Racial disparity of Crouzon syndrome in maxilla and mandible

The racial disparity of facial features in craniosynostosis patients is not fully understood. The aim of this study was to explore the difference in maxillary and mandibular morphology and spatial position in Asian and Caucasian Crouzon syndrome patients. Ninety-one computed tomography scans were included (12 Asian Crouzon syndrome patients, 22 Asian controls; 16 Caucasian Crouzon syndrome patients, 41 Caucasian controls) and measured using Materialise software. The maxillary and mandibular volumes of Asian patients were both reduced by 19% (P = 0.102 and P = 0.187), and those of Caucasian patients were reduced by 15% (P = 0.142) and 14% (P = 0.211) when compared to the respective race-specific controls. Maxilla length of Asian patients was reduced by 6.36 mm (14%, P = 0.003), while the reduction in Caucasian patients was 4.88 mm (10%, P = 0.038). ANS was retracted 11.99 mm (P < 0.001) in Asian patients and 11.54 mm (P < 0.001) in Caucasian patients. The ANB angle was narrowed by 13.17° (P < 0.001) in Asian patients compared to Asian controls, and by 7.02° (P < 0.001) in Caucasian patients compared to Caucasian controls. The retrusive midface profiles of Asian and Caucasian Crouzon syndrome look similar; both result from the combined effect of hypoplastic size and backward displacement. However, the insufficiency was found to be more a failure of the anteroposterior maxillary length in Asian patients, and more due to posterior maxillary positioning in Caucasian patients. Therefore, prognathism in Crouzon syndrome patients is more likely caused by displacement rather than elongation of mandibular length in both races. Crouzon syndrome results in the same extent of overall volume deficiency of the maxilla and mandible in these races.     

A longitudinal study of dental arch morphology in children with the syndrome of Crouzon or Apert

The aim of this study was to compare changes in dental arch morphology between patients with Crouzon syndrome or Apert syndrome and controls. Children between 4 and 14 yr of age with Crouzon syndrome (n = 40) or Apert syndrome (n = 28) were compared with non‐syndromic controls (n = 457) in terms of arch widths, depths, and length dimensions. Multilevel statistical modeling techniques were used to evaluate changes over time. Dental arch dimensions were found to be smaller in patients with Crouzon syndrome or Apert syndrome compared with control subjects. Maxillary intercanine width for patients with Apert syndrome were increased, whilst other arch width variables showed no change. Patients with Crouzon syndrome showed increases in maxillary intercanine width, whilst intermolar width showed no change over time. Dental arch dimensions in syndromic patients were thus found to be consistently smaller than in control subjects between 4 and 14 yr of age, implying that patients with Crouzon syndrome and Apert syndrome had a diminished growth potential.     

Orbit,zygoma, and maxilla growth patterns in Crouzon syndrome

The facial malformations of Crouzon syndrome involve the entire cranio-orbito-zygomatic region. The detailed sequence of changes in orbit, zygoma, and maxilla over time, the mutual influence among these three anatomical structures, and their relationship with the cranial base were studied to determine the sequence and timing of deformity. Preoperative CT scans of 36 patients with Crouzon syndrome (mean age 10.84 ± 14.70 years; 14 male, 22 female) and CT scans of 54 control subjects (mean age 8.53 ± 13.22 years; 29 male, 25 female) were divided into five subgroups by age: 0–6 months, 6 months–2 years, 2–6 years, 6–18 years, and 18–62 years. Craniofacial morphometric cephalometrics were analyzed using Materialise software. Crouzon orbit anteroposterior length was shorter before 6 months (P = 0.021) and remained shorter into adulthood (P < 0.001). Globe projection was greater across all age subgroups (P < 0.001), reaching a peak at 6 months to 2 years (P < 0.001). The increased medial orbital width was the most remarkable and persistent secondary deformity (P < 0.001). The zygoma anterior protrusion was retruded before 6 months of age (P < 0.001), but then improved gradually. The width of maxilla was greater by 24% in the Crouzon cohort (P < 0.001), with a difference of 16% before 6 months (P = 0.024), and was developed earlier than the shortened anteroposterior length. Crouzon high and shallow orbital walls are distinctive. Maxillary widening developed before the malformation of sphenoid. The anteroposterior position of zygoma is likely a principal deformity, rather than a reflection of the intrinsic shape of the bone.Level of Evidence: II     

