Development of the dentition in cieidocranial dyspiasia
Birgit Leth Jensen and Sven Kreiborg Institute of Pediatric Dentistry, Royal Dental College, Copenhagen, Denmark
Jensen BL, Kreiborg S: Development of the dentition in cieidocranial dysplasia, J Oral Pathol Med 1990; 19: 89-93, The purpose of the present investigation was to describe the formation, maturation and eruption of the dentition, including supernumerary teeth in a sample of patients with cieidocranial dysplasia. The dentition was evaluated from orthopantomograms, intraoral radiographs, cephalometric films, surgically removed teeth and intraoral photographs in 19 patients (9 men, 10 women), aged 3,5 to 34 years. Formation of primary teeth was normal, whereas all patients but one had supernumerary permanent teeth. Frequency of supernumerary teeth ranged from 22% in the maxillary incisor region to 5% in the molar regions. Supernumerary teeth were formed lingually and occlusally to the normal teeth. Maturation of the primary dentition was normal, while permanent teeth were delayed from 1 to 4 yr. Supernumerary teeth were delayed about 4 years in relation to normal permanent teeth. Eruption of primary teeth was normal, whereas all patients had severe eruption problems of permanent teeth. It was hypothesized that the dental lamina for both primary and permanent dentition is normal, but does not resolve completely and therefore may form supernumerary teeth. Abnormalities of tooth morphology is related to inadequate space and arrested eruption. Delayed or arrested eruption is probably caused by diminished resorption of bone and of primary teeth and to the presence of multiple supernumerary teeth.
Cieidocranial dysplasia (CCD) is an autosomal dominant skeletal dysplasia with severe dental abnormalities. The most characteristic clinical sign is the eruption failure of the permanent dentition. The primary dentition seems to develop in a relatively normal way (1-8), while the permanent dentition is severely disturbed with predisposition for multiple supernumerary teeth, failure of eruption, ectopia and abnormal tooth morphology, especially involving the roots (9-19), Although CCD is a generalized bone dysplasia (20-21), the extensive literature is mainly focused on dental problems (5, 7, 18, 22-26), because of the patients' concern about their dentofacial appearance. Dental treatment is difficult and time consuming, often requiring surgical, orthodontic and prosthodontic interventions. Most reports in the literature represent case histories dealing with these complex therapeutic problems. Longitudinal studies of the development of the permanent dentition and supernumerary teeth have not yet been carried out and literature on dental maturity is extremely scarce (27, 28), The explanation for the absence of longitudinal information appears to be related to the fact that CCD patients are not
aware of any problems until the age of about 10 yr, when the eruption difficulties in the permanent dentition have become a serious psychosocial problem. The purpose of the present investigation is, as part of a larger craniofacial study on CCD, to describe the formation, maturation and eruption of the dentition, including supernumerary teeth, in a relatively large sample. Material and methods
The patient population consisted of 22 white subjects (10 males, 12 females) with CCD, ascertained through the Institutes of Pediatric Dentistry and Orthodontics at the Royal Dental College in Copenhagen, Most patients ( H = 1 5 ) were followed longitudinally during their growth period. Age range was 31^ to 34 yr. The number of observations per patient ranged from 1 to 21, In the longitudinal series several patients were followed with annual examinations. Pedigrees were available for 14 patients (Fig. 1), Intraoral radiographs, orthopantomograms and/or cephalometric films in several projections were available for all subjects. Recording of supernumerary teeth, dental maturity and dental morphology were judged from intraoral ra-
Key vi/ords: cieidocranial dyspiasia; dental lamina; supernumerary teeth; tooth eruption; tooth formation; tooth maturation; tooth morphology, Birgit Leth Jensen, Institute of Pediatric Dentistry, Royai Dental Coliege, 20 Nr, Alie, DK-2000 Copenhagen N, Denmark, Accepted for publication November 12, 1989,
diographs, orthopantomograms, intraoral photographs and from surgically removed teeth. Dental maturity was scored according to the assessment system of DEMIRJAN et al. (29), and
com-
pared to available normative data. Three patients were excluded from the present study because of multiple extractions or incomplete material. The youngest patient had to be excluded from registration of abnormal tooth formation, Orthopantomograms of 14 patients could be evaluated for dental maturity. The differences between the means were tested using Student's t test. The level of significance chosen was 0,01, Results Tooth formation
