cherubism—clinical picture and treatment

Oral Diseases (2001) 7, 123–130 2001 Munksgaard All rights reserved 1354-523X/01

www.munksgaard.dk

CASE REPORT

Cherubism—clinical picture and treatment

M Kozakiewicz1, W Perczynska-Partyka1, J Kobos2

1Clinic of Maxillofacial Surgery, Institute of Dentistry, Medical University of Lodz, Kopcinskiego 22 PL-90153 Lodz, Poland;2Laboratory of Patomorphology, Institute of Pediatry, and Chair and Department of Patomorphology Medical University of Lodz,UI. Sporna 36/50, PL-91738 Lodz, Poland

Cherubism is a rare, painless, disfigurating disease prim-arily affecting bones of the jaws.OBJECTIVE: To report on five patients with cherubism.The symptoms of the disease, methods of managementand possible mode of inheritance are discussed and litera-ture is reviewed.PATIENTS: The study involves five cherubs, members ofone family. The diagnoses were based on history, physicalexamination, laboratory tests, X-ray parameters, andclinical follow-up. One member of the family had surgicalintervention. The remaining cases were left for obser-vation.RESULTS: Good aesthetic and long lasting effect wasreached in the operated patient.CONCLUSIONS: Treatment is unnecessary unless func-tional or emotional disturbances develop. An autosomalrecessive pattern of inheritance is suggested for thesecases, although autosomal dominant transmission hasbeen previously established.Oral Diseases (2001) 7, 123–130

Keywords: cherubism; inheritance; treatment; literaturereview

Introduction

Cherubism is a non-neoplastic disease of bones clinicallycharacterized by bilateral, painless enlargements of the jaws(Jones, 1933; Pinborget al, 1971). The appearance of theaffected children is normal at birth. Between 2 and 7 yearsof life swellings within mandibular body or tuberosities ofmaxilla appear. Males are affected twice as often asfemales. The rare frequency of the disease is confirmed bythe fact that during the post-war period in Poland only twofamilies affected by cherubism have been described

Correspondence: Marcin Kozakiewicz, D.D.S., Ph.D, Clinic of Maxillofa-cial Surgery, Institute of Dentistry, Medical University of Lodz Kopcinski-ego 22, PL-90153 Lodz, Poland. Fax 0048 42 656 6547The photographs in this paper have been published with the permissionof the patients concerned.Received 10 May 1999; revised 24 November 1999, 22 May 2000 and31 July 2000; accepted 9 August 2000

(Rydosz, 1960; Gabryelewicz and Wejroch-Kowalska,1975; Lagowska-Adamczyk and Wolan´ska-Karut, 1994).During the 50-year existence of the Clinic of MaxillofacialSurgery in Lodz, only one family was observed, in 1954.Cherubism has also rarely been observed in other countriessuch as Japan and Peru (Suyama and Uragou, 1954; Hitomiet al, 1997).

In cherubism the middle and lower parts of the facialskull are widened and the face is circular. Tuberosities ofmaxilla are usually deformed. Fibrous changes can alsoappear in the suborbital skeleton, the front part of the max-illa and the bottom of the orbit (Zachariadeset al, 1985).Augmentation of the alveolar processes deforms the palate.It takes the form of a reversed letter V and a nivelation ofthe palatal arch occurs (Khosla and Korobkin, 1970). Eye-balls are moved upwards which makes the characteristicfacies. Because of that Jones (1933) named the diseasecherubism. In most cases the rapid growth of the size ofthe maxilla and mandible has occurred within 2 years sincethe manifestation of the first symptoms (Topazian andCostich, 1965). Before the 10th year of life the osseousgrowth changes either stop or their development slows untilthe maturation period. Before the 20th year of life thechanges spontaneously regress, first in the maxilla then inthe mandible (Arnott, 1978; Riefkohlet al, 1985). Theappearance of the face may regain its almost normal formbefore the 4th or 5th decade of life.

The aim of this work is to present five patients from afamily affected with cherubism and to review the literatureconcerning the disease.