克鲁宗综合征临床常用的手术方法和进展

克鲁宗综合征是一种颅缝早闭所致的常染色体显性遗传性疾病,表现为尖头畸形,面孔异常,如面中部凹陷和眼球突出等。针对此疾病的治疗方法有手术治疗、物理治疗和基因治疗等,其中以手术治疗为主要手段并针对各种不同的并发症治疗。手术治疗只能作为克鲁宗综合征治疗的一种补救措施,未来能否在胚胎发生期通过基因技术检测克鲁宗综合征,通过药物预防和阻断克鲁宗综合征的发生是临床研究的重点。本文就克鲁宗综合征治疗的手术时机和常用的外科治疗方法等研究进展作一综述。     

Midface correction in patients with Crouzon syndrome is Le Fort III distraction osteogenesis with a rigid external distraction device the gold standard?

Introduction

Le Fort III distraction osteogenesis with a rigid external distraction device is a powerful procedure to correct both exorbitism and impaired airways in faciocraniosynostosis. The aim of this study was to investigate treatment effect, perioperative parameters and volumetric outcomes after Le Fort III distraction osteogenesis in patients with Crouzon syndrome in a retrospective study design and to explore potential strengths and weaknesses of this procedure.

Excessive ossification of the bandeau in Crouzon and Apert syndromes

BackgroundThe study aimed at assessing the variations in thickness of the supra-orbital bar in Crouzon (CS) and Apert syndromes (AS) before and after fronto-facial monobloc advancement (FFMBA) using CT-scan data.MethodsAll CS or AS patients who underwent FFMBA between 2008 and 2018 with available clinical and CT-scan data were included. Frontal bone thickness was assessed at five locations of the supra-orbital bar using the Half-Maximum-Height protocol and plotted over the bone surface.ResultsWe included 210 CT-scans from 25 CS to 10 AS patients and 25 controls. Before FFMBA, CS children only had a significantly thicker frontal bone (+0.772 mm ± 0.312, p = 0.017). After FFMBA, a significant increase in frontal bone thickness was reported for CS at 3 months (+0.637 mm ± 0.141, p < 0.001), 6 months (+0.910 mm ± 0.120, p < 0.001) and 12 months (+1.099 mm ± 0.124, p < 0.001) post-operatively as well as in AS at 1 month (+2.069 mm ± 0.441, p < 0.001), 6 months (+1.247 mm ± 0.406, p = 0.003) and 12 months (+2.360 mm ± 0.284, p < 0.001) post-operatively. For both syndromes, age at FFMBA and specific FGFR2 mutations significantly influenced post-operative frontal bone thickness.ConclusionsCraniofacial surgeons should be aware of the potential need for secondary surgery of the supra-orbital after FFMBA in CS and AS. Furthermore, a thicker supra-orbital bar is part of the CS phenotype, illustrating the role of FGFR2 in craniofacial growth.     

Growth patterns of the airway in Crouzon syndrome patients with different types of cranial vault suture synostosis

