CE: ; SCS-20-0487; Total nos of Pages: 100; SCS-20-0487 EDUCATIONAL SUPPLEMENT European Guideline Craniofacial microsomia R.W. Renkema, the ERN CRANIO working group on craniofacial microsomia Index Summary 00 Chapter 1. Introduction 00 1.1 Incentive for making the guideline 00 1.2 Purpose of the guideline 00 1.3 Scope of the guideline 00 1.4 Relationship to other congenital facial malformations 00 1.5 Intended users of the guideline 00 1.6 About craniofacial microsomia 00 1.7 European Reference Networks 00 Chapter 2. Methodology 00 2.1 Validity of the guideline 00 2.2 General information 00 2.3 Aim and target audience guideline 00 2.3.1 Aim of the Guideline 00 2.3.2 Target audience 00 2.3.3 Patient population 00 2.4 Steering group 00 2.5 Conflicts of interest 00 2.6 Patient perspectives 00 2.7 Implementation 00 2.8 Methods 00 2.8.1 Bottleneck analysis 00 2.8.2 Questions and outcomes 00 2.8.3 Literature search and selection of literature 00 2.8.4 Quality assessment of individual studies 00 2.8.5 Summary of literature 00 2.8.6 Quality of evidence 00 2.8.7 Formulating conclusions 00 2.8.8 Considerations 00 2.8.9 Formulating recommendations 00 2.8.10 Conditions (organisation of care) 00 2.8.11 Knowledge gap 00 2.8.12 Evaluation and authorisation phase 00 Chapter 3. Diagnostic criteria for craniofacial microsomia 00 3.1 Based on which criteria is a child or adult with craniofacial microsomia diagnosed? 00 Chapter 4. Screening, monitoring and indication for treatment 00 4.1 Breathing difficulties in craniofacial microsomia 00 4.1.1 What is the type, prevalence and severity of breathing difficulties in craniofacial microsomia? 00 From the Department of Plastic and Reconstructive Surgery and Hand Surgery and department of Maxillofacial Surgery, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, The Netherlands. Received March 29, 2020. Accepted for publication April 21, 2020. [email protected] An initiative from the European Reference Network for rare and/or complex craniofacial anomalies and ear, nose and throat (ENT) disorders (ERN CRANIO) Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www.jcraniofacialsurgery.com). Copyright # 2020 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000006691 The Journal of Craniofacial Surgery Volume 00, Number 00, Month 2020 1 Copyright © 2020 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. CE: ; SCS-20-0487; Total nos of Pages: 100; SCS-20-0487 Renkema The Journal of Craniofacial Surgery Volume 00, Number 00, Month 2020 4.1.2 What is the policy for screening and monitoring of breathing difficulties (OSA) in patients with craniofacial microsomia? 00 4.1.3 What are the indications and policy for treatment of breathing difficulties (OSA) in patients with craniofacial microsomia? 00 4.2 Feeding difficulties in craniofacial microsomia 00 4.2.1 What is the type, prevalence and severity of feeding difficulties in craniofacial microsomia? 00 4.2.2 What is the policy for screening and monitoring of feeding difficulties in patients with craniofacial microsomia? 00 4.2.3 What are the indications and policy for treatment of feeding difficulties in patients with craniofacial microsomia? 00 4.3 Speech and Language difficulties in craniofacial microsomia 00 4.3.1 What is the type, prevalence and severity of speech difficulties in craniofacial microsomia? 00 4.3.2 What is the policy for screening and monitoring of speech and language difficulties in patients with craniofacial microsomia? 00 4.3.3 What are the indications and policy for treatment of speech difficulties in patients with craniofacial microsomia? 00 4.4 Hearing difficulties in craniofacial microsomia 00 4.4.1 What is the type, prevalence and severity of hearing difficulties in craniofacial microsomia? 00 4.4.2 What is the policy for screening and monitoring of hearing difficulties in patients with craniofacial microsomia? 00 4.4.3 What are the indications and policy for treatment of hearing difficulties in patients with craniofacial microsomia? 00 4.5 Eye anomalies in craniofacial microsomia 00 4.5.1 What is the type, prevalence and severity of eye anomalies in craniofacial microsomia? 00 4.5.2 What is the policy for screening and monitoring of eye anomalies in patients with craniofacial microsomia? 00 4.5.3 What are the indications and policy for treatment of eye anomalies in patients with craniofacial microsomia? 00 4.6 Dental deformities in craniofacial microsomia 00 4.6.1 What is the type, prevalence and severity of dental deformities in craniofacial microsomia? 00 4.6.2 What is the policy for screening and monitoring of dental deformities in patients with craniofacial microsomia? 00 4.6.3 What are the indications and policy for treatment of dental deformities in patients with craniofacial microsomia? 00 4.7 Vertebral anomalies in craniofacial microsomia 00 4.7.1 What is the type, prevalence and severity of vertebral anomalies in craniofacial microsomia? 