Cleidocranial Dysplasia - the Jerusalem Approach: part 4
Published: February 2016
Bulletin #52 February 2016
Cleidocranial dysplasia – the Jerusalem Approach: part 4
Parts 1, 2 and 3 of this series on the treatment of Cleidocranial dysplasia (CCD), according to the Jerusalem Approach, were presented in the September, October and November 2013 bulletins on this website (bulletins #25, 26 and 27 respectively). They describe and illustrate the rationale for the orthodontic and surgical modality of treatment and its effective execution in 3 of the 4 distinct stages that are a unique feature of the Jerusalem approach.
The first stage relates to orthopedic protraction of the under-developed maxilla,1-3 which may be usefully carried out in those CCD patients who show a tendency to a class 3 skeletal development, provided they are seen early enough. The second and third stages are the principal elements in this approach and they are concerned with extraction of the over-retained deciduous teeth, surgical elimination of the supernumerary teeth, attachment bonding to the impacted teeth of the normal series and their assisted eruption and alignment. 1-3
The present bulletin represents the last of the series describing the comprehensive treatment of CCD according to the Jerusalem Approach. It addresses the inter-jaw relations that are the result of the late growth spurt/persistent maxillary under-development or merely exacerbation of the earlier, frequently-occurring and initially mild, class 3 inter-arch relations. Most CCD patients are not seen early enough, for a minority the skeletal discrepancy is too severe, while others may not be adequately compliant with the cumbersome mechanics involved to benefit from pre-orthodontic skeletal protraction. It is therefore inevitable that, for a significant number of late adolescents/young adults who have completed the eruption and alignment of all their permanent teeth and who display skeletal class 3 relationships, orthognathic surgery will be indicated.
We have reported that there is a very characteristic 3-4 years delay in dental development in CCD patients in relation to their chronologic age1-3 and there may also be a lesser degree of delay in the onset of puberty and circumpubertal growth. For this reason, the timing of the procedure aimed at surgically harmonizing dysgnathic jaw relations should also be delayed. The considerations involved in this last phase in the treatment of CCD by the Jerusalem approach will be illustrated with clinical views of the same patient described in bulletins #26 and #27.
The incisor-erupting stage began in July 2006. In a departure from protocol in this particular patient, the eruption and alignment of the four permanent canines was included in this first stage, together with the incisors. As the result, this phase of the treatment was particularly long and was only completed in 34 months, in April 2009. At its conclusion, the 6 maxillary and mandibular anterior teeth had been brought to their place and into good alignment, although in a marked crossbite relation due to the worsening skeletal 3 relation. The second tooth-erupting phase involved the premolar teeth and was initiated in August 2009. Surgery involved the removal of 3 remaining supernumerary teeth in the premolar areas and the exposure and bonding of eyelets to each of these teeth.
Fig. 1. A periapical view of the right premolar area showing the “forgotten” supernumerary tooth, at the end of the treatment.
Although it had been identified prior to the surgery, one supernumerary tooth in the mandibular right premolar area was forgotten and only re-discovered on the post-surgical panoramic film. Its presence did not prove to be an obstacle to the successful eruption and alignment of the adjacent teeth and it was left in situ (Fig. 1).
Fig. 2. Intra-oral views of the occlusion immediately prior to the orthognathic surgery.
The remaining orthodontic procedures in this phase were completed in December 2011 with the angulation of the incisor teeth having been decompensated in line with the planned post-surgical outcome, thereby producing a very large negative overjet (Fig. 2).
Shortly after completion, the patient was drafted into the army, where he spent the next 3 years completing his national service. By the end of the first year, it was hoped that the final orthosurgical stage of his treatment would be undertaken during his army service and new records were taken in February 2013.
Fig. 3. Panoramic view of the dentition prior to the extraction of the third and fourth maxillary molars.
His orthodontic appliances were left in place and the maxillary third and fourth (!) permanent molars (Fig. 3) were extracted in October 2014, in readiness. Circumstances did not permit and the orthognathic surgery was delayed until his release, in February 2015.
Orthognathic surgery
Fig. 4. Intra-oral views of the dentition a few days before the surgery, showing the inserted T-pins (Power Pins, TP Orthodontics, Inc.) to be used as hooks for the intermaxillary fixation.
Surprisingly minimal damage to the orthodontic appliances was found at the first visit that the patient made following his discharge from the army and arrangements were made for the jaw surgery to correct his extreme jaw discrepancy, to bring his teeth into proper occlusion and to improve his overall facial appearance. In the week prior to the surgery, rectangular cross-section 0.028”x0.0215” stainless steel archwires were placed and Power Pins were slotted into the vertical slots of the brackets of all the teeth, to provide multiple locations for intermaxillary elastic fixation in the immediate post-operative period (Fig. 4).
Fig. 5. The lateral cephalogram showing the inter-relations of the hard tissue structures.
The skeletal class 3 relation was due both to underdevelopment of the maxilla and an over-sized mandible (Fig. 5). In conference with the surgical team, led by Prof. Nardi Caspi, there was clearly the need for a 2-jaw procedure, not the least in view of the very large discrepancy between the jaws. In the final analysis, a LeFort 1 osteotomy was performed in the maxilla, with a 6mm advancement and four 1.5mm AO plates (DePuy Synthes) were used for fixation. A bilateral vertical ramus osteotomy was employed to set back the mandible by 4 mm, followed by intermaxillary fixation. The chin was treated with a 6mm advancement genioplasty and fixated with two 1.5 mm AO plates. The patient was discharged from hospital 7 days after surgery, having been intubated for 2 days in ICU following respiratory complications during the recovery period.
