Objective To study the phenotype and overview the clinical management on Cleft Lip and/or Palate (CL/P) with Nevoid Basal Cell Carcinoma Syndrome (NBCCS) patients in Peking University School and Hospital of Stomatology. Design Case series study. Main Outcome Measures To describe the clinical phenotype of 12 CL/P with NBCCS patients who fulfilled the diagnostic criteria as well as to explore clinical management. Read More
The peer-reviewed abstracts presented at the 74th Annual Meeting of the ACPA are published as submitted by the authors. For financial conflict of interest disclosure, please visit http://meeting.acpa-cpf. Read More
Dear CPCJ Peer Reviewers, As we start 2017, it is again time to say thank you to all the 2016 peer reviewers. Published bimonthly with a global readership, CPCJ is the official organ of the American Cleft Palate-Craniofacial Association. For the six issues of Volume 53, 2016, 114 articles were published in 963 pages with 756 print pages and 207 online-only pages. Read More
Objective: To evaluate speech in adults treated for unilateral cleft lip and palate with one-stage or two-stage palate closure and compare the speech of the patients with that of a noncleft control group.
Design: Cross-sectional study with long-term follow-up.
Participants/setting: All unilateral cleft lip and palate patients born from 1960 to 1987 and treated at Uppsala University Hospital, Sweden, were invited (n = 109). Read More
Craniofrontonasalsyndrome (CFNS) is a very rare genetic disorder, the common physical malformations of which include coronal synostosis, widely spaced eyes, clefting of the nasal tip, and various skeletal anomalies. Mutations of EFNB1, which encodes a member of the ephrin family of transmembrane ligands for Eph receptor tyrosine kinases, is the cause of CFNS. Although familial CFNS cases have been reported, no studies in the literature describe familial cases of CFNS expressing bilateral cleft lip and palate. Read More
Objective: We aim to establish consistent time points for evaluating palatal fistula incidence to standardize reporting practices and clarify prospective literature.
Design: An institutional retrospective chart review was conducted on 76 patients with unilateral or bilateral complete cleft lip and palate who underwent secondary alveolar bone grafting between 2006 and 2015.
Main Outcome Measures: Early fistula incidence rates were reported prior to maxillary expansion, and late fistula rates were reported at the time of alveolar bone grafting. Read More
Craniosynostosis (CS) has a prevalence of approximately 1 in every 2000 live births and is characterized by the premature fusion of one or more cranial sutures. Failure to maintain the cell lineage boundary at the coronal suture is thought to be involved in the pathology of some forms of CS. The Ephrin family of receptor tyrosine kinases consists of membrane-bound receptors and ligands that control cell patterning and the formation of developmental boundaries. Read More
Introduction: Secondary or residual cleft lip and nasal deformities following primary unilateral or bilateral cleft lip repair are common. Many classification systems have been proposed to describe congenital cleft lip and palate deformities before repair. This article proposes a one-of-a-kind classification system for residual cleft deformities and describes its application to 136 cleft lip revision cases from cleft outreach missions worldwide. Read More
Objective: Bone grafts in patients with cleft lip and palate can undergo a significant amount of resorption. The aim of this study was to investigate the effects of bisphosphonates (BPs) on the success of bone grafts in rats.
Design: Thirty-five female 15-week-old Fischer F344 Inbred rats were divided into the following experimental groups, each receiving bone grafts to repair an intraoral CSD: (1) Graft/saline: systemic administration of saline and (2) systemic administration of zoledronic acid immediately following surgery (graft/BP/T0), (3) 1 week postoperatively (graft/BP/T1), and (4) 3 weeks postoperatively (graft/BP/T2). Read More
Objective: To test the reliability of the modified Huddart-Bodenham (MHB) numerical scoring system and its agreement with the GOSLON Yardstick categorization for assessing the dental arch relationships in unilateral cleft lip and palate (UCLP) cases.
Design: A retrospective study.
Setting: Faculty of Dentistry, The University of Hong Kong. Read More
Objective: The objective of this study was to assess the efficacy of presurgical nasoalveolar molding (PNAM) on long-term nasal symmetry and shaping after primary cheiloplasty in patients with unilateral complete cleft lip/palate (UCL/P).
Design: This was a two-group, parallel, prospective, randomized clinical trial.
