Prosthodontic therapy for cleidocranial dysostosis: report of case Cleidocranial dysostosis is a disease o f un known causation, characterized by disabling oral anomalies. The restoration of function and appearance via a multidisciplinary approach to therapy is discussed and illustrated.
Gerald S. Weintraub, DDS
R e p o rt o f case
Irving L. Yalisove, DDS, Philadelphia
Objectives of treatment of the oral manifestations of cleidocranial dysostosis should include restor ing the vertical dimension of occlusion, building out the maxilla, establishing a functional occlusion, improving appearance and phonation, and improv ing the patient’s mental well-being.
A 14-year-old girl with a history of basically good health sought dental treatment because of the retention o f primary teeth and dissatisfaction with her appearance. The clavicles were underde veloped bilaterally and virtually absent on the right side. Body proportions were within normal limits. A lateral view of the face showed a concav ity o f the maxillary region. These physical charac teristics were suggestive o f cleidocranial dysos tosis. The patient’s half sister was born with a clubfoot; there was no other history o f anomalies. The patient’s health history showed that she was a full-term baby weighing 3,780 gm (8 lb, 7 oz). She had the usual childhood diseases. A con genital hip dislocation was treated surgically at age 12. Her parents reported that she sucked her thumb until 6 or 7 years of age. Professional den tal care was not sought. JADA, Vol. 96, February 1978 ■ 301
Fig 1 ■ Pretreatm ent radiograph shows no restorations.
Results o f an oral examination showed 14 per manent teeth had erupted. The maxillary arch contained the right lateral incisor, right premolar, and first and second molars bilaterally. The erupted permanent mandibular teeth included the central and lateral incisors and the first and sec ond molars. Primary teeth present in the maxilla were the central incisors, left lateral incisor, canines, first molars, and left second molar. Man dibular primary teeth present were the canines and molars. There was carious involvement of many primary and permanent teeth. N o restora tions were present (Fig 1). An oral radiographic survey disclosed numer ous unerupted teeth present in the mandible and the maxilla. Supernumerary teeth were noticed in all four premolar regions. N o supernumerary teeth were present in the molar region. Results o f the oral examination and radiographic findings that indicated an underdevel oped maxilla, normal mandibular growth and de velopment, pseudoprognathism, retention o f pri mary teeth, failure o f eruption of succedaneous teeth, numerous unerupted supernumerary teeth predominantly in the premolar region, and nor mal eruption o f permanent molars— all peculiar to cleidocranial dysostosis— led to a diagnosis of that condition.
Treatment The treatment plan was divided into three phases, the first of which was to establish a mandibular plane of occlusion by surgical and orthodontic intervention, thus avoiding the use of fixed or removable prosthetic devices in the mandibular 302 ■ JADA, Vol. 96, February 1978
arch. The second and third phases involved re habilitation o f the maxillary arch. ■ Phase 1: Cast gold crowns were placed on the maxillary right and left first molars and the man dibular left first molars to assist in establishing the vertical dimension o f occlusion. The patient was hospitalized for the surgical removal o f all man dibular primary and supernumerary teeth. At the same time, the permanent canines and premolars were exposed and orthodontic therapy initiated to achieve eruption and alignment. N o agreement o f the results o f orthodontic therapy was found in the literature. Although Lubowitz1 reported about a patient treated suc cessfully with orthodontics, Kalliala and Taskinen2 described orthodontic measures to bring
THE AUTHORS
WEINTRAUB
YALISOVE
Dr. W eintraub is assistant professor and d i re ctor o f removable prosthodontics, and Dr. Yalisove is associate professor, departm ent of restorative dentistry, University of Pennsylvania School of Dental Medicine, 4001 Spruce St, A1, Philadelphia, 19104.
