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Paget’s Disease: Report of a Case
PAGET’S DISEASE: REPORT OF A CASE By A l l e n T . N e w m a n ,* D .D .S ., and M i l t o n H y m a n ,f D .D .S ., N e w Y o rk , N. Y .
A G E T ’s disease, or osteitis deform ans, is o f unknow n etiology. It is characterized by an initial bone re sorption, w h ich is follow ed b y bone d ep osition. T h e deposited bone is thick and porous, in volvin g b oth the cortex and spongiosa, w ith a consequent increase in bone volum e. T h e entire process is a slow one. T h e bones most com m on ly affected are the tibia, skull, pelvis and fem u r.1 These becom e thickened and deform ed, and the lim bs bow ed. T h e skull b e comes enlarged and square across the front. C yst form ation m ay occu r in the legs. T h e patients often com plain that they m ust b u y new hats, since their old ones are too sm all. T h e disease is m ost com m on in m iddle life or later.1’ 2 T h a t the condition has been considered rela tively rare, Stafn e and A u stin 3 attribute to the fa ct th at only the m ore advanced forms have been recognized. The roentgenographic pictu re of P aget’s disease probably is dependent on the stage a t w h ich the disease is seen. Since this is a den tal report, only the skull and jaw s w ill b e considered. Osteoporosis circum scripta is considered an early osteolytic phase affectin g the skull. In the later stage, the skull has a typical “ cotton-w ool” appearance. In a study o f 138 know n cases o f P aget’s disease reported by Stafn e and A ustin, 58 per cent o f skull pictures presented definite evidence o f the disease. In only tw entythree cases did they find definite in volve m ent o f tha m a xillae and m andible, tw enty in the m a xillae and three in the
P
From the New York University College of Dentistry. *Dean. fA ssistant Professor of diagnosis.
Jour. A.D.A., Vol. 30, February 1, 1943
m andible. T h is seems to b ear out other findings th at the m andible is rarely a f fected. T h e cases reported in d en tal literature show a w id e varian ce in roentgeno graphic findings. F o x 4 fou n d cystic in volvem ent o f the apices o f the m axillary teeth, hypercem entosis o f the roots and a feath ery spongious appearan ce of the m axillary bone. Seldin6 described a b ila t eral involvem ent of the m andible, w ith alternate areas of resorption and calci fication, and hypercem entosis, evid ent in the roentgenogram . C a h n 6 reported a characteristic hazy structure of the bone due to an increase in the n um ber of trabeculae. Islands of hypercalcification w ere also noted. Stafne and A u stin w ere fortu n ate in being able to follow up cases o f P aget’s disease. A s a result o f their study, they stated that the roen tgenographic ap p ear ance probably depends on the stage at w hich the disease is seen. In the early stages, the dom inant featu re is probably osteoporosis, w hile, in the ad vanced stages, the presence o f osteosclerotic areas m ay result in an uneven m ottled ap p ear ance. In 17 per cen t o f their cases, they found fine, close trabeculations, w hich in variably occurred in those patients show ing P aget’s disease o f the skull. H ow ever, they considered these va ria tions to be w ith in norm al limits. REPORT OF CASE
History.— F. H ., a man, white, aged 70 , com plained of pains, of three years’ dura tion, in the right leg, arm, both knees and back and across the chest. D uring the past year, he had been having trouble w ith his hearing. H e had been treated b y physicians without relief. H e wished to have his teeth 237
238
T h e J o u r n a l o f t h e A m e r ic a n D e n t a l A s s o c ia t io n
Fig. i.-— Paget’s disease involving the m axillae; showing osteoporosis of anterior region and radiopaque appearance of remainder of alveolar bone. T he mandibular bone is normal.
Fig. 2.— Skull showing characteristic “ cotton-wool” or “ cotton-puff” appearance. The bones of the calvarium are thickened, with very fuzzy inner and outer tables.
