Congenital Absence of Teeth

Congenital Absence of Teeth

592 T he Journal of th e A m e r ic a n а. K ö n i g , F.: Entzündlichen Processe am H als; in Billroth and Lücke: Deutsche Chir., No. 36, 1882...

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а. K ö n i g , F.: Entzündlichen Processe am H als; in Billroth and Lücke: Deutsche Chir., No. 36, 1882, p. 20. 3. S e m o n , F.: Tr. Roy. M ed. Chir. Soc., London, 78 : 1 8 1 , 189 5. 4. B o e h l e r , G.: Thèse de Paris, No. 2 9 5 , 188 5. 5 . D a v i s , G. G.: Ann. Surg., 44 : 1 7 5 , 190 6 . б . T homas , T . T .: Ann. Surg., 47 : 1 6 1 , 19 0 8 .

7 . P r i c e , J. W .: Ann. Surg., 4 8 :6 4 9 , 190 8 . 8. G r a y , H. Anatom y of the Human Body. Ed. ao. Philadelphia: L ea and Febiger, 1918, P-

393-

9 . T i l l a u x , P. J. : Traite d’anatomie topo­

graphique, Paris, p. 4 3 8 , 1 8 7 7 . 10. P o u l s e n , K . : Deutsche Ztschr. : , IÖ -

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11. H amann , C. A .: Cleveland M . ]., 4:387, 1899. 12. A s h u r s t , A . P. C .: Ludw ig’s Angina. Arch. Surg., 18:2054, M ay 1929. 13. B lair , V . P.: Surgery and Diseases of M outh and Jaws. St. Louis: C. V . Mosby Co., 1920, p. 410. 14. F ink , G. J.: Swelling of Face, Jaws and Neck. J.A .D .A ., 26 :1292, August 1939. 15. A l d e n , A. M .: Neck Infections of Den­ tal Origin. J.A .D .A ., 26:1266, August 1939. 16. B ohlman , H. R . : Sulphanilamide in Surgical Practice. Am. J. Surg., 42:824, D e­ cember 1938. 17. W illiams , A. C .: Ludw ig’s Angina. Surg., Gynec. & Obst., ¡147, February 1940.

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T h e Warren Chambers.

CONGENITAL ABSENCE OF TEETH By R a l p h W. E d w a r d s , B.S., D.D.S., Kansas City, Mo. in t r o d u c t io n

U M E R IC A L aberrations in the human dentition result from the presence of supernumerary teeth or the absence of teeth. The latter con­ dition is designated as “anodontia” if there is a total absence of teeth, and as “oligodontia” if several teeth are miss­ ing. The maxillary second incisor, the mandibular first incisor, the maxillary and mandibular second premolars and the maxillary third molar are missing more frequently than other teeth, al­ though any tooth or any combination of teeth can be involved. Occasionally, there is a tendency to symmetrical varia­ tion, with the same tooth missing on each side of the arch, the same tooth missing on one side of the arch and in each jaw or the same tooth missing on each side of the arch and in each jaw.

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From the Department of Oral Surgery, Kansas City-Western Dental College. Read at the Eighteenth Annual M eeting of the International Association for Dental R e­ search, Philadelphia, Pa., M arch 13, 1940.

Jour. A.D.A., Vol. 28, April 1941

Various theories have been offered as to the cause of this phenomenon of miss­ ing teeth. Syphilis, endocrine gland dis­ turbance, rickets, tuberculosis and hered­ ity are listed as etiologic factors, with a possibility that each may be responsible, in certain instances, for suppression of the dentition. Heredity seems to have the strongest case of all, but, in general, the etiology of dental dystrophy is ob­ scure and can be ascribed to some un­ known condition resulting in partial or complete aplasia of the dental lamina. lite r a tu r e

Fifty-seven years ago, Guilford1 (1883) reported the case of a man, 48 years of age, who had been edentulous from birth. While it is true that the x-rays had not been discovered at that time, and there­ fore the possibility of unerupted teeth existed, it is nevertheless evident, from the clinical description and the illustra­ tions, that it was a case of anodontia as­ sociated with ectodermal dysplasia of the anhidrotic type. Similar cases of com-