Fig. 6a. Intra-oral views of the final alignment and occlusion of the teeth.
Fig. 6b. Occlusal views of the two jaws showing the 3-3 bonded twistflex wire retention splints.
He was seen again several times between June and December for some minor adjustments in my Orthodontic office, before being debonded on 14 December 2015 (Fig. 6a). Fixed 3-3 twistflex bonded retainers were placed in both jaws on the same day and no other form of retention was used (Fig. 6b).
Post-treatment appraisal
Fig. 7. In the panoramic view, the method of fixation (1.5 mm AO plates) may be seen.
Fig. 8. The post-surgical lateral cephalogram illustrates the normalization of the skeletal relations and the chin enhancement.
The patient’s overall facial appearance and profile have
undergone marked improvement, both from the point of view of the soft tissues (Fig.
7) and the bony skeleton (Fig. 8). The
face is harmonious and symmetrical, although for reasons of patient
confidentiality, these are not shown here. The upper lip is well supported by
the dentition and there is a good degree of lip competence. 
Fig. 9. An excellent relationship between the maxillary anterior teeth and the lips at rest has been achieved.
The “smile line” of
the incisal edges of the upper teeth in relation to the curvature of the lower
lip is excellent (Fig. 9). Although the pre-surgical records were taken in 2013
(when the patient was 21 years of age) and the immediate post-surgical records
only in 2015 (when he was 23), a comparison of the soft tissue profile is
valid, since growth changes had come to a standstill some time previously. 
Fig. 10. Much of the considerable improvement in the patient’s appearance may be seen in this juxtaposition of the lateral cephalograms of the patient before and after surgery and “photoshopped” to bring out the texture of the soft tissues of the face, nose and chin.
Accordingly, the major clinical benefits that were achieved in the orthosurgical phase of the treatment (Fig. 10) may be summarized as follows:-
1. Increased lower third of the face
2. Improved chin profile
3. Improved nasal profile
4. Relatively reduced nasal length due to the newly supported upper lip and a consequent forward displacement of soft tissue A-point.
5. Intra-orally, the teeth are well aligned and the occlusal relations feature good class 1 dental interdigitation with normal overjet and overbite.
Fig. 11. A periapical view of the splinted maxillary incisors at the completion of treatment, showing stunted roots and bone loss, not present elsewhere in the mouth.
.The maxillary central incisors have long clinical crowns due to a degree of gingival recession (Fig. 6a) and, while the periodontal tissues are healthy, the panoramic and periapical radiographs show bone loss around the maxillary incisors (Fig. 11), which may be causally related to the fact that supernumerary teeth were located on the lingual side of the unerupted incisors at the outset. A large bony defect had resulted following their surgical removal. A relative lack of fill-in of new bone during the healing stage, together with the forced eruption of the labially and superiorly displaced incisors did not stimulate a sufficiently positive response on the part of the alveolar bone, to follow the dental development. The left maxillary central and lateral incisors also have short roots, but before one label this as root resorption, it is entirely possible that their development was stunted because of their initial extreme height displacement into close relation with the floor of the nasal cavity.
Fig. 12a, b. A comparison of the panoramic views pre-treatment in 2005 and post-treatment in 2015, respectively.
In the mandible, a conscious decision was made not to disturb the unerupted supernumerary premolar and the unerupted third molars in the mandible. At this stage, these teeth cannot adversely affect the orthodontic result and, therefore, the determination if and when to extract them is no longer an orthodontic decision. The recommendation was that they should be left in place, monitored clinically and radiographically on a periodic basis, maintaining the option for their extraction as and when indicated. Panoramic views of the dentition prior to treatment and immediately post-treatment can be seen in Fig. 12a and 12b, respectively.
Fig. 13. Superimposed tracings of the cephalograms (a) May
2005 in black and February 2013 in red, (b) February 2013 in black and January
2016 in red, (c) May 2005 in black and January 2016 in red . Superimpositions by Orthodata, Jerusalem.
Superimposed tracings of the cephalometric superimpositions prior to commencement of treatment (May 2005), at completion of the eruption phase of all the permanent teeth with the exception of third molars (February 2013) and at the completion of the orthognathic surgery phase and removal of all appliances (January 2016) can be seen in Fig. 13a-c. The parameters measured and their values can be read in table 1 below.
Table 1: Comparison of cephalometric values between pre-treatment (May 2005), post-eruption (Feb. 2013) and completion of the comprehensive treatment (Jan. 2016). With thanks to Orthodata, Jerusalem.
References
1. Becker A, Lustmann J, Shteyer A. Cleidocranial dysplasia: part 1 - General principles of the Orthodontic and Surgical Treatment Modality. American Journal of Orthodontics and Dentofacial Orthopedics 111:28-33, 1997.
2. Becker A, Shteyer A, Bimstein E, Lustmann J. Cleidocranial dysplasia: part 2 - a Treatment Protocol for the Orthodontic and Surgical Modality. American Journal of Orthodontics and Dentofacial Orthopedics 111:173-183,1997.
3. Becker A. Orthodontic Treatment of Impacted Teeth, 3rd edition, 2012. Oxford: Wiley-Blackwell Publishers. 2012. Chapter 14.
