The Cleft Palate Craniofacial Journal

To compare dental arch relationship, craniofacial form, and nasolabial aesthetic outcomes among cleft centers using distinct methods of presurgical infant orthopedics (PSIO).

Retrospective cohort study.

Four cleft centers in North America.

One hundred ninety-one children with repaired complete unilateral cleft lip and palate (CUCLP).

Dental arch relationship was assessed using the GOSLON Yardstick. Craniofacial form was assessed by 12 cephalometric measurements. Nasolabial aesthetics were assessed using the Asher-McDade system. Assessments were performed between 6 and 12 years of age.

The center that used no PSIO achieved the most favorable dental arch relationship and maxillomandibular relationship, with a median GOSLON score of 2.3 (P < .01) and an ANB angle of 5.1° (P < .05). The proportion of children assigned a GOSLON score of 4 or 5, predictive of the need for orthognathic surgery in adolescence, was 16% at the center that used no PSIO and no secondary surgery, compared to 76% at the centers that used the Latham appliance and early secondary lip and nose surgery (P < .01). The center that used no PSIO and no secondary surgery achieved significantly less favorable nasolabial aesthetic outcomes than the centers using Latham appliance or nasoalveolar molding (NAM) (P < .01).

Effects of active PSIO are multifaceted and intertwined with use of revision surgery. In our study, centers using either the Latham appliance combined with early revision surgery or the NAM appliance without revision surgery achieved better nasolabial aesthetic outcomes but worse maxillary growth, compared to a center using no PSIO and secondary surgery.

This study uses administrative data to assess the optimal timing for surgical repair of craniosynostosis and to identify factors associated with risk of perioperative complications.

Statistical analysis of the Healthcare Cost and Utilization Project Kids’ Inpatient Database (2006, 2009, 2012).

KID-participating hospitals in 44 states. Patients: Children 0 to 3 years of age with ICD-9 codes for surgical correction of craniosynostosis (756 and 0124, 0125, 0201, 0203, 0204, or 0206).

Age-based cohorts were assessed for perioperative complications. We performed a multivariable analysis to determine characteristics associated with increased risk of complications.

21 million admissions were screened and 8417 visits met criteria for inclusion. Seventy-five percent of procedures occurred before age 1. Complications occurred in 8.6% of patients: 6.6% of patients at age 0 to 6 months, 10.3% of patients aged 7 to 12 months, and 13.9% of patients 12 to 36 months. Patients with acrocephalosyndactyly or associated congenital anomalies experienced complications in 22.9% of cases (OR = 3.07, 95% CI = 2.33, 4.03).

Craniosynostosis repair is safe; however, the risk of complications increases with age at intervention. Presence of a syndromic congenital deformity at any age carries the greatest increased risk of perioperative complications. This suggests that optimal timing of intervention is within the first year of life, especially in those cases with additional factors increasing perioperative risk. These data support the importance of counseling patients of the increased risk associated with delaying craniosynostosis repair.

To compare nasolabial appearance outcomes of patients with complete unilateral cleft lip and palate (CUCLP) in preadolescence from 4 cleft centers including a center using nasoalveolar molding (NAM) and primary nasal reconstruction.

Retrospective cohort study.

Four cleft centers in North America.

135 subjects with repaired CUCLP.

Frontal and profile facial pictures were assessed using the Asher-McDade rating scale. Intra- and interrater reliability were tested using weighted Kappa statistics. Median scores by center were compared with Kruskal-Wallis statistics.

Intrarater reliability scores were moderate to good. Interrater reliability scores were moderate. Significant differences (P < .05) among centers were found. For nasal form, center G (median = 2.83) had better scores than centers C and D (C median = 3.33, D median = 3.17). For nose symmetry, center G had better scores (median = 2.33) than all other centers (B median = 2.67, C median = 2.83, D median = 2.83). For vermillion border, center G had better scores (median = 2.58) than centers B and C (B median = 3.17, C median = 3.17). For nasolabial profile, center G (median score = 2.67) had better scores than center C (median = 3.00). For total nasolabial score, center G (median = 2.67) had better scores than all other centers (B median = 2.83, C median = 3, D median = 2.83).

The protocol followed by center G, the only center that performed NAM and primary nasal reconstruction, produced better results in all categories when compared to center C, the only center that did not perform presurgical orthopedics or lip/nose revisions. When compared to centers that performed traditional presurgical orthopedics and surgical revisions (B and D), center G was not consistently better in all categories. As with other uncontrolled, retrospective intercenter studies, it is not possible to attribute the outcomes to a specific protocol component.

