Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia

Abstract

Cleidocranial dysplasia (CCD) is a rare congenital condition characterized by patients presenting with craniofacial anomalies, multiple supernumerary and impacted teeth, retained primary teeth, and delayed eruption, necessitating multi-stage and innovative treatment mechanics to facilitate tooth eruption. This case report highlights the orthodontic management of a 21-year-old female with CCD using a sequential, interdisciplinary approach including the extraction of retained primary and supernumerary teeth, and exposure, bonding, and forced eruption of impacted teeth. The latter was achieved with a custom-fabricated lower appliance and a modified transpalatal arch designed to reinforce anchorage and facilitate simultaneous traction of multiple impacted teeth via a closed surgical technique, achieving their successful retrieval and good orthodontic outcomes.

Introduction

Cleidocranial dysplasia (CCD), also named as cleidocranial dysostosis and Marie-Sainton syndrome, is a condition that mainly affects the intramembranous bones and is linked to several dental anomalies.^1–3 CCD affects 1 in 100 000 live births. The inheritance pattern is autosomal dominant with equal prevalence in males and females. ⁴ CCD results from a mutation in the Cbfa1 (also known as RUNX2) gene, leading to haploinsufficiency of this osteoblast-specific gene, and a lack of adequate functional protein to properly guide the differentiation of bone cells and the ossification process. This results in delayed and defective bone formation. The main clinical features of CCD that can be recognized during early childhood include a short stature, delayed closure of fontanels, prominent forehead, and abnormal dental development (Table 1).

Midface hypoplasia and a markedly prognathic mandible are the 2 craniofacial anomalies frequently associated with this condition. This occurs due to the dysplastic and reduced growth of the cranial base.^5–9 The mandible is typically less affected by the ossification disorder and develops relatively normally, such that the observed “mandibular prognathism” is primarily relative to the underdeveloped maxilla. Some of the characteristic facial features include hypoplastic maxillary, lacrimal, nasal, and zygomatic bones, and underdeveloped maxillary and paranasal sinuses.^10–12

Table 1.

Summary of Clinical Findings in Cleidocranial Dysplasia.

Skeletal features	Craniofacial features	Dentoalveolar features
Open fontanels or delayed closure of fontanels	Brachycephalic skull	Retained deciduous teeth
Wormian bones	Prominent frontal, parietal, and occipital bones	Delayed eruption of permanent teeth; delay in root development; short, thin roots
Complete or partial absence of clavicles, resulting in an excessive shoulder mobility	Skeletal Class III tendency due to an underdeveloped maxilla	Multiple impacted supernumerary teeth
Small and bell-shaped thoracic cage; pes planus (flat feet); genu valgum (knock-knee deformity); and scoliosis	Hypoplasia of the maxillary, lacrimal, nasal, and zygomatic bones	Absence or lack of cellular cementum on the roots of permanent teeth
Short stature; short bones of hands and feet, especially distal and middle phalanges of second and fifth fingers	Discontinuous zygomatic arch	Markedly reduced vertical development of the alveolar bone with a shallow buccal and lingual sulcus
Premature closure of epiphyseal growth plates leading to shortened bones	Underdeveloped and narrow paranasal sinuses	High narrow arched palate; infrequently cleft palate

The dental manifestations of CCD include delayed exfoliation of primary teeth, delayed root development and eruption of permanent teeth, and multiple supernumerary teeth.^13–15 The mutation in the RUNX2 gene hinders the resorption of bone over developing permanent teeth, thus blocking their eruption. ¹⁶ Some studies have shown that failure of eruption of teeth is due to an increase in the amount of acellular cementum and the absence of cellular cementum in the affected roots.^17,18 The vertical development of the alveolar bone is impeded due to the failure or significant delayed eruption of permanent teeth. Mutations in the RUNX2 gene also interfere with the formation of the tooth bud. ¹⁹ The supernumerary teeth in CCD may result from the lack of inhibition or incomplete resorption of tooth bud formation, or from the remnants of dental laminae following dental extraction. ¹⁷ Some tooth germs are malformed and rudimentary, resulting in unusually shaped teeth (eg, peg-like crowns) or abnormally developed roots (root dilaceration or excessive length), and enamel or cementum hypoplasia contributing to thin or underdeveloped enamel/cementum.^18,20

