Citations

Objective Assess if velopharyngeal gap size during speech is associated with consonant accuracy and whether hypernasality severity and compensatory misarticulation errors (CMAs) mediate this relationship. Design Cross-sectional. Setting Metropolitan hospital. Participants Eighty-one patients undergoing assessment for velopharyngeal insufficiency (VPI). Assessment(s) Each patient completed a preoperative magnetic resonance imaging protocol during sustained /i/ production and a perceptual speech evaluation. Velopharyngeal gap size was measured in millimeters. Hypernasality was rated using the Cleft Audit Protocol for Speech-Augmented-Americleft Modification. Percent consonants correct (PCC) and percent of CMAs used were derived from transcriptions of the American English sentence or phrase sample. Main Outcome Measure(s) A generalized structural equation model was used to examine whether velopharyngeal gap size is associated with PCC indirectly through hypernasality severity and CMA use. Results Larger velopharyngeal gap sizes were associated with more severe hypernasality ( P < .001), greater hypernasality severity was associated with increased CMA use ( P < .001), and greater CMA use was associated with lower PCC ( P < .001). Velopharyngeal gap size was not directly associated with PCC ( P = .400). However, the full indirect pathway from gap size to PCC through hypernasality severity and CMA use was significant ( P = .001); gap size was significantly associated with PCC when accounting for both direct and indirect pathways combined ( P = .005). Conclusions These findings suggest hypernasality severity is the key mediator between gap size and speech accuracy. Patients with more severe hypernasality may benefit from timely surgical remediation of VPI to increase overall consonant accuracy.

Purpose: Nasopharyngoscopy is an imaging method used to visualize the velopharyngeal mechanism. To quantify the movement of the mechanism during speech, an international working group recommended standardized rating guidelines. While these guidelines are widely cited in research, the extent to which cleft teams apply them in a clinical setting is unclear. Method: This cross-sectional study examined clinical nasopharyngoscopy reports from 11 cleft teams in North America. Descriptive statistics were used to summarize the proportion of reports that included ratings for the extent of velar movement, left and right lateral pharyngeal wall movement, gap size, and a surgical recommendation. Fisher's exact and chi-square tests were used to assess differences in reporting by cleft team and whether the inclusion of quantitative ratings was associated with a surgical recommendation being included. Results: A total of 188 nasopharyngoscopy reports were included. The extent of velar movement was reported in 68% ( n = 127) of reports, the left and right lateral pharyngeal wall movement was reported in 38% ( n = 71), and the size of the gap was reported in 70% ( n = 132). Only 34% ( n = 63) of reports included all three quantitative ratings. Inclusion of all three quantitative ratings varied significantly ( p < .001) across teams. Teams either never included all the ratings ( n = 4/11) or inconsistently included all ratings ( n = 7/11). Conclusions: Findings suggest that what is documented clinically in nasopharyngoscopy reports varies both within and across teams. To achieve more consistent reporting within individual teams, providers may consider implementing standardized clinical templates. To achieve consistency across teams, these findings suggest a need for renewed efforts at developing consensus on the nasopharyngoscopy elements important to clinicians.

Objective Assess the patient compliance rate for nasopharyngoscopy as documented in clinical reports. Design Cross-sectional. Setting Thirteen cleft teams in North America. Patients Patients aged 3 to 21 years old with a repaired cleft palate. Interventions Nasopharyngoscopy. Main Outcome Measure Patient compliance rate for nasopharyngoscopy. Results Patient compliance was documented in 128 of 158 reports (81%). Of the 128 reports, patient compliance was reported as “good/excellent” in 65% (n = 83), “marginal/fair” across 10% (n = 13), “poor” in 16% (n = 20), and “other” in 9% (n = 12). Patients with “poor” compliance had lower rates of documented imaging ratings, however 55% (n = 11) of reports included a surgical recommendation. Conclusions Among children completing nasopharyngoscopy for velopharyngeal insufficiency surgery planning, at least one-fourth of reports indicate significant compliance issues that limits velopharyngeal port imaging. These findings suggest that some cleft teams and surgeons may be proceeding with surgical management of velopharyngeal insufficiency without adequate visualization or ratings of velopharyngeal anatomy.

