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1.
Cell Biochem Funct ; 42(6): e4106, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39140697

RESUMEN

Myostatin, a member of the transforming growth factor-ß superfamily, is a pivotal regulator of skeletal muscle growth in mammals. Its discovery has sparked significant interest due to its multifaceted roles in various physiological processes and its potential therapeutic implications. This review explores the diverse functions of myostatin in skeletal muscle development, maintenance and pathology. We delve into its regulatory mechanisms, including its interaction with other signalling pathways and its modulation by various factors such as microRNAs and mechanical loading. Furthermore, we discuss the therapeutic strategies aimed at targeting myostatin for the treatment of muscle-related disorders, including cachexia, muscular dystrophy and heart failure. Additionally, we examine the impact of myostatin deficiency on craniofacial morphology and bone development, shedding light on its broader implications beyond muscle biology. Through a comprehensive analysis of the literature, this review underscores the importance of further research into myostatin's intricate roles and therapeutic potential in human health and disease.


Asunto(s)
Músculo Esquelético , Miostatina , Miostatina/metabolismo , Humanos , Músculo Esquelético/metabolismo , Animales , Transducción de Señal , MicroARNs/metabolismo , MicroARNs/genética , Enfermedades Musculares/metabolismo , Enfermedades Musculares/patología , Enfermedades Musculares/tratamiento farmacológico , Desarrollo de Músculos
2.
Sleep Breath ; 28(5): 1889-1897, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39012434

RESUMEN

PURPOSE: Craniofacial morphology is integral to Sleep Breathing Disorders (SBD), particularly Obstructive Sleep Apnea (OSA), informing treatment strategies. This review assesses the utility of two-dimensional (2D) photogrammetry in evaluating these metrics among OSA patients. METHODS: Following PRISMA guidelines, a systematic review was conducted. PubMed, Embase, and Lilacs databases were systematically searched for studies utilizing 2D photography in SBD. Findings were narratively synthesized. RESULTS: Thirteen studies involving 2,328 patients were included. Significant correlations were found between craniofacial measurements-specifically neck parameters and facial width-and OSA severity, even after BMI adjustment. Ethnic disparities in craniofacial morphology were observed, with photogrammetry effective in predicting OSA in Caucasians and Asians, though data for other ethnicities were limited. Pediatric studies suggest the potential of craniofacial measurements as predictors of childhood OSA, with certain caveats. CONCLUSION: 2D photogrammetry emerges as a practical and non-invasive tool correlating with OSA severity across diverse populations. However, further validation in various ethnic cohorts is essential to enhance the generalizability of these findings.


Asunto(s)
Cara , Fotogrametría , Fotograbar , Apnea Obstructiva del Sueño , Humanos , Fotogrametría/métodos , Cara/anatomía & histología , Síndromes de la Apnea del Sueño/diagnóstico
3.
Clin Oral Investig ; 28(7): 409, 2024 Jul 02.
Artículo en Inglés | MEDLINE | ID: mdl-38954126

RESUMEN

OBJECTIVES: Orofacial clefts are complex congenital anomalies that call for comprehensive treatment based on a thorough assessment of the anatomy. This study aims to examine the effect of cleft type on craniofacial morphology using geometric morphometrics. MATERIALS AND METHODS: We evaluated lateral cephalograms of 75 patients with bilateral cleft lip and palate, 63 patients with unilateral cleft lip and palate, and 76 patients with isolated cleft palate. Generalized Procrustes analysis was performed on 16 hard tissue landmark coordinates. Shape variability was studied with principal component analysis. In a risk model approach, the first nine principal components (PC) were used to examine the effect of cleft type. RESULTS: We found statistically significant differences in the mean shape between cleft types. The difference is greatest between bilateral cleft lip and palate and isolated cleft palate (distance of means 0.026, P = 0.0011). Differences between cleft types are most pronounced for PC4 and PC5 (P = 0.0001), which together account for 10% of the total shape variation. PC4 and PC5 show shape differences in the ratio of the upper to the lower face, the posterior mandibular height, and the mandibular angle. CONCLUSIONS: Cleft type has a statistically significant but weak effect on craniofacial morphological variability in patients with non-syndromic orofacial clefts, mainly in the vertical dimension. CLINICAL RELEVANCE: Understanding the effects of clefts on craniofacial morphology is essential to providing patients with treatment tailored to their specific needs. This study contributes to the literature particularly due to our risk model approach in lieu of a prediction model.


