Cleft Palate-craniofacial Journal

OBJECTIVE: To determine the prevalence of treatment of early childhood caries (ECC) using general anesthesia (GA) in children with cleft lip and/or palate (CL/P). DESIGN: Retrospective chart review of children followed by the Manitoba Cleft Lip and Palate Program (MCLPP) to determine the frequency of treatment for ECC under GA. SETTING: Children's Hospital, Winnipeg, Canada (a tertiary care centre). PATIENTS: Children registered with MCLPP between January 1, 2008- December 31, 2019...

Osteosarcomas arising within the pterygomaxillary/infratemporal fossa region are rare among the pediatric population. Survival rates are most influenced by tumor resection with negative margins, which can be dependent on surgical accessibility of the tumor site. The pterygomaxillary/infratemporal fossa location poses several challenges to safe and adequate tumor resection, including proximity of the facial nerve and great vessels and scarring associated with traditional transfacial approaches. In this article, we present the case of a 6-year-old boy with an osteosarcoma of the left pterygomaxillary/infratemporal fossa region successfully managed with an "oncoplastic" approach, incorporating the use of CAD/CAM and mixed reality technologies...

This study used three-dimensional measurements to provide a nasolabial analysis of patients with unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), and controls across different races and ethnicities. A retrospective comparative study. Tertiary care pediatric institution. The study included 90 patients with UCLP, 43 patients with BCLP, and 90 matched controls. Patients are separated as self-identified Caucasian, Hispanic, or African American. Nasal length, nasal protrusion, columellar height, columellar width, tip width, alar width, alar base width, nasolabial angle, upper lip length, philtrum length, nostril height, and nostril width...

OBJECTIVE: To translate and validate CLEFT-Q© , patient-reported outcome measure for patients with cleft lip and/or palate (CL and/or P), into Indonesian. CLEFT-Q© covers the domains of appearance, facial function, health-related quality of life and consists of scales describing outcomes after cleft surgery. DESIGN: The CLEFT-Q© instrument was translated according to the International Society of Pharmacoeconomics and Outcomes Research guidelines, including translation, cognitive debriefing, and field-testing...

OBJECTIVE: Accurate nationwide epidemiological evidence is vital to study the seasonal, geographic, and ethnic influence on the trends of orofacial cleft prevalence in Kuwait. DESIGN: Data obtained from the National Center for Health Information and Ministry of Health Hospital digital records were reviewed retrospectively to identify patients with orofacial clefts (OFC) using the ICD-10 diagnostic codes. The Jonckheere-Terpstra test was used to assess the trend of birth prevalence across the different years...

BACKGROUND: Children born with Trisomy 13 or 18 (T13/18) often have multiple congenital anomalies, many of which drastically shorten their lifespan. Among these defects are cleft lip and palate, the repair of which presents an ethical dilemma to the surgeon given the underlying comorbidities associated with T13/18. The authors present an ethical discussion and institutional experience in navigating this dilemma. METHODS: The authors analyzed existing literature on T13 and T18 surgery and mortality...

OBJECTIVE: To assess modelled facial development of infants with unilateral cleft lip (CL) and cleft lip and palate (UCLP) compared to controls up to two years of age. DESIGN AND PARTICIPANTS: A total of 209 facial images of children (CL: n = 37; UCLP: n = 39; controls: n = 137) were obtained in four age categories (T0 = 0.2-0.5; T1 = 0.6-1.0; T2 = 1.1-1.5; T3 = 1.6-2...

OBJECTIVE: To identify characteristics of malpractice litigations involving skull deformity in infants (craniosynostosis and deformational plagiocephaly). DESIGN: Retrospective review of all lawsuits with jury verdicts or settlements involving infant skull deformity as the primary diagnosis using the Westlaw Legal Database. SETTING: United States. PATIENTS, PARTICIPANTS: Plaintiffs with skull deformity as the primary diagnosis...

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...

OBJECTIVE: To investigate condylar bone density (BD) in children with craniofacial microsomia (CFM) and identify factors that contribute to early stage condylar resorption (CR) after mandibular distraction osteogenesis (MDO). DESIGN: Retrospective study. SETTING: Craniofacial department of a plastic surgery hospital. PATIENTS: Fifty-one children with CFM classified as Pruzansky IIa based on complete pre-(T0) and post-MDO (T1) computed tomography (CT) data...

OBJECTIVE: Develop a model for the study of Electronic Nicotine Device (ENDS) exposure on craniofacial development. DESIGN: Experimental preclinical design followed as pregnant murine dams were randomized and exposed to filtered air exposure, carrier exposure consisting of 50% volume of propylene glycol and vegetable glycine (ENDS Carrier) respectively, or carrier exposure with 20 mg/ml of nicotine added to the liquid vaporizer (ENDS carrier with nicotine)...