The severity of obstructive respiratory difficulty varies among affected Crouzon syndrome patients. The aim of this study was to investigate the correlation between the restricted airway volume in Crouzon syndrome and the associated type of cranial vault suture synostosis. Computed tomography scans of 68 unoperated Crouzon syndrome patients and 89 control subjects were subgrouped into four types: type I, bilateral coronal synostosis; type II, sagittal synostosis; type III, pansynostosis; type IV, perpendicular combinations of synostoses. Measurements were made using Mimics software. Of type I Crouzon patients, 42% had a restricted nasal airway (P = 0.002), while the pharyngeal airway volume was not significantly reduced. Type II Crouzon patients grew normal segmental airway volumes. Crouzon patients of type III developed simultaneously reduced nasal and pharyngeal airway volumes in infancy, by 38% (P = 0.034) and 51% (P = 0.014), respectively. However, the nasal airway achieved a normal volume by 2 years of age without any intervention, while the pharyngeal airway remained significantly reduced up to 6 years of age, by 42% (P = 0.013), compared to controls. Type IV Crouzon patients developed a reduced nasal airway volume (32%, P = 0.048) and a non-significant restricted pharyngeal airway (18%, P = 0.325). Airway compromise in Crouzon syndrome is variable when associated with different craniosynostosis fusion patterns. Type II (sagittal synostosis) Crouzon patients grew a normal nasopharyngeal airway volume. Those with types I (bicoronal synostosis) and IV (perpendicular synostoses) had significantly restricted nasal airways and a tendency towards a reduced pharyngeal volume. Type III (pansynostosis) Crouzon infants had the worst restriction of both airways, although there was some improvement with age.     

Racial disparity in stage at diagnosis and survival among adults with oral cancer in the US

OBJECTIVES: To explore distribution of stage at diagnosis and relative survival rates among US adults with oral cavity cancer in relation to race, and over time. METHODS: We obtained 1973-2002 oral cancer incidence data from the Surveillance, Epidemiology, and End Results (SEER) Program, and computed proportions for each oral cavity site by stage at diagnosis, tumor size, and 5-year relative survival rates among Whites and Blacks. RESULTS: A total of 46 855 cases of oral cavity cancer were reported to the SEER registry among adults > or =20 years between 1973 and 2002. African-Americans had a significantly higher proportion of cancer, mainly in the tongue, that had spread to a regional node or to a distant site at diagnosis than Whites: 67% versus 49% of tongue cancers reported from 1973 to 1987 (P < 0.001), and 70% versus 53% of those reported from 1988 to 2002 (P < 0.001). They had a significantly higher proportion of tongue cancer that were >4 cm in diameter at time of diagnosis (59% versus 44%; P < 0.001), and black men in particular experienced lower 5-year relative survival rates than white men, in particular, for tongue cancer (25% versus 43% from 1973 to 1987, and 31% versus 53% from 1988 to 2002). CONCLUSION: There are significant racial disparities with respect to stage at diagnosis and survival among adults with oral cancer reported to the SEER registry from 1973 to 2002. One possible explanation for the lower survival among Blacks may be a difference in access to, and utilization of, healthcare services.     

Racial and ethnic disparity in oral cancer awareness and examination: 2003 New york state BRFSS

OBJECTIVES: This study documents the level of oral cancer awareness and examination among New York State adults and evaluates the determinants of disparities in oral cancer detection. METHODS: The 2003 New York State Behavioral Risk Factor Surveillance System gathered information on current awareness of oral cancer and receipt of oral cancer examination from 5,544 adults. To assess whether the racial/ethnic factor remains important for the awareness, receipt, and source of oral cancer examination after controlling for other socioeconomic and health care access variables, bivariate and multiple logistic regression analyses were conducted using SAS and SUDAAN. RESULTS: Even though a majority of State adults (80.4 percent) had heard about oral cancer, about three-quarters of these adults (74.3 percent) had never heard about an oral cancer test or examination. Only 35 percent of the adults reportedly received an oral cancer examination in their lifetime. Adults with Hispanic origin were less likely to have heard about and received an oral cancer examination. Regarding the source of the examination, some 72 percent of the examinations were conducted by a dental professional; the remaining 28 percent were performed by a physician, nurse, or nurse practitioner. Non-Hispanic Blacks were more likely to have received an oral cancer examination from health care providers other than a dentist or dental hygienist. CONCLUSIONS: These data suggest the need to improve the oral cancer awareness and examination rate in New York State. Routine examination of the mouth by primary care providers as part of a physical examination would provide the best opportunity for improving the low oral cancer examination rates in minority populations.     

