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1.
Am J Orthod Dentofacial Orthop ; 157(2): 259-268, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32005478

RESUMO

This case report presents the unique treatment of a patient with varying asymmetries at different levels within the face. The patient was a 17-year-old male with a diagnosis of right unilateral coronal synostosis. He had frontal and superior orbital retrusion on the fused side, and bossing of the contralateral side. The middle and lower portions of his face were rotated toward the nonfused side. This unique diagnosis presented particular challenges to surgical and orthodontic correction. The treatment approach, which included a combination of Le Fort I and II osteotomies, bilateral sagittal split osteotomy, and orthodontic treatment with 4 premolar extractions, enabled simultaneous correction of nasal, midfacial, lower facial, and dental asymmetries. The orthodontist and surgeon integrated their efforts to correct dentofacial asymmetry in all 3 planes of space. Facial esthetics and dental function were significantly improved with no appreciable relapse occurring over a 2-year retention period.


Assuntos
Craniossinostoses , Estética Dentária , Assimetria Facial , Osteotomia de Le Fort , Adolescente , Craniossinostoses/complicações , Craniossinostoses/cirurgia , Assimetria Facial/cirurgia , Ossos Faciais , Humanos , Masculino
3.
Plast Reconstr Surg ; 145(2): 493-503, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31985646

RESUMO

BACKGROUND: Secondary fusion of initially patent cranial sutures after primary correction of nonsyndromic craniosynostosis is rarely reported. This study's aim is to report the incidence and analyze whether there are variables that may predispose to such fusion. METHODS: A single-institution, retrospective, case-control study was conducted of all nonsyndromic patients who underwent operative treatment for craniosynostosis from April of 2008 to May of 2017. Patients with less than 1 year of follow-up and/or without a 1-year postoperative computed tomographic scan were excluded. Preoperative, intraoperative, and postoperative variables were analyzed using univariate and multivariate analyses. RESULTS: Sixty-six patients were included in the study, with a mean 2.57-year postoperative follow-up. Six patients (8.8 percent) were found to have secondary craniosynostosis, all of whom had fusion of sutures that were initially patent and refusion of the primary pathologic suture(s). Fifty percent of secondary fusions presented as pansynostosis. On univariate analysis, suturectomy with barrel staving (p < 0.01) was significantly associated with secondary suture fusion. On multivariate analysis, bilambdoid suture involvement (p = 0.03) and suturectomy with barrel staving (p = 0.01) were significantly associated with secondary suture fusion. CONCLUSIONS: Secondary cranial suture fusion may be a relatively common complication after primary craniosynostosis correction. Suturectomy with barrel staving was independently associated with secondary craniosynostosis. Wide surgical separation of the dura from the cranium and osteotomies across patent sutures may predispose to secondary craniosynostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.


Assuntos
Suturas Cranianas/cirurgia , Craniossinostoses/cirurgia , Estudos de Casos e Controles , Pré-Escolar , Craniossinostoses/diagnóstico , Feminino , Humanos , Lactente , Masculino , Recidiva , Reoperação , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Tomografia Computadorizada por Raios X , Resultado do Tratamento
4.
Plast Reconstr Surg ; 145(2): 382e-390e, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31985648

