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Clinical evaluation of osteopetrosis
ORAL PATHOLOGY General Section .
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CLINICAL
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EVALUATION
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OF OSTEOPETROSIS
Report of a Case Ralph 8. Kaslick, B,rooklyn, N. Y. Department
A.B., D.D.S.,
a.nd Henry
C. Brustein,
B.S., D.M.D.,
of Dentistry, Jewish Chronic Disease Hospital
a rare disorder cha.racterized by hardness and brittleness of the bones,l has a va.riety of interesting medical and dental manifestations. Only 200 C’RSCSof this disease ha.vc ~CCI~ w]m-ted sinec 1904 when Albcrs-Schiinherg recognized osteopet.rosis a~ a separa.te clinical cntit.y.ll
0
STROPRTROSIS,
RRVLEW
OY THE
MEDICAL
LITRRATURE
EtioZoqy.-The cause of osteopetrosis is unknown, but. inheritance is said to play a role in the development of the diseasr. The mode of inheritance is sometimes recessive and sometimes d0minant.l Ynth.oloyy.-The pathologic changes in this disease are primarily in the bone. The cortex, as well as the trabcculac, is thickened. In the shaft, the ma.rrow spaces arc reduced in volume. The marrow nlay contain abnormal cells, or it n1a.y undergo fibrous changes. The foramina for cranial nerves are often constricted by overgrowth of bone. In the skull, the base, which is of cartilaginous origin, is extremely dense and sclerotic.‘, If The sella tureica is sclcrot,ic,’ and the clinoid process is club shaped.‘” “The chemical composition of the bones is normal,” and the serum calcium, inorganic phosphorus, and phosphatasc lcvcls are also within the normal range.‘, 2 The disease process is generally attributed to a lack of ostcoclastic bone resorption without excessive bone formation.” According to Bailey’s Textbook of Histology,20 “ The fact that osteoclasts arc usually present where bono and Long
Presented before a joint laland Jewish Hospitals
Clinical on Nov.
Oral Conference 18, 1959, at the i.1
of the Jewish Chronic Jewish Chronic Disease
Disease Hospital.
and
c&Aied cartilage arc un(lcrgoirlg ;~l~sor~~fic)tl l(vl t 48 Illi* itll’c~rcwc: l.llill tllc~)\\‘(‘I? the cells responsible IOP rcsorpt.ion. II is ill-t txvli~ IL 10 at1 rihul(s tllv ;iC)sc,rpiiotl ol’ bone to these cells, but. t 1~17~ is IIO cliwc4 (~\+ltwvc~ 1 II;I~ 1111~y in I-(’ t Ircb ;~vti\:c z1gcnt.sin resorption of either ~)OIIP or (‘:I rtilag(1.” As Wcinmann and 8iclu.P l)oitlt, out. “‘I’Ii(~ lavlr 01’ t~c~sorption S(:(~IIIH to h(a the consequence of failure iii liming 01’ hon(* i~~)~~sitiofi ;lnc.l rcsorpt.ioii.” Osteopctrosis may dcrclop when (~ndo~hond~;lI ossificaation is dist-ut$rtl 1,~ a lack of resorption of the cart ilaginons intc~r(*~ll~ll;1r g~*owltl suhstancc. Isl;~ntls oi this partly calcified giv~nntl siihstanw. which ntldc~r nornlal c*ontlitioris ilrt’ roplaced by bone, persist and Hr(I f’oa11d in I hcl sh~~ft’tsol’ the lmw. Snapper’ stat.rs that. utll’ort,un:ltclyr? p~~tscx~t-day fincliugs indicate that the pathologic changes of OSIclopct.rosis c*annot 1~ cbxpl;lincd by decreased ~JOIW rcsorpbion alonr. Ent.icknap mclntionrd thilt. in the hone tihtilincd at the antops> of his pa.t.irnt. with osteopctrosisY both ostcoblastic and ostcoclastic activit.y were found to br within normal 1in1it.s.’ The Swc4ish scimltists, Xngfelclt? Engstriim7 and Zettcrstrtim, drmonstrattul thilt. in the abnormal bone the filling-up of the! marrow spaces was caused by both escessive ralcifiaa.tion ant1 int.ense bone resorption which W~PC going on a.t the sil.lu(b time>.’ Since typical areas OCl)cwe resorption MII 1~ tlrrnonstrated. a11 inhibition of osteoclastic bone rcsorptioll (~illl~lot 1~ th(b only C;IWW of ostcopcbt rosis. This negat.es most of the current. ~lsI)lana.tions oC t hc pathogcnesis of th(b disease.’ Snapper” continues : ” The Swedish investiga.tors (Engfeldt ct. al.) cunelude t.hat the normal bonc~ rcso~~lwil by ostooclastic~ activity is t*cl>litcetl by immature hone? which II~~VVI: clcvc~lops to mat.urity. The replncrmc~nt of this immature bone is then inhibited. ’ * In the Y;L~ILC win, Zcttc~rst.riinl” c~nclut Lcs tha 1. the! ostropcttrous lm~e is built. up by all t.hose tyljcs of skeletill t.issucr which appear during the normal d(~velopment of bot~s, the Ill;litl abnormality bcitlg it lack of removal of this new ske1cta.l tissue onw it hs lwcu formed. S~ln.ppcr and Zet.tcrst.riim il.pl)e;lp to diWct* OIL the question OC whether or Snappcbr states that ostoopctrosis IlOt, ostcopct~rosis is il 1Il~~fill~i~li~! lllIllOlaI~lillit~. is not. the result of an alnlormality of calcium mcta.bolism, whereas Zetterstriinl stat.cs that osteoixltrosis d~vc~10ps as I he eonsequcnre ot’ ;I. tllct.abolic tlysi~unction of the bone-forming tissue. Metabolism is clcfinctl :IS the sum of chemical tissue changcs.L!’ Chcrnskin and LangleyIs help to c1ariC.v the situation somcwhal; by saying that t.hc changes in ostcopctrosis are causctl not, by a chemical alt.eratiou in the bonr but 1,~ a structural defect. The disease afYcct.s th(l CII~iron sk(ll(?ton.” Thcrc ;Ic
Volllmr Numlwr
1i
I
OSTEOPBTROSIS
73
a scarcity of collagen fihrils has also been reported.’ “The fragility of bone in marble bonr! disease is thr eonsequcncc! of its sf.ructurc)? not its chrmical composition. “‘J The outstanding feature ot’ osteopetrosis is the bilateral symmetrical increase in tlcnsity of the bout with loss of the trabcculnr pattctrn. The cliscase is always syinmet.t*ici~l .2 &mptoms.-Tho symptoms of the disease arc varied. A hypochromie a.nemia devel~q~s first and is usu:~lly followed IJY a mpclophthisic. anemia.‘? I2 11 shortening of the lift spat1 (hcntolysis) of the: rrd ~11s may also contribulc! to the ;lnenGa.‘, ” Mentality is ~or~ntll, although chronic illness, blindness, or ilcafnc~ may itltcr~cro with mental dcvt~lqn~ent.~ IZrittlrncsn of the imws predisposes to fracture.” 2plz ‘l’hesc fract.ures are l)t*olmbly due to fragi1it.y of I~OIU! caused hy the lack of irlaturcb lamc~llar bone and a high ratio of inorganic to organic matter.’ Osteomyelitis is frequent in the mandible. or the masill;l.Z, !J,12 l’iatt’ discusses a cast of ostcopctric osteomyelitis of the mandihle secondary to dental infection following tooth extraction. This occurred when the patient. was 10 years of age, and surgical rcmov;ll of the mandible was necessary one year liltt??. In ost,copetrosis, the head is square11 sl I1 and somewhat cnlargcd.’ Palmer” stat.es that unusual skull changes, such as defects in the vault and enlarged sphcnoid bones, wcrc found in his casts. ‘J’hc manclihlcs were, PC’culi;u~ly straighti?nc~il, and in all caxcs in Pallncr ‘s st.ud>- thrrc wtW nniltiplr fracturcbs and c~xophthulmos. One author statchs that t.hc teeth arc abnormal in