Patients and methods

At the Clinic of Maxillofacial Surgery of the Institute ofDentistry of the Medical University of Lodz we haveobserved familial occurring dystrophic changes in the facialbones of five children—offspring of two brothers. Theirdisease was noticed between the ages of 4 to 7 and it tookthe form of facial deformity caused by bilateral painlessswelling of the body and angle of the mandible. During thefirst few years submandibular lymphatic nodes wereenlarged (diameter 1–2 cm), painless, elastichard, and sep-arated from the bone. Rapid loss of teeth was noticed. X-ray examination revealed a bilateral blurring of bone struc-

Cherubism—clinical picture and treatmentM Kozakiewicz et al

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Figure 1 Dispersed multinucleated giant cells among fibrous stromawith a few thin-walled vessels. Original magnification3 200

ture or cyst-like formation within the body and angles ofthe mandible. Scintigraphic examinations of all the patientsshowed tracer uptake within the mandible and, to lesserextent, in the maxilla. However, no changes were observedin other bones. The concentration of calcium, phosphatesand hormones: TSH, FSH, LH, T3, T4, 17-corticostreroids,17-(OH)-steroids in blood serum remained in the range ofnormal values. The level of alkaline phosphatase in bloodserum was increased in four of five patients in all the lab-oratory tests performed between the 7 to 17 years of life.

The deformation process in four patients stopped duringthe adolescence period (13–17 year of life). In the case ofthe eldest patient, with the most advanced cherubicchanges, the inhibition of the pathologic process wasnoticed at 26 years of age.

Karyotype tests were performed in all the patients. Thekaryotypes were normal: 46 XX for women and 46 XYfor men. The pattern of inheritance in our patients may berecessive and not sex-linked. These tests were performedin the Medical Genetic Institute of Medical University ofLodz (Professor Bogdan Kaluzewski, MD, PhD). Clinicalfollow-up was performed at 6-month periods. Teeth whichwere a source of infection, loosened or displaced with peri-radicular granulation tissue were extracted. The surgicallyremoved tissue was histopathologically examined (Figures1–3). Microscopic examination revealed osteoclastic-like

Figure 2 Numerous inflammatory cells in fibrous stroma from the biop-sied clinically granulation tissue (the second described patient—W.S.).Original magnification3 200

Figure 3 Fibrous tissue with many interspersed multinucleated giantcells. Original magnification3 200

multinucleated giant cells in a moderately loose fibrousstroma. Eosinophilic cuff-like deposits surrounding smallvessels were not observed in evaluated cases.

Based on these findings the diagnosis of cherubismwas established.

Case 1The propositus, KS aged 7 (case record no. 178/76, 302/87,6671, 284/95) referred to the health care centre due to sym-metrical painless, hard with blurred outlines, augmentationson both mandibular angles and at lateral parts of the max-illa. Abnormal pattern of mandibular teeth eruption wasnoted. The facial deformities progressed during the 10-yearobservation period. They tended to slow down during thenext 3 years. Exophthalmos and right bulb displacementwere observed at 20 years old as well as the expansion ofthe right malar region. The disease was found to stabilizeat the age of 26, but no regression was observed. Retainedand displaced teeth with root resorption were noticed.

Second patientWS (303/87) was referred at age 17 for facial asymmetrydue to painless augmentation of the left mandibular angle.Thickening of the mandibular bone body was palpablebilaterally. At age of 25 years the patient lost his teeth: 12,26, 37, 46, 47, and the focus of granulation tissue visiblewithin the alveolar part of mandible together with teethrotated and displaced, as well as edentulous areas, partlydue to removals of loosened teeth and probably due to par-tial hypodontia were noted. Teeth root resorption wasnoticed.

Third patientAn.S, the sister (case record no. 93/88, 4/90) was admittedto the hospital at age 15 due to a thickening of the mandibu-lar body. Multilocular radiolucent areas and impacted teeth(47 and 48) were noticed in the radiological examination.At age 19 the patient insisted, because her marriage wasto take place, on corrective surgery. An osteoplasty wasperformed: the shape of the body and mandibular ramuswas contoured. The procedure was done during a stabiliz-ation period of the disease. Histopathological examinationof the removed bone revealed numerous, multinucleated,


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Figure 4 Two oldest patients. On the right patient WS (303/87) aged 6,on the left his older brother KS aged 7 (case record no. 178/76, 302/87,6671, 284/95). KS referred to the health care centre due to symmetricalaugmentations on both mandibular angles

giant cells of the osteoclastic type in a dense fibrous stroma.Foci of haemorrhage were noticed. The successful result ofthe treatment has remained after 7 years follow-up.