RESUMO

BACKGROUND: The purpose of this study was to determine what craniometric changes occur to both orbits of unicoronal craniosynostosis patients undergoing fronto-orbital advancement and remodeling, and which of these changes are associated with new onset of postoperative strabismus. METHODS: A retrospective analysis was performed of the preoperative and postoperative orbits of 24 unicoronal craniosynostosis patients and the orbits of 24 control subjects, totaling 144 orbits. Eight parameters were evaluated using multivariate logistic regression analysis. One of the parameters was modified orbital index, an indicator of severity of harlequin deformity. RESULTS: Significant differences in orbital dimensions and angles were present bilaterally in unicoronal craniosynostosis orbits when compared to controls. Fronto-orbital advancement and remodeling increased the ipsilateral unicoronal craniosynostosis orbital volume from 13,184 ± 2003 mm to 16,220 ± 2323 mm (p < 0.001). Ipsilateral horizontal cone angles were increased from 48 ± 5 degrees to 54 ± 7 degrees (p = 0.004). Ipsilateral vertical cone angles were decreased from 73 ± 8 degrees to 66 ± 10 degrees (p = 0.003). Ipsilateral modified orbital index improved from 0.83 ± 0.06 to 0.88 ± 0.06 (p = 0.003). Three of the 19 unicoronal craniosynostosis patients developed transient postoperative strabismus. Logistic regression analysis displayed a strong significant association between new-onset strabismus and a change in modified orbital index with a coefficient of 30.84 ± 14.51 (p < 0.05). CONCLUSIONS: The orbital dysmorphology in unicoronal craniosynostosis is bilateral in nature, and it is not wholly treated with conventional fronto-orbital advancement and remodeling. The severity of ipsilateral orbital dysmorphology is correlated with the incidence of postoperative strabismus following conventional fronto-orbital advancement and remodeling. Future research is needed to develop strategies to mitigate the risk of development of strabismus in this group of patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.


Assuntos
Craniossinostoses/complicações , Osso Frontal/cirurgia , Órbita/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Estrabismo/etiologia , Estrabismo/cirurgia , Estudos de Casos e Controles , Feminino , Humanos , Lactente , Modelos Logísticos , Masculino , Órbita/patologia , Estudos Retrospectivos , Estrabismo/patologia
5.
Hum Genet ; 139(4): 461-472, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31980905

RESUMO

SKI pathogenic variations are associated with Shprintzen-Goldberg Syndrome (SGS), a rare systemic connective tissue disorder characterized by craniofacial, skeletal and cardiovascular features. So far, the clinical description, including intellectual disability, has been relatively homogeneous, and the known pathogenic variations were located in two different hotspots of the SKI gene. In the course of diagnosing Marfan syndrome and related disorders, we identified nine sporadic probands (aged 2-47 years) carrying three different likely pathogenic or pathogenic variants in the SKI gene affecting the same amino acid (Thr180). Seven of these molecular events were confirmed de novo. All probands displayed a milder morphological phenotype with a marfanoid habitus that did not initially lead to a clinical diagnosis of SGS. Only three of them had learning disorders, and none had intellectual disability. Six out of nine presented thoracic aortic aneurysm, which led to preventive surgery in the oldest case. This report extends the phenotypic spectrum of variants identified in the SKI gene. We describe a new mutational hotspot associated with a marfanoid syndrome with no intellectual disability. Cardiovascular involvement was confirmed in a significant number of cases, highlighting the importance of accurately diagnosing SGS and ensuring appropriate medical treatment and follow-up.


Assuntos
Aracnodactilia , Craniossinostoses , Proteínas de Ligação a DNA/genética , Proteínas de Ligação a DNA/metabolismo , Síndrome de Marfan , Mutação , Proteínas Proto-Oncogênicas/genética , Proteínas Proto-Oncogênicas/metabolismo , Adolescente , Adulto , Aracnodactilia/diagnóstico , Aracnodactilia/genética , Aracnodactilia/metabolismo , Criança , Pré-Escolar , Craniossinostoses/diagnóstico , Craniossinostoses/genética , Craniossinostoses/metabolismo , Feminino , Humanos , Masculino , Síndrome de Marfan/diagnóstico , Síndrome de Marfan/genética , Síndrome de Marfan/metabolismo , Pessoa de Meia-Idade , Patologia Molecular
6.
Anim Genet ; 51(1): 122-126, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31691328

RESUMO

A GWAS was performed for inborn X-linked facial dysmorphia with severe growth retardation in Labrador Retrievers. This lethal condition was mapped on the X chromosome at 17-21 Mb and supported by eight SNPs in complete LD. Dams of affected male puppies were heterozygous for the significantly associated SNPs and male affected puppies carried the associated alleles hemizygously. In the near vicinity to the associated region, RPS6KA3 was identified as a candidate gene causing facial dysmorphia in humans and mice known as Coffin-Lowry syndrome. Haplotype analysis showed significant association with the phenotypes of all 18 animals under study. This haplotype was validated through normal male progeny from a dam with the not-associated haplotype on both X chromosomes but male affected full-sibs with the associated haplotype.