Objective To assess the impact of renal replacement therapy in people with lupus nephritis (LN)–associated end‐stage kidney disease (ESKD) and support the development of the 2024 American College of Rheumatology LN treatment guidelines. Methods We conducted a systematic literature review and meta‐analysis to address three Population, Intervention, Comparison, and Outcome (PICO) questions related to renal replacement therapy for ESKD due to LN, including comparisons of kidney transplant versus dialysis, hemodialysis versus peritoneal dialysis, and preemptive kidney transplant versus no preemptive kidney transplant. Outcomes of interest included mortality, cardiovascular (CV) events, infections, lupus flares, disease‐related damage, graft failure, and quality of life. We conducted a meta‐analysis and analyzed hazard ratios for time‐to‐event analyses and risk ratios for dichotomous outcomes, as well as absolute risk estimates. Results Sixteen comparative observational studies addressed at least one of the three PICO questions. Kidney transplant was found to reduce the risks of all‐cause mortality, CV mortality, infection‐related mortality, and CV events compared with dialysis (high certainty). Dialysis modality (peritoneal vs hemodialysis) was not associated with mortality (high certainty) or with other outcomes of infection, CV complications, and systemic lupus erythematosus flares (low certainty). Preemptive kidney transplant was associated with lower risks of graft failure and mortality (low certainty). Conclusion This systematic review identified improved outcomes with kidney transplant versus dialysis for people with LN‐associated ESKD and potential benefits of preemptive kidney transplant. This evidence supports the use of kidney transplant as a preferred renal replacement therapy for people with LN‐ESKD.

Objective To assess the relationship in ratings of velopharyngeal closure between connected speech and sustained speech, as visualized on nasopharyngoscopy. Design Prospective observational study. Setting Craniofacial clinics in the United States and Canada. Participants Fifty-two participants with velopharyngeal insufficiency (VPI) who underwent nasopharyngoscopy and produced both connected speech and sustained speech of either /i/or /s/ during the procedure. Main outcome measure(s) Correlations and differences between connected speech and sustained speech for velopharyngeal closure percentage, velar movement, lateral pharyngeal wall movement, and velopharyngeal closure pattern. Results There were moderate to very strong correlations observed between connected speech and sustained /i/ for velopharyngeal closure percentage ( P < .01); extent of velar movement ( P < .01), and left ( P < .01) and right ( P < .01) lateral pharyngeal wall movement. Findings were similar between connected speech and sustained /s/. Differences between ratings made during connected and sustained speech were small: for velopharyngeal closure percentage, there was a 5% difference in the mean between connected speech and sustained /i/ (77.5% vs 72.5%) and a 7% difference for /s/ (79.3% vs 86.7%). Overall differences across ratings ranged from 2% to 12%. Closure pattern was rated the same for both connected speech and sustained /i/ in 90% of videos (n = 44/49; P = .38) and the same for connected speech and sustained /s/ in 95% of videos (n = 42/44; P = 1.00). Conclusions Ratings of velopharyngeal movement during nasopharyngoscopy are similar between connected speech and sustained /i/ and /s/ suggesting elicitation of only sustained /i/ and /s/ may be adequate in most cases when assessment of connected speech is not possible.

Purpose: The purpose of this study was to compare the height of velopharyngeal (VP) closure in relation to the palatal plane between individuals with velopharyngeal insufficiency (VPI) with or without cleft palate and individuals without cleft palate and with normal speech, considering the effects of age and sex. Method: Magnetic resonance imaging was used to analyze the height of VP closure in the midsagittal view during phonation of /i/ and /s/ sounds among 105 individuals with VPI and 45 individuals without cleft palate and normal speech. Results: Independent-samples t tests demonstrated that individuals with VPI display a significantly lower mean height of VP closure (3.7 mm) compared to individuals without cleft palate and normal speech during phonation of /i/ and /s/ sounds ( p < .001). Analysis of variance was used to evaluate the impact of age and sex on the height of VP closure. Significant differences were observed between the height of VP closure in individuals with VPI and those without cleft palate and normal speech across the age span during the production of /i/ and /s/ sounds. However, nonsignificant differences were found between the height of VP closure in males and females of the VPI group and those without cleft palate and normal speech during the production of these sounds. Conclusions: The results revealed the variations of the height of VP closure in relation to the palatal plane. These findings provide insights into the presurgical planning for individuals with VPI undergoing palatal surgeries, especially the location of pharyngeal flap insertion.

Objective To provide evidence‐based and expert guidance for the treatment and management of non‐renal systemic lupus erythematosus (SLE); treatment and management of lupus nephritis are addressed in a separate guideline. Methods Clinical questions for treatment and management of SLE were developed in the PICO format (population, intervention, comparator, and outcome). Systematic literature reviews were developed for each PICO question, and the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to assess evidence quality and formulate recommendations. The Voting Panel achieved a consensus of ≥70% agreement on the direction (for or against) and strength (strong or conditional) of each recommendation. Results We present recommendations and ungraded, consensus‐based good practice statements for the treatment and management of SLE that are applicable to pediatric and adult patients. Recommendations emphasize uniform treatment with hydroxychloroquine, limiting duration of glucocorticoid use, and early introduction of conventional and/or biologic immunosuppressive therapies to achieve and maintain control of SLE inflammation (remission or a low level of disease activity), reduce SLE‐related morbidity and mortality, and minimize medication‐related toxicities. Conclusion This guideline presents direction regarding treatment and management of SLE and provides a foundation for well‐informed, shared clinician–patient decision‐making. These recommendations should not be used to limit or deny access to therapies, as treatment decisions may vary due to the unique clinical situation and personal preferences of each person with SLE.

