Cleidocranial dysplasia is a rare genetic condition caused by mutations in the RUNX2 gene. It is characterized by hypoplastic or aplastic clavicles, open or delayed closure of cranial sutures, dental abnormalities like supernumerary teeth and eruption defects, and short stature. Treatment involves a combination of surgical removal of extra teeth and exposure of impacted teeth followed by orthodontic treatment to actively erupt the impacted permanent teeth through various approaches depending on the severity and extent of impactions.

1. Cleidocranial dysplasia
Becker 1997
https://youtu.be/3kvCknYyr-c
1. CDD is an autosomal dominant condition
2. 1 per million individuals worldwide
3. Cleidocranial dysplasia is caused by a mutation in the runt-related transcription factor 2 gene
(RUNX2), and factor alpha 1 (CFBA1) both are responsible for the initial differentiation of
osteoblasts to form skeletal structures [11,12].
Features
A. General features:
 Hypoplasia or aplasia of the clavicles,
 Delayed closure of the cranial sutures, the skull sutures may take years to close or, in some
cases, may never close thus patients with CCD have a broad forehead thus helmets are
suggested when engaging in physical activities and sports.
 Defective bone formation
 Short stature
 Short and tapered fingers and broad thumbs
 Narrow chest; and scoliosis.
 Adult patients may develop osteoporosis.
B. Dental features
 Supernumerary teeth,
 Defective cementum formation
 Abnormal permanent tooth eruption [9,10] caused by impedance of the migration of the
permanent teeth toward the oral cavity by defects in the osteoclastic and resorptive activity of
the alveolar bone.
 Reduced height of the lower third of the face and a skeletal Class III tendency due to
underdevelopment of the maxilla and to an upward and forward mandibular rotation.
 The vertical development of alveolar bone is markedly reduced, with a shallow buccal and
lingual sulcus.

2. Management
1. Historically, there were two main approaches
a. Prosthetic replacement by means of dentures, with or without extraction of the impacted
teeth. In some instances, the impacted teeth are exposed and used to support
overdentures.
b. A surgical approach consisting of the removal of supernumerary teeth, followed by
surgical repositioning or transplantation of the permanent teeth.
2. Currently, combination of surgical and orthodontic treatment with the aim of actively erupting
and aligning the impacted permanent teeth. Three distinct and contrasting approaches have
been suggested over the past few years and each of these has its relative merits
a. The Toronto-Melbourne approach. This method consisted of a series of surgical
procedures: . (Smylski 1974;Hall, 1978)
 1st surgery at age of 5-6 years: Removal of the anterior deciduous teeth with its timing
dependent on appropriate root development of the permanent teeth. At the intervention,
supernumerary teeth and bone overlying the crypts of the corresponding, unerupted, permanent
teeth are removed.
 2nd surgery: Only much later, after the spontaneously erupted first molars have been banded, a
more localized surgical re-exposure of the permanent incisors is performed and the exposed
area packed. Attachments are placed on the permanent incisors after healing has occurred.
 43rd surgery: Later still, between 9 to 12 years, the premolars are surgically exposed, the
supernumerary teeth removed, and the exposed area packed. After complete healing and
removal of the packs, brackets are placed on the premolars and canines.
Limitations
 In this method, the patient is under treatment for many years, starting from a very early age
and requiring several recommended and fairly extensive surgical interventions
 The deciduous anterior teeth are removed at an early stage. This means that the patient is
anteriorly edentulous for some considerable time.
 The placement of attachments to the deeply sited permanent teeth is not performed at the time
of surgery, but some time later, after full healing (by secondary intention) has occurred and the
surgical packs have been removed. Thus, at each surgical stage, valuable time is lost between
the exposure and the force application needed to encourage the eruption of the teeth.