Fig 2 ■ Final cast of m axilla in preparation fo r con structio n o f im m ediate interim prosthesis.
about the mechanical eruption of a few strategic teeth for use as abutments for either fixed or re movable partial dentures as being futile. They indicated that complete dentures eventually have to be made. Orthodontic treatment o f the patient in this case report resulted in a reasonable mandibular plane o f occlusion (with the exception o f the left canine), against which the maxillary arch was restored. ■ Phase 2: An immediate interim maxillary re movable partial denture was planned before the construction of a “ definitive” prosthesis. All pri mary teeth were extracted except the left primary canine, which was to serve as an anterior vertical stop along with the permanent right lateral in cisor. Two maxillary right premolars, which had erupted during the course of orthodontic therapy— one malposed palatally to the first molar and the other only minimally erupted, also were
extracted (Fig 2). The impacted teeth remained undisturbed. There are divergent opinions on what should be done with the unerupted teeth. Douglas and G reene3 suggested extraction o f all unerupted teeth because of the danger of cyst formation with subsequent bone destruction. Kelly and Nakamoto4 proposed a conservative approach, indi cating that unerupted teeth should remain un disturbed unless cyst formation is observed. The embedded teeth appear to serve a purpose in the formation o f a well-rounded ridge, thus enhancing the stability and retention of a removable pros thesis. In addition, the presence of these embed ded teeth may lessen bone resorption. The objectives of the interim prosthesis were to establish and maintain a vertical dimension of oc clusion, to increase the masticatory surface, to determine the labial placement o f the anterior arti ficial teeth, and to alleviate the emotional prob lems o f the patient as related to the poor appear ance o f the maxillary arch. The surgical extraction o f the designated teeth and insertion o f the immediate interim prosthesis was uncomplicated (Fig 3). Normal healing prog ressed. It was gratifying to notice an immediate and dramatic improvement in the patient’s ap pearance and self-image. The only dissatisfaction that the patient ex pressed at this time concerned the appearance of the overlaid maxillary permanent lateral incisor and primary canine (Fig 3). This problem was considered in the third phase of treatment. ■ Phase 3: The maxillary arch was prepared to receive a crown and sleeve-coping removable par-
Fig 3 ■ Occlusal (left) and labial (above) views of interim prosthesis (arrows) at tim e of insertion. A rrows indicate overlaid an terior natural teeth.
W eintraub— Yalisove: CLEIDOCRANIAL DYSOSTOSIS, PROSTHODONTIC THERAPY ■ 303
Fig 5 ■ Palatal surface of com pleted prosthesis. Notice relief in resin to allow fu rth e r eruption of prem olar (arrow). Fig 4 ■ M axillary arch prepared fo r crow n and sleeve-coping re m ovable partial denture. Notice newly erupted prem olar (arrow).
tial denture as described by Y alisove.5-7 This type of prosthesis is a definite consideration in partially edentulous patients where the vertical dimension o f occlusion is to be restored and supported by the prosthesis. This device does not cause injury to the abutment teeth in that the secondary crowns, seated on the sleeve-copings, pivot and rotate when horizontal forces are delivered to the pros thesis, thus relieving the abutment teeth from the application of potentially destructive forces. Gold copings were placed on the first and second mo lars. The primary canine was extracted and the permanent lateral incisor received a short, wellrounded coping after intentional endodontic treatment (Fig 4). This provided a solution to the
esthetic problem encountered with the interim prosthesis. Observation of other patients, in our experi ence, has indicated that extraction of primary teeth does not stimulate eruption o f the perma nent teeth. A lso, embedded teeth are not gener ally stimulated to erupt by the pressure exerted by denture bases. Wilbanks8 reported a case where all erupted teeth were extracted in preparation for complete dentures. The patient had worn the den tures more than six years and none of the 18 impacted teeth erupted as a result of pressure by the denture. It is interesting to note that a tooth erupted in the left premolar region, perhaps as a result o f stimulation by the denture base. Provi sion was made for further eruption o f this tooth by relieving the resin in the new prosthesis (Fig 4,5).
Fig 6 ■ Occlusal (left) and righ t side views of com pleted restoration.
