Treatment of oral conditions associated with cleidocranial dysostosis is discussed in the follow ing three reports of cases. The firs t by M iller and associates concerns collaboration between the oral surgeon and orthodontist; the second by W eintraub and Yalisove emphasizes prosthodontic therapy; and the third by Maw, oral surgery and prosthodontics. The rationale and objectives fo r treatm ent in each particular case are given, and restoration of function and appearance bythe m ultidisciplinary approach is emphasized.
Cleidocranial dysostosis: a multidisciplinary approach to treatment
R. Miller, DDS, San Antonio, Tex E. Sakamoto, DDS, Hilo, Hawaii Allan Zell, DDS A. Arthur, DDS G. T. Stratigos, DDS, MSD, Bronx, NY
To ensure eruption of the dentition in patients with cleidocranial dysostosis, close collaboration be tween the oral surgeon and the orthodontist is es sential to determine the sequence of extraction, the teeth to be ligated, the preferred technique of ligation, and the approach for surgical exposure.
2 96 ■ JADA, V ol. 96, February 1978
Cleidocranial dysostosis is a rare syndrome of unknown causation, first described by Marie and Sainton1 in 1897. They were the first to draw attention to the following chief characteristics: various degrees o f aplasia o f one or both clavicles, delayed ossification o f the sutures and increased width o f the skull, defects in the teeth, and heredi tary transmission. Since then, extensive reviews o f the syndrome have been made and over 100 associated anom alies have been described.2,3 The syndrome is believed to be transmitted as an autosomal dom inant trait, but many cases have arisen spontane ously.4'6 The causation is unknown, but many support the mutation theory proposed by M iles7 in 1940 and Soule8 in 1946. Seldon and as sociates,9 in 1950, suggested there is evidence that hypophyseal dysfunction is the cause. It is generally believed that the acceleration o f eruption o f the succedaneous teeth is difficult, if not impossible, regardless o f the method used. Some clinicians have tried unsuccessfully to in crease the rate of eruption by giving the patient vitamins or mineral salts.2 Archer10 attempted to uncover impacted teeth by giving the patient a quarter o f a grain o f thyroid extract daily; how ever, he found no appreciable eruption. Others
have attempted unsuccessfully to stimulate the eruptive process by removing tissue over the un erupted crowns.2,1112 Orthodontists have achieved some results in correcting malposed, erupted teeth that were to be used as bridge abutments.2,5 The only excep tion to this is a case reported by Lubowitz.6 He uncovered the maxillary lateral incisors, canines, and first and second premolars, and the mandibu lar left first and second premolars and right and left canines; during a 2Vi year period, the teeth erupted into satisfactory occlusion. Although he moved the teeth a large distance to achieve satis factory occlusion, the orthodontic movement did not improve jaw growth. In young patients, conservative treatment calls for the restoration and retention of carious pri mary teeth for as long as possible because their extraction does not induce the eruption of their permanent successors.4 Others recommend the surgical removal of all impacted teeth because there is little chance for them to erupt normally.12 H owever, the early re moval of the supernumerary teeth and impacted permanent teeth could sacrifice the existing alveo lar ridge and force the patient into requiring the premature placement of a prosthetic device.
