Craniofacial Morphology of Children with Williams Syndrome

1993 ◽  
Vol 30 (3) ◽  
pp. 343-349 ◽  
Author(s):  
Eliyahu Mass ◽  
Leon Belostoky

Facial dysmorphology is considered to be a major diagnostic feature in patients with Williams syndrome (WS). The dysmorphology is composed of soft tissue and skeletal components. In this study the skeletal component of the facial dysmorphology was examined by analyzing the lateral cephalometric radiographs of 8 children with WS. Seven anatomic areas were judged by means of the analysis of 48 cephalometric variables. Four skeletal features contributed to the facial appearance of children with WS: (1) the anterior cranial base was short, although the cranial base angle (N-S-Ba) was normal; (2) the angle of the mandibular plane was steep, although total facial height was normal; (3) despite the normal facial height, there was an unusual proportion of upper to lower anterior facial height and posterior to anterior facial height; and (4) the chin button (pogonion to N-B line) was deficient, although the mandible could not be classified as retrognathic. The common skeletal components were not dominant enough to characterize the facial dysmorphology completely.

2022 ◽  
Vol 11 ◽  
pp. 270-278
Author(s):  
Ahmet Karaman ◽  
Esra Genc

Objectives: The purpose of this study was to evaluate the facial soft tissue and craniofacial morphological structures in adolescent obese individuals with different skeletal patterns. Materials and Methods: The study was carried out on 292 adolescents examined under three groups based on their body mass indexes (BMIs) as obese, healthy, and overweight. The subjects were also categorized based on skeletal classes as Class I, Class II, and Class III. Results: The glabella, nasion, labiale inferius, labiomentale, and pogonion values of the female patients were significantly higher in obese group. In the obese and overweight groups, effective midfacial and mandibular length, anterior and posterior facial heights, and SN values of the females were higher than males. In the obese group, the mean effective midfacial and mandibular length (Co-A and Co-Gn), anterior and posterior facial heights (S-Go and N-Me), and anterior cranial base (SN) values were significantly higher than other groups. Conclusion: Soft-tissue thicknesses increase as BMI value increases. Craniofacial morphology reveals significant differences between BMI groups.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Ascensión Vicente ◽  
Luis-Alberto Bravo-González ◽  
Ana López-Romero ◽  
Clara Serna Muñoz ◽  
Julio Sánchez-Meca

AbstractThe aim of this study was to evaluate the craniofacial cephalometric characteristics of individuals with Down syndrome (DS), comparing them with healthy subjects. An electronic search was made in Pubmed, Embase, Lilacs, Scopus, Medline and Web of Science without imposing limitations on publication date or language. Studies were selecting following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement. The PECO acronym was applied as follows: P (population), individuals with DS; E, (exposition) diagnosis of DS; C (comparison), individuals without DS; O (outcomes) craniofacial characteristics based on cephalometric measurements. Independent reviewers performed data extraction and assessed the methodological quality of the articles using the Newcastle–Ottawa Quality-Assessment-scale. Seven case–control studies were included in meta-analysis. Given the variability of the cephalometric measurements used, only those that had been reported in at least three or more works could be included. Anterior cranial base length (SN), posterior cranial base length (SBa), total cranial base length (BaN), effective length of the maxilla (CoA), sagittal relationship between subspinale and supramentale (ANB), anterior facial height (NMe), and posterior facial height (SGo) values were significantly lower in the DS population than among control subjects. No significant differences were found in sagittal position of subspinale relative to cranial base (SNA) and sagittal position of supramentale relative to cranial base (SNB). Summarizing, individuals with DS present a shorter and flatter cranial base than the general population, an upper jaw of reduced sagittal dimension, as well as a tendency toward prognatic profile, with the medium third of the face flattened and a reduced anterior and posterior facial heights.


2017 ◽  
Vol 74 (11) ◽  
pp. 1048-1053
Author(s):  
Vladanka Vukicevic ◽  
Jasna Pavlovic ◽  
Amila Vujacic ◽  
Brankica Martinovic ◽  
Mirjana Kostic ◽  
...  