Objective To provide evidence‐based and expert guidance for the treatment and management of non‐renal systemic lupus erythematosus (SLE); treatment and management of lupus nephritis are addressed in a separate guideline. Methods Clinical questions for treatment and management of SLE were developed in the PICO format (population, intervention, comparator, and outcome). Systematic literature reviews were developed for each PICO question, and the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to assess evidence quality and formulate recommendations. The Voting Panel achieved a consensus of ≥70% agreement on the direction (for or against) and strength (strong or conditional) of each recommendation. Results We present recommendations and ungraded, consensus‐based good practice statements for the treatment and management of SLE that are applicable to pediatric and adult patients. Recommendations emphasize uniform treatment with hydroxychloroquine, limiting duration of glucocorticoid use, and early introduction of conventional and/or biologic immunosuppressive therapies to achieve and maintain control of SLE inflammation (remission or a low level of disease activity), reduce SLE‐related morbidity and mortality, and minimize medication‐related toxicities. Conclusion This guideline presents direction regarding treatment and management of SLE and provides a foundation for well‐informed, shared clinician–patient decision‐making. These recommendations should not be used to limit or deny access to therapies, as treatment decisions may vary due to the unique clinical situation and personal preferences of each person with SLE.

Objective Prior work has identified numerous barriers to standardized outcome measurement in cleft care. The objective of this study was to evaluate a multifaceted implementation strategy to collect standardized outcomes for children with cleft lip and/or palate (CL/P). Design A one-armed, single-site pilot study of a multifaceted implementation strategy. Setting A metropolitan children's hospital. Patients Five-year-old children with CL/P. Interventions A multifaceted implementation strategy for standardized outcome measurement. Main Outcomes Measures The primary outcome measure was penetration of the intervention, defined as the proportion of children for whom the standardized measures of aesthetic, dental, and speech outcomes were collected. Penetration pre-implementation and 12 months post-implementation were compared. The secondary outcome was acceptability of standardized outcome measurement among providers and staff, evaluated with the Acceptability of Intervention Measure. Results The implementation strategy resulted in high penetration of standardized outcome measurement: measurement of aesthetic outcomes increased from 7% to 82% ( p < .0001); measurement of dental outcomes increased from 0% to 56% ( p < .0001); measurement of speech outcomes increased from 29% to 77% ( p = .0001). Acceptability of standardized outcome measurement was high, with 93% of cleft team providers and staff (n = 14/15) reporting that they somewhat or completely agreed that standardized outcome measurement met their approval, was appealing, was welcomed, and was liked. Penetration continued to improve after the implementation period, with high rates of outcome measure collection sustained for four years following implementation. Conclusions Deployment of a multifaceted implementation strategy led to substantial increases in the collection of aesthetic, dental, and speech outcomes among children with CL/P.

Background: There remains significant uncertainty about the fistula rate following palate repair with the Furlow double opposing Z-plasty (Furlow DOZ) technique compared with a straight-line/mucosal incision with intravelar veloplasty (IVVP) technique. Moreover, the relative impact of technique and surgical skill on fistula incidence remains unclear. Methods: A prospective, observational study of cleft palate repair was conducted. Enrollment occurred at 18 U.S.-based cleft centers, including 64 cleft surgeons. The primary outcome was postoperative fistula. Results: Overall, 1219 participants underwent cleft palate repair, 1178 of whom presented for follow-up. Of these, 581 (49.3%) underwent palate repair with the Furlow DOZ technique, 483 (41.0%) with the straight-line/IVVP technique, and 114 (9.7%) with an alternative technique. The overall fistula incidence was 5.3% (62 of 1162 analyzed for fistula occurrence). The fistula incidence was significantly lower in the Furlow DOZ group (2.4% [14 of 564]) than in the straight-line/IVVP group (7.8% [37 of 427]), with a propensity score–weighted risk difference of 5.7% (95% CI, 1.6% to 9.9%; P = 0.007). The fistula incidence varied substantially among surgeons: the top quartile’s fistula rate was 0% (0 fistulas among 417 repairs), whereas the bottom quartile’s fistula rate was 18.9% (31 fistulas among 164 repairs). Conclusions: Fistula incidence following cleft palate repair was significantly lower when using the Furlow DOZ technique, compared with the straight-line/IVVP technique. The effect of surgical technique was smaller, however, than the variation observed between the top and bottom quartiles of surgeons. These findings suggest that changing surgical technique may lead to small changes in fistula incidence, but greater opportunities exist to improve outcomes by improving surgical skill.