Fibro-osseous lesion of the mandible mimicking chronic osteomyelitis

oral surgery oral medicine oral pathology with secrionson endodontics Volume

40, Number

and

dental

4, October.

radiology

1975

oral surgery Editor: ROBERT

B. SHIRA,

D.D.S.

School of Dental Medicine, Tufts University 1 Kneeland St. Boston, Massachusetts 02111

Fibro-osseous lesion of the mandible mimicking chronic osteomyelitis S. Jocobsso~~,0. Hn116~1,I,. Hollcltder, C.-G. Hcrnsso~l,crud J. Ihdstviinl, Gb’feborg, SwedeIt DEPARTMENT

OF

EN’I’,

ORAL

RADIOLOGY,

AND

PATHOLOGY,

UNIVERSITY

OF

GijTEBORG An account is given of the clinical, radiologic, and pathologic, features of a special form of fibro-osseous lesion of the mandible. The material consists of fifteen cases (eleven females and four males). Two cases are descrilwd in detail. Together with Case 1, a probably unique series of radiographic pictures is presented, showing the development of the disease.

F

ibrous dysplasia was first described by Lichtenstein’ in 1938. A more detailed description of its clinical course, histopathology, and radiographic appearance was published by Jaffe and Lichtenstein* in 1942. The disease is characterized by fibro-osseouschanges in bone tissue, Three different types are recognized : (1) the monostotic form, (2) the polyostotic form, and (3) Albright’s syndrome, which, in addition to polyostotic skeletal changes, also displays pigmentation of the skin and endocrine disturbances. Fibrous dysplasia localized to the bones of the face is often monostotic, 433

and the caurrellt ol)inion is thitt it nl;rllif thcl tlisclascl into two forms-one form with continuous symptont-free growth, an(l th(l other with intcrmittcnt growth combined and subfebrilit,y. The lsttcr type is with local pain, trn(lerncss on palpation, illustrated in two wws (two f~lalos with monostotic fibrous clyspla.sia in thcb right side of the mantliblt~). The aim of this stud!, UYIS to sh~tl f’urther light upon thr spe&l type of fibroosseous lesion with recurrent cpisotlcs oi’ pain ant1 swclliag. This type of disease has until now rcmainccl relativcl;v unBno~-II and has mostly bcrn reported as isolatc~cl c*asc:s of fihrom tl,vspl;k (ieviatiflg from the usual (+linicd appearance.4- 5, 7 MATERIAL Some fifty caases of fibrous tlysplasin of the facial bones were diagnosed, assessed, and followed in (Gtcborg betwcctl 1960 and 1973. During that period a special group c~rystallixetl, in which thtl clinical characteristics were recurrent episodes of growth with simultaneous pain, s~clling, and ocdcasionally an elevated erythrocytc> sedimentation rate ant1 l’cvcr. E’iftclcn such cases (eleven females and four males) WYC rcgistcrccl anal followed up during the period of 1960 to 1973. ln all fifteen of thcst, patients the fibro-osseous changes were localized to the manclihlc. Fig. 1 shows the sexual clistriln~tion, the age of the patients at the time of appearance of the tlisoasc~, ilt~(l thr localiswtioll in the mandible. Tooth extractions, if any, at the time of appc’arancc of the tliscnse arc intlicatetl. CLINICAL

FEATURES

Common to all wscs was ill) intermittent growth of the involved area. In connection with such a period the patients suffered from tenderness and pain in the affected area. The exacerbations occnrrctl at intervals varying from months to years and had, as a rule, a duration of I to 2 weeks. In all 11 of the women the disease started in the right half of the mantlible. Only in t1v-o eases (IT. B. and R. W.) did the process em&roach upon the midline (Fig. 1). In the four male patients the changes were localized to the left half of the mandible. The time of onset seemed to rarp widely. No prediction of any particularly susceptible age could be made. In one case (S.-B. P.) the exacerbations appeared regularly in connection with menstruation and could bc subdued by progesterone therapy. In other casts such a correlation was not found. Several patients showed, in connection with an cxacderbation, an elevation in tcmperaturc and/or ESR. Most of the patients at some point rcccivcd antibiotic therapy, and in the majority of cases this appeared to have I)ositivc effects, such as relief of pain and inhibition of gro\vth of the lesion. and in some cases there was a regression of the local swelling. It should bc pointed out, howcvcr, that spontaneous remissions also occurred.