304 ■ JADA, Vol. 96, February 1978
Fig 7 ■ Com parison of phase 1 posttreatm ent
C o n c lu s io n There is no specific treatment for the skeletal abnormalities that characterize cleidocranial dysostosis. Treatment o f the oral condition is im portant, not only to restore function but also to resolve the problems o f appearance associated with this disease. Generally, prosthodontic treatment is preferred. To preserve the primary dentition and the per manent teeth that may erupt, it is important to recognize this disorder early in the patient’s life and to initiate appropriate preventive and restora tive procedures. Kelly and Nakamoto4 listed the objectives of treatment o f the oral manifestations o f cleidocranial dysostosis as restorating the ver tical dimension o f occlusion, building out the maxilla, establishing a functional occlusion, im
and phase 3 posttreatm ent (right) results.
proving appearance and phonation, and improv ing the patient’s mental well-being. These objec tives have been realized in the treatment o f our patient (Fig 6,7).
1. Lubow itz, A.H. C leidocranial dysostosis: a case report. Angle Orthod 38:150 A pril 1968. 2. Kalliala, E., and Taskinen, P.J. Cleidocranial dysostosis: re po rt of six typical cases and one atypical case. Oral Surg 15:808 July 1962. 3. Douglas, B.L., and Greene, H.J. Cleidocranial dysostosis: re po rt of case. J Oral Surg 27:41 Jan 1969. 4. Kelly, E., and Nakamoto, R.Y. Cleidocranial dysostosis— a prostho don tic problem . J Prosthet Dent 31:518 May 1974. 5. Yalisove, I.L. Crown and sleeve-coping retainers fo r remova ble partial prostheses. J Prosthet Dent 16:1069 Nov-Dee 1966. 6. Yalisove, I.L. Removable periodontal prosthesis: the crown and sleeve-coping retainer. A lpha Omegan Sept 1972. 7. Yalisove, I., and Dietz, J. Telescopic prosthe tic therapy. Philadelphia, George F. Stickley Co., 1977. 8. W ilbanks, J.L. C leidocranial dysostosis: report of a case. Oral Surg 17:797 June 1964.
W eintraub— Yalisove: CLEIDOCRANIAL DYSOSTOSIS, PROSTHODONTIC THERAPY ■ 305
Gerald S. Weintraub, DDS
R e p o rt o f case
Irving L. Yalisove, DDS, Philadelphia
Objectives of treatment of the oral manifestations of cleidocranial dysostosis should include restor ing the vertical dimension of occlusion, building out the maxilla, establishing a functional occlusion, improving appearance and phonation, and improv ing the patient’s mental well-being.
A 14-year-old girl with a history of basically good health sought dental treatment because of the retention o f primary teeth and dissatisfaction with her appearance. The clavicles were underde veloped bilaterally and virtually absent on the right side. Body proportions were within normal limits. A lateral view of the face showed a concav ity o f the maxillary region. These physical charac teristics were suggestive o f cleidocranial dysos tosis. The patient’s half sister was born with a clubfoot; there was no other history o f anomalies. The patient’s health history showed that she was a full-term baby weighing 3,780 gm (8 lb, 7 oz). She had the usual childhood diseases. A con genital hip dislocation was treated surgically at age 12. Her parents reported that she sucked her thumb until 6 or 7 years of age. Professional den tal care was not sought. JADA, Vol. 96, February 1978 ■ 301
Fig 1 ■ Pretreatm ent radiograph shows no restorations.
Results o f an oral examination showed 14 per manent teeth had erupted. The maxillary arch contained the right lateral incisor, right premolar, and first and second molars bilaterally. The erupted permanent mandibular teeth included the central and lateral incisors and the first and sec ond molars. Primary teeth present in the maxilla were the central incisors, left lateral incisor, canines, first molars, and left second molar. Man dibular primary teeth present were the canines and molars. There was carious involvement of many primary and permanent teeth. N o restora tions were present (Fig 1). An oral radiographic survey disclosed numer ous unerupted teeth present in the mandible and the maxilla. Supernumerary teeth were noticed in all four premolar regions. N o supernumerary teeth were present in the molar region. Results o f the oral examination and radiographic findings that indicated an underdevel oped maxilla, normal mandibular growth and de velopment, pseudoprognathism, retention o f pri mary teeth, failure o f eruption of succedaneous teeth, numerous unerupted supernumerary teeth predominantly in the premolar region, and nor mal eruption o f permanent molars— all peculiar to cleidocranial dysostosis— led to a diagnosis of that condition.