Asunto(s)
Puntos Anatómicos de Referencia , Cefalometría , Labio Leporino , Fisura del Paladar , Humanos , Fisura del Paladar/patología , Labio Leporino/patología , Masculino , Femenino , Adolescente , Niño , Análisis de Componente Principal
4.
J Oral Rehabil ; 51(3): 581-592, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37962252

RESUMEN

BACKGROUND: Obstructive sleep apnoea (OSA) is a common sleep disorder characterized by repetitive episodes of upper airway collapse during sleep associated with arousals with or without oxygen desaturation. OBJECTIVE: This study aims to assess and analyse the morphological and neurological factors associated with obstructive sleep apnoea using polysomnography study data and two-dimensional cephalometric analysis of airway and skeletal parameters and their correlation in the patients with varying severities of obstructive sleep apnoea. METHODS: This study included 892 patients who underwent a complete work up, including a thorough history, clinical examination, standard polysomnography study and 2D cephalometric analysis to diagnose obstructive sleep apnoea. This study divided the participants into two groups based on the AHI score from the PSG study: AHI < 15 and AHI > 15 groups. The groups were further divided into male and female groups to study the prevalence of OSA. The analysis involved 13 cephalometric parameters: Seven linear and six angular measurements. The airway parameters measured in this study were minimum posterior airway space (PAS_min), hyoid bone to the mandibular plane (H_MNP) and soft palate length (SPL). All the subjects in this study underwent a standard overnight polysomnography study at the sleep centre in Samsung Medical Center. RESULTS: A total of 892 adult participants (M: F = 727:165, mean age: 50.6 ± 13.2 years and age range: 18-85 years). AHI >15 group was significantly older with higher BMI, NC and WC compared to the AHI < 15 groups in both male and female groups. There was statistical significance observed in N1, N3, AI, ODI, lowest saturation (%) and apnoea max length between the groups (p < .001). The arousal index (AI), especially the respiratory arousal index was considerably higher in the male group. There were significantly higher values in all the PSG parameters in the male group. In the airway parameters, hyoid bone position and soft palate length showed significant differences (p < .001), whereas the PAS did not show any differences (p = .225) between the AHI <15 and AHI >15 groups. The overall skeletal cephalometric parameters showed no significant differences between the groups, whereas the gonial angle and AB to mandibular plane angle showed significant differences in the female group (p = .028, p = .041 respectively). CONCLUSION: The partial correlation of cephalometric parameters with AHI showed a stronger correlation between the H_MNP and AHI in both men and women. The position of the hyoid bone and the soft palate length influences the progression of OSA, especially in male patients. This study found no direct association between the minimum PAS and varying severities of OSA in men and women. We speculate that more than the craniofacial morphological factors such as the sagittal and vertical position of the maxilla and the mandible, the position of the hyoid bone might be more responsible for the severity of OSA.


Asunto(s)
Apnea Obstructiva del Sueño , Adulto , Humanos , Masculino , Femenino , Adolescente , Adulto Joven , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Factores Sexuales , Apnea Obstructiva del Sueño/diagnóstico por imagen , Apnea Obstructiva del Sueño/complicaciones , Cefalometría/métodos , Mandíbula/diagnóstico por imagen , Hueso Hioides
5.
Cleft Palate Craniofac J ; 61(4): 584-591, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36628544

RESUMEN

OBJECTIVE: To characterise the morphology of temporal bone in patients with craniofacial microsomia (CFM). DESIGN: A retrospective study. SETTING: A craniofacial centre. PATIENTS: Ninety-four patients with unilateral craniofacial microsomia. INTERVENTIONS: Mimics 21.0 (Materialise Inc., Belgium) was used to locate temporal bone landmarks on preoperative computed tomography data. The spatial Cartesian coordinate system was established in 3-matic 13.0 (Materialise Inc., Belgium). The coordinates of each landmark and the distances and angles between the landmarks were calculated. A classification system was used to quantify the severity of the zygomatic arch deformity. MAIN OUTCOME MEASURE(S): The bilateral differences in coordinates, linear and angular measurements, and the severity of temporal bone deformity (TTL δ, Psag δ, Paxiδ, and Tsag δ) among the groups were compared using the paired t-test and Kruskal-Wallis test, respectively. RESULTS: Compared to those of the unaffected side, the landmarks of the inner ear and petrous part on the affected side showed a decrease in the Z-coordinate or an increase in the X-coordinate. A superolateral rotation tendency of the temporal bone on the affected side was found. There were no significant differences in the linear and angular measurements between the groups. The degree of zygomatic arch deformation was lower in the mild group; however, no significant difference was found between the moderate and severe groups. CONCLUSIONS: In patients with CFM, asymmetry of the temporal bone and its inner organs (vestibulocochlear organ, facial nerve, and vessels) exists in multiple dimensions; its severity is not completely consistent with the degree of mandibular involvement.