The objective was to assess differences in psychosocial adjustment between adolescents with and without craniofacial microsomia (CFM).

This is a case-control follow-up study in adolescents with and without CFM.

Participants were originally recruited as infants from 26 cities across the United States and Canada.

Participants included 142 adolescents with CFM (cases) and 316 peers without CFM (controls), their caregivers, and their teachers.

Social and behavior measures from the Achenbach System of Empirically Based Assessments (ASEBA), the PedsQL: Core Version, and the Children’s Communication Checklist–2nd edition (CCC-2) were used. Linear regression was used to estimate case-control differences and corresponding standardized effect sizes (ES) and 95% confidence intervals after adjustment for sociodemographic confounds. We also examined case-control differences by facial phenotype and hearing status.

The magnitude and direction of case-control differences varied across assessment and respondent, but were generally modest (ES = –0.4 to 0.02, P values ranged from .003 to .85). There was little evidence for variation in case-control differences across different facial phenotypes or as a function of hearing status.

Our results suggest that in spite of multiple risk factors, adolescents with CFM exhibit behavior problems no more frequently than their peers without CFM. Future studies of individuals with CFM should focus on resilience and social coping mechanisms, in addition to maladjustment.

Since the implementation of centralized services in the United Kingdom for those affected by cleft lip and/or palate (CL/P), several studies have investigated the impact of service rationalization on the delivery of care. While large-scale quantitative studies have demonstrated improvements in a range of patient outcomes, and smaller studies have reported on the benefits and challenges of centralization from the views of health professionals, little research has attempted to capture the patient perspective. Furthermore, few studies have investigated the views of adult “returners” who have undergone treatment both pre- and postcentralization.

Qualitative data relevant to the subject of this article were extracted from 2 previous larger studies carried out between January 2013 and March 2014. A total of 16 adults born with CL/P contributed data to the current study. These data were subjected to inductive thematic analysis.

The findings suggest that centralization of CL/P services has considerably enhanced the patient experience. Specifically, the overall standard and coordination of care has improved, service delivery has become more patient centered, and access to professional psychological support and peer support has greatly improved patients’ capacity to cope with the associated emotional challenges.

The data collected provide additional insight into the impact of centralization from the perspective of a largely unexplored patient population. In combination with other literature, these findings are also relevant to future efforts to centralize other specialist services around the world.

The aim of this study is to assess the treatment outcome of complete unilateral cleft lip and palate (CUCLP) patients using modified Huddart/Bodenham scoring system (MHB). To determine whether there is an association of congenital and postnatal factors with the treatment outcome.

Retrospective observational study.

Two regional cleft-referral centers.

In the current study, 101 pairs of dental models of non-syndromic CUCLP patients were retrieved from hospital archives. Each occlusal relationship from central incisor till the first permanent molars were scored except the lateral incisor. Sum of 10 occlusal relationships in each study sample gave a total occlusion score. The primary outcome was the mean total occlusion score.

According to MHB, a mean (standard deviation) total occlusion score of –8.92 (6.89) was determined. Based on treatment outcome, 66 cases were favorable (grades 1, 2, and 3) and 35 cases were unfavorable (grades 4 and 5). Chi-square tests indicated, difference of cheiloplasty (P = .001) and palatoplasty (P < .001) statistically significant. Five variables—gender, family history of cleft, cleft side, cheiloplasty, and palatoplasty—were analyzed with a logistic regression model.

Final model indicated that cases treated with modified Millard technique (cheiloplasty) and Veau-Wardill-Kilner method (palatoplasty) had higher odds of unfavorable treatment outcome.

Dexmedetomidine is a parenteral agent that combines the benefits of cooperative sedation, anxiolysis, and analgesia without the risks of respiratory depression. Off-label use has been reported in children. We have introduced dexmedetomidine for use in patients having undergone alveolar bone graft (ABG). The objective is to demonstrate the value and safety of postoperative dexmedetomidine infusion in a non-ICU setting following ABG.

A retrospective review was performed on patients who underwent ABG by the senior author. Patients were divided into 2 groups: those who received postoperative dexmedetomidine and those who received patient-controlled anesthesia.

The primary study outcome measures included patient demographics, adverse events, length of stay, pain scores, and doses of narcotics during admission were collected.

Inclusion criteria were met by 54 patients; 39 received dexmedetomidine whereas 15 did not. There were no significant differences between groups in age, gender, and length of stay. The patients who received dexmedetomidine used oral narcotics less often (P = .01). In addition, more patients reported no pain after surgery (P = .05) and at the time of discharge if they received dexmedetomidine (P < .01). There were no reported adverse effects.