Fourth patientThe youngest of four, Ag.S, was reported for initial exam-ination at age 6, due to bilateral cortical expansion of themandibular angles (case record no. 94/88, 3/90). At age 13,the progression of the disease stopped, but no regressionwas noted.

Fifth patientAr.S, has been followed up since the age of 8 (case recordno. 108/88). Intraorally, cortical expansion of the mandibu-lar angle extending into the retromolar region was observedbilaterally. At age 15, the progression of the diseasestopped and the patient lost his teeth: 31, 32, 36, 37, 41,42, 46 and 47. Multilocular, radiolucent cyst-like lesions inentire mandible persisted.

Figures 4–15 and the Table 1 present the patients andtheir family history.

Figure 5 Patient KS aged 26. The exophthalmos and the right bulb dis-placement were revealed at the age of 20 as well as the right malar regionaugmentation. The disease was found to stop at the age of 26 but noregression was observed

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Figure 6 Patient KS aged 26. Computed tomography of the right orbitrevealed the displacement of the rear-lateral wall backward. Its opticaldensity matches the fibro-osseous tissue. The optic nerve and the medialrectus muscle were displaced mesially, the lateral rectus muscle was notvisible (probably due to atrophy secondary to compression) (arrow)

Figure 7 Patient KS aged 26. Computed tomography of mandibleshowed multiple foci compatible with soft-tissue (arrows)

Discussion

Many theories have tried to explain the aetiology of cherub-ism. Yet its pathogenesis remains unknown since the firstreported case (WA Jones, MD, meeting of North AmericaRadiological Association, 1931). Histopathological evalu-ation of cherubic lesions shows proliferating fibrous con-nective tissue containing numerous multinucleated giantcells. Southgateet al (1998) proved that these cells areosteoclasts.

Cherubism is a rare disease. It is considered to be heredi-tary with autosomal dominant pattern (McKusik, 1992)with 100% penetration in men and 50–70% in women(Betts et al, 1993). However, a number of cases withoutfamiliar background have been described (Gru¨nebaum andTiqva 1973; Zachariadeset al, 1985; Bianchiet al, 1987;Kaugarset al, 1992). These may be examples of recessiveinheritance with features such as the ones observed in the


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Figure 8 The siblings. From left to right: KS aged 17, An.S aged 15,Ag.S aged 6 and WS aged 16. Well visible changes in contour of face inKS and An.S

Figure 9 Posterio-anterior radiograph of paranasal sinuses: diffusedilution of the bony structures, maxillary sinuses are opaque, diminishedwith blurred outlines

present study or the result of a mutant gene. Mangionetal (1999) performed a genome-wide linkage search in twoaffected families, attempting to map the gene for cherub-ism. They have found a gene in chromosome 4. Within4p16.3 region a strong candidate is the gene for fibroblast

Figure 10 Patient An.S—the sister (case record no. 93/88, 4/90). Thepicture shows the look of the face at the age of 17 when the progress ofdeformations stopped, and after 2 years we observed the remission. Atthat time the patient insisted on having correction surgery of mandibularcontouring because of her marriage was to take place. The operationwas performed

growth factor receptor 3 (FGFR3). Mutations in this genehave been implicated in a diverse set of disorders of bonedevelopment (Mangionet al, 1999).

The characteristic appearance of the face of the patients(Figures 8 and 10) is explained in various ways. Khoslaand Korobkin (1970) suggest it is a result of enlargementof the bottom of the orbit and a weak support of lowereyelid. We agree with their hypothesis. In one of our cases(KS) we observed a deformation of the orbit on the rightside and movement of the eyeball resulting in the ‘lookingat the sky’ sign; while on the opposite side, where the bot-tom of the orbit was unchanged, the middle part of the faceremained normal. The classic appearance of patients withthe disease is not expected to be observed in all patients,since the clinical expression is variable and the mandiblemay be the only affected bone (Gru¨nebaum and Tiqva,1973). This is confirmed by the course of the disease inthree of five children presented here.

