Cleft Palate-Craniofacial Journal最新文献

Objective: Craniofacial microsomia (CFM) is a broad clinical term used to describe a congenital condition most commonly involving the underdevelopment of the external ear, mandible, soft tissues, and facial nerve. Despite medical advances, understanding of the psychological health and healthcare experiences of individuals with CFM and their caregivers remains limited. This article describes a research program designed to address these knowledge gaps, and identify opportunities for psychosocial intervention and improved healthcare provision.

Design: The Craniofacial microsomia: Accelerating Research and Education (CARE) research program aims to: 1) Conduct up to 160 narrative interviews with individuals and caregivers to validate a conceptual framework; 2) Administer an online international survey of up to 800 individuals with CFM and caregivers to identify predictors of psychological distress; 3) Perform up to 60 semi-structured interviews with healthcare providers and advocacy leaders to examine the extent to which current healthcare provisions address identified patient needs; and 4) Establish a participant registry to build a longitudinal database and develop an international community.

Results: Teams in the USA and UK have been established, alongside an international, interdisciplinary Advisory Committee. Data analysis for Aim 1 is ongoing and informing the delivery of Aims 2-3. Aim 4 is also in development. A dedicated website serves as a recruitment tool, educational resource, and mechanism for engaging with the CFM community.

Conclusions: The CARE program provides a comprehensive approach to understanding the experiences of individuals with CFM and their caregivers. Challenges encountered and lessons learned are shared for the benefit of the community.

Objective: To evaluate the outcomes of Secondary Alveolar Bone Grafting (SABG) in patients treated either in daycare or with multiple day hospitalization (MDH) in relation to costs and complication rates.

Design: Retrospective comparative cohort study.

Setting: The data was collected from two settings: Postoperative daycare or MDH after oral cleft surgery in an Academic Medical Center in The Netherlands.

Patients: Data of 137 patients with unilateral Cleft lip, alveolus, and palate (CLAP) treated between 2006-2018 were evaluated. Registered clinical variables: age, gender, cleft subtype, bone donor site, type of hospitalization, length of stay, additional surgery, complications, surgeons, and costs.

Interventions: Closure of the alveolar cleft with/without closure of the anterior palate.

Main outcome measures: Univariate analyses.

Results: Of the 137 patients, 46.7% were treated in MDH, and 53.3% in daycare. Total costs for daycare were significantly lower (P < .001). All patients treated in daycare received mandibular symphysis bone, whereas in MDH, 46.9% received iliac crest bone instead. Bone donor site was associated with postoperative care type. Complication rates were slightly but not significantly higher in daycare (26%) vs. MDH (14.1%) (P = .09). Most were Grade I (minor) according to Clavien Dindo classification.

Conclusions: Daycare after alveolar cleft surgery is about as safe as MDH, but significantly cheaper.

Objective: To compare the soft tissue measurements of the upper lip and the nose on the cleft side and the noncleft side before surgery, immediately after cheiloplasty and two months after surgery.

Design: Descriptive clinical study with one group, prospective.

Setting: Department of Odonto-Stomatology, Children's Hospital 1, at Ho Chi Minh City - Vietnam.

Patients: 31 patients with a unilateral complete cleft lip participated in this study, 30 patients were evaluated at the time two months after surgery.

Interventions: Interventions include PNAM and cheiloplasty by modified Millard technique.

Main outcome measures: Patients proceed to acquire 3D images of lips and nose, define landmarks and measure dimensions. There are 11 evaluators to be compared, p < 0.05 was considered statictical significance.

Results: After two months of surgery, on cleft side and noncleft side, the lengths of the upper lip were 10.87 ± 0.80 and 11.92 ± 0.78 (mm), the widths of the upper lip were 16.06 ± 1.10 and 16.40 ± 1.02 (mm), the heights of the nostril were 4.85 ± 0.44 and 5.93 ± 0.43 (mm), the lengths of columella were 4.08 ± 0.37 and 4.93 ± 0.38 (mm), and the widths of the nostril were 9.07 ± 0.37 and 8.37 ± 0.40 (mm).

Conclusion: Cheiloplasty by modified Millard technique on the patients who used PNAM showed that after two months of surgery, the morphology of the upper lip and nose has a slight disproportion with the nasolabial measurements of the cleft side being smaller than on the noncleft side.

Objective: Identification of at risk patients before surgery could facilitate improved clinical communication, care pathways and postoperative pain management.

Design: A retrospective cohort study was performed in all infants who had undergone cleft palate repair.

Setting: Tertiary Institutional.

Participants: Infants < 36 months of age who underwent primary repair of cleft palate between March 2016 and July 2022.

