Crouzon syndrome: A comprehensive review

Kyprianou, Chrystalla; Chatzigianni, Athina

doi:10.2478/bjdm-2018-0001

Cited by 5 publications

(5 citation statements)

References 52 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The prevalence rate of AS has been estimated to be between 1/65,000 new-borns, and that of CS is estimated to be 1/25000 new-borns, without prediction by sex (Munarriz et al, 2020;Munib et al, 2023). AS and CS are associated with advanced paternal age, maternal infections, maternal drug consumption, and cranial in ammatory processes (Fernandes et al, 2016;Kyprianou & Chatzigianni, 2018;Lu et al, 2019;Sakamoto et al, 2021). Fibroblast growth factor (FGFR2) gene-speci c missense pathogenic mutations at chromosomal region 10q25-10q26 cause more than 98% of AS and CS patients (Azoury et al, 2017;Morice et al, 2020).…”

Section: Introductionmentioning

confidence: 99%

FGFR2 gene Related Apert and Crouzon Syndrome Patients with Different Craniofacial Dysmorphisms: A Systematic Review and Meta-analysis

Dhiman,

Panigrahi,

Padhi

et al. 2024

Preprint

View full text Add to dashboard Cite

Background This study aimed to compare the prevalence of craniofacial dysmorphisms, such as maxillary, mandibular, and dental arch dimensions, and cranial suture fusion in Apert and Crouzon syndrome patients from publicly available scientific information and to provide insights to improve the findings of further studies. Over a large-scale interval from January 2000 to January 2023, a comprehensive search was performed on different database platforms: PubMed, Google Scholar, Cochrane, Web of Science, and the Wiley online library. The preferred reporting item for Systematic Review and Meta-Analyses (PRISMA) guidelines were followed to conduct this systematic review. The protocol was submitted to the International Prospective Register of Systematic Reviews (CRC42023395454; https://www.crd.york.ac.uk/prospero/record_email.php) on 11 February 2023. We collected the data from different databases and ranked the publications based on their adherence to the Newcastle‒Ottawa Quality Assessment Scale. The meta-analysis was carried out by calculating the random effects model and pooled mean proportions with 95% confidence intervals (CIs). Results A total of 53 studies were considered worthy, but 39 were excluded due to unusable data formats. The meta-analysis was carried out by calculating the random effects model and pooled mean proportions with 95% confidence intervals (CIs). Patients with Apert syndrome were noted as having AS, and Crouzon syndrome was noted as having CS; different studies were included in the systematic review. A total of seven studies reported outcomes. The I2 index provides a better way of assessing effect size heterogeneity. Forest plots were generated to visualize the heterogeneity of the individual outcomes. Subgroup analyses were performed for each outcome to assess the potential differences in effect sizes. The effect size and heterogeneity of the dental arch were greater in the CS group (I2: 58%, 95% CI=0.01, 0.29; P=0.12) than in the AS group (I2: 52%, 95% CI=0.01, 0.27; P=0.15). Effect size and heterogeneity of the maxilla of AS patients (I2: 91%, 95% CI 0.09; 0.47, P<0.01) and CS patients (I2: 94%, 95% CI 0.07; 0.64, P<0.01). We observed significant heterogeneity in AS and CS patients. Conclusion This review demonstrated the large variation in cephalometric measurements between CS and AS patients. The CS patient had a smaller skull and mandible volume than the AS patient. The CS procedure did not change the maxillary intercanine width or intermolar width, but the maxillary intercanine width increased in patients with AS. With the growth period of the children, the maxillary and mandibular intercanine indices increased in the CS, whereas no change in mandibular or maxillary intercanine indices during the growth period was predicted in the AS. The anterior maxillary region is more affected in AS patients than in HCs but less affected in CS patients. ASs had an anterior crossbite (p<0.001), and CSs had an edge-to-edge bite (p<0.011). CSs tend to have short and flat cranial bases, smaller orbital volumes, and cleft palates.

show abstract

Section: Introductionmentioning

confidence: 99%

FGFR2 gene Related Apert and Crouzon Syndrome Patients with Different Craniofacial Dysmorphisms: A Systematic Review and Meta-analysis

Dhiman,

Panigrahi,

Padhi

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

“…Chewing, speaking, and breathing are severely impaired, and some psychological disorders as well as malnutrition are also aggravated by the problem. Clefts are either isolated or associated with syndromes such as Cleido-Cranial dysplasia, Crouzon syndrome, and Apert syndrome [2], [3], [4].…”

Section: Introductionmentioning

confidence: 99%

Comparing Surgical Advancement Outcomes of Retruded Maxilla in a Group of Egyptian Cleft Lip and Palate Subjects