The increased level of alkaline phosphatase in bloodserum in four of our five patients confirms the observationof Hitomi et al, (1996).

In cherubism ocular disturbances can occur (Riefkohletal, 1985; Hawes, 1989). Riefkohlet al have described a


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Figure 11 Patient An.S 4 years after operation. The face is harmonic

case of visual disturbances caused by eyeball dislocationwhich resulted in imbalance among external eye muscles.This was noted in our patient KS. Also, displacement ofthe right optic nerve canal caused nerve pressure. In caseof worsening symptoms decompression of the nerve willbe considered. To the best of our knowledge, only one caseof optic nerve damage has been previously reported (alGazaliet al, 1993).

Retained and displaced teeth as well as root resorptionsform a supplementary factor influencing a correct diag-nosis, this sign being especially important in abortive formsof cherubism. The displacement of teeth is caused by thereplacement of bone by fibrous tissue, which in turn leadsto malocculsion (Morley and Stoneman, 1984; Zachariadeset al, 1985; Patel, 1987; Corduant and Gugny, 1989;Hawes, 1989; Levineet al, 1991).

Radiological data form the base for the diagnostic pro-cess (Hitomiet al, 1996). Computed tomographic scans(Bianchiet al, 1987; Wackeneet al, 1987; Marck and Kud-ryk, 1992; Hitomiet al, 1996) and scintiscans (Wells andSty, 1985; Hitomiet al, 1996, 1997) as well as plain radio-graphs provide the main evidence of benign osseouschanges. Between the 18th month and 2nd year of lifecherubism can be diagnosed mainly on radiological data(Faircloth et al, 1991). The following changes can beobserved: irregular, multilocular, well separated cyst-liketranslucencies which cause bone expansion, leaving only a

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Figure 12 The youngest of four (Ag.S) was reported for her initial exam-ination at the age of 6, due to augmentations within both mandibularangles (case record no. 94/88, 3/90)

thin layer of cortical bone. Axial CT scans show disruptionof the cortexes, which is more evident on the facial ratherthan on the lingual side (Hitomiet al, 1996). This findingwas noted in one of our cases. Teeth are displaced,unerupted, retained or ‘floating’ within cyst-like spaces(Fairclothet al, 1991; Katzet al, 1992; Hitomiet al, 1996).When the changes occur in alveolar bone and angles of themandible, the mandibular canal is often displaced(Zachariadeset al, 1985). The areas of radiolucency usuallyoccur within the coronoid process, but the condylar processof the mandible is not affected (Hitomiet al, 1996, 1997).No subperiosteal bone apposition is observed (Gru¨nebaumand Tiqva, 1973). Radiologic changes in the maxilla andmandible are similar. Maxillary sinuses can be entirelyopacified but when disease regression occurs, they regaintheir normal pneumatic condition (Caffey and Williams,1951). Contrast-enhanced T1 weighted magnetic resonanceimaging revealed lobulated gross masses in the jaws thatwere well enhanced. Whole-body skeletal bone scans with99mTc methylene diphosphonate revealed cold areas in bothjaws. 67Ga-citrate-scintigrams clearly confirms a non-neo-plastic bone lesions (Hitomiet al, 1996, 1997). Betweenthe 8th and 12th year of life formation of a delicate osseoustrabeculae within the translucent areas, starting from theinside of alveolar processes can be observed (Seward and


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Figure 13 Patient Ag.S at the age of 13—the malady stopped. Well vis-ible swelling in the regions of corpus and angle of mandible

Figure 14 Patient Ar.S (case record no. 108/88). In the picture a visiblecyst-like, multilocular lesion in the left body and angle of mandible isobserved

Hankey, 1957). The bone structure comes close to normalbetween the 3rd and 4th decade of life (Khosla and Korob-kin, 1970). In our study there was no significant correlationbetween the age of first symptoms, and the duration ofactive growth of the disease (Table 1). Our patients are inII 0 and III0 grade of cherubism according to Fordyce’s clini-cal classification of the disease (1976) (Arnott, 1978). Inthose cases in which the clinical and histopathologicalresults are not in accordance with the radiographic findings,two main groups of diseases have to be considered in thedifferential diagnosis from the radiological point of view:(1) lesions that originate from the dental tissues and theneighbouring bone, (2) lesions that are part of a generaldisease that affects the bony skeleton (Gru¨nebaum andTiqua, 1973; Kaugarset al, 1992). The above radiologicalpoint of view is completed by clinical comparison ofcherubism, central giant cell granuloma and giant celltumour (Table 2).