Intervention: Requirement for analgesic intervention in the post operative care unit.

Main outcome measure: Adverse perioperative event defined as pain or distress. Secondary outcomes were the incidence of airway obstruction, hypoxemia or unplanned intensive care admission.

Results: Two hundred and ninety one patients (14.6 months,10.1 kg weight) were included. Cleft distribution included submucous (5.2%), Veau I (23.4%), Veau 2 (38.1%), Veau 3 (24.4%), and Veau 4 (8.9%). Overall 35% of 291 infants undergoing cleft palate repair experienced pain or distress requiring opiate intervention in the first hour after surgery. Infants with a Veau 4 cleft palate had 1.8 times and Veau 2 cleft palate had 1.5 times the risk of postoperative pain compared to infants with Veau 1 cleft palate (relative risk 1.82, 95%CI 1.04-3.18 and 1.49, 95%CI 0.96-2.32 respectively). The use of bilateral above elbow arm splints was significantly associated with postoperative pain or distress (odds ratio 2.23, 95%CI 1.01-5.16).

Conclusions: Post operative pain requiring intervention in PACU is common despite adequate intraoperative multimodal analgesia, local anaesthesia infiltration and postoperative opiate infusions. Infants undergoing soft palate alone or submucous palate repair may require less perioperative opiates.

Objective: To evaluate a method of measuring the change in palatal length and shape following maxillary advancement using synchronous lateral videofluoroscopy and voice recording in order to understand how movement of the maxilla may affect VPI risk in patients with cleft lip and/or palate (CL/P).

Design: Retrospective cohort study of children with cleft lip and/or palate.

Setting: Single center, tertiary children's hospital.

Participants: Patients with cleft lip and/or palate who underwent maxillary advancement between 2016-21 inclusive.

Interventions: Maxillary advancement surgery, including those who underwent concurrent mandibular procedures.

Main outcome measures: The length of the soft palate and the genu angle were measured throughout palatal dynamic range. Pre- and post-operative measurements were compared using a one sided T-test, with subgroup analysis for patients with clinical VPI.

Results: Ten patients were examined. The mean distance of maxillary advancement was 10.5 mm. The average increase in pre-genu soft palate length was 2.8 mm in the resting position and 2.9 mm in the closed position. The genu angle decreased in the closed position by 16.3 degrees.

Conclusions: The soft palate showed limited ability to lengthen following maxillary advancement and this may explain the risk of VPI. There was partial compensation by the muscle sling of the palate as demonstrated by a more acute post-operative genu angle and this suggests one reason for the variability of VPI reported. Future research is required to investigate how length and shape changes measured using this method can predict VPI risk.

Objective: Our goal was to compare data collected from 3- and 7-day Infant with Clefts Observation Outcomes (iCOO) diaries.

Design: Secondary data analysis of an observational longitudinal cohort study. Caregivers completed the daily iCOO for 7 days before cleft lip surgery (T0) and for 7 days after cleft lip repair (T1). We compared 3- and 7-day diaries collected at T0 and 3- and 7-day diaries collected at T1.

Setting: United States.

Participants: Primary caregivers of infants with cleft lip with and without cleft palate (N  =  131) planning lip repair and enrolled in original iCOO study.

Main outcomes measure(s): Mean differences and Pearson correlation coefficients.

Results: Correlation coefficients were high for global impressions (>0.90) and scaled scores (0.80-0.98). Mean differences were small across iCOO domains at T0. T1 comparisons reflected the same pattern.

Conclusions: Three-day diary data is comparable to 7-day diaries for measuring caregiver observations using iCOO across T0 and T1.

Objective: Taking advantage of the broad coverage of Wireless Application Protocol (WAP), we developed a Content Management System (CMS)-programmed mobile learning application. This application can help the undergraduate to obtain a comprehensive understanding of concepts in Cleft lip and palate Phenotype, and Embryonic development (CPE). The present study aims to evaluate the feasibility and efficacy of the concept acquisition teaching model on the basis of WAP in a practical undergraduate course of CPE.

Design: The concept acquisition teaching model based on WAP was programmed by CMS, covering definitions of various cleft lip and palate, the mechanisms underlying the phenotypes, practical medical cases, as well as corresponding tests after learning.

Setting: The CPE concept acquisition teaching model was distributed to a total of 524 undergraduate students and 46 tutors participated in CPE teaching from seven highly ranked schools of stomatology in China since April 2022.

Participants: 524 undergraduate students and 46 tutors from seven highly ranked schools of stomatology in China.

Interventions: The CPE concept acquisition teaching model.

Main outcome measures: The effectiveness of the CPE teaching model.