OF THR DENTAL LITERATURE
Congenital or early osteopetrosis, as it is discussctl in the literature, has many int.eresting dental and oral ramifications. SnappeP states that decay of the teeth is cx(*essive. Stai’m? says that I’(+sistance to infection is decreased with a tendency toward ostcomyclitix. Ill addition, both Stafnc and Pckarsky’ st,atc that dmwt.ur~d dt++rt.s ma.? (j~ur in these teeth and that their development and eruption may be retarded. Stafnc
concludes that in a. document&i C~SV the? pulp callamt)(hrs wc~re oblit(~t*;lt(~d ntrcl t II;I I the roots were small and d~arf~~d. Rocrlt~~~nog1.aI)lli~e~i?-~ Wittic~hl” also fw~~cl SOlllC failnrc Of root fortttalion aTIt it11 >tl)l)itIV’ttl ohlit(!ratiott 01’ Sottt(’ l)ltlI~ c~ha.tnbcrs in a case involving a 4,~e;~r-old rhild. In this caw, Wittivti ttlso fount! il dclaycd eruptive process. Only sfwtt t fwtlt ww ps rtially or .foll~- c!i*ul~tcCI. ilIli1 their occlusion was faulty. In order to inc~rcasc! I’llllCtiCJIlill c+Iicieiqv anil cxthctii3, all erupted teeth were cst txmii and fill I &nturcw ww mist rwtcvl. Thoma” statc:s that the rtangc~l’ of jaw tleerossis is so gIVilt tht vsi rat*1 ion ol’ it11 the tCCtl1 1XfOIP any bNWtll~! infec+ctl 1la.S l)c(~tl il(l\.O('ittd ilS il ~)t’WCtlt ivcs measure. Before any clstriictions atrl attcinptc4, however. certain prc~;~utiotis should be taken. The usual body drt’cnsrs against, infection and trauma ;It’(’ not. adequate, so postcstract.ion int’c4otl and ~IYIC~ un ;lw clasily p~du~tl. Thus, the exodontht ~110uld take 11nnsua1 prcIcaut.ions to prevent a I’racturta. ,Intibiotic therapy should bc institutctl prior to estractions to t*(4ucfc 1tic possibilit> of postoperative infection.‘” There is a tcndcncy toward fracture of the jaws iu osteopetrosis,‘. !‘, ‘I. I”. ” and in lateral plates t.ho tchc?th may appear to partieipatc in the discss(x process and become sclcrosetl. il.S iti the cast to lw t*(q)ot+rtl in this art.iclc. SWWill %c+tc~rstriitu.” authoI*s claim that th(l juwbotlc~s t)c~c*oltlc~c~stramc4y ~cMc.!‘~ “’ however, says that thcht*e is IIO itlcreasc in tlcnsity of the IttCltlt)l*ilII()us bones (for example, the llultlclit)le NII~ maxilla), although thr I)OIIC~S Itlil?- lt~ thickeuctl. M~I~~~MIIOL~S t)ollt’s ilrc altercltl littIc* if ;It iIll, it1 ttl:l rhle b011tl disease. ’ ’ la general? the older basal portion of t hr skull is proformcd in int.racartilage, whereas tlic ~NVPI* facial and roofing bones arc foimd ltlcalhratlonsly. There is, howe\-(1t*, so much fusion ;111(1overlapping 0-f these parts 21s t.hey bcwmct moltlrtl into ;I. coh(hrcnt whot(b that it is unwiscs to tl*y to draw too sharp it distinct.ion between thetll OIL the l);1sis of t~tt~lo(‘hOtttlt~ttl (It’ Ttlcl mechanic4 insufCionc,v ol’ the tl~nsc~, altercr! intramctrrlbraxious origin.‘” base of the skull Icads to a. mcrh;lnical insuficic~ncy 01: thcb whole c~rsnirun, ant1 iltI ,Scvoiidarily, tliv formtitiotl osteophytic rc~iriforcctncnt 011 the VilUlt is tllcb 1Wlllt. of ostcophytcs may load to a11 ostcoscIc!rosisof tlw hottrs of tttrh skull which :IW not primarily aficcted (for itistanco, thcr IllilSillil) .!’ Finally, it has been not ~1 that the air sinuses :I re oblitera.1 (VI 0~’ invisi,lc 11,12,1; &fore we begin the discussion of our findings in a c’asc of osteopetrosis. it might be well to discuss briefly the work of Bergman. In lZAF Rergtiian cbxtr>l(ttcd three deciduous tcclh of a fi-year-old boy afflicted with osteoprtrosis. The t&h wcrc practically 11or111al in cst.c~lal shape, but cxamini~tioll Of gIWlltl(l and decalcified scct.ions of the teeth rcrcaled rcsorpt.ion of roots, w-i&led lines of Owen in the dentine, slight, cnamt4 hypoplasiu, ilnd circumpulpal irrc>guL I* scheondarydentine. 1)ent;ll radiographs indi(a;lted aplasia of FXWHXI p~rm~~(?nt l.eeth, impaction of most of ttir deciduous t rcth. and ma1formation Of the iw~\VllS of the permanent upper incjsors.“’ :[n 1956, Bergman examincsd another (ails(’of ostcopetrosis, this time in ;I 5-year-old girl. Only the deciduous central incisors had erupted. These t.eet.h wcrc mobile and were cliscolored toward the gingivac. Radiographs showrd
tl~ar~itml destruction of bone in the incisor region, malforma~tion of the crowns au11 roots of many deciduous and permanent teeth, and aplasia of several twth. A study WRS made of dcca.lcified and ground se&ions of the unerupted tcbc’th. Five teeth were removed and sectiouecl. Macroscopically, four teeth had tlcformcd crowns and poorly devclopcd roots. !l’hcre was enamel hypoplasia in I’our teeth, but. in all five tect.h the dentinal structnrc was fairly regular. Thth pulp was rclativcly normal in all the t&h. and miueraliza.tion of &utinc was al)l)arelltly normal wit.h small interglolmlar sl)aCes apl~rariug in only two of the tec4h. l.‘arts of the jawbone attached to the teeth were studied, and an inunaturc~ tibrillar bout wit.h few signs of remodeling was tliscovcrrtl. Osteomyelitis was also evident.‘:’ ~~I*~I~I;IIL postulates two possible reasons for the cilaol(~l tlcfect,s : 1. The factors
giving
rise to the skeletul
changes directby
a.fectrd
This theory is cont.raindicatcd, howev(lr, because in some Teetb teeth thr enamel was normal whereas in others it was abnormal. dcvc4oping simult.ancously should present similar dcfttcts, but it is also possible for the teet.h which dcvelopcd enamel hypoplasia to have greater sensitivity to the general factors of the disease. 2. Local fnr:tors ore the ccr.?hse of eaanre[ defects. The dist.urbances in development of t.hc enamel duch to systemic factors gcncrall- have their counterpart in the dent.inc iu the form of tlisinrbanrcs in the mincralization (for example, rickets a.nd fluorosis) . 1 lere, there is no dist.urbancc iu dcntinc mineralization. The presence of a nonspecific chronic osteomyelitis ma.y be responsible for the enamel hppoplasia. and In osteopetmsis resorption of oven the disturbance of root formation. the bone is Mardcd. Kormal tooth growth and eruption requires active? remodeling of the surrounding bone. Normal t.oot h dc~velopmrnt may br inhibited merely by a lack of xpacc for tha growing germ.
