Craniofacial, oral, and cervical morphological characteristics in Japanese patients with Apert syndrome or Crouzon syndrome

Author(s):  
Yukiho Kobayashi ◽  
Kenji Ogura ◽  
Rina Hikita ◽  
Michiko Tsuji ◽  
Keiji Moriyama

Summary Background and objectives Mutations in the fibroblast growth factor receptor 2 (FGFR2) gene are responsible for both Apert syndrome (AS) and Crouzon syndrome (CS). These diseases share phenotypic characteristics, including midfacial hypoplasia and premature fusion of the calvarial suture(s). Given the extensive range of craniofacial growth and developmental abnormalities, management of these patients requires a multidisciplinary approach. This study aimed to compare craniofacial, oral, and cervical morphological characteristics in Japanese orthodontic patients with AS or CS. Subjects and methods Lateral cephalograms, orthopantomograms, dental casts, medical interview records, facial photographs, and intraoral photographs of 7 AS patients and 12 CS patients on initial visits were used in this study. Cephalometric analyses were performed, and standard scores were calculated based on age- and sex-matched Japanese standard values. Results Cephalometric analysis revealed that AS patients had significantly more severe maxillary hypoplasia in two dimensions and increased clockwise mandibular rotation. Additionally, 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. Multiple fusions between cervical vertebrae C2, C3, C5, and C6 were observed in the AS patients. Limitations Small sample size. Conclusions/implications Our study shows that AS patients have more severe craniofacial and maxillofacial deformities than CS patients.

2012 ◽  
Vol 49 (2) ◽  
pp. 185-193 ◽  
Author(s):  
Jacobus H. Reitsma ◽  
Edwin M. Ongkosuwito ◽  
Peter H. Buschang ◽  
Birte Prahl-Andersen

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.


2016 ◽  
Vol 144 (1-2) ◽  
pp. 15-22
Author(s):  
Milena Trajkovic ◽  
Emira Lazic ◽  
Nenad Nedeljkovic ◽  
Zorana Stamenkovic ◽  
Branislav Glisic

Introduction. Orthodontic diagnosis includes the interpretation of the relations between the craniofacial and cervical system, given the potential impact of the irregularities from one system to another. Objective. The aim of this study was to examine morphological characteristics of the cervical spine, depending on the parameters of the vertical craniofacial growth and gender in adult subjects. Methods. The sample comprised lateral cephalograms of 120 subjects with different vertical facial growth, aged 17.5-35 years. Measured parameters were the following: anterior and posterior vertebral body height (ABHC2-C5, PBHC2-C5), anterior and posterior intervertebral space (AISC2-C5, PISC2-C5), distance between vertebrae and point sella (SC2, SC3, SC4), pterygomaxillare (PmC2), gonion (GoC2) and basion (BaC4); cervical spine angulation (OPT/CVT) and inclination (OPT/HOR, CVT/HOR). Results. Results showed that subjects with anterior facial growth rotation have greater values for BaC4, OPT/HOR, CVT/HOR, OPT/CVT, anterior and posterior vertebral body heights and intervertebral spaces, and lower values for GoC2 and PmC2. Higher values in males were found for anterior and posterior vertebral body heights, distances SC2, SC3, SC4, and BaC4. In females, the greater values were found for GoC2 and ???/?VT. Conclusion. Subjects with anterior facial growth rotation have greater cervical spine inclination and angulation, higher cervical vertebrae and intervertebral spaces, longer upper cervical spines and shorter distances GoC2 and PmC2. Males show smaller cervical column curvature, but higher cervical vertebrae and greater length of the upper cervical spine.


2006 ◽  
Vol 43 (4) ◽  
pp. 499-506 ◽  
Author(s):  
Mariko Takashima ◽  
Noriyuki Kitai ◽  
Shumei Murakami ◽  
Satoshi Takagi ◽  
Ko Hosokawa ◽  
...  

Objective To present orthodontic treatment combined with dual segmental distraction osteogenesis in a patient with Apert syndrome. Patient A 15-year-old boy exhibited severe midfacial hypoplasia with retruded and hypoplastic maxilla and anterior open bite. The patient was treated with a rigid external distraction II system for distraction osteogenesis, a preadjusted edgewise appliance, and a modified maxillary protraction headgear. The concave profile with midfacial hypoplasia was improved. A tight occlusal relationship between maxillary and mandibular teeth was achieved. Postoperative treatment results have been stable for 1 year.


2006 ◽  
Vol 17 (1) ◽  
pp. 68-70 ◽  
Author(s):  
Adriana Sasso Stuani ◽  
Andréa Sasso Stuani ◽  
Maria Bernadete Sasso Stuani ◽  
Maria da Conceição Pereira Saraiva ◽  
Mírian Aiko Nakane Matsumoto

The purpose of this study was to compare the dental pattern of patients with anterior open bite malocclusion to that of individuals with normal overbite by utilization of lateral cephalograms, panoramic radiographs and study casts. The findings showed that there was no significant difference in the inclination of the occlusal plane (SN.PlO) and position of the maxillary and mandibular incisors (1-NA, 1-NB) between both groups of individuals; but the angles of inclination of the maxillary and mandibular incisors (1.1, 1.NA and 1.NB) differed statistically between patients with anterior open bite of the individuals that presented normal overbite, which suggests that the anterior open bite may be of dental origin.


