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1.
Cleft Palate Craniofac J ; : 10556656241257101, 2024 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-38778755

RESUMO

OBJECTIVE: This study aimed to (1) assess layperson preferences for how surgical information is presented; (2) evaluate how the format of visual information relates to layperson comfort with undergoing surgery, perceptions of surgeon character traits, and beliefs about artistic skill impacting plastic surgery practice; and (3) identify sociodemographic characteristics associated with these outcomes. DESIGN: A survey was developed in which one of five standardized sets of information depicting a unilateral cleft lip repair was presented as (1) text alone, (2) quick sketches, (3) simple drawings, (4) detailed illustrations, or (5) photographs. SETTING: Online crowdsourcing platform. PARTICIPANTS: Raters aged 18 years and older from the United States. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): After viewing the surgical information, participants answered three sets of Likert scale questions. Ratings were averaged to produce three composite scores assessing (1) comfort with undergoing surgery (2) perceptions of surgeon character traits, and (3) beliefs about plastic surgery and artistry. RESULTS: Four hundred seventy-nine participants were included. Surgeon character traits score was highest among participants who viewed detailed illustrations at 4.46 ± 0.59, followed by photographs at 4.43 ± 0.54, text alone at 4.28 ± 0.59, simple drawings at 4.17 ± 0.67, and quick sketches at 4.17 ± 0.71 (p = 0.0014). Participants who viewed detailed illustrations rated surgical comfort score and plastic surgery and artistry score highest, although differences did not achieve statistical significance. CONCLUSIONS: Viewing detailed cleft lip repair illustrations was significantly associated with positive perceptions of surgeon character traits. Our data help to contextualize methods of communication and education valued by the public when seeking cleft care.

2.
J Craniofac Surg ; 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38710067

RESUMO

BACKGROUND: This study assesses nasal airway volumes in skeletally mature patients with CLP and healthy controls and examines the relationship among nasal volumes, cleft laterality, and facial asymmetry. METHODS: Computed tomography images from patients with CLP and controls were analyzed using Mimics Version 23.0 (Materialise, Leuven, Belgium). Relationships among nasal airway volume, cleft laterality, and facial asymmetry were compared. RESULTS: The 89 patients in this study included 66 (74%) CLP and 23 (17%) controls. Nasal airway volumes in CLP were more asymmetric than controls (26.8±17.5% vs. 17.2±14.4%; P=0.015). In UCLP, the smaller nasal airway was on the cleft side 81% of the time (P<0.001). Maximum airway stenosis was on the cleft side 79% of the time (P<0.001), and maximum stenosis was on the same side as the smaller airway 89% of the time (P<0.001). There was a mild linear relationship between nasal airway asymmetry and maximum stenosis (r=0.247, P=0.023). On 3-dimensional image reconstruction, the septum often bowed convexly into the cleft-sided nasal airway with a caudal deviation towards the noncleft side. Nasal airway asymmetry was not associated with facial midline asymmetry (P>0.05). CONCLUSION: The nasal airway is more asymmetric in patients with cleft lip and palate compared with the general population, with the area of maximum stenosis usually occurring on the cleft-sided airway. In patients with unilateral cleft lip and palate, the septum often bows into the cleft side, reducing the size of that nasal airway. Nasal airway asymmetry did not correlate with facial asymmetry.