3. b. The Belfast-Hamburg approach (Behlfelt, 1987)
 One of the principal aims of this approach is to limit the inescapable need for extensive surgery
to a single episode at age of 12-14 years
 all deciduous and supernumerary teeth are removed and all unerupted teeth are exposed
simultaneously
 a surgical pack is placed over them to prevent bony healing and soft-tissue closure over the
teeth. Healing is by secondary intention.
 Over an extended period, these surgical packs are changed frequently, until such time as
orthodontic brackets may be bonded to each of the unerupted teeth, under conditions that are
less likely to lead to bond contamination than those present during the actual surgical exposure
procedure.
 Often, spontaneous eruptive movements occur with some of the teeth, although it is never
sufficient or reliable enough to eliminate the need for extrusive mechanics.
 Orthodontic appliances must be placed on the few teeth that are fully erupted and elastic thread
is tied between the bonded brackets on the unerupted teeth and the arch wires to encourage
eruption.
 The same wide exposure with radical bone removal has been shown to create a compromised
bony support for the tooth after eruption has been achieved and, thereby, a reduced periodontal
integrity.
 The early surgical removal of a follicle that surrounds an immature and deeply impacted tooth
allows the tissues to come in direct contact with the enamel. Replacement resorption may occur
in the crowns of impacted teeth, in time, after the tooth follicle atrophies or otherwise
disappears
Limitations
 The immediate advantage of this policy is very clear and encouraging, although its drawbacks
are of considerable consequence and not so obvious. By delaying treatment until this late age,
the teeth of the normal series will have been held deep down in basal bone by the
supernumerary teeth, particularly in the lateral incisor/canine/premolar area, for an extended
period of time. Their roots will have reached an advanced stage of development in these
cramped circumstances, which is likely to exaggerate the tendency for a stunted, tortuous and
distorted root morphology. Removal of the unwanted extra teeth at this late stage will relieve
the impaction of the permanent teeth of the normal series, but it will do so at a time when they

4. exhibit even less potential for spontaneous eruption, particularly in the incisor region, since the
root apices will already have been completed.
 During growth in a normal child and with the eruption of permanent teeth, the vertical
development of the alveolar processes that occurs makes a significant contribution to the height
of the lower face. It also leads to the establishment of deep vestibular and lingual sulci, with a
clear differentiation of wide zones of oral mucosa and attached gingiva. In the untreated
cleidocranial dysplasia patient, vertical growth of the alveolar bone appears to be markedly
diminished. This brings about the typically reduced height of the lower third of the face that is
so frequently a feature of the condition.
 This necessitates the removal of considerable quantities of bone [14] and, as recommended by
several authors, the placement of a surgical pack over and around the crowns and necks of the
teeth to prevent bony healing-over and to encourage spontaneous eruption.
c. The Bronx approach
 It developed recently by Berg in 2011 is similar to the Belfast– Hamburg approach but involve
additionally another Phase 2 where orthognathic surgery, bone augmentation and dental
implants are placed under at the same time.
d. The Jerusalem approach
 Intervention 1: dental age 7-8 years which is equal to 10-12 years of chronological age
A. extract the anterior deciduous teeth,
B. extract all supernumerary teeth in the anterior and, as far as reasonably possible, posterior areas
are extracted.
C. expose permanent incisor teeth (root development should be two-thirds their expected length),
D. Bond attachments immediately. It is recommended to bond a small metal attachment to an
impacted tooth immediately after the surgeon has exposed the tooth and prior to flap closure,
Despite the apparent difficulty of avoiding contamination with blood and saliva, successful
bonding is nearly always possible. Ligation is made with a fine wire that extends from the
attachment in the direction of the dental arch or vertically through the fully replaced surgical
flap.
E. Close the surgical flaps fully without the need for packing. The use of surgical packs need

5.  The maintenance of patency of the exposure sites.
 Their presence causes pain and nuisance and it compromises oral hygiene and normal function.
 There is a bad taste in the mouth, with accompanying halitosis.
 The regeneration of alveolar bone will be slow because of the surgical method that relies on
healing by secondary intention. This will further delay eruption and the more deeply sited teeth
may still cover over with surrounding soft tissue.
F. The immature posterior permanent teeth are not expose.
 Intervention 2: dental age 10-11 years which is equal to +13 years of chronological age
A. extract remaining deciduous teeth,
B. expose unerupted premolars and canines,
C. bond attachments immediately,
D. close the surgical flaps fully.
Orthodontic Treatment Strategy
A. Space for the unerupted teeth is provided by:
1. anteroposterior expansion of the dental arches
2. in the vertical plane, by the extraction of deciduous and supernumerary teeth.
3. The necessity for extraction should only be determined after complete eruption has been
achieved and, from our experience, this is avoidable.
B. The appliance used for forced eruption should:
 Have a sufficient number of erupted anchor teeth. Typically, the permanent molars erupt quite
normally and, in many cases, one or two incisors may also be present in each jaw.
 A rigid appliance frame to withstand the distortion from oral function,
 The application of light forces with a good range of action to individual and groups of
unerupted teeth.
C. Orthodontic appliances:
I. Modified Becker lingual arch: In cases with several impacted permanent teeth in the alveolar
bone area, dental implants are indicated instead of screws. The implants are combined with
orthodontic appliances such as lingual arches to serve as indirect anchorage units. If only