Primary dentition - The youngest patient, a 3'/, yr old girl, had a complete primary dentition with teeth of nonnal size and shape. Several older patients also had a complete primary dentition at the first examination and all but one patient had several persisting deciduous teeth. No patients showed signs of abnormal tooth formation or deviation in number. Permanent dentition - Of 18 eval-
uated patients, 17 had supernumerary
90
JENSEN & KREIBORG
normal tooth. The frequency of supernumerary maxillary central incisors was D—r-O 1 more than ten times higher than the D I • n D-r-« n an n frequency for lateral incisors. Also, the n frequency of supernumerary first premoiars was about two times higher than for second premoiars in both jaws. Two patients had a nearly complete n 107 T n *9153 0 2760 K 1065 Z supernumerary dentition anterior to the DI 5739 P, 5740 P, in 9153 0, 9154 0 G 1513 first molars. In one of these patients, 5741 P the only "missing" teeth in the supernumerary dentition were the upper lateral • AFFECTED MALE incisors and the lower central incisors. D UNAFFECTED The other patient "lacked" only one D-p-O I O-r—O AFFECTED FEMALE maxillary second premolar and the O UNAFFECTED D-]-i n mandibular four incisors. • ) MUSCULAR From the longitudinal series of ortho• m @ DYSTROPHY pantomograms and roentgencephalo1 A MONOZYGOTIC grams it was observed that the supernuE L 5053 5731 U 345 0 TWINS merary tooth germs developed lingually in 3425 R to the normal teeth, and in the canine Fig. I. Pedigrees, and premolar regions they developed occlusally to the normal teeth (Figs, 2A, B). Table 1, Distribution of supernumerary teeth according to sex About one third of the 18 patients Individuals iSupernumerary had supernumerary molars. One pan teeth n X SD P t tients had an extra second mandibular molar bilaterally situated between the Males 9 11 8,6 1.1 2,0 0,05 Females 9 6,4 58 3,8 first and second molars, the other extra molars all developed distal to the third molar as fourth and even fifth molars. It is noteworthy that the monozygotic Table 2, Percentage of supernumerary teeth according to main region, « = 135 female twins (Fig, I A), both produced supernumerary teeth only in the incisor Incisor Canine Premolar Molar Total and canine regions; however, one had 8 and the other 10 extra teeth. Maxilla 22,2 15,6 11,9 5,2 54,8 Morphology of supernumerary teeth Mandible 12,6 11,9 5,9 45,2 14,7 always resembled the corresponding Total 34,8 27,5 26,6 11,1 100,0 normal teeth. However, malformed crowns and roots were observed frequently. Among the normal permanent teeth in addition to the normal teeth. gion of the maxilla; the frequency in the teeth no malformed crowns were obThe number ranged from 1 to 21 extra mandibular incisor region and in the served, but malformed roots with tooth germs in individual cases with a canine and premolar regions was fairly marked defections (Fig, 3) were mean of 8 teeth. The frequency of super- similar, ranging from 12 to 15%, The common. numerary teeth was somewhat higher in frequency of supernumerary molars was males than in females (Table I), How- about 5% in each jaw. In general there ever, the difference was not significant was a high degree of symmetry in the Tooth maturation at the 0,01 level. occurrence of supernumerary teeth. In Primary dentition - Maturity of the priIn Table 2 the location of the super- Table 3 the distribution of supernumer- mary teeth could not be assessed in any numerary teeth is tabulated according ary teeth is tabulated according to indi- detail because of the age distribution of to main regions. The highest frequency vidual tooth type. No case revealed the sample. It should be noted, however, (22%) was observed in the incisor re- more than one supernumerary tooth per that the youngest child in the study at D—pO
I
D—|-HD I
Table 3, Distribution of supernumerary teeth in 18 patients with Cieidocranial Dysplasia according to individual teeth Incisor
Premolar
Central n %
Lateral n %
Canine n %
n
%
Second n %
Maxilla Mandible
28 9
20,8 6,7
2 8
1,5 5,9
21 16
15,6 11,9
II 12
8,1 8,8
5 8
3,7 5,9
Total
37
27,5
10
7,4
37
27,5
23
16,9
13
9,6
First
c
Dist, mol n %
n
%
2
_ I ,5
7 6
5,1 4,4
74 61
54,8 45,2
2
1,5
13
9,6
135
100,0
Molar
n _
Total
J
Dentition in cieidocranial dysplasia 91 group. Thereafter, the delay became progressively marked, the average being about 4 yr in the 18-23 yr age group. Supernumerary teeth were markedly delayed in all patients when compared to both controls and to the earlier forming normal permanent teeth. Longitudinal observations of the premolar regions revealed about a 4-yr delay. Also, when fourth and fifth molars developed, their formation was initiated approximately 4 yr after formation of the preceding molar. In general, calcification of supernumerary teeth was initiated when the preceding tooth showed completion of crown formation. Tooth eruption
Fig. 2. A, orthopantomogram illustrating formation of supernumerary teeth in premolar region, B, same patient at a later developmental stage.