anaelogsnesis.
Wo nmy deduct from the foregoing facts that nomruption of the teeth may be connected with the absence of bone rrsorption, with t.bc ost.comJ-elitis, and with the arrested dovclopment of the roots.‘:’ J.t appears most likely that the dental changes in ostcopetrosis are (1011~ ncetcd wholly or principally with the skeletal discasc which, on the other hand, probably has no prinmry influence on the dcveloprucnt of the teeth. The dist urbances in dental development appear instead to bc indirectly connected with the skeletal disease and to be caused by the retarded rcmodcling process of tbc bone in combination with the apparclntly not iinconi~noii chronic ostromyelitis of the jawbone. I3 CASE
REPORT
lwrn
Micdicwl on Jww
Findings 17, 1955,
and and
Comments. The patient, n white boy of Italian died on OeD. 2, 1959. \Vlwn tlw child was 8 days
extraction, was old, his motllel
During but the torso vory thin but creased from 27 inches on
the! ensuing years, the! c-hilt1 ~l~~~lol~c~~l iill VX! rcmr~l~ Iargt~ hc~n11 ant1 racldlo-nostl, and extremities romainctl rcblativcly normal in proportion. The patient became muscular, and hc had an cxti-emoly JJZLIV coml~lcsiou. TIN: head mcasurcmcnt in23 inches on Oct. 1.1, 1957, to 23.5 iuchcs on Ckt. 19. 1957, and eventually roached July 10, 1.959. Kormally, t.lu’ value of thcl head mrasurcmont 011 July 10, 1959, should hsvc l~ccn npproxinlatcly 20 inc*hcs .\ sligll t hylmcllromic’ iLll~Wli~l :1180 d~~v~~lop(!tl. A comp1et.c: nasal ol,struc.tion c~~~ilunll~~l~c~lopt~~l IWC~UW of’ an overgrowth of thr inferior t.urbinatcs, ctnd a progrckvch ol)tie :ttrol>lly alsO appearetil. Sarrowing of the facial canal led to involvement of the farial nerve. Wit: patient suffnrc~l a right-si(lt!tl seventhnerve paralysis, and later there was :L left,-sided paralysis, alrectinp the ~nuscles of facial expression, the stylohyoitl anI1 platynnln muscles, antI tbc posterior l~elly of tbc tligastric muscle. A narrowing of the foramen I~I~~IIIIIII also occurr(4. .\ pIIeumoe~lcephalogrnnl showed a bilateral hydrocephalus. The boy was rather poorly nourishe~l in 1he lotcar stages of the tliseuse, eating only pureed regular foods and some I)al)y foods. At the age of 3 years 5 months, the chill1 hall the emotional and intellectual development of a l-year-old. Steroid therapy was atte~nptc(I, sinr*e osteoporosis is sometimes a side eftcct of cortisone therapy. Meticorten was a(lminist.ered from July 2, 1956, until Nor. 2: l95G, with no definitely observable effects. On Feb. 14, 1957, a chronic acidosis was observed. Ik. 1. S. Friedman, attending physician, commented on the medical chart as follows: “ Chronic acidosis and osteopetrosis are an unusual combination. The acidosis is primarily renal tubular in origin. There is
1’01 ume I .;
OSTEOPETROSIS
Number I
77
It is difficult to account for the abnormal bone calcificaalso increased K+ in the urine. tion. Ordinarily tinder conditions of acidosis there is calcium loss.” Hp Sept. 30, 1959, however? the aci(losis had disappeared and :L pH reading of 7.35 was recorded. Ha~liogrttphs showe(l II diffuse sclerosis oli all hottes ant1 an extreme thicktdng of the skull. Ott Jntt. 16, 1956, 1)r. E. M;Iolt,on, a hospital radiologist, stated: “Examination of the skull (I*?#. :! ) rc~~:~ls c!citl(~ttc*c of nmrkd w(.h*roxis involving tltca frontal, ntaxillnr~, trmporal. Marked selcrosis is :tIso prrsettt in portiotts ttintidil~ular, ttrul it ~mrtioti of tlto occ:ipit:tl Ilotte. of t.hc ribs and 1mUt clxvioles. Sclerotic. patches are also seen in both humeri, radii, and Fig.
Pig. Fig.
2.-Lateral t.-Lateral
view view
Fig. of skull of skull
2.
3. at the WC of 7 months. at 3 years 8 months.
ulnae. The lower extremities reveal patches of sclerosis in both femurs, and right tibia.” On Feb. D LL, 1956, Dr. A. Lrwitan, director of radiology, noted nmrkd sclerosis of the base of the skull. A pncumoc!llc~cpltto~r~ltt on I?ol~ IX, 1957, ~howc11 ntt oldrtdiott, probably below t.ho fourth vcntriclc. Tltc hydrnecphah~a appeared grcaater on the right side on Ort. 30, 1957.
