- Email: [email protected]
Cephalometric findings in three cases of cleidocranial dysostosis
Cephalometric findings in three eases of cleidocranial dysostosis Seppo Jarvinen, Dr. odont. Kuopio,
Finlund Three cuses oj’cleidocruniul dysostosis, invoh?ng one boy und two girls 13 to 15 yur., oj’agr, ~t’ere unulyzed cephalometricully. All the cuses were characterized by real maxillary prominence in addition to mandibular prognuthism. Although the skeletul development of the face in the present cases was utypicul ruther than typicul oj cleidocruniul dysostosis, the possible individual vuriution should be noticed when u person with abnormal skeletul de\,elopment is analyzed cephalometricull~ for diugnostic purposes. Key words: Orthodontics, cephalometrics, malformations, craniofacial morphology
cleidocranial
dysostosis, congenital
T
he facial and dental symptoms of cleidocranial dysostosis have been described by various authors. In patients affected by this syndrome, the most common orthodontic problems have been reported as disharmony between the jaws due to retrusion of the middle face and mandibular prognathism, the formation of multiple supernumerary teeth, and the failure of many normal permanent teeth to erupt.2, 6* B,g, 11, I3 Case reports The patients reported in this article were three Finnish children with cleidocranial dysostosis , one boy and two girls, 13 to 15 years of age. The girl in Case 2 was the sister of the boy in Case 1. Frontal cephalic radiographs of the children (Figs. 1A, lB, and 1 C) showed changes typical of cleidocranial dysostosis-limited union of the frontal bones and open fontanelles. lo The dental development of these children, with occurrence of multiple supernumerary teeth and delay or failure of many permanent teeth to erupt (Figs. 2A, 2B, and 2C), has been previously described.6 Cephalometric measurements in these cases were made from tracings of lateral cephalometric radiograms (Fig. 3), which were analyzed by the Jarabak cephalometric analysis method.5 The angular measurements were made within an accuracy of 0.5”, and the dimensional measurements within an accuracy of 0.5 mm. The results of the analyses are presented in Figs. 4 to 6. DISCUSSION Facial disharmony in patients affected by cleidocranial dysostosis has been described as characterized by retrusion of the maxilla, with real or false mandibular prognathism.2’g’1’ The present findings are partly divergent. From
184
the Department
of Dentistry,
University
of Kuopio
0002.9416/81/020184+08$00.80/0
0
1981 The C. V. Mosby Co.
Vdume 19 Number 2
Fig. 1A. Case 1. Posteroanterior cephalic frontal bones and open fontanelles.
Cleidocranial
radiograph
of 13-year-old
boy showing
dysostosis
limited
union
185
of the
In each case described in this article the saddle angle (NSAr) was unusually small (104 to 112 degrees). Such a circumstance generally causes facial prognathism. ‘7 7 According to this finding, the values of the SNA angle were remarkably high (90 to 97 degrees), indicating an anterior relation of the maxilla to the anterior cranial base. Likewise, the very high values of the SNB angle (87.5 to 97 degrees) indicated marked mandibular prognathism. Further, the Class III tendency was specially shown by the unusual lingual inclination of the lower incisors (65.5 to 76 degrees). A slight anteroposterior disharmony between the jaws, the mandible being anteriorly related to the maxilla, seemed to exist in Cases 1 and 3, where the ANB angle’* was 0. By contrast, the ANB angle (3 degrees) in Case 2 was within the limits of the normal range. However, the impression of normal or nearly normal sagittal relation between the jaws in the present cases is regarded as misleading. As demonstrated by Jacobson,” the ANB angle can be erroneous in persons with facial prognathism. According to the morphologic traits discussed above, the mandible was anteriorly related to the maxilla in the present cases.
