Cleidocranial dysostosis: report of case
Ralph B. Maw, DDS, Long Beach, Calif
In the treatm ent of oral problems of patients with cleidocranial dysostosis, the m ultidisciplinary ap proach should be considered. The collective abili ties of the oral surgeon, the prosthodontist, and the o rthodontist should be utilized to determ ine the most effective treatm ent.
The history and treatment o f multiple possible anomalies o f cleidocranial dysostosis have been widely published and are almost universally ac cepted; therefore, no review of them is under taken in this paper. The diagnosis is usually made by identification o f the complete or partial lack of calcification of the clavicles with resultant hypermobility o f the shoulders, allowing them to be approximated anteriorly and by radiographs of the skull showing patent fontanelles and wormian bone in unclosed suture lines. An excellent re view of this disease is provided by Kalliala and Taskinen.1
Report of case A 21-year-old, well-developed, well-nourished white man came to the hospital dental clinic for correction of oral problems connected with previ ously diagnosed cleidocranial dysostosis. Ra diographs of the skull and thorax showed a slight amount of wormian bone in the skull, an almost complete absence o f the clavicles, and a few de formed ribs. H e was slight of build (Fig 1) but physically active. H is performance as a hospital corpsman indicated an above average level of intelligence and dexterity. 306 ■ JADA, Vol. 96, February 1978
Dental radiographs showed 46 teeth— 23 in each arch, 18 of which were visible in the mouth (8 maxillary and 10 mandibular) (Fig 2). Only one primary tooth, the mandibular left canine, was present. N o cysts were evident on the radiograph, but many teeth were dilacerated (Fig 3). All teeth were asymptomatic and in good repair. The gingi val tissues were healthy, and 13 o f the erupted teeth were in relatively good positions in the den tal arches. The patient’s facial profile was good, with only a very slightly apparent prognathism. His only complaints were related to the lack of anterior teeth and misalignment o f those present, resulting in an ungainly smile and a reduced ability to incise (Fig 4). He enjoyed the attention gener ated by his ability to touch his shoulders together in front of him (Fig 5). Medical history, review of system s, and results of the remainder of his physi cal examination were within normal limits. N o other member o f his family was known to have a similar condition. After thorough evaluation and numerous con sultations, it was decided to stage the removal of all impacted teeth and those erupted teeth that could not be used or would be unattractive when combined with partial dentures. The first stage,
THE AUTHOR
Dr. Maw, an oral surgeon, is a captain in the US Navy Dental Corps. Address requests fo r re prints to Dr. Maw at 7500 E Car son St, Long Beach, Calif 90822. MAW
the remaining impacted teeth were removed with the patient under general anesthesia, in three stages over the next IV2 years (Fig 6); the final tooth was removed from a lingual approach with the patient under local anesthesia. After complete healing, removable partial dentures were con structed. Fourteen months later, all erupted teeth were stable and asymptomatic, and the patient was pleased with the appearance and function of his partial dentures.
Discussion
Fig 1 ■ Normal, relaxed appearance of patient w ith cleidocranial dysostosis.
with the patient under local anesthesia, included removal of all designated erupted teeth and all impacted teeth in the anterior maxilla. After this, preliminary partial dentures were constructed to provide esthetics and some incisive ability. Thir teen erupted teeth were maintained. All but one of
Because this patient worked in the same hospital as we did, the multiple-operation approach was used to minimize his discomfort at any one time and resulted in less lost working time for him. Total surgical time was about six hours because of the large amount of bone surgery; dilaceration of teeth; and position of teeth near the inferior bor der, approximating the maxillary sinuses, and wedged between roots o f teeth that we were trying to salvage. In addition, extreme care was taken to preserve as much bone as possible. Although no cysts were evident by radiograph, each impacted tooth had at least a partial sac around the crown. The fear of creating iatrogenic fracture, al though a possibility, was far outweighed by the fear o f a traumatic fracture in the future with a multitude o f impacted teeth in the line(s) o f frac ture and the increased possibility of nonunion(s), infections, and so forth. Any mandible that would be fractured in the course of removing these im pacted teeth surely would require very little trauma to fracture it if teeth were left undisturbed.
Maw: CLEIDOCRANIAL DYSOSTOSIS ■ 307
Fig 3 ■ Extreme dilacerations found in teeth of patient.
Once the teeth are removed and healing takes place, the resultant bone is far stronger than be fore. The desire to preserve a bony denture base is, of course, a primary consideration; however, those teeth immediately under the denture have been shown to result in erosion of the overlying bone.2 The deep impactions are removed from the buccal aspect, leaving the alveolar bone relatively untouched. Comparison of Figures 2 and 6 shows that despite the removal of a large number of impacted teeth, there was no significant alveolar bone loss. Obviously, one does not resort to surgery on the very young patient; however, as the patient reaches maturity it is usually apparent which teeth will not be of value. Bony repair is unimpeded in these patients, and the presence of partial dentures provides the function necessary to help preserve alveolar bone. Surgical correction of apparent prognathism can be accomplished through maxillary or man dibular osteotom ies, or both. The problem is
Fig 5 ■ Absence of clavicles allow s patient to alm ost touch shoulders in m idline.
created by an underdeveloped maxilla; with the techniques currently available, maxillary surgery would probably be the logical choice when feasi ble. The appearance and function o f the patient’s dentition was satisfactory; therefore, orthog nathic surgery was not considered necessary. Prosthetics played an important part in the treat ment o f our patient. Several different appliances were required during the course of his care and greatly enhanced the overall success of the treat ment.