The significant disruption of dental formation and eruption, along with growth anomalies, pose challenges in the dental treatment of CCD patients. Because orthodontic treatment can last several years, it is very important to constantly motivate the patient to sustain compliance and maintain good oral hygiene. The therapy requires a staged interdisciplinary approach and collaboration among the orthodontist, the oral surgeon, and the patient and their family, with the goal of improving esthetics and providing a functional masticatory apparatus. A multistage orthodontic treatment involving surgical extraction of supernumerary teeth and exposure and traction of the impacted teeth is required for successful outcomes. Mechanically, a complete engagement of the teeth present in the arch with an orthodontic fixed appliance helps in the forced eruption of the impacted teeth. Presurgical orthodontics is done to prepare for subsequent orthognathic surgery, typically involving maxillary advancement and mandibular setback, followed by post-surgical orthodontics to achieve good occlusion and esthetic outcomes.

The successful retrieval of the multiple impacted teeth with limited anchorage offered by the few erupted teeth in CCD patients requires complex mechanics and appliances. Currently, there is a lack of information regarding the appropriate mechanics that can be used to reliably retrieve teeth under these circumstances. This case report describes the orthodontic management of an adult female CCD patient with multiple impacted teeth in both arches. There were few teeth available in the mandibular arch to be used as effective anchors for retrieving the unerupted teeth. Thus, a custom appliance serving as a rigid anchor was fabricated for forced eruption using power threads of multiple unerupted mandibular permanent teeth. Fixed appliances and a modified transpalatal arch (TPA) with rigid wire extensions were used to retrieve maxillary teeth. Following the successful retrieval of teeth, the patient is currently undergoing preparation for orthognathic surgery by alignment of teeth and space closure. An additional case is used to demonstrate the entire spectrum of treatment, including retrieval of impacted teeth and orthodontic-surgical outcomes in the Supplemental Figures.

Case Presentation

Pretreatment Findings

A 21-year-old female presented with a chief complaint, “I have missing and crooked teeth, and it's hard to chew.” She had a short stature, concave profile with mesofacial pattern, midfacial hypoplasia, mandibular prognathism, and a confirmed diagnosis of CCD. The patient's mother also has CCD, accompanied by short stature and impacted teeth, illustrating the autosomal dominant mode of inheritance. Intraoral examination (Figure 1) revealed Class III molar relation by 4 mm on the right side and 6 mm on the left side, negative overjet of 13 mm, and 50% deep overbite. Severe crowding was present in the mandibular arch, with the mandibular midline deviated to the right by 5 mm. The arch form was distorted and presented with 2 planes of occlusion for both maxillary and mandibular arches due to the unerupted teeth and dental compensations.

Figure 1.

Pre-treatment records showing (A) intraoral photographs and panoramic radiograph; (B) cephalometric and volumetric CBCT radiographs; and (C) segmented 3D models highlighting multiple impacted, supernumerary and retained primary teeth, and Class III skeletal and dental discrepancy.

The initial panoramic radiograph and CBCT volumetric reconstructions (Figure 1) show multiple retained primary teeth (mandibular primary canines, first molars, second molars, and maxillary primary canines and second molars), 2 supernumerary teeth in the mandibular premolar regions, and impacted permanent teeth (mandibular permanent canines, first and second premolars, second molars, and maxillary lateral incisors, canines and second premolars). The third molars are in the developing stages. The lateral cephalogram demonstrates skeletal III malocclusion due to a prognathic mandible and retrognathic maxilla, validated by Steiner and Wits analyses.

Treatment Objectives and Plan

The treatment objectives are to optimize facial balance by addressing midface deficiency and mandibular prognathism, achieve functional occlusion, correct the arch form, level the occlusal plane, and retrieve as many impacted teeth as possible. To achieve these objectives, a multi-phase treatment plan was developed that included (1) extraction of the retained primary and supernumerary teeth; (2) extraction of the upper permanent second premolars and lower permanent first premolars due to crowding and the predicted difficulty in successfully retrieving them; (3) exposure, bonding and forced eruption of impacted permanent teeth; (4) leveling and aligning; (5) space closure and arch coordination; (6) orthognathic surgery involving maxillary advancement and mandibular setback to correct the skeletal discrepancies; (7) finishing and detailing; and (8) retention.