3.
J Craniofac Surg ; 2024 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-38743289

RESUMO

OBJECTIVE: Metopic craniosynostosis (MCS) can be difficult to differentiate from metopic ridge (MR) or normal frontal morphology. This study assess whether the supraorbital notch-nasion-supraorbital notch (SNS) angle can help identify MCS. METHODS: Records of 212 patients with preoperative three-dimensional computed tomography scans were examined. The SNS angles, surgeon craniofacial dysmorphology rankings, and CranioRate metopic severity scores (MSSs) were compared with the Spearman rank correlation coefficient. Receiver operating characteristic (ROC) curves with Youden J-statistic and cross-validation of regression models assessed the ability of these measures to predict surgery. RESULTS: A total of 212 patients were included, consisting of 78 MCS, 37 MR, and 97 controls. Both the mean SNS angle (MCS: 111.7 ± 10.7 degrees, MR: 126.0 ± 8.2 degrees, controls: 130.7 ± 8.8 degrees P < 0.001) and MSS (MCS: 5.9 ± 2.0, MR: 1.4 ± 1.9, controls: 0.2 ± 1.9, P < 0.001) were different among the cohorts. The mean SNS angle (111.5 ± 10.7 versus 129.1 ± 8.8, P < 0.001) was lower in those who had surgery and CranioRate score (5.9 ± 2.1 versus 0.8 ± 2.2, P < 0.001) was higher in those who underwent surgery. SNS angles were positively correlated with surgeon craniofacial dysmorphology rankings (r = 0.41, P < 0.05) and CranioRate MSS (r = 0.54, P < 0.05). The ROC curve requiring high sensitivity revealed an SNS angle of 124.8 degrees predicted surgery with a sensitivity of 88.7% and a specificity of 71.3%. A ROC curve using the CranioRate MCC values ≥3.19 predicted surgery with 88.7% sensitivity and 94.7% specificity. CONCLUSION: Orbital dysmorphology in patients with MCS is well captured by the supraorbital-nasion angle. Both the SNS angle and CranioRate MSS scores accurately predict surgical intervention.

5.
Childs Nerv Syst ; 2024 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-38568218

RESUMO

OBJECTIVE: Patients with normocephalic pansynostosis, who have a grossly normal head shape, are often overlooked early in life and present late with elevated intracranial pressure (ICP) requiring timely cranial vault expansion. This study evaluates the long-term clinical outcomes of patients with normocephalic pansynostosis. METHODS: We retrospectively reviewed patients with a clinical and radiographic diagnosis of primary pansynostosis who underwent vault reconstruction between 2000 and 2023. Clinical and treatment course after craniofacial interventions was followed in patients with normocephaly to assess outcomes. RESULTS: Thirty-five patients with primary pansynostosis were identified, of which eight (23.5%) had normocephaly and underwent initial vault expansion at a mean age of 5.0 ± 2.4 years. All eight patients (50.0% male) presented with symptoms of elevated ICP including headaches (50.0%), nausea and vomiting (50.0%), and developmental delay (62.5%) and/or signs of elevated ICP including papilledema (75.0%) and radiologic thumbprinting on head computed tomography scan (87.5%). Three of the four normocephalic patients who had over 7 years of postoperative follow-up developed subjective headaches, vision changes, or learning and behavioral issues in the long-term despite successful vault reconstruction. CONCLUSIONS: Our longitudinal experience with this rare but insidious entity demonstrates the importance of timely intervention and frequent postoperative monitoring, which are critical to limiting long-term neurological sequelae. Multidisciplinary care by craniofacial surgery, neurosurgery, ophthalmology, and neuropsychology with follow-up into adolescence are recommended to assess for possible recurrence of elevated ICP secondary to cranio-cerebral disproportion.

6.
Ther Innov Regul Sci ; 2024 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-38622455

RESUMO

The classification of medical devices by the Food and Drug Administration (FDA) involves rigorous scrutiny from specialized panels that designate devices as Class I, II, or III depending on their levels of relative risk to patient health. Posterior rigid pedicle screw systems were first classified by the FDA in 1984 and have since revolutionized the treatment of many spine pathologies. Despite this early classification by the FDA, posterior cervical pedicle and lateral mass screws were not reclassified from unclassified to Class III and then to Class II until 2019, nearly 35 years after their initial classification. This reclassification process involved a decades-long interplay between the FDA, formal panels, manufacturers, academic leaders, practicing physicians, and patients. It was delayed by lawsuits and a paucity of data demonstrating the ability to improve outcomes for cervical spinal pathologies. The off-label use of thoracolumbar pedicle screw rigid fixation systems by early adopters assisted manufacturers and professional organizations in providing the necessary data for the reclassification process. This case study highlights the collaboration between physicians and professional organizations in facilitating FDA reclassification and underscores changes to the current classification process that could avoid the prolonged dichotomy between common medical practice and FDA guidelines.