Midfacial morphology in adult unoperated complete unilateral cleft lip and palate patients

OBJECTIVE: To examine lateral cephalometric radiographs of adult unoperated cleft lip and palate patients for the purpose of clarifying whether maxillary deficiencies observed in treated cleft patients result from intrinsic defects or surgical intervention early in life. MATERIALS AND METHODS: This retrospective study examined lateral cephalograms of 30 adult patients with nonsyndromic complete unilateral cleft lip and palate (CUCLP). The lateral cephalograms were traced and evaluated for size and position of the cranial base, maxilla, maxillary dentition, mandible, and mandibular dentition as well as for vertical relationships. Comparisons with 30 adult noncleft individuals were made. RESULTS: In unoperated adult cleft lip and palate patients, the cranial base angle was increased with the anterior cranial base reduced in length. The maxilla was found to be normal in size and somewhat prognathic in position. Both the maxillary and mandibular incisors were relatively upright. The mandible was smaller in size and posteriorly positioned. CONCLUSIONS: The potential for normal growth of the maxilla exists in patients with CUCLP. It is likely that disturbances of maxillary growth in surgically operated cleft patients are related primarily to the surgical intervention.     

Racial disparity and prognosis in patients with mouth and oropharynx cancer in Brazil

Background Oral and oropharyngeal cancer (OPC) is an important cause of morbidity and mortality worldwide. Populations in situations of social vulnerability tend to have higher incidences of cancer, a higher proportion of late diagnosis, greater difficulties in accessing health services, and, consequently, worse prognosis. The aim of this study was to evaluate the relationship between race/skin color and OPC prognosis in Brazil.Material and Methods This is a cross-sectional epidemiological study using OPC data from the National Cancer Institute between the years 2000 and 2019. The selected variables were: gender, race/skin color, age, education, smoking and alcohol consumption, stage of the disease and disease status at the end of the 1st treatment.Results 154,214 cases were recorded. Black men, in the 6th decade of life, were the most affected population. Blacks had a lower level of education when compared to non-blacks (p<0.001). Blacks were more exposed to smoking and alcohol consumption (p<0.001). At the time of diagnosis, the black population was at the most advanced stage when compared to non-blacks (p<0.001). At the end of the 1st treatment, more black patients had disease in progression, as well as more black patients died (p<0.001).Conclusions Blacks had a worse prognosis for OPC in Brazil. Despite the limitations, these results are important to elucidate the scenario of health disparities in relation to the race/skin color of the Brazilian population. Key words:Head and neck, oncology, cancer, oral cavity, oropharynx.     

未手术成人腭裂患者颅面形态的X线头影测量分析

目的:应用X线头影测量方法对未接受手术修复的成年腭裂患者(年龄为16～27岁)颅面形态进行测量分析,探讨腭裂对颌骨发肓的影响。方法:用X线头影测量机获取15例(男7例,女8例)未手术成年腭裂患者的头颅定位侧位片,联合采用Downs和Steiner分析法进行定点测量分析,测量结果均值与广西正常成年人群牙颌资料进行比较,以t检验进行统计学分析。结果:未手术修复成人单纯腭裂患者上牙槽座角SNA,上颌体长度ANS-Ptm(FH),下中切牙-下颌平面角L1-MP的均值与正常颌比较均变小,而Po-NB距与正常组比较则变大,其差异均有统计学意义(P<0.05或0.01)。结论:未手术成年腭裂患者颅面畸形主要表现为上颌骨前后向发育不足,垂直向发育未受影响;前颅底和下颌骨发育正常,而下牙槽突代偿性后缩可导致成年腭裂患者的假性颏前突。     