Assuntos
Craniossinostoses/veterinária , Doenças do Cão/genética , Cães/genética , Genes Letais , Proteínas Quinases S6 Ribossômicas 90-kDa/genética , Animais , Craniossinostoses/genética , Feminino , Estudos de Associação Genética/veterinária , Haplótipos , Masculino , Linhagem , Fenótipo , Polimorfismo de Nucleotídeo Único , Cromossomo X/genética
7.
Anim Genet ; 51(1): 127-131, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31774195

RESUMO

Missense variants are associated with various phenotypic traits and disorders in dogs. The canine P2RX7 gene, coding the ATP-gated P2X7 receptor ion channel, contains four known missense variants. The current study aimed to examine the presence of these variants in a random sample of pedigree and mixed-pedigree dogs. Exons 3, 8, 11 and 13 of the P2RX7 gene, encoding these four respective variants, in 65 dogs were assessed by Sanger sequencing and combined with existing sequencing data from another 69 dogs. The distribution of these variants was then evaluated in all 134 dogs combined and separately within individual breeds including 35 different pure breeds. The rs23314713 (p.Phe103Leu) and rs23315462 (p.Pro452Ser) variants were present in 47 and 40% of all dogs studied respectively, with the rs23314713 variant associated with brachycephalic breeds. Among pedigree dogs, the rs23314713 and rs23315462 variants were associated with brachycephalic and non-brachycephalic breeds respectively. The rs851148233 (p.Arg270Cys) and rs850760787 (p.Arg365Gln) variants were present only in dogs of Cocker Spaniel and Labrador Retriever pedigrees respectively. No other missense variants were found in exons 3, 8, 11 and 13 of the P2RX7 gene within the dogs. In conclusion, the rs23314713 and rs23315462 missense variants of the P2RX7 gene are present in a large proportion of dogs, with the rs23314713 variant associated with a number of brachycephalic breeds. However, the association of this variant with dogs of bulldog ancestry, not brachycephaly per se, cannot be excluded.


Assuntos
Craniossinostoses/veterinária , Doenças do Cão/genética , Cães/genética , Mutação de Sentido Incorreto , Receptores Purinérgicos P2X7/genética , Animais , Cruzamento , Craniossinostoses/genética , Linhagem
8.
Spec Care Dentist ; 40(1): 127-133, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31850547

RESUMO

AIM: The functional and structural complexities accompanying syndromic craniosynostosis make dental care for these patients particularly challenging. We report a case of long-term care for a syndromic craniosynostosis patient. The objective of this report is to introduce special care guidance and clinical recommendation, so that oral health care providers, as key members of a multidisciplinary care team, can provide optimal diagnosis, treatment, and management for the patient with syndromic craniosynostosis. CASE REPORT: The patient of this case report had a medical history of syndromic craniosynostosis involving multiple comorbidities. Over the past 20 years, a multidisciplinary care team has successfully treated the patient. Dental and medical procedures that the patient has received include cranial surgeries, prophylactic dental care, caries control, growth hormone therapy, comprehensive orthodontic treatment in conjunction with orthognathic surgeries, and plastic surgery. CONCLUSION: Oral health care providers can play essential roles in multidisciplinary care for patients with craniosynostosis by understanding the patients' unique oral health conditions and dentofacial deformities. To provide optimal oral health care in a multidisciplinary team, clear communication between the members of the care team is crucial.