7.
Head Neck ; 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38576311

RESUMO

BACKGROUND: The National Surgical Quality Improvement Program surgical risk calculator (SRC) estimates the risk for postoperative complications. This meta-analysis assesses the efficacy of the SRC in the field of head and neck surgery. METHODS: A systematic review identified studies comparing the SRC's predictions to observed outcomes following head and neck surgeries. Predictive accuracy was assessed using receiver operating characteristic curves (AUCs) and Brier scoring. RESULTS: Nine studies totaling 1774 patients were included. The SRC underpredicted the risk of all outcomes (including any complication [observed (ob) = 35.9%, predicted (pr) = 21.8%] and serious complication [ob = 28.7%, pr = 17.0%]) except mortality (ob = 0.37%, pr = 1.55%). The observed length of stay was more than twice the predicted length (p < 0.02). Discrimination was acceptable for postoperative pneumonia (AUC = 0.778) and urinary tract infection (AUC = 0.782) only. Predictive accuracy was low for all outcomes (Brier scores ≥0.01) and comparable for patients with and without free-flap reconstructions. CONCLUSION: The SRC is an ineffective instrument for predicting outcomes in head and neck surgery.

8.
Ann Surg ; 2024 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-38647147

RESUMO

OBJECTIVE/ SUMMARY BACKGROUND DATA: We propose the first classification scheme for macroglossia in patients with Beckwith-Wiedemann Syndrome (BWS), the BWS Index of macroGlossia (BIG). METHODS: Patients with molecularly confirmed BWS seen from 2004-2023 were included to develop this system. Relationships among BIG scores, tongue reduction surgery, BWS clinical score, percent mosaicism, and polysomnography findings were examined. RESULTS: Patients were classified from BIG0 to BIG3. BIG0 includes those without macroglossia; BIG1 includes those with macroglossia not protruding beyond the teeth/alveolus; BIG2 includes those with tongue protrusion past the teeth/alveolus to the lips but that can be contained within the mouth; and BIG3 includes those with tongues that protrude beyond the teeth/alveolus and lips but that cannot be closed within the mouth. Of the 459 patients with molecularly confirmed BWS, 266 (58.0%) patients were scored. One hundred and eleven (41.7%) were BIG0, 44 (16.5%) were BIG1, 90 (33.8%) were BIG2, and 21 (7.9%) were BIG3. As scores increased, patients had an increased incidence of tongue reduction surgery (BIG0: 0% versus BIG1: 20.5% versus BIG2: 51.1% versus BIG3: 100%; r=0.66, P <0.01). Higher BIG scores were associated with elevated BWS clinical scores (r=0.68, P <0.01) and increased tissue mosaicism (r=0.50, P <0.01) as well as trends towards worse obstructive apnea-hypopnea indices (r=0.29, P =0.02) and lower SpO 2 nadirs (r=-0.29, P =0.02). CONCLUSION: In this large series of patients with Beckwith-Wiedemann Syndrome, increased BIG score correlates with undergoing tongue reduction surgery and increased phenotypic severity. Adoption of the BIG scoring system may facilitate communication and risk stratification across institutions.

9.
Endocrine ; 2024 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-38551756

RESUMO

PURPOSE: Multiple groups have created clinical practice guidelines (CPGs) for the management of primary hyperparathyroidism (PHPT). This report provides a rigorous quality assessment using the Appraisal of Guidelines for Research & Evaluation Instrument (AGREE II) to identify high-performing guidelines and areas for improvement. METHODS: A systematic review was conducted to isolate CPGs addressing the management of PHPT. Guideline data was extracted and quality ratings were assigned by four independent reviewers. Intraclass correlation coefficients (ICC) were calculated to ensure interrater reliability. RESULTS: Twelve guidelines were assessed. The American Association of Endocrine Surgeons (AAES) guideline had the highest mean scaled score across all domains (73.6 ± 31.4%). No other published guideline achieved a "high" quality designation. The highest scoring domain was "clarity of presentation" (mean 60.5 ± 26.5%). The lowest scoring domain was "applicability" (mean 19.8 ± 18.2%). Scoring reliability was excellent, with ICC ≥ 0.89 for all AGREE II 6 domains. CONCLUSION: Although several working groups have developed guidelines to address PHPT management, only those published by the AAES meet all methodologic quality criteria necessary to ensure incorporation of recommendations into clinical practice. Future guidelines would benefit from the development of tools, resources, monitoring criteria that enhance applicability.