2019 ◽  
Vol 35 (3) ◽  
pp. 501-507
Author(s):  
Ankita Mondal ◽  
Naiara Rodriguez-Florez ◽  
Justine O’Hara ◽  
Juling Ong ◽  
N. u. Owase Jeelani ◽  
...  

2020 ◽  
Vol 3 (11) ◽  
pp. 352-354
Author(s):  
Pooja Gaur

Defined as a rare type I acrocephalosyndactyly syndrome which is clinically characterized by dysmorphic facial features, craniosynostosis, and severe syndactyly of the hands and feet, Apert Syndrome represents an autosomal dominant inheritance which occurs due to the gene mutations in the receptors of the fibroblast growth factor. Oral lesions include tooth crowding, reduction in the size of the maxilla, impacted teeth, anterior open-bite, ectopic eruption, delayed eruption, thick gingiva and supernumerary teeth. The present case report describes a 58 year old female patient reported with the features of Apert’s syndrome such as dysmorphic facial features, occular anomalies, syndactyly and oral features. The case was referred to a specialized centre of clinical care for further treatment.


2012 ◽  
Vol 10 (4) ◽  
pp. 310-314 ◽  
Author(s):  
David F. Jimenez ◽  
Constance M. Barone

Patients with Apert syndrome commonly present with ocular proptosis due to bilateral coronal craniosynostosis and midfacial hypoplasia. Severe proptosis can cause visual compromise and damage, which is most commonly treated with bilateral orbital frontal advancement. The authors present the case of a patient who was treated at 8 weeks of age with endoscope-assisted bilateral coronal craniectomies followed by treatment with a custom-made postoperative cranial orthosis. The patient underwent the procedure without any complications. Over the ensuing months, the patient's proptosis corrected, the forehead and orbital rims advanced without the need for an orbital frontal advancement and craniotomies. This approach may provide an alternative treatment modality for these patients.


2020 ◽  
Vol 19 (4) ◽  
pp. 10-14
Author(s):  
Z. Masna

The individual face shape, the ratio of its proportions, the articulation capabilities of each person to a significant extent depend on the characteristics of occlusion and occlusion, in turn, are determined by the size, shape and ratio of dental, alveolar and basal arches. Two of them - alveolar and basal - pass at the corresponding levels of the alveolar areas of the jaws, which means that it is almost impossible to correct them, therefore they play the role of landmarks for setting the dental arch during dental prosthetics. A full-fledged aesthetic and functional restoration of the maxillofacial region after prosthetics largely depends on the dentist taking into account the individual morphological characteristics of the jaws, their cellular areas and specifically the morphometric parameters of the collar and basal arches. In order to determine the possible variants of the shape of the alveolar and basal arches of the upper jaw and the patterns of their ratio during the routine dental examination, 55 people (27 men and 28 women) aged 21-60 years with preserved maxillary dentition were examined. It has been established that the alveolar and basal arches of the upper jaw can have the shape of five geometric shapes: an oval, a semicircle, an ellipse, a trapezoid or a square. In this case, the shape of the collar and basal arches can coincide or be combined in various combinations. The analysis of the obtained results showed that in men the alveolar arch most often had the shape of an oval (70%), in women - an oval or semicircle (43%, respectively). With the aim of possible variants of the alveolar and basal arches form determination and peculiarities of their correlation 55 individuals (27 men and 28 women) in age of 21-60 years with the preserved teeth row were examined during planned dental examination. All examined were patients of the “Dental clinic of dr. Dakhno” (Kyiv). Computer tomographic investigation was made to these patients according to medical indications, scanning was made parallely to the occlusal plane. Image reconstruction was conducted with the use of highly dimentional bone algorithm. Alveolar and basal arch form of the upper jaw were determined on the images. It was established that alveolar and basal arches of the upper jaw can have a form of five geometrical figures: oval, semicircle, ellipse, trapeze or square. Form of the alveolar and basal arches can match or can combine in different combinations. Received results analysis testified that alveolar arch has a form of the oval in men and oval or semicircle – in women. Basal arch in men also most frequently has a form of oval, less often – semicircle, square and trapeze. In women basal arch more often has a form of trapeze or semicircle, less often – oval or square. In men alveolar and basal arches form matched in 41% of cases, in women – only in 31% of cases among total amount of all examined individuals. Most often alveolar and basal arches matched in the form of oval and semicircle, only in one case arches of the trapeze form matched in man, in woman – square form. Maxillary alveolar and basal arches form variants analysis let to determine areas, where distance between alveolar crests was maximal. Most variable this index was in individuals with the oval form of the alveolar arch on the level of 16-26, 17-27 or 18-28 teeth. Peculiarities of the alveolar and basal arches form of the alveolar process of the upper jaw in male and female individuals are characterized with expressed individual variability. These peculiarities consideration during dental prosthesis let avoid series of complications connected with the pressure redistribution on the osseous tissue of the jaws during articulation. 


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