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plete or partial absence of teeth associ­ ated with this affliction have been re­ corded by Roberts2 (1929), H ill3 (1933), Thom a4 (1934), Thannhauser5 (1936), Lord and W olfe6 (1938) and Cook7 (1939). Gardner and Dillery8 (1934) discuss the case o f a boy, aged 15, with twenty-three permanent teeth missing. K eeler9 (1935) lists the pedigrees o f sev­ eral families with missing teeth. Battersby10 (1936) presented the case of a

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second premolar missing. M iller13 (1937) presented the case o f a 13-year-old girl with nine permanent teeth missing. Sly14 (1938) described a 12-year-old girl with the m axillary second incisors and the mandibular second premolars missing. G ray16 (1938) published an account o f a 9-year-old boy with six deciduous and thirty permanent teeth absent. W arr16 (1938) recorded the case o f a 12-year-old boy, with the deciduous m axillary second

Fig. 1 (C h ild 1 ) .— X -ra y survey show ing absence of eleven teeth.

F ig. 2 (C h ild 3 ) .— X -ra y exam ination show ing sixteen teeth missing.

boy, aged 9, with complete absence of both dentitions. Steuer11 (1936) de­ scribed an 1 1 -year-old girl with all the permanent teeth missing except the m ax­ illary first incisors and the mandibular first molars. Dahlberg12 (1937) reported a 12-year-old boy with the m axillary second incisors, the mandibular first and second incisors and the right mandibular

incisors and all permanent teeth missing. T h e child was otherwise normal, and no evidence o f dental anomalies could be traced on either side o f the fam ily. Schultz17 (1938) reported the case o f a 3-year-old girl, normal except for com­ plete absence o f tooth development as shown by an x-ray examination. Werther and Rothenberg18 (1939) described a 15-

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year-old girl with all the teeth missing except the following :R -6--% j * ¿-g- L .* In a previous communication,19 oli­ godontia in two of a fam ily of eight chil­ dren was reported. Inadvertently, be­ cause of undue credence in parental information, the statement was made that numerical variation did not exist in the remaining six children. A n opportunity for additional study o f this fam ily has revealed that five o f the eight children have congenitally missing teeth.

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7 1. H is m o th er d ie d a t th e age o f 55. A n x -ra y su rv ey o f his teeth w as m a d e J a n u a ry 1 1 ,1 9 3 8 a n d th e d en titio n w as fo u n d to b e n orm al. T h e fo llo w in g teeth w e re m issing, w ith e v i­ d en ce th at th e y h a d been rem o ved : • jj-j w e re m issing, w ith n o re c o lle ctio n of tooth rem o val. 4 3 1 0 h a v e sin ce been rem oved. T h e m o th er, L .M .G ., w a s born in A r k ­ ansas o f Irish -D u tc h p a re n tage , N o ve m b er 14, 1888. H e r fa th e r d ie d a t the age o f 76 and her m o th er at 73. E a c h p a re n t h a d m ost of the n atu ra l teeth re m a in in g a t death , bu t a p o ssib ility o f tooth d e ficie n cy existed.

Fig. 3 (C h ild 4 ) .— X -ra y exam ination show ing tw enty-nine teeth missing.

Fig. 4 (C h ild 5 ) .— F ourteen teeth revealed as missing in x -ra y survey. h is t o r y o f

“ c ” f a m il y

T h e fa th e r, G .A .C ., b o rn in G re e c e , o f G re e k parents, in D e c e m b e r 1888. H is fath e r w as still liv in g in G re e c e a t the age o f abou t * I n this system of n otation of the teeth, figures in arabic num erals sign ify the perm a­ nen t teeth, and sm all letters represent the deciduous teeth.

