Singh, Davinder

Background: Tranexamic acid (TXA) is widely used in craniosynostosis surgery to reduce blood loss. Although the efficacy of TXA administration has been documented in the literature, dosing in the perioperative setting is variable and without a standard protocol. The purpose of this study is to determine the efficacy of continuing TXA postoperatively for patients undergoing suturectomy for metopic or unicoronal synostosis. Methods: We conducted a retrospective cohort study evaluating the use of postoperative TXA infusion in patients undergoing suturectomy for metopic and unicoronal craniosynostosis. All patients received a TXA bolus of 10 mg/kg at the time of surgical prep, followed by a TXA infusion at 5 mg/kg/hour during the procedure. Patients undergoing suturectomy for metopic or unicoronal synostosis in 2020 to 2021 received postoperative TXA infusion at 5 mg/kg/hour for the first 4 hours after surgery. Following a protocol change in anemia management at the institution in 2022, patients undergoing this surgery in 2022 did not receive a postoperative TXA infusion. Chart review was performed, and mean hemoglobin values were compared between patients who received postoperative TXA and those who did not, with analyses stratified by suture involvement to determine the impact of the protocol change. Results: Twenty-eight patients met the inclusion criteria: 14 patients received postoperative TXA, and 14 patients did not. Both groups included 8 patients undergoing metopic suturectomy and 6 patients undergoing unicoronal suturectomy. For patients who underwent metopic suturectomy and received postoperative TXA, the mean preoperative hemoglobin was 11.4 g/dL, and on postoperative day 1 (POD1) was 8.1 g/dL. For patients who underwent metopic suturectomy and did not receive postoperative TXA, the mean preoperative hemoglobin was 11 g/dL and on POD1 was 9.2 g/dL ( P =0.27). The differences in mean preoperative and postoperative hemoglobin were not statistically significant between the 2 cohorts, P =0.15 and P =0.20, respectively. For patients who underwent unicoronal suturectomy and received postoperative TXA, the mean preoperative hemoglobin was 12 g/dL, and on POD1 was 8.7 g/dL. For patients who underwent unicoronal suturectomy and did not receive postoperative TXA, the mean preoperative hemoglobin was 11.4 g/dL and on POD1 was 9.1 g/dL ( P =0.36). The mean preoperative and postoperative hemoglobin were not statistically different between the 2 cohorts, P =0.42 and P =0.92, respectively. Hospital length of stay averaged 1 day across both cohorts. No patient received a postoperative blood transfusion. Conclusion: In patients undergoing suturectomy for metopic or unicoronal craniosynostosis, postoperative TXA infusion made no significant difference in postoperative hemoglobin levels, length of stay, or postoperative transfusion. Discontinuing postoperative TXA could potentially decrease monitoring needs and lower hospital costs.

Objective To evaluate the incidence and characteristics of secondary synostosis following spring-mediated cranioplasty (SMC) for nonsyndromic sagittal craniosynostosis. Design Retrospective cohort study. Setting Single tertiary pediatric hospital. Patients/Participants Ten patients with isolated sagittal craniosynostosis who underwent primary SMC between 2021 and 2023. Patients with syndromic diagnoses or prior cranial surgery were excluded. Interventions SMC was performed using 2 to 3 stainless-steel springs following sagittal strip craniectomy. Springs were typically removed 3 to 4 months postoperatively. Follow-up included review of clinical photographs, radiographs, and operative records. Main Outcome Measures Change in cephalic index (CI) from preoperative to postoperative assessment and occurrence of secondary suture fusion, particularly coronal synostosis. Results Mean age at surgery was 4 months (range, 3-6 months). Mean CI improved from 68.5 to 79 following expansion. Three patients (30%) developed left unicoronal synostosis (UCS) during the perioperative period prior to spring removal. One patient required secondary coronal suturectomy for significant frontal asymmetry, while 2 were observed without intervention. Conclusions Secondary synostosis, particularly UCS, may represent an underrecognized sequela of SMC. Altered biomechanical forces or asymmetric cranial remodeling during distraction may contribute to this finding. Larger, multicenter studies with longitudinal imaging are warranted to determine incidence, risk factors, and preventive strategies.

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.