N e w m a n a n d H y m a n — P a g e t ’s D is e a s e
239
exam ined to determ ine w hether they might be a focus of infection. Physical Examination. — T he patient weighed 156 pounds and was 5 feet 7 inches tall. T here was nothing of special im por tance in the mouth. T he occlusion was within norm al lim its, but the m axillary ridges were very heavy, ranging from 1 to 1i inches in width. Roentgenographic Findings.— 1. M ax illary region: T he m axillae showed m arked altera tion. (Fig. 1.) Osteoporosis was noted in the region from the upper left central in cisor to the cuspid, and the right central
Fig. 4.— Osteoporosis between central in cisors and central and lateral incisors. (Com pare Fig. 5.)
Fig. 3.— Right femur with cyst formation, most common in leg bones.
incisor to the second bicuspid regions. The rem ainder of the bone seemed to have lost its norm al trabeculation. It was radiopaque, granular and hazy in appearance, the trabec ulae being liner and more closely woven. No hypercementosis of the roots was observed. 2. M andible: The m andible was normal. 3. Skull: T here was a typ ical “cotton wool” appearance of Paget’s disease. (Fig. 2-)
4. R ight fem ur: Cyst formation was evi dent. (Fig. 3.)
Fig. 5.— Normal anterior radiolucency, most marked between central incisors and right central and lateral incisors in patient aged 47. M edical Report .— On the first visit, M ay 31, 1940, the patient com plained of burning pain of the right thigh. A roentgenogram suggested central chondroma or chondro
240
T h e J o u r n a l o f t h e A m e r ic a n D e n t a l A s s o c ia t io n
sarcoma. Aspiration biopsy disclosed an in flammatory lesion, possibly xanthomatosis. The patient was given small doses of the x-rays in this area. October 1940, there was no change in right femur, but the left femur presented the appearance of Paget’s disease. The roentgenologist stated that the x-ray appearance of the right thigh might be due to a healed cystic condition, and' that both lesions were probably due to Paget’s disease. com m ent
Stafn e and A ustin a ttach m uch im portance, from a diagnostic view point, to the osteoporosis fou n d in the m axillary bone. (F ig. 4.) H ow ever, these authors wish to stress the inadvisability of p la c ing too m uch em phasis on this point alone. W h ile the osteoporosis m ay be
Fig. 6.— Normal bone trabeculations in m axilla; showing size, shape and number of trabeculae as compared with those in Figure 7. Since the patient was 64 years of age, any variation due to age can be ruled out.
m arked, a sim ilar rad iolu cen t appearance can often be noted in otherwise norm al cases. (Fig. 5.) T h is norm al radiolucence m ay be due to the thinness o f the alveolar plate at th at point, or it m ay be an op tical illusion created b y the contrast betw een the radiopaque, roentgenograp hic appearance o f the tooth as com pared w ith that o f the ad jacen t bone. H ow ever, the bone trabeculation of the m axillae does possess diagnostic value, as dem onstrated by this case. G reen field7 describes the norm al cancellous
bone as m ade up o f fibrils of bone that interlace and form a lattice-like or trab e cu late structure. R oentgen ographically, one finds a w hite netw ork enclosing ir regu lar black spaces. In the upper jaw , the b lack spaces appear sm aller and m ore regu lar than in the lower. T h e w hite netw ork is thinner. (Fig. 6.) E xam in ation o f the roentgenogram s in the case here reported shows th at the black spaces betw een the w hite trabeculae a p p ear very sm all and fine, and a great m an y trab ecu lae crow d the space n or m ally occu pied b y a single trabecula. (Fig. 7.) T h is increase in n um ber of the trabeculae has also been reported on m icroscopic exam ination. T his fine trabecu lation can even be seen in the osteo-
Fig. 7.— Paget’s bone trabeculations.