10.
Plast Reconstr Surg ; 2024 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-38507550

RESUMO

INTRODUCTION: Most patients with Beckwith-Wiedemann syndrome (BWS) have macroglossia with some requiring tongue reduction surgery (TRS). This study reports correlations between levels of affected cells (mosaicism) and BWS clinical score in patients evaluated for TRS. We also show correlations of clinical score and mosaicism with obstructive sleep apnea (OSA) severity. METHODS: Blood mosaicism levels and BWS clinical score were recorded in patients with macroglossia referred to plastic surgery for evaluation. Associations among blood mosaicism, BWS clinical score, TRS, and OSA were assessed with appropriate statistics. RESULTS: Of the 225 patients included, BWS blood testing was available in 128 (56.9%). Mosaicism levels were higher in those who underwent TRS compared to those who did not (85.9 85.9 (56.5-95.9)% vs. 29.7 (2.8-73.1)%, p<0.001). BWS clinical score was also higher in those requiring TRS (9.0 (8.0-11.0) versus 7.0 (6.0-9.0), p<0.001). There was a positive correlation between clinical score and obstructive apnea-hypopnea index (r=0.320, p=0.011). Receiver operating characteristic curve analysis showed a clinical score ≥11 had 100% specificity and 36.4% sensitivity for detecting patients requiring TRS. Blood mosaicism ≥80% had 63.6% sensitivity and 83.6% specificity for predicting surgery. A combined criteria of BWS clinical score ≥11 or mosaicism ≥80% had 72.7% sensitivity and 83.6% specificity for predicting TRS. CONCLUSION: Blood mosaicism levels and higher BWS clinical scores appear associated with a greater frequency of having surgery in patients with macroglossia referred for surgical evaluation. Elevations in BWS clinical scoring are associated with increased OSA severity while increased blood mosaicism is not.

11.
Plast Reconstr Surg ; 2024 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-38507553

RESUMO

BACKGROUND: This study assesses operative trends over time and outcomes of five osteotomy techniques used to treat the Apert midface. Using clinical and photogrammetric data, we present our institution's selection rationale for correcting specific dysmorphologies of the Apert midface based on the individual phenotype. METHODS: We retrospectively reviewed patients with Apert syndrome who underwent midface distraction from 2000 to 2023. Patients were temporally divided by the year 2012 to assess differences in surgical approach. Postoperative facial dimension changes, surgical and perioperative characteristics, and complications data were compared across techniques. RESULTS: Thirty-nine patients with Apert syndrome underwent 41 midface distraction procedures: 23 (56%) in the early cohort and 18 (44%) in the late cohort. The use of segmental osteotomies for frontofacial advancement increased from 0% before 2012 to 61% from 2012 onwards (p<0.001). Monobloc with bipartition was the only technique that decreased intercanthal distance (p=0.016), and Le Fort II with zygomatic repositioning achieved the greatest median change in bilateral canthal tilt of 8.7° (IQR 1.3°, 8.7°; p=0.068). Monobloc with Le Fort II achieved the greatest median change in facial convexity of -34.9° (IQR -43.3°, -29.2°; p=0.031). Severity of complications, stratified by Clavien-Dindo grade, was greater in transcranial than subcranial procedures but similar between segmental and non-segmental osteotomies (p=0.365). CONCLUSIONS: In studying the Apert midface and attempting to resolve its varying functional and aesthetic issues, we document an evolution towards multi-piece osteotomies over time. With an appreciation for differential midface hypoplasia, segmentation is associated with more effective normalization of the Apert face.