Kxt raorally, the maxilla and mandi talc appcarcd c~nlargc~d, primarily in the anterior gave the patient a typically This ~~henomcnon region with only slight posterior enlargement. ’ ‘ square head and jaw. ” A slight asymmetry was not.ed. The ala-tragns line measured 134 mm. on the right side but only 130 mm. on the left. 4 slight deviation of the nasal septum WlS I!\itl,!nt. Thn right-sided paralysis of the screnth nerw, iuvolving the platysma and the powterior IwIly of the digantric muscle, caused a cwt.ain amonut, of limitation of fnnction and deviation. There was a left-sided deviation on jaw opening. Tlio jaw was deviated, perhaps beeanw the mandible was drawn to one side by tht! action of the digastric mnselc! on the same sidc.l; The digastric muscle on the opposite side was paralyxcd. The digast.ric can deprws tho mandil~lc only wl~(w the hyoid bone is fixed.17 The ph~t~yama mnselo clcprosws the mandible lmt only against rcrititant 2(a,16 and this occurs only wl~tw th neck is tcwrrc.li
Fig.
5.-Intraoral
photograph
showing
relationship
of teeth
in
closed
position
of mandible.
In this child the wvcwth-iiww paralysis also somfwliat inhibited swallowing. Upon intraoral examination (Fig. B), it, was ol~serrc~l that tlw upper mueolul~ial fold had lwcn oblitcratad lay the ovrrgrowtli of the maxilla. The loww mncolabial fold was normal. Gingival rwession wax found on the upper right deciduous ctwtral incisor, and the gingiva appeared dry, red, and enlarged around the anterior teeth. This dry mont.h and gingirit.is wore duo part,ly to month breathing canwd by the uasal obrtrwt.ion and partly to poor oral hygiene. A slight ororljite was also prwent, a11d tlw uplwr and lower right deciduous enspids were the only tcclth that mad,~ caontaet in th(l c?losnd position of t.llc mandible (Fig. 5). Finally, the intraoral examination rcThe! 4iilt17 thcrc:forc, could not V~IPH.his food at, all. waled a somewhat mlargcd tongue and a tongue-thrust habit. There was also a wiclc wpariition lwtwwri tlw uplwr and lowc~r anterior twth in the c*lwwd posit.ion of t.hc m:ujdiljlr (opeu-1Ao j . Kadiogral~hic: clxamination (Fig. 6) revealed virtually no sArosis of t.ooth strnctnro ahical1 wcw not olditerated. The roots of the tcet.11 wcrc wid ahow~~d normal pulp el1amlwrs gcwrally timall and dwarfed, and tl1e alveolar l~ono appwwed very tl~nsc:. Frw strwtnral defects ww lnwont, although thcw swmc~tl t.0 Ire some ~wtiii1~1 lypoplasia (particularly in the twth roots of
of the lowr arch). t.lw upyr anterior
Iu
additioii,
thcrc:
was
a pwnliar
planting
arrangement
of
t.lw
maxillary sinus waH not visible. .Ln fact, there was a lack of pncwmatizat.ion (air content) of all the parauasal sinnscs. !l’hc! entire mandible was nclwosrd. the mental foramon was oblitwated, and the! mandihll~ WIR markedly ineroased in siw. A
ctoml~inat.io~t
of
t&h.
int,raoral
Tl~o
a~~1 ratliogralAie
were present and ernptctl and that all pwmaucnt
widcnc:c!
lrnds were
intlic~nlt~(l that in cvidcww.
all
deciduous
Honevcr,
teeth
the origi-
Textbook of Pediatriw, (~1. 4, Philadclphin, 194i, IV. 1. Mitclwll, -1. G., and Nelson, D-. $2.: 13. Hnunders Company, p. 1 l!M. 2. Rllapp", 1.: Isone Diseases, ,“r’cw York, l!Ki, Grune C Stratton, lq). .I, 1751XIl. s. \‘.: I&eases of the Mbuth, et]. 5, Ht,. l.ouis, I!Ml), The C. V. Moshy (:o~I3. Meml. Iklly, I’. x15. L. E., and McIntosh, R.: Diseases of Infuucy and Childhoo(l, ed. ‘II, Sew \Yorl(, +. Holt, 1939, Appleton-Century Company, Inc., p. 54X. 14:. C.: Philadelphia, 195S, W. 1% SauntJcrs 5. Htal’ne, Oral Roentgenographic l)lagnosis, ( VoInpaIIy, pp. 457, L’5x. 6. Palmer, 1’. E. H., and Thomas, J. E. P.: Ontcwpctrosis, &it. ,I. Ratliol. 31: 705, 195% 76: 1119, 1!XX. i. Piutt, A. I).: Benign Osteopetrosis, Am. .I. Roentgenol. 8. Pekarsky, R. L., and Grohowrski, A. J..: Osteopetrosis, NV\V York .J. Metl. 56: 001,
19Rfi 9. Weinmann, J. P., ant1 Sichcr, H. : Hone and Bones, cd. 8, St. Idouis, 1955, ‘rho (!. Y. Yosby Company, pp. l.BO-lfi3. 10. Wittich, H. C., Chaudhry, A. P., Gorlin, R. J., and Ktocsz, A. I~.: Osteopetrosis, J. Ibent. Chiltlren 24: 41, 1956. Review of the Literat.uret I~il~liothetca Pawliatrica II. Zetterstriim, R.: Osteopet.rosis; 66: 438, 1958. 12. Thoma, I<. H.: Oral Pathology, Pt. Louis, 1954, The (1. V. Mosby Company, pp. 513514,
IX 14. 1.5. 16. .l’i. 1.8. 19. ‘0. -*’ 1.
fi:WRl~i.
Isergman, G., Borggren, M. I<., ant1 Engfeltlt, I<.: Stuilies on Mineralize(1 1)ental TisPUCII, Acta odont. scandinav. 14: Xl, 19.56. Amlcrxon, W. A. I).: Pathology, ed. 3, St. Louis, 1957, The (1. V. hToslq- Company, pp. 1 l’f(i-l“o0. Cheraskin, IX., and Langley, L. J,.: Dynamics of Oral I)iagnoais, Chicago, 1956, The Tear Hook Publishers, Inc., p. 233. Cunningham, I). J.: Textbook of Anatomy, J,ontlon, 1!)53, Oxford University Press, p. +‘)c. Shapiro, H. Il.: Xaxillofacial Anatomy, Philadelphia, 1954, J. 11. Lippincott. Company, pp. 241, 2511. Patten, n. XL: Human Embryology, cd. 2, Sew Pork, 1953, The Rlakirton Company, p. 977. Stetlman’s Medical I)ictionary, Raltimore, 1953, Williams & \Vilkins Company, p. 8-E. Smith, P. E., and Copenhaver, W. M.: lsailey’s Textbook of Histology, ~1. 33, Daltimore, 1953, Williams & Wilkins Company, pp. 1’2, 123. Herbut, P. A.: Pathology, ed. 2, Philadelphia, 1959, Lea & Fchiger, p. lL’2!).