Objective: Our objective is to examine which sociodemographic factors may influence the time of presentation of patients with craniosynostosis, which can impact perioperative outcomes. Design: Retrospective cohort study. Setting: Tertiary pediatric hospital. Patients, Participants: All patients with nonsyndromic sagittal synostosis who underwent surgery at a tertiary pediatric hospital from January 1, 2007 to January 2, 2024 were eligible for inclusion. Interventions: Comparing demographic variables between those undergoing early and late craniosynostosis repair. Main Outcome Measure(s): Demographic variables, social vulnerability index (SVI) and area deprivation index (ADI), median household income, education rate, and employment rates. Results: 77.5% (n = 234) of patients underwent minimally invasive repair. 22.5% (n = 68) underwent open cranial vault remodeling. Age at consultation differed significantly between those undergoing early repair compared to late repair (0.18 years v. 1.01 years, P < .0001). Patients undergoing late repair were more likely to be Hispanic (42.6% v. 24.4%, P = .0378), on public insurance (66.2% v. 45.3%, P = .0079), have lower parental education rates (24.78% vs. 31.64%, P = .0019), and lower median household income ($65 657 vs. $75 281, P = .0226). Interestingly, ADI scores and SVI scores did not significantly differ between groups. Conclusions: Patients who presented later for treatment of nonsyndromic sagittal synostosis had lower median household income, were more likely to be Hispanic, had lower parental education rates, and were more likely to be on public insurance compared to those who received early treatment.

Objective: Describe changes in symptoms of velopharyngeal insufficiency (VPI)—hypernasality, audible nasal emission, and VPI-related quality of life—following LeFort I osteotomy in individuals with cleft palate +/- cleft lip. Design: Single-center, retrospective cohort study. Participants: Twenty-one patients with a history of cleft palate repaired in infancy were included in this study. Ten patients had no prior history of VPI surgery and eleven had a pharyngeal flap in place at the time of LeFort I osteotomy. The mean age at time of surgery was 17.2 years. Main Outcome Measure: Pre- and postoperative hypernasality severity, frequency of audible nasal emission, and total score on the VPI Effects on Life Outcomes youth questionnaire (VELO-Y) before and after LeFort osteotomy I. Fischer exact test was used to assess differences between groups for categorical variables and a t test was used for continuous variables. Results: Patients with no prior history of VPI management had a significant increase in hypernasality severity compared with those with a pharyngeal flap in place at the time of surgery ( P =0.004). Patients without a prior history of VPI management also had a significant increase in frequency of audible nasal emission, while patients with a pharyngeal flap had decreased audible nasal emission ( P =0.004). There was not a significant difference between groups for change in the total score on the VELO-Y ( P =0.16). Conclusions: Findings from this study may improve preoperative counseling provided to patients and caregivers on the risk of acquiring VPI after LeFort I osteotomy based on prior VPI surgical history.

Background: Positioning the pharyngeal flap base high along the posterior pharyngeal wall is essential for optimizing speech outcomes. Objective data on where to place the flap base are lacking. Further, adenoid tissue can restrict cephalad positioning of the flap. This study aimed to improve the design of the pharyngeal flap by measuring the distance from the first cervical vertebrae (C1) to the palatal plane, and the adenoid depth in children undergoing evaluation for velopharyngeal insufficiency. Methods: This retrospective cross-sectional study analyzed magnetic resonance imaging (MRI) scans of the velopharynx and measured the distance between C1 and the palatal plane, and the adenoid depth at the level of the palatal plane in millimeters. Results: Thirty-four patients met the inclusion criteria. The mean age at the time of MRI was 7.4 years (range: 3.9–11.9 y). The anterior tubercle of C1 was below the palatal plane in 97% (n = 33) of patients. On average, this landmark was 10.5 mm (SD = 5.0) below the palatal plane. Adenoid tissue was present at the level of the palatal plane in 91% (n = 31) of patients. Conclusions: Positioning the pharyngeal flap base at C1 is too low to aid with velopharyngeal closure. Further, adenoid tissue is frequently present at the level of velopharyngeal closure, limiting superior positioning of the pharyngeal flap base. When this occurs, surgeons should consider adenoidectomy before pharyngeal flap surgery. Preoperative MRI may be beneficial for planning pharyngeal flap positioning relative to C1 and assessing adenoid tissue at the palatal plane.