12.
Cleft Palate Craniofac J ; : 10556656241234804, 2024 Feb 29.
Artigo em Inglês | MEDLINE | ID: mdl-38425129

RESUMO

OBJECTIVE: Identify and describe factors associated with retention and attrition of patients during longitudinal follow-up at multidisciplinary cleft clinic. DESIGN: Retrospective cohort study. SETTING: Single, tertiary care center. PATIENTS, PARTICIPANTS: Patients born between 1995 and 2007 with a diagnosis of cleft palate with or without cleft lip attending multidisciplinary cleft clinic. INTERVENTIONS: None tested, observational study. MAIN OUTCOME MEASURE(S): Age at last clinical appointment with a multidisciplinary cleft team provider. Attrition was defined as absence of an outpatient appointment following 15 years of age. RESULTS: Six hundred seventy-eight patients were included. The average age at last appointment across the entire cohort was 13.1 years (IQR 6.6-17.2). Patients who were Black (HR 1.60, 95% CI 1.10-2.32, p = 0.014) and other races (HR 1.90, 95% CI 1.22-2.98, p = 0.004) were more likely to be lost to follow-up compared to white patients. Publicly insured patients were more likely to experience attrition than those who were privately insured (HR 1.30, 95% CI 1.03-1.65, p = 0.030). Estimated income was not significantly associated with length of follow-up (p = 0.259). Those whose residence was in the fourth quartile of driving distance from our center experienced loss to follow-up significantly more than those who lived the closest (HR 2.04, 95% CI 1.50-2.78, p < 0.001). CONCLUSIONS: There is a high degree of follow-up attrition among patients with cleft lip and palate. Race, insurance status, and driving distance to our center were associated with attrition in a large, retrospective cohort of patients who have reached the age of cleft clinic graduation.

13.
Cleft Palate Craniofac J ; : 10556656241236369, 2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38436069

RESUMO

OBJECTIVE: To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs). DESIGN: Cross-sectional retrospective chart review. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period. MAIN OUTCOME MEASURES: CLEFT-Q. RESULTS: Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales. CONCLUSIONS: Patient perception of cleft outcomes is linked to psychosocial factors.

14.
J Craniomaxillofac Surg ; 52(4): 472-476, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38378367

RESUMO

This study compares condylar volumetric asymmetry and facial asymmetry in patients with cleft lip and/or palate (CLP) and controls. The mandibular condyle is important to facial growth, but its role in facial asymmetry for those with CLP has not been described. Condylar volumes and mandibular asymmetry were retrospectively calculated using Mimics Version 23.0 (Materialise, Leuven, Belgium) from patients with CLP undergoing computed tomography (CT) imaging and a cohort of controls. A total of 101 participants, 60 with CLP and 41 controls, had mean condylar volumetric asymmetry of 16.4 ± 17.4 % (CLP) and 6.0 ± 4.0 % (controls) (p = 0.0002). Patients with CLP who had clinically significant chin deviation (>4 mm) had more asymmetric condyles than those without significant chin deviation (p = 0.003). The chin deviated toward the smaller condyle in patients with facial asymmetry more often than in patients without facial asymmetry (81 % vs. 62 %, p = 0.033). While controls had some degree of condylar asymmetry, it tended to be milder and not associated with facial asymmetry. There is a greater degree of condylar volumetric asymmetry in patients with CLP compared to individuals in the general population. Clinically significant facial asymmetry in CLP is associated with a higher degree of condylar asymmetry, with the facial midline deviating toward the smaller condyle.


Assuntos
Fenda Labial , Fissura Palatina , Humanos , Fenda Labial/complicações , Fenda Labial/diagnóstico por imagem , Côndilo Mandibular/diagnóstico por imagem , Côndilo Mandibular/cirurgia , Estudos Retrospectivos , Assimetria Facial/diagnóstico por imagem , Assimetria Facial/complicações , Fissura Palatina/complicações , Fissura Palatina/diagnóstico por imagem , Imageamento Tridimensional
15.
J Craniofac Surg ; 2024 Feb 29.
Artigo em Inglês | MEDLINE | ID: mdl-38421207

RESUMO

INTRODUCTION: This study describes the development and explores the academic impact of a cleft and craniofacial research fellowship. MATERIALS AND METHODS: Research and career outcomes from 3 cleft and craniofacial surgeons, 14 clinical fellows, and 25 research fellows between 2010 and 2023 were examined. Academic productivity was measured by the number of peer-reviewed publications indexed in PubMed and podium presentations at national/international meetings. Residency match statistics were recorded for eligible research fellows. RESULTS: Over this 14-year period (11 with research fellows), the team produced 500 publications in 96 peer-reviewed journals, with 153 (31%) in Plastic and Reconstructive Surgery, 117 (23%) in the Journal of Craniofacial Surgery, and 32 (6%) in The Cleft Palate-Craniofacial Journal. Yearly publications increased from 15.3±7.6 per year (before fellowship) to 23.0±5.3 (with 1 fellow) to 38.3±12.9 (2 fellows) to 81.0±5.7 (3 fellows; P<0.001). There was a strong annual linear growth in publications since the beginning the research fellowship position (r=0.88, P<0.001). All (100%) clinical research fellows developed strong relationships with senior surgeons, and all who applied to plastic surgery residency matched a significantly higher success rate than the national average (P<0.05). CONCLUSION: Implementing a structured cleft and craniofacial clinical research fellowship was associated with a broad impact across all cleft and craniofacial team members, as reflected by increased academic output and high match rates among fellows. The fellowship also strengthens the talent pipeline into plastic surgery by fostering meaningful mentor/mentee relationships and provides a model that can be adopted in both surgical and nonsurgical fields.