porotic regions o f F igu re 4, w h ile the norm al trabeculation shows quite clearly in the area o f anterior radiolucency in F igu re 5. T h e hypercem entosis reported by other investigators is not in evidence in this case, and m a y not be a constant factor. S U M M A R Y AND CONCLUSION
In a m an aged 70, roentgenogram s disclosed P aget’s disease involvin g the skull, fem u r and m axillae. T h e skull show ed the typical “ cotton-w ool” a p p e ar ance, the fem ur, cyst form ation. T w o separate phases w ere seen in the m a xillae : osteoporosis and osteosclerosis. T h e os-
S e id e s — M o u t h R e h a b il it a t io n
teoporosis m igh t be confused w ith norm al anterior radiolucency. T h e bone deposition took the form of an increase in trabeculae, giv in g the bone a hazy appearance. T h e m an d ibu lar bone was norm al. N o hypercem entosis was present. B I B L IO G R A P H Y
1. G e s c h ic k t e r , C . F., and C o p e l a n d , M. M .: Tumors of Bone. New York: American Journal of Cancer, 1936 , pp. 788 -79 2 . 2 . T h o m a , K . H.: O ral Pathology. St.
241
Louis: C. V . Mosby Co., 19 4 1 , pp. 866 -904 . 3. S t a f n e , E . C ., and' A u s t i n , L . T . : Study of Dental Roentgenograms in Cases of Paget’s Disease. J .A .D .A ., 2 5 :1 2 0 2 , August 1938 . 4 . Fox, L e w i s : Paget’s Disease (Osteitis Deformans) and Its Effect on M axillary Bone. J.A.D .A., 2 0 : 1823 , October 1933 . 5 . S e l d i n , N. A .: Paget’s Disease— Clinical Case. D. Cosmos, 7 5 :6 9 1 , July 19 3 3 . 6 . C a h n , L. R .: Paget’s Disease of Bone. Arch. Clin. & Oral Path., 1 : 1 4 1 , June 19 3 7 . 7 . G r e e n f ie l d , A. L . : X -R ay Technic and Interpretation of Dental Roentgenograms. Brooklyn: Dental Items of Interest Publishing Co., 1936 , p. 123 .
FACTORS OF OCCLUSION IN RELATION TO MOUTH REHABILITATION By H
arry
M . S e i d e s , D .D .S., N ew Y o rk , N . Y .
O U T H rehabilitation im plies that im pairm ent o f the elem ents w h ich contribute to th e m ain tenance o f m asticatory efficien cy has taken place. In some instances, this m ay result in loss o f the norm al m a xillo m an d ibular ve rtica l dim ension, often m od ifying fa cia l contour. Since m astica tory efficiency depends on a thoroughly equilibrated occlusion, the prim ary req uisite fo r successful treatm ent o f m outh rehabilitation is an understanding o f the factors in norm al occlusion and the m ethod o f correctin g discrepancies. T h e follo w in g is offered as a standard ized procedure fo r the purpose o f estab lishing a diagnosis and as a basis fo r treatm ent p la n n in g : 1. C lin ica l exam ination o f the m outh. 2. E valu ation o f habits as factors in occlusion. 3. A p praisal o f occlusal discrepancies a fte r m oun ting o f casts on an adjustable articulator. A clin ical exam ination o f the m outh is m ade and p a th o lo gic conditions o f the teeth, supporting parodontium and con
M
Jour. A.D.A., Vol. 30, February 1, 1943
tiguous structures are recorded. A n in tra-oral roen tgenograp h ic exam ination is m ade, and w here tem porom andibular in volvem ent is suspected, the tem poro m and ibu lar joints are also roentgenographed. W hen occlusal disturbances are evident, there is a b reakdow n in the o c clusal m echanism w ith resultant im p a ir m ent o f fu n ction . C lin ica l exam ination o f the occlusion should be given carefu l consideration an d the degree o f destruc tion o f the d ental tissues observed and recorded, in clu d in g the status an d fu n c tion o f the m u scu lature o f the jaw s. Sequelae o f occlusal discrepancies that m a y m anifest them selves clin ically are a ttritio n ; d riftin g o f th e teeth, occasion a lly coupled w ith alveolar resorption, and disturbances in the tem porom andib u la r capsule an d its ligam ents, o r a com bin ation o f these conditions. T h ese con ditions m a y result from th e loss o f a tooth o r part o f a tooth w ith a subse qu en t deficiency w h ich m a y also b e m an i fested as a disharm ony betw een con d ylar m ovem ent and tooth gu id in g inclines. (Fig- !•)