age 314 y showed completed root formation of the primary teeth. Thus, she had no marked delay in dental maturity, if any. Permanent dentition - Dental maturi-
ty of the normal permanent teeth, assessed by the method of DEMIRJAN et al. (29), is presented graphically in Fig, 4, On the average dental maturity was delayed about 1 yr in the 7-9 yr old age
Primary dentition - Eruption of primary teeth was uneventful in all patients except one girl, who had delayed eruption of a second primary molar (erupting at about 8 yr of age). Retention of primary teeth was not observed in any case. Permanent dentition - All patients had eruption problems in the permanent dentition, both in regions with and without supernumerary teeth, A single patient without supernumerary teeth showed delayed, but spontaneous eruption of all teeth except a maxillary canine. The remaining patients had a range of spontaneous eruption from 4 to 22 permanent teeth. It was noteworthy that all first permanent molars erupted spontaneously, but with considerable delay (about 3 yr). Apart from third molars, the premoiars and canines showed the least tendency to erupt, Ectopie tooth position was most frequent in the premolar and canine regions. However, normal permanent tooth germs were fonned at their normal sites in the dental arch and then, during the very slow eruption process, and while supernumerary teeth were developing, they were displaced to an aberrant eruption path and became retained in the jaws (Figs, 2A and B), In the many cases with failure of permanent tooth eruption, the primary teeth persisted; in fact, one patient presented with a full primary dentition at the age of 16, Especially around unerupted second and third molars dental cysts developed leading to a gross displacement of the teeth in several cases (Fig, 5), Discussion
Fig. 3. Surgically removed teeth from a CCD patient.
The formation, maturation and eruption of the primary teeth in the CCD
92
JENSEN & KREIBORG
DENTAL AGE
5
DENTAL MATURITY IN PATIENTS WITH CLEIDOCRANIAL DYSPLASIA N = 14
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23 AGE IN YEARS
Fig. 4. Graph of dental maturity in 14 CCD patients.
patients was relatively normal. In fact the only anomaly observed in the present study was a slight delay in eruption in a few cases. This finding is in agreement with several previous studies (1, 3—4, 13), The most marked and consistent disturbance observed was the extreme delay or arrest of physiologic root resorption and shedding of the teeth. This finding is probably related to the generalized reduced bone resorption observed in the jaws in CCD (30) and to the lack of eruption of the permanent teeth. The formation and maturation of the permanent teeth was fairly normal in the early stages of development. However, with a delay of about 3-4 yr, all but one patient developed supernumerary teeth. The delay in the development of the supernumerary teeth has previously been described by one of us (28) in one of the cases in the present study.
but we have found no mention of it in previous studies, probably because of the lack of longitudinal data. It was noteworthy that supernumerary teeth developed upon completion of normal crown formation in the permanent dentition at predictable locations lingually and occlusally to the normal permanent teeth. Supernumerary teeth developed in all regions (incisor, canine, premolar, molar). More than one supernumerary tooth per normal tooth was never observed. In cases with multiple supernumerary teeth, the relative onset of formation of these teeth corresponded to that observed in the normal permanent dentition. When supernumerary molars were observed they generally developed distally to the third molar as fourth and even fifth molars, invariably being fonned at the time of crown completion of the preceding molar in a manner similar to that normally
Fig. 5. Orthopantomogram with denial cysts around the mandibular third molars.
observed in development of second and third molars. Two of the longitudinal cases developed a nearly complete supernumerary permanent dentition anterior to the first molars. In one of these cases the "second permanent dentition" only had "aplasia" of the maxillary lateral incisors and the mandibular central incisors, imitating the localization often seen in normal individuals. Similarly, the other case lacked only one maxillary second premolar and the mandibular four incisors in the supernumerary dentition. Formation of supernumerary permanent teeth in CCD can be explained by incomplete or severely delayed resorption of the dental lamina, which is then reactivated at the time of crown completion in the normal permanent teeth, A similar mechanism would appear to operate in the molar region. If the dental lamina for the molars is not resolved, it may extend distally to form supernumerary molars with the "expected" time intervals. The hypothesis of incomplete or markedly delayed resorption of the dental laminas is in accordance with previously reported abnormal bone remodeling in CCD with diminished bone resorption (5, 7, 30), Thus, it would seem that development of supernumerary teeth in CCD can be directly related to the generalized bone dysplasia. Malformed crowns and roots of supernumerary teeth can be explained by poor spacing in the jaws during their development and to their retention in the jaws. As already mentioned, crown morphology of the first permanent teeth is normal, although their roots most often become abnormal and dental maturity becomes progressively delayed (27), Both findings ean be explained by lack of eruption. In previous literature, the severely delayed or arrested eruption of permanent teeth has been ascribed to various factors: 1) The presence of multiple supernumerary teeth (31), 2) malformed roots with lack of cellular cementum (32, 33), 3) the jaw bone being too dense (9), and 4) abnormal resorption of bone and of primary teeth (5, 6, 9), As already indicated, we favor the last hypothesis which will now be further substantiated. The finding that primary teeth and the permanent first molars always erupt, but with some delay, may be explained by their superficial localization in the jaws with minimal bone coverage. The finding of arrested eruption even in regions without supernumerary teeth supports the hypothesis of abnormal re-
Dentition in cieidocranial dysplasia 93 modeling, which may also explain the delayed or arrested resorption of the primary roots. Supernumerary teeth never erupted spontaneously, and their presence occlusal to the nonnal permanent teeth undoubtedly contributes to arrested eruption, secondary migration and to ectopie localization of these teeth. The chaotic picture of the dentition in CCD most often reported in the literature, (multiple supernumerary teeth, multiple tooth abnormalities, lack of tooth eruption, ectopie localization of teeth etc) now has a simpler interpretation on the basis of this study. Two probably interrelated factors seem to be of primary importance: Incomplete resorption of the dental lamina and of primary teeth and markedly delayed bone resorption. Both findings are explained by the generalized bone dysplasia in CCD, Incomplete resorption of the dental lamina creates multiple supernumerary teeth in a rather,predictable fashion and the presence of these combined with delayed bone and primary root resorption are responsible for the multible retentions, which again lead to malformed roots and migration of permanent teeth. These findings are important for improved treatment strategies of the dental abnormalities in CCD, Based on the findings in the present study and the findings in the literature it would seem conceivable that: 1) the dental lamina for the primary and permanent dentition is normal, 2) the dental lamina does not completely resolve at the expected time, 3) at the time of completed crown formation of the permanent teeth, the remnants of the dental lamina are reactivated to form supernumerary teeth, 4) the dental lamina for the molars is not resolved in all patiens, but extends distally to form supernumerary molars, with "expected" intervals, 5) abnormalities of supernumerary crown and root morphology can be related to the inadequate space in the jaws during their development and to their retention in the jaws, 6) the abnormalities of root morphology in normal permanent teeth are secondary to arrested eruption, 7) ectopie location of normal permanent teeth is caused by migration secondary to arrested eruption and interference with supernumerary teeth in the region, and 8) severely delayed or arrested eruption of normal permanent
J Roentgenol 1974; 121: 5-16,
teeth is probably caused primarily by diminished bone resorption, diminished resorption of the roots of the primary teeth, and secondarily by the presence of multiple supernumerary teeth.