Treatment The treatment plan was divided into three phases, the first of which was to establish a mandibular plane of occlusion by surgical and orthodontic intervention, thus avoiding the use of fixed or removable prosthetic devices in the mandibular 302 ■ JADA, Vol. 96, February 1978
arch. The second and third phases involved re habilitation o f the maxillary arch. ■ Phase 1: Cast gold crowns were placed on the maxillary right and left first molars and the man dibular left first molars to assist in establishing the vertical dimension o f occlusion. The patient was hospitalized for the surgical removal o f all man dibular primary and supernumerary teeth. At the same time, the permanent canines and premolars were exposed and orthodontic therapy initiated to achieve eruption and alignment. N o agreement o f the results o f orthodontic therapy was found in the literature. Although Lubowitz1 reported about a patient treated suc cessfully with orthodontics, Kalliala and Taskinen2 described orthodontic measures to bring
THE AUTHORS
WEINTRAUB
YALISOVE
Dr. W eintraub is assistant professor and d i re ctor o f removable prosthodontics, and Dr. Yalisove is associate professor, departm ent of restorative dentistry, University of Pennsylvania School of Dental Medicine, 4001 Spruce St, A1, Philadelphia, 19104.
Fig 2 ■ Final cast of m axilla in preparation fo r con structio n o f im m ediate interim prosthesis.
about the mechanical eruption of a few strategic teeth for use as abutments for either fixed or re movable partial dentures as being futile. They indicated that complete dentures eventually have to be made. Orthodontic treatment o f the patient in this case report resulted in a reasonable mandibular plane o f occlusion (with the exception o f the left canine), against which the maxillary arch was restored. ■ Phase 2: An immediate interim maxillary re movable partial denture was planned before the construction of a “ definitive” prosthesis. All pri mary teeth were extracted except the left primary canine, which was to serve as an anterior vertical stop along with the permanent right lateral in cisor. Two maxillary right premolars, which had erupted during the course of orthodontic therapy— one malposed palatally to the first molar and the other only minimally erupted, also were
extracted (Fig 2). The impacted teeth remained undisturbed. There are divergent opinions on what should be done with the unerupted teeth. Douglas and G reene3 suggested extraction o f all unerupted teeth because of the danger of cyst formation with subsequent bone destruction. Kelly and Nakamoto4 proposed a conservative approach, indi cating that unerupted teeth should remain un disturbed unless cyst formation is observed. The embedded teeth appear to serve a purpose in the formation o f a well-rounded ridge, thus enhancing the stability and retention of a removable pros thesis. In addition, the presence of these embed ded teeth may lessen bone resorption. The objectives of the interim prosthesis were to establish and maintain a vertical dimension of oc clusion, to increase the masticatory surface, to determine the labial placement o f the anterior arti ficial teeth, and to alleviate the emotional prob lems o f the patient as related to the poor appear ance o f the maxillary arch. The surgical extraction o f the designated teeth and insertion o f the immediate interim prosthesis was uncomplicated (Fig 3). Normal healing prog ressed. It was gratifying to notice an immediate and dramatic improvement in the patient’s ap pearance and self-image. The only dissatisfaction that the patient ex pressed at this time concerned the appearance of the overlaid maxillary permanent lateral incisor and primary canine (Fig 3). This problem was considered in the third phase of treatment. ■ Phase 3: The maxillary arch was prepared to receive a crown and sleeve-coping removable par-
Fig 3 ■ Occlusal (left) and labial (above) views of interim prosthesis (arrows) at tim e of insertion. A rrows indicate overlaid an terior natural teeth.
