Cleft Palate-Craniofacial Journal最新文献

The Timing of Primary Surgery (TOPS) trial was published August 2023 in the New England Journal of Medicine and is a milestone achievement for a study focused on cleft palate. Due to the complexity of outcome reporting in cleft and the rarity of such comparative trials, TOPS presents a useful opportunity to critically review the design, analysis and reporting strategies utilised. This perspective article focused on the inclusion of participants, the choice of the primary outcome measure and the analysis of ordinal data within the trial. Considerations for future comparative studies in cleft care are discussed.

The TP63 gene is essential for epithelial proliferation, differentiation, and maintenance during embryogenesis. Despite considerable clinical variability, TP63-related symptoms are characterized by ectodermal dysplasia, distal limb malformations, and orofacial clefts. We identified a novel TP63 variant (c.619A > G, p.K207E) in a seven-month-old Chinese patient with orofacial clefts and ectrodactyly but no evident signs of ectodermal dysplasia. This phenotype was rarely reported before. We summarized the presence of the three main TP63-related manifestations in the literature and noted different distributions of CP- and CL/P-related variants regarding p63 structural domains.

Objective: To conduct a systematic review of the data in peer-reviewed medical literature and evaluate the effectiveness of lip taping as a pre-surgical naso-alveolar molding (NAM) technique in infants with cleft lip and/or palate.

Design: An electronic search of various databases for relevant studies, regardless of date, from inception to June 2023 was carried out and evaluated. After completing the electronic search and applying our inclusion/exclusion criteria, 6 studies-2 randomized control trials, 2 non-randomized studies, and 2 case series-were included. Data extraction of relevant articles was done independently by 2 authors. Quality assessment was done using the JBI prevalence critical appraisal tool and certainty of evidence was carried out by GRADE approach.

Main outcome measures: Nasolabial Aesthetics, Dentoalveolar Relationship.

Results: A total of six studies were included in the current review. Meta-analysis was carried out, and forest plots were obtained for a single mean from the lip-taping group. 3 studies had a low risk of bias, while 3 studies displayed a serious risk of bias. Significant improvement in various outcome measures was noted with lip taping when compared with the control group although the certainty of evidence was very low.

Conclusion: When compared to no therapy, lip taping appears to ameliorate dentoalveolar measurements and nasolabial aesthetics. To increase our knowledge of lip taping, more research will be needed in the future, as there are not many studies to prove lip taping is better than other treatment approaches.

Objective: To evaluate the safety of same-day discharge for patients undergoing primary cleft palate repair.

Design: Single-surgeon retrospective review.

Setting: Tertiary care institution.

Patients/participants: 40 consecutive patients that underwent primary cleft palate repair by a single surgeon from September 2018 to June 2023.

Interventions: Same-day discharge versus overnight admission after primary palatoplasty.

Main outcome measures: 30-day readmission, reoperation, wound and all-cause complication rate and 1-year fistula incidence.

Results: Of 40 total cases, 20 patients were discharged on the same calendar day and 20 patients were admitted for overnight stay following primary cleft palate repair. In the same-day discharge group, readmission incidence was 10%(n = 2), wound complication incidence was 5%(n = 1), and postoperative complication incidence was 15%(n = 3). In comparison, patients admitted overnight had a readmission incidence of 5%(n = 1, P = 1.00), wound complication incidence of 10%(n = 2, P = 1.00), and postoperative complications of 20%(n = 4, P = 1.00) No patients had 30-day reoperations or fistulas at 1 year. A higher proportion of admitted patients held a preoperative diagnosis of unilateral cleft palate and alveolus (Veau 3) as compared to patients discharged on the same day (P = .019). During the postoperative hospital course, admitted patients received significantly more oxycodone at median of 2 doses (IQR 1.00-3.75) and acetaminophen at a median of 4 doses (IQR 3.00-5.00) than patients with same-day discharge with a median of 1 dose (IQR 0.00 -1.00, P < .001).

Conclusions: In a low-risk patient population, same-day discharge following primary cleft palate repair may be safely undertaken and result in similar short-term outcomes and 1-year fistula incidence as patients admitted for overnight stay.

Numerous surgical techniques for median cleft lip repair have been described; however, most cause excessively sharp peaks or the collapse of Cupid's bow. We report a technique for median cleft lip repair using a mucosal skin flap and full-thickness skin graft and 15 years of follow-up. Our technique provides acceptable formation of the columella, philtrum, and the two peaks of Cupid's bow. In this paper, we cite our previously reported techniques and add new findings and discussion based on the long-term postoperative outcomes of this procedure. Advantages and disadvantages of this technique are discussed, and a possible solution to achieve a more satisfactory result is suggested. Advantages and disadvantages of this new technique are discussed, and a possible solution to achieve a more satisfactory result is suggested.

Objective: The inaugural Cleft Summit aimed to unite experts and foster interdisciplinary collaboration, seeking a collective understanding of velopharyngeal insufficiency (VPI) management.

Design: An interactive debate and conversation between a multidisciplinary cleft care team on VPI management.

Setting: A two-hour discussion within a four-day comprehensive cleft care workshop (CCCW).

Participants: Thirty-two global leaders from various cleft disciplines.

Interventions: Cleft Summit that allows for meaningful interdisciplinary collaboration and knowledge exchange.

Main outcome measures: Ability to reach consensus on a unified statement for VPI management.

Results: Participants agreed that a patient with significant VPI and a dynamic velum should first receive a surgery that lengthens the velum to optimize patient outcome. A global, multicenter prospective study should be done to test this hypothesis.