16, MONTEIL M, ViALATEL C, Le probleme dentaire dans la dysostose cleidocranienne, Actualites Odonto-Stomatol 1974; 105: 441-51, 17, JARVINEN S, Dental findings in three cases of cieidocranial dysostosis, Proc Finn Dent Soc 1980; 76: 56-61, Acknowledgment - We are grateful to Drs ARNE BjflRK, Copenhagen, and BIRTE PRAHL18, HuTTON CE, BiXLBR D, GARNER LD, ANDERSEN, Amsterdam for giving us access Cleidoeranial dysplasia - treatment of to their material on CCD patients. Also, we dental problems: report of a case, J Dent wish to thank Dr, MICHAEL M, COHEN for Child 1981; 11: 456-62,
his critical appraisal of the English, The study was supported by a grant from "Ingeborg and Leo Dannin's Fund",
19,
KlRSON L E , SCHEIBER R E , TOMARO A J ,
Multiple impacted teeth in cieidocranial dysostosis. Oral Surg 1982; 54: 604-5, 20, SPRANGER J, Cieidocranial dysplasia. In: SPRANGER J, LANGER LO, WIEDEMANN
References 1, EiSEN D, cieidocranial dysostosis, Radiotogy 1953; 61: 21-31, 2, KALLIALA E , TASKINEN PJ, Cieidocranial
dysostosis. Report of six typical cases and one atypical case, Orat Surg 1962; 15: 808-22, 3, BACH C , FAUR^ C , SCHAHFER P, JOLLY J,
La dysostose cleido-cranienne. Etude de six observations. Association a des manifestations neurologiques, Ann Pediatr •1966; 42: 67-77, 4, HASLER JF, VANDERMER J, Cieidocranial
dysplasia. Birth Defects 1974; 10: 524-6, 5, HitCHiN AD, FAIRLY JM, Dental management in eleido-cranial dysostosis, Br J Oral Surg 1974; 12: 46-55, 6, HALL RK, HYLAND AL, Combined sur-
gical and orthodontic management of the oral abnormalities in children with cieidocranial dysplasia, Int J Oral Surg 1978; 7: 267-73, 7, MiGLiORisi JA, BLENKINSOPP P T , Oral surgical management of cieidocranial dysostosis, BrJ Orat Surg 1980; 18: 212-20, 8, MoNASKY GE, WINKLER S, ICENHOWER JB, RuANE AS, FIELDING AF, DH-
FRANCisis D, Cieidocranial dysostosis. Two case reports, N Y State Dent J 1983; 49: 235-8, 9, RusHTON MA, The failure of eruption in eleido-cranial dysostosis, Br Dent J 1937; 63: 641-5, 10, PusEY RF, DuRiE JF, A case of cieidocranial dysostosis showing failure of
eruption of teeth, Br Dent J 1943; 75: 11-3, 11, CHIPPS JE, Multiple supernumerary
teeth in eleidocranial dysostosis, Orat Surg 1951; 4: 25-8, 12, KiTTNER E, Zahniiberzahl bei Dysostosis eleidocranialis, Dtsch Zahnaertzl Z 1955; 10: 493-5, 13, JUNG C , Befunde im Zahn- und Kiefersystem bei Dysostosis eleidocranialis, Fort.ichr Kieferorthop 1962; 23: 273-9, 14, LEVIN EJ, SONNENSCHEIN H , Cieidocrani-
al dysostosis, N Y State J Med 1963; 63: 1562-6, 15, JARVIS JL, KEATS TE, Cieidocranial dy-
sostosis, A review of 40 new cases. Am
H-R, eds. Bone dysplasias. Stuttgart: Fischer Verlag 1974: 254, 21, GORLIN RJ, PINDBORG JJ, COHEN MM,
Cieidocranial dysplasia. In: GORLIN RJ, PINDBORG JJ, COHEN MM,
eds.
