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An automated cleft speech evaluator, available globally, has the potential to dramatically improve quality of life for children born with a cleft palate, as well as eliminating bias for outcome collaboration between cleft centers in the developed world. Our automated cleft speech evaluator interprets resonance and articulatory cleft speech errors to distinguish between normal speech, velopharyngeal dysfunction and articulatory speech errors. This article describes a significant update in the efficiency of our evaluator. Speech samples from our Craniofacial Team clinic were recorded and rated independently by two experienced speech pathologists: 60 patients were used to train the evaluator, and the evaluator was tested on the 13 subsequent patients. All sounds from 6 of the CAPS-A-AM sentences were used to train the system. The inter-speech pathologist agreement rate was 79%. Our cleft speech evaluator achieved 85% agreement with the combined speech pathologist rating, compared with 65% agreement using the previous training model. This automated cleft speech evaluator demonstrates good accuracy despite low training numbers. We anticipate that as the training samples increase, the accuracy will match human listeners., (Copyright © 2018. Published by Elsevier Ltd.)

Background: Sculpted physical models and castings of the anatomy of cleft lip and palate are used for parent, patient, and trainee education of cleft lip and palate conditions. In this study, we designed a suite of digital 3-dimensional (3D) models of cleft lip and palate anatomy with additive manufacturing techniques for patient education., Methods: CT scans of subjects with isolated cleft palate, unilateral and bilateral cleft lip and palate, and a control were obtained. Soft tissue and bony structures were segmented and reconstructed into digital 3D models. The oral soft tissues overlying the cleft palate were manually molded with silicone putty and scanned using CT to create digital 3D models. These were then combined with the original model to integrate with segmentable soft tissues. Bone and soft tissues were 3D printed in different materials to mimic the rigidity/softness of the relevant anatomy. These models were presented to the parents/patients at our craniofacial clinic. Visual analog scale (VAS) surveys were obtained pertaining to the particular use of the models, to ascertain their value in parental education., Results: A total of 30 parents of children with cleft conditions completed VAS evaluations. The models provided the parents with a better understanding of their child's condition with an overall evaluation score of 9.35 ± 0.5., Conclusions: We introduce a suite of 3D-printed models of cleft conditions that has a useful role in patient, parental, and allied health education with highly positive feedback.

Perceptual evaluation remains the gold-standard evaluation of cleft speech, but with any human interpretation, there can be bias. Eliminating bias, allowing comparison of speech data between units, is labor and time intensive. Globally, there is a shortage of listeners. We have developed a computer learning system to evaluate cleft speech. Our automated cleft speech evaluator interprets resonance and articulatory cleft speech errors. Speech recognition engines typically ignore voice characteristics and speech errors of the speaker, but in cleft speech evaluation, these features are paramount. Our evaluator targets these to distinguish between normal speech, velopharyngeal dysfunction and articulatory speech errors. Speech samples from our Craniofacial Team clinic were recorded and rated independently by two experienced speech pathologists: 60 patients were used to train the evaluator, and the evaluator was tested on the 13 subsequent patients. The inter-speech pathologist agreement rate was 79%. Our cleft speech evaluator achieved 77% on its best sentence and a median of 65% for all sentences. This automated cleft speech evaluator has applications for global cleft speech evaluation when no speech pathologist is available, and for unbiased evaluation, facilitating collaboration between teams. We anticipate that as the training samples increase, the accuracy will match human listeners., (Copyright © 2017 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Objective: To assess patient experiences in obtaining dental care in a team setting without an affiliated dental school., Design and Participants: Three hundred seventy-four patients in the cleft team database met inclusion criteria of diagnosis of cleft lip and/or palate, and current age of 7 to 12 years. Demographic information and experiences in obtaining dental care were ascertained using a standardized series of questions. A callback protocol was employed to maximize response rate. Results were analyzed with t-tests using contingency tables., Outcome Measures and Results: One hundred seventy-one parents/caregivers were interviewed of a possible 374 (response rate 45.7%). Mean age was 9.87 years. The insurance distribution was as follows: 113 private insurance (66.1%), 35 Medicaid (20.5%), and 23 had no insurance (13.5%). The dental checkup distribution was as follows: 145 regular dental checkups (84.8%) and 26 no regular checkups (15.2%). Patients with private insurance were more likely to obtain dental care than were patients with Medicaid (p = .002) or patients without insurance (p = .0027). Patients with Medicaid were more likely to report provider refusal of care than were patients with private insurance (p = .0001) or patients without insurance (p = .0001). Patients with private insurance were more likely to report satisfaction with their dental care than were patients with Medicaid (p = .0003)., Conclusions: We report an 84.8% regular checkup rate among our study population and a significantly different reported experience in obtaining care depending on insurance type. The reasons underlying the differences between privately insured patients and Medicaid patients appear multifactorial.

Background: Patients with palatal clefts are predisposed to developing speech and language abnormalities. Emerging evidence indicates that children with cleft lip and/or cleft palate have higher rates of learning disabilities than the general population and differences in brain morphology., Methods: Magnetic resonance imaging of 12 individuals with isolated unilateral complete clefts of the lip and palate produced functional images during three lexical processing tasks: generation of verbs, opposites, and rhymes. Direct statistical comparisons were made between subjects with cleft lip and palate and controls (matched for age and performance) from an extant data set, both as a group and individually., Results: Two types of differences were found. Compared with unaffected controls, subjects with clefts showed a delayed and elongated blood oxygen level-dependent response in regions found throughout the cerebrum, including in the prefrontal cortex, cingulate gyrus, right precuneus, and right temporal gyrus. A right middle frontal gyrus region was activated by these tasks in controls but not in subjects with clefts. Developmental analysis showed that subjects 14.5 years and older (n = 5) had a larger number of age-related regions differing in blood oxygen level-dependent response from controls than did younger subjects (n = 7). Single-patient analysis demonstrated substantial individual variability., Conclusions: Children with cleft lip and palate, performing lexical processing tasks at a comparable level of proficiency, use a similar but nonidentical functional neuroanatomy than peers without clefts. Differing neural circuitry for language tasks and differing developmental trajectories could help explain the predisposition to velopharyngeal dysfunction and learning disabilities in this population.

Background: The purpose of this study was to quantitate preoperative osseous dysmorphology in a homogeneous group of 3-month-old infants with unilateral complete cleft lip and palate., Methods: High-resolution computed tomography scans of 28 infants with unilateral complete cleft lip and palate were the basis for study. Coordinate data from 43 landmarks on the skull were collected using surface-rendered reconstructions of scan data. Euclidean distance matrix analysis was used to assess the degree of asymmetry between the cleft and noncleft sides of the craniofacial skeleton., Results: Linear distances involving primary and secondary landmarks (those that are located on or within the bony cleft and those that are near the cleft in the adjacent oronasal area, respectively) were highly asymmetric, with significantly greater distances on the cleft side. In addition, small (1 to 5 percent) but statistically significant asymmetries in linear distances were found involving tertiary landmarks (those that are not directly associated with the cleft or adjacent oronasal area). Most linear distances involving the nasion, zygomaxillare superius, and frontozygomatic junction were significantly greater on the cleft side, and certain linear distances in and around the middle cranial fossa were significantly smaller on the cleft side., Conclusions: The extreme asymmetry of primary and secondary landmarks is explained by the cleft itself and the obvious displacement of the premaxilla toward the noncleft side. The subtler, statistically significant asymmetry of the tertiary landmarks supports the idea that the unilateral cleft affects development of the entire face and possibly the cranial base. Euclidean distance matrix analysis of computed tomography landmark data is a useful methodology for the quantitative morphometry of children with untreated unilateral cleft lip and palate.

Epignathus, a congenital oropharyngeal teratoma, is a rare clinical entity with variable clinical outcomes described in the literature. Even fewer cases of epignathus with intracranial extension have been reported, all with poor outcomes. In this manuscript, the authors present a case of epignathus with intracranial extension, emphasizing clinical presentation, imaging findings, a staged surgical approach, multidisciplinary management, and outcome.