Asunto(s)
Síndrome de Goldenhar , Humanos , Síndrome de Goldenhar/diagnóstico por imagen , Estudios Retrospectivos , Mandíbula , Hueso Temporal/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Asimetría Facial
6.
Cleft Palate Craniofac J ; : 10556656241275964, 2024 Aug 12.
Artículo en Inglés | MEDLINE | ID: mdl-39135453

RESUMEN

OBJECTIVE: In managing sagittal craniosynostosis, strip craniectomy of the affected suture is commonly paired with barrel-stave osteotomies to allow for additional cranial remodeling. However, the effect of these osteotomies is not well-established. This study aimed to evaluate the effect of the length of barrel-stave osteotomies on outcomes in patients with sagittal craniosynostosis. DESIGN: A retrospective review of operative records and pre-operative and one-year post-operative three-dimensional images. SETTING: Tertiary care pediatric institution. PATIENTS: Forty-five patients with sagittal craniosynostosis. INTERVENTIONS: Sagittal strip craniectomy and either long, medium, or short barrel-stave osteotomy lengths followed by helmet therapy. MAIN OUTCOME MEASURES: Operative and three-dimensional craniometric outcomes. RESULTS: Operative time, estimated blood loss, and hospital length of stay were significantly decreased in the short group (P = .003; 0.002; 0.027). The cranial index was normalized in all groups, but the long group was significantly lower (P = .007; 0.025). Head circumference was similar between groups. All indexes were within the normal percentiles in all groups. The medium group had a significantly decreased scaphocephalic index (P = .031; .035). The short group had significantly greater occipital bulleting than the medium group (P = .001). The long group had significantly greater narrowing than the short group (P = .036). CONCLUSIONS: Strip craniectomy with the addition of long, medium, or short barrel staves all resulted in clinically successful outcomes. Our findings suggest that increased barrel-stave osteotomy length may not be necessary for a successful outcome while avoiding more extensive dissection, potential risk, increased operative time, and hospital length of stay.

7.
Cleft Palate Craniofac J ; 61(1): 166-171, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-35918810

RESUMEN

Spontaneous reossification following a cranial defect is described by only a few case reports. A 6-month-old male with epidural hematoma underwent decompressive craniotomy, subsequently complicated by scalp abscess requiring removal of the bone flap. On serial outpatient follow-up, the patient demonstrated near-complete resolution of cranial defect over the course of 18 months, thus deferring the need for future cranioplasty. Prior articles have identified this occurrence in children and young adults; however, the present case is the first to report of this phenomenon in an infant less than 1 year of age. A brief review of the literature is provided with the proposed physiologic underpinning for the spontaneous reossification observed. While prior studies propose that recranialization is mediated by contact with the dura mater and pericranium, new investigations suggest that calvarial bone repair is also mediated by stem cells from the suture mesenchyme.


Asunto(s)
Craniectomía Descompresiva , Procedimientos de Cirugía Plástica , Lactante , Niño , Humanos , Masculino , Craniectomía Descompresiva/efectos adversos , Complicaciones Posoperatorias/epidemiología , Cráneo/diagnóstico por imagen , Cráneo/cirugía
8.
Cleft Palate Craniofac J ; 61(1): 150-154, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-36147021

RESUMEN

Binder's syndrome is a rare congenital deformity characterized by midface hypoplasia, particularly around the nasomaxillary area. Genetic etiology or developmental failure caused by prenatal exposure to teratological agents has been considered. In this article, we present 3 related rhesus monkeys born with orofacial deformities similar to those found in infants with the Binder phenotype. For the first time, a primate biomodel for this condition is presented. The clinical description and association with management and environmental factors are discussed. These findings reinforce the knowledge about the relationship between possible vitamin K metabolism interference and Binder's syndrome.


Asunto(s)
Anomalías Maxilofaciales , Nariz , Humanos , Lactante , Embarazo , Femenino , Animales , Nariz/anomalías , Macaca mulatta , Maxilar/anomalías
9.
Eur J Orthod ; 46(6)2024 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-39364771

