Table 2 Characteristics of the study included in the systematic review.
From: Craniofacial morphology in Apert syndrome: a systematic review and meta-analysis
No | References | Country | Study design | participants | Age range (years) | Sex (M/F) | Method | Craniofacial findings |
|---|---|---|---|---|---|---|---|---|
1 | Kobayashi et al.34 | Japan | Cohort | Apert—7 Comparator—12 | Case: 12.3(5)* Comparator: 10.8(2.89)* | Case 4/3 Comparator: 6/6 | Cephalometric analysis | AS patients had significantly more severe maxillary hypoplasia in two dimensions and increased clockwise mandibular rotation |
Cleft of the soft palate, anterior open bite, severe crowding in the maxillary dental arch, and congenitally missing teeth occurred more frequently among AS patients | ||||||||
2 | Lu et al.23 | U.S.A | Case–control | Apert—25 Control—20 | Case: 2 days to 6 years Control: 4 days to 16 years | Apert: 12/13 Control: 9/11 | CT scan with Cephalometric landmark | N-S-BA and N-SO-BA angles of AS were more narrowed compared to normal |
3 | Meazzini et al.35 | Italy | Cohort | Apert—19 Control—38 | 1–12 (age matched) | Case: N/A Control: 20/18 | CT scan | AS showed a significant earlier ossification of all sutures compared to the nonsyndromic group |
Care should be taken when planning any maxillary orthopedics, such as expansion or maxillary protraction, given the high frequency of early fusion of circummaxillary sutures | ||||||||
4 | Morice et al.32 | France | Case–control | Apert—12 Control—12 | 8.9(9.2)* (age matched) | Case—6/6 Control: matched | CT scan with DICOM raw data using 3D Slicer | Open gonial angle, short ramus height, and high and prominent symphysis |
Short ramus height appeared more pronounced in Apert than in Crouzon syndrome | ||||||||
5 | Lu et al.26 | U.S.A | Case–control | Apert—33 Control—54 | 0–62 (age matched) | Case: 18/15 Control: 29/25 | CT scan with Cephalometric analysis | Initially significant shortening of the mandibular width and length, and, subsequently, reduced height |
Apert has less shortening in mandibular height with the more shortened posterior cranial base length | ||||||||
Limited nasopharyngeal and oropharyngeal airway space | ||||||||
6 | Lu et al.24 | U.S.A | Case–control | Apert—18 Control 36 | Case: 4 days to 24 years Control: 5 days to 24 years | Apert: 10/8 Control: 22/14 | CT scan with Cephalometric landmark | The zygoma markedly retruded |
Maxillary anterior posterior dimension was 22% shorter than normal, transverse width of the zygoma increased 39% between 6 months and 2 years of age | ||||||||
7 | Lu et al.25 | U.S.A | Cohort | Apert—18 Control—36 | 0–24 (age matched) | Case:10/8 Control:22/14 | CT scan with Cephalometric analysis | The angulation changes occur earlier in development, than linear distance deformity (largely shortening) in AS patients compared with controls |
The initial facial deformity of AS occurs in maxilla, while the orbit deformity develops later | ||||||||
8 | Forte et al.33 | Brazil | Case–control | Apert—19 Control—17 | 6–13(age matched) | N/A | Cephalometric analysis | Midface retrusion associated with altered sphenoid morphology (widened and retruded pterygoid plates) |
A flatter and wider maxilla, suggesting diminished growth inferiorly and anteriorly | ||||||||
9 | Reitsma et al.30 | Netherlands | Population based Case–control l | Apert—28 Control—451 | Case—3.9–15.1 Control—N/A | Case: 10/18 Control: 225/226 | Panoramic radiographs | Girls with AS had a statistically significant less mature dental maturity compared with controls |
Dental maturation was more delayed than control | ||||||||
10 | Reitsma et al.29 | Netherlands | Population based Case–control | Apert—7 Control—486 | Case: 12–19 years Control: 4–22 | Case—0/7 Control—N/A | Cephalometric analysis | The SNA, ANB, and SN/PP angles were significantly smaller in the syndromic patients, and the LFH ratio was significantly larger than control values |
11 | Reitsma et al.31 | Netherlands | Population based Case–control | Apert—28 Control—457 | 4-14 years (age matched) | Case—12/16 Control—216/241 | CT scan with Cephalometric analysis | Maxillary intercanine width for patients with AS were increased, whilst other arch width variables showed no change |
Dental arch dimensions were found to be consistently smaller with a diminished growth potential | ||||||||
12 | Wink et al.27 | U.S.A | Case–control | Apert—9 Control—9 | Case: 12–17 Control: 1–18 | Case—4/5 Control—5/4 | CT scan with Cephalometric analysis | The mandible deformities in the population with AS are likely to be secondary to maxillary hypoplasia and, possibly, the degree of advancement and end point position from maxillary growth |
13 | Boutros et al.28 | U.S.A | Case–control | Apert—2 Control—60 | 5–15 (age matched) | Case—N/A Control-30/30 | Cephalometric analysis | Significant reduction in bicondylar width compared with normal |
The ramus appears torqued inward, forming a greater angle with the cranial base |
