Background Pierre Robin Sequence (PRS) is characterized by micrognathia, glossoptosis, and upper airway obstruction. Early recognition and appropriate perinatal management is crucial for optimizing outcomes. This study aimed to evaluate 20-week fetal ultrasounds to determine if specific mandibular measurements could predict PRS diagnosis and disease severity. Methods A retrospective case-control study of 48 patients with PRS and gender-matched controls was performed. Medical records were reviewed for respiratory and surgical interventions. Three parameters to assess micrognathia were measured on mid-sagittal profile ultrasound images: frontal nasal-mental angle (FNMA), facial-maxillary angle (FMA), and alveolar overjet. Student's t-test and univariate logistic regression was performed. P ≤ 0.05 was considered statistically significant. Results Patients with PRS demonstrated a significantly smaller mean FNMA compared to the control group, 129.3 ± 8.6° and 137.4 ± 3.2°, respectively (p < 0.0001), as well as significantly smaller mean FMA, 63.2 ± 9.2° and 74.8 ± 6.1°, respectively (p < 0.0001). The PRS group also demonstrated significantly larger mean alveolar overjet compared to the control group, 3.9 ± 1.4 mm and 2.1 ± 0.9 mm, respectively (p < 0.0001). The odds of respiratory intervention increased among cases when FMA was <68°. Additionally, there was a significant difference in median overjet between patients with PRS who did and did not require respiratory intervention. Conclusions Mandibular features on the 20-week ultrasound can be measured to predict diagnosis and severity of PRS. This is an important first step to prepare for potential respiratory intervention at delivery to minimize perinatal hypoxia. Alveolar overjet, previously not described in prenatal ultrasound literature, is measurable and has utility in prenatal screening for PRS, as do FMA and FNMA.

Purpose Persistent velopharyngeal insufficiency (VPI) following primary palatoplasty remains a difficult problem to treat. This study evaluates speech outcomes following revision palatoplasty with tissue augmentation using buccal myomucosal flaps (BMF) as an alternative to pharyngoplasty for patients with VPI. Methods A retrospective single-center review of revision palatoplasty with tissue augmentation at a tertiary pediatric hospital Cleft-Craniofacial Center between January 2017 and March 2021 was conducted. Patients with a history of previous palatoplasty, a diagnosis of persistent or recurrent VPI, and comprehensive pre- and postoperative speech evaluations who underwent revision palatoplasty with BMF were included. Results Twenty patients met inclusion criteria (35% female, 20% syndromic). Mean age at the time of revision palatoplasty with BMF was 9.7 years. Preoperatively, all patients had stigmatizing speech and received the recommendation for speech surgery; the mean Pittsburgh Weighted Speech Score (PWSS) was 14.3 ± 4.9. The mean postoperative PWSS at the most recent assessment was 4.2 ± 2.3, representing a statistically significant improvement from preoperative scores ( P < .001). Mean follow-up time was 8.9 months. Following revision palatoplasty with BMF, only one patient has received the recommendation for further speech surgery. No complications were noted. Conclusion In patients with VPI following primary palatoplasty, revision palatoplasty with tissue augmentation offers an alternative to pharyngoplasty. This approach preserves dynamic velopharyngeal function, improves speech outcomes, and should be considered an option when treating patients with post-primary palatoplasty VPI.

Background: The COVID-19 pandemic had multiple effects on the provision of health care, including the suspension of elective and nonessential surgeries. The objective of this study was to determine the early effect of the COVID-19 pandemic on the surgical care of patients with cleft lip and/or palate at a high-volume cleft center. Methods: A retrospective comparative cohort study of patients with cleft lip and/or palate undergoing lip adhesion, cleft lip and nose repair, and palatoplasty before and during the pandemic was conducted. There were 50 patients in the prepandemic cohort and 53 in the pandemic cohort. Results: Mean age at lip adhesion was 3.1 ± 1.1 months prepandemic (n = 8) and 3.5 ± 2.5 months in the pandemic cohort (n = 8) (P = 0.75). One lip adhesion was delayed by 1.6 months. Mean age at cleft lip and nose repair was 6.6 ± 1.9 months prepandemic (n = 23) and 8.0 ± 2.1 months in the pandemic cohort (n = 23) (P = 0.03). Six pandemic cleft lip and nose repairs were delayed; the mean delay was 2.6 ± 1.8 months. The mean age at palatoplasty was 13.9 ± 2.2 months prepandemic (n = 26) and 14.1 ± 2.9 months in the pandemic cohort (n = 26) (P = 0.79). Seven pandemic palatoplasties were delayed; the mean delay was 3.3 ± 1.4 months. Conclusions: The COVID-19 pandemic caused delays at each stage of repair for cleft lip and/or palate-related procedures; however, only cleft lip and nose repair were significantly affected. This study emphasizes the importance of remaining vigilant regarding the care of this vulnerable population during this challenging time.

Introduction Velopharyngeal insufficiency (VPI), a stigmatizing hallmark of palatal dysfunction, occurs in a wide spectrum of pediatric craniofