Table 3 Statements that reached > 80% agreement in Round 2, comprising the best practice guidelines
From: Best practice guidelines in managing the craniofacial aspects of skeletal dysplasia
| Â | Strongly agree | Agree | Neutral | Disagree | Strongly disagree |
|---|---|---|---|---|---|
1. Patients with skeletal dysplasia are more likely than the general population to have abnormal upper airway morphology and function, which can contribute to increased morbidity/mortality | 13 (100%) | 0 | 0 | 0 | 0 |
2. The mortality and morbidity risks for patients with skeletal dysplasia undergoing surgery are greater than the general population | 12 (100%) | 0 | 0 | 0 | 0 |
3. Clinicians should evaluate for signs and symptoms of upper airway obstruction and sleep disordered breathing in patients with skeletal dysplasia at each clinic visit | 8 (61.5%) | 5 (38.5%) | 0 | 0 | 0 |
4. Polysomnography should be performed in patients with skeletal dysplasia who have snoring or signs and symptoms of sleep disordered breathing | 9 (69.2%) | 4 (30.8) | 0 | 0 | 0 |
5. MRI of the craniocervical junction should be considered in infants with achondroplasia and sleep disordered breathing | 7 (53.8%) | 6 (46.2%) | 0 | 0 | 0 |
6. Hearing loss is more prevalent in patients with skeletal dysplasia than in the general population | 10(76.9%) | 3 (23.1%) | 0 | 0 | 0 |
7. Patients with skeletal dysplasia should have hearing assessed at birth or time of diagnosis and at age 5Â years | 3 (23.1%) | 8 (61.5%) | 2 (15.4%) | 0 | 0 |
9. Comprehensive audiologic evaluation should be performed on any child with skeletal dysplasia who has speech delay, suspicion of hearing difficulties, or signs/symptoms of middle ear disease | 11 (84.6%) | 2 (15.4%) | 0 | 0 | 0 |
10.Children with skeletal dysplasia who have otitis media with effusion are at increased risk of speech, language, or learning problems | 4 (30.8%) | 7 (53.8%) | 1 (7.7%) | 1 (7.7%) | 0 |
11. Tympanostomy tube insertion may be performed in children with skeletal dysplasia and unilateral or bilateral otitis media with effusion that is unlikely to resolve quickly, as reflected by a type B (flat) tympanogram or persistence of effusion for 3Â months or longer | 2 (15.4%) | 11 (84.6%) | 0 | 0 | 0 |
12. At the time of tympanostomy tube placement in children with achondroplasia, the surgeon should look for otoscopic signs of a high jugular bulb | 8 (61.5%) | 5 (38.5%) | 0 | 0 | 0 |
13. Children with skeletal dysplasia and a history of recurrent acute otitis media should be assessed for persistent middle ear disease | 5 (38.5%) | 8 (61.5%) | 0 | 0 | 0 |
14. Children with skeletal dysplasia and acute otitis media should be managed as per established guidelines for the general population | 3 (23.1%) | 10 (76.9%) | 0 | 0 | 0 |
15. Adenoidectomy and/or tonsillectomy should be considered first-line therapy for children with skeletal dysplasia and obstructive sleep apnea | 6 (46.1%) | 7 (53.9%) | 0 | 0 | 0 |
16. Non-invasive positive pressure ventilation is a treatment option for patients with skeletal dysplasia and obstructive sleep apnea | 9 (69.2%) | 4 (30.8%) | 0 | 0 | 0 |
17. Children with skeletal dysplasia should undergo polysomnography before adenoidectomy and/or tonsillectomy is performed | 8 (61.5%) | (38.5%) | 0 | 0 | 0 |
18. Children with skeletal dysplasia who undergo adenoidectomy and/or tonsillectomy for obstructive sleep apnea should be monitored overnight for respiratory difficulties after surgery | 8 (61.5%) | (38.5%) | 0 | 0 | 0 |
19. Children with skeletal dysplasia have a higher prevalence of soft and hard palate abnormalities compared to the general population | 8 (61.5%) | (38.5%) | 0 | 0 | 0 |
20. Children with skeletal dysplasia have a higher prevalence of midfacial, dental and jaw abnormalities compared to the general population | 8 (61.5%) | (38.5%) | 0 | 0 | 0 |
21. Specialized dental and orthodontic care are part of the core clinical management of patients with skeletal dysplasia, starting in early childhood | 9 (69.2%) | 4 (30.8%) | 0 | 0 | 0 |
22. Stridor or hoarseness in patients who have skeletal dysplasia warrants further evaluation that may include imaging and/or visualization of the larynx | 4 (30.8%) | 9 (69.2%) | 0 | 0 | 0 |
23. In infants with diastrophic dysplasia, auricular cystic swelling may occur. Incision and drainage techniques do not appear to improve outcomes | 9 (69.2%) | 4 (30.8%) | 0 | 0 | 0 |