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.
CLINICAL
.
.
.
.
EVALUATION
.
.
.
.
.
.
.
.
.
.
...*..
OF OSTEOPETROSIS
Report of a Case Ralph 8. Kaslick, B,rooklyn, N. Y. Department
A.B., D.D.S.,
a.nd Henry
C. Brustein,
B.S., D.M.D.,
of Dentistry, Jewish Chronic Disease Hospital
a rare disorder cha.racterized by hardness and brittleness of the bones,l has a va.riety of interesting medical and dental manifestations. Only 200 C’RSCSof this disease ha.vc ~CCI~ w]m-ted sinec 1904 when Albcrs-Schiinherg recognized osteopet.rosis a~ a separa.te clinical cntit.y.ll
0
STROPRTROSIS,
RRVLEW
OY THE
MEDICAL
LITRRATURE
EtioZoqy.-The cause of osteopetrosis is unknown, but. inheritance is said to play a role in the development of the diseasr. The mode of inheritance is sometimes recessive and sometimes d0minant.l Ynth.oloyy.-The pathologic changes in this disease are primarily in the bone. The cortex, as well as the trabcculac, is thickened. In the shaft, the ma.rrow spaces arc reduced in volume. The marrow nlay contain abnormal cells, or it n1a.y undergo fibrous changes. The foramina for cranial nerves are often constricted by overgrowth of bone. In the skull, the base, which is of cartilaginous origin, is extremely dense and sclerotic.‘, If The sella tureica is sclcrot,ic,’ and the clinoid process is club shaped.‘” “The chemical composition of the bones is normal,” and the serum calcium, inorganic phosphorus, and phosphatasc lcvcls are also within the normal range.‘, 2 The disease process is generally attributed to a lack of ostcoclastic bone resorption without excessive bone formation.” According to Bailey’s Textbook of Histology,20 “ The fact that osteoclasts arc usually present where bono and Long
Presented before a joint laland Jewish Hospitals
Clinical on Nov.
Oral Conference 18, 1959, at the i.1
of the Jewish Chronic Jewish Chronic Disease
Disease Hospital.
and
c&Aied cartilage arc un(lcrgoirlg ;~l~sor~~fic)tl l(vl t 48 Illi* itll’c~rcwc: l.llill tllc~)\\‘(‘I? the cells responsible IOP rcsorpt.ion. II is ill-t txvli~ IL 10 at1 rihul(s tllv ;iC)sc,rpiiotl ol’ bone to these cells, but. t 1~17~ is IIO cliwc4 (~\+ltwvc~ 1 II;I~ 1111~y in I-(’ t Ircb ;~vti\:c z1gcnt.sin resorption of either ~)OIIP or (‘:I rtilag(1.” As Wcinmann and 8iclu.P l)oitlt, out. “‘I’Ii(~ lavlr 01’ t~c~sorption S(:(~IIIH to h(a the consequence of failure iii liming 01’ hon(* i~~)~~sitiofi ;lnc.l rcsorpt.ioii.” Osteopctrosis may dcrclop when (~ndo~hond~;lI ossificaation is dist-ut$rtl 1,~ a lack of resorption of the cart ilaginons intc~r(*~ll~ll;1r g~*owltl suhstancc. Isl;~ntls oi this partly calcified giv~nntl siihstanw. which ntldc~r nornlal c*ontlitioris ilrt’ roplaced by bone, persist and Hr(I f’oa11d in I hcl sh~~ft’tsol’ the lmw. Snapper’ stat.rs that. utll’ort,un:ltclyr? p~~tscx~t-day fincliugs indicate that the pathologic changes of OSIclopct.rosis c*annot 1~ cbxpl;lincd by decreased ~JOIW rcsorpbion alonr. Ent.icknap mclntionrd thilt. in the hone tihtilincd at the antops> of his pa.t.irnt. with osteopctrosisY both ostcoblastic and ostcoclastic activit.y were found to br within normal 1in1it.s.’ The Swc4ish scimltists, Xngfelclt? Engstriim7 and Zettcrstrtim, drmonstrattul thilt. in the abnormal bone the filling-up of the! marrow spaces was caused by both escessive ralcifiaa.tion ant1 int.ense bone resorption which W~PC going on a.t the sil.lu(b time>.’ Since typical areas OCl)cwe resorption MII 1~ tlrrnonstrated. a11 inhibition of osteoclastic bone rcsorptioll (~illl~lot 1~ th(b only C;IWW of ostcopcbt rosis. This negat.es most of the current. ~lsI)lana.tions oC t hc pathogcnesis of th(b disease.’ Snapper” continues : ” The Swedish investiga.tors (Engfeldt ct. al.) cunelude t.hat the normal bonc~ rcso~~lwil by ostooclastic~ activity is t*cl>litcetl by immature hone? which II~~VVI: clcvc~lops to mat.urity. The replncrmc~nt of this immature bone is then inhibited. ’ * In the Y;L~ILC win, Zcttc~rst.riinl” c~nclut Lcs tha 1. the! ostropcttrous lm~e is built. up by all t.hose tyljcs of skeletill t.issucr which appear during the normal d(~velopment of bot~s, the Ill;litl abnormality bcitlg it lack of removal of this new ske1cta.l tissue onw it hs lwcu formed. S~ln.ppcr and Zet.tcrst.riim il.pl)e;lp to diWct* OIL the question OC whether or Snappcbr states that ostoopctrosis IlOt, ostcopct~rosis is il 1Il~~fill~i~li~! lllIllOlaI~lillit~. is not. the result of an alnlormality of calcium mcta.bolism, whereas Zetterstriinl stat.cs that osteoixltrosis d~vc~10ps as I he eonsequcnre ot’ ;I. tllct.abolic tlysi~unction of the bone-forming tissue. Metabolism is clcfinctl :IS the sum of chemical tissue changcs.L!’ Chcrnskin and LangleyIs help to c1ariC.v the situation somcwhal; by saying that t.hc changes in ostcopctrosis are causctl not, by a chemical alt.eratiou in the bonr but 1,~ a structural defect. The disease afYcct.s th(l CII~iron sk(ll(?ton.” Thcrc ;Ic
Volllmr Numlwr
1i
I
OSTEOPBTROSIS
73
a scarcity of collagen fihrils has also been reported.’ “The fragility of bone in marble bonr! disease is thr eonsequcncc! of its sf.ructurc)? not its chrmical composition. “‘J The outstanding feature ot’ osteopetrosis is the bilateral symmetrical increase in tlcnsity of the bout with loss of the trabcculnr pattctrn. The cliscase is always syinmet.t*ici~l .2 &mptoms.-Tho symptoms of the disease arc varied. A hypochromie a.nemia devel~q~s first and is usu:~lly followed IJY a mpclophthisic. anemia.‘? I2 11 shortening of the lift spat1 (hcntolysis) of the: rrd ~11s may also contribulc! to the ;lnenGa.‘, ” Mentality is ~or~ntll, although chronic illness, blindness, or ilcafnc~ may itltcr~cro with mental dcvt~lqn~ent.~ IZrittlrncsn of the imws predisposes to fracture.” 2plz ‘l’hesc fract.ures are l)t*olmbly due to fragi1it.y of I~OIU! caused hy the lack of irlaturcb lamc~llar bone and a high ratio of inorganic to organic matter.’ Osteomyelitis is frequent in the mandible. or the masill;l.Z, !J,12 l’iatt’ discusses a cast of ostcopctric osteomyelitis of the mandihle secondary to dental infection following tooth extraction. This occurred when the patient. was 10 years of age, and surgical rcmov;ll of the mandible was necessary one year liltt??. In ost,copetrosis, the head is square11 sl I1 and somewhat cnlargcd.’ Palmer” stat.es that unusual skull changes, such as defects in the vault and enlarged sphcnoid bones, wcrc found in his casts. ‘J’hc manclihlcs were, PC’culi;u~ly straighti?nc~il, and in all caxcs in Pallncr ‘s st.ud>- thrrc wtW nniltiplr fracturcbs and c~xophthulmos. One author statchs that t.hc teeth arc abnormal in