16.
Pediatr Surg Int ; 40(1): 59, 2024 Feb 27.
Artigo em Inglês | MEDLINE | ID: mdl-38411786

RESUMO

PURPOSE: Several clinical practice guidelines (CPGs) have been produced to optimize the diagnosis and management of pediatric foreign body aspiration and ingestion. However, to date there have been no critical evaluations of their methodological rigor or quality. Herein, we address this need via the Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument. METHODS: A literature search of Embase, MEDLINE via PubMed, and Scopus was performed up until February 25, 2021. Identified CPGs were then assessed by four independent reviewers trained in AGREE II. A scaled domain score of >60% was indicated as satisfactory quality. Intraclass correlation coefficients (ICC) were calculated to assess inter-reviewer agreement. RESULTS: 11 guidelines were assessed with only one being classified as high quality and others being either average (two) or low quality (eight). Domain 4 (clarity of presentation) achieved the highest mean score (66.41 ± 13.33%), while domain 5 (applicability) achieved the lowest score (10.80 ± 10.37%). ICC analysis revealed generally strong agreement between reviewers with a range of 0.60-0.98. CONCLUSION: Quality appraisal using the AGREE II instrument suggests that the methodologic rigor and quality of current guidelines for the diagnosis and management of pediatric foreign body aspiration and ingestion need significant improvement.


Assuntos
Ingestão de Alimentos , Aspiração Respiratória , Criança , Humanos , Guias de Prática Clínica como Assunto
17.
Plast Reconstr Surg Glob Open ; 12(1): e5558, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38264445

RESUMO

Apert syndrome classically presents with craniosynostosis at birth, most commonly of the bilateral coronal sutures, which may lead to cephalocranial disproportion and elevated intracranial pressure, the latter of which is associated with optic atrophy, visual loss, and developmental delays. A small number of patients with syndromic craniosynostosis demonstrate open sutures at birth; however, all previously reported patients of this subtype have been reported to develop premature suture fusion in the early postnatal period and/or require cranial vault expansion for increased intracranial pressure. Here, we report on a patient with Apert syndrome who did not have closed sutures at birth, and only began to demonstrate unilateral coronal suture fusion between ages 4 and 6 years, yet neither developed phenotypic signs of craniosynostosis nor evidence of intracranial hypertension. Moreover, despite demonstrating patency of the spheno-occipital synchondrosis, the patient developed progressive midface hypoplasia, requiring a subcranial Le Fort 3 advancement with external distraction at age 9. Now at skeletal maturity, this patient has a normal cranial shape and will likely never require cranial vault surgery for functional or aesthetic concerns. We are not aware of any prior reports of a patient with Apert syndrome who did not require intracranial surgery over long-term follow-up.

18.
Plast Reconstr Surg ; 153(3): 612e-616e, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-37053449

RESUMO

SUMMARY: Conventional methods to reconstruct cortical bone defects introduced by pediatric cranial vault remodeling (CVR) procedures have shortcomings. Use of bone burr shavings as graft material leads to variable ossification, and harvesting split-thickness cortical grafts is time-intensive and often not possible in thin infant calvaria. Since 2013, the authors' team has used the SafeScraper, originally developed as a dental instrument, to harvest cortical and cancellous bone grafts during CVR. The authors assessed the effectiveness of this technique by analyzing postoperative ossification using computed tomography scans of 52 patients, comparing cohorts treated with the SafeScraper versus those who received conventional methods of cranioplasty during fronto-orbital advancement. The SafeScraper cohort had a greater reduction in total surface area of all defects (-83.1% ± 14.9 versus -68.9% ± 29.8; P = 0.034), demonstrating a greater and more consistent degree of cranial defect ossification compared with conventional methods of cranioplasty, suggesting potential adaptability of this tool. This is the first study that describes the technique and efficacy of the SafeScraper in reducing cranial defects in CVR. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.