Dexmedetomidine provided superior pain control after surgery and at the time of discharge, as well as a significant decrease in the use of oral narcotics. In our institution, it has since replaced the PCA as a postoperative pain control modality. Absent the risk for respiratory depression, dexmedetomidine has demonstrated a safe option for postoperative pain control in our focused group of pediatric patients.

To investigate whether awake endoscopy can diagnose base-of-tongue obstruction as reliably as sleep endoscopy in infants with Pierre Robin sequence (PRS).

The study was retrospective with the clinicians blinded to patient identity. Endoscopy findings were assessed and measured by the performing pediatric otolaryngologist.

Tertiary care children’s hospital.

All infants with PRS managed between January 2005 and July 2015 were included. There were 141 patients, of which 35 underwent both awake endoscopy (AE) and drug-induced sleep endoscopy (DISE).

Bedside AE and DISE in the operating room.

Presence of moderate or severe base-of-tongue collapse was assessed. Sensitivity, specificity, and positive likelihood ratio of AE findings as well as intertest differences between AE and DISE were calculated.

AE had 50.0% sensitivity (95% confidence interval [CI] 27.2%-72.8%) and 86.7% specificity (95% CI 59.5%-98.3%) for detecting base-of-tongue obstruction compared to DISE; false negative rate was 50.0% (n = 10). Positive likelihood ratio was 3.75 (CI 0.96-14.65). Compared to AE, DISE demonstrated significantly more cases of base-of-tongue obstruction (P = .039).

Bedside AE has low sensitivity for detecting base-of-tongue collapse in infants with PRS. Because of the substantial false negative rate, AE may not be a reliable diagnostic modality for ruling out base-of-tongue obstruction in this susceptible population. DISE may be indicated in high-risk patients to avoid underdiagnosing upper airway obstruction.

To investigate the dimensions of the tensor veli palatini (TVP) muscle in adults with and without cleft palate.

Prospective study.

There were a total of 14 adult participants, 8 noncleft and 6 with cleft palate.

Analysis and comparison of the TVP muscle and surrounding structures was completed using 3D MRI data and Amira 5.5 Visualization Modeling software. TVP muscle volume, hamular process distance, mucosal thickness, TVP muscle length, and TVP muscle diameter were used for comparison between participant groups based upon previous research methods.

Mann-Whitney U tests revealed a significantly smaller (U < .001, P = .002) TVP muscle volume in the cleft palate group (median = 536.22 mm³) compared to individuals in the non–cleft palate group (median = 895.19 mm³). The TVP muscle was also significantly shorter (U = 1.00, P = .003) in the cleft palate group (median = 18.04 mm) versus the non–cleft palate (median = 21.18 mm). No significant differences were noted for the other measured parameters.

Significant differences in the TVP muscle volume and length among the noncleft participants found in this study may insights into the reported increased incidence of otitis media with effusion (OME) seen in the cleft population. Results from this study contribute to our understanding of the underlying anatomic differences among individuals with cleft palate.

The present study investigates the efficacy of performing a “palate rerepair” utilizing a double-opposing z-palatoplasty (DOZ) following primary Furlow palatoplasty.

Retrospective study.

Tertiary referral academic center for craniofacial surgery.

15 consecutive patients who presented with velopharyngeal insufficiency (VPI) after primary Furlow palatoplasty.

All subjects were evaluated using the perceptual speech assessment (PSA) scale. Criteria for inclusion in the study were (1) velopharyngeal gap size on phonation of 7 mm or less and (2) lateral wall motion at least 40% normal.

Mean PSA score was 7.13 + 3.31 (range 3-13) preoperatively, and decreased to 1.80 + 2.83 (range 0-11; P < .001 vs baseline) 3 months or more after surgery. A sphincter pharyngoplasty was performed as a tertiary procedure in 2 patients due to persistent nasal air emission. There was no symptomatic airway compromise following secondary or tertiary management in all patients. Presence of a cleft lip, lateral wall motion, and velopharyngeal gap size did not impact outcomes.

We have demonstrated that a DOZ performed secondarily is anatomically possible following Furlow palatoplasty and can restore function of the levator muscle despite significant scarring following primary repair. This approach respects anatomic principles of palatoplasty without eliminating the possibility for extrapalatal procedures should velopharyngeal competence not be achieved.

To compare facial expressiveness (FE) of infants with and without craniofacial microsomia (cases and controls, respectively) and to compare phenotypic variation among cases in relation to FE.

