UNASSIGNED: This study aimed to determine the orbital dimensions of Omani subjects who had been referred for computed tomography (CT) of the brain at Sultan Qaboos University Hospital, Muscat, Oman. Good knowledge of the normal orbital dimensions is clinically essential for successful surgical outcomes. Racial, ethnic and regional variations in the orbital dimensions have been reported.
UNASSIGNED: A total of 273 Omani patients referred for CT scans of the brain were retrospectively evaluated using an electronic medical records database. The orbital dimensions were recorded using both axial and sagittal planes of CT images.
UNASSIGNED: The mean orbital index (OI) was found to be 83.25 ± 4.83 mm and the prevalent orbital type was categorised as the mesoseme. The mean orbital index was 83.34 ± 5.05 mm and 83.16 ± 4.57 mm in males and females, respectively, with their difference not being statistically significant (P = 0.76). However, a statistically significant association was observed between the right and left orbits regarding horizontal distance (P <0.05) and vertical distance (P <0.01) of orbit and OI (P <0.05). No significant difference between the OI and age groups was observed in males and females. The mean interorbital distance and interzygomatic distance were found to be 19.45 ± 1.52 mm and 95.59 ± 4.08 mm, respectively. These parameters were significantly higher in males (P <0.05).
UNASSIGNED: Results of the present study provide reference values of orbital dimensions in Omani subjects. Mesoseme, a hallmark of Caucasian people, is discovered to be the prevalent orbital type of Omani subjects.

The surgical management of hypertelorism is challenging for plastic surgeons, and limited long-term outcome data are available. The purpose of this long-term study was to report a single-surgeon experience with a staged reconstructive protocol for hypertelorism correction.
This retrospective study reviewed the records of patients with hypertelorism who were surgically managed by a single surgeon between 1978 and 2000. Bone (orbital box osteotomy and orthognathic surgery) and soft tissue (rhinoplasty and epicanthoplasty) surgeries were performed based on a patient-specific surgical protocol. Included patients were divided into a childhood group and an adolescence or adulthood group according to their age at orbital repositioning (≤12 and >12 years, respectively). Patients were invited for clinical interviews in February 2020 to evaluate whether requests for revision surgery had been made. The photogrammetric analysis-based hypertelorism index was calculated at preoperative and long-term postoperative times. Satisfaction with the long-term outcome was judged by both surgical professionals and laypeople.
In total, 14 patients with hypertelorism of different etiologies were included, with no request for revision surgery during an average follow-up of 29 years. The preoperative hypertelorism index was higher than the long-term postoperative evaluation (all, P < 0.05) for both childhood and adolescence or adulthood groups. Intergroup comparison revealed no significant difference for the hypertelorism index and panel assessment-based satisfaction with long-term outcome analysis (all, P > 0.05).
Considering the complexity and wide spectrum of clinical presentation of soft tissue and bone deformities in hypertelorism and current outcomes, the surgical approach to these patients should be staged and individualized for achievement of a balanced result between functional (orbital, occlusion, and psychosocial) and aesthetic parameters.

In this case study, we investigate a child presenting with patent ductus arteriosus, short philtrum, duck-bill lips, strabismus, a flat nasal bridge, a broad forehead, low-set ears, hypertelorism, up-slanting palpebral fissures, almond-shaped eyes, and hypodontia, all leading to the clinical diagnosis of Char syndrome. Genetic analysis showed heterozygosity for the novel variant c.851T>C, p. Leu284Ser in the TFAP2B gene. Family analysis suggested that at least 20 members, extending six generations back, were affected. All 10 members available for genetic testing were heterozygous for the novel pathogenic variant. Qualitative analysis of the facial dysmorphology in the proband and three of the affected family members using three-dimensional surface scanning showed that the major deviations were observed in the forehead/eyebrow, nose, upper lip, and chin regions with, for example, a flattened nose and reduced height of the upper lip and the face. Furthermore, it is suggested that Char syndrome is associated with disturbances of tooth formation and eruption.

BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS) is a hereditary autosomal dominant syndrome presenting with a number of signs and symptoms in different population groups.
METHODS:   The investigators implemented a 40-year retrospective analysis of the clinical and radiological features of South Africans affected by NBCCS presenting at the Departments of Oral Surgery, Pathology and Radiology of two major referral hospitals. Details of age, gender, ethnic origin, clinical, and radiological findings were recorded and compared to previous reports. A list of diagnostic criteria for diagnosis of NBCCS in this population was complied. Descriptive statistics were computed, and the P value was set at 0.05 or less.
RESULTS: The sample was composed of 15 patients. The mean age at the time of diagnosis was 22.7years (SD 20.9) with eight (53.3%) patients diagnosed before 20years of age (P=0.0001). The male: female ratio was 2:1. The most frequent major criteria were keratocystic odontogenic tumors (KCOTs) (100%), calcification of falx cerebri (40%), palmo-plantar pits (26.7%), and basal cell carcinomas (BCCs) (20%). The most frequent minor criteria were bifid ribs (20%), skull anomalies (20%), and hypertelorism (20%).
CONCLUSIONS: The results of this study indicate that there was a low frequency of falx cerebri calcifications, BCCs, skull, and rib anomalies in this sample compared to other population groups. These differences could be attributed to genetic, racial, and environmental factors. Future studies are needed to compile diagnostic criteria specific to different population groups.

BACKGROUND: The aim of this study was to identify variables that affect orbital relapse after hypertelorbitism correction.
METHODS: The authors retrospectively reviewed the medical records of patients who underwent hypertelorbitism correction at a single institution between 1975 and 2005. Bony interorbital distance was measured postoperatively and at long-term follow-up. Orbital relapse was defined as the difference between bony interorbital distance measurements at these time points. Patients were stratified into groups based on age at primary surgical correction (early, <8 years; late, ≥ 8 years), the severity of the initial deformity (moderate, bony interorbital distance ≤ 40 mm; severe, bony interorbital distance >40 mm), and the type of surgical technique used (facial bipartition versus box osteotomy). Differences in relapse between the stratified groups were analyzed using paired t tests.
RESULTS: A total of 22 patients met inclusion criteria for this study. Patients who underwent surgery before 8 years of age had a significantly higher degree of orbital relapse compared with older patients (5.9 mm versus 1.8 mm; p = 0.0142). There was no significant difference in orbital relapse based on the severity of the deformity or the operative technique used.
CONCLUSIONS: Surgical correction of hypertelorbitism in patients younger than 8 years leads to a significantly higher rate of bony interorbital distance relapse compared with patients who undergo surgery at an older age. Neither the initial degree of severity nor the type of surgical technique correlates with relapse. The authors therefore recommend that in the absence of urgent factors necessitating early intervention, hypertelorbitism correction should be performed after 8 years of age.

We aimed to investigate if orbital dysmorphisms were more common in patients with long-standing and advanced nasal polyposis. A total of 42 nasal polyposis patients and 55 healthy controls were enrolled in this study. The distances between medial and lateral canthi were measured using a sliding caliper, and distances between medial and lateral orbital walls were evaluated on paranasal sinus CT scans. The results were compared using Student\'s t test and Mann-Whitney U test. The distance between medial canthi was found to be significantly increased in nasal polyposis patients compared to controls (P = 0.004). No difference was observed between patients and controls in terms of distances between lateral canthi, medial orbital walls and lateral orbital walls. Anatomic changes regarding orbital morphology may occur in patients with long-standing and advanced nasal polyposis. Early diagnosis and appropriate treatment is of paramount importance in the management of nasal polyposis, since significant anatomic changes leading to orbital dysmorphisms may occur in advanced and long-standing cases. Studies on larger series must be conducted to unveil the morphologic changes and genetic predisposition in nasal polyposis.

In order to contribute to clinical delineation of midline facial defects with hypertelorism (MFDH) and to etiologic diagnosis of the isolated form, 31 patients with MFDH unaffected by known syndromic associations were evaluated. Group A included patients personally examined by the authors, while Group B included those previously evaluated by other geneticists. Among the 14 patients from Group A, there were 7 with distinct pictures of multiple congenital anomalies. In Group B, 5 of the 17 patients also exhibited a distinct pattern of defects. Among isolated MFDH, there was association with anomalies of the skull and facial bones (13/14), otorhinologic (11/16), central nervous system (9/16), and ocular (6/7), and audiologic (3/16); 1/3 of the cases had a relevant gestational intercurrences. Isolated FNM may have involvement of environmental components in some cases; the possibility of a syndromic picture should be extensive investigated. Follow-up of such patients must include the examinations herein performed.