Treatment Progress

The first stage of treatment involved the extraction of the retained mandibular primary teeth (primary canines, first molars, and second molars), the mandibular supernumerary teeth (in the mandibular premolar region), the permanent lower first premolars, and the mandibular right permanent second molar. This was followed by surgical exposure and bonding of a button with a gold chain on each of the mandibular impacted permanent teeth to aid in traction. The buttons were bonded on the cusp tips of the canines and on the labial surfaces of molars. The extractions were done first in the mandibular arch and then in the maxillary arch to minimize post-surgical morbidity. The teeth extracted in the maxillary arch included the primary canines, first, and second molars. A maxillary frenectomy was also performed because of the fibrous frenum in the interdental space between the maxillary central incisors, which could have prevented space closure.

Since the first molars and incisors were the only teeth present in the mandibular arch, they offered limited anchorage for forced eruption of the mandibular canines and premolars. A custom appliance consisting of two 0.030″ stainless steel labial-lingual arch wires soldered to molar bands was fabricated to provide the necessary support (Figure 2). Horizontal arms with short stops were soldered to the labial and lingual arches at regular intervals to serve as attachment points for elastics and allow for precise force application. These stops ensured that a vertical pull was applied during traction on the impacted teeth. Elastic threads were tied from the gold chains to the appliance, thus applying extrusive forces on the impacted teeth from a rigid anchor. Activation was done by changing the elastic threads every 4 weeks. Hooks were added to the horizontal bars to prevent the sliding of the elastic thread during traction. In addition to banding the molars, resin-based glass ionomer (Band Lok, Reliance Orthodontic Products, Inc) was added to the mandibular anterior teeth to help secure the appliance anteriorly for improved stability. The presence of wires on both the labial and lingual sides enhanced the appliance's stability and rigidity, ensuring effective eruption of the deeply embedded mandibular teeth.

Figure 2.

Progress photographs at 6 months of treatment showing the custom-fabricated mandibular appliance being used for the traction of impacted teeth.

For the maxillary arch, while the incisors, premolars, and molars were considered to offer adequate anchorage for traction, a TPA was placed to enhance transverse stability (Figure 3). The TPA was combined with a palatal arm extending to the canine area on the left side and bilateral buccal extensions from the molar bands that provide the option to retrieve impacted teeth with a variety of different force vectors. Spaces required for the impacted teeth were developed using open coil springs, and traction was applied with elastic threads from buttons on impacted teeth to the cantilever arms. The progress of forced eruption of teeth in both arches is shown in Figure 4A.

Figure 3.

Progress photographs at 12 months of treatment showing incorporation of maxillary appliance with transpalatal arch (TPA) for anchorage to retrieve impacted maxillary teeth with continued traction in the mandibular arch.

Figure 4.

Treatment progress records at (A) 20 months of treatment showing clinically visible teeth in both arches; and (B) at 22 months of treatment when all teeth were bonded to initiate levelling and alignment.

Following the retrieval of the impacted teeth, the labial wire and horizontal arms were removed from the mandibular appliance, thus converting it into a lingual arch. The cantilever arms on the TPA were removed, and the TPA was maintained for stability during levelling and alignment (Figure 4B). At present, all permanent teeth have been successfully retrieved into the arch (Figure 5), and once more fully erupted, the brackets were repositioned to optimize tooth positions. Space closure by protracting the lower molars and premolars and arch coordination will complete the pre-surgical treatment plan. To demonstrate the entire range of treatment provided to CCD patients, a similar completed case demonstrating retrieval of impacted teeth, surgical orthodontics, and treatment completion is presented in Supplemental Figures 1 to 7. Two difficult-to-retrieve teeth were left in situ for this patient.

Figure 5.

Progress intraoral photographs and panoramic radiographs at 24 months of treatment depicting the retrieval of all impacted teeth.

Discussion

CCD is of clinical significance to dentistry due to prolonged retention of deciduous teeth, multiple impacted permanent teeth, and supernumerary teeth.^18–22 The delayed dental development is believed to be caused by mutations in the RUNX2 gene, which is essential for both tooth development and in regulating the differentiation of root progenitor cells.^23,24 Management of the orofacial manifestations of CCD is lengthy and challenging, and requires careful planning and execution by an interdisciplinary team. Simply extracting the primary or supernumerary teeth itself does not usually promote the eruption of unerupted permanent teeth, thus highlighting the need for orthodontic intervention to guide the eruption of impacted teeth. Due to the disease's rarity, there are few guidelines in the literature for treating CCD cases. Early diagnosis and timing of intervention are important, and it is essential to develop an individualized treatment plan and mechanics for each CCD patient.