Am. .I. Onhod. Fehruai-j 198 I
186 Jiirvinrn
Fig. 16. Case 2. Posteroanterior cephalic frontal bones and open fontanelles.
radiograph
of 14-year-old
girl showing
limited
union
of the
Another interesting finding was the remarkable length of the mandibular ramus in each case (57 to 66.5 mm.). Because of the relative height of the posterior face, the face height ratios were within a range of 70 to 83 percent, indicating a counterclockwise growth of the mandible. In Cases 1 and 3 the maxilla also was anteriorly rotated. The three cases of cleidocranial dysostosis presented in this article were characterized by real maxillary prominence in addition to mandibular prognathism. Previous articles have emphasized pseudoprognathism as a result of the midface deficiency and underdevelopment of the maxilla and the maxillary sinuses.2* 9, ” A comparison between the present findings and earlier clinical reportsgj I1 seems to indicate that the skeletal development of the face, as well as the dental development,” in cases of cleidocranial dysostosis can be characterized by marked individual variation. Because of the great number of cases with retrusion of the maxilla and pseudoprognathism presented or referred to in the literature,2, g, 11, l3 it may be considered that the present three cases showed features that are atypical rather than typical of cleidocranial dysostosis.
Vl?lume 19 Number 2
Cleidocrunial
Fig. 1 C. Case 3. Posteroanterior cephalic fronl tal bones and open fontanelles.
Fig. 2A. Case 1. Orthopantomogram dela y or failure of many permanent
radiograph
of 19year-old teeth to erupt.
of 1 F&year-old
boy showing
girl showing
formation
dysostosis
limited
of supernumerary
187
union
teeth
of the
188
Am. J. Orrhod. February 1981
Jiirvinen
Fig. 28. Case 2. Orthopantomogram delay or failure of many permanent
of 14-year-old teeth to erupt.
girl showing
formation
of supernumerary
teeth
and
Fig. 2C. Case 3. Orthopantomogram delay or failure of many permanent
of 1 B-year-old teeth to erupt.
girl showing
formation
of supernumerary
teeth
and
Volume 19 Number 2
Cleidorranial
dysostosis
199
Fig. 3. Cephalometric points, planes, and angles used in the study. S-N: Sella-nasion plane (anterior cranial base). 4Ac Sella-articulare plane (posterior cranial base). Ar-Go: Ramus line (ramus height). Go-Gn: Mandibular plane (corpus length). Pm-SP: Palatal plane. 1s: Upper incisor length axis. $1 Lower incisor length axis. N-Gn: Anterior face height. S-Go: Posterior face height. NSAc Saddle angle. SArGo: Articular angle. ArGoGn: Gonial angle. S-N/I,: Upper incisor inclination. GoGnll,: Lower incisor inclination. WA: Maxillary apical base relation to anterior cranial base. Despite the analysis method described, the point A (subspinale) by Downs3 was used. SNB: Mandibular apical base relation to anterior cranial base. ANB: Sagittal jaw relation (mathematical difference between the SNA and SNB angles). S-N/Go-Gn: Mandibular plane angle. S-N/Pm-Sp: Palatal plane angle. SNA=
9o”
74 mm
Anterior = 125 mm Posterior 2 91 mm
Fig. 4. Case
Face face
height height
1. Cephalometric tracing of 19year-old boy. In spite of the ANB angle of 0 degrees, which indicates only slight sagittal disharmony between the jaws, the mandible is clearly prognathic. The skeletal profile is characterized by total facial prognathism. Observe the high SNA angle (90 degrees), the low saddle angle (112 degrees), the long mandibular ramus (59 mm.), and the unusual lingual inclination of the lower incisors (74 degrees). The face height ratio is 73 percent.