W eintraub— Yalisove: CLEIDOCRANIAL DYSOSTOSIS, PROSTHODONTIC THERAPY ■ 303
Fig 5 ■ Palatal surface of com pleted prosthesis. Notice relief in resin to allow fu rth e r eruption of prem olar (arrow). Fig 4 ■ M axillary arch prepared fo r crow n and sleeve-coping re m ovable partial denture. Notice newly erupted prem olar (arrow).
tial denture as described by Y alisove.5-7 This type of prosthesis is a definite consideration in partially edentulous patients where the vertical dimension o f occlusion is to be restored and supported by the prosthesis. This device does not cause injury to the abutment teeth in that the secondary crowns, seated on the sleeve-copings, pivot and rotate when horizontal forces are delivered to the pros thesis, thus relieving the abutment teeth from the application of potentially destructive forces. Gold copings were placed on the first and second mo lars. The primary canine was extracted and the permanent lateral incisor received a short, wellrounded coping after intentional endodontic treatment (Fig 4). This provided a solution to the
esthetic problem encountered with the interim prosthesis. Observation of other patients, in our experi ence, has indicated that extraction of primary teeth does not stimulate eruption o f the perma nent teeth. A lso, embedded teeth are not gener ally stimulated to erupt by the pressure exerted by denture bases. Wilbanks8 reported a case where all erupted teeth were extracted in preparation for complete dentures. The patient had worn the den tures more than six years and none of the 18 impacted teeth erupted as a result of pressure by the denture. It is interesting to note that a tooth erupted in the left premolar region, perhaps as a result o f stimulation by the denture base. Provi sion was made for further eruption o f this tooth by relieving the resin in the new prosthesis (Fig 4,5).
Fig 6 ■ Occlusal (left) and righ t side views of com pleted restoration.
304 ■ JADA, Vol. 96, February 1978
Fig 7 ■ Com parison of phase 1 posttreatm ent
C o n c lu s io n There is no specific treatment for the skeletal abnormalities that characterize cleidocranial dysostosis. Treatment o f the oral condition is im portant, not only to restore function but also to resolve the problems o f appearance associated with this disease. Generally, prosthodontic treatment is preferred. To preserve the primary dentition and the per manent teeth that may erupt, it is important to recognize this disorder early in the patient’s life and to initiate appropriate preventive and restora tive procedures. Kelly and Nakamoto4 listed the objectives of treatment o f the oral manifestations o f cleidocranial dysostosis as restorating the ver tical dimension o f occlusion, building out the maxilla, establishing a functional occlusion, im
and phase 3 posttreatm ent (right) results.
proving appearance and phonation, and improv ing the patient’s mental well-being. These objec tives have been realized in the treatment o f our patient (Fig 6,7).
1. Lubow itz, A.H. C leidocranial dysostosis: a case report. Angle Orthod 38:150 A pril 1968. 2. Kalliala, E., and Taskinen, P.J. Cleidocranial dysostosis: re po rt of six typical cases and one atypical case. Oral Surg 15:808 July 1962. 3. Douglas, B.L., and Greene, H.J. Cleidocranial dysostosis: re po rt of case. J Oral Surg 27:41 Jan 1969. 4. Kelly, E., and Nakamoto, R.Y. Cleidocranial dysostosis— a prostho don tic problem . J Prosthet Dent 31:518 May 1974. 5. Yalisove, I.L. Crown and sleeve-coping retainers fo r remova ble partial prostheses. J Prosthet Dent 16:1069 Nov-Dee 1966. 6. Yalisove, I.L. Removable periodontal prosthesis: the crown and sleeve-coping retainer. A lpha Omegan Sept 1972. 7. Yalisove, I., and Dietz, J. Telescopic prosthe tic therapy. Philadelphia, George F. Stickley Co., 1977. 8. W ilbanks, J.L. C leidocranial dysostosis: report of a case. Oral Surg 17:797 June 1964.
W eintraub— Yalisove: CLEIDOCRANIAL DYSOSTOSIS, PROSTHODONTIC THERAPY ■ 305
