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Craniofacial Surgery in Children With Down Syndrome: A 6-decade Scoping Review
John Warner-Levy1, Henry S Chan1, Loren Wilkins1
1From the Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom.
Insights
Children with Down syndrome often need craniofacial interventions, but guidelines are lacking. This study found significant geographic variations in procedures and proposes preliminary guidelines to improve care.
Area of Science:
- Medical research
- Pediatric surgery
- Genetics
Background:
- Children with Down syndrome (DS) frequently exhibit craniofacial anomalies requiring surgical, dental, or orthodontic interventions.
- Existing guidance for managing these craniofacial aspects in pediatric DS care is limited.
- Geographic disparities in the availability and reporting of craniofacial procedures for DS patients are notable.
Purpose of the Study:
- To investigate the landscape of craniofacial procedures performed on children with Down syndrome globally.
- To identify geographic variations in the availability of these interventions.
- To assess the current state of guidelines and algorithms for craniofacial care in pediatric DS.
Main Methods:
- A systematic literature search was conducted across PubMed, Web of Science, and African Journals OnLine from 1960 to 2024.
- Studies reporting elective craniofacial surgical, dental, or orthodontic interventions in pediatric Down syndrome patients were included.
- Data on procedure types, patient demographics, and age at intervention were extracted.
Main Results:
- A total of 92 studies detailed 2815 craniofacial procedures, with the United States, Canada, and Germany reporting the highest numbers.
- No procedures were recorded in Africa or South Asia.
- Mean ages at intervention varied significantly by procedure, from palatal plate therapy (2.2 years) to hypoglossal nerve stimulator implantation (14.8 years).
- No comprehensive craniofacial care guidelines were identified, though four care algorithms were found.
Conclusions:
- There is a significant gap in established craniofacial care guidelines for children with Down syndrome.
- The study highlights the need for developing preliminary guidelines, emphasizing early screening and timely reconstructive care.
- Further research is required to validate and refine proposed guidelines for optimal patient outcomes.
Background:
Children with Down syndrome often present with craniofacial anomalies, some of which may be addressed through early surgical interventions. Unfortunately, there is limited guidance on considerations surrounding these procedures. This study aimed to examine craniofacial procedures across different countries, highlighting geographic variations and the availability of interventions and guidelines regarding craniofacial aspects of care for children with Down syndrome.
Methods:
PubMed, Web of Science, and African Journals OnLine were searched from January 1, 1960, to March 31, 2024. Eligible studies reported elective craniofacial surgical, dental, or orthodontic interventions in pediatric Down syndrome patients and included at least 1 age statistic.
Results:
The 92 eligible studies comprised 2815 procedures. The United States contributed the largest number of procedures (1727, 61.4%), followed by Canada (345, 12.3%) and Germany (226, 8.0%). No recorded procedures were performed within Africa or South Asia. Globally, the mean ages of patients (in years) at the time of surgery, along with SD and coefficient of variation, were as follows: palatal plate therapy, 2.2 (3.1) (141.4%); tonsillectomy, 5.5 (3.9) (70.6%); extraocular muscle realignment, 6.9 (4.4) (63.5%); mastoidectomy, 10.1 (4.3) (42.7%); glossectomy, 10.0 (3.5) (35.1%); and hypoglossal nerve stimulator implantation, 14.8 (3.1) (21.2%). Although no established craniofacial guidelines were identified, 4 algorithms addressing craniofacial aspects of care were found.
Conclusions:
To address this gap, we propose the development of preliminary craniofacial care guidelines for children with Down syndrome, prioritizing early screening measures to facilitate timely access to reconstructive care. Subsequent studies will be essential to validate and refine these guidelines.
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