Am. J. Orthod. Frbruury 198 1
SNA-
Posterior = 85.5 mm
face
90.5’
height
Fig. 5. Case 2. Cephalometric tracing of 14-year-old girl. The ANB angle is 3 degrees, indicating a normal sagittal relation between the jaws. However, the mandible is prognathic, and the skeletal profile is characterized by total facial prognathism. Observe the high SNA angle (90.5 degrees), the low saddle angle (110 degrees), the long mandibular ramus (57 mm.), and the very strong lingual inclination of the mandibular incisors (65.5 degrees). The face height ratio is 70 percent. Summary
and
conclusions
The purpose of this article was to analyze cephalometrically three Finnish children, 13 to 15 years of age, affected by cleidocranial dysostosis. The results revealed the following: 1. The three cases of cleidocranial dysostosis analyzed were characterized by real maxillary prominence in addition to mandibular prognathism. Because of the great number of cases with retrusion of the maxilla and pseudoprognathism presented in previous reports, it might be considered that the present children had atypical rather than typical cases of cleidocranial dysostosis. The findings, however, seemed to indicate that the skeletal development of the face in cases of this syndrome can be characterized by marked individual variation. 2. In persons with abnormal skeletal development, the use of a single angle (for example, the ANB angle) as a diagnostic aid in cephalometric analysis can be misleading. The diagnosis should be determined, therefore, by individual criteria in every case, with evaluation of the individual characteristics of the whole facial skeleton. REFERENCES I. Bjiirk, A.: ORTHOD.
The nature 37:
106-124,
of facial
prognathism
and its relation
to normal
occlusion
of the teeth,
2. BjOm, H., and Grahnbn, H.: Cleido-cranial dysostosis, Odontol. Revy 17: 167-175, 1966. 3. Downs, W. B.: Variation in facial relationships, their significance in treatment and prognosis, ORTHOD.
34: 812-840,
AM.
J.
AM.
J.
1951.
1948.
Volume 79 Number 2
Cleidocranial
\ )/ 73.5
Anterior = 116 mm Posterior =96mm
Fig. 6. Case
face face
SNA= 97’ SNB97’ ANB=O”
mm
height height
3. Cephalometric tracing of 15year-old slight sagittal disharmony between the jaws. However, characterized by total facial prognathism. Observe the low saddle angle (104 degrees), the long mandibular the mandibular incisors (76 degrees). The face height rotated (S-N/Pm-SP = -5 degrees).
girl. The ANB angle the Class Ill tendency unusually high SNA ramus (66.5 mm.), ratio is 83 percent.
is 0 degrees, indicating only is obvious, and the face is angle (97 degrees), the very and the lingual inclination of Also, the maxilla is anteriorly
4. Jacobson, A.: Application of the “Wits” appraisal, AM. J. ORTHOD. 70: 179-189, 5. Jarabak, J. R., and Fizzell, J. A.: Technique and treatment with light-wire edgewise Louis, 1972, The C. V. Mosby Company, vol. 1, pp. 128-166. 6. Jarvinen, S.: Dental findings in three cases of cleidocranial dysostosis, Proc. Finn. 1980. 7. Jarvinen, S.: Relation of the SNA angle to the saddle angle, AM. J. ORTHOD. 78: 8. Kalliala, E., and Taskinen, P. J.: Cleidocranial dysostosis: Report of six typical cases Oral Surg. 15: 808-822, 1962. 9. Magnus, W. W., and Sands, N. R.: Cleidocranial dysostosis: Report of a case, 638-643,
191
dysostosis
1976
appliances, Dent.
Sot.
670-673,
ed. 2, St. 76: 56-61, 1980.
and one atypical AM.
J. ORTHOD.
case, 65:
1974.