OF THR DENTAL LITERATURE
Congenital or early osteopetrosis, as it is discussctl in the literature, has many int.eresting dental and oral ramifications. SnappeP states that decay of the teeth is cx(*essive. Stai’m? says that I’(+sistance to infection is decreased with a tendency toward ostcomyclitix. Ill addition, both Stafnc and Pckarsky’ st,atc that dmwt.ur~d dt++rt.s ma.? (j~ur in these teeth and that their development and eruption may be retarded. Stafnc
concludes that in a. document&i C~SV the? pulp callamt)(hrs wc~re oblit(~t*;lt(~d ntrcl t II;I I the roots were small and d~arf~~d. Rocrlt~~~nog1.aI)lli~e~i?-~ Wittic~hl” also fw~~cl SOlllC failnrc Of root fortttalion aTIt it11 >tl)l)itIV’ttl ohlit(!ratiott 01’ Sottt(’ l)ltlI~ c~ha.tnbcrs in a case involving a 4,~e;~r-old rhild. In this caw, Wittivti ttlso fount! il dclaycd eruptive process. Only sfwtt t fwtlt ww ps rtially or .foll~- c!i*ul~tcCI. ilIli1 their occlusion was faulty. In order to inc~rcasc! I’llllCtiCJIlill c+Iicieiqv anil cxthctii3, all erupted teeth were cst txmii and fill I &nturcw ww mist rwtcvl. Thoma” statc:s that the rtangc~l’ of jaw tleerossis is so gIVilt tht vsi rat*1 ion ol’ it11 the tCCtl1 1XfOIP any bNWtll~! infec+ctl 1la.S l)c(~tl il(l\.O('ittd ilS il ~)t’WCtlt ivcs measure. Before any clstriictions atrl attcinptc4, however. certain prc~;~utiotis should be taken. The usual body drt’cnsrs against, infection and trauma ;It’(’ not. adequate, so postcstract.ion int’c4otl and ~IYIC~ un ;lw clasily p~du~tl. Thus, the exodontht ~110uld take 11nnsua1 prcIcaut.ions to prevent a I’racturta. ,Intibiotic therapy should bc institutctl prior to estractions to t*(4ucfc 1tic possibilit> of postoperative infection.‘” There is a tcndcncy toward fracture of the jaws iu osteopetrosis,‘. !‘, ‘I. I”. ” and in lateral plates t.ho tchc?th may appear to partieipatc in the discss(x process and become sclcrosetl. il.S iti the cast to lw t*(q)ot+rtl in this art.iclc. SWWill %c+tc~rstriitu.” authoI*s claim that th(l juwbotlc~s t)c~c*oltlc~c~stramc4y ~cMc.!‘~ “’ however, says that thcht*e is IIO itlcreasc in tlcnsity of the IttCltlt)l*ilII()us bones (for example, the llultlclit)le NII~ maxilla), although thr I)OIIC~S Itlil?- lt~ thickeuctl. M~I~~~MIIOL~S t)ollt’s ilrc altercltl littIc* if ;It iIll, it1 ttl:l rhle b011tl disease. ’ ’ la general? the older basal portion of t hr skull is proformcd in int.racartilage, whereas tlic ~NVPI* facial and roofing bones arc foimd ltlcalhratlonsly. There is, howe\-(1t*, so much fusion ;111(1overlapping 0-f these parts 21s t.hey bcwmct moltlrtl into ;I. coh(hrcnt whot(b that it is unwiscs to tl*y to draw too sharp it distinct.ion between thetll OIL the l);1sis of t~tt~lo(‘hOtttlt~ttl (It’ Ttlcl mechanic4 insufCionc,v ol’ the tl~nsc~, altercr! intramctrrlbraxious origin.‘” base of the skull Icads to a. mcrh;lnical insuficic~ncy 01: thcb whole c~rsnirun, ant1 iltI ,Scvoiidarily, tliv formtitiotl osteophytic rc~iriforcctncnt 011 the VilUlt is tllcb 1Wlllt. of ostcophytcs may load to a11 ostcoscIc!rosisof tlw hottrs of tttrh skull which :IW not primarily aficcted (for itistanco, thcr IllilSillil) .!’ Finally, it has been not ~1 that the air sinuses :I re oblitera.1 (VI 0~’ invisi,lc 11,12,1; &fore we begin the discussion of our findings in a c’asc of osteopetrosis. it might be well to discuss briefly the work of Bergman. In lZAF Rergtiian cbxtr>l(ttcd three deciduous tcclh of a fi-year-old boy afflicted with osteoprtrosis. The t&h wcrc practically 11or111al in cst.c~lal shape, but cxamini~tioll Of gIWlltl(l and decalcified scct.ions of the teeth rcrcaled rcsorpt.ion of roots, w-i&led lines of Owen in the dentine, slight, cnamt4 hypoplasiu, ilnd circumpulpal irrc>guL I* scheondarydentine. 1)ent;ll radiographs indi(a;lted aplasia of FXWHXI p~rm~~(?nt l.eeth, impaction of most of ttir deciduous t rcth. and ma1formation Of the iw~\VllS of the permanent upper incjsors.“’ :[n 1956, Bergman examincsd another (ails(’of ostcopetrosis, this time in ;I 5-year-old girl. Only the deciduous central incisors had erupted. These t.eet.h wcrc mobile and were cliscolored toward the gingivac. Radiographs showrd
tl~ar~itml destruction of bone in the incisor region, malforma~tion of the crowns au11 roots of many deciduous and permanent teeth, and aplasia of several twth. A study WRS made of dcca.lcified and ground se&ions of the unerupted tcbc’th. Five teeth were removed and sectiouecl. Macroscopically, four teeth had tlcformcd crowns and poorly devclopcd roots. !l’hcre was enamel hypoplasia in I’our teeth, but. in all five tect.h the dentinal structnrc was fairly regular. Thth pulp was rclativcly normal in all the t&h. and miueraliza.tion of &utinc was al)l)arelltly normal wit.h small interglolmlar sl)aCes apl~rariug in only two of the tec4h. l.‘arts of the jawbone attached to the teeth were studied, and an inunaturc~ tibrillar bout wit.h few signs of remodeling was tliscovcrrtl. Osteomyelitis was also evident.‘:’ ~~I*~I~I;IIL postulates two possible reasons for the cilaol(~l tlcfect,s : 1. The factors
giving
rise to the skeletul
changes directby
a.fectrd
This theory is cont.raindicatcd, howev(lr, because in some Teetb teeth thr enamel was normal whereas in others it was abnormal. dcvc4oping simult.ancously should present similar dcfttcts, but it is also possible for the teet.h which dcvelopcd enamel hypoplasia to have greater sensitivity to the general factors of the disease. 2. Local fnr:tors ore the ccr.?hse of eaanre[ defects. The dist.urbances in development of t.hc enamel duch to systemic factors gcncrall- have their counterpart in the dent.inc iu the form of tlisinrbanrcs in the mincralization (for example, rickets a.nd fluorosis) . 1 lere, there is no dist.urbancc iu dcntinc mineralization. The presence of a nonspecific chronic osteomyelitis ma.y be responsible for the enamel hppoplasia. and In osteopetmsis resorption of oven the disturbance of root formation. the bone is Mardcd. Kormal tooth growth and eruption requires active? remodeling of the surrounding bone. Normal t.oot h dc~velopmrnt may br inhibited merely by a lack of xpacc for tha growing germ.