chief complaint of bleeding gingiva. Examination showed moderate, generalized gingivitis and sev eral carious primary teeth. The child’s medical history showed that she had had hip operations at ages 3 and 5. Her mother denied any other contributory incident to the med ical history. The patient’s physical stature was small and had been since birth. Examination of her head and neck region showed frontal and parietal bossing and pseudoprognathism as a result of a midface deficiency. Intraorally, she had a high, narrow, arched palate and overretained primary teeth. Her dentition consisted of her four permanent first molars and all of her primary teeth, except the mandibular right central incisors. A panoramic radiograph showed multiple supernumerary teeth and unerupted permanent teeth (Fig 1). A provisional diagnosis of cleido cranial dysostosis was made, and a complete radiographic workup was done. The radiographic findings were consistent with the diagnosis of cleidocranial dysostosis. There was no familial history, and results of routine blood and urine analyses were normal. The decision was made to extract all carious primary teeth. The extractions were done two weeks later (Fig 2). The patient was followed up by the oral surgery department on a monthly basis for seven months. At that time, because of no appreciable eruption of any teeth, the decision was made to admit the patient for surgical removal of the supernumerary
Report of case In March 1972, a 9-year-old girl was brought to the dental clinic for consultation because o f the
THE AUTHORS
MILLER
SAKAMOTO
ZELL
Dr. M iller is in the US A ir Force in San A ntonio, Tex, and Dr. Sakam oto is in private practice. B oth are form er senior resi dents, departm ent of dental and oral surgery, Lincoln Medical & Mental Health Center, Bronx, NY. Dr. Zell, an o rthod ontist, is c linical in s tru c to r in dentistry, and Dr. A rthu r is assistant clinical professor of dentistry, A lbert Einstein College of Medicine, Bronx, NY. Dr. S tratigos is associate professor of
ARTHUR
STRATI GOS
dentistry, A lbert Einstein C ollege of M edicine, and director, departm ent of dental and oral surgery, Lincoln Medical & Mental Health Center. Address requests fo r reprints to Dr. Stratigos, Lincoln Medical & Mental Health Center—A lbert Einstein C ollege of M edicine, 234 E 149th St, Bronx, NY 10451.
M iller— others: CLEIDOCRANIAL DYSOSTOSIS, MULTIDISCIPLINARY APPROACH ■ 297
Fig 1 ■ Initial panoram ic radiograph taken in March 1972.
Fig 2 ■ Panoram ic radiograph taken in A pril 1972, after removal of selected prim ary teeth.
Fig 3 ■ Panoram ic radiograph taken in O ctober 1972, after removal of 19 supernum erary teeth and fo u r prim ary molars.
teeth. On Oct 11, the patient was taken to the operating room and the four primary second mo lars and 19 impacted and unerupted supernumer ary teeth were removed from the maxilla and the mandible (Fig 3). Closure was done with 3-0 chromic sutures. She was discharged three days later, and healing was uncomplicated. After being followed up in the outpatient clinic for a year, there was no evidence o f any eruption of her per 298 ■ JADA, Vol. 96, February 1978
manent dentition. The only visible dentition at this time were the permanent first molars, man dibular central incisors, and maxillary left and mandibular right lateral incisors (Fig 4). Because o f the lack of any eruptive process after a year, on Oct 10,1973, the patient was taken to the operating room and the unerupted perma nent dentition (maxillary canines and first and second premolars, and mandibular lateral incisors
Fig 4 ■ P a tie n ts existing d e ntition in September 1973, before surgicai exposure and ligation of teeth.
> ■ Panoram ic radiographs taken in O ctober 1973, after ligation of unerupted lanent de ntition.
Fig 6 ■ Patient's d e ntition in December 1973, before initiatio n of o rth o d o n tic treatm ent.
and first and second premolars) was uncovered. These teeth were found to be covered by a dense, fibrotic tissue, which was reflected with great dif ficulty. The teeth were further exposed surgically with bone burs; in each quadrant, the first and second premolars and lateral incisors were ligated with no. 26-gauge wires. At this time, it became
evident that forces from the circumdental wires had a tendency to cause avulsion o f these un erupted teeth because o f the conical shape o f the roots o f these teeth. Surgical cement* on gauzet was packed gently around these surgically ex posed teeth. Overlying redundant alveolar tissue was excised, and 3-0 black silk sutures were used to maintain the position of the impregnated gauze (Fig 5). The patient was discharged two days lat er. The packing and sutures were removed during the next three weeks because small segments of the packing became loose. By mid-December, all o f the central and lateral incisors, first and second premolars, and first molars were visualized intraorally (Fig 6). H owever, the panoramic radio graph showed that this apparent eruption was caused only by the removal o f tissue that previ ously has been covering these teeth and that the dentition’s relative position had not changed radiographically. The patient then was referred to the consulting orthodontist for follow-up. In January 1974, banding of individual teeth was begun, with a couple o f teeth being banded weekly because of the timidness o f the patient. By the beginning o f April, orthodontic forces were being applied to the maxillary dentition via a maxillary arch wire and wire springs to upright the teeth. By July, similar orthodontic forces were applied to the mandibular teeth. It was noticed that the teeth began to erupt only when orthodon tic forces were applied. By October, the eruption o f the dentition was apparent clinically (Fig 7). It was evident from the panoramic radiograph taken in October 1975 (Fig 8), three years later from the time o f removal of the supernumerary teeth, that the root formation of the permanent teeth had been satisfactory and the periodontal condition o f all teeth appeared clinically normal; however, radiographically there was some indica tion of periodontal involvement, which actually was the result of the removal o f bone and the supernumerary teeth. As function of the teeth improves, it is expected that the alveolar bone growth will advance, with the ultimate prognosis being that of a satisfactory and periodontically sound dentition. Furthermore, the facial appear ance of the patient has improved greatly at this time.