Setting: The setting for this study was the Chang Gung Craniofacial Center in Taoyuan, Taiwan. Read More
Purpose: Patient follow-up after cleft missions is imperative if we are to critically assess the quality of care provided in these settings. The adoption of mobile telephones among disadvantaged families abroad may enable such an undertaking in a cost-effective manner. This project aimed to assess the efficacy of cellular phone-based follow-up in a developing country following a cleft mission to Thailand. Read More
Introduction: Many societies and organizations are using social media to reach their target audience. The extent to which parents of patients with craniofacial anomalies use social media has yet to be determined. The goal of this study is to characterize and describe the use of social media by the parents of children with cleft lip and palate as it pertains to the care of their child. Read More
Objectives: The aim of this study is to define the candidate target genes for TFII-I and AP2α regulation in neural crest progenitor cells.
Design: The GTF2I and GTF2IRD1 genes encoding the TFII-I family of transcription factors are prime candidates for the Williams-Beuren syndrome, a complex multisystem disorder characterized by craniofacial, skeletal, and neurocognitive deficiencies. AP2α, a product of the TFAP2A gene, is a master regulator of neural crest cell lineage. Read More
Objective: To investigate the reliability of ordinal versus visual analogue scaling (VAS) ratings for perceptual judgments of nasal resonance, nasal airflow, understandability, and acceptability in speakers with cleft palate.
Objective: Oronasal fistula is an important complication of cleft palate repair that is frequently used to evaluate surgical quality, yet reliability of fistula classification has never been examined. The objective of this study was to determine the reliability of oronasal fistula classification both within individual surgeons and between multiple surgeons.
Design: Using intraoral photographs of children with repaired cleft palate, surgeons rated the location of palatal fistulae using the Pittsburgh Fistula Classification System. Read More
Manyspeakers with cleft palate develop atypical consonant productions, especially for pressure consonants such as plosives, fricatives, and affricates. The present study investigated the nature of nasal sound errors. The participants were eight female and three male speakers with cleft palate between the ages of 6 to 20. Read More
Background: A series of epidemiological studies were conducted to investigate the association between transforming growth factor alpha (TGFA) polymorphism and nonsyndromic cleft lip and/or palate (CL/P) risk, but the findings remain conflicting. The present meta-analysis summarizes the association between the TGFA Taq I polymorphisms and nonsyndromic CL/P risk.
Methods: We searched PubMed, EMBASE, Web of Science, and Chinese Biomedical Literature databases from their inception to May 1, 2015. Read More
Background: Humanitarian surgical organizations provide palatoplasties for patients without access to surgical care. Few organizations have evaluated the outcomes of these trips. This study evaluates the palatal fistula rate in patients from two cohorts in rural China and one in the United States. Read More
Objective: The aim of this systematic review was to identify patient-reported outcome (PRO) instruments used in research with children/youth with conditions associated with facial differences to identify the health concepts measured.
Design: MEDLINE, EMBASE, CINAHL, and PsycINFO were searched from 2004 to 2016 to identify PRO instruments used in acne vulgaris, birthmarks, burns, ear anomalies, facial asymmetries, and facial paralysis patients. We performed a content analysis whereby the items were coded to identify concepts and categorized as positive or negative content or phrasing. Read More
Background: Nonsyndromic cleft lip with or without cleft palate (nsCL±P) and nonsyndromic cleft palate (nsCP) are caused by a combination of genetic and environmental risk factors. We investigated gene-environment and gene-gene joint effects in a large multicenter study of case-parent triads.
Methods: The nsCL±P or nsCP triads were recruited in 11 European countries between 2001 and 2005. Read More
Objective: To develop a reliable and easy-to-use method to assess the nasolabial appearance of 18-year-old patients with unilateral cleft lip and palate (CLP).
Design: Retrospective analysis of nasolabial aesthetics using a 5-point ordinal scale and newly developed photographic reference scale: the Cleft Aesthetic Rating Scale (CARS). Three cleft surgeons and 20 medical students scored the nasolabial appearance on standardized frontal photographs. Read More
Congenital macrostomia, or Tessier number 7 cleft, is a rare craniofacial anomaly. We present a unique patient with bilateral macrostomia that consisted of a "double" transverse cleft on the left side and a single transverse cleft on the right side. A staged reconstructive approach was used to repair the "double" left-sided clefts. Read More
Objective: Maxillary growth in patients with cleft lip and palate is highly variable. The authors' aim was to investigate associations between preoperative cleft lip measurements and maxillary growth determined cephalometrically in patients with complete unilateral cleft lip and palate (cUCLP).
Design: Retrospective cross-sectional study. Read More
Objective: To compare facial growth characteristics in patients with cleft palate who have undergone pharyngeal flap with those who had palatal lengthening or pharyngoplasty and to control subjects who have not had surgery for velopharyngeal insufficiency (VPI).
Design: Matched retrospective cohort study.
Setting: Multidisciplinary cleft care center. Read More
Objective: Quantitative measures of facial form to evaluate treatment outcomes for cleft lip (CL) are currently limited. Computer-based analysis of three-dimensional (3D) images provides an opportunity for efficient and objective analysis. The purpose of this study was to define a computer-based standard of identifying the 3D midfacial reference plane of the face in children with unrepaired cleft lip for measurement of facial symmetry. Read More
Objective: The first part of this study validated an automated computer-based method of identifying the three-dimensional midfacial plane in children with unrepaired cleft lip. The purpose of this second part is to develop computer-based methods to quantify symmetry and to determine the correlation of these measures to clinical expectations.
Participants: A total of 35 infants with unrepaired unilateral cleft lip and 14 infant controls. Read More
Background: Our institution has incorporated the use of objective structured clinical examinations (OSCE) in our residency curriculum. The OSCE provides trainee education and evaluation while addressing the six Accreditation Council for Graduate Medical Education (ACGME) core competencies required within training programs. We report our program's experience with the first cleft OSCE ever conducted. Read More
Objective: The purpose of this study was to evaluate the pedicled buccal fat pad flap for its applicability in the reconstruction of surgically created oral defects. We highlighted the technique of flap harvesting, its different applications, and limitations.
Design: Prospective analysis of patients with intraoral defects repaired by the buccal fat pad flap. Read More
Objective: The aim of this study was to radiographically investigate the prevalence of dental anomalies outside the cleft area in a group of Brazilian patients with nonsyndromic cleft lip and/or palate (NSCL/P).
Design, Participants, And Setting: A retrospective analysis of 207 panoramic radiographs of patients with NSCL/P aged 12 to 45 years without history of tooth extraction and orthodontic treatment was performed.
Results: Dental anomalies were found in 75. Read More
Objective: Although numerous studies have confirmed that consumption of folic acid (FA) during early pregnancy reduces the risk of oral facial clefts in newborn infants, the optimal dose of FA for reducing this risk remains unknown. We evaluated various doses of FA for their ability to reduce the incidence of all-trans retinoic acid (ATRA)-induced cleft palate in mice.
Methods: Pregnant C57BL/6J mice were randomly assigned to eight groups dosed with corn oil (control group), ATRA (80 mg/kg), FA (40 mg/kg), or ATRA (80 mg/kg) + FA (2. Read More
For full-arch reconstruction of an atrophied cleft maxilla with missing premaxilla, a prefabricated microvascular free bony flap is a relevant option. A fibula flap was prefabricated in a cleft patient who received six dental implants and an epithelial layer. Six weeks later, maxillary reconstruction was performed. Read More
Transabdominal ultrasound examination carried out at 11.3 weeks' gestation suggested the diagnosis of holoprosencephaly (HPE). Transvaginal three-dimensional (3D) scan performed using the niche-mode technique enabled diagnosis of HPE, hypotelorism, and cleft lip (CL). Read More
We present a 3-month-old girl who displayed typical clinical characteristics of blepharophimosis, ptosis, and epicanthus inversus syndrome (BPES). She was referred to our clinic with an initial diagnosis of Down syndrome. Clinical features of elevated follicle stimulating hormone and low estradiol levels in the case were diagnosed as BPES syndrome and were consistent with BPES type 2. Read More
With the current widespread use of three-dimensional (3D) facial surface imaging in clinical and research environments, there is a growing demand for high-quality craniofacial norms based on 3D imaging technology. The principal goal of the 3D Facial Norms (3DFN) project was to create an interactive, Web-based repository of 3D facial images and measurements. Unlike other repositories, users can gain access to both summary-level statistics and individual-level data, including 3D facial landmark coordinates, 3D-derived anthropometric measurements, 3D facial surface images, and genotypes from every individual in the dataset. Read More
Objective: To assess the interdental septal thickness of grafted bone bridges using cone beam computed tomography (CBCT).
Patients: Of 71 patients with cleft lip and/or palate having undergone alveolar bone grafting for the first time at least 6 months previously, 52 patients with 57 grafted sites rated type I or II based on the Bergland scale using occlusal radiographs were selected.
Interventions: CBCT was performed for each bone-grafted alveolar cleft within 1 week after the occlusal radiographs were taken. Read More