Volume 40 Number 4

Pibro-osseous First

Sex and age (year)

appearance ksle)

lesion

mimicking

Localization

chronic osteomyelitis Tooth extraction at debut

MO 0 24

No

LD

914

Yes

ES

022

No

YB

913

E-LC?

16

10

No

13

Yes

KA

915

13

Yes

JE

d33

26

Yes

S-BP? 43

33

Yes

BP

35

Yes

MG 056

47

Yes

RW

958

52

iit

RM

0’63

53

ti!d

EE

063

53

Yes

OJ

d75

63

No

KJ

d80

73

No

050

Pig. 1. Patient

data.

Yes

Yes

435

Oral October,

Table

I. (‘linic*al

fintlitlgs Fever ( > 20 mm./lrr.)

M. IA E. Y.

0. I). R. H.

E.-L. c. I<. A. .I. E. E.-L. c R. 1’.

F, 24 F, 14 F, 22 F, 13 F. 16 F; 15 M, 33 F, 43

F; 50 F.

56

M, F. AI; M,

63 63 i5 80

I<.:58

0. J. I<. J.

Surg. 1975

+ ? + +

(>

37.5”

+ + + +

-

+

? + + + -

-. + + + B

Positive C.)

effect

of.

antibiotics

No No Yes Yes No Yes No No 8 Yes Yes Yes Yes Yes

Table I shows the ESR, presence of fever, and the effect of antibiotic therapy in the treatment of an acute exacerbation. The elevated ESR (> 20 mm. per hour) varied between 25 and 58 mm. per hour. The elevation in the ESR compared t,o thcl asymptomatic intcrral was 70 to 38 mm. per hour. Fever more than 37.5O C. rrachetl a maximum of 38.s” (1. V-penicillin and ampicillin were the antibiotics primarily usctl. Xo diffrrrncc in effect between these antibiotics was fOUlltl. Jl1 l’itticllt M. C;., antibiotic therapy was not attempted. Many of the patients wcrc at first thought to have osteomyelitis, and were trcatccl accordingly. Cultures taken were negative, except in Patients Y. B. and R. XI., in whom sparse growth of streptococci was seen on one occasion only, and in another patient ($1. 0.) thcrc was a sparse grofth of Actinomyces. However, in none of these patients \vas thercb any rc>al proof of the presence of an inflammatorv agent. Two patients (1~. 11. and K. ,4.) werI’c subjected to an extensive immunologic*al examination, the findings of which wcrc completely normal. After biopsies in c~onllcc~tion with an acute caxacerbation, patients were, as a rnle, completely free of pain. Thus, the therapeutic approach in some patients (T. B., K. A., E. E., K. ,J.) was to carry out a dccortication, whereby cortical bone was removed within an arca approximately 3 by 1 cm. over the affected regions. This procedure in all eases resulted in a relief of pain and increased the interval betlveen periods of pain. The effect, however, was only transient, and exacerbat,ions recurred after some time (SW case report). RADIOLOGIC

FEATURES

ln a few cases the primary radiographic examination was made in the initial stage of the cliseasr. In me patient (K. A.), no changes at all could at first be depicted in intraoral periapical radiographs. About 2 weeks later, however, structural ehangcls simulating osttlompclitis appearetl. At this stage, ill-defined osteolytie areas as well as zones of sclerotic, bone were found. Later on, the bone became more sclerotic and noticeably increased in volume. Formation of new pcriostcal boric of thn tvpc tlesc*rihetl by Lautcnbac~h and T)ockhorn” was seen and

Fib-o-osseous

lesion mimicking

chronic

osteomyelitis

437

Big. 8. Patient K. A. (Feb. 9, 1973). Nine months after the onset of clinical symptoms. Note the changes in structure and size of the right side of the mandible. Osteolytic areas can he seen below and close to the mandibular canal. Fig. 3. Patient E.-L. C. Appearance of the right side of the mandible in February, 1973. Compare with Fig. 2.

prevailed at subsequent radiographic examinations (Fig. 2). Similar changes were found in the other young patients (Fig. 3). In the majority of cases radiographs were not taken until in later stages of the disease. The dominating feature then was an almost homogeneously sclerotic bone that had no sharp demarcation from neighboring, unaffected bone. In discrete areas, however, osteolytic zones

438

Jacobssoa

et al.