Mossaad

Abdel-Rahman

Hassan

et al. 2022

Open Access Maced J Med Sci

View full text Add to dashboard Cite

BACKGROUND: Cleft lip and palate (CLP) is one of the most common congenital deformities involving intervention in several sub-specialties. AIM: The present study was conducted to investigate the amount of maxillary advancement obtained by three different methods. METHODS: A retrospective comparative study was conducted on 24 CLP patients who were treated with three surgical maxillary advancement techniques: Group A was treated with Le Fort I (LFI) orthognathic surgery with bone grafting and rigid fixation (LFI). Group B was treated with intraoral maxillary bone distraction (MIDO). Group C was treated with orthodontic traction by facemask (orthodontic facemasks [OFM]) plus corticotomy. All pre-operative data were collected, which included intraoral and extraoral clinical photos and dental casts. Pre-operative radiographic assessment was compared with post-operative values using digital panorama, multi-slice computed tomography and lateral cephalometric X-ray measuring Sella-nasion-A point; point A-nasion-point B points, with a follow-up period of 6 months. RESULTS: All approaches showed statistically significant success in maxillary advancement with p < 0.01. LFI has produced the highest advancement obtained with regard to the pre-operative advancement required (8.6 ± 1.4) and post-operative advancement achieved (7.8 ± 0.8). MIDO technique is an alternative method to LFI, but it gives less achieved post-operative maxillary advancement (6.25 ± 0.8) and is indicated for moderate cases. OFM gave the least advancement results; however, it has been the most convenient less-invasive method and was more suitable for unsevere cases. CONCLUSIONS: The three approaches produced satisfactory results in rehabilitating deficient maxilla in cleft patients, although each technique has limitations and indications. Future research is recommended to assess the technique’s long-term stability.

show abstract

“…[5][6][7][8][9] There is no doubt that surgeries and orthodontic procedures can significantly improve the deformity in almost all cases. 10 However, sometimes the surgical complications can be fatal despite strict vigilance about perioperative safety and anesthetic management. 11 Separation of the pterygoid junction is found to be technically difficult because of the important arteries surrounding the bones.…”

mentioning

confidence: 99%

“…There is no doubt that surgeries and orthodontic procedures can significantly improve the deformity in almost all cases 10 . However, sometimes the surgical complications can be fatal despite strict vigilance about perioperative safety and anesthetic management 11 …”

mentioning

confidence: 99%

Characteristics of the Sphenoid Bone in Crouzon Syndrome

Yang

Gui

Teng

et al. 2021

Journal of Craniofacial Surgery

View full text Add to dashboard Cite

Background: The goal of this study is to analyze the safety of reconstructive surgeries for Crouzon syndrome, and to understand the deformities and complications related to the surgical procedure. Methods: Thirty-nine subjects underwent preoperative computed tomographic scans were included (Crouzon, n ¼ 19; controls, n ¼ 20) in this study. Craniofacial cephalometric measurements were analyzed by Materialise software. Results: The overall average distance from the pterygoid junction to the coronal plane in the patients with Crouzon syndrome was 21.34 mm (standard deviation [SD] 5.13), which was deeper than that in the controls by 35% (P ¼ 0.000).The overall average distances between the left and right foramen ovale and pterion on the sphenoid bone in the subjects were 64.93 mm (SD 7.56) and 67.83 mm (SD 8.57), which were increased by 13% (P ¼ 0.001) and 14% (P ¼ 0.001) compared with those in the controls.The overall distances between the most inferior point of the left and right lateral pterygoid plate and the medial pterygoid plate in the subjects were 51.09 mm (SD 6.68) and 51.51 mm (SD 10.98), which was not statically different from the controls (P ¼ 0.887, P ¼ 0.991, respectively). Conclusions: This study characterized the surgically relevant anatomy of the sphenoidal bone. The pterygomaxillary junction is located in the posterior of the skull. Though there are some anatomical differences due to age, it is well known that the cranial cavity of the sphenoid side is likely to be enlarged in Crouzon syndrome, which may contribute to the other complications of the disease.

show abstract

Crouzon syndrome: A comprehensive review

Cited by 5 publications

References 52 publications

FGFR2 gene Related Apert and Crouzon Syndrome Patients with Different Craniofacial Dysmorphisms: A Systematic Review and Meta-analysis

FGFR2 gene Related Apert and Crouzon Syndrome Patients with Different Craniofacial Dysmorphisms: A Systematic Review and Meta-analysis

Comparing Surgical Advancement Outcomes of Retruded Maxilla in a Group of Egyptian Cleft Lip and Palate Subjects

Characteristics of the Sphenoid Bone in Crouzon Syndrome

Contact Info

Product

Resources

About