Assuntos
Crânio , Tomografia Computadorizada por Raios X , Criança , Lactente , Humanos , Crânio/cirurgia , Osteogênese , Transplante Ósseo/métodos , Coleta de Tecidos e Órgãos , Estudos Retrospectivos
19.
Otolaryngol Head Neck Surg ; 170(1): 20-33, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37694597

RESUMO

OBJECTIVE: Identify and appraise clinical practice guidelines (CPGs) for radioactive iodine (RAI) indications in differentiated thyroid carcinoma (DTC), and the treatment for radioactive iodine refractory (RAI-R) DTC using the Appraisal of Guidelines for Research and Evaluation II tool. DATA SOURCES: MEDLINE (Pubmed), Ovid (EMBASE), and Scopus. REVIEW METHODS: A systematic literature search was conducted to identify CPGs addressing RAI in DTC. CPGs were appraised by 4 independent reviewers in 6 distinct areas of quality. Scaled domain scores were subsequently calculated for each domain. Intraclass correlation coefficients were calculated for each domain to assess interrater reliability. RESULTS: Sixteen guidelines were found addressing RAI indications for DTC. Of these 16, 9 also addressed the treatment of RAI-R DTC. A further 6 unique guidelines were identified that exclusively address RAI-R DTC, bringing the total number of guidelines to 22. The American Thyroid Association (ATA) guidelines for adult thyroid cancer were the highest scoring with a mean score of 83.5%. Two guidelines scored >60% in 5 or more domains, qualifying as "high" quality: ATA and British Thyroid Association. The highest scoring domain was domain 4: clarity of presentation (80.4%) while the lowest scoring domain was domain 5: applicability (38.6%). CONCLUSION: Of the 22 guidelines identified, only two were "high quality." CPGs exclusively addressing the treatment of RAI-R DTC were weak with most guidelines scoring in the "low" quality range. This report reveals an unmet need for rigorously developed guidelines addressing indications for RAI in DTC, as well as the treatment for RAI-R DTC.


Assuntos
Neoplasias da Glândula Tireoide , Adulto , Humanos , Radioisótopos do Iodo/uso terapêutico , Reprodutibilidade dos Testes , Neoplasias da Glândula Tireoide/cirurgia
20.
Otolaryngol Head Neck Surg ; 170(1): 112-121, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37538005

RESUMO

OBJECTIVE: Management of the difficult airway can be a challenging process, which necessitates actionable recommendations from well-established guidelines. Herein, clinical practice guideline (CPG) quality is evaluated using the Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument. STUDY DESIGN: A systematic literature search was performed using Scopus, EMBASE, and MEDLINE via PubMed. SETTING: Literature database. METHODS: Data were abstracted from relevant guidelines and appraised by 4 expert reviewers in the 6 domains of quality defined by AGREE II. Intraclass correlation coefficients (ICC) were calculated across domains to quantify interrater reliability. RESULTS: Twelve guidelines met the inclusion criteria. With a mean quality score of 83.1%, the highest quality guideline was authored by the American Society of Anesthesiologists (ASA). Low-quality content was observed in CPGs authored by the Japanese Society of Anesthesiologists (JSA) and the Chinese Collaboration Group for Emergency Airway Management (CCGEAM). Overall, deficits were most pronounced in domains describing the involvement of stakeholders, developmental rigor, and editorial independence. These findings were consistent among the panel of independent reviewers, with high ICC inter-rater reliability scores of 58.0% to 70.0% for the referenced domains. CONCLUSION: By providing a comprehensive appraisal of guidelines, this report may serve as a reference for clinicians seeking to understand and improve upon the developmental quality of difficult airway management resources. According to AGREE II criteria for the quality of the guideline creation process, the 2022 ASA guideline outperforms its predecessors.


Assuntos
Manuseio das Vias Aéreas , Humanos , Bases de Dados Factuais , Reprodutibilidade dos Testes , Guias de Prática Clínica como Assunto
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