Positive and negative affect was elicited in response to standardized emotion inductions, video recorded, and manually coded from video using the Facial Action Coding System for Infants and Young Children.

Five craniofacial centers: Children’s Hospital of Los Angeles, Children’s Hospital of Philadelphia, Seattle Children’s Hospital, University of Illinois–Chicago, and University of North Carolina–Chapel Hill.

Eighty ethnically diverse 12- to 14-month-old infants.

FE was measured on a frame-by-frame basis as the sum of 9 observed facial action units (AUs) representative of positive and negative affect.

FE differed between conditions intended to elicit positive and negative affect (95% confidence interval = 0.09-0.66, P = .01). FE failed to differ between cases and controls (ES = –0.16 to –0.02, P = .47 to .92). Among cases, those with and without mandibular hypoplasia showed similar levels of FE (ES = –0.38 to 0.54, P = .10 to .66).

FE varied between positive and negative affect, and cases and controls responded similarly. Null findings for case/control differences may be attributable to a lower than anticipated prevalence of nerve palsy among cases, the selection of AUs, or the use of manual coding. In future research, we will reexamine group differences using an automated, computer vision approach that can cover a broader range of facial movements and their dynamics.

This study compares the change in midface position following Le Fort III advancement using either rigid external distraction (group 1) or internal distraction (group 2). We hypothesized that, with reference to right-facing cephalometry, internal distraction would result in increased clockwise rotation and inferior displacement of the midface.

Le Fort III osteotomies and standardized distraction protocols were performed on 10 cadaveric specimens per group. Right-facing lateral cephalograms were traced and compared across time points to determine change in position at points orbitale, anterior nasal spine (ANS), A-point, and angle ANB.

Institutional.

Twenty cadaveric head specimens.

Standard subcranial Le Fort III osteotomies were performed from a coronal approach and adequately mobilized. The specified distraction mechanism was applied and advanced by 15 mm.

Changes of position were calculated at various skeletal landmarks: orbitale, ANS, A-point, and ANB.

Group 1 demonstrated relatively uniform x-axis advancement with minimal inferior repositioning at the A-point, ANS, and orbitale. Group 2 demonstrated marked variation in x-axis advancement among the 3 points, along with a significant inferior repositioning and clockwise rotation of the midface (P < .0001).

External distraction resulted in more uniform advancement of the midface, whereas internal distraction resulted in greater clockwise rotation and inferior displacement. External distraction appears to provide increased vector control of the midface, which is important in creating a customized distraction plan based on the patient’s individual occlusal and skeletal needs.

The present study aimed to determine the structure and morphology of the maxilla in patients with cleft lip and palate to ensure safe Le Fort I osteotomy.

A total of 34 sides of 17 patients with unilateral cleft lip and palate (nonsyndromic cases) were included in this study. The control group included 10 sides of 5 patients who exhibited skeletal mandibular protrusion without malformation.

Finite element analysis was performed to examine the distribution of occlusal force over the maxilla, and continuous 3-dimensional measurement was performed at the sites of stress concentration.

In patients with cleft lip and palate, bones at the lateral border of the piriform aperture and the anterior wall of the maxillary sinus were significantly thicker than those in controls (P < .05). Furthermore, the attachment of the pterygomaxillary junction was wider and thicker (P < .05), and the anterior distance to the descending palatine artery was shorter (P < .01) in patients with cleft lip and palate than in controls. Our results further indicated that alveolar bone grafting may significantly influence bone thickness and the attachment state of the pterygomaxillary junction.

Our results suggest that the complications of LFI can be reduced in patients with cleft lip and palate by ensuring proper understanding of each patient’s maxillary anatomy and bone thickness, as well as the location of the descending palatine artery and the attachment state of the pterygomaxillary junction.

Cleft lip and/or cleft palate (CL/P) are congenital anomalies of the face and have multifactorial etiology, with both environmental and genetic risk factors playing crucial roles. Though at least 40 loci have attained genomewide significant association with nonsyndromic CL/P, these loci largely reside in noncoding regions of the human genome, and subsequent resequencing studies of neighboring candidate genes have revealed only a limited number of etiologic coding variants. The present study was conducted to identify etiologic coding variants in GREM1, a locus that has been shown to be largely associated with cleft of both lip and soft palate.

We resequenced DNA from 397 sub-Saharan Africans with CL/P and 192 controls using Sanger sequencing. Following analyses of the sequence data, we observed 2 novel coding variants in GREM1. These variants were not found in the 192 African controls and have never been previously reported in any public genetic variant database that includes more than 5000 combined African and African American controls or from the CL/P literature.