A n x -ra y su rv ey o f th e eden tulo us ja w s of L .M .C . w as m a d e J a n u a ry 5 , 1939. N o u n ­ eru p ted teeth w e re p resen t. T h e p a tien t had lost th e re m a in d e r o f h e r n a tu ra l teeth a t 32. T h e re w as a re c o lle ctio n o f to oth deficien cy, bu t n o th in g d efin ite c o u ld be determ ined . A c c o rd in g to h e r description , she w as “ sn aggle-too th ed ,”

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teeth

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m issing. D a t a b e y o n d the p aren ts are n ot a v a ila b le. T o this co u p le, e ig h t ch ild re n w e re born, a ll o f w h o m are liv in g . A ll of these ch ild re n are of n o rm a l in te llig e n ce , b u t sm all of stature. A l l a re d e v o id o f p o lyd a c ty lism , o lig o d a c ty lism or e cto d e rm a l anom alies. P h y sic a l a n d se ro lo gic exam in ation s w ere

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M a r c h 20, 1936 a n d a son b o rn J a n u a ry 2, 1938. A n x -ra y su rv ey o f th e te e th of E .C .G . w as m a d e F e b ru a ry 9, 1939. (F ig . 1.) T h e fo llo w in g teeth w e re present, a cc o rd in g to th e fo rm u la : R 7 ? 432i l ' 234 ^ 8 1 6 - h a d been re c e n tly rem oved. M issin g teeth w e re : | s 1 5 7 1 8 ; a to ta l of 11.

Fig. 5 (C h ild 7).-— X -ra y survey revealin g fou rteen teeth missing.

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Pediqree o f C fam//tj . F ig. 6.— Stud y of tooth deficien cy in “ C ” fam ily. n eg ative . N o in fo rm a tio n w as a v a ila b le as to to o th a n o m alies o r n u m erica l d eficien cy in th e d ecid u ou s d en titio n o f an y o f these ch ild ren . C h ild 1, E .C .G ., a girl, born D e ce m b er 15, 19 17 , a n d m a rried , h a d a d au g h ter born

C h ild 2, D .C ., a g irl, b o rn J a n u a ry 19, 1919, h a d an x -ra y su rvey m a d e J a n u a ry 13, 1940. T h e den titio n w as fo u n d to be n orm al, e x c e p t th a t JSJ w a s m issing, w ith n o re c o l­ lection of h a v in g h a d th e to o th rem o ved . C h ild 3, J .C ., a b o y, born F e b ru a ry 2,

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1922, h a d an x -ra y e xa m in a tio n m a d e D e ­ cem b er 18, 1937. (F ig . 2.) T h e fo llo w in g teeth w e re present: p R

7 6 3 c l l l c 3 6 7 T 6 4 3 2 I a 2 3 4 6 - u*

T h e m issing teeth w ere: b t b ? 1x » t I a to ta l o f 16. C h ild 4 , K .C ., a girl, b o m O c to b e r 15, 1923, h a d an x -ra y su rvey m a d e F e b ru a ry 1 2 ,1 9 3 7 . (F ig . 3.) T h e fo llo w in g te e th w ere present: p

6 C I c 6 7 e d c b | c d e

t

T h e m issing teeth w e re : 8 7 5 4 3 2 1 1 1 2 3 4 5 8 8 7 6 5 4 3 2 1 | 1 2 3 4 5 0 7

f f 1 f l U l i l l U1 ¿ y .

C h ild 5, G .C ., a girl, born J u ly 14, 1925, h a d a n x -ra y su rvey m a d e J a n u a ry 28, 1939. (F ig . 4.) T h e fo llo w in g teeth w e re presen t:

R

7 f l e 4 c 3 2 1 l l 3 c 4 e 6 7 T e 4 3 2 1 | 2 3 4 e *-*

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a to tal o f 14. A n a d d itio n a l x -ra y survey of | | | w a s m a d e F e b ru a ry 10, 1940. N o m a x illa r y o r m a n d ib u la r th ird m o lars w ere present. C h ild 8, R .C ., a b o y, b o rn J u n e 5, 1931, h a d an x - r a y su rvey m a d e J a n u a ry 28, 1939. A ll m a x illa r y d ecid u o u s te e th w e re present; a ll m a n d ib u la r d ecid u ou s teeth w e re present e xce p t th e incisors. B o th m a x illa r y and m a n d ib u la r first p e rm a n en t m olars, a n d th e m a n d ib u la r first in cisors w e re f u lly erupted. T h e p e rm a n e n t d e n titio n w a s fou nd , to be n orm al, w it h n o e vid en ce o f th ird m o la r c a l­ cificatio n . A n e xtra -o ral x -ra y su rv ey of -3 | | w a s m a d e F e b ru a ry 10, 1940. N o m a x illa r y o r m a n d ib u la r th ird m olars w ere present.

T a b le 1.— M a x illa ry T e e t h T h a t A r e M issin g .

Child / Child 3 Child 4 Childs Child 7 0 “

Sa 9
8 7 6 S 4- 3 z 0 0 0 0 0 0 0 0 0 a 0 0 0 0 0 0

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/

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3

0

0

4 s 6 7 8 0 0 0 0

0 0 0 0 0

0

0

0 0 0 0 0

0 0 0

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f u '/ s s in g

T a b le 2.— M a n d ib u la r T e e t h T h a t A r e M issin g . Sex CkUJ / ? ChUd 3
cu u s ? auj 7 d

T h e m issing teeth w ere: a to tal o f 14.

8 7 6 & 4 3 0 0 0 0 0 00 0 0 0 0 0 0 0 Û

0 0 0

8 7 I H ^ H H b rs,

C h ild 6, R .C ., a b o y, born J a n u a ry 9, 1927, h a d an x -ra y su rvey m a d e J a n u a ry 21, I 939 - T h e den titio n w as fo u n d to b e n o r­ m al. T h e m a n d ib u la r th ird m o la r buds w ere presen t; th e m a x illa r y th ird m o la r buds m iss­ in g. A n a d d itio n al x -ra y su rvey of -§ [ | w as m a d e F e b ru a ry 10, 1940. T h e m a n d ib ­ u la r th ird m o lars w e re d evelo p in g. N o m a x ­ illa r y th ird m olars w e re present. C h ild 7, D .C ., a boy, born A u g u s t 21, 1929, h a d an x -ra y su rvey m a d e J a n u a ry 2 1, J939 - (F ig . 5 .) T h e fo llo w in g teeth w ere „



.

Tj

present: R.

7 6 8 3 2 1 1 X 2 3 «

5 a 3 2 12 3 a e

7

L.

T h e m issin g teeth w ere: - 3 7 g * j \ ° g T 8



!

z

/

0

0 0 0 0 0

z

3 4

s

0 0

6 7 8 0 0 0 0

0 c 000 0 0L 0 0 0 0 00 00 0 0 com m ent

In these five afflicted children, a total of eighty-four teeth are missing. (Tables 1 and 2.) Both maxillary and mandibular third molars are missing in all children of this group. The left maxillary and mandibular second premolars and the left mandibular second molars are ab­ sent in all of the affected group. In chil­ dren 1, 3, 4 and 5, the right maxillary and mandibular second premolars and the left maxillary second incisor are miss­ ing. In children 3, 4, 5 and 7, the right mandibular second molar and the left mandibular first incisor are missing. In