Anterior convex lateral orbital wall: distinctive morphology in Apert syndrome

Bony malformations of the orbit and alterations to the soft tissue in Apert syndrome contribute to ophthalmic dysfunction. Recognised structural malformation of the sphenoid and ethmoid sinuses, together with corresponding deformities in the anterior and middle cranial base, are characteristic. Our aim was to explore the underlying structural components of disfigurement and the consequent development of the orbit in patients with Apert syndrome over time by studying 18 preoperative computed tomographic (CT) scans of affected patients and 36 scans from controls. Cephalometric measurements related to the orbit were collected, and analysed with Materialise software. The patients with Apert syndrome had larger than normal external orbital horizontal angles between the ages of 6 months and 2 years. The inside horizontal angle was narrower at 16.36° before 6 months, and continued to decrease into adulthood. The ethmoid and sphenoid side angles in affected patients consistently increased, starting at 7.93% and 14.68% of the external horizontal angle, respectively, during the first 6 months of age, and becoming 20.55% and 11.69%, respectively, in adulthood. In unaffected patients, both angles were less than 3% of the external horizontal angle overall. The orbital vertical angle also changed synchronously, with increasingly wide lateral orbits and shortened anteroposterior orbits. The anterior protrusion of the lateral orbital wall resulted from superior and posterior rotation of a curved, greater wing of the sphenoid, while the widened median orbital wall was caused by the widened ethmoid sinus. These resulted in bony deformities of the orbit, which predisposed to the visual impairments of Apert syndrome.     

Reliable manifestations of increased intracranial pressure in patients with syndromic craniosynostosis

Purpose

Systematic examination of increased intracranial pressure (ICP) is important during the follow-up period after surgical repair of syndromic craniosynostosis. In these patients, postoperative progress can be unclear due to the involvement of multiple sutures and the high incidence of relapse due to the progressive nature of the disease and to genetic variability. In this study, we investigated the clinical manifestations of increased ICP in syndromic craniosynostosis patients before and after surgery.

成年女性未手术不全腭裂患者颌面部软组织形态学研究

目的：通过对成年后未接受手术治疗的女性不全腭裂患者颌面部软组织形态的测量、分析,研究此类畸形对颌面部软组织发育的影响。方法：收集具有完整临床资料的湖北籍不全腭裂女性患者10例。采用颌面部软组织X线头影测量方法,对软组织侧貌进行测量,参考湖北地区女性正常牙合成人颅颌面软组织结构参数,以t检验进行统计学分析。结果：不全腭裂患者的上、下面高比例基本协调,上唇长度和厚度的均值比正常值略小,下唇厚度和突度的均值比正常值略大,但均无显著性差异。结论：不全腭裂对颌面部软组织发育的影响比较小。     

Enlarged anterior cranial fossa and restricted posterior cranial fossa,the disproportionate growth of basicranium in Crouzon syndrome

Crouzon syndrome patients develop normal intracranial volume and potential restricted posterior cranial fossa volume with growth. This study aims to trace the segmental anterior, middle and posterior cranial fossae volume, and structural morphology in these patients, in order to help discern more focused and individualized surgical treatment plan. Ninety-two preoperative CT scans (Crouzon, n = 36; control, n = 56) were included, and divided into 5 age related subgroups. CT scans were measured using Mimics and 3-matics software. Overall, Crouzon syndrome patients grew to a 27% (p = 0.011) increased anterior cranial fossa volume and a 20% (p = 0.001) decreased posterior cranial fossa volume, with normal middle cranial fossa and entire intracranial volume measurement. The posterior cranial fossa of Crouzon syndrome initially developed significantly reduced volume (19%, p = 0.032), compared to normals, from 6 months of age, and remained reduced thereafter. The 7.63 mm shortening of posterior cranial fossa length contributed most to the shortened entire cranial length (9.30 mm, p = 0.046). Although the entire cranial volume of Crouzon syndrome is normal overall, the segmental anterior, middle and posterior cranial fossae developed disproportionately. The early significant and lifelong restricted posterior cranial fossa addresses the importance of early posterior cranial expansion. Ideally expansion would have vectors in all three dimensions.