Conclusions
Fig 4 ■ Spaces and teeth before treatment. 308 ■ JADA, Vol. 96, February 1978
Oral problems in cleidocranial dysostosis, like so many other entities, are best approached by a team rather than by an individual. The oral sur geon, the prosthodontist, and the orthodontist, as a minimum, should all be involved in treatment planning.
Fig 6 ■ Panoram ic radiograph taken a week after surgery was com pleted except fo r rem aining lingu ally impacted prem olar. Notice presence of ample alveolar bone despite considerable surgery.
The first step should be to relieve pain, if any; then a complete medical history, radiographs, models, and an evaluation o f the patient’s mental and physical conditions should be accomplished to establish his ability to cooperate and to tolerate treatment. These data will allow the team to determine which teeth are to be salvaged; the requirements for prosthetic appliances; the need, if any, for orthodontics, orthognathic surgery, speech ther apy, and so forth; and the staging of treatment. With a complete tentative treatment plan, the patient or his parents, or both, are counseled and their own desires and expectations consid ered within the bounds of feasible treatment. A final plan is formulated and accepted before treatment is initiated. This is a logical course to follow in any condi tion to be treated. The team approach is especial ly important in cleidocranial dysostosis because
o f its complexities and should be used whenever possible to ensure the best possible treatment of these patients.
S u m m a ry A case of cleidocranial dysostosis was presented. Each case should be approached individually using sound surgical and clinical procedures. The op in ions o r assertions contained herein are the private ones of the author and are n ot to be construed as o fficia l or as reflecting the views of the Navy Department or the naval service at large.
1. Kalliala, E., and Taskinen, P.J. C leidocranial dysostosis. Oral Surg 15:808 June 1962. 2. Douglas, B.C., and Greene, H.J. C leidocranial dysostosis: re po rt of a case. J Oral Surg 27:41 Jan 1969.
Maw: CLEIDOCRANIAL DYSOSTOSIS ■ 309
Ralph B. Maw, DDS, Long Beach, Calif
In the treatm ent of oral problems of patients with cleidocranial dysostosis, the m ultidisciplinary ap proach should be considered. The collective abili ties of the oral surgeon, the prosthodontist, and the o rthodontist should be utilized to determ ine the most effective treatm ent.
The history and treatment o f multiple possible anomalies o f cleidocranial dysostosis have been widely published and are almost universally ac cepted; therefore, no review of them is under taken in this paper. The diagnosis is usually made by identification o f the complete or partial lack of calcification of the clavicles with resultant hypermobility o f the shoulders, allowing them to be approximated anteriorly and by radiographs of the skull showing patent fontanelles and wormian bone in unclosed suture lines. An excellent re view of this disease is provided by Kalliala and Taskinen.1
Report of case A 21-year-old, well-developed, well-nourished white man came to the hospital dental clinic for correction of oral problems connected with previ ously diagnosed cleidocranial dysostosis. Ra diographs of the skull and thorax showed a slight amount of wormian bone in the skull, an almost complete absence o f the clavicles, and a few de formed ribs. H e was slight of build (Fig 1) but physically active. H is performance as a hospital corpsman indicated an above average level of intelligence and dexterity. 306 ■ JADA, Vol. 96, February 1978
Dental radiographs showed 46 teeth— 23 in each arch, 18 of which were visible in the mouth (8 maxillary and 10 mandibular) (Fig 2). Only one primary tooth, the mandibular left canine, was present. N o cysts were evident on the radiograph, but many teeth were dilacerated (Fig 3). All teeth were asymptomatic and in good repair. The gingi val tissues were healthy, and 13 o f the erupted teeth were in relatively good positions in the den tal arches. The patient’s facial profile was good, with only a very slightly apparent prognathism. His only complaints were related to the lack of anterior teeth and misalignment o f those present, resulting in an ungainly smile and a reduced ability to incise (Fig 4). He enjoyed the attention gener ated by his ability to touch his shoulders together in front of him (Fig 5). Medical history, review of system s, and results of the remainder of his physi cal examination were within normal limits. N o other member o f his family was known to have a similar condition. After thorough evaluation and numerous con sultations, it was decided to stage the removal of all impacted teeth and those erupted teeth that could not be used or would be unattractive when combined with partial dentures. The first stage,
THE AUTHOR
Dr. Maw, an oral surgeon, is a captain in the US Navy Dental Corps. Address requests fo r re prints to Dr. Maw at 7500 E Car son St, Long Beach, Calif 90822. MAW
the remaining impacted teeth were removed with the patient under general anesthesia, in three stages over the next IV2 years (Fig 6); the final tooth was removed from a lingual approach with the patient under local anesthesia. After complete healing, removable partial dentures were con structed. Fourteen months later, all erupted teeth were stable and asymptomatic, and the patient was pleased with the appearance and function of his partial dentures.