Syn-
dromes of the head and neck. New York: MacGraw-Hill, 1975: 180-4, 22, MULLER EE, Transplantation of teeth in cieidocranial dysostosis. In: HUSTED E , HJORTING-HANSEN E , eds. Oral Surg Transactions of 2nd Congress. Copenhagen: Munksgaard 1967: 375-9, 23, NoRDENRAM A, Autotransplantation of teeth in cieidocranial dysostosis, Odontol Revy 1971; 22: 363-70, 24, OKSALA E , FAGERSTROM G , A two-stage
autotransplantation of 14 teeth in a patient with cieidocranial dysostosis, Suom Hammaslaak Toim 1971; 67: 333-8, 25, BIXLER D , Heritable disorders affecting cementum and the periodontal structiire. In: STEWART RE, PRESCOTT GH, eds.
Oral facial genetics. St, Louis: Mosby 1976: 262, 26, BEHLFELT K M , GUNDLACH K K H , Dys-
plasia eleidocranialis, Fortschr KiefefGesichtschir 1986; 31: 175-8, 27, MCssiG HD, Untersuchung zur dentalen und sketettaten Entwicktung von Patienten mit Dysostosis-cteido-craniatis. Thesis, Erlangen, Nurnberg: Fdedrich-Alexander-Universitat, 1986, 28, KREIBORG S, Syndromer med alvorlige dentitionsafvigelser. In: HJORTING-HAN-
SEN E, ed, Odontologi '89. Kobenhavn: Munksgaard 1989: 161, ,' ; 29, DEMIRJIAN A , GOLDSTEIN H , TA^JNER
JM, A new system of dental age assessment. Hum Biol 1973; 45: 211-27, 30, KREIBORG
S, JENSEN
BL,
BJORK
A,
SKIELLER V, Abnormalities of the c'ranial base in cieidocranial dysostosis. Am J Orthod 1981; 79: 549-57, 31, MAGNUS WW, SANDS NR, Cieidocranial
dysostosis. Report of a case. Am J Orthod 1974; 65: 6 3 8 ^ 3 , 32, RusHTON MA, An anomaly of cementum in eleido-cranial dysostosis, Br Dent J 1956; 100: 81-3, 33, ALDERSON C G P , Hereditary eleido-cranial dysostosis, A case report, Br Dent J 1960; 108: 157-9,
Birgit Leth Jensen and Sven Kreiborg Institute of Pediatric Dentistry, Royal Dental College, Copenhagen, Denmark
Jensen BL, Kreiborg S: Development of the dentition in cieidocranial dysplasia, J Oral Pathol Med 1990; 19: 89-93, The purpose of the present investigation was to describe the formation, maturation and eruption of the dentition, including supernumerary teeth in a sample of patients with cieidocranial dysplasia. The dentition was evaluated from orthopantomograms, intraoral radiographs, cephalometric films, surgically removed teeth and intraoral photographs in 19 patients (9 men, 10 women), aged 3,5 to 34 years. Formation of primary teeth was normal, whereas all patients but one had supernumerary permanent teeth. Frequency of supernumerary teeth ranged from 22% in the maxillary incisor region to 5% in the molar regions. Supernumerary teeth were formed lingually and occlusally to the normal teeth. Maturation of the primary dentition was normal, while permanent teeth were delayed from 1 to 4 yr. Supernumerary teeth were delayed about 4 years in relation to normal permanent teeth. Eruption of primary teeth was normal, whereas all patients had severe eruption problems of permanent teeth. It was hypothesized that the dental lamina for both primary and permanent dentition is normal, but does not resolve completely and therefore may form supernumerary teeth. Abnormalities of tooth morphology is related to inadequate space and arrested eruption. Delayed or arrested eruption is probably caused by diminished resorption of bone and of primary teeth and to the presence of multiple supernumerary teeth.
