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Craniofacial morphology in children with cystic fibrosis
E Hellsing1, V Brattström, B Strandvik
1Department of Orthodontics, Karolinska Institutet, Huddinge, Sweden.
Insights
Children with cystic fibrosis (CF) exhibit distinct facial morphology, including open bite and altered facial heights. These changes are more pronounced in those with respiratory issues, resembling patterns seen in nasal obstruction.
Area of Science:
- Craniofacial morphology
- Pediatric genetics
- Respiratory medicine
Background:
- Cystic fibrosis (CF) is a genetic disorder characterized by abnormal mucus production, leading to airway obstruction and pancreatic insufficiency.
- Progressive respiratory disease in CF can cause respiratory insufficiency and cor pulmonale.
- Facial morphology alterations are increasingly recognized in various chronic childhood conditions.
Purpose of the Study:
- To investigate and compare the facial morphology of children diagnosed with cystic fibrosis (CF).
- To identify potential differences in facial structure between CF patients with gastrointestinal versus predominantly respiratory symptoms.
- To compare CF patients' facial morphology to that of healthy controls.
Main Methods:
- Lateral skull radiographs were obtained from 11 children with CF (divided into gastrointestinal and respiratory subgroups) and 11 healthy controls.
- Radiographs were digitized, and linear and angular cephalometric variables were calculated.
- Statistical comparisons were performed between the groups.
Main Results:
- Children with CF demonstrated an open bite, reduced posterior facial height, and increased mandibular and craniocervical inclination compared to controls.
- CF patients with respiratory insufficiency showed greater morphological deviations from controls than those with gastrointestinal disorders.
- The observed facial patterns in CF children were similar to those seen in children with nasal respiratory obstruction.
Conclusions:
- Children with cystic fibrosis exhibit characteristic craniofacial morphology differences, including open bite and altered facial dimensions.
- Respiratory insufficiency in CF patients is associated with more significant facial morphological changes.
- The findings suggest a potential link between CF-related respiratory compromise and craniofacial development, similar to other conditions causing nasal obstruction.
Abstract:
Cystic fibrosis (CF) is a hereditary metabolic disorder with clinical symptoms of abnormal mucus production. This blocks the airways, gives pancreatic insufficiency, and increases sweat electrolytes. The progressive respiratory disease often leads to respiratory insufficiency and cor pulmonale. The aim of the present investigation was to examine the facial morphology in children with cystic fibrosis. The sample comprised 11 children with cystic fibrosis, who were divided in two groups, one with gastrointestinal disorders and the other with predominantly respiratory insufficiency. Eleven healthy children with normal occlusions were selected as controls. Lateral skull radiographs obtained in natural head posture were digitized, and linear and angular variables for the different groups calculated and compared statistically. The cystic fibrosis group showed open bite, decreased posterior facial height, increased mandibular and craniocervical inclination. Additionally, within the CF-group, the children with respiratory insufficiency differed more from the controls than the children with gastrointestinal disorders. Despite the small number of subjects, the facial morphology of the CF children showed a similar pattern to that of children with nasal respiratory obstruction due to enlarged adenoids or tonsils.