Results: The response rate to the survey was 100%. The grading of the questionnaires indicated that the students were satisfied with the usability, practicality, and outcome, whereas the tutors were more positive with the contents, cooperation, and outcome.

Conclusions: The present study demonstrated the feasibility and efficacy of the WAP-based concept acquisition teaching model of CPE and a high level of satisfaction among undergraduate students and tutors who major in Stomatology.

Objective: To evaluate the applicability of transplanted teeth in young patients with craniofacial anomalies.

Design: Observational study.

Setting: Comprehensive Centre for Cleft Palate and Craniofacial Malformations.

Patients/participants: Patients with craniofacial anomalies who underwent tooth transplantation. Only children with complete clinical and radiological documentation and a follow-up period of at least 1.5 years were included.

Interventions: Tooth transplantation.

Main outcome measure(s): Retrospective evaluation of clinical records, pre- and postoperative radiographs, and operative charts. Clinical characteristics of patients, preoperative parameters and postoperative outcome parameters were collected.

Results: A total of 17 patients with 23 tooth transplantations were included. The median follow-up period was 6.7 years. The pooled survival and success rates were 91%. Notably, one out of two teeth that were transplanted into the bone grafted alveolar cleft site had to be extracted, which might indicating a higher risk for this procedure. In total, two transplanted teeth had to be extracted during the follow-up period, one due to external resorption and the other one due to perio-endo lesion. One patient needed endodontic treatment due to pulp necrosis.

Conclusion: We consider tooth transplantation to be a reliable and suitable procedure in the dental rehabilitation of young patients with craniofacial anomalies and fitting concomitant circumstances. We encourage craniofacial teams to reconsider this option more frequently in appropriate cases.

Objective: Posterior cranial distraction (PCD) is a surgical technique to address craniosynostosis, especially in syndromic patients. The technique has the ability to significantly expand the cranium, while requiring minimal dural dissection, compared to cranial remodeling. Our goals were to determine the patient characteristics and surgical outcomes of PCD. The two questions that we sought to answer were: 1) What is the average published complication rate and the most common complications of PCD? and 2) How much intracranial volume expansion can one expect with PCD?

Design: A PubMed database search of articles on PCD was performed. Case reports and articles with overlapping patients were excluded. A systematic review was performed using the remaining articles.

Main outcome measures: Patient data were extracted in order to determine the total number of patients, patients with a syndrome, types of syndromes, mean age at surgery, mean distraction distance, mean increase in intracranial volume, and complications.

Results: 18 articles representing 325 patients were analyzed. A syndrome was present in 68.6% of patients. The mean age at time of surgery was 22.1 months. Mean distraction amount was 24.7 mm. Mean increase in intracranial volume was 253.2 cm³. The overall complication rate was 32.2%, with the most common complications being surgical-site infection, hardware-related complications and delayed wound healing.

Conclusions: PCD is a powerful technique in the management of syndromic craniosynostosis, although complication rates are significantly higher than traditional remodeling techniques. Future studies should compare the effects of supratorcular and infratorcular osteotomies on intracranial volume, cosmesis and complications.

Objective: Paired box 7 (PAX7) has been considered as a candidate gene for non-syndromic cleft lip with or without palate (NSCL/P). However, there is no research for the XXX, and previous studies concentrated on limited variants. This study aimed to conduct sufficiently dense and powerful scans of variants at PAX7 and explored the roles of variants at PAX7 in NSCL/P among the XXX.

Design: Targeted region sequencing was performed to thoroughly screen variations, followed by a two-phase association analysis. 159 NSCL/P cases and 542 controls were analyzed in phase 1. Then in phase 2, the validation study was performed using 1626 cases and 2255 controls. We also explored the roles of variants at PAX7 gene in NSCL/P subtypes. Additionally, indirect associations were found by calculating LD and haplotypes.

Setting: The study was conducted in XXX.

Patients, participants: 159 NSCL/P cases and 542 controls were analyzed in phase 1. Then in phase 2, the validation study was performed using 1626 cases and 2255 controls.

Interventions: Blood samples were collected.

Main outcome measures: To explore the association analysis between variants at PAX7 and NSCL/P in XXX.

Results: The results showed that rs2236810, rs114882979 and rs2236804 were significantly associated with NSCL/P, which were predicted to have regulatory functions. Besides, variants at PAX7 function differently in the NSCL/P subtypes. We also discovered a PAX7 missense variant, NM_001135254 p.A369 V (NM_002584.2:c.1106C > T).

Conclusions: In summary, we confirmed 3 SNPs at PAX7 were significantly associated with NSCL/P in XXX and identified a missense variant, NM_001135254 p.A369 V (NM_002584.2:c.1106C > T).