Cieidocranial dysplasia (CCD) is an autosomal dominant skeletal dysplasia with severe dental abnormalities. The most characteristic clinical sign is the eruption failure of the permanent dentition. The primary dentition seems to develop in a relatively normal way (1-8), while the permanent dentition is severely disturbed with predisposition for multiple supernumerary teeth, failure of eruption, ectopia and abnormal tooth morphology, especially involving the roots (9-19), Although CCD is a generalized bone dysplasia (20-21), the extensive literature is mainly focused on dental problems (5, 7, 18, 22-26), because of the patients' concern about their dentofacial appearance. Dental treatment is difficult and time consuming, often requiring surgical, orthodontic and prosthodontic interventions. Most reports in the literature represent case histories dealing with these complex therapeutic problems. Longitudinal studies of the development of the permanent dentition and supernumerary teeth have not yet been carried out and literature on dental maturity is extremely scarce (27, 28), The explanation for the absence of longitudinal information appears to be related to the fact that CCD patients are not
aware of any problems until the age of about 10 yr, when the eruption difficulties in the permanent dentition have become a serious psychosocial problem. The purpose of the present investigation is, as part of a larger craniofacial study on CCD, to describe the formation, maturation and eruption of the dentition, including supernumerary teeth, in a relatively large sample. Material and methods
The patient population consisted of 22 white subjects (10 males, 12 females) with CCD, ascertained through the Institutes of Pediatric Dentistry and Orthodontics at the Royal Dental College in Copenhagen, Most patients ( H = 1 5 ) were followed longitudinally during their growth period. Age range was 31^ to 34 yr. The number of observations per patient ranged from 1 to 21, In the longitudinal series several patients were followed with annual examinations. Pedigrees were available for 14 patients (Fig. 1), Intraoral radiographs, orthopantomograms and/or cephalometric films in several projections were available for all subjects. Recording of supernumerary teeth, dental maturity and dental morphology were judged from intraoral ra-
Key vi/ords: cieidocranial dyspiasia; dental lamina; supernumerary teeth; tooth eruption; tooth formation; tooth maturation; tooth morphology, Birgit Leth Jensen, Institute of Pediatric Dentistry, Royai Dental Coliege, 20 Nr, Alie, DK-2000 Copenhagen N, Denmark, Accepted for publication November 12, 1989,
diographs, orthopantomograms, intraoral photographs and from surgically removed teeth. Dental maturity was scored according to the assessment system of DEMIRJAN et al. (29), and
com-
pared to available normative data. Three patients were excluded from the present study because of multiple extractions or incomplete material. The youngest patient had to be excluded from registration of abnormal tooth formation, Orthopantomograms of 14 patients could be evaluated for dental maturity. The differences between the means were tested using Student's t test. The level of significance chosen was 0,01, Results Tooth formation
Primary dentition - The youngest patient, a 3'/, yr old girl, had a complete primary dentition with teeth of nonnal size and shape. Several older patients also had a complete primary dentition at the first examination and all but one patient had several persisting deciduous teeth. No patients showed signs of abnormal tooth formation or deviation in number. Permanent dentition - Of 18 eval-
uated patients, 17 had supernumerary
90
JENSEN & KREIBORG
normal tooth. The frequency of supernumerary maxillary central incisors was D—r-O 1 more than ten times higher than the D I • n D-r-« n an n frequency for lateral incisors. Also, the n frequency of supernumerary first premoiars was about two times higher than for second premoiars in both jaws. Two patients had a nearly complete n 107 T n *9153 0 2760 K 1065 Z supernumerary dentition anterior to the DI 5739 P, 5740 P, in 9153 0, 9154 0 G 1513 first molars. In one of these patients, 5741 P the only "missing" teeth in the supernumerary dentition were the upper lateral • AFFECTED MALE incisors and the lower central incisors. D UNAFFECTED The other patient "lacked" only one D-p-O I O-r—O AFFECTED FEMALE maxillary second premolar and the O UNAFFECTED D-]-i n mandibular four incisors. • ) MUSCULAR From the longitudinal series of ortho• m @ DYSTROPHY pantomograms and roentgencephalo1 A MONOZYGOTIC grams it was observed that the supernuE L 5053 5731 U 345 0 TWINS merary tooth germs developed lingually in 3425 R to the normal teeth, and in the canine Fig. I. Pedigrees, and premolar regions they developed occlusally to the normal teeth (Figs, 2A, B). Table 1, Distribution of supernumerary teeth according to sex About one third of the 18 patients Individuals iSupernumerary had supernumerary molars. One pan teeth n X SD P t tients had an extra second mandibular molar bilaterally situated between the Males 9 11 8,6 1.1 2,0 0,05 Females 9 6,4 58 3,8 first and second molars, the other extra molars all developed distal to the third molar as fourth and even fifth molars. It is noteworthy that the monozygotic Table 2, Percentage of supernumerary teeth according to main region, « = 135 female twins (Fig, I A), both produced supernumerary teeth only in the incisor Incisor Canine Premolar Molar Total and canine regions; however, one had 8 and the other 10 extra teeth. Maxilla 22,2 15,6 11,9 5,2 54,8 Morphology of supernumerary teeth Mandible 12,6 11,9 5,9 45,2 14,7 always resembled the corresponding Total 34,8 27,5 26,6 11,1 100,0 normal teeth. However, malformed crowns and roots were observed frequently. Among the normal permanent teeth in addition to the normal teeth. gion of the maxilla; the frequency in the teeth no malformed crowns were obThe number ranged from 1 to 21 extra mandibular incisor region and in the served, but malformed roots with tooth germs in individual cases with a canine and premolar regions was fairly marked defections (Fig, 3) were mean of 8 teeth. The frequency of super- similar, ranging from 12 to 15%, The common. numerary teeth was somewhat higher in frequency of supernumerary molars was males than in females (Table I), How- about 5% in each jaw. In general there ever, the difference was not significant was a high degree of symmetry in the Tooth maturation at the 0,01 level. occurrence of supernumerary teeth. In Primary dentition - Maturity of the priIn Table 2 the location of the super- Table 3 the distribution of supernumer- mary teeth could not be assessed in any numerary teeth is tabulated according ary teeth is tabulated according to indi- detail because of the age distribution of to main regions. The highest frequency vidual tooth type. No case revealed the sample. It should be noted, however, (22%) was observed in the incisor re- more than one supernumerary tooth per that the youngest child in the study at D—pO
I
D—|-HD I
Table 3, Distribution of supernumerary teeth in 18 patients with Cieidocranial Dysplasia according to individual teeth Incisor
Premolar
Central n %
Lateral n %
Canine n %
n
%
Second n %
Maxilla Mandible
28 9
20,8 6,7
2 8
1,5 5,9
21 16
15,6 11,9
II 12
8,1 8,8
5 8
3,7 5,9
Total
37
27,5
10
7,4
37
27,5
23
16,9
13
9,6
First
c
Dist, mol n %
n
%
2
_ I ,5
7 6
5,1 4,4
74 61
54,8 45,2
2
1,5
13
9,6
135
100,0
Molar
n _
Total
J
Dentition in cieidocranial dysplasia 91 group. Thereafter, the delay became progressively marked, the average being about 4 yr in the 18-23 yr age group. Supernumerary teeth were markedly delayed in all patients when compared to both controls and to the earlier forming normal permanent teeth. Longitudinal observations of the premolar regions revealed about a 4-yr delay. Also, when fourth and fifth molars developed, their formation was initiated approximately 4 yr after formation of the preceding molar. In general, calcification of supernumerary teeth was initiated when the preceding tooth showed completion of crown formation. Tooth eruption
Fig. 2. A, orthopantomogram illustrating formation of supernumerary teeth in premolar region, B, same patient at a later developmental stage.
age 314 y showed completed root formation of the primary teeth. Thus, she had no marked delay in dental maturity, if any. Permanent dentition - Dental maturi-
ty of the normal permanent teeth, assessed by the method of DEMIRJAN et al. (29), is presented graphically in Fig, 4, On the average dental maturity was delayed about 1 yr in the 7-9 yr old age
Primary dentition - Eruption of primary teeth was uneventful in all patients except one girl, who had delayed eruption of a second primary molar (erupting at about 8 yr of age). Retention of primary teeth was not observed in any case. Permanent dentition - All patients had eruption problems in the permanent dentition, both in regions with and without supernumerary teeth, A single patient without supernumerary teeth showed delayed, but spontaneous eruption of all teeth except a maxillary canine. The remaining patients had a range of spontaneous eruption from 4 to 22 permanent teeth. It was noteworthy that all first permanent molars erupted spontaneously, but with considerable delay (about 3 yr). Apart from third molars, the premoiars and canines showed the least tendency to erupt, Ectopie tooth position was most frequent in the premolar and canine regions. However, normal permanent tooth germs were fonned at their normal sites in the dental arch and then, during the very slow eruption process, and while supernumerary teeth were developing, they were displaced to an aberrant eruption path and became retained in the jaws (Figs, 2A and B), In the many cases with failure of permanent tooth eruption, the primary teeth persisted; in fact, one patient presented with a full primary dentition at the age of 16, Especially around unerupted second and third molars dental cysts developed leading to a gross displacement of the teeth in several cases (Fig, 5), Discussion
Fig. 3. Surgically removed teeth from a CCD patient.
The formation, maturation and eruption of the primary teeth in the CCD
92
JENSEN & KREIBORG
DENTAL AGE
5
DENTAL MATURITY IN PATIENTS WITH CLEIDOCRANIAL DYSPLASIA N = 14
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23 AGE IN YEARS
Fig. 4. Graph of dental maturity in 14 CCD patients.
patients was relatively normal. In fact the only anomaly observed in the present study was a slight delay in eruption in a few cases. This finding is in agreement with several previous studies (1, 3—4, 13), The most marked and consistent disturbance observed was the extreme delay or arrest of physiologic root resorption and shedding of the teeth. This finding is probably related to the generalized reduced bone resorption observed in the jaws in CCD (30) and to the lack of eruption of the permanent teeth. The formation and maturation of the permanent teeth was fairly normal in the early stages of development. However, with a delay of about 3-4 yr, all but one patient developed supernumerary teeth. The delay in the development of the supernumerary teeth has previously been described by one of us (28) in one of the cases in the present study.
but we have found no mention of it in previous studies, probably because of the lack of longitudinal data. It was noteworthy that supernumerary teeth developed upon completion of normal crown formation in the permanent dentition at predictable locations lingually and occlusally to the normal permanent teeth. Supernumerary teeth developed in all regions (incisor, canine, premolar, molar). More than one supernumerary tooth per normal tooth was never observed. In cases with multiple supernumerary teeth, the relative onset of formation of these teeth corresponded to that observed in the normal permanent dentition. When supernumerary molars were observed they generally developed distally to the third molar as fourth and even fifth molars, invariably being fonned at the time of crown completion of the preceding molar in a manner similar to that normally
Fig. 5. Orthopantomogram with denial cysts around the mandibular third molars.
observed in development of second and third molars. Two of the longitudinal cases developed a nearly complete supernumerary permanent dentition anterior to the first molars. In one of these cases the "second permanent dentition" only had "aplasia" of the maxillary lateral incisors and the mandibular central incisors, imitating the localization often seen in normal individuals. Similarly, the other case lacked only one maxillary second premolar and the mandibular four incisors in the supernumerary dentition. Formation of supernumerary permanent teeth in CCD can be explained by incomplete or severely delayed resorption of the dental lamina, which is then reactivated at the time of crown completion in the normal permanent teeth, A similar mechanism would appear to operate in the molar region. If the dental lamina for the molars is not resolved, it may extend distally to form supernumerary molars with the "expected" time intervals. The hypothesis of incomplete or markedly delayed resorption of the dental laminas is in accordance with previously reported abnormal bone remodeling in CCD with diminished bone resorption (5, 7, 30), Thus, it would seem that development of supernumerary teeth in CCD can be directly related to the generalized bone dysplasia. Malformed crowns and roots of supernumerary teeth can be explained by poor spacing in the jaws during their development and to their retention in the jaws. As already mentioned, crown morphology of the first permanent teeth is normal, although their roots most often become abnormal and dental maturity becomes progressively delayed (27), Both findings ean be explained by lack of eruption. In previous literature, the severely delayed or arrested eruption of permanent teeth has been ascribed to various factors: 1) The presence of multiple supernumerary teeth (31), 2) malformed roots with lack of cellular cementum (32, 33), 3) the jaw bone being too dense (9), and 4) abnormal resorption of bone and of primary teeth (5, 6, 9), As already indicated, we favor the last hypothesis which will now be further substantiated. The finding that primary teeth and the permanent first molars always erupt, but with some delay, may be explained by their superficial localization in the jaws with minimal bone coverage. The finding of arrested eruption even in regions without supernumerary teeth supports the hypothesis of abnormal re-
Dentition in cieidocranial dysplasia 93 modeling, which may also explain the delayed or arrested resorption of the primary roots. Supernumerary teeth never erupted spontaneously, and their presence occlusal to the nonnal permanent teeth undoubtedly contributes to arrested eruption, secondary migration and to ectopie localization of these teeth. The chaotic picture of the dentition in CCD most often reported in the literature, (multiple supernumerary teeth, multiple tooth abnormalities, lack of tooth eruption, ectopie localization of teeth etc) now has a simpler interpretation on the basis of this study. Two probably interrelated factors seem to be of primary importance: Incomplete resorption of the dental lamina and of primary teeth and markedly delayed bone resorption. Both findings are explained by the generalized bone dysplasia in CCD, Incomplete resorption of the dental lamina creates multiple supernumerary teeth in a rather,predictable fashion and the presence of these combined with delayed bone and primary root resorption are responsible for the multible retentions, which again lead to malformed roots and migration of permanent teeth. These findings are important for improved treatment strategies of the dental abnormalities in CCD, Based on the findings in the present study and the findings in the literature it would seem conceivable that: 1) the dental lamina for the primary and permanent dentition is normal, 2) the dental lamina does not completely resolve at the expected time, 3) at the time of completed crown formation of the permanent teeth, the remnants of the dental lamina are reactivated to form supernumerary teeth, 4) the dental lamina for the molars is not resolved in all patiens, but extends distally to form supernumerary molars, with "expected" intervals, 5) abnormalities of supernumerary crown and root morphology can be related to the inadequate space in the jaws during their development and to their retention in the jaws, 6) the abnormalities of root morphology in normal permanent teeth are secondary to arrested eruption, 7) ectopie location of normal permanent teeth is caused by migration secondary to arrested eruption and interference with supernumerary teeth in the region, and 8) severely delayed or arrested eruption of normal permanent
J Roentgenol 1974; 121: 5-16,
teeth is probably caused primarily by diminished bone resorption, diminished resorption of the roots of the primary teeth, and secondarily by the presence of multiple supernumerary teeth.
16, MONTEIL M, ViALATEL C, Le probleme dentaire dans la dysostose cleidocranienne, Actualites Odonto-Stomatol 1974; 105: 441-51, 17, JARVINEN S, Dental findings in three cases of cieidocranial dysostosis, Proc Finn Dent Soc 1980; 76: 56-61, Acknowledgment - We are grateful to Drs ARNE BjflRK, Copenhagen, and BIRTE PRAHL18, HuTTON CE, BiXLBR D, GARNER LD, ANDERSEN, Amsterdam for giving us access Cleidoeranial dysplasia - treatment of to their material on CCD patients. Also, we dental problems: report of a case, J Dent wish to thank Dr, MICHAEL M, COHEN for Child 1981; 11: 456-62,
his critical appraisal of the English, The study was supported by a grant from "Ingeborg and Leo Dannin's Fund",
19,
KlRSON L E , SCHEIBER R E , TOMARO A J ,
Multiple impacted teeth in cieidocranial dysostosis. Oral Surg 1982; 54: 604-5, 20, SPRANGER J, Cieidocranial dysplasia. In: SPRANGER J, LANGER LO, WIEDEMANN
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