10. Meschan, 1.: Analysis of roentgen signs in general radiology. 1ntroductioniSkeletal system (including joints, skull and spine), Philadelphia, 1973, W. B. Saunders Company, Vol. 1, pp. 182-229. 11. Miller, R., Sakamoto, E., Zell, A., Arthur, A., and Stratigos, G. T.: Cleidocranial dysostosis: A multidisciplinary approach to treatment, J. Am. Dent. Assoc. 96: 296-300, 1978. 12. Riedel, R. A.: An analysis of dentofacial relationships, AM. J. ORTHOD. 43: 103-119, 1957. 13. Weintraub, G. S., and Yalisove, I. L.: Prosthodontic therapy for cleidocranial dysostosis: Report of case, J. Am. Dent. Assoc. 96: 301-305. 1978. Karjusaari SF-15270
Luhti
24, Finlund
Finlund Three cuses oj’cleidocruniul dysostosis, invoh?ng one boy und two girls 13 to 15 yur., oj’agr, ~t’ere unulyzed cephalometricully. All the cuses were characterized by real maxillary prominence in addition to mandibular prognuthism. Although the skeletul development of the face in the present cases was utypicul ruther than typicul oj cleidocruniul dysostosis, the possible individual vuriution should be noticed when u person with abnormal skeletul de\,elopment is analyzed cephalometricull~ for diugnostic purposes. Key words: Orthodontics, cephalometrics, malformations, craniofacial morphology
cleidocranial
dysostosis, congenital
T
he facial and dental symptoms of cleidocranial dysostosis have been described by various authors. In patients affected by this syndrome, the most common orthodontic problems have been reported as disharmony between the jaws due to retrusion of the middle face and mandibular prognathism, the formation of multiple supernumerary teeth, and the failure of many normal permanent teeth to erupt.2, 6* B,g, 11, I3 Case reports The patients reported in this article were three Finnish children with cleidocranial dysostosis , one boy and two girls, 13 to 15 years of age. The girl in Case 2 was the sister of the boy in Case 1. Frontal cephalic radiographs of the children (Figs. 1A, lB, and 1 C) showed changes typical of cleidocranial dysostosis-limited union of the frontal bones and open fontanelles. lo The dental development of these children, with occurrence of multiple supernumerary teeth and delay or failure of many permanent teeth to erupt (Figs. 2A, 2B, and 2C), has been previously described.6 Cephalometric measurements in these cases were made from tracings of lateral cephalometric radiograms (Fig. 3), which were analyzed by the Jarabak cephalometric analysis method.5 The angular measurements were made within an accuracy of 0.5”, and the dimensional measurements within an accuracy of 0.5 mm. The results of the analyses are presented in Figs. 4 to 6. DISCUSSION Facial disharmony in patients affected by cleidocranial dysostosis has been described as characterized by retrusion of the maxilla, with real or false mandibular prognathism.2’g’1’ The present findings are partly divergent. From
184
the Department
of Dentistry,
University
of Kuopio
0002.9416/81/020184+08$00.80/0
0
1981 The C. V. Mosby Co.
Vdume 19 Number 2
Fig. 1A. Case 1. Posteroanterior cephalic frontal bones and open fontanelles.
Cleidocranial
radiograph
of 13-year-old
boy showing
dysostosis
limited
union
185
of the
In each case described in this article the saddle angle (NSAr) was unusually small (104 to 112 degrees). Such a circumstance generally causes facial prognathism. ‘7 7 According to this finding, the values of the SNA angle were remarkably high (90 to 97 degrees), indicating an anterior relation of the maxilla to the anterior cranial base. Likewise, the very high values of the SNB angle (87.5 to 97 degrees) indicated marked mandibular prognathism. Further, the Class III tendency was specially shown by the unusual lingual inclination of the lower incisors (65.5 to 76 degrees). A slight anteroposterior disharmony between the jaws, the mandible being anteriorly related to the maxilla, seemed to exist in Cases 1 and 3, where the ANB angle’* was 0. By contrast, the ANB angle (3 degrees) in Case 2 was within the limits of the normal range. However, the impression of normal or nearly normal sagittal relation between the jaws in the present cases is regarded as misleading. As demonstrated by Jacobson,” the ANB angle can be erroneous in persons with facial prognathism. According to the morphologic traits discussed above, the mandible was anteriorly related to the maxilla in the present cases.