RESUMEN

BACKGROUND/OBJECTIVES: It is unclear whether palatal canine impaction is related to genetic or local/environmental factors. If a genetic origin is assumed, then it could be expected that palatal canine impaction is associated with overall craniofacial development. Within this context, the aim of this study was to evaluate the craniofacial morphology of individuals with palatal canine impaction and compare it to a matched group of normal controls. MATERIALS/METHODS: The sample for this investigation comprised 404 individuals (232 females and 172 males). Half of these individuals presented with unilateral or bilateral palatal canine impaction confirmed clinically and radiographically. The other half were matched for sex and age with the first half and comprised individuals without tooth impaction, apart from third molars. The shape of the craniofacial structures was outlined on calibrated cephalometric images through 15 curves and 127 landmarks (11 fixed and 116 semi-landmarks). Shape configurations were superimposed using Procrustes Superimposition and the resulting shape coordinates were reduced into principal components for all subsequent analyses. The effect of palatal canine impaction on craniofacial shape was assessed with regression models, separately in females and males. All statistical tests were performed assuming a type-1 error of 5%. RESULTS: Individuals with palatally impacted canines appear to have a less convex face, a more brachyfacial skeletal pattern, and a sagittally extended premaxilla. In females effect sizes ranged between η2 = 0.136-0.397 (P < 0.05) and in males between η2 = 0.125-0.396 (P < 0.05, apart from the entire craniofacial configuration: P = 0.259). LIMITATIONS: Palatal canine impaction was not confirmed through cone beam computer tomography images in all patients, however, in those cases, the treatment history confirmed the diagnosis. CONCLUSIONS/IMPLICATIONS: Palatal canine impaction is related to a distinct craniofacial shape in females and males. These findings allow for speculation that palatal canine impaction is affected by genetic pathways involved in overall craniofacial development.


Asunto(s)
Cefalometría , Diente Canino , Diente Impactado , Humanos , Masculino , Femenino , Diente Canino/diagnóstico por imagen , Diente Impactado/diagnóstico por imagen , Diente Impactado/patología , Cefalometría/métodos , Adolescente , Adulto Joven , Adulto , Niño , Estudios de Casos y Controles , Puntos Anatómicos de Referencia , Maxilar/diagnóstico por imagen , Cráneo/diagnóstico por imagen , Cráneo/anatomía & histología , Cráneo/patología
10.
Eur J Orthod ; 46(1)2024 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-37932128

RESUMEN

BACKGROUND: Prevalence of adolescent obesity has markedly increased from 5.2% in 1974 to 19.7% in 2021. Understanding the impacts of obesity is important to orthodontists, as growth acceleration and greater pre-pubertal facial dimensions are seen in children with elevated body mass index (BMI). METHODS: To identify whether adolescent obesity shifts the timing and rate of craniofacial growth resulting in larger post-treatment dimensions, we evaluated cephalometric outcomes in overweight/obese (BMI > 85%, n = 168) and normal weight (n = 158) adolescents (N = 326 total). Cephalometric measurements were obtained from pre- and post-treatment records to measure growth rates and final dimensions and were statistically evaluated with repeated measures analysis of variance and linear regression models. RESULTS: Overweight and obese adolescents began and finished treatment with significantly larger, bimaxillary prognathic craniofacial dimensions, with elevated mandibular length [articulare-gnathion (Ar-Gn)], maxillary length [condylion-anterior nasal spine (Co-ANS), posterior nasal spine-ANS (PNS-ANS)], and anterior lower face height (ANS-Me), suggesting overweight children grow more overall. However, there was no difference between weight cohorts in the amount of cephalometric change during treatment, and regression analyses demonstrated no correlation between change in growth during treatment and BMI. BMI percentile was a significant linear predictor (P < 0.05) for cephalometric post-treatment outcomes, including Ar-Gn, Co-ANS, ANS-Me, upper face height percentage (UFH:total FH, inverse relationship), lower face height percentage (LFH:total FH), sella-nasion-A-point (SNA), and SN-B-point (SNB). LIMITATIONS: The study is retrospective. CONCLUSIONS: Growth begins earlier in overweight and obese adolescents and continues at a rate similar to normal-weight children during orthodontic treatment, resulting in larger final skeletal dimensions. Orthodontics could begin earlier in overweight patients to time care with growth, and clinicians can anticipate that overweight/obese patients will finish treatment with proportionally larger, bimaxillary-prognathic craniofacial dimensions.