Background/Aim. Nasal breathing plays an important role in overall physical growth and mental development, as well as in the growth of the craniofacial complex. Oral breathing over a long period of time, can cause changes in position of the head relative to the cervical spine and jaw relationship. It can cause an open bite and the narrowness of the maxillary arch due to increased pressure of strained face. The aim of this study was to analyze the position of the head and craniofacial morphology in oral breathing children, and compare the values obtained compared with those of the same parameters in nasal brething children. Methods. We analyzed the profile cephalometric radiographs of 60 patients who had various orthodontic problems. In the first group there were 30 patients aged 8?14 years, in which oral breathing is confirmed by clinical examination. In the second group there were 30 patients of the same age who had orthodontic problems, but did not show clinical signs of oral breathing. The analyses covered the following: craniocervical angle (NS/OPT), the length of the anterior cranial base (NS), anterior facial height (N-Me), posterior facial height (S-Go), the angle of maxillary prognathism (SNA), angle of mandibular prognathism (SNB), difference between angles SNA and SNB (ANB angle), the angle of the basal planes of the jaws (SpP/MP), cranial base angle (NSB), and the angle of facial convexity (NA/Apg). Results. The average value of the craniocervical angle (NS/OPT) was significantly higher in OB children (p = 0.004). There were significantly different values of SNA (p < 0.001), ANB (p < 0.001), NA/APg (p < 0.001) and length of the anterior cranial base (NS) (p = 0.024) between groups. Conclusion. Oral breathing children have pronounced retroflexion of the head in relation to the cervical spine compared to nasal breathing children, and the most prominent characteristics of the craniofacial morphology of skeletal jaw relationship of class II and increased facial convexity.


Author(s):  
G Geethanjali ◽  
Amarshree A Shetty ◽  
Amitha M Hegde

Williams syndrome is a rare genetic disorder that occurs due to deletion on chromosome 7q11.23. The prevalence is said to be 1:7500-1:20,000. The typical presentation of the syndrome includes dysmorphic facial features, cardiovascular malformations and intellectual disability. Craniofacial features of these children include short anterior cranial base, protrusive maxilla, increased Mandibular Plane- Occlusal angle, steep mandibular plane and reduced ratio of posterior to anterior facial height. The unique characteristics of these children are over friendliness, out-going personality, hyper-acusis and tendency to get easily distracted. This is a unique case of Williams syndrome in an eight-year-old female patient.


1996 ◽  
Vol 33 (1) ◽  
pp. 32-36 ◽  
Author(s):  
Yildiz Öztürk ◽  
Nil Cura

Craniofacial morphology of children with and without clefts has been reported to differ because of a combination of factors including, morphogenetic pattern, adaptive changes, and lip and palate management. The purpose of this study was to make a cephalometric evaluation of possible differences in craniofacial morphology of Turkish children with and without unilateral cleft lip and palate (UCLP). Twenty UCLP children (7 girls, 13 boys) with a mean age of 10.75 years (SD = 2.58 years) were compared with a control group of children without UCLP, who were matched for age (mean = 10.64 years, SD = 2.58 years) and sex. No patient had received orthodontic treatment. Linear and angular variables were measured from tracings of lateral cephalometric radiographs and the resulting data were evaluated statistically. Compared with children in the control group, the children in the cleft palate group demonstrated a greater flattening of the cranial base, a more retrognathic and posteriorly inclined maxilla with decreased length, a larger mandibular plane and gonial angle, larger anterior facial height, and decreased posterior and upper-posterior facial heights.


2018 ◽  
Vol 23 (6) ◽  
pp. 48-55 ◽  
Author(s):  
Waqar Jeelani ◽  
Mubassar Fida ◽  
Attiya Shaikh

ABSTRACT Introduction: Maxillary incisal display is one of the most important attributes of smile esthetics. Objective: The aim of this study was to determine the relationship between maxillary incisal display at rest (MIDR) and various soft tissue, hard tissue and dental components. Methods: A cross-sectional study was conducted on 150 subjects (75 males, 75 females) aged 18-30 years. The MIDR was recorded from the pretreatment orthodontic records. The following parameters were assessed on lateral cephalograms: ANB angle, mandibular plane angle, palatal plane angle, lower anterior and total anterior facial heights, upper incisor inclination, upper anterior dentoalveolar height, and upper lip length, thickness and protrusion. The relationship between MIDR and various skeletal, dental and soft tissue components was assessed using linear regression analyses. Results: The mean MIDR was significantly greater in females than males (p = 0.011). A significant positive correlation was found between MIDR and ANB angle, mandibular plane angle and lower anterior facial height. A significant negative correlation was found between MIDR and upper lip length and thickness. Linear regression analysis showed that upper lip length was the strongest predictor of MIDR, explaining 29.7% of variance in MIDR. A multiple linear regression model based on mandibular plane angle, lower anterior facial height, upper lip length and upper lip thickness explained about 63.4% of variance in MIDR. Conclusions: Incisal display at rest was generally greater in females than males. Multiple factors play a role in determining MIDR, nevertheless upper lip length was found to be the strongest predictor of variations in MIDR.


2021 ◽  
Vol 45 (6) ◽  
pp. 433-440
Author(s):  
Sunock Yun ◽  
Jae Hyun Park ◽  
Na-Young Chang ◽  
Hye Young Seo ◽  
Jae-Hyun Sung ◽  
...  