00 4.7.2 What is the policy for screening and monitoring of vertebral anomalies in patients with craniofacial microsomia? 00 4.7.3 What are the indications and policy for treatment of vertebral anomalies in patients with craniofacial microsomia? 00 4.8 Psychosocial difficulties in craniofacial microsomia 00 4.8.1 What are the type, prevalence and severity of psychosocial difficulties in craniofacial microsomia? 00 4.8.2 What is the policy for the screening and monitoring of psychosocial difficulties in patients with craniofacial microsomia? 00 4.8.3 What are the indications and policies for the treatment of psychosocial difficulties in patients with craniofacial microsomia? 00 Chapter 5. Surgical treatment 00 5.1 Mandible & Maxilla 00 5.1.1 What is the indication for surgical treatment of mandibular and maxillary deformity in patients with craniofacial microsomia? 00 5.1.2 What is the most optimal treatment modality and its timing for mandibular/maxillary deformity in patients with craniofacial 00 microsomia regarding severity, breathing problems, occlusal problems and aesthetics? 5.2 Facial nerve 00 5.2.1 What is the indication for surgical treatment of facial nerve anomaly in patients with craniofacial microsomia? 00 5.2.2 What is the most optimal treatment modality for facial nerve anomaly in patients with craniofacial microsomia 00 related to functional deficits and aesthetics? 5.3 Soft tissues 00 5.3.1 What is the indication for surgical treatment of soft tissue deficiency in patients with craniofacial microsomia? 00 5.3.2 What is the most optimal treatment modality for soft tissue deficiency in patients with craniofacial microsomia 00 related to severity and its timing? 5.4 Microtia 00 5.4.1 What is the indication for surgical treatment of ear deformity in patients with craniofacial microsomia? 00 5.4.2 What is the most optimal treatment modality for ear deformity in patients with craniofacial microsomia related to its timing? 00 Chapter 6. Organisation of care 00 Minimal care standards and monitoring outcomes 00 6.1 What are the minimal care standards to treat patients with craniofacial microsomia and how should outcomes of care be monitored? 00 Appendix 1. Additional questions 00 Appendix 2. Literature searches 00 Appendix 3. Bottlenecks from patient’s perspective 00 Appendix 4. Definitions 00 Appendix 5. Reporting speech outcomes 00 Appendix 6. Evidence table 00 2 # 2020 Mutaz B. Habal, MD Copyright © 2020 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. CE: ; SCS-20-0487; Total nos of Pages: 100; SCS-20-0487 The Journal of Craniofacial Surgery Volume 00, Number 00, Month 2020 European Guideline Craniofacial microsomia SUMMARY 4.1.2 What is the policy for screening and monitoring of his guideline provides the following chapters: breathing difficulties (OSA) in patients with craniofacial micro- T somia? Chapter 3 Diagnostic criteria for craniofacial microsomia Chapter 4 Screening, monitoring and indication for treatment All patients with craniofacial microsomia should be 4.1 Breathing difficulties in craniofacial microsomia screened with a questionnaire biannually, at least up to 4.2 Feeding difficulties in craniofacial microsomia the age of six, in the outpatient department for a clinical history of obstructive sleep apnoea. 4.3 Speech difficulties in craniofacial microsomia If there is a suspicion of obstructive sleep apnoea based 4.4 Hearing difficulties in craniofacial microsomia on a questionnaire, a polysomnography (sleep study) 4.5 Eye anomalies in craniofacial microsomia has to be performed. 4.6 Dental deformities in craniofacial microsomia 4.7 Vertebral anomalies in craniofacial microsomia 4.8 Psychosocial difficulties in craniofacial microsomia Chapter 5 Surgical treatment of craniofacial microsomia All patients who have Pruzansky-Kaban IIb or III mandibles and/or are bilaterally affected have to 5.1 Mandible & Maxilla undergo a polysomnography (sleep study) to screen 5.2 Facial nerve for obstructive sleep apnoea in the first year of life. 5.3 Soft tissues 5.4 Microtia Chapter 6 Organisation of care 4.1.3 What are the indications and policy for treatment of breathing difficulties (OSA) in patients with craniofacial micro- somia? The following recommendations were agreed on: Chapter 3. Diagnostic criteria for craniofacial microsomia 3.1 On which criteria is a child or adult with craniofacial Treatment of children with craniofacial microsomia and microsomia diagnosed? obstructive sleep apnoea has to be discussed in a multidisciplinary team. Terminology It is advised to exclusively use the term craniofacial microsomia. Discard the use of other terms such as Treatment of children with craniofacial microsomia and Goldenhar syndrome, hemifacial microsomia or aur- obstructive sleep apnoea depends on the age of the child, iculo-oculo-vertebral spectrum.