Fig. 4. Patient E. E. A, November, the mandibular canal, and the continuous the osteolytic changes at the mandibular

1969. Ii, May,

1972. Note the osteolytic clvange in form of the mandibular angle in R.

changes around angle. Also note

with nonsharp margins were to he found (Fig. 1, 11 and B). These seemedto he closely related to the acute periods, and in many cw~s pain appeared to he localized to these very areas, which, in addition, were more painful on palpation than other regions. Formation of periosteal boric was a frequent finding. Changes in form and size were seen in all patients, preclominantl;v as an increase in width. In two cases a &w-case in superoinfcrior dimension occurred (Fig. 4) while

Pibro-osseous

Volume Number

40 4

Table

II. Histopathologic findings Not calcified semioalcified trabeculae

Patient M. 0. E. S. Ey.2. c. K: A. S.-B. P. ::

ii.

+ + + + f + + + + + +

lesion mimicking

or Osteoblastic rimming + + +

Presence of multinucleated cells + + +

chronic

osteomyelitis

Celllar connective tissue stroma

439

Inflammatory cells

+ + + + + + + + + + +

+

the increase in width remained. An increase in all dimensions was usually seenand a frequent feature was a change in the mandibular angle (Figs. 2 to 4). With the exception of the initial stages in young persons, the radiographic appearances of the bone structure were similar in all fifteen cases,and formation of periosteal bone was seen in most of them. These findings lend support to the assumption that the radiographic findings play an important part in diagnosis. PATHOLOGIC

FEATURES

Biopsy specimens were taken from all patients. In most patients, specimens were taken on more than one occasion, mostly in connection with an acute exacerbation. The biopsied material was fixed in 10 per cent formalin, and decalcification was performed in Parengy’s solution containing nitric acid and chromic trioxide. Embedded in paraffin, the 3-micron thick sections were stained according to van Gieson and with hematoxylin and eosin. In four cases (Patients L. D., J. E., M. G., R. W.), only fragments of cortical bone or periosteal connective tissue were seen and this material was not considered to be representative. However, sufficient material was obtained in eleven patients for biopsy, and this material contained part of the cortical bone as well as tissue from the medullary cavity. The results of the histologic investigation are summarized in Table II. A constant finding in these cases was poorly oriented trabeculae of various thickness, and these trabeculae were either not calcified or only semicalcified. In two cases (K. A., E.-L. C.), woven bone spiculae were seen, which are characteristic of fibrous dysplasia. Furthermore, bone spiculae with rimming of osteoblastic cells were seen in three cases,but these also showed trabeculae without osteoblastic rimming. In two of these cases,multinucleated giant cells were seenalso. Between the trabeculae there was a cellular and vascular stroma. In one case (0. J.), inflammatory cells were also found in one of the biopsy specimens, making the diagnosis of chronic osteomyelitis questionable. CASE

REPORTS

In order to illustrate mandible, two case histories

the clinical course are presented.

with

this

type

of

fibro-osseous

change

in

the

Oral surg. October 7 197.7’

Fi,q. 5. Patient E. E. The cortex is very thin and the bone trabeculae are poorly oriented and of varying thickness. The stroma is celtular connective tissue. (Hematoxylin and rosin. Magnification, x100.) Pig. 6. Patient E. E. Semicalcified bone spiculac with osteoblastic rimming in some areas. There is a cellular and vascular stroma between the trabeculac. (Hrmatoxylin and eosin. Magnification, xl 80.)