Objective: The aim of this study was to compare the craniomaxillofacial changes when using high-pull J-hook headgear (HPJH) and mini-implants (MIs) as maxillary anchorage in adolescents. Study Design: 40 female adolescents with dentoalvolar protrusion were divided into 2 groups; the HPJH group (n=20) and the MI group (n=20). Lateral cephalograms taken before treatment (T0) and after anterior tooth retraction (T1) were superimposed on the stable structures and then craniomaxillofacial changes were evaluated. Results: The cranial base angle, SNB, and facial angle decreased in the HPJH group but increased in the MI group. ANB decreased more in the MI group than in the HPJH group. Mandibular plane angle increased in the HPJH group but decreased in the MI group. Facial height index increased in the MI group while it showed no change in the HPJH group. Mandibular true rotation occurred clockwise in the HPJH group and counterclockwise in the MI group. Maxillary central incisors were intruded and retracted more in the MI group than in the HPJH group. Maxillary first molars were extruded in the HPJH group and were intruded in the MI group. Maxillary first molars were protracted more in the HPJH group than in the MI group. Mandibular central incisors were retracted more in the HPJH group than the MI group. Mandibular first molars were extruded more in the MI group than in the HPJH group. Conclusion: More favorable craniomaxillofacial changes occurred in the MI group than in the HPJH group.


2003 ◽  
Vol 50 (3) ◽  
pp. 120-123
Author(s):  
Ivana Scepan ◽  
Branislav Glisic ◽  
Marko Babic ◽  
Zorana Nikolic ◽  
Dusan Djordjevic

The aim of this investigation was to assess the craniofacial morphology of five male individuals with 46,XX chromosomal constitution, and to compare them with normal male individuals (46,XY) and normal female participates (46,XX). The investigation was carried out on the lateral cephalomestic radiographs of five male individuals with sex reverse syndrome (46,XX chromosomal constitution), 62 normal female (46,XX) and 31 normal male (46,XY chromosomal constitution) participants. The following angular variables were measured: SNA, SNB, ANB, SN/SpP, SN/MP and SpP/MP. For each variable the mean value and standard deviation were calculated. The Student t-test was used to compare the result obtained between the group of patients with sex reverse syndrome and group of normal male, as well as of normal female participants. The male individuals with 46, XX chromosomal constitution showed slight bimaxillary retrognathism, and class III sagittal jaw relationship. Due to decreased inclination of the upper jaw to the anterior cranial base, the angle of vertical jaw relationship slightly increased in the group of patients with sex reverse syndrome, compared to normal male and female participants.


1993 ◽  
Vol 30 (4) ◽  
pp. 376-381 ◽  
Author(s):  
Leopoldino Capelozza ◽  
Sheyla Miki Taniguchi ◽  
Omar Gabriel Da Silva

The craniofacial morphology of 26 white unoperated complete unilateral cleft lip and palate patients (13 males, 13 females) was analyzed with cephalometry and compared with a control (normal) group. The results show that in the cleft group, the maxilla is smaller and more protruded, the lower anterior facial height is much larger, and the mandible shows well-defined differences (body, ramus, gonial angle, and mandibular plane angle).


2008 ◽  
Vol 78 (2) ◽  
pp. 262-269 ◽  
Author(s):  
Barbara Obloj ◽  
Piotr Fudalej ◽  
Zofia Dudkiewicz

Abstract Objective: To establish gender-specific normative data for Polish children at the age of 10 years. Materials and Methods: Thirty-nine boys and 34 girls (mean age 10.37, SD = 0.52) of Polish ethnicity were selected based on the following criteria: Class I molar relationship, lack of crossbite or scissor-bite, positive overjet and overbite less than 5 mm, adequate amount of space in both dental arches, no visible asymmetry, and good facial proportions. Lateral cephalograms of each subject were scanned and analyzed with the use of NemoCeph NX2005 software. Descriptive statistics (mean and standard deviation) were calculated for all measured variables. Independent t-tests were performed to assess the intergender differences. The results were compared to the published norms of other white populations. Normative data were presented in the tables. Results: Intergender differences included anterior cranial base length (sella-nasion), total and lower anterior facial height (nasion-menton and ANS-menton, respectively), posterior facial height (sella-gonion), SNA angle, point A to nasion perpendicular distance, pogonion to nasion perpendicular distance, and facial axis. Conclusions: Polish 10-year-old boys had a larger anterior cranial base, and larger total anterior, lower anterior and posterior facial heights than girls. The maxillae and chin protrusion were more pronounced in girls.


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