Am. .I. Onhod. Fehruai-j 198 I
186 Jiirvinrn
Fig. 16. Case 2. Posteroanterior cephalic frontal bones and open fontanelles.
radiograph
of 14-year-old
girl showing
limited
union
of the
Another interesting finding was the remarkable length of the mandibular ramus in each case (57 to 66.5 mm.). Because of the relative height of the posterior face, the face height ratios were within a range of 70 to 83 percent, indicating a counterclockwise growth of the mandible. In Cases 1 and 3 the maxilla also was anteriorly rotated. The three cases of cleidocranial dysostosis presented in this article were characterized by real maxillary prominence in addition to mandibular prognathism. Previous articles have emphasized pseudoprognathism as a result of the midface deficiency and underdevelopment of the maxilla and the maxillary sinuses.2* 9, ” A comparison between the present findings and earlier clinical reportsgj I1 seems to indicate that the skeletal development of the face, as well as the dental development,” in cases of cleidocranial dysostosis can be characterized by marked individual variation. Because of the great number of cases with retrusion of the maxilla and pseudoprognathism presented or referred to in the literature,2, g, 11, l3 it may be considered that the present three cases showed features that are atypical rather than typical of cleidocranial dysostosis.
Vl?lume 19 Number 2
Cleidocrunial
Fig. 1 C. Case 3. Posteroanterior cephalic fronl tal bones and open fontanelles.
Fig. 2A. Case 1. Orthopantomogram dela y or failure of many permanent
radiograph
of 19year-old teeth to erupt.
of 1 F&year-old
boy showing
girl showing
formation
dysostosis
limited
of supernumerary
187
union
teeth
of the
188
Am. J. Orrhod. February 1981
Jiirvinen
Fig. 28. Case 2. Orthopantomogram delay or failure of many permanent
of 14-year-old teeth to erupt.
girl showing
formation
of supernumerary
teeth
and
Fig. 2C. Case 3. Orthopantomogram delay or failure of many permanent
of 1 B-year-old teeth to erupt.
girl showing
formation
of supernumerary
teeth
and
Volume 19 Number 2
Cleidorranial
dysostosis
199
Fig. 3. Cephalometric points, planes, and angles used in the study. S-N: Sella-nasion plane (anterior cranial base). 4Ac Sella-articulare plane (posterior cranial base). Ar-Go: Ramus line (ramus height). Go-Gn: Mandibular plane (corpus length). Pm-SP: Palatal plane. 1s: Upper incisor length axis. $1 Lower incisor length axis. N-Gn: Anterior face height. S-Go: Posterior face height. NSAc Saddle angle. SArGo: Articular angle. ArGoGn: Gonial angle. S-N/I,: Upper incisor inclination. GoGnll,: Lower incisor inclination. WA: Maxillary apical base relation to anterior cranial base. Despite the analysis method described, the point A (subspinale) by Downs3 was used. SNB: Mandibular apical base relation to anterior cranial base. ANB: Sagittal jaw relation (mathematical difference between the SNA and SNB angles). S-N/Go-Gn: Mandibular plane angle. S-N/Pm-Sp: Palatal plane angle. SNA=
9o”
74 mm
Anterior = 125 mm Posterior 2 91 mm
Fig. 4. Case
Face face
height height
1. Cephalometric tracing of 19year-old boy. In spite of the ANB angle of 0 degrees, which indicates only slight sagittal disharmony between the jaws, the mandible is clearly prognathic. The skeletal profile is characterized by total facial prognathism. Observe the high SNA angle (90 degrees), the low saddle angle (112 degrees), the long mandibular ramus (59 mm.), and the unusual lingual inclination of the lower incisors (74 degrees). The face height ratio is 73 percent.
