Preoperative Velopharyngeal Closure Predicts Hypernasality Outcomes of Secondary Furlow Double-Opposing Z-Plasty.

OBJECTIVE: To determine if preoperative velopharyngeal closure percentage (VCP) is predictive of successful Furlow double opposing Z-plasty (DOZP) and subsequently determine the optimal velopharyngeal closure cutoff for successful DOZP. DESIGN: Retrospective study. SETTING: Tertiary academic center. PATIENTS: 110 patients with repaired cleft lip and palate having hypernasality treated with DOZP. INTERVENTIONS: Speech videofluoroscopy images were used to obtain the preoperative VCP and other measurements. MAIN OUTCOME MEASURES: Changes in hypernasality scores using the Cleft Audit Protocol for Speech-Augmented-Americleft Modification (CAPS-A-AM) rating system were used as the primary outcome measure. A successful DOZP was defined as a postoperative hypernasality score of ≤ 1 or an improvement of 2 or more scores from baseline. A receiver operating characteristic (ROC) curve was calculated to determine preoperative VCP cutoff. RESULTS: There were 110 patients who underwent DOZP for treatment of velopharyngeal insufficiency. Of these patients, 94 (85%) had successful surgery as determined by their postoperative CAPS-A-AM hypernasality score. Preoperative VCP was a statistically significant predictor of successful DOZP (P < .0001). The ROC curve with Youden index (J) determined a cutoff (c*) of 55% preoperative VCP or greater to optimize surgical success rate. Grouping by preoperative VCP showed that surgical success increases directly with preoperative VCP, and patients with low VCP had above a 50% success rate in reducing hypernasality scores. CONCLUSIONS: Preoperative VCP was significantly associated with improved hypernasality ratings postoperatively. A preoperative VCP of ≥55% may be used to help predict success of Furlow palatoplasty treatment. Patients with lower VCP can still benefit from secondary DOZP.

Multicenter comparison of head shape outcomes for three minimally invasive strip craniectomy techniques for sagittal craniosynostosis.

OBJECTIVE: A literature gap exists comparing whole head shape outcome following correction of sagittal craniosynostosis. The objective of this multicenter study was to provide an analysis of long-term results following three different endoscopic strip craniectomy techniques for correction of sagittal craniosynostosis: 1) spring-assisted strip craniectomy, 2) wide-strip craniectomy with biparietal and bitemporal barrel-stave wedge osteotomies plus helmet orthosis, and 3) narrow-strip craniectomy plus orthosis without barrel staves. METHODS: Pre- and postoperative 3D stereophotogrammetric images were collected from patients who underwent craniosynostosis surgery. Procedures were divided among institutions as follows: spring-assisted strip craniectomies were performed at Atrium Health Wake Forest Baptist Hospital; narrow-strip craniectomies were performed at St. Louis Children's Hospital by one craniofacial surgeon; and wide-vertex craniectomies were performed at St. Louis Children's Hospital prior to 2010, and then continued at Children's Medical Center Dallas. Pre- and postoperative 3D whole-head composite images were generated for each procedure to visually represent outcomes at final follow-up and compared with age-matched normal controls. RESULTS: Patients in the spring-assisted strip craniectomy group showed normalization of frontal bossing and skull height compared with age-matched controls, whereas patients undergoing wide-strip craniectomy showed greater correction of occipital protrusion. Patients in the narrow-strip craniectomy cohort had intermediate results between these outcomes. Nested aggregate head shapes showed good correction of head shapes from all techniques. CONCLUSIONS: This large, retrospective, multicenter study illustrated whole head shape outcomes from three different craniectomy procedures. Although each procedure showed some differences in loci of primary correction, all three surgical methods demonstrated good correction of primary scaphocephalic deformity.

3D imaging of vascular anomalies using raster-scanning optoacoustic mesoscopy.