Various clinical protocols have been proposed to treat patients with CCD depending on the patient's goals, social and economic circumstances, the patient's age, periodontal and endodontic health, and the eruption stage of permanent dentition as follows^8,25,26:

The Toronto-Melbourne approach ⁸ is based on timed, serial extractions of primary teeth. It depends on the extent to which the roots of the permanent teeth have developed. The surgical procedures usually begin at about 5 to 6 years of age with the extraction of the anterior primary teeth. At 6 or 7 years of age, the primary incisors are extracted, and healing is allowed. Orthodontic brackets are placed on the permanent incisors as soon as possible, and the primary posterior teeth are extracted. When the patient is 9 or 10 years of age, the permanent premolars are exposed and allowed to heal. Thereafter, orthodontic brackets are placed on the permanent canines and premolars. During each procedure, which is performed under general anesthesia, the supernumerary teeth are also extracted together with the bone covering the underlying permanent teeth.

The Jerusalem approach ⁸ is based on at least 2 surgical interventions, depending on the root development of the permanent dentition. In the first phase, which begins approximately at 10 to 10 years of age, the anterior teeth are involved. In the second phase, performed at age 13 years and older, the posterior primary teeth are extracted, unerupted canines and premolars are exposed, and orthodontic treatment is completed. The extraction and exposure are carried out simultaneously in both jaws under general anesthesia. One of the goals of this approach is to immediately deal with the absence of anterior teeth by placing an orthodontic appliance to erupt the anterior teeth first.

The Bronx Approach ⁸ uses an interim overdenture prosthesis for functional and esthetic reasons due to the long course of treatment. The age is not specified; however, the initial preference is to allow spontaneous eruption, followed relatively quickly by orthodontically aided eruption. The final surgical step consists of a Le Fort I osteotomy, maxillary down graft, and replacement by dental implants of any teeth that fail to erupt.

The Belfast-Hamburg approach ⁸ involves only a single surgical episode. All deciduous and supernumerary teeth are extracted, and all unerupted permanent teeth are exposed simultaneously under general anesthesia. Surgical packing is placed, and healing occurs by secondary intention. The surgical packs are changed frequently until brackets can be bonded to the teeth. Orthodontic appliances are placed on the few fully erupted teeth, and elastics are tied to the unerupted teeth to encourage eruption.

A temporary option is to retain the primary maxillary anterior teeth and to restore esthetics by performing a composite build-up until the patient is ready to undergo surgical intervention. In the short-term, the treatment may be considered successful and effective in improving the esthetics of the patient. ²⁵ Cosmetic recontouring of teeth can be a conservative and esthetic alternative to re-establish the form, shape, and color. ²⁶

Various other techniques have been mentioned in the literature for managing impacted teeth in CCD using other modified appliances. Davies et al ²⁷ reported the use of a chin cup with inter-maxillary elastics to apply traction. Soheilifar et al ²⁸ used a 0.036-inch fixed lower lingual arch, a TMA labial wire with 4 loops (2 anterior to the molars and 2 at the canine area), and high-pull headgear with J-hooks. The attachments of the impacted teeth are ligated to this labial wire. Stainless steel J-hooks are engaged in the anterior canine loops and attached to a high-pull headgear with 90-gram force on each side. After levelling and aligning the other teeth, an elastic thread or chain is used for the traction of the impacted teeth. Other methods involve the use of temporary anchorage devices (TADs). Using mini screws, Shetty et al ²⁹ placed an “L”-shaped miniplate modified by soldering a 0.035″ stainless steel wire with hooks for attachment of elastics in the lower anterior region below the root apices. An extrusive force is applied from the hooks to the impacted teeth with elastics. This, however, is an invasive option. Kuroda et al ³⁰ recommended enhancing anchorage on a modified lingual arch using 2 titanium screws placed in the palate and applying a traction force of 50 g via elastics from the screw-supported lingual arch to the impacted teeth, initiated 4 weeks post-surgery with a monthly change of elastics. However, potential complications include TAD instability and peri-implant soft tissue overgrowth. Overall, the aforementioned methods, while effective for the traction of impacted teeth, require a high level of patient compliance or use of adjunctive appliances such as TADs, which, although minimally invasive, introduce additional procedural considerations and potential risks. The customized-fabricated appliance used in this case provided sufficiently robust anchorage for successful and stable orthodontic traction of multiple teeth without the need for additional anchorage devices.