A G E T ’s disease, or osteitis deform ans, is o f unknow n etiology. It is characterized by an initial bone re sorption, w h ich is follow ed b y bone d ep osition. T h e deposited bone is thick and porous, in volvin g b oth the cortex and spongiosa, w ith a consequent increase in bone volum e. T h e entire process is a slow one. T h e bones most com m on ly affected are the tibia, skull, pelvis and fem u r.1 These becom e thickened and deform ed, and the lim bs bow ed. T h e skull b e comes enlarged and square across the front. C yst form ation m ay occu r in the legs. T h e patients often com plain that they m ust b u y new hats, since their old ones are too sm all. T h e disease is m ost com m on in m iddle life or later.1’ 2 T h a t the condition has been considered rela tively rare, Stafn e and A u stin 3 attribute to the fa ct th at only the m ore advanced forms have been recognized. The roentgenographic pictu re of P aget’s disease probably is dependent on the stage a t w h ich the disease is seen. Since this is a den tal report, only the skull and jaw s w ill b e considered. Osteoporosis circum scripta is considered an early osteolytic phase affectin g the skull. In the later stage, the skull has a typical “ cotton-w ool” appearance. In a study o f 138 know n cases o f P aget’s disease reported by Stafn e and A ustin, 58 per cent o f skull pictures presented definite evidence o f the disease. In only tw entythree cases did they find definite in volve m ent o f tha m a xillae and m andible, tw enty in the m a xillae and three in the
P
From the New York University College of Dentistry. *Dean. fA ssistant Professor of diagnosis.
Jour. A.D.A., Vol. 30, February 1, 1943
m andible. T h is seems to b ear out other findings th at the m andible is rarely a f fected. T h e cases reported in d en tal literature show a w id e varian ce in roentgeno graphic findings. F o x 4 fou n d cystic in volvem ent o f the apices o f the m axillary teeth, hypercem entosis o f the roots and a feath ery spongious appearan ce of the m axillary bone. Seldin6 described a b ila t eral involvem ent of the m andible, w ith alternate areas of resorption and calci fication, and hypercem entosis, evid ent in the roentgenogram . C a h n 6 reported a characteristic hazy structure of the bone due to an increase in the n um ber of trabeculae. Islands of hypercalcification w ere also noted. Stafne and A u stin w ere fortu n ate in being able to follow up cases o f P aget’s disease. A s a result o f their study, they stated that the roen tgenographic ap p ear ance probably depends on the stage at w hich the disease is seen. In the early stages, the dom inant featu re is probably osteoporosis, w hile, in the ad vanced stages, the presence o f osteosclerotic areas m ay result in an uneven m ottled ap p ear ance. In 17 per cen t o f their cases, they found fine, close trabeculations, w hich in variably occurred in those patients show ing P aget’s disease o f the skull. H ow ever, they considered these va ria tions to be w ith in norm al limits. REPORT OF CASE
History.— F. H ., a man, white, aged 70 , com plained of pains, of three years’ dura tion, in the right leg, arm, both knees and back and across the chest. D uring the past year, he had been having trouble w ith his hearing. H e had been treated b y physicians without relief. H e wished to have his teeth 237
238
T h e J o u r n a l o f t h e A m e r ic a n D e n t a l A s s o c ia t io n
Fig. i.-— Paget’s disease involving the m axillae; showing osteoporosis of anterior region and radiopaque appearance of remainder of alveolar bone. T he mandibular bone is normal.
Fig. 2.— Skull showing characteristic “ cotton-wool” or “ cotton-puff” appearance. The bones of the calvarium are thickened, with very fuzzy inner and outer tables.