Assuntos
Craniossinostoses , Procedimentos Cirúrgicos Reconstrutivos , Humanos , Síndrome
9.
Am J Orthod Dentofacial Orthop ; 156(6): 779-790, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31784011

RESUMO

INTRODUCTION: LeFort III distraction osteogenesis may be indicated in the treatment of syndromic craniosynostosis with severe midface retrusion. This study investigates long-term changes in patients undergoing distraction as children, and compares outcomes to an unaffected, untreated control. METHODS: Fifteen patients (9 males, 6 females) with syndromic craniosynostosis treated by LeFort III distraction at an average age of 4.9 ± 1.5 years were identified. Lateral cephalograms at predistraction, immediate, 1-, 5-, and 10-year postdistraction were superimposed using the best-fit of cranial base details. An untreated, unaffected matched control was obtained from the American Association of Orthodontists Foundation Legacy Collection. Differences in landmark location and cephalometric relationships were assessed between time points and between treatment and control groups. RESULTS: LeFort III distraction produced an average advancement of 14.86 ± 5.14 mm at A-point and 10.54 ± 3.78 mm at orbitale. This advancement produced overcorrection of anteroposterior occlusal relationships and phenotypic correction of midface position. Surgical stability over a 10-year follow-up was excellent. Posttreatment growth was characterized by absent anteroposterior maxillary growth, preservation of dentoalveolar development and maxillary remodeling, and delayed mandibular growth. Subsequent growth resulted in a long-term phenotypic relapse of pretreatment Class III maxillomandibular relationship and negative overjet. CONCLUSIONS: LeFort III distraction osteogenesis produces stable advancement of the midface. Overcorrection is required for long-term phenotypic stability because of deficient postdistraction sagittal midface growth. Late mandibular growth contributes to underestimation of the amount of distraction required to produce long-term phenotypic correction.


Assuntos
Craniossinostoses , Osteogênese por Distração , Osteotomia de Le Fort , Cefalometria , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Mandíbula , Maxila , Resultado do Tratamento
10.
J Craniomaxillofac Surg ; 47(12): 1891-1897, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31812312

RESUMO

INTRODUCTION: Sagittal synostosis leading to scaphocephaly is the most common type of craniostenosis being operated. Different treatment options are known, but the optimal treatment method is still controversial. Head growth indicated by measurements of the head´s circumference and cephalic index (CI) are valid surrogate parameters for normal head shapes in children. The aim of the study was to analyze if osteoclastic craniectomy (OC) in scaphocephaly children at four to ten months of age results in normal head shapes. PATIENTS AND METHODS: Twenty-seven patients with scaphocephaly underwent OC between 2003 and 2011. The mean patient age at the time of surgery was 6.75 months. The body weight was between 6.1 and 9.3 kg, mean 8.0 kg. The average duration of surgery was 108 minutes. The mean blood loss during the procedure was 168 ml and the mean amount of erythrocyte transfusion was 152 ml. The mean time spent on the ICU was 1.48 days and the mean of total hospital stay was 5.81 days. The operative method is described. During the mean follow-up time of 6.3 years (min 3.8, max 10.4, median 7.1) focus was set on the patient´s head growth and cephalic index (CI) following OC. For statistical reason the follow up period was divided into three groups: follow up 2-4 years, 5-7 years and 8-10 years. RESULTS: For all cases the total head growth was 9.5cm (mean) during the follow up period of 6.3 years. Analyzing the mean head growth by bootstrapping analysis, the three observational groups showed a significant increase of the head circumference in all cases being analyzed: group 1 p=0.003, group 2 p=0.005 and group 3 p=0.028 Evaluation of the CI showed a statistically significant change from a pathologic value of 0.67 (mean) preoperatively to a normal value of 0.78 (mean) postoperatively during the follow up analyzing all patients. To precise these findings, the bootstrapping analysis showed in the first period an increase of the mean CI not reaching statistical significance (p=0.351). Analyzing the second and third period the CI significantly increased in both groups (p=0.016 and p=0.037). All patients showed a nearly complete re-ossification during the follow up period. No secondary operation was necessary in any patient of this cohort. CONCLUSION: As shown in this single-center observational study, the surgical intervention significantly improved the cephalic index and resulted in a symmetric head shape with excellent aesthetic appearance. The results were not dependent on postoperative helmet therapy, and compliance of caregivers. Re-ossification reached 100% within the observation period. According to these data, we recommend osteoclastic craniectomy as the method of choice in infants six to twelve months of age.


