Cleft Palate-Craniofacial Journal最新文献

Objective: The present study aimed to investigate the risk factors, complication profiles, and clinical outcomes of cleft and noncleft patients undergoing single jaw (mandibular or LeFort 1) and bimaxillary (BSSO + LeFort 1).

Design: Retrospective Cross-sectional Study Setting: National Surgical Quality Improvement Program database 2018-2019.

Patients: Pediatric patients.

Interventions: Outcomes for mandibular, LeFort 1, and bimaxillary osteotomy were retrospectively evaluated for cleft and noncleft patients.

Main outcome measures: Multivariate logistic regression was used to determine the odds of complications and length of stay for cleft and noncleft patients undergoing single jaw and double jaw surgery.

Results: 669 pediatric patient underwent orthognathic surgery in the study period; the majority received LF1 only (n = 385; 58.3%), followed by mandible only (n = 179; 27.1%), and bimaxillary (n = 105; 15.9%%). Cleft differences were present in 56% of LFI patients, 32% of mandibular patients, and 22% of bimaxillary patients. After multivariate adjustment, ASA class III was associated with nearly 400% increased odds of any complication including readmission and reoperation (OR = 5.99; CI [[1.54-23.32]], p < 0.01, and 65% increased LOS (β-coefficient = 1.65, CI [1.37-1.99], p < 0.01). Presence of cleft was not significantly associated with odds of any complication (p = 0.69) nor increased LOS (p = 0.46) in this population.

Conclusion: Complications remained low between surgery types among cleft and noncleft patients. The most significant risk factor in pediatric orthognathic surgery was not the presence of cleft but rather increased ASA class. Though common in patients seeking orthognathic surgery, cleft differences did not cause additional risk after adjustment for other variables.

Objective: To assess the characteristics of popular videos on YouTube about the feeding of infants with cleft lip and palate (CLP) and analyze the adequacy of information provided by them.

Design: A cross-sectional design was used.

Methods: YouTube was systematically searched for consecutive relevant videos about the feeding of infants with CLP, using predefined keyword combinations, without any limitations on language or duration. Scrutiny of the top 50 videos for each keyword combination was performed and a self-designed data-extraction sheet was used. A content adequacy index was developed by an expert group, and used to assess content adequacy, classifying it into categories from excellent to poor.

Results: From an initial retrieval of 200 videos, 42 were included in the final assessment. The videos originated from nine different countries, with more than half coming from the USA and in English. Five of the videos came from the channel of the American Cleft Palate-Craniofacial Association. Content adequacy analysis showed that no video could be classified as excellent, while 33.3% were classified as optimal, 21.4% as suboptimal and 45.2% as poor.

Conclusions: The content adequacy of the majority of videos on YouTube, relating to the feeding of infants with CLP was inadequate, with only one third of them achieving optimal content adequacy. Efforts must be made to develop informative and standardized videos for social media and video-sharing platforms, perhaps through professional associations to ensure that families with an infant with CLP receive appropriate information.

Objective: To identify specific areas for improvement in cleft lip repair teaching.

Design: Secondary analysis of prospectively-collected, blinded data.

Setting: Three residency programs rotating at a single academic children's hospital.

Participants: Plastic surgery residents, and craniofacial/pediatric plastic surgery fellows.

Main outcome measures: Mean scores for each skill in an 18-item Unilateral Cleft Lip Repair competency assessment tool (UCLR) (1-3 scale for each item) were rank ordered. Correlation between level of training (PGY) and performance on steps of the procedure was examined using Pearson R.

Results: Simulation participants (n = 26) scored highest on skills in the "Marking" subscale (2.38-2.63 mean score). Procedural steps that scored lowest were: closing the nasal floor (2.00), repairing oral mucosa (2.15) and avoiding over/under-dissection (2.19). Interestingly, none of these skills correlated with PGY, suggesting they do not improve with training.

Conclusions: These results suggest that marking cleft lip repair is taught well in our current teaching environment, while steps like closing the nasal floor and repairing the oral mucosa are taught less well. Improved teaching of these steps could be achieved with deliberate instruction, video, digital simulation, and high fidelity simulation.

Objective: To provide a comprehensive understanding of the pathophysiology of cleft palate (CP) and future perspectives.

Design: Literature review.

Setting: Setting varied across studies by level of care and geographical locations.

Interventions: No interventions were performed.

Main outcome measure(s): Primary outcome measures were to summarize our current understanding of palatogenesis in humans and animal models, the pathophysiology of CP, and potential future treatment modalities.

Results: Animal research has provided considerable insight into the pathophysiology, molecular and cellular mechanisms of CP that have allowed for the development of novel treatment strategies. However, much work has yet to be done to connect our mouse model investigations and discoveries to CP in humans. The success of innovative strategies for tissue regeneration in mice provides promise for an exciting new avenue for improved and more targeted management of cleft care with precision medicine in patients. However, significant barriers to clinical translation remain. Among the most notable challenges include the differences in some aspects of palatogenesis and tissue repair between mice and humans, suggesting that potential therapies that have worked in animal models may not provide similar benefits to humans.

Conclusions: Increased translation of pathophysiological and tissue regeneration studies to clinical trials will bridge a wide gap in knowledge between animal models and human disease. By enhancing interaction between basic scientists and clinicians, and employing our animal model findings of disease mechanisms in concert with what we glean in the clinic, we can generate a more targeted and improved treatment algorithm for patients with CP.

