Facial growth in patients with apert and crouzon syndromes compared to normal children
Jacobus H Reitsma1, Edwin M Ongkosuwito, Peter H Buschang
1Department of Orthodontics, Academic Centre of Dentistry, Amsterdam, The Netherlands. j.reitsma@acta.nl
Insights
Children with Apert and Crouzon syndromes exhibit distinct facial growth patterns compared to controls. Syndromic patients show worsened midfacial underdevelopment and mandibular rotation.
Area of Science:
- Craniofacial development
- Genetics
- Orthodontics
Background:
- Apert and Crouzon syndromes are rare genetic disorders affecting craniofacial development.
- Understanding their specific growth patterns is crucial for timely intervention.
Purpose of the Study:
- To evaluate and compare vertical and sagittal facial growth in children with Apert and Crouzon syndromes against a nonsyndromic control group.
Main Methods:
- A case-control study involving 62 syndromic patients (Apert and Crouzon) and 482 controls.
- Lateral cephalograms were analyzed for horizontal and vertical cephalometric measurements (SNA, SNB, ANB, NSMe, palatal plane angles, lower facial height ratio).
Main Results:
- Syndromic groups showed no change in SNA angle during growth, with lower SNA in Apert syndrome. ANB angles were smaller in syndromic patients.
- Vertical analysis revealed increased lower facial height ratios in syndromic groups. A counterclockwise rotation of the palatal plane was observed in syndromic patients.
Conclusions:
- Significant differences in sagittal and vertical jaw relationships exist between Apert syndrome, Crouzon syndrome, and control subjects.
- Syndromic patients experience aggravated midfacial hypoplasia and anterior mandibular rotation, impacting facial structure.
Objective:
To evaluate vertical and sagittal facial growth in children with Apert and Crouzon syndromes and compare it to the growth patterns of a nonsyndromic control group.
Design:
Case-control study.
Setting:
Department of Orthodontics, Children's Hospital Erasmus Medical Centre, Sophia, Rotterdam, The Netherlands.
Patients, Participants:
Sixty-two patients (37 patients with Crouzon syndrome and 25 patients with Apert syndrome) born between 1971 and 2001 (age range 3.9 to 32 years) and 482 nonsyndromic children as a control group.
Interventions:
Lateral cephalograms performed prior to any midfacial surgery of 62 patients and 482 nonsyndromic children were traced and horizontal and vertical measurements were digitized.
Main Outcome Measures:
Cephalometric measurements of SNA, SNB, ANB, NSMe, and SN/palatal plane angles and lower facial height ratio.
Results:
Horizontal measurements for the syndromic groups showed no change in SNA angle during growth. SNA angles were lower in patients with Apert syndrome compared to patients with Crouzon syndrome. The syndromic groups showed smaller values for ANB angles compared to the nonsyndromic group. Vertical measurements showed increased lower facial height ratios for the syndromic groups compared to control subjects. There was an increasing counterclockwise rotation of the palatal plane in relation to the anterior cranial base in syndromic patients. NSMe angles among the three groups were not significantly different.
Conclusions:
Based on the growth differences identified, the sagittal and vertical jaw relationships differ in patients with Crouzon syndrome, patients with Apert syndrome, and control subjects. Syndromic patients show aggravation of midfacial underdevelopment and anterior rotation of the mandible.
Related Concept Videos
Assessment of Airway, Skin Color, and Use of Accessory Muscles
Introduction
The initial evaluation of a patient's respiratory system...
Sutures of the Skull
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
Signs of Puberty
Changes in the Appendicular Skeleton with Age
Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
Facial Feedback Hypothesis
Cranial Bones: Lateral View
The temporal bone forms the lower lateral side of the skull. The temporal bone is subdivided into several regions. The flattened upper portion is the squamous portion of the temporal bone. Below this area and projecting anteriorly is the zygomatic process of the temporal bone, which forms the posterior portion of the zygomatic arch. Posteriorly is the mastoid portion of the temporal bone. Projecting...