OBJECTIVES: Vascular anomalies such as capillary malformations (CMs) and infantile hemangiomas (IHs) are common pediatric vascular disorders that are treated with therapeutic laser. The treatment method, however, relies on subjective evaluation of clinical findings and can have unpredictable results. Raster-scanning optoacoustic mesoscopy (RSOM) is an innovative imaging technology using pulsed-light laser to excite hemoglobin, generating ultrasound waves that are converted into three-dimensional images of tissues. RSOM can provide objective information about superficial structures such as the microvasculature of vascular anomalies. MATERIALS AND METHODS: In this study, we explore the clinical potential of RSOM to study vascular anomalies before and after laser treatment. We scanned nine patients with CM (n = 6) and IH (n = 3) who underwent laser treatment and calculated the blood vessel volume. RESULTS: Overall, there was a posttreatment volume increase in CM, and a decrease in IH. CONCLUSION: These findings support the possibility that RSOM may have a role in developing an objective method of evaluating these lesions, leading to a tailored treatment approach and avoidance of adverse outcomes.

Anterior "W" Tongue Reduction for Macroglossia in Beckwith-Wiedemann Syndrome.

INTRODUCTION: Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by surgeons. The purpose of this study is to evaluate the postoperative outcomes of the anterior "W" tongue reduction technique in patients with BWS. METHODS: A retrospective review was conducted of all patients diagnosed with BWS that underwent an anterior "W" tongue reduction for macroglossia in the past 7 years, performed by 2 surgeons. Demographics, procedural characteristics, perioperative outcomes, and complications were assessed. RESULTS: A total of 19 patients met inclusion criteria consisting of 8 male and 11 female patients. The mean age at the time of surgery was 405 days, mean surgeon operating time was 1.06 h, and mean length of follow-up was 467 days. Postoperative oral competence was observed in 100% of patients. There was no reported history of sleep apnea or airway compromise. Speech delay was seen in 4 patients pre- and postoperatively. Feeding issues decreased from 7 patients preoperatively to 1 patient postoperatively. Preoperative prevalence of class III malocclusion (53%) and isolated anterior open bite (26%) decreased postoperatively to 37% and 16%, respectively. The only reported complications were superficial tip wound dehiscence in 3 patients treated with nystatin antifungal therapy. None of the patients required revisional surgery. CONCLUSION: Patients treated with the anterior "W" tongue reduction technique had low rates of perioperative complications and significant improvements in oral competence. Anterior "W" tongue reduction is safe and effective for the correction of macroglossia in patients with BWS.

Assessing outcomes of ear molding therapy by health care providers and convolutional neural network.

Ear molding therapy is a nonsurgical technique to correct certain congenital auricular deformities. While the advantages of nonsurgical treatments over otoplasty are well-described, few studies have assessed aesthetic outcomes. In this study, we compared assessments of outcomes of ear molding therapy for 283 ears by experienced healthcare providers and a previously developed deep learning CNN model. 2D photographs of ears were obtained as a standard of care in our onsite photography studio. Physician assistants (PAs) rated the photographs using a 5-point Likert scale ranging from 1(poor) to 5(excellent) and the CNN assessment was categorical, classifying each photo as either "normal" or "deformed". On average, the PAs classified 75.6% of photographs as good to excellent outcomes (scores 4 and 5). Similarly, the CNN classified 75.3% of the photographs as normal. The inter-rater agreement between the PAs ranged between 72 and 81%, while there was a 69.6% agreement between the machine model and the inter-rater majority agreement between at least two PAs (i.e., when at least two PAs gave a simultaneous score < 4 or ≥ 4). This study shows that noninvasive ear molding therapy has excellent outcomes in general. In addition, it indicates that with further training and validation, machine learning techniques, like CNN, have the capability to accurately mimic provider assessment while removing the subjectivity of human evaluation making it a robust tool for ear deformity identification and outcome evaluation.

Progressive Postnatal Pansynostosis in Crouzon Syndrome.

ABSTRACT: Crouzon syndrome is a known craniosynostosis syndrome with a heterogenous presentation. Suture closure can rapidly develop postnatally and involve multiple sutures, requiring vigilant clinical monitoring to prevent delay in diagnosis and developing intracranial hypertension. The authors present the case of a male patient with Crouzon syndrome who developed postnatal pansynostosis at 18 months of age when several missed appointments led to diagnosis after complete closure of all sutures and signs of elevated intracranial pressure. Posterior cranial vault distraction was performed soon after, and the patient improved clinically. There is a need for improved monitoring and management of these patients to prevent delay in diagnosis and potential permanent sequelae as well as minimizing radiation exposure through watchful CT scanning.