anaelogsnesis.
Wo nmy deduct from the foregoing facts that nomruption of the teeth may be connected with the absence of bone rrsorption, with t.bc ost.comJ-elitis, and with the arrested dovclopment of the roots.‘:’ J.t appears most likely that the dental changes in ostcopetrosis are (1011~ ncetcd wholly or principally with the skeletal discasc which, on the other hand, probably has no prinmry influence on the dcveloprucnt of the teeth. The dist urbances in dental development appear instead to bc indirectly connected with the skeletal disease and to be caused by the retarded rcmodcling process of tbc bone in combination with the apparclntly not iinconi~noii chronic ostromyelitis of the jawbone. I3 CASE
REPORT
lwrn
Micdicwl on Jww
Findings 17, 1955,
and and
Comments. The patient, n white boy of Italian died on OeD. 2, 1959. \Vlwn tlw child was 8 days
extraction, was old, his motllel
During but the torso vory thin but creased from 27 inches on
the! ensuing years, the! c-hilt1 ~l~~~lol~c~~l iill VX! rcmr~l~ Iargt~ hc~n11 ant1 racldlo-nostl, and extremities romainctl rcblativcly normal in proportion. The patient became muscular, and hc had an cxti-emoly JJZLIV coml~lcsiou. TIN: head mcasurcmcnt in23 inches on Oct. 1.1, 1957, to 23.5 iuchcs on Ckt. 19. 1957, and eventually roached July 10, 1.959. Kormally, t.lu’ value of thcl head mrasurcmont 011 July 10, 1959, should hsvc l~ccn npproxinlatcly 20 inc*hcs .\ sligll t hylmcllromic’ iLll~Wli~l :1180 d~~v~~lop(!tl. A comp1et.c: nasal ol,struc.tion c~~~ilunll~~l~c~lopt~~l IWC~UW of’ an overgrowth of thr inferior t.urbinatcs, ctnd a progrckvch ol)tie :ttrol>lly alsO appearetil. Sarrowing of the facial canal led to involvement of the farial nerve. Wit: patient suffnrc~l a right-si(lt!tl seventhnerve paralysis, and later there was :L left,-sided paralysis, alrectinp the ~nuscles of facial expression, the stylohyoitl anI1 platynnln muscles, antI tbc posterior l~elly of tbc tligastric muscle. A narrowing of the foramen I~I~~IIIIIII also occurr(4. .\ pIIeumoe~lcephalogrnnl showed a bilateral hydrocephalus. The boy was rather poorly nourishe~l in 1he lotcar stages of the tliseuse, eating only pureed regular foods and some I)al)y foods. At the age of 3 years 5 months, the chill1 hall the emotional and intellectual development of a l-year-old. Steroid therapy was atte~nptc(I, sinr*e osteoporosis is sometimes a side eftcct of cortisone therapy. Meticorten was a(lminist.ered from July 2, 1956, until Nor. 2: l95G, with no definitely observable effects. On Feb. 14, 1957, a chronic acidosis was observed. Ik. 1. S. Friedman, attending physician, commented on the medical chart as follows: “ Chronic acidosis and osteopetrosis are an unusual combination. The acidosis is primarily renal tubular in origin. There is
1’01 ume I .;
OSTEOPETROSIS
Number I
77
It is difficult to account for the abnormal bone calcificaalso increased K+ in the urine. tion. Ordinarily tinder conditions of acidosis there is calcium loss.” Hp Sept. 30, 1959, however? the aci(losis had disappeared and :L pH reading of 7.35 was recorded. Ha~liogrttphs showe(l II diffuse sclerosis oli all hottes ant1 an extreme thicktdng of the skull. Ott Jntt. 16, 1956, 1)r. E. M;Iolt,on, a hospital radiologist, stated: “Examination of the skull (I*?#. :! ) rc~~:~ls c!citl(~ttc*c of nmrkd w(.h*roxis involving tltca frontal, ntaxillnr~, trmporal. Marked selcrosis is :tIso prrsettt in portiotts ttintidil~ular, ttrul it ~mrtioti of tlto occ:ipit:tl Ilotte. of t.hc ribs and 1mUt clxvioles. Sclerotic. patches are also seen in both humeri, radii, and Fig.
Pig. Fig.
2.-Lateral t.-Lateral
view view
Fig. of skull of skull
2.
3. at the WC of 7 months. at 3 years 8 months.
ulnae. The lower extremities reveal patches of sclerosis in both femurs, and right tibia.” On Feb. D LL, 1956, Dr. A. Lrwitan, director of radiology, noted nmrkd sclerosis of the base of the skull. A pncumoc!llc~cpltto~r~ltt on I?ol~ IX, 1957, ~howc11 ntt oldrtdiott, probably below t.ho fourth vcntriclc. Tltc hydrnecphah~a appeared grcaater on the right side on Ort. 30, 1957.