Conclusion This case illustrates several important points in
M iller— others: CLEIDOCRANIAL DYSOSTOSIS, MULTIDISCIPLINARY APPROACH ■ 299
Fig 8 ■ Panoram ic radiograph taken in O ctober 1975. Fig 7 ■ P atient’s existing m axillary and m andibular d e ntition in O ctober 1974.
the management of a patient with cleidocranial dysostosis. In this pathological entity, any me chanical obstruction appears to completely obstruct the rate of eruption. The fibrous tissue overlying the permanent dentition, in this case, was extremely dense and thick, and it delayed the eruption. The complete uncovering o f the crowns of the permanent teeth was found to be essential to eruption. Even then, eruption was negligible until orthodontic forces were put on the teeth. The conical nature o f these succedaneous teeth, which is a result of their cemental defect, should deter the surgeon from excessive manipu lation o f these erupting teeth because they can be avulsed easily from their bony crypt. The screw or pin appliance13 is least traumatic and therefore indicated for orthodontic traction. The oral surgical approach to such a case re quires close collaboration with the orthodontist to determine the sequence o f extraction, the teeth to be ligated, the preferred technique o f ligation, and the approach for surgical exposure. Because of the protracted nature of the total treatment of these patients, cooperation and understanding between these two disciplines of dentistry are imperative.
Summary A case o f cleidocranial dysostosis has been pre sented. Its oral surgical and orthodontic ap
300 ■ JADA, Vol. 96, February 1978
proaches and rationale for treatment have been discussed. We hope this presentation will contri bute to a more aggressive approach in the total treatment of this disease.
•W ard’s surgical cement, Westward Dental P roducts Co., San Francisco, 94109. fld o fo rm gauze, Acme C otton P roducts Co., Inc., Valley Stream, NY 11582. 1. Marie, P., and Sainton, P. Cited by Rock, W.P. Cleido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 2. Kalliala, E „ and Taskinen, P.J. C leidocranial dysostosis: re po rt of six typical cases and one atypical case. Oral Surg 15:808 July 1962. 3. G orlin, R.J., and Goldman, H.M., eds. Thom a’s oral pathology, ed 6. St. Louis, C. V. Mosby Co., 1970, vol 1, p 534. 4. Shafer, W.G.; Hine, M.K.: and Levy, B.M. Oral pathology. Philadelphia, W. B. Saunders Co., 1974, p 625. 5. W inkler, S., and Jung, E.L. C leidocranial dysostosis: report of a case. Dent Dig 77:24 Jan 1971. 6. Lubow itz, A.H. C leidocranial dysostosis: a case report. Angle O rthod 38:150 Jan 1968. 7. Miles, P.W. Cited by Rock, W.P. C leido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 8. Soule, A.B. Cited by Rock, W.P. C leido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 9. Seldin, H.M., and others. Cited by Rock, W.P. Cleido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 10. Archer, W.H. Oral surgery, ed4. Philadelphia, W. B. S aunders Co., 1966, p 227. 11. W inther, J.E., and Khan, M.W. C leidocranial dysostosis: re p o rt of fo u r cases. Dent Pract Dent Rec 22:215 Feb 11, 1972. 12. Thoma, K.H. Oral surgery, ed 5. St. Louis, C. V. Mosby Co., 1969, pp 172, 380. 13. Porter, W.J., and Rider, E.A. A m ethod of anchorage fo r orth o d o n tic movement of unerupted teeth. J Oral Surg 32:513 July 1974.