Purpose: The aim of this study was to investigate whether successful surgical management of velopharyngeal insufficiency (VPI) aids in the remediation of compensatory misarticulation errors (CMAs) among children with VPI and CMAs. Method: Fourteen participants with VPI and use of CMAs from a larger study were included in this retrospective cohort study. The mean age at the time of preoperative evaluation was 8.9 years ( SD = 1.1). Perceptual ratings of hypernasality, phonetic transcription, and anatomic measurements from magnetic resonance imaging were performed by raters blinded to the participants' medical and surgical history. The mean percentage of CMAs produced on the American English Sentence Sample was calculated. The Wilcoxon signed-ranks test was used to compare the change in CMA use pre- and postoperatively. During the study period, 71% ( n = 10) of participants received speech therapy. Results: Nine participants had resolved hypernasality after surgery, and five had persistent hypernasality. Among those with resolved hypernasality, the mean percentage of CMAs significantly decreased from 14.6% preoperatively to 1.1% postoperatively ( p = .028). For participants with persistent hypernasality, the mean percentage of CMAs decreased from 27.6% to 22%; this change was not significant ( p = .586). Conclusions: Correction of VPI may aid in the remediation of CMAs as participants have more normal anatomy to achieve velopharyngeal closure. These findings suggest correction of VPI may reduce the amount of speech therapy needed to treat CMAs.

Objective Describe surgical decision making and outcomes in a series of patients with persistent VPI after pharyngeal flap placement that were all treated with revision palatoplasty. Design Retrospective, case series. Participants Five patients with nonsyndromic cleft palate and persistent hypernasality following a pharyngeal flap. On MRI, 3 patients presented with an incohesive levator veli palatini muscle, and 4 patients had a pharyngeal flap that was below the palatal plane. Interventions One patient underwent straight-line intravelar veloplasty (IVVP), 2 patients underwent pharyngeal flap take-down and IVVP, and 2 patients underwent pharyngeal flap take-down and palate lengthening with buccal myomucosal flaps. Main Outcome Measure Pre- and postoperative resonance. Results Four of five patients (80%) achieved normal resonance 12 months postoperation. Conclusions Revision palatoplasty may be an effective approach for treating patients with persistent hypernasality following a pharyngeal flap. MRI may aid in surgical selection based on patient-specific anatomical findings.

Objective Evaluate the effectiveness of a digital home monitoring program for infants with cleft palate with or without cleft lip (CP ± L), compared to monitoring through in-person clinic visits. Design Retrospective cohort study. Setting One metropolitan pediatric hospital. Patients Eight-eight infants with CP ± L: 41 infants received digital home monitoring and 47 infants were monitored solely through in-person visits. Interventions Beginning in September 2022, all infants with CP ± L were enrolled in a digital home monitoring program, in which caregivers weighed their child weekly at home and submitted those weights, along with subjective evaluations of their child's feeding, using a secure website. Submissions were monitored by the cleft team nurse coordinator. Main Outcome Measures The primary outcome was the incidence of malnutrition at 4 months of age. The secondary outcome was the average number of in-person clinic visits during the first 4 months of life. Results There was not a significant difference ( P = .764) in the incidence of malnutrition between infants monitored in-person compared to infants in the home monitoring program (13% vs 17%). Infants in the home monitoring program had fewer in-person visits with speech–language pathology (5.4 vs 3.9; P < .001). Across groups, malnutrition was associated with Child Protective Services involvement ( P = .001) and presence of a syndrome ( P = .014). Conclusions The digital home monitoring program did not decrease the incidence of malnutrition, but it did reduce the number of speech–language pathology in-person visits. The program appears to distinguish infants who are gaining weight appropriately from those with feeding challenges, but it does not fully address the multifactorial contributors to malnutrition.

Purpose: The purpose of this study was to investigate the relationship between perceptual ratings of hypernasality made during connected speech and velopharyngeal (VP) gap size measured in millimeters in the sagittal plane during sustained vowel production using magnetic resonance imaging (MRI). Method: A retrospective cross-sectional analysis was completed. A subgroup of 110 participants from another study with an M age of 10.1 years presenting for management of VP insufficiency was included. Perceptual ratings of hypernasality during connected speech and measurement of gap size during sustained /i/ production on MRI were performed by raters blinded to the participants' medical and surgical history. Results: There was a moderate-to-strong, positive correlation ( r = .61; p < .001) between hypernasality ratings and VP gap size measured on MRI using sustained /i/. The odds of a higher hypernasality rating increased as the gap size increased (odds ratio = 1.34; 95% CI [1.20, 1.49]; p < .001). The predicted probability for hypernasality ratings of none/minimal/mild steadily decreased as the gap size increased indicating that lower ratings of hypernasality were associated with smaller gap sizes. For the rating of “moderate” hypernasality, the predicted probability of the rating steadily increased up to 8 mm and then decreased as the gap size continued to increase. The predicted probability for a hypernasality rating of “severe” consistently increased as the gap size increased. Conclusions: Hypernasality ratings made at the connected speech level were significantly associated with VP gap size as measured during sustained vowel production. These findings suggest sustained vowel production elicited on MRI may adequately characterize VP gap size in the evaluation of VP insufficiency.