CASE

1

I(. A., a 15-year-old girl, previously in good health, presented in the spring of 19i2, with a furuncle on the right foot. Some days later a swelling and tenderness were noted in the right knee. Two weeks later pain developed in the right lolver jam, 4-6 region. The dentist corrected the filling in 4-6, which was considered to be the cause of the pain. An x-ray film taken on May 9, 19T2, was normal (Fig. 8, A). However, pain remained in the lower jaw. Xew radiographs taken on June 8 and 26, together wit,h the clinical course, gave reason to suspect osteomyelitis (Fig. 8, H and C). The patient was treated with dicloxacillin, which wduced the pain, l)ut as soon as the patient had completed the course of dicloxacillin therapy, a renewed period of pain developed, and each time there was an increased swelling of the right half of the mandible. Radiographs taken on July 6 and 13, showed further progression of the lesion (Fig. 8, D and E). Tooth 4-6 was extracted; material taken for culture was nega-

and

Fig. 7. Patient K. eosin. Magnification,

Pibro-osseous

lesion mimicking

A.

trabeculae

Not x189.)

calcified

in

highly

chronic

cellular

osteomyelitis

stroma.

441

(Hematoxylin

tive. In connection with the period of pain the patient was subfebrile with a temperature between 37.5” and 38” C. The ESR during these periods varied between 10 and 15 mm. per hour and thus was not significantly elevated. The patient was admitted to the ENT Clinic at Sahlgrenska Hospital in Goteborg on March 7, 1973. The course of the disease and its development as shown by radiographic changes then suggested fibrous dysplasia (Fig. 2). A biopsy specimen was taken. The histopathologic examination showed fibrous dysplasia and gave no support for osteomyelitis. After biopsy the patient was free of pain for some months without antibiotic therapy. She was readmitted to the clinic on May 28, because of recurring pain. A decortication was carried out within 1 by 3 cm. area in the pathologically changed region of the mandible. The patient remained free of symtoms until November, 1973, when she had a new exacerbation, which was terminated by the use of V-penicillin. Since then the patient has had one further period of pain which also was terminated by the use of V-penicillin. Cultured material has given negative findings, and an extensive immunologic examination showed completely normal values. The etiology of the disease has not been established. CASE

2 (see

E.

Fig 4)

E., a 63-year-old woman, previously in good health, entered menopause in 1961. In 1962, pain occurred on the right side of the lower jaw. A filling was then made in 4-7. During 1963, pain recurred in the same area on several occasions. Tooth 4-7 was then extracted. At the time of a new period of pain in August, 1963, a swelling of the mandible in the region of extraction was seen. The patient was subjectively free of symptoms after therapy with V-penicillin. In 1964, there were repeated periods of pain in the right mandible in connection with increased swelling. These periods occurred at intervals of 1 to 2 months and lasted approximately 10 days. Ampicillin therapy reduced the pain, and the mandibular swelling decreased. However, new periods of pain arose. Repeated biopsy specimens from the affected area showed granulomatous changes only. In August, 1966, a decortication was carried out, and a large biopsy specimen was subjected to histopathologic examination. The diagnosis was fibrous dysplasia. The patient remained free of symptoms for 6 months postoperatively. Thereafter, new exacerbations developed at approximately l-month intervals. As a rule, antibiotic therapy had a good subjective effect. Antiepileptic therapy (Tegretol) was tried but had no convincing pain-reducing effect. In November, 1968, apical curettage and retrograde amalgam fillings in 4-5 and 4-4 were carried out, after which there was a pain-free period of approximately 2 months. In May, 1969, a new decortication was carried out. The diag-

Oral Ortober,

Fiy. E, .July

8. Patient 13, 1972.

I<. A. A, May

9, 1972.

B, June

8, 1972.

C, June

26, 1972.

D, July

Surg. 1975

6, 1972.

uosis was again fibrous dysplasia. The patient afterward remained free of symptoms for approximately 6 months. ln connection with new exacerbations, subfcbrility of approximately 38” C. and a slight elevation in the ESR from 10 to 20 mm. were noticed. Repeated cultures taken from the pathologically changed awa of the mandible showed no bacterial growth. The patient is currently Iwing treated as an outpatient. No ctiologic agent or causal therapy has yet been discoverrd.