A possible treatment alternative for the patient in our case report included composite build-up and the retention of primary teeth. This option was not recommended because it would compromise optimal function and esthetics. Prosthetic surgery could also have been an alternative course of treatment. Cases of oral rehabilitation using implant-supported fixed dental prostheses in middle-aged CCD patients have been reported.^31,32 However, the use of implants is generally contraindicated due to the presence of multiple unerupted teeth, which reduces the amount of available bone and increases the risk of bone fragility. ³³ It was thus decided to opt for orthodontic traction followed by bi-jaw orthognathic surgery to ensure the best results. Young patients have a relatively high probability of successful retrieval of impacted teeth. The combination of surgical exposure of permanent teeth and orthodontic traction ensured the patient's natural dentition would be maintained, and to help achieve good function and esthetics. Orthodontic traction can also promote bone formation around the erupting impacted teeth.^8,32 For the surgical exposure of impacted teeth, a closed eruption surgical technique was preferred as it allows acceptable periodontal outcomes, minimizes wound contamination and infection, and promotes healing by primary intention.

The greatest biomechanics challenge was the lack of an adequate number of permanent teeth in the mandibular arch to serve as anchors. To ensure the successful retrieval of the impacted teeth, a rigid appliance was fabricated to aid in traction. In retrospect, the short stops soldered on the horizontal arms could have been avoided, as they might have been a source of irritation to the mucosa. Also, nickel-titanium coil springs, which apply low, constant forces, could have been used as an alternative to elastics for the forced eruption. This requires fewer reactivations and thus reduces the number of appointments. Orthognathic surgery represents a key stage in the overall management. It allows improvement in both function and esthetics. In this patient, Le Fort I maxillary advancement combined with bilateral sagittal split mandibular setback surgery will be performed to improve the profile and occlusion similar to that shown for the patient in Supplemental Figures 1 to 7. Retention following completion of treatment will involve the use of Hawley retainers and follow-up visits at 2, 6, and 12 months.

Conclusion

CCD is a complex congenital disease characterized by multiple dental and skeletal abnormalities. A multi-phase, long-term orthodontic and surgical intervention is required for treatment, and thorough planning is crucial to its success. Performing traction of multiple impacted teeth is a challenge because of inadequate anchorage. Lack of adequate information in the literature provide a limited roadmap or protocols on appliances to use and how to perform the traction to result in adequate outcomes.

In this case report, special emphasis has been given to an individualized treatment protocol with the use of a custom-fabricated appliance to facilitate the traction of the impacted teeth in cases where there are very few teeth erupted in the arch to offer anchorage. The interdisciplinary approach involving surgical and orthodontic teams allows for predictable results, preservation of natural teeth, and long-term stability. Continued documentation of such cases will help establish more defined treatment guidelines and improve outcomes for patients in the future.

Supplemental Material

sj-docx-1-cpc-10.1177_10556656261447538 - Supplemental material for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia

Supplemental material, sj-docx-1-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

Supplemental Material

sj-pdf-2-cpc-10.1177_10556656261447538 - Supplemental material for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia

Supplemental material, sj-pdf-2-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

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sj-pdf-3-cpc-10.1177_10556656261447538 - Supplemental material for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia

Supplemental material, sj-pdf-3-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

Supplemental Material

sj-pdf-4-cpc-10.1177_10556656261447538 - Supplemental material for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia

Supplemental material, sj-pdf-4-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

Supplemental Material

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Supplemental material, sj-pdf-5-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

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Supplemental material, sj-pdf-6-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

Supplemental Material

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Supplemental material, sj-pdf-7-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal

Supplemental Material

sj-pdf-8-cpc-10.1177_10556656261447538 - Supplemental material for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia

Supplemental material, sj-pdf-8-cpc-10.1177_10556656261447538 for Optimized Approach for Retrieval of Multiple Impacted Teeth in Cleidocranial Dysplasia by Roohi Sobti, Shilpa Upadhyay, Andrew Paige, Juliana Panchura, Yi Lin and Sunil Kapila in The Cleft Palate Craniofacial Journal