Holistic Communication Assessment for Young Children With Cleft Palate Using the International Classification of Functioning, Disability and Health:Children and Youth.

Purpose Children with a cleft palate (± cleft lip; CP±L) can have difficulties communicating and participating in daily life, yet speech-language pathologists typically focus on speech production during routine assessments. The International Classification of Functioning, Disability and Health: Children and Youth Version (ICF-CY; World Health Organization, 2007) provides a framework for holistic assessment. This tutorial describes holistic assessment of children with CP±L illustrated by data collected from a nonclinical sample of seven 2- to 3-year-old children, 13 parents, and 12 significant others (e.g., educators and grandparents). Method Data were collected during visits to participants' homes and early childhood education and care centers. Assessment tools applicable to domains of the ICF-CY were used to collect and analyze data. Child participants' Body Functions including speech, language, and cognitive development were assessed using screening and standardized assessments. Participants' Body Structures were assessed via oral motor examination, case history questionnaires, and observation. Participants' Activities and Participation as well as Environmental and Personal Factors were examined through case history questionnaires, interviews with significant others, parent report measures, and observations. Results Valuable insights can be gained from undertaking holistic speech-language pathology assessments with children with CP±L. Using multiple tools allowed for triangulation of data and privileging different viewpoints, to better understand the children and their contexts. Several children demonstrated speech error patterns outside of what are considered cleft speech characteristics, which underscores the importance of a broader assessment. Conclusion Speech-language pathologists can consider incorporating evaluation of all components and contextual factors of the ICF-CY when assessing and working with young children with CP±L to inform intervention and management practices.

Integrated assessment of differentially expressed plasma microRNAs in subtypes of nonsyndromic orofacial clefts.

BACKGROUND: Orofacial clefts include cleft lip only (CLO), cleft palate only (CPO), and cleft lip with palate (CLP). Previously, we reported the expression profile of plasma microRNAs in CLO, CPO, and CLP, respectively. However, the interaction of each subtype remains poorly investigated. METHODS: In this study, we integrated the expression profiles of plasma miRNAs in these 3 subtypes, and assessed the distinct and overlapping dysregulated miRNAs using Venn diagrams. Their respective target genes reported in the literature were further analyzed using pathway analysis. RESULTS AND CONCLUSION: The results showed that distinct or overlapping signaling pathways were involved in CLO, CPO, and CLP. The common key gene targets reflected functional relationships to the Wnt, Notch, TGF-beta, and Hedgehog signaling pathways. Further studies should examine the mechanism of the potential target genes, which may provide new avenues for future clinical prevention and therapy.

GAND classification and volumetric assessment of unilateral cleft lip and palate malformations using cone beam computed tomography.

The aim of this study was to propose a classification for unilateral cleft lip and palate (UCLP) malformations based on cone beam computed tomography (CBCT) images, as well as to estimate the amount of bone necessary for grafting, and to evaluate the relationship of this volume with scores obtained using the classification. CBCT images of 33 subjects with UCLP were evaluated according to gap, arch, nasal, and dental parameters (GAND classification). Additionally, these defects were segmented and the amount of graft needed for alveolar bone grafting was estimated. The reproducibility of GAND classification was analyzed by weighted kappa test. The association of volume assessment with the classification (gap and nasal parameters) was verified using analysis of variance, while the intra-observer agreement was analyzed using the intra-class correlation coefficient. The intra-observer reproducibility of the classification ranged from 0.29 to 0.92 and the inter-observer agreement ranged from 0.29 to 0.91. There were no statistically significant values when evaluating the association of the volume with the classification (P>0.05). The GAND classification is a novel system that allows the quick estimation of the extent and complexity of the cleft. It is not possible to estimate the amount of bone needed for alveolar bone grafting based on the classification; individualized surgical planning should be done for each patient specifically.

Dental treatment need and dental general anesthetics among preschool-age children with cleft lip and palate in northern Finland.

Cleft lip and palate incidence is high in northern Finland. This study aimed to investigate the proportion of children in need of restorative dental treatment among cleft lip and palate patients in northern Finland, as well as their need for dental treatment under general anesthesia. The records of 183 cleft lip and palate patients, treated in Oulu University Hospital from 1997 to 2013, were reviewed. Data on dental caries were analyzed in association with cleft type, considering also the presence of syndromes. The frequency of dental general anesthetic (DGA) use, and of treatments, were also analyzed. Dental treatment need was most frequently observed, in this rather limited study population, in patients with the most severe deformities, namely bilateral cleft lip and palate, of whom 60% had caries. Among the study population, 11.5% (n = 21) had a syndrome. Of those, 57.1% had dental caries at the age of 3 or 6 yr, and only four could be treated without a DGA. Dental treatment under general anesthesia was performed in 14.8% of cleft patients without a syndrome, but in 38.1% of those with a syndrome. General anaesthesia is required for the provision of dental care more often in cleft (17.5%) than in non-cleft (0.2%) patients, and especially for those with a syndrome.

Volumetric assessment of secondary alveolar bone grafting using cone beam computed tomography.

OBJECTIVE: To assess the radiographic outcome of secondary alveolar bone grafting in individuals with nonsyndromic unilateral or bilateral cleft lip and palate using cone beam computed tomography. METHODS: This prospective study was conducted at the University of California at San Francisco Center for Craniofacial Anomalies on 21 consecutive nonsyndromic complete cleft lip and palate individuals between 8 and 12 years of age who required alveolar bone grafting. Seventeen unilateral and four bilateral cleft lip and palate individuals had preoperative and postoperative cone beam computed tomography scans that were analyzed using Amira 3.1.1 software. RESULTS: The average volume of the preoperative alveolar cleft defect in unilateral cleft lip and palate was 0.61 cm(3), and the combined average volume of the right and left alveolar cleft defects in bilateral cleft lip and palate was 0.82 cm(3). The average percentage bone fill in both unilateral cleft lip and palate and bilateral cleft lip and palate was 84%. The outcome of alveolar bone grafting was assessed in relation to (1) type of cleft, (2) size of preoperative cleft defect, (3) presence or absence of lateral incisor, (4) root development stage of the maxillary canine on the cleft side, (5) timing, and (6) surgeon. None of these parameters significantly influenced the radiographic outcome of alveolar bone grafting. CONCLUSIONS: Secondary alveolar bone grafting of the cleft defect in our center was successful, based on radiographic outcome using cone beam computed tomography scans. Volume rendering using cone beam computed tomography and Amira software is a reproducible and practical method to assess the preoperative alveolar cleft volume and the adequacy of bone fill postoperatively.

Hearing and speech assessment of cleft palate patients after palatal closure. Long-term results.

OBJECTIVE: The goal of this study was the development of a clinical methodology to assess speech and hearing impairment 5 years after the primary surgical repair of the cleft and, further, to determine the relative importance and long-term consequences of each cleft type and age to the velopharyngeal and eustachian tube function in patients who did not undergo pharyngeal flap surgery following primary palatoplasty. MATERIALS AND METHODS: We evaluated with a certain assessment protocol hearing and speech abilities of 42 patients between 5 and 15 years of age: 9 with CP (cleft of the soft and hard palate), 19 with unilateral cleft lip and palate (UCLP), 14 with bilateral cleft lip and palate (BCLP), surgically treated by a team of surgeons using two different surgical techniques between 18 and 24 months of age. ACCORDING TO OUR RESULTS WE WERE ABLE TO EVALUATE: (a) the impact of hearing impairment to the development of speech in these patients. (b) The relation of hypernasality with compansatory articulation. (c) The influence of cleft type, by means of extent of the cleft palatal musculature, to speech integrity. RESULTS: Sixty-nine percent of our patients presented with mild and moderate hearing loss. Hypernasality was observed in 40.5%, compensatory articulation in 28.5% of our patients. CONCLUSIONS: Our findings indicated: (a) a simultaneous appearance of speech and hearing impairment at the same age for each cleft type post-surgically in our patients; (b) that the muscular and vomer complex rather than the anatomic extent of the cleft is a significant factor for speech outcome after surgical repair; and (c) that hypernasality is exacerbated by compensatory articulation.

A quantitative three-dimensional assessment of abnormal variations in facial soft tissues of adult patients with cleft lip and palate.

OBJECTIVE: To supply quantitative information about the facial soft tissues of adult operated patients with cleft lip and palate (CLP). DESIGN, SETTING, AND PATIENTS: The three-dimensional coordinates of soft tissue facial landmarks were obtained using an electromagnetic digitizer in 18 Caucasian patients with CLP (11 males and 7 females aged 19 to 27 years) and 162 healthy controls (73 females and 89 males aged 18 to 30 years). From the landmarks, 15 facial dimensions and two angles were calculated. Data were compared with those collected in healthy individuals by computing z-scores. Two summary anthropometric measurements for quantifying craniofacial variations were assessed in both the patients and reference subjects: the mean z-score (an index of overall facial size), and its SD, called the craniofacial variability index (an index of facial harmony). RESULTS: In treated patients with CLP, facial size was somewhat smaller than in normal individuals, but in all occasions the mean z-score fell inside the normal interval (mean +/- 2 SD). Almost all patients had a craniofacial variability index larger than the normal interval, indicating a global disharmonious appearance. Overall, in patients pronasale, subnasale, and pogonion were more posterior, the nose was shorter and larger, the face was narrower, and the soft tissue profile and upper lip were flatter than in the reference population. CONCLUSIONS: The facial soft tissue structures of adult operated patients with CLP differed from those of normal controls of the same age, sex, and ethnic group. In this patient group, surgical corrections of CLP failed to provide a completely harmonious appearance, even if the deviations from the reference were limited. Further analyses of larger groups of patients are needed.

Aspects of assessment and management of velopharyngeal dysfunction in developing countries.

Proper assessment of patients with velopharyngeal valve incompetence is a mandatory prerequisite for optimal management. The protocol of assessment of Ain Shams University, Phoniatric Department, uses three levels of assessment of velopharyngeal valve incompetence according to the complexity of the armamentarium used. This allows application of those parts of the protocol that suit the needs of the different socioeconomic levels and geographical locations. Firstly, the elementary diagnostic procedures, which are rather simple, noninvasive, but essentially subjective. Despite the clinical feasibility of these procedures, documentation of the data is made utilizing the tools at the second level of assessment in that protocol (clinical diagnostic aids). This level comprises video-nasofiberscopy and high fidelity voice recording. An attempt to extract quasi-quantitative measures from the hitherto qualitative video-nasofiberscopy is made. The third level of assessment, namely additional instrumental measures, comprises CT scanning of the velopharyngeal port, aerodynamics, and acoustic analysis. The results of the three levels of the protocol are presented. Their significance and clinical efficacy are discussed. Some community-related problems that have faced the cleft palate team are outlined. Their sociocultural significance in a developing country is discussed.