The novel variants include p.Pro164Ser in an individual with soft palate cleft only and p.Gly61Asp in an individual with bilateral cleft lip and palate. The proband with the p.Gly61Asp GREM1 variant is a van der Woude (VWS) case who also has an etiologic variant in IRF6 gene.

Our study demonstrated that there is low number of etiologic coding variants in GREM1, confirming earlier suggestions that variants in regulatory elements may largely account for the association between this locus and CL/P.

To determine the usage of otolaryngology services by children with cleft palate at a pediatric tertiary care facility.

Retrospective case series.

Specialty clinic at a pediatric tertiary care hospital.

Children born between January 1, 1999, and December 31, 2002, with the diagnosis of cleft palate or cleft lip and palate. A total of 41 female and 48 male patients were included.

Total number of otolaryngology clinic visits and total number of otolaryngologic surgeries (tympanostomy tube placements and other otologic or upper airway procedures).

In the first 5 years of life, these children utilized an average of 8.2 otolaryngology clinic visits (SD = 5.0; range: 1-22) and underwent 3.3 tympanostomy tube surgeries (SD = 2.0; range: 0-10). Seventy-three had their first tube placed at the time of palate repair, and 4 at the time of lip repair. Fifty-one (57.3%) required other otologic or upper airway procedures, including tonsillectomy and/or adenoidectomy (27 children), removal of tympanostomy tubes (24 children), tympanomastoidectomy (3 children), and tympanoplasty (14 children). Of the children who underwent other procedures, they underwent a mean of 1.67 (SD = 0.84; range: 1-4) surgeries.

Children with cleft palate are at increased risk for eustachian tube dysfunction, frequently utilize otolaryngology care, and typically receive multiple sets of tympanostomy tubes. This study found that children with cleft palate receive on average of approximately 3 sets of tympanostomy tubes, and the majority required another otologic or upper airway surgery.

To determine if cropping facial images affects nasolabial aesthetics assessments in unilateral cleft lip patients and to evaluate the effect of facial attractiveness on nasolabial evaluation.

Two cleft surgeons and one cleft orthodontist assessed standardized frontal photographs 4 times; nasolabial aesthetics were rated on cropped and full-face images using the Cleft Aesthetic Rating Scale, and total facial attractiveness was rated on full-face images with and without the nasolabial area blurred using a 5-point Likert scale.

Cleft Palate Craniofacial Unit of a University Medical Center.

Inclusion criteria: nonsyndromic unilateral cleft lip and an available frontal view photograph around 10 years of age. Exclusion criteria: a history of facial trauma and an incomplete cleft. Eighty-one photographs were available for assessment.

Differences in mean CARS scores between cropped versus full-face photographs and attractive versus unattractive rated patients were evaluated by paired t test.

Nasolabial aesthetics are scored more negatively on full-face photographs compared to cropped photographs, regardless of facial attractiveness. (Mean CARS score, nose: cropped = 2.8, full-face = 3.0, P < .001; lip: cropped = 2.4, full-face = 2.7, P < .001; nose and lip: cropped = 2.6, full-face = 2.8, P < .001).

Aesthetic outcomes of the nasolabial area are assessed significantly more positively when using cropped images compared to full-face images. For this reason, cropping images, revealing the nasolabial area only, is recommended for aesthetical assessments.

To investigate the age effects of cleft palate repair on middle ear function and hearing level in patients who underwent cleft palate repair at different ages by audiologic examination.

Medical histories were gathered in detail, and audiologic tests (ie, tympanometry and pure tone hearing threshold) were conducted in 126 patients after palatoplasty. The patients were divided into the following 4 groups according to their ages when they underwent cleft palate repair: group I (0-3 years, 73 patients), group II (4-7 years, 29 patients), group III (8-11 years, 16 patients), and group IV (12 years and older, 8 patients). The data regarding tympanograms, hearing levels, and the average hearing thresholds of each group were analyzed using chi-square tests.

The prevalence of middle ear dysfunction and hearing loss in the patients who underwent palatoplasty before 3 years old (27.4% and 2.0% respectively) was significantly lower than that in patients who underwent palatopalsty at 12 years or older (75.0% and 43.7%, respectively). Linear-by-linear association revealed that the prevalences of middle ear dysfunction and hearing loss among the 4 groups were significantly different (P < .05).

The prevalence of middle ear dysfunction and hearing loss tended to increase with advancing age at the time of cleft palate repair. From an audiologist’s perspective, palatoplasty at an early age is very beneficial in helping children with cleft palates acquire better middle ear function and hearing level.