Fibrodysplasia ossificans progressiva (FOP) is a rare condition characterized by progressive heterotopic ossifications and congenital hallux valgus deformities. The common underlying genetic cause is an ACVR1 mutation, resulting in altered bone morphogenetic protein (BMP) regulation. Trauma and/or minor procedures aggravate the abnormal bony formation in soft tissues. This report presents a 3-year-old child with this condition who presented pseudo-ankylosis of the temporomandibular joint (TMJ) after minor craniofacial trauma. Abnormal ossification in the medial pterygoid muscle was identified as the causative abnormality for the presentation with trismus.

Objective: To verify the effect of orthognathic surgery (OS) on nasal dimensions and its correlation with respiratory symptoms in patients operated on over the past 20 years.

Design: Retrospective study.

Setting: Tertiary level craniofacial hospital.

Participants: 535 patients with CLP previously repaired, without syndromes, age over 18, who performed OS between 2000 and 2019 and rhinomanometric evaluation pre and post OS.

Interventions: Maxillary advancement isolated and combined with turbinectomy and/or mandibular osteotomy.

Main outcome measures: Minimum nasal cross-sectional area (CSA) assessed by posterior (PR) and anterior rhinomanometry (AR), nasopharyngeal cross-sectional area (NCSA) verified by modified AR, and self-perceived respiratory symptoms through a questionnaire.

Results: After OS, there was a significant increase in CSA assessed by PR (p < .001) and AR (p < .001), while there was no significant difference in NCSA (p = 0.319). Regarding respiratory symptoms after OS, 26.3% showed improvement in nasal obstruction, 28.5% in oronasal breathing, 18.5% in snoring, and 5.2% in respiratory obstruction during sleep. However, a weak correlation between increased CSA and improvement of symptoms was observed. In the first decade, lower values of CSA (p < .001) and NCSA (p < .001) were observed compared to the second decade. Both periods showed a significant increase in CSA (p < .001).

Conclusions: Nasal dimensions and breathing symptoms were improved by OS when combined with turbinectomy. Comparing the results of patients operated on between two different decades, patients had more favorable conditions for breathing in the most recent decade of care. However, this was not statistically correlated with the improvement in patient symptoms in this study.

Traditional imaging modalities used to assess velopharyngeal insufficiency (VPI) do not allow for direct visualization of underlying velopharyngeal (VP) structures and musculature which could impact surgical planning. This limitation can be overcome via structural magnetic resonance imaging (MRI), the only current imaging tool that provides direct visualization of salient VP structures. MRI has been used extensively in research; however, it has had limited clinical use. Factors that restrict clinical use of VP MRI include limited access to optimized VP MRI protocols and uncertainty regarding how to interpret VP MRI findings. The purpose of this paper is to outline a framework for establishing a novel VP MRI scan protocol and to detail the process of interpreting scans of the velopharynx at rest and during speech tasks. Additionally, this paper includes common scan parameters needed to allow for visualization of velopharynx and techniques for the elicitation of speech during scans.

The aim of this paper is to describe a technique that can be utilised for the targeted correction of the Whistle Deformity and accompanying upper lip subunits. All patients were post-primary cleft lip repair, either unilateral or bilateral cleft lips, with a resulting Whistle Deformity post-operatively. They all elected to undergo surgical correction of the Whistle Deformity using bilateral philtral dermofascial flaps to correct their Whistle Deformity. Post-operative clinical photographs demonstrate the results of the technique. We assessed for the resolution of the Whistle Deformity and improvement of the appearance of the upper lip subunits, including; philtral, columella, and nostril sill elements. The patients treated with this technique, with Whistle Deformities following unilateral and bilateral cleft lip repair, had satisfactory results from the procedure and recovered with no complications. We propose this is an effective method for treating up to moderate-severe Whistle deformities following both unilateral and bilateral primary cleft lip repairs.

Aarskog-Scott syndrome (AAS), also known as facio-digito-genital syndrome, is a rare heterogenous syndrome characterized by facial dysmorphism, brachydactyly, and genetic abnormalities. Although severe craniofacial abnormalities have been reported in AAS, little is known about speech and resonance issues in AAS. Specifically, published data to date have only indicated reports of hypernasality associated with a cleft palate in AAS. This case report provides clinical and anatomic information surrounding hypernasal speech in the absence of an overt cleft palate in a patient with AAS.

Objective: To determine the accuracy of the Pediatric Sleep Questionnaire (PSQ) as a screening tool for obstructive sleep apnea in children with craniofacial anomalies.

Design: Retrospective cohort study.

Setting: Multidisciplinary cleft and craniofacial clinic at a tertiary care center.

Patients: Children with craniofacial anomalies 2 to ≤18 years of age who both completed a PSQ screen and underwent polysomnography (PSG) without interval surgery.

Main outcome measures: Sensitivity and specificity of the PSQ in detecting an obstructive apnea-hypopnea index (AHI) ≥ 5 events/hour.

Results: Fifty children met study criteria, with 66% (n = 33) having an associated syndrome. Mean patient age at time of PSQ was 9.6 + 4.0 years. Overall, 33 (64%) screened positive on the PSQ, while 20 (40%) had an AHI ≥ 5. The sensitivity and specificity for identifying AHI ≥ 5 was 70% and 40%, respectively. With subgroup analysis, the sensitivity and specificity were higher (100% and 50%) in children with non-syndromic palatal clefting but lower (65% and 31%) in children with a syndrome or chromosomal anomaly. There was no correlation detected between PSQ score and AHI severity (p = 0.25). The mean obstructive AHI in the study population was 10.1 ± 22.7 despite 44% (n = 22) undergoing prior adenotonsillectomy.

Conclusions: The PSQ was less sensitive and specific in detecting an AHI ≥ 5 in children with craniofacial anomalies than in a general population, and particularly poor in for children with syndrome-associated craniofacial conditions. Given the high prevalence of OSA in this patient population, a craniofacial-specific validated screening tool would be beneficial.