Assuntos
Craniossinostoses/cirurgia , Craniotomia/métodos , Criança , Pré-Escolar , Suturas Cranianas , Feminino , Cabeça , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento
11.
Head Face Med ; 15(1): 29, 2019 Dec 11.
Artigo em Inglês | MEDLINE | ID: mdl-31829202

RESUMO

BACKGROUND: 22q11.2 deletion syndrome is a medical condition that results from genomic loss at chromosome 22. Affected patients exhibit large variability that ranges from a severe condition to mild symptoms. In addition, the spectrum of clinical features differs among populations and even within family members. The facial features related to this syndrome are not an exception, and although part of its variation arises through development, few studies address this topic in order to understand the intra and inter-population heterogeneities. Here, we analyze the ontogenetic dynamics of facial morphology of Mexican patients with del22q11.2 syndrome. METHODS: Frontal facial photographs of 37 patients (mean age = 7.65 ± 4.21 SE) with del22q11.2DS and 200 control subjects (mean age = 7.69 ± 4.26 SE) were analyzed using geometric morphometric methods. Overall mean shape and size differences between patients and controls were analyzed, as well as differences in ontogenetic trajectories (i.e. development, growth, and allometry). RESULTS: We found that Mexican patients show typical traits that have been reported for the Caucasian population. Additionally, there were significant differences between groups in the facial shape and size when all the ontogenetic stages were considered together and, along ontogeny. The developmental and allometric trajectories of patients and controls were similar, but they differed in allometric scaling. Finally, patients and controls showed different growth trajectories. CONCLUSION: The results suggest that the typical face of patients with del22q11.2DS is established prenatally; nonetheless, the postnatal ontogeny could influence the dysmorphology and its variability through size-related changes.


Assuntos
Craniossinostoses , Síndrome de DiGeorge , Face , Síndrome de Marfan , Variação Biológica da População , Criança , Pré-Escolar , Síndrome de DiGeorge/complicações , Face/anormalidades , Humanos , Fenótipo
12.
Vet J ; 253: 105392, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31685131

RESUMO

Brachycephalic dog breeds are prone to breathing difficulties because of their upper airway anatomy. Several surgical techniques exist to correct anatomical pathologies and common surgical approaches aim to correct functional abnormalities in the nares and/or the soft palate. However, further research is needed to improve clinical outcomes. This study evaluated air pressure and airflow resistance in the upper airways and trachea in nine sedated, sternally recumbent dogs of different skull types (dolichocephalic, n=3; mesocephalic, n=3; brachycephalic, n=3). CT images were acquired from the nostrils to the caudal border of the lungs and geometrical reconstruction of the upper airway and trachea was performed. Analysis of computational fluid dynamics was performed using inspiratory flow adapted to bodyweight for each dog. Flow (L/min) and pressure (cmH2O) were computed for the entire upper airway and trachea. Resistance (cmH2O/L/min) was calculated using pressure differences between the nose, larynx, and trachea. In this pilot study, statistical comparisons were not performed. Pressure maps, airflow, and resistance were similar in dolichocephalic and mesocephalic breeds. Median pressure difference (3.76cmH2O) and resistance (0.154cmH2O/L/min) between the nose and larynx were numerically higher in brachycephalic dogs than in other breeds (0.45cmH2O and 0.016cmH2O/L/min, respectively). Median pressure difference (0.205cmH2O) and resistance (0.009cmH2O/L/min) between the larynx and trachea was numerically similar in all dogs, except for the English bulldog. The methodology used in this preliminary study to quantify airflow characteristics such as pressure and resistance could improve the understanding of brachycephalic obstruction airway syndrome.