Cleidocranial dysostosis: a multidisciplinary approach to treatment
R. Miller, DDS, San Antonio, Tex E. Sakamoto, DDS, Hilo, Hawaii Allan Zell, DDS A. Arthur, DDS G. T. Stratigos, DDS, MSD, Bronx, NY
To ensure eruption of the dentition in patients with cleidocranial dysostosis, close collaboration be tween the oral surgeon and the orthodontist is es sential to determine the sequence of extraction, the teeth to be ligated, the preferred technique of ligation, and the approach for surgical exposure.
2 96 ■ JADA, V ol. 96, February 1978
Cleidocranial dysostosis is a rare syndrome of unknown causation, first described by Marie and Sainton1 in 1897. They were the first to draw attention to the following chief characteristics: various degrees o f aplasia o f one or both clavicles, delayed ossification o f the sutures and increased width o f the skull, defects in the teeth, and heredi tary transmission. Since then, extensive reviews o f the syndrome have been made and over 100 associated anom alies have been described.2,3 The syndrome is believed to be transmitted as an autosomal dom inant trait, but many cases have arisen spontane ously.4'6 The causation is unknown, but many support the mutation theory proposed by M iles7 in 1940 and Soule8 in 1946. Seldon and as sociates,9 in 1950, suggested there is evidence that hypophyseal dysfunction is the cause. It is generally believed that the acceleration o f eruption o f the succedaneous teeth is difficult, if not impossible, regardless o f the method used. Some clinicians have tried unsuccessfully to in crease the rate of eruption by giving the patient vitamins or mineral salts.2 Archer10 attempted to uncover impacted teeth by giving the patient a quarter o f a grain o f thyroid extract daily; how ever, he found no appreciable eruption. Others
have attempted unsuccessfully to stimulate the eruptive process by removing tissue over the un erupted crowns.2,1112 Orthodontists have achieved some results in correcting malposed, erupted teeth that were to be used as bridge abutments.2,5 The only excep tion to this is a case reported by Lubowitz.6 He uncovered the maxillary lateral incisors, canines, and first and second premolars, and the mandibu lar left first and second premolars and right and left canines; during a 2Vi year period, the teeth erupted into satisfactory occlusion. Although he moved the teeth a large distance to achieve satis factory occlusion, the orthodontic movement did not improve jaw growth. In young patients, conservative treatment calls for the restoration and retention of carious pri mary teeth for as long as possible because their extraction does not induce the eruption of their permanent successors.4 Others recommend the surgical removal of all impacted teeth because there is little chance for them to erupt normally.12 H owever, the early re moval of the supernumerary teeth and impacted permanent teeth could sacrifice the existing alveo lar ridge and force the patient into requiring the premature placement of a prosthetic device.
chief complaint of bleeding gingiva. Examination showed moderate, generalized gingivitis and sev eral carious primary teeth. The child’s medical history showed that she had had hip operations at ages 3 and 5. Her mother denied any other contributory incident to the med ical history. The patient’s physical stature was small and had been since birth. Examination of her head and neck region showed frontal and parietal bossing and pseudoprognathism as a result of a midface deficiency. Intraorally, she had a high, narrow, arched palate and overretained primary teeth. Her dentition consisted of her four permanent first molars and all of her primary teeth, except the mandibular right central incisors. A panoramic radiograph showed multiple supernumerary teeth and unerupted permanent teeth (Fig 1). A provisional diagnosis of cleido cranial dysostosis was made, and a complete radiographic workup was done. The radiographic findings were consistent with the diagnosis of cleidocranial dysostosis. There was no familial history, and results of routine blood and urine analyses were normal. The decision was made to extract all carious primary teeth. The extractions were done two weeks later (Fig 2). The patient was followed up by the oral surgery department on a monthly basis for seven months. At that time, because of no appreciable eruption of any teeth, the decision was made to admit the patient for surgical removal of the supernumerary
Report of case In March 1972, a 9-year-old girl was brought to the dental clinic for consultation because o f the
THE AUTHORS
MILLER
SAKAMOTO
ZELL
Dr. M iller is in the US A ir Force in San A ntonio, Tex, and Dr. Sakam oto is in private practice. B oth are form er senior resi dents, departm ent of dental and oral surgery, Lincoln Medical & Mental Health Center, Bronx, NY. Dr. Zell, an o rthod ontist, is c linical in s tru c to r in dentistry, and Dr. A rthu r is assistant clinical professor of dentistry, A lbert Einstein College of Medicine, Bronx, NY. Dr. S tratigos is associate professor of
ARTHUR
STRATI GOS
dentistry, A lbert Einstein C ollege of M edicine, and director, departm ent of dental and oral surgery, Lincoln Medical & Mental Health Center. Address requests fo r reprints to Dr. Stratigos, Lincoln Medical & Mental Health Center—A lbert Einstein C ollege of M edicine, 234 E 149th St, Bronx, NY 10451.