Conclusion: The 1st Cleft Summit successfully distilled global expertise into actionable best-practice guidelines through iterative discussions, fostering interdisciplinary collaboration and paving the way for a transformative multi-center prospective study on VPI care.

Objective: To explore the experiences and perceptions of barriers of parents and family members of patients with cleft lip and palate in accessing cleft services in remote northwest Nigeria.

Design: Face-to-face semi-structured audio recorded interviews were used to obtained qualitative textual data. Thematic analysis using interpretative descriptive techniques was employed to understand the participants' lived experiences with barriers and accessibility to cleft services.

Setting: Participants were from Sokoto, Kebbi and Zamfara states in remote northwest, Nigeria.

Participants: Consisted of 22 caregivers (17 parents and 5 extended family members) were purposively sampled between 2017 and 2020.

Main outcome measures: Barriers experienced while accessing cleft services were identified during thematic analysis.

Result: Over three quarter of the respondents had patients with both cleft lip and palate and without any previous family history (n = 20). About two-thirds of the participants (n = 15) were females. Most of the interviews were conducted before the surgeries (n = 15).

Five themes emerged: lack of information, financial difficulty, misrepresentation from health workers, multiple transportation and previous disappointment.

Conclusions: Areas of poor awareness, misinformation from primary health care workers, financial hurdles, multiple transportation logistics and others were identified. Aggressive broadcasting of information through radio, timely treatment and collaboration with influential religious leaders were emphasized. Support, grants and subsidies from government and voluntary agencies are encouraged to mitigate the huge out of pocket cost of cleft care in the region.

Objective: Assess the Health-Related Quality of Life in children and adolescents with non-syndromic craniosynostosis and compare it with participants without craniosynostosis.

Design: Non-experimental, cross-sectional design.

Setting: The assessment was done remotely and the instrument was sent via chat or email.

Patients/participants: Participants (ages 8-17) with non-syndromic craniosynostosis (n = 27) and without craniosynostosis (n = 26).

Main outcome measure(s): We used an adapted version for the Mexican population of the Health-Related Quality of Life Questionnaire for Children and Adolescents -KIDSCREEN-52.

Results: All scores were in the average clinical range and both groups scored similarly in all domains except those with craniosynostosis were significantly lower in the Social Support and Peers domain (r_pb= 0.48).

Conclusions: Children and adolescents with non-syndromic craniosynostosis reported similar Health-Related Quality of Life as the control group, except for the Social Support domain, which should be investigated in future studies.

Objective: To design the technique of 'Suspension Palatoplasty' for Velopharyngeal Insufficiency (VPI) post Cleft Palate (CP) based on optimal spatial positioning of palate at the time of VPI correction, by using a non-obstructive, high, midline pharyngeal flap for predictable velopharyngeal closure and normal speech. To evaluate the results of CP patients with VPI operated using the technique of 'Suspension palatoplasty'.

Design: An ambi-spective longitudinal clinical study.

Setting: Comprehensive cleft care clinic in a private trust hospital.

Patients, participants: Patients operated using the 'Suspension Palatoplasty' technique for VPI post CP repair between 2014 and 2018 with a minimum follow-up period of 5 years.

Interventions: 'Suspension Palatoplasty' - Double Opposing Z (DOZ) plasty with palatal myoplasty is used to revise soft palate and a narrow superiorly based pharyngeal flap is used to suspend it for a dynamic velopharyngeal closure.

Main outcome measure: Speech outcome and surgical complications.

Results: 70 out of 119 studied were found to have normal speech (59%), and another 25 patients (21%) had acceptable speech. Thus 95 out of 119 patients (80%) had normal or near-normal speech and did not require any further speech therapy or surgeries. 12 patients had snoring without difficulty in breathing. One patient had symptoms suggestive of obstructive sleep apnea. Younger patients had a higher percentage of normal speech outcomes. Many of our adult patients also attained normal speech.

Conclusion: 'Suspension Palatoplasty' aims to achieve normal speech with little effort. It has minimal side effects. The author has performed 403 cases to date.

Objective: To evaluate the outcomes associated with two techniques of periorbital steroid administration in bilateral fronto-orbital advancement (FOA).

Design: Multi-institutional retrospective chart review.

Setting: Two high volume, tertiary US craniofacial centers.

Patients, participants: Patients who underwent FOA between 2012 and 2021.

Interventions: Patients were divided into three cohorts based on method of steroid administration. Groups GEL and INJ represent those who received steroids in the form of triamcinolone soaked gelfoam or direct injection of dilute triamcinolone to the frontal/periorbital region, respectively. Group NON did not receive any periorbital steroids.

Main outcome measure(s): Peri-operative outcomes including hospital length of stay and complications were evaluated based on method of periorbital steroid administration. Variables predictive of infectious complications were assessed using stepwise logistic regression.

Results: Four hundred and twelve patients were included in our sample (INJ:249, GEL:87, NON:76). Patients in the INJ group had a higher ASA class (P < .001) while patients in the NON group were significantly more likely to be syndromic (P < .001) and have multisuture craniosynostosis (P < .001). Rate of infectious complications for each cohort were NON: 2.6%, INJ: 4.4%, and GEL: 10.3%. There was no significant difference between groups in hospital length of stay (P = .654) or rate of post-operative infectious complications (P = .061). Increased ASA class (P = .021), increased length of stay (P = .016), and increased intraoperative narcotics (P = .011) were independent predictors of infectious complications.

Conclusions: We identified a dose-dependent relationship between periorbital steroids and rate of postoperative infections, with key contributions from ASA class, hospital length of stay, and dose of intraoperative narcotics.