Introduction
A multidisciplinary conference was convened in March 2010 with the charge to develop parameters of care for patients with craniosynostosis. The 52 participants represented 16 medical specialties and 16 professional societies. Herein, we present the dental, orthodontic, and surgical care recommendations for those with craniosynostosis, with special emphasis on craniosynostosis syndromes.
Methods
Plenary and small-group iterative discussions were held to draft specialty-specific parameters of care. All participants reviewed and discussed each specialty-specific document. Special care was taken to ensure cross-discipline interactions, recognizing the importance of interdisciplinary team care.
Results
A unified document was produced delineating longitudinal care parameters from prenatal assessment and consultation to adulthood in all the represented specialty areas. The dental and orthodontic care parameters from infancy to adulthood are explained in terms of stages of development and coordinated with interdisciplinary assessments and interventions.
Conclusions
The consensus document provides a detailed description of physical, functional, and cognitive development in persons with craniosynostosis and recommends staged team observations and interventions. The expectation is that the document will help to ensure state-of-the-art care for patients with craniosynostosis and provide a generally acceptable framework for collaborative studies.
Craniosynostosis is the premature fusion of at least 1 cranial suture. This relatively common developmental anomaly affects approximately 1 in 2000 live births worldwide. In most cases, this condition affects only 1 cranial suture and is described as “isolated” craniosynostosis. Premature suture fusion results in abnormal head shape caused by growth inhibition at the affected suture and compensative growth at other patent sutures. Although any cranial suture can prematurely fuse, the sagittal suture is most commonly affected; sagittal suture fusion results in scaphocephaly (ie, a narrow, elongated skull shape). Craniosynostosis can occur in association with more than 130 syndromes. The most common craniosynostosis syndromes are Crouzon and Pfeiffer (each, 1 in 60,000 live births), but Muenke (1 in 140,000 live births) and Apert (1 in 160,000 live births) syndromes are also commonly seen at referral centers. Whereas most persons with a craniosynostosis syndrome have acquired a new gene mutation, Mendelian inheritance transmission patterns are well known. Intensive genetics investigations have discovered more than 100 mutations in genes such as FGFR1-3 , NELL-1 , MSX2 , TWIST , and GLI3 . In addition, mechanotransduction signaling pathways have been implicated in the etiopathogenesis of craniosynostosis.
Physical findings in craniosynostosis syndromes can include skull deformity, hydrocephalus, midface hypoplasia, and extremity anomalies. Developmental delays can also occur. Because of the complexity of the functional and structural abnormalities, the care of a patient with craniosynostosis should be managed by a multi-specialty team providing interdisciplinary care. The American Cleft Palate-Craniofacial Association defines interdisciplinary team care in the “Parameters for the Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies.” For the management of craniosynostosis, these interdisciplinary teams can be comprised of professionals from the following disciplines: anesthesiology, craniofacial surgery, genetics, hand surgery, intensive care, neurosurgery, nursing, ophthalmology, oral and maxillofacial surgery, orthodontics, otolaryngology, pediatrics, pediatric dentistry, prosthodontics, psychology, radiology, social work, and speech and language pathology. If multidisciplinary team parameters as proposed by the consensus conference are adopted by craniofacial teams, standard study designs and reporting protocols could be developed to yield outcome studies better than the level 5 (expert opinion) and 4 (case series) evidence currently in the public domain. Herein, we focus on the general oral health needs and orthodontic and surgical management of patients with craniosynostosis, with particular emphasis on those with craniosynostosis syndromes.
A multidisciplinary meeting was held from March 4 to 6, 2010, entitled “Craniosynostosis: Developing Parameters for Diagnosis, Treatment, and Management,” with the goal to create parameters of care for persons with craniosynostosis. A detailed description of the proceedings and the entire document resulting from the proceedings have been published in the Cleft Palate-Craniofacial Journal .
In addition to the established routine oral health care needs, people with craniosynostosis, particularly those with syndromic craniosynostosis, have unique oral health and craniofacial growth problems. The dental providers should, therefore, have special training in the care of these complicated conditions ( Table I ). Children with craniosynostosis often have an increased need for dental care because of missing or malformed teeth, delayed and ectopic eruptions, dental crowding, dental caries, propensity for periodontal disease, and problems with motivation and the physical ability to perform regular oral hygiene procedures. Dental caries can result in difficult and costly treatment, and diminish the overall quality of life. Furthermore, healthy teeth and gingival tissues are essential for the required orthodontic treatment.
| 1. Board certification or board eligibility in their respective specialties and state specialty licensure where applicable |
| 2. Membership in a craniofacial team |
| 3. A case load that ensures experience in care of patients with isolated and syndromic craniosynostosis |
| 4. Ongoing continuing education in the craniofacial field |
| 5. Formal training in craniofacial and special-care orthodontics (orthodontic specialist) |
The basis of preventive oral health education and dental care begins with perinatal and infant oral health ( Table II ). The early establishment of a dental home is essential. Oral examination must be provided at least by the eruption of the first tooth and no later than 12 months of age. Anticipatory guidance, including oral hygiene and dietary counseling and caries risk assessment, is a critical component of early dental intervention.