The Pittsburgh Oral-Facial Cleft study was begun in 1993 with the primary goal of identifying genes involved in nonsyndromic orofacial clefts in a variety of populations worldwide. Based on the results from a number of pilot studies and preliminary genetic analyses, a new research focus was added to the Pittsburgh Oral-Facial Cleft study in 1999: to elucidate the role that associated phenotypic features play in the familial transmission patterns of orofacial clefts in order to expand the definition of the nonsyndromic cleft phenotype. The purpose of this paper is to provide a comprehensive review of phenotypic features associated with nonsyndromic orofacial clefts. These features include fluctuating and directional asymmetry, non-right-handedness, dermatoglyphic patterns, craniofacial morphology, orbicularis oris muscle defects, dental anomalies, structural brain and vertebral anomalies, minor physical anomalies, and velopharyngeal incompetence.

Objective: To test the hypothesis that maxillary growth and lower lip form in patients with van der Woude syndrome (VDW) is different from patients with non-syndromic cleft lip and palate., Design and Setting: Retrospective, case-control study at a tertiary cleft center, Chang Gung Memorial Hospital, Taipei, Taiwan., Patients and Participants: Records of 53 patients with VDW, who presented for treatment during the years 1968 through 1998 were obtained. Twenty-three of 53 patients had received at least one lateral cephalogram during the course of their treatment. Of these 23, in 17 it was possible to find non-syndromic case controls with identical cleft type, sex, and method of cleft palate repair, with year of birth matching within 1 year of the corresponding VDW patient. For these 17 pairs of VDW and non-syndromic cleft controls, cephalogram acquisition dates were checked to see how well the corresponding pairs matched. A total of 43 pairs of cephalograms were deemed to be acceptably matched because the ages at acquisition differed by less than 15% of the VDW patient's age., Main Outcome Measures: Thirteen measurements were derived from the 11 standard lateral cephalometric landmarks recorded on each cephalogram. The data were classified into five groups according to age at time of cephalogram, and sets of paired non-syndromic cleft and VDW measurements were tested for differences using a Wilcoxon signed rank sum test in two ways, first including all cleft types and then including only those patients with complete bilateral cleft lip and palate. A longitudinal growth analysis considering the movement of the skeletal A and B points was performed on the patients with complete bilateral cleft lip and palate., Results: For the osseous measurements, anteroposterior maxillary length as described by the anterior nasal spine (ANS)-posterior maxillary point distance was statistically significantly shorter in the VDW patients of age 13 years and older, by 5.3 mm. Maxillary height, as described by the nasion-ANS distance was shorter in the VDW patients, closely approaching statistical significance in the age range 7 through 11 years. The lip soft tissue measurements showed significantly greater protrusion over several age ranges in the VDW patients. The longitudinal growth analysis showed a significantly more inferior vertical position of the B point in the controls., Conclusions: This study demonstrates a few statistically significant differences in maxillary growth and lip conformation between VDW and matching controls. Small sample sizes for each age group hamper the ability to fully interpret or generalize the pattern of these differences.

Objective: To determine whether, in performing palatoplasty, fracture of the pterygoid hamulus is beneficial, detrimental, or neutral with respect to intraoperative and perioperative complications, hearing outcome, and speech outcome., Design: Prospective, alternating., Setting: Institutional, tertiary cleft palate center, Chang Gung Memorial Hospital, Taipei, Taiwan., Participants: A total of 173 patients enrolled in the study, of whom 161 had charts available for analysis., Interventions: During the performance of palatoplasty, 85 patients received hamulus fracture and 76 patients did not. All palatoplasties were performed by the same surgeon., Main Outcome Measures: (1) Surgical outcomes, including patient demographic data, palatoplasty type and duration, blood loss, incidences of oronasal fistulae, temporary mucosal dehiscence, and postoperative bleeding; (2) otolaryngological outcomes, including hearing results as judged by auditory brainstem response testing, myringotomy tube data describing rates of tube extrusion, and culture results from sampled effusions; and (3) preliminary speech outcomes as described by judgments of overall velopharyngeal function from perceptual speech samples., Results: No statistically significant differences in any of the measured surgical, otolaryngological, or preliminary speech outcomes were found between the groups who did and did not receive hamulus fracture., Conclusions: On the basis of these results, we are unable to advocate the performance of hamulus fracture as an operative maneuver during the performance of primary palatoplasty. The historical rationale and theoretical advantage of this maneuver have not been demonstrated here nor have any detrimental effects of the maneuver been measured.