N e w m a n a n d H y m a n — P a g e t ’s D is e a s e
239
exam ined to determ ine w hether they might be a focus of infection. Physical Examination. — T he patient weighed 156 pounds and was 5 feet 7 inches tall. T here was nothing of special im por tance in the mouth. T he occlusion was within norm al lim its, but the m axillary ridges were very heavy, ranging from 1 to 1i inches in width. Roentgenographic Findings.— 1. M ax illary region: T he m axillae showed m arked altera tion. (Fig. 1.) Osteoporosis was noted in the region from the upper left central in cisor to the cuspid, and the right central
Fig. 4.— Osteoporosis between central in cisors and central and lateral incisors. (Com pare Fig. 5.)
Fig. 3.— Right femur with cyst formation, most common in leg bones.
incisor to the second bicuspid regions. The rem ainder of the bone seemed to have lost its norm al trabeculation. It was radiopaque, granular and hazy in appearance, the trabec ulae being liner and more closely woven. No hypercementosis of the roots was observed. 2. M andible: The m andible was normal. 3. Skull: T here was a typ ical “cotton wool” appearance of Paget’s disease. (Fig. 2-)
4. R ight fem ur: Cyst formation was evi dent. (Fig. 3.)
Fig. 5.— Normal anterior radiolucency, most marked between central incisors and right central and lateral incisors in patient aged 47. M edical Report .— On the first visit, M ay 31, 1940, the patient com plained of burning pain of the right thigh. A roentgenogram suggested central chondroma or chondro
240
T h e J o u r n a l o f t h e A m e r ic a n D e n t a l A s s o c ia t io n
sarcoma. Aspiration biopsy disclosed an in flammatory lesion, possibly xanthomatosis. The patient was given small doses of the x-rays in this area. October 1940, there was no change in right femur, but the left femur presented the appearance of Paget’s disease. The roentgenologist stated that the x-ray appearance of the right thigh might be due to a healed cystic condition, and' that both lesions were probably due to Paget’s disease. com m ent
Stafn e and A ustin a ttach m uch im portance, from a diagnostic view point, to the osteoporosis fou n d in the m axillary bone. (F ig. 4.) H ow ever, these authors wish to stress the inadvisability of p la c ing too m uch em phasis on this point alone. W h ile the osteoporosis m ay be
Fig. 6.— Normal bone trabeculations in m axilla; showing size, shape and number of trabeculae as compared with those in Figure 7. Since the patient was 64 years of age, any variation due to age can be ruled out.
m arked, a sim ilar rad iolu cen t appearance can often be noted in otherwise norm al cases. (Fig. 5.) T h is norm al radiolucence m ay be due to the thinness o f the alveolar plate at th at point, or it m ay be an op tical illusion created b y the contrast betw een the radiopaque, roentgenograp hic appearance o f the tooth as com pared w ith that o f the ad jacen t bone. H ow ever, the bone trabeculation of the m axillae does possess diagnostic value, as dem onstrated by this case. G reen field7 describes the norm al cancellous
bone as m ade up o f fibrils of bone that interlace and form a lattice-like or trab e cu late structure. R oentgen ographically, one finds a w hite netw ork enclosing ir regu lar black spaces. In the upper jaw , the b lack spaces appear sm aller and m ore regu lar than in the lower. T h e w hite netw ork is thinner. (Fig. 6.) E xam in ation o f the roentgenogram s in the case here reported shows th at the black spaces betw een the w hite trabeculae a p p ear very sm all and fine, and a great m an y trab ecu lae crow d the space n or m ally occu pied b y a single trabecula. (Fig. 7.) T h is increase in n um ber of the trabeculae has also been reported on m icroscopic exam ination. T his fine trabecu lation can even be seen in the osteo-
Fig. 7.— Paget’s bone trabeculations.