Asunto(s)
Mandíbula , Obesidad Infantil , Niño , Humanos , Adolescente , Estudios Retrospectivos , Sobrepeso , Índice de Masa Corporal , Maxilar , Cefalometría/métodos
11.
BMC Pediatr ; 23(1): 163, 2023 04 06.
Artículo en Inglés | MEDLINE | ID: mdl-37024864

RESUMEN

BACKGROUND: When analyzing the relationship between adenotonsillar hypertrophy and craniofacial morphology, researchers generally regarded hypertrophied adenoids and tonsils as a whole. It remains unclear whether different enlarged sites of pharyngeal lymphoid tissue would correlate with multiple craniofacial subtypes. We hypothesized there would be craniofacial subtypes correlated with different locations of hypertrophied adenoid and tonsil. METHODS: Lateral cephalometric radiographs were obtained from 466 children (171 boys and 295 girls, aged 12.27 ± 2.69 years). They were divided into four groups according to different sites of enlarged pharyngeal lymphoid tissue: adenoid hypertrophy group (AG, n = 126), tonsillar hypertrophy group (TG, n = 59), adenotonsillar hypertrophy group (ATG, n = 69) and control group (CG, n = 212). Five commonly used angles for cephalometric measurements were investigated: SNA (Sella-Nasion-Point A), SNB (Sella-Nasion-Point B), ANB (Point A-Nasion-Point B), mandibular plane angle (MP/SN) and Y-axis angle (SGn/FH). RESULTS: Children with isolated tonsillar hypertrophy correlated with increased SNA (unstandardized regression coefficient B = 1.38, p = 0.009) and SNB (B = 1.99, p = 0.001) compared with controls. However, children with isolated adenoid hypertrophy correlated with decreased SNB (B=-0.94, p = 0.036), increased ANB (B = 0.74, p = 0.014) and increased MP/SN (B = 2.22, p < 0.001). Similarly, children with adenotonsillar hypertrophy correlated with decreased SNB (B=-1.36, p = 0.015), increased ANB (B = 1.35, p < 0.001) and increased MP/SN (B = 2.64, p = 0.001). CONCLUSIONS: Isolated adenoid hypertrophy correlated with a retrognathic mandible, an increased maxillo-mandibular sagittal discrepancy, and an increased mandibular plane angle. Isolated tonsillar hypertrophy correlated with maxillary and mandibular protrusion. Adenotonsillar hypertrophy did not show a superimposed craniofacial pattern of the above two but showed the same craniofacial pattern as isolated adenoid hypertrophy.


Asunto(s)
Tonsila Faríngea , Tonsila Palatina , Masculino , Niño , Femenino , Humanos , Tonsila Palatina/diagnóstico por imagen , Mandíbula , Hipertrofia , Cefalometría
12.
Clin Oral Investig ; 27(9): 5367-5376, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37468596

RESUMEN

OBJECTIVES: Masticatory function, including masticatory muscle activity and occlusal function, can be affected by craniofacial morphology. This study aimed to investigate the relationship between craniofacial morphology and masticatory function in participants who had completed orthodontic treatment at least two years before and had stable occlusion. MATERIALS AND METHODS: Fourty-two healthy participants were prospectively enrolled and divided into three vertical cephalometric groups according to the mandibular plane angle. Masticatory muscle activity (MMA) in the masseter and anterior temporalis muscles was assessed using surface electromyography. The occlusal contact area (OCA) and occlusal force (OF), defined as occlusal function in this study, were evaluated using occlusal pressure mapping system. Masticatory muscle efficiency (MME) was calculated by dividing MMA by OF. The craniofacial morphology was analyzed using a lateral cephalogram. The masticatory function was compared using one-way analysis of variance. Pearson correlations were used to assess relationships between craniofacial morphology and masticatory function. RESULTS: The hypodivergent group had the lowest MMAand the highest MME in the masseter (167.32 ± 74.92 µV and 0.14 ± 0.06 µV/N, respectively) and anterior temporalis muscles (0.18 ± 0.08 µV/N, p < 0.05). MMA in the masseter showed a positive relationship with mandibular plane angle (r = 0.358), whereas OCA (r = -0.422) and OF (r = -0.383) demonstrated a negative relationship (p < 0.05). The anterior temporalis muscle activity negatively correlated with ramus height (r = -0.364, p < 0.05). CONCLUSIONS: Vertical craniofacial morphology was related to masticatory function. Hypodivergent individuals may have low MMA and high occlusal function, resulting in good masticatory muscle efficiency. CLINICAL RELEVANCE: Hypodivergent individuals require careful consideration in orthodontic diagnosis and prosthetic treatment planning.