Assuntos
Obstrução das Vias Respiratórias/veterinária , Resistência das Vias Respiratórias , Craniossinostoses/veterinária , Doenças do Cão/fisiopatologia , Palato Mole/anormalidades , Obstrução das Vias Respiratórias/fisiopatologia , Animais , Craniossinostoses/fisiopatologia , Cães , Feminino , Masculino , Palato Mole/fisiopatologia , Linhagem
13.
Vet J ; 253: 105391, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31685136

RESUMO

Brachycephalic obstructive airway syndrome (BOAS) is a common disorder presenting enlarged soft palate, stenotic nares and abnormal turbinate growth. Surgical correction of BOAS abnormalities with caudal palatoplasty is an elective therapy. This prospective study aimed to compare the effect of an air plasma device and diode laser in dogs undergoing palatoplasty. Outcome measures were as follows: (1) intra-operative and immediate post-operative complications; (2) evaluation of thermal injury in histological tissue sections of the excised soft palate. Twenty dogs with enlarged soft palates underwent palatoplasty, using an air plasma device (n=10) and diode laser (n=10). Soft palate specimens underwent masked histopathological analysis to assess post-operative thermal injury. In this pilot study, no differences were observed in surgical times; all dogs were discharged 24h after surgery and had stable respiration. In the air plasma group, post-operative bleeding occurred in two cases and revision surgery was performed. No difference in thermal injury was observed using the two devices (P>0.05). The air-plasma device was a viable surgical option for palatoplasty in dogs with BOAS.


Assuntos
Obstrução das Vias Respiratórias/veterinária , Queimaduras/veterinária , Craniossinostoses/veterinária , Doenças do Cão/cirurgia , Lasers Semicondutores/efeitos adversos , Palato Mole/anormalidades , Obstrução das Vias Respiratórias/cirurgia , Animais , Craniossinostoses/cirurgia , Cães , Feminino , Masculino , Palato Mole/patologia , Projetos Piloto , Complicações Pós-Operatórias/veterinária , Estudos Prospectivos , Resultado do Tratamento
14.
J Craniomaxillofac Surg ; 47(11): 1706-1711, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31677988

RESUMO

The treatment of non-syndromic scaphocephaly with spring-activated cranioplasty offers acceptable outcomes with the potential for reduced surgical morbidity when compared with cranial vault remodelling procedures. A disadvantage of this technique is the need for a second operative intervention to remove the implanted devices. There are many descriptions of the surgical technique for performing spring-activated cranioplasty available in the literature; however, little is documented regarding the procedures used for device removal. The published accounts of spring removal demonstrate a wide range of approaches, from the reopening and dissection of the entire previous surgical field, to attempts to limit the incisions and dissection. In this study we describe our technique for the minimally invasive removal of cranial springs used in the treatment of scaphocephaly. Our technique focuses on minimal soft tissue disruption and uses a Kirschner wire cutter to divide the spring at its mid-point so as to relieve any residual internal forces acting on the footplates.


Assuntos
Craniossinostoses/cirurgia , Craniotomia/instrumentação , Craniotomia/métodos , Procedimentos Cirúrgicos Reconstrutivos , Craniossinostoses/diagnóstico , Humanos , Lactente , Crânio/cirurgia , Instrumentos Cirúrgicos
15.
J Craniofac Surg ; 30(8): 2609-2613, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31689735

RESUMO

Ventricular shunting procedures represent the classical surgical treatment for hydrocephalus. Slit ventricle syndrome (SVS) with craniocerebral disproportion (CCD) and secondary craniosynostosis (SCS) is a well-known but uncommon complication following cerebrospinal fluid (CSF) shunting in children. Its general management includes shunt upgrade or revision, placement of anti-siphon devices, lumbo-peritoneal shunting, and endoscopic third ventriculostomy. Cranial expansion is generally considered a major procedure and is indicated when less invasive treatments fail. In these cases, SVS and associated SCS have been usually managed through anterior cranial vault expansion. This procedure aims to decrease the risk of further shunt revisions and to improve neurologic symptoms, but it takes the disadvantage of considerable iatrogenic alterations of the facial proportions. The authors report a case of a 6-year old boy with SVS and SCS, who maintained eumorphic face and was treated by an innovative post-coronal vault expansion ensuring a wide volume increase while avoiding any change of facial conformation.