Kxt raorally, the maxilla and mandi talc appcarcd c~nlargc~d, primarily in the anterior gave the patient a typically This ~~henomcnon region with only slight posterior enlargement. ’ ‘ square head and jaw. ” A slight asymmetry was not.ed. The ala-tragns line measured 134 mm. on the right side but only 130 mm. on the left. 4 slight deviation of the nasal septum WlS I!\itl,!nt. Thn right-sided paralysis of the screnth nerw, iuvolving the platysma and the powterior IwIly of the digantric muscle, caused a cwt.ain amonut, of limitation of fnnction and deviation. There was a left-sided deviation on jaw opening. Tlio jaw was deviated, perhaps beeanw the mandible was drawn to one side by tht! action of the digastric mnselc! on the same sidc.l; The digastric muscle on the opposite side was paralyxcd. The digast.ric can deprws tho mandil~lc only wl~(w the hyoid bone is fixed.17 The ph~t~yama mnselo clcprosws the mandible lmt only against rcrititant 2(a,16 and this occurs only wl~tw th neck is tcwrrc.li
Fig.
5.-Intraoral
photograph
showing
relationship
of teeth
in
closed
position
of mandible.
In this child the wvcwth-iiww paralysis also somfwliat inhibited swallowing. Upon intraoral examination (Fig. B), it, was ol~serrc~l that tlw upper mueolul~ial fold had lwcn oblitcratad lay the ovrrgrowtli of the maxilla. The loww mncolabial fold was normal. Gingival rwession wax found on the upper right deciduous ctwtral incisor, and the gingiva appeared dry, red, and enlarged around the anterior teeth. This dry mont.h and gingirit.is wore duo part,ly to month breathing canwd by the uasal obrtrwt.ion and partly to poor oral hygiene. A slight ororljite was also prwent, a11d tlw uplwr and lower right deciduous enspids were the only tcclth that mad,~ caontaet in th(l c?losnd position of t.llc mandible (Fig. 5). Finally, the intraoral examination rcThe! 4iilt17 thcrc:forc, could not V~IPH.his food at, all. waled a somewhat mlargcd tongue and a tongue-thrust habit. There was also a wiclc wpariition lwtwwri tlw uplwr and lowc~r anterior twth in the c*lwwd posit.ion of t.hc m:ujdiljlr (opeu-1Ao j . Kadiogral~hic: clxamination (Fig. 6) revealed virtually no sArosis of t.ooth strnctnro ahical1 wcw not olditerated. The roots of the tcet.11 wcrc wid ahow~~d normal pulp el1amlwrs gcwrally timall and dwarfed, and tl1e alveolar l~ono appwwed very tl~nsc:. Frw strwtnral defects ww lnwont, although thcw swmc~tl t.0 Ire some ~wtiii1~1 lypoplasia (particularly in the twth roots of
of the lowr arch). t.lw upyr anterior
Iu
additioii,
thcrc:
was
a pwnliar
planting
arrangement
of
t.lw
maxillary sinus waH not visible. .Ln fact, there was a lack of pncwmatizat.ion (air content) of all the parauasal sinnscs. !l’hc! entire mandible was nclwosrd. the mental foramon was oblitwated, and the! mandihll~ WIR markedly ineroased in siw. A
ctoml~inat.io~t
of
t&h.
int,raoral
Tl~o
a~~1 ratliogralAie
were present and ernptctl and that all pwmaucnt
widcnc:c!
lrnds were
intlic~nlt~(l that in cvidcww.
all
deciduous
Honevcr,
teeth
the origi-
Textbook of Pediatriw, (~1. 4, Philadclphin, 194i, IV. 1. Mitclwll, -1. G., and Nelson, D-. $2.: 13. Hnunders Company, p. 1 l!M. 2. Rllapp", 1.: Isone Diseases, ,“r’cw York, l!Ki, Grune C Stratton, lq). .I, 1751XIl. s. \‘.: I&eases of the Mbuth, et]. 5, Ht,. l.ouis, I!Ml), The C. V. Moshy (:o~I3. Meml. Iklly, I’. x15. L. E., and McIntosh, R.: Diseases of Infuucy and Childhoo(l, ed. ‘II, Sew \Yorl(, +. Holt, 1939, Appleton-Century Company, Inc., p. 54X. 14:. C.: Philadelphia, 195S, W. 1% SauntJcrs 5. Htal’ne, Oral Roentgenographic l)lagnosis, ( VoInpaIIy, pp. 457, L’5x. 6. Palmer, 1’. E. H., and Thomas, J. E. P.: Ontcwpctrosis, &it. ,I. Ratliol. 31: 705, 195% 76: 1119, 1!XX. i. Piutt, A. I).: Benign Osteopetrosis, Am. .I. Roentgenol. 8. Pekarsky, R. L., and Grohowrski, A. J..: Osteopetrosis, NV\V York .J. Metl. 56: 001,
19Rfi 9. Weinmann, J. P., ant1 Sichcr, H. : Hone and Bones, cd. 8, St. Idouis, 1955, ‘rho (!. Y. Yosby Company, pp. l.BO-lfi3. 10. Wittich, H. C., Chaudhry, A. P., Gorlin, R. J., and Ktocsz, A. I~.: Osteopetrosis, J. Ibent. Chiltlren 24: 41, 1956. Review of the Literat.uret I~il~liothetca Pawliatrica II. Zetterstriim, R.: Osteopet.rosis; 66: 438, 1958. 12. Thoma, I<. H.: Oral Pathology, Pt. Louis, 1954, The (1. V. Mosby Company, pp. 513514,
IX 14. 1.5. 16. .l’i. 1.8. 19. ‘0. -*’ 1.
fi:WRl~i.
Isergman, G., Borggren, M. I<., ant1 Engfeltlt, I<.: Stuilies on Mineralize(1 1)ental TisPUCII, Acta odont. scandinav. 14: Xl, 19.56. Amlcrxon, W. A. I).: Pathology, ed. 3, St. Louis, 1957, The (1. V. hToslq- Company, pp. 1 l’f(i-l“o0. Cheraskin, IX., and Langley, L. J,.: Dynamics of Oral I)iagnoais, Chicago, 1956, The Tear Hook Publishers, Inc., p. 233. Cunningham, I). J.: Textbook of Anatomy, J,ontlon, 1!)53, Oxford University Press, p. +‘)c. Shapiro, H. Il.: Xaxillofacial Anatomy, Philadelphia, 1954, J. 11. Lippincott. Company, pp. 241, 2511. Patten, n. XL: Human Embryology, cd. 2, Sew Pork, 1953, The Rlakirton Company, p. 977. Stetlman’s Medical I)ictionary, Raltimore, 1953, Williams & \Vilkins Company, p. 8-E. Smith, P. E., and Copenhaver, W. M.: lsailey’s Textbook of Histology, ~1. 33, Daltimore, 1953, Williams & Wilkins Company, pp. 1’2, 123. Herbut, P. A.: Pathology, ed. 2, Philadelphia, 1959, Lea & Fchiger, p. lL’2!).