, Masel LF, Vischer symmetrical osteomyelitis. Ann Radio! (Pans)

D. Subacute
1972:15:329-342

and chronic

2. Bjorksten B, Gustavson KH, Enksson B, Undholm A, Nordstrom S. Chronic recurrent multifocal osteomyelitis and pustulosis palmoplantans. J Pediatr

1980:51:37-41