M iller— others: CLEIDOCRANIAL DYSOSTOSIS, MULTIDISCIPLINARY APPROACH ■ 297
Fig 1 ■ Initial panoram ic radiograph taken in March 1972.
Fig 2 ■ Panoram ic radiograph taken in A pril 1972, after removal of selected prim ary teeth.
Fig 3 ■ Panoram ic radiograph taken in O ctober 1972, after removal of 19 supernum erary teeth and fo u r prim ary molars.
teeth. On Oct 11, the patient was taken to the operating room and the four primary second mo lars and 19 impacted and unerupted supernumer ary teeth were removed from the maxilla and the mandible (Fig 3). Closure was done with 3-0 chromic sutures. She was discharged three days later, and healing was uncomplicated. After being followed up in the outpatient clinic for a year, there was no evidence o f any eruption of her per 298 ■ JADA, Vol. 96, February 1978
manent dentition. The only visible dentition at this time were the permanent first molars, man dibular central incisors, and maxillary left and mandibular right lateral incisors (Fig 4). Because o f the lack of any eruptive process after a year, on Oct 10,1973, the patient was taken to the operating room and the unerupted perma nent dentition (maxillary canines and first and second premolars, and mandibular lateral incisors
Fig 4 ■ P a tie n ts existing d e ntition in September 1973, before surgicai exposure and ligation of teeth.
> ■ Panoram ic radiographs taken in O ctober 1973, after ligation of unerupted lanent de ntition.
Fig 6 ■ Patient's d e ntition in December 1973, before initiatio n of o rth o d o n tic treatm ent.
and first and second premolars) was uncovered. These teeth were found to be covered by a dense, fibrotic tissue, which was reflected with great dif ficulty. The teeth were further exposed surgically with bone burs; in each quadrant, the first and second premolars and lateral incisors were ligated with no. 26-gauge wires. At this time, it became
evident that forces from the circumdental wires had a tendency to cause avulsion o f these un erupted teeth because o f the conical shape o f the roots o f these teeth. Surgical cement* on gauzet was packed gently around these surgically ex posed teeth. Overlying redundant alveolar tissue was excised, and 3-0 black silk sutures were used to maintain the position of the impregnated gauze (Fig 5). The patient was discharged two days lat er. The packing and sutures were removed during the next three weeks because small segments of the packing became loose. By mid-December, all o f the central and lateral incisors, first and second premolars, and first molars were visualized intraorally (Fig 6). H owever, the panoramic radio graph showed that this apparent eruption was caused only by the removal o f tissue that previ ously has been covering these teeth and that the dentition’s relative position had not changed radiographically. The patient then was referred to the consulting orthodontist for follow-up. In January 1974, banding of individual teeth was begun, with a couple o f teeth being banded weekly because of the timidness o f the patient. By the beginning o f April, orthodontic forces were being applied to the maxillary dentition via a maxillary arch wire and wire springs to upright the teeth. By July, similar orthodontic forces were applied to the mandibular teeth. It was noticed that the teeth began to erupt only when orthodon tic forces were applied. By October, the eruption o f the dentition was apparent clinically (Fig 7). It was evident from the panoramic radiograph taken in October 1975 (Fig 8), three years later from the time o f removal of the supernumerary teeth, that the root formation of the permanent teeth had been satisfactory and the periodontal condition o f all teeth appeared clinically normal; however, radiographically there was some indica tion of periodontal involvement, which actually was the result of the removal o f bone and the supernumerary teeth. As function of the teeth improves, it is expected that the alveolar bone growth will advance, with the ultimate prognosis being that of a satisfactory and periodontically sound dentition. Furthermore, the facial appear ance of the patient has improved greatly at this time.