Assuntos
Face/cirurgia , Crânio/cirurgia , Síndrome do Ventrículo Colabado/cirurgia , Derivações do Líquido Cefalorraquidiano , Craniossinostoses/cirurgia , Estética , Humanos , Lactente , Masculino , Procedimentos Neurocirúrgicos
18.
J Craniofac Surg ; 30(8): 2526-2529, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31609944

RESUMO

While many studies have examined potential risk factors for nonsyndromic craniosynostosis, there have been no publications to date investigating the role of ethnicity in the United States. The current study was undertaken as the first multi-center investigation to examine the relationship between ethnicity and nonsyndromic craniosynostosis, looking at both overall prevalence as well as potential correlation between ethnicity and pattern of affected suture site. A chart review of patients diagnosed with nonsyndromic craniosynostosis treated at four major children's hospitals was performed to obtain ethnicity data. Analysis was preformed based on ethnic group as well as suture site affected. To account for potentialOne regional selection bias, the KID database (1997-2012) was utilized to identify all cases of craniosynostosis on a national level. This data was analyzed against birth rates by ethnicity obtained from CDC WONDER natality database.Amongst the 2112 cases of nonsyndromic craniosynostosis at all institutions, Caucasians and African Americans were consistently the predominant ethnic groups. There was a statistically significant difference in the distribution of affected suture type with African Americans more likely to present with unicoronal synostosis and Caucasians more likely to present with metopic synostosis (P = 0.005). The national data revealed that there were more cases of craniosynostosis in Caucasians and fewer in African Americans than expected when compared to population birth rates. Our findings demonstrate that the Caucasian race is associated with increased rates of synostosis.


Assuntos
Craniossinostoses/epidemiologia , Craniossinostoses/cirurgia , Grupos Étnicos , Grupo com Ancestrais do Continente Europeu , Humanos , Prevalência , Fatores de Risco , Suturas , Estados Unidos/epidemiologia
19.
Plast Reconstr Surg ; 144(4): 932-940, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31568307

RESUMO

BACKGROUND: Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations, with few data on the relative benefit of various surgical interventions. The authors compare the incidence and resolution of Chiari malformations and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) and patients undergoing conventional cranial vault remodeling. METHODS: Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 to 2016 at a single academic hospital, with adequate radiographic assessments, were reviewed. Demographics, interventions, the presence of a Chiari malformation on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher's exact tests were used as appropriate. RESULTS: Forty-nine patients underwent PVDO, and 23 patients underwent cranial vault remodeling during the study period. Median age at surgery (p = 0.880), sex (p = 0.123), and types of syndrome (p = 0.583) were well matched. Patients who underwent PVDO had a decreased incidence of developing Chiari malformations postoperatively compared with the cranial vault remodeling cohort (2.0 percent versus 17.4 percent; p = 0.033). Not surprisingly, no significant difference was found between the groups with regard to the incidence of postoperative hydrocephalus requiring shunt placement (PVDO, 4.1 percent; cranial vault remodeling, 4.3 percent; p = 0.999). CONCLUSIONS: As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a Chiari malformation; however, prospective evaluation is needed to determine causality. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.


Assuntos
Malformação de Arnold-Chiari/etiologia , Craniossinostoses/complicações , Craniossinostoses/cirurgia , Hidrocefalia/etiologia , Osteogênese por Distração/métodos , Crânio/cirurgia , Feminino , Humanos , Lactente , Masculino , Indução de Remissão , Estudos Retrospectivos , Síndrome
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