Asunto(s)
Músculo Masetero , Músculos Masticadores , Humanos , Estudios Prospectivos , Músculos Masticadores/fisiología , Músculo Masetero/fisiología , Músculo Temporal/fisiología , Electromiografía
13.
Clin Oral Investig ; 27(3): 1063-1070, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36036293

RESUMEN

BACKGROUND AND OBJECTIVE: Agenesis of the maxillary lateral incisor occurs in up to 4% of all individuals and requires multidisciplinary treatment. Its developmental origins, however, are not fully understood. Earlier studies documented genetic factors contributing to agenesis but also an association with craniofacial morphology. In this study, we assessed the association between maxillary morphology and lateral incisor agenesis by a geometric morphometric approach to disentangle the roles of developmental plasticity and genetic factors. MATERIALS AND METHODS: We quantified the maxillary alveolar ridge by 19 two-dimensional landmarks on cross-sectional images of 101 computed tomography scans. We compared the shape and size of the alveolar ridge across patients with unilateral or bilateral agenesis of maxillary lateral incisors and patients with extracted or in situ incisors. RESULTS: The maxillary alveolar ridge was clearly narrower in patients with agenesis or an extracted incisor compared to the control group, whereas the contralateral side of the unilateral agenesis had an intermediate width. Despite massive individual variation, the ventral curvature of the alveolar ridge was, on average, more pronounced in the bilateral agenesis group compared to unilateral agenesis and tooth extraction. CONCLUSIONS: This suggests that pleiotropic genetic and epigenetic factors influence both tooth development and cranial growth, but an inappropriately sized or shaped alveolar process may also inhibit normal formation or development of the tooth bud, thus leading to dental agenesis. CLINICAL RELEVANCE: Our results indicate that bilateral agenesis of the lateral incisor tends to be associated with a higher need of bone augmentation prior to implant placement than unilateral agenesis.


Asunto(s)
Anodoncia , Incisivo , Humanos , Incisivo/anatomía & histología , Anodoncia/genética , Maxilar/anatomía & histología , Proceso Alveolar/anatomía & histología , Tomografía Computarizada por Rayos X
14.
Cleft Palate Craniofac J ; 60(5): 521-525, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-35538850

RESUMEN

The diagnosis of late-presentation sagittal suture craniosynostosis (SCS) can be challenging, especially in the setting of subtle physical exam findings. The clinical significance of clinocephaly-a retro-coronal concavity along the midvault-in this context remains unknown. The aim of this study is to evaluate the predictive value of clinocephaly in identifying late-presentation SCS.A retrospective chart review of all patients >1 year old presenting to the craniofacial clinic with a concern for SCS was performed. The presence or absence of SCS in the setting of clinocephaly was recorded following diagnostic imaging. Student's t test, Chi Square test, and multivariate logistic regression analysis were performed to determine predictors for SCS.75 patients met inclusion criteria. 32 patients (42.7%, 6% female) were diagnosed with SCS. No difference in age between patients with and without SCS was detected. Stratification of patients by age (1-2, 2-4, and >4 years) revealed a higher rate of SCS in younger patients (P = 0.04). The cephalic index (C.I.) of those with sagittal synostosis was significantly smaller but within the normal range, indicating a more scaphocephalic shape (P = 0.003). Logistic regression analysis revealed that C.I. was a strong predictor for SCS (P = 0.003). Of those with SCS, a mix of complete and partial fusion of the sagittal suture was appreciated.This study found that 42.7% of patients with clinocephaly had SCS. C.I. was the only predictor for SCS and unique suture fusion patterns were identified in those with SCS. This study suggests that clinocephaly should be considered a core component of the exam and work-up for SCS. Future studies aimed at evaluating the positive predictive value of this exam finding and identifying risk factors associated with late-presentation SCS are underway.


Asunto(s)
Relevancia Clínica , Craneosinostosis , Lactante , Humanos , Femenino , Preescolar , Masculino , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/cirugía , Suturas Craneales/diagnóstico por imagen , Suturas Craneales/cirugía
15.
Cleft Palate Craniofac J ; : 10556656231184967, 2023 Jun 25.
Artículo en Inglés | MEDLINE | ID: mdl-37437901

RESUMEN

INTRODUCTION: Treacher Collins syndrome is a rare congenital disease characterized by the multiple craniofacial malformations. Although the deformities affecting patients with Treacher Collins syndrome have been well characterized, the effects of these malformations to clinical severity of the syndrome are not well understood. OBJECTIVE: To determine the association of specific Treacher Collins mandibular malformations with clinical severity. DESIGN: A retrospective radiographic observational study. SETTING: Study conducted at a single institution, a quaternary craniofacial care center. PATIENTS: 54 patients with Treacher Collins syndrome. INTERVENTIONS: Computed tomography (CT), clinical photographs and medical history were included in this analysis. Mandibles were isolated from CT data and reconstructed in three dimensions using Mimics software. Cephalometric measurements were performed on CT data. Clinical severity was determined by Teber and Vincent scores. Association of craniofacial dysmorphology to clinical severity was determined by Spearman rank coefficient. MAIN OUTCOME MEASURES: The main results obtained were the measurements of the mandibles and the quantification of the malformations of the evaluated patients. RESULTS: Among the most frequent findings in the sample are hypoplasia of the zygomatic complex, descending palpebral cleft and mandibular hypoplasia. Patients with a lower ramus/corpus ratio had a higher (more severe) Teber and Vincent classification. CONCLUSION: Patients with the most compromised mandible are also the patients with the highest number of malformations, thus, the most severe patients.