Conclusion This case illustrates several important points in
M iller— others: CLEIDOCRANIAL DYSOSTOSIS, MULTIDISCIPLINARY APPROACH ■ 299
Fig 8 ■ Panoram ic radiograph taken in O ctober 1975. Fig 7 ■ P atient’s existing m axillary and m andibular d e ntition in O ctober 1974.
the management of a patient with cleidocranial dysostosis. In this pathological entity, any me chanical obstruction appears to completely obstruct the rate of eruption. The fibrous tissue overlying the permanent dentition, in this case, was extremely dense and thick, and it delayed the eruption. The complete uncovering o f the crowns of the permanent teeth was found to be essential to eruption. Even then, eruption was negligible until orthodontic forces were put on the teeth. The conical nature o f these succedaneous teeth, which is a result of their cemental defect, should deter the surgeon from excessive manipu lation o f these erupting teeth because they can be avulsed easily from their bony crypt. The screw or pin appliance13 is least traumatic and therefore indicated for orthodontic traction. The oral surgical approach to such a case re quires close collaboration with the orthodontist to determine the sequence o f extraction, the teeth to be ligated, the preferred technique o f ligation, and the approach for surgical exposure. Because of the protracted nature of the total treatment of these patients, cooperation and understanding between these two disciplines of dentistry are imperative.
Summary A case o f cleidocranial dysostosis has been pre sented. Its oral surgical and orthodontic ap
300 ■ JADA, Vol. 96, February 1978
proaches and rationale for treatment have been discussed. We hope this presentation will contri bute to a more aggressive approach in the total treatment of this disease.
•W ard’s surgical cement, Westward Dental P roducts Co., San Francisco, 94109. fld o fo rm gauze, Acme C otton P roducts Co., Inc., Valley Stream, NY 11582. 1. Marie, P., and Sainton, P. Cited by Rock, W.P. Cleido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 2. Kalliala, E „ and Taskinen, P.J. C leidocranial dysostosis: re po rt of six typical cases and one atypical case. Oral Surg 15:808 July 1962. 3. G orlin, R.J., and Goldman, H.M., eds. Thom a’s oral pathology, ed 6. St. Louis, C. V. Mosby Co., 1970, vol 1, p 534. 4. Shafer, W.G.; Hine, M.K.: and Levy, B.M. Oral pathology. Philadelphia, W. B. Saunders Co., 1974, p 625. 5. W inkler, S., and Jung, E.L. C leidocranial dysostosis: report of a case. Dent Dig 77:24 Jan 1971. 6. Lubow itz, A.H. C leidocranial dysostosis: a case report. Angle O rthod 38:150 Jan 1968. 7. Miles, P.W. Cited by Rock, W.P. C leido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 8. Soule, A.B. Cited by Rock, W.P. C leido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 9. Seldin, H.M., and others. Cited by Rock, W.P. Cleido-cranial dysostosis: a case report. Br Dent J 126:85 Jan 21, 1969. 10. Archer, W.H. Oral surgery, ed4. Philadelphia, W. B. S aunders Co., 1966, p 227. 11. W inther, J.E., and Khan, M.W. C leidocranial dysostosis: re p o rt of fo u r cases. Dent Pract Dent Rec 22:215 Feb 11, 1972. 12. Thoma, K.H. Oral surgery, ed 5. St. Louis, C. V. Mosby Co., 1969, pp 172, 380. 13. Porter, W.J., and Rider, E.A. A m ethod of anchorage fo r orth o d o n tic movement of unerupted teeth. J Oral Surg 32:513 July 1974.