Discussion
Fig 1 ■ Normal, relaxed appearance of patient w ith cleidocranial dysostosis.
with the patient under local anesthesia, included removal of all designated erupted teeth and all impacted teeth in the anterior maxilla. After this, preliminary partial dentures were constructed to provide esthetics and some incisive ability. Thir teen erupted teeth were maintained. All but one of
Because this patient worked in the same hospital as we did, the multiple-operation approach was used to minimize his discomfort at any one time and resulted in less lost working time for him. Total surgical time was about six hours because of the large amount of bone surgery; dilaceration of teeth; and position of teeth near the inferior bor der, approximating the maxillary sinuses, and wedged between roots o f teeth that we were trying to salvage. In addition, extreme care was taken to preserve as much bone as possible. Although no cysts were evident by radiograph, each impacted tooth had at least a partial sac around the crown. The fear of creating iatrogenic fracture, al though a possibility, was far outweighed by the fear o f a traumatic fracture in the future with a multitude o f impacted teeth in the line(s) o f frac ture and the increased possibility of nonunion(s), infections, and so forth. Any mandible that would be fractured in the course of removing these im pacted teeth surely would require very little trauma to fracture it if teeth were left undisturbed.
Maw: CLEIDOCRANIAL DYSOSTOSIS ■ 307
Fig 3 ■ Extreme dilacerations found in teeth of patient.
Once the teeth are removed and healing takes place, the resultant bone is far stronger than be fore. The desire to preserve a bony denture base is, of course, a primary consideration; however, those teeth immediately under the denture have been shown to result in erosion of the overlying bone.2 The deep impactions are removed from the buccal aspect, leaving the alveolar bone relatively untouched. Comparison of Figures 2 and 6 shows that despite the removal of a large number of impacted teeth, there was no significant alveolar bone loss. Obviously, one does not resort to surgery on the very young patient; however, as the patient reaches maturity it is usually apparent which teeth will not be of value. Bony repair is unimpeded in these patients, and the presence of partial dentures provides the function necessary to help preserve alveolar bone. Surgical correction of apparent prognathism can be accomplished through maxillary or man dibular osteotom ies, or both. The problem is
Fig 5 ■ Absence of clavicles allow s patient to alm ost touch shoulders in m idline.
created by an underdeveloped maxilla; with the techniques currently available, maxillary surgery would probably be the logical choice when feasi ble. The appearance and function o f the patient’s dentition was satisfactory; therefore, orthog nathic surgery was not considered necessary. Prosthetics played an important part in the treat ment o f our patient. Several different appliances were required during the course of his care and greatly enhanced the overall success of the treat ment.
Conclusions
Fig 4 ■ Spaces and teeth before treatment. 308 ■ JADA, Vol. 96, February 1978
Oral problems in cleidocranial dysostosis, like so many other entities, are best approached by a team rather than by an individual. The oral sur geon, the prosthodontist, and the orthodontist, as a minimum, should all be involved in treatment planning.
Fig 6 ■ Panoram ic radiograph taken a week after surgery was com pleted except fo r rem aining lingu ally impacted prem olar. Notice presence of ample alveolar bone despite considerable surgery.
The first step should be to relieve pain, if any; then a complete medical history, radiographs, models, and an evaluation o f the patient’s mental and physical conditions should be accomplished to establish his ability to cooperate and to tolerate treatment. These data will allow the team to determine which teeth are to be salvaged; the requirements for prosthetic appliances; the need, if any, for orthodontics, orthognathic surgery, speech ther apy, and so forth; and the staging of treatment. With a complete tentative treatment plan, the patient or his parents, or both, are counseled and their own desires and expectations consid ered within the bounds of feasible treatment. A final plan is formulated and accepted before treatment is initiated. This is a logical course to follow in any condi tion to be treated. The team approach is especial ly important in cleidocranial dysostosis because
o f its complexities and should be used whenever possible to ensure the best possible treatment of these patients.
S u m m a ry A case of cleidocranial dysostosis was presented. Each case should be approached individually using sound surgical and clinical procedures. The op in ions o r assertions contained herein are the private ones of the author and are n ot to be construed as o fficia l or as reflecting the views of the Navy Department or the naval service at large.
1. Kalliala, E., and Taskinen, P.J. C leidocranial dysostosis. Oral Surg 15:808 June 1962. 2. Douglas, B.C., and Greene, H.J. C leidocranial dysostosis: re po rt of a case. J Oral Surg 27:41 Jan 1969.
Maw: CLEIDOCRANIAL DYSOSTOSIS ■ 309