OBJECTIVE: Characterize the interventions and assistance employed by a cleft nurse navigator (CNN) which have mediated improvement in care equity at our institution. DESIGN: Retrospective study. SETTING: Academic tertiary care center. PATIENTS, PARTICIPANTS: Patients presenting with cleft lip and/or cleft palate presenting between August 2020 and August 2021 with exclusions for syndromic diagnosis, Pierre-Robin sequence, late (> 6 months) presentation, and prior cleft surgery at outside institutions...

Palatal foreign bodies remain relatively rare, consequently, delays in diagnosis and misdiagnosis can occur leading to unnecessary anxiety and invasive investigations. We report three children with a reflective disc from inside a confetti balloon masquerading as a fistula of the hard palate. Awareness of this foreign body phenomenon enabled timely diagnosis in subsequent patients; hence the need to highlight such cases to the global cleft community. Crucially, while the foreign body remains in the oral cavity, there is an ongoing risk of airway aspiration which could be life threatening...

OBJECTIVE: The aim of this study was to evaluate the volumetric airway changes using three-dimensional images following unilateral vertical mandibular distraction osteogenesis (uVMD) among patients with hemifacial microsomia (HFM). DESIGN: This retrospective study analyzed cone-beam computed tomography (CBCT) scans of patients with HFM at three different timepoints; pretreatment (T0), posttreatment (T1), and at least 6 months post-distraction (T2). The individuals underwent uVMD between December 2018-Januaray 2021...

To investigate the relationship between auditory-perceptual ratings of resonance and nasometry scores in children with cleft palate. Factors which may impact this relationship were examined including articulation, intelligibility, dysphonia, sex, and cleft-related diagnosis. Retrospective, observational cohort study. Outpatient pediatric cranio-facial anomalies clinic. Four hundred patients <18 years of age identified with CP ± L, seen for auditory-perceptual and nasometry evaluations of hypernasality as well as assessments of articulation and voice...

UNLABELLED: To determine the sensitivity and specificity of velar notching seen on nasopharyngoscopy for levator veli palatini (LVP) muscle discontinuity and anterior positioning. Nasopharyngoscopy and MRI of the velopharynx were performed on patients with VPI as part of their routine clinical care. Two speech-language pathologists independently evaluated nasopharyngoscopy studies for the presence or absence of velar notching. MRI was used to evaluate LVP muscle cohesiveness and position relative to the posterior hard palate...

OBJECTIVE: Cleft lip and/or palate (CL/P) can have long-lasting effects on the appearance, function, and psychosocial wellbeing of patients. The CLEFT-Q questionnaire is a patient-reported outcomes instrument specifically designed to assess the health-related quality of life of patients with CL/P. The aim of this study was to produce and linguistically validate a Finnish version of the CLEFT-Q questionnaire. DESIGN: The CLEFT-Q questionnaire was translated into Finnish following guidelines of the International Society for Pharmacoeconomics and Outcomes Research...

METHODS: Retrospective case note review was undertaken of all patients treated in the clinic over a two-year period. RESULTS: 20 patients underwent HA filler injections to the upper lip 26 times. Most were female (F:M = 3:1) and patients were aged 18-58 years. Most patients had a unilateral cleft lip +/- palate (n = 13, 65%). The most common indication was to address upper lip volume (n = 13, 65%). Other indications included vermillion notch (n = 5, 25%), cupid bow peak height asymmetry (n = 4, 20%), scar asymmetry (n = 1, 5%) and nasal sill flattening (n = 1, 5%)...

OBJECTIVE: International outreach for cleft lip and/or palate care has traditionally been characterized by foreign groups and individuals holding surgical outreach trips in low- and middle-income countries. However, this "magic bullet" approach has often been criticized for prioritizing short-term results that may disrupt local workflow. The presence and impact of local organizations that support cleft care and take on capacity building initiatives has not been well explored. DESIGN: Eight countries previously studied as having the highest Google search demand for CL/P were chosen for the scope of the study...

The purpose of this study is to analyze the clinical characteristics of patients with Van der Woude syndrome (VWS) and to detect variations in each patient. Finally, the combination of genotype and phenotype can make a clear diagnosis of VWS patients with different phenotype penetrance. Five Chinese VWS pedigree were enrolled. Whole exome sequencing of the proband was performed, and the potential pathogenic variation was further verified by Sanger sequencing in the patient and their parents. The human mutant IRF6 coding sequence was generated from the human full-length IRF6 plasmid by site-directed mutagenesis and cloned into the GV658 vector, RT-qPCR and Western blot were used to detect the expression of IRF6 ...