| Age | Recommendations |
|---|---|
| Birth-2 years | Baseline diagnostic records (eg, full head CT scans; cephalograms; intraoral, facial, and head photographs) |
| Oral examination: intraoral clefts, bifid uvula, palatal swellings, neonatal teeth, maxillomandibular relationships. Needs to be repeated every 6 months or as indicated by risk status or susceptibility | |
| Caries risk assessment | |
| Anticipatory guidance or counseling (oral hygiene, dietary, injury prevention, nonnutritive habits) | |
| Fluoride supplementation assessment | |
| Dental prophylaxis and topical fluoride | |
| Monitor craniofacial growth and development, including the deciduous dentition | |
| Midface surgical treatment if indicated (planning through cephalometric analysis and surgical predictions; appliance selection; fabrication and insertion of intraoral appliances; postsurgical follow-up and documentation) | |
| 3-6 years | Oral examination: needs to be repeated every 6 months or as indicated by risk status or susceptibility |
| Caries risk assessment | |
| Anticipatory guidance or counseling (oral hygiene, dietary, injury prevention, nonnutritive habits) | |
| Fluoride supplementation assessment | |
| Dental prophylaxis and topical fluoride | |
| Pit and fissure sealant assessment | |
| Monitor dental development (delayed dental eruption, missing teeth, dental crowding, abnormal intra-arch and interarch relationships) | |
| Collection and analysis of longitudinal growth records at appropriate intervals (CT scans, CBCT scans, cephalograms, photographs, dental casts) | |
| Midface surgical treatment if indicated (planning through cephalometric analysis and surgical predictions; appliance selection; fabrication and insertion of intraoral appliances; postsurgical follow-up and documentation) | |
| Transitional dentition, 7-15 years | Oral examination: needs to be repeated every 6 months or as indicated by risk status or susceptibility |
| Caries risk assessment | |
| Periodontal disease assessment | |
| Anticipatory guidance or counseling (oral hygiene, dietary, injury prevention, nonnutritive habits) | |
| Fluoride supplementation assessment | |
| Dental prophylaxis and topical fluoride | |
| Pit and fissure sealant assessment | |
| Phase 1 orthodontic treatment | |
| Provide active eruption guidance for permanent teeth that are severely delayed | |
| Retention between active orthodontic treatment phases | |
| Surgical treatment if indicated (planning through cephalometric analysis and surgical predictions; appliance selection; fabrication and insertion of intraoral appliances; postsurgical follow-up and documentation) | |
| Evaluation of molar positions before surgical procedures and planning with surgeon for the best approach to prevent damage to unerupted teeth | |
| Permanent dentition, 12-21 years | Oral examination: needs to be repeated every 6 months or as indicated by risk status or susceptibility |
| Caries risk assessment | |
| Periodontal disease assessment | |
| Anticipatory guidance or counseling (oral hygiene, dietary, injury prevention, nonnutritive habits, timing of dental care) | |
| Fluoride supplementation assessment | |
| Dental prophylaxis and topical fluoride | |
| Pit and fissure sealant assessment | |
| Phase 2 orthodontic treatment | |
| Consult with patient’s dentist or prosthodontist on general dental treatment objectives (eg, periodontal considerations, replacement of missing teeth, space requirements for dental implants, restoration to full anatomic dimension of peg or abnormally shaped teeth, and other long-term maintenance and preventive dental care needs) | |
| Management of dental impactions with surgical exposure and orthodontic assisted eruption | |
| Referral for intraoral soft-tissue procedures (eg, frenectomy, reduction of palatal swellings, periodontal surgery) | |
| Referral for dental extractions required for orthodontic treatment | |
| Referral for third molar extractions | |
| Orthodontic preparation for craniofacial and orthognathic surgery (eg, facial bipartition procedure, midface advancement or distraction osteogenesis, LeFort I advancement, or 2-jaw surgical correction) at skeletal maturity | |
| Skeletal maturity assessment by analysis of serial lateral cephalograms, closure of the epiphyseal growth plates in the hand-wrist film, and systematic collection and evaluation of body and height data | |
| Surgical treatment (planning through cephalometric analysis and surgical predictions; appliance selection; fabrication and insertion of intraoral appliances; postsurgical follow-up and documentation) | |
| Evaluation of molar positions before surgical procedures and planning with surgeon for the best approach to prevent damage to unerupted teeth | |
| Complete postsurgical orthodontic treatment and provide long-term orthodontic retention | |
| Obtain final records | |
| Refer to appropriate dental professionals for replacement of missing teeth and general maintenance and preventive dental care | |
| Skeletal maturity, 21 years onward | Long-term follow-up assessment at appropriate intervals |
| Pertinent records and treatment as necessary |
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