COMMENTS

Common to the patients in this study was an intermittent growth of the diseased part of the mandible, with simultaneous local pain and tenderness. Characteristic of the condition was the clinical similarity to the inflammatory changes in osteomyelitis. In all vases the c~ondition was primarily interpreted as an inflammatory process, which in many casesresulted in apparently unnecessary extraction of teeth. Other authors have reported similar vases in which the

Volume Number

40 4

Pibro-osseous

lesion mimicking

chrovlic

osteomyelitis

443

symptoms were explained as inflammatory episodes of fibrous dysplasia or were considered to be a variant of osteomyelitis.7 The present cases differ both clinically and radiographically from the classic form of fibrous dysplasia, although the histopathologic features in many cases are in accordance with that type of disease. However, there arc some patients in whom the histopathologic features are confusing or uncertain. We are aware, therefore, that among these cases there might be concealed other types of fibro-osseous lesion and/or even osteomyelitis. Further study might reveal their true nature. Some of the cases in this material have been presented at various symposia. From the subsequent discussions it appears that patients with similar clinical symptoms have been found in different parts of the country and have always constituted a diagnostic and therapeutic problem. There is a noticeable predominance of females among the patients. A curious thing is that all eleven of the female patients in this study had changes in the right half of the mandible, whereas the four male patients had changes in the left half of the mandible (compare Lautenbach and Dockhorn6). Pain is the dominant clinical characteristic and constitutes a difficult therapeutic problem. Most patients were free of pain following biopsy at exacerbation. Therefore, in some patients (Y. B., K. A., E. E., K. J.), we performed a “decortication.” NO doubt this surgical treatment has been of good help when we were trying to arrest a progression of the disease or to attain relief of pain, and so has been antibiotic therapy in many eases. How this latter treatment could have any effect is obscure, since no convincing inflammatory agent was found. The positive cultures in Patients Y. B., R. M., and M. 0. are in all probability the result of contamination. Only temporary therapeutic results have been obtained. In all cases, exacerbations have occurred independent of chosen therapy. At present, no causal therapy exists. Recent investigation of some patients has shown the presence of anaerobic gram-positive bacteria in diseased areas. In these cases, histochemical analysis has revealed areas with total lack of enzymatic activity, pointing toward tissuenecrosis and some type of chronic osteomyelitis. Whether the condition is a primary chronic or a secondary infection in a fibrous dysplasia is not certain at the moment. It has been shown that thcrc is an extremely high uptake of sgmTc in bone changed by fibrous dysplasia.8 We, therefore, plan a study with g”mTc-scintigraphy in an attempt to evaluate (1) the ability of ‘)nxl’Tc as a diagnostic tool, (2) its usability for long-term follow-ups, and (3) control of treatment. In addition, such a study might throw more light upon the nature of the disease. Our hope is that a battery of different tests will provide a more distinct classification of this type of fibro-osseous lesion, so that we perhaps can then find more accurate therapeutic alternatives.

1. Liehtenstein, L.: Polyostotic 2. Jaffe, H. L., and Lichtenstein, 1942.

Fibrous Dysplasia, Arch. L.: Fibrous Dysplasia

Surg. 36: 874, of Bone, Arch.

1938. Pathol.

33:

777-816,

Oral October,

Surg. 1975

4. I)ahlgrtw, S. E., Lind, P. O., Lindl~lom, A., and MBrttwsson, CT.: Fibrous Dysplasia of Jan Hones, d\cta Otolnryngol. 68: 257.2i0, 1969. 3. Kiirjii, J., and KBsLuen, 0.: Fibrous Dysplasia of the Jaw Bones, Acta Otolaryugol. 74: 130-138, 19i2. 6. Lautenbach, E., and Dockhorn, R. : FibrSse Kic,frrerkrankungen, Stuttgart, 1968, Georg Thieme Verlag. 7. Fitzpatrick, R. N.: Fibrous Dysplasia and Infection of the Mandible, ORAL SURG. 22: 209-216, 1966. 8. Kohlin, M., anil Nosslin, B. : Personal Communication, 1974. Reprint

requests

to:

Dr. S. Jacol)sson ENT Department University of Giiteborg Giiteborg, Sweden