16.
Cleft Palate Craniofac J ; : 10556656231176876, 2023 May 17.
Artículo en Inglés | MEDLINE | ID: mdl-37198893

RESUMEN

OBJECTIVE: Unilateral lambdoid synostosis (ULS) is characterized by occipital flattening, mastoid bulging, and contralateral parietal bossing. Anterior craniofacial features are less well-defined. This study utilizes volumetric, craniometric, and composite heat maps of three-dimensional (3D) rendered CT scans to analyze anterior craniofacial asymmetry in ULS and compared to controls. DESIGN: A retrospective review of three-dimensional CT scans. SETTING: Tertiary care pediatric institution. PATIENTS, PARTICIPANTS: 30 ULS and 30 control patients. MAIN OUTCOME MEASURE(S): Volumetric and craniometric analysis of the anterior fossa, orbits, zygomas, maxilla, and mandible was performed. RESULTS: The anterior fossa volume was greater bilaterally (0.047, 0.038), and the fossa angle was more anterior contralaterally (<0.001) and more anterior bilaterally than controls (0.038, 0.033). The orbits had greater height and lesser depth bilaterally compared to controls (0.006, 0.009; < 0.001, < 0.001). Zygoma length was significantly greater on the contralateral side than controls (0.048; < 0.001). Nasal contralateral deviation of 3.57 ± 1.97°. The maxillary length was longer on the contralateral side (0.045). The mandibular angle was more anterior on the ipsilateral side and posterior on the contralateral side (<0.001) compared to controls (0.042, < 0.001). Chin had a contralateral deviation of 1.04 ± 3.74°. CONCLUSIONS: ULS has significant asymmetry in the anterior craniofacial skeleton. There is a bilateral expansion of the anterior cranial fossa with greater frontal bossing on the contralateral side. Increased orbital height and decreased depth. Contralateral zygomatic and mandibular body lengthening with posterior mandibular deviation. These features may provide more effective diagnosis and potential clinical management strategies.

17.
Cleft Palate Craniofac J ; : 10556656231179068, 2023 May 29.
Artículo en Inglés | MEDLINE | ID: mdl-37248557

RESUMEN

OBJECTIVE: This study identifies risk factors for late positional plagiocephaly (PP) diagnosis and impact on helmet therapy. DESIGN: We conducted a retrospective review of all patients diagnosed with PP over 10 years at five Southern California hospitals. SETTING: Patients diagnosed with PP at an included hospital. PATIENTS: 25,332 patients were diagnosed with PP over 10 years. INTERVENTIONS: Patients diagnosed with PP early (< = 6 months) and late (>6 months) were compared. MAIN OUTCOME MEASURES: Cohorts were evaluated for demographics, gestational history, associated conditions, and hospitalizations through direct comparison, logistic regression, and correlation analyses. Rates of referrals and helmet orders were compared. RESULTS: Of patients reviewed, 4.8% (n = 1216) were diagnosed late. On multivariate analysis, late diagnoses were more likely Hispanic or Black/African-American. Early gestational age, hydrocephalus, and VP shunt were more frequent in late diagnoses. Patients diagnosed late had longer NICU and overall hospital stays. Earlier gestational age, longer NICU or overall hospital stay correlated with later age at PP diagnosis. 8.9% of patients were referred for helmet therapy evaluation. Patients diagnosed late were 2.63 and 1.64 times as likely to be referred and require helmet therapy, respectively. CONCLUSIONS: Patients who are Hispanic or Black/African-American, premature, have hydrocephalus, or VP shunt have higher rates of delayed PP diagnosis. Shorter gestational age or longer NICU or hospital stay correlates with later diagnosis, which increases helmet therapy requirements. Additional interventions are needed for at risk patients to routinely evaluate for and minimize the risk of developing PP.