6. several teeth are unerupted in one-quarter of dental arches and the other teeth are well aligned,
screws can also be preferred
II. Becker lingual arch
 Preformed bands are adapted to the first molars in both jaws.
 After a compound impression is made, these bands are replaced in the impression and a model
is poured.
 A lingual arch (0.036-inch stainless steel wire) is fashioned well clear of the incisor region in
both jaws and soldered to maxillary and mandibular bands on the model.
 A round 0.036-inch buccal tube is then soldered to each band, parallel to the occlusal plane and
to the buccal surfaces of the deciduous teeth.
 The accurate orientation of the buccal tubes is critical to the efficient working of the appliance.
Thus, if a first permanent molar is tipped lingually or buccally out of the line of the arch, ideal
buccal tube alignment will be impossible. In this situation, a simple removable plate should
first be used to tip the molar into a more suitable position, thereby facilitating the construction
of the basic appliance.
 For each of the two jaws, two arch wires are prepared on the plaster model. The first of these,
the "incisor erupting" archwire, is made of heavy 0.036-inch stainless steel round wire, which
slides into the buccal tubes on the molars and is "stopped" at a bayonet bend on each side
 This self-supported arch wire extends mesially on each side, buccal to the line of the deciduous
teeth, and 2 to 3 mm gingival to the occlusal plane. In the incisor area, it is held 3 to 4 mm
labial to the incisors, thereby slightly displacing the lips.
 An S-shaped hook is soldered in the canine area of each side on both the maxillary and
mandibular arch wire, with the gingivally directed arm of the S pointing distally and the
occlusally directed arm mesially. In the midline area, a small wire frame is solder mounted on
the arch wire and points vertically toward the depth of the sulcus in each jaw
 The arch wire is secured in its place by stretching an elastic chain module from the distal of
the molar tube to the mesially pointing arm of the S-shaped hook. A light, large-diameter elastic
will later be extended from one distally facing hook to another in the upper arch wire and across
to the same two hooks on the lower, to form an intermaxillary "box" elastic. The elastic is held
away by the soldered wire frame to prevent impingement of the tissues in the midline area.

7. 

8. 
The second arch wire, the "incisor-aligning" arch wire, consists of two tubes of 0.020-inch
internal diameter and external diameter 0.036 inch welded to each of the molar bands,. These
are cut to a length that brings their mesial extremity just distal to the ideal site of the canine. A
single length of 0.0155-inch or 0.0175-inch multistranded wire is then drawn into these tubes
and friction fitted into the larger 0.020-inch gauge by placing small bends in that portion of the
wire that will remain in the tubes. This provides a flexible middle section to the otherwise
rigidsided composite arch wire and represents a modification of Johnson's twin wire arch.
 The incisors will erupt well but with a lingual inclination; and, at that time, their initial eyelet
attachments are removed and the operator's standard or prescription brackets of choice are
placed at the ideal height and midlabial position. The composite Johnsontype "incisor-aligning"
arch wire is substituted and its flexible multistrand wire middle portion is ligated to the newly
placed brackets, where it will rapidly and efficiently align, rotate, and level these teeth
 Meanwhile, the buccal tubes provide robust resistance to wire distortion and exert a strong
influence in determining the level of extrusion the incisors will reach. A single, intermaxillary,
light "box" elastic is placed from lateral incisor bracket to lateral incisor bracket on each side
of each jaw. This is aimed at reinforcing the anchorage and encouraging concomitant vertical
development of the alveolar ridges associated with the erupting teeth.

9.  Once the anterior teeth have been aligned, the middle twistflex portion of the composite arch
wire is replaced by a 0.016-inch and subsequently by a more substantial 0.018-inch or 0.020-
inch wire and is secured in the same two side piece tubes in the same way as with the first arch
wires.
 Same procedure repated when premoalrs exposed

10. D. Retention
 Vertical relapse of the extruded teeth does not occur after treatment
 the use of fixed lingual arches provides good control of buccal expansion. The correction of
severely rotated teeth and the labiolingual positioning of the anterior teeth are the most
challenging aspects of therapy.
 To determine labiolingual incisor position in patients with cleidocranial dysplasia , it is better
to place these teeth well forward, with the incisal edges below the level of the upper lip,
 The use of bonded multistrand wire retainers 32-34 is recommended from canine to canine in
both jaws to hold this position and to prevent rotational relapse.