porotic regions o f F igu re 4, w h ile the norm al trabeculation shows quite clearly in the area o f anterior radiolucency in F igu re 5. T h e hypercem entosis reported by other investigators is not in evidence in this case, and m a y not be a constant factor. S U M M A R Y AND CONCLUSION
In a m an aged 70, roentgenogram s disclosed P aget’s disease involvin g the skull, fem u r and m axillae. T h e skull show ed the typical “ cotton-w ool” a p p e ar ance, the fem ur, cyst form ation. T w o separate phases w ere seen in the m a xillae : osteoporosis and osteosclerosis. T h e os-
S e id e s — M o u t h R e h a b il it a t io n
teoporosis m igh t be confused w ith norm al anterior radiolucency. T h e bone deposition took the form of an increase in trabeculae, giv in g the bone a hazy appearance. T h e m an d ibu lar bone was norm al. N o hypercem entosis was present. B I B L IO G R A P H Y
1. G e s c h ic k t e r , C . F., and C o p e l a n d , M. M .: Tumors of Bone. New York: American Journal of Cancer, 1936 , pp. 788 -79 2 . 2 . T h o m a , K . H.: O ral Pathology. St.
241
Louis: C. V . Mosby Co., 19 4 1 , pp. 866 -904 . 3. S t a f n e , E . C ., and' A u s t i n , L . T . : Study of Dental Roentgenograms in Cases of Paget’s Disease. J .A .D .A ., 2 5 :1 2 0 2 , August 1938 . 4 . Fox, L e w i s : Paget’s Disease (Osteitis Deformans) and Its Effect on M axillary Bone. J.A.D .A., 2 0 : 1823 , October 1933 . 5 . S e l d i n , N. A .: Paget’s Disease— Clinical Case. D. Cosmos, 7 5 :6 9 1 , July 19 3 3 . 6 . C a h n , L. R .: Paget’s Disease of Bone. Arch. Clin. & Oral Path., 1 : 1 4 1 , June 19 3 7 . 7 . G r e e n f ie l d , A. L . : X -R ay Technic and Interpretation of Dental Roentgenograms. Brooklyn: Dental Items of Interest Publishing Co., 1936 , p. 123 .
FACTORS OF OCCLUSION IN RELATION TO MOUTH REHABILITATION By H
arry
M . S e i d e s , D .D .S., N ew Y o rk , N . Y .
O U T H rehabilitation im plies that im pairm ent o f the elem ents w h ich contribute to th e m ain tenance o f m asticatory efficien cy has taken place. In some instances, this m ay result in loss o f the norm al m a xillo m an d ibular ve rtica l dim ension, often m od ifying fa cia l contour. Since m astica tory efficiency depends on a thoroughly equilibrated occlusion, the prim ary req uisite fo r successful treatm ent o f m outh rehabilitation is an understanding o f the factors in norm al occlusion and the m ethod o f correctin g discrepancies. T h e follo w in g is offered as a standard ized procedure fo r the purpose o f estab lishing a diagnosis and as a basis fo r treatm ent p la n n in g : 1. C lin ica l exam ination o f the m outh. 2. E valu ation o f habits as factors in occlusion. 3. A p praisal o f occlusal discrepancies a fte r m oun ting o f casts on an adjustable articulator. A clin ical exam ination o f the m outh is m ade and p a th o lo gic conditions o f the teeth, supporting parodontium and con
M
Jour. A.D.A., Vol. 30, February 1, 1943
tiguous structures are recorded. A n in tra-oral roen tgenograp h ic exam ination is m ade, and w here tem porom andibular in volvem ent is suspected, the tem poro m and ibu lar joints are also roentgenographed. W hen occlusal disturbances are evident, there is a b reakdow n in the o c clusal m echanism w ith resultant im p a ir m ent o f fu n ction . C lin ica l exam ination o f the occlusion should be given carefu l consideration an d the degree o f destruc tion o f the d ental tissues observed and recorded, in clu d in g the status an d fu n c tion o f the m u scu lature o f the jaw s. Sequelae o f occlusal discrepancies that m a y m anifest them selves clin ically are a ttritio n ; d riftin g o f th e teeth, occasion a lly coupled w ith alveolar resorption, and disturbances in the tem porom andib u la r capsule an d its ligam ents, o r a com bin ation o f these conditions. T h ese con ditions m a y result from th e loss o f a tooth o r part o f a tooth w ith a subse qu en t deficiency w h ich m a y also b e m an i fested as a disharm ony betw een con d ylar m ovem ent and tooth gu id in g inclines. (Fig- !•)