18.
Cleft Palate Craniofac J ; 60(1): 110-114, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-34755550

RESUMEN

Proboscis lateralis (PL) is a rare congenital craniofacial anomaly and it is represented by rudimentary, tube-like nasal structure measuring 2-3 centimetre (cm) length and generally attaches to medial canthal region. A 22-month-old male patient was referred to our clinic with a PL hanging from the right medial canthus and a coloboma on the medial third of the right lower eyelid. Physical examination revealed that the PL was 4 cm long and 1.5 cm in diameter. There was a blind cavity with dimple at its distal segment. The right side of the nose and the nasal airway was aplastic. Before surgical correction, neuroradiological evaluation should be performed to obtain the characteristics and relationship of the lesion with adjacent structures and associated anomalies Many reconstructive options have been described in the literature, however, these options are insufficient to obtain natural contour and define alar crease, especially. We described the laterally-based skin flap from the medial wall of the left heminose and adapted to the lateral edge of the de-epithelialized skin of PL for defining supra-alar crease.


Asunto(s)
Masculino , Humanos , Lactante
19.
Cleft Palate Craniofac J ; 60(9): 1071-1077, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-35437035

RESUMEN

To assess the clinical impacts of prenatal consultation with a multidisciplinary cleft team on infants with cleft lip with or without cleft palate (CL ± P).Retrospective cases series.Tertiary pediatric hospital.Infants with CL ± P whose mothers received prenatal consultation with a pediatric otolaryngology team from June 2005 to December 2019 were identified. A random sample of infants with CL ± P without prenatal consultation from June 2005 to December 2019 was also identified.The primary outcomes were the length of hospitalization during the first 12 weeks of life, timing of surgical repair, length of postsurgical hospitalization, and number of unplanned clinic visits and phone calls for feeding evaluation.Time to cleft lip repair differed significantly between the 2 groups with repair performed at 13.4 (±0.9) weeks for the prenatal consultation group (n = 73) and 15.3 (±2.1) weeks for the control group (n = 80), (P < .05). If hospitalization was required for feeding difficulties during the first 12 weeks of life, length of stay was 4.9 (± 1.7) days for infants with prenatal consultation and 11.5 (± 7.2) days for control infants (P < .05). Unplanned clinic visits with a speech-language pathologist (SLP) for feeding difficulties were needed for 2.7% of prenatal consultation infants and 11.3% of control infants (P < .05).Prenatal consultation regarding CL ± P resulted in infants with decreased duration of early hospitalizations, earlier cleft lip repair, and decreased engagement with the SLP feeding clinic for feeding difficulties when compared with infants without prenatal consultation.


Asunto(s)
Labio Leporino , Fisura del Paladar , Embarazo , Femenino , Lactante , Humanos , Niño , Fisura del Paladar/cirugía , Labio Leporino/cirugía , Estudios Retrospectivos , Madres , Derivación y Consulta
20.
Cleft Palate Craniofac J ; 60(7): 804-809, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35249396

RESUMEN

OBJECTIVE: This multicenter study aimed to compare demographic, operative, and short-term outcomes data between open and minimally invasive surgical approaches for craniosynostosis repair utilizing the American College of Surgeon's National Surgical Quality Improvement Program Pediatric (NSQIP-P) database and highlight surgical disparities among races and ethnicities. DESIGN: Retrospective review of large multicenter database. SETTING: Freestanding general acute care children's hospitals, children's hospitals within a larger hospital, specialty children's hospitals, or general acute care hospitals with a pediatric wing. PATIENTS AND PARTICIPANTS: A total of 4931 pediatric patients underwent craniosynostosis correction within the NSQIP-P database from 2013 to 2019. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): Demographic information included age at surgery, sex, race, and ethnicity. Operative and outcomes measures included operative time, anesthesia time, days until discharge, postoperative complications, blood transfusions, 30-day readmission, and 30-day unplanned return to operating room. RESULTS: Patients who underwent minimally invasive surgery had significantly shorter operative and anesthesia times (p < .001; p < .001), fewer days until discharge (p < .001), fewer postoperative complications (p < .05), and less blood transfusions (p < .001). The proportion of White patients was significantly higher in the minimally invasive surgery group (p < .01), whereas Black and Hispanic patients had a significantly higher proportion in the open surgery group (p < .001; p < .001). Additionally, the percentage of patients undergoing minimally invasive surgery increased from 3.8% in 2014 to over 13% in 2019. CONCLUSIONS: This study adds to a growing consensus that minimally invasive surgery has significantly decreased operative time, anesthesia time, transfusion rates, length of hospital stay, and postoperative complications compared to open surgery. Racial and ethnic surgical disparities showed larger proportions of Black and Hispanic populations undergoing open procedures.


Asunto(s)
Craneosinostosis , Mejoramiento de la Calidad , Humanos , Niño , Estados Unidos , Craneosinostosis/cirugía , Craneosinostosis/complicaciones , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Estudios Retrospectivos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología
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