Treatment protocols for cherubism are not well estab-lished. Spontaneous regression of the disease has beennoticed (Katzet al, 1992), but the frequency of its occur-rence is unknown, since most of the recorded cases havebeen surgically treated before reaching puberty (Riefkohlet al, 1985). Southgateet al (1998) suggest that the geneticdefect responsible for the localized increase in osteoclastsin cherubism is overridden and normalised by the physio-logically increased synthesis of sex steroids when childrenreach puberty. According to Hamner and Ketcham (1969)four methods of treatment can be distinguished: (1) await-ing for spontaneous stabilisation and remission of cherubiclesions, (2) teeth extraction from the sites of fibrouschanges, (3) osteoplasty of the jaws, and (4) curettage ofthe pathological lesions. Moreover during recent yearsexperimental use of calcitonin in treatment of cherubism isdescribed (Wadaet al, 1996; Southgateet al, 1998; Hartet al, 2000).

Davis suggests curettage of the affected tissue, preserv-ing the teeth as long as possible. He recommends bonegrafting only when there is risk of pathologic fracture(Daviset al, 1983). In some patients it is possible to removethe pathologic tissue by liposuction (Dubin and Jackson,1990). Riefkohlet al (1985) reported numerous recurrencesfollowing conservative curettage in a teenage patient. Basedon their experience, these authors recommended fullintraosseous curettage of pathologic tissue at a young age.Zachariadeset al (1985) totally removed the lesions andfilled the defects with lyophilised bone. They have observedgood results in the mandible but not in the maxilla. Onceattempted, radiation therapy (Rydosz, 1960) has been aban-doned due to a high risk of osteoradionecrosis, delay ondevelopment of the facial skeleton and potential failure offurther surgical interventions (McCledonet al, 1962; Pet-ers, 1979; Riefkohlet al, 1985). The question of optimalpatients’ age for surgery remains unanswered.

Joneset al (1950) found removal of teeth within theaffected bone followed by curettage, as the most effectivemethod. This would support the hypothesis that pathogen-esis is associated with development of the permanent den-tition. Therefore, extractions might be warranted (Jones,1933, 1965). In our clinic we have adapted a treatment pro-tocol, which does not differ from Jones’. It is based on


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Figure 15 Pedigree of family affected with cherubism. Phenotypic manifestation of cherubism (K P) was revealed only in one of five generations.Explanation of symbols: (K) affected male, (P) affected female, (l) normal male, (s) normal female, arrow—the propositus

Table 1 Data of disease in the family affected by cherubism

Patient Sex Age of first clinical Duration periods of Location Effect of operationalmanifestation active development treatment

(in years) (in years)

KS 6 13 Maxilla and mandible

WS 5 11 Mandible

An.S 5 9 Mandible Good

Ag.S 5 8 Mandible

Ar.S 6 9 Mandible

Table 2 Clinical comparison of cherubism, central giant cell granuloma,and giant cell tumour (Kaugerset al, 1992)

Cherubism CGCG GCT

Genetic transmission Autosomal No Nodominat

Age at diagnosis (yr) 1.5–10 10–30 20–40

Sex predilection Male Female Female

Jaw location Entire jaw Anterior Raremandible

Painful No No Yes

Pathologic fracture No No Yes

Bilateral Yes Yes/No No

Recurrences rate — 13% 30% to 62%

CGCG, central giant cell granuloma; CGT, giant cell tumour

permanent, systematic observation and follow-up. Surgicalindications to extraction are: teeth significantly displacedand/or considerably loosened at pathologic foci. The treat-ment regimen involves also surgical intervention to contourimprove the shape of the face in specific cases.

Concluding, the autosomal recessive pattern of cherub-ism inheritance can be suspected in selected families.Further observation of the family presented here is neces-sary to confirm this pattern. Surgical treatment is unnecess-ary unless functional or emotional disturbances develop.

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cherubism—clinical picture and treatment

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