OBJECTIVE: The goal of this study was to use three-dimensional (3D) analysis to characterize the primary facial deformities in children with unilateral cleft lip and palate (UCLP) and then serially analyze the relationships between facial deformities and maxillofacial growth from infancy to adolescence.
METHODS: Twenty-one Japanese subjects with unilateral cleft lip and alveolus (UCLA) and 20 with UCLP who had been operated on and then followed up for more than 15 years were enrolled in this study.
METHODS: Facial cast models taken at cheiloplasty were scanned with a 3D laser scanner. Lateral cephalographs taken when subjects were 15 years of age or older were traced, and linear and angular measurements were calculated. The correlation between primary facial forms and maxillofacial morphology in adolescence was analyzed.
RESULTS: Three-dimensional analysis showed larger ocular hypertelorism, wider cleft, greater deviation of the columella base, and more severe retruded position of the affected nasal alar base in subjects with UCLP than those with UCLA. Total surface area of the upper lips in subjects with UCLP was significantly smaller than those with UCLA. Correlation analyses revealed that the width of cleft lip, deviation of the columella base, difference of the nose base width, and surface area of the upper lip were statistically correlated with the maxillary length, the anterior position of the maxillary alveolar base, the posterior facial height, and the high angle of the mandible.
CONCLUSIONS: The subjects who had less severe facial deformities and more tissue volume of the upper lips at cheiloplasty showed better maxillofacial growth.

To identify potential risk factors among the therapeutic regimen and life style which may increase the risk for stroke, a pair matched case-control study was conducted in Gaza Strip among 112 patients, who had been hospitalized for acute stroke and history of hypertension, and 224 controls with history of hypertension. Conditional logistic regression models show significant associations between stroke and medication not taking as prescribed (OR = 6.07; 95% CI: 1.53, 24.07), using excessive salt at meals (OR = 4.51; 95% CI: 2.05, 9.90), eating diet high in fat (OR = 4.67; 95% CI: 2.09, 10.40), and high level of stress (OR = 2.77; 95% CI: 1.43, 5.38). No significant association between smoking and the development of stroke (OR = 2.12; 95% CI: 0.82, 5.51) was found. Regular physical exercise was a protective factor (OR=0.26; 95% CI: 0.12, 0.57). Using excessive salt at meals was a significant risk factor (OR = 16.61; 95% CI: 4.40, 62.80) in people having low level of stress, whereas it was not significant in people having high level of stress. (OR = 1.76; 95% CI: 0.58, 5.33). Smoking in combination with low level of stress was a significant risk factor for stroke (OR = 9.88; 95% CI: 2.52, 38.78), but a non-significant protective factor in combination with high level of stress (OR=0.52; 95% CI: 0.14, 1.99). An increase in compliance with the pharmacological and non-pharmacological therapeutic regimen might be a key to a reduction of stroke incidence and prevalence among hypertensive patients.

The objective of the study is to describe the prevalence of a number of condition variables and their interrelations in children with craniofacial anomalies (CFAs). The participants were 217 children with CFAs (125 boys and 92 girls), aged 5 to 16 years. The medical files and brain imaging provided information on most condition variables except for the variable phenotypical expression, on which information was obtained in a rating experiment. Brain anomalies were present in 77 subjects (36%), absent in 79 subjects (36%), and undefined in 61 subjects (28%). Craniosynostosis occurred in 160 children (74%), a syndromic diagnosis in 108 (50%), and clinical hypertelorism in 72 (33%). The mean phenotypical expression score was 4.8 (SD = 2.1), the mean number of hospitalizations was 6.2 (SD = 5.6), and mean age at craniotomy was 10.9 months (SD = 9.3). Many of the condition variables were significantly interrelated. Brain anomalies occur frequently (36%) in syndromic and nonsyndromic forms of CFAs. The most salient condition variables are the presence of brain anomalies, a syndromic diagnosis, clinical hypertelorism, a severe phenotypical expression, and female gender. Individuals presenting with one or more of these condition variables probably form the most severely affected group and require more treatment.