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children 4, 5 and 7, all m and ibu lar m olars are absent. In child 4, all m a n ­ dibular teeth are missing. A n interesting observation in child 3 is th at the missing teeth are b ilaterally id en tical in each jaw . T h e pedigree of this fam ily is rep re­ sented in F igu re 6. T h e children in F 2 are too y o u n g to be studied fo r tooth defi­ cien cy in the perm anent dentition. J a n u ­ a ry 17, 1940, these children w ere ex a m ­ ined and foun d to be norm al. T h e oral exam ination o f G .G . (fem ale) revealed a norm al com plem ent o f deciduous teeth w ith ou t anom alies. T h e oral exam ination o f K .E .G . (m ale) revealed norm al d ecid u ­ ous teeth, fu lly erupted, excep t ? 1 I , but the age o f the child could accou n t fo r lack o f eruption o f these teeth. C h ildren 2, 6 and 8 (the un affected group ) all h ave some third m olar defi­ ciency. C h ild 2 has the right m axillary third m olar m issing; child 6 has both m a xillary third m olars missing, and child 8 has all third m olars missing. Ju ly 17, 1940, the m outh o f K .E .G ., the son o f C h ild 1, w as exam ined. A ll deciduous second m olars w ere eru p ted .* I t w ill be observed in F igu re 6 that this characteristic o f tooth deficiency a p ­ p aren tly follow s a hereditary pattern that is not sex linked. Burks,20 in review in g the pedigree o f this fam ily, w as o f the opinion th at the hereditary predisposition cam e from both m other and fath er, the one h avin g a m ultiple tooth deficiency, the oth er a third m olar deficiency. BIB LIO G R APH Y 1. G u i l f o r d , S. H .: Dental Anomaly. D. Cosmos, : i i , M arch 1883. 2. R o b e r t s , E l m e r : Inheritance o f A n ­ hidrosis Associated with Anodontia. : , July 27, 1929.

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•Exam ination made subsequently presentation of the paper.

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3. H i l l , A. M .: Hereditary Ectodermal Dysplasia of Anhidrotic Type. Arch. Derm. & Syph., : , July 1933. 4. T h o m a , K . H .: Clinical Pathology of Jaws. Springfield, .: Charles C. Thomas,

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5. T h a n n h a u s e r , S. J.: Hereditary Ecto­ dermal Dysplasia of Anhydrotic Type. J.A. M .A ., :go , M arch 14, 1936. 6. L o r d , L . W., and W o l f e , W. D .: Her­ editary Ectodermal Dysplasia of Anhidrotic Type. Arch. Derm. & Syph., 38:893. Decem­ ber 1938. 7. C o o k , T . J.: Hereditary Ectodermal Dysplasia of Anhidrotic Type. Am. J. Ortho­ dontics, :1008, October 1939. 8 . G a r d n e r , B . S., and D i l l e r y , J. L . : Congenital Absence of Twenty-Three Perma­ nent Teeth. J.A .D .A ., 21:2032, November

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9. K e e l e r , C . E .: Heredity in Dentistry. D. Cosmos, 7 7 :1 1 4 7 . December 1935. 10. B a t t e r s b y , J o h n : Anodontia. D. Mag. & Oral Top., 53:427, M ay 1936. 11. S t e u e r , I. F . : Report of Congenitally Missing Teeth. D . Cosmos, 78:770, July 1936. 12. D a h l b e r g , A . A .: Inherited Congenital Absence of Six Incisors, Deciduous and Per­ manent. J. D. Res., 16:59. February 1937. 13. M i l l e r , H. A .: Dental Abnormalities in Patient with Achondroplasia. Jnternat. J. Orthodontia, 23:296, M arch 1937. 14. S l y , W. J.: Neutroclusion Case Involv­ ing Congenitally Absent Teeth. Am. J. Ortho­ dontics, 2 4 :15 1 , February 1938. 15. G r a y , D. S . : Congenital Absence of Deciduous and Permanent Teeth. J.A .D .A ., 25:748, M ay 1938. 16. W a r r , V . C .: Case of Complete A b ­ sence of Permanent Dentition. /. Brit. D . A ., 64:327, M arch 1938. 17. S c h u l t z , L. W .: Anodontia: Report of Case. D . Digest, 44:212, M a y 1938. 1 8. W e r t h e r , R . , and R o t h e n b e r g , F.: Anodontia. Am. J. Orthodontics, : i, Jan­ uary 1939. 19. P o r t e r , C . G ., and E d w a r d s , R. Congenital Absence of Tw enty-Nine Perma­ nent Teeth. J.A .D .A ., 24:1852, November

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20. B u r k s , B a r b a r a S.: Personal Commu­ nication. Carnegie Institution of Washington, Department of Genetics, Cold Spring Harbor, Long Island, N. Y .

1108 East Tenth Street.

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