Am. J. Orthod. Frbruury 198 1
SNA-
Posterior = 85.5 mm
face
90.5’
height
Fig. 5. Case 2. Cephalometric tracing of 14-year-old girl. The ANB angle is 3 degrees, indicating a normal sagittal relation between the jaws. However, the mandible is prognathic, and the skeletal profile is characterized by total facial prognathism. Observe the high SNA angle (90.5 degrees), the low saddle angle (110 degrees), the long mandibular ramus (57 mm.), and the very strong lingual inclination of the mandibular incisors (65.5 degrees). The face height ratio is 70 percent. Summary
and
conclusions
The purpose of this article was to analyze cephalometrically three Finnish children, 13 to 15 years of age, affected by cleidocranial dysostosis. The results revealed the following: 1. The three cases of cleidocranial dysostosis analyzed were characterized by real maxillary prominence in addition to mandibular prognathism. Because of the great number of cases with retrusion of the maxilla and pseudoprognathism presented in previous reports, it might be considered that the present children had atypical rather than typical cases of cleidocranial dysostosis. The findings, however, seemed to indicate that the skeletal development of the face in cases of this syndrome can be characterized by marked individual variation. 2. In persons with abnormal skeletal development, the use of a single angle (for example, the ANB angle) as a diagnostic aid in cephalometric analysis can be misleading. The diagnosis should be determined, therefore, by individual criteria in every case, with evaluation of the individual characteristics of the whole facial skeleton. REFERENCES I. Bjiirk, A.: ORTHOD.
The nature 37:
106-124,
of facial
prognathism
and its relation
to normal
occlusion
of the teeth,
2. BjOm, H., and Grahnbn, H.: Cleido-cranial dysostosis, Odontol. Revy 17: 167-175, 1966. 3. Downs, W. B.: Variation in facial relationships, their significance in treatment and prognosis, ORTHOD.
34: 812-840,
AM.
J.
AM.
J.
1951.
1948.
Volume 79 Number 2
Cleidocranial
\ )/ 73.5
Anterior = 116 mm Posterior =96mm
Fig. 6. Case
face face
SNA= 97’ SNB97’ ANB=O”
mm
height height
3. Cephalometric tracing of 15year-old slight sagittal disharmony between the jaws. However, characterized by total facial prognathism. Observe the low saddle angle (104 degrees), the long mandibular the mandibular incisors (76 degrees). The face height rotated (S-N/Pm-SP = -5 degrees).
girl. The ANB angle the Class Ill tendency unusually high SNA ramus (66.5 mm.), ratio is 83 percent.
is 0 degrees, indicating only is obvious, and the face is angle (97 degrees), the very and the lingual inclination of Also, the maxilla is anteriorly
4. Jacobson, A.: Application of the “Wits” appraisal, AM. J. ORTHOD. 70: 179-189, 5. Jarabak, J. R., and Fizzell, J. A.: Technique and treatment with light-wire edgewise Louis, 1972, The C. V. Mosby Company, vol. 1, pp. 128-166. 6. Jarvinen, S.: Dental findings in three cases of cleidocranial dysostosis, Proc. Finn. 1980. 7. Jarvinen, S.: Relation of the SNA angle to the saddle angle, AM. J. ORTHOD. 78: 8. Kalliala, E., and Taskinen, P. J.: Cleidocranial dysostosis: Report of six typical cases Oral Surg. 15: 808-822, 1962. 9. Magnus, W. W., and Sands, N. R.: Cleidocranial dysostosis: Report of a case, 638-643,
191
dysostosis
1976
appliances, Dent.
Sot.
670-673,
ed. 2, St. 76: 56-61, 1980.
and one atypical AM.
J. ORTHOD.
case, 65:
1974.
10. Meschan, 1.: Analysis of roentgen signs in general radiology. 1ntroductioniSkeletal system (including joints, skull and spine), Philadelphia, 1973, W. B. Saunders Company, Vol. 1, pp. 182-229. 11. Miller, R., Sakamoto, E., Zell, A., Arthur, A., and Stratigos, G. T.: Cleidocranial dysostosis: A multidisciplinary approach to treatment, J. Am. Dent. Assoc. 96: 296-300, 1978. 12. Riedel, R. A.: An analysis of dentofacial relationships, AM. J. ORTHOD. 43: 103-119, 1957. 13. Weintraub, G. S., and Yalisove, I. L.: Prosthodontic therapy for cleidocranial dysostosis: Report of case, J. Am. Dent. Assoc. 96: 301-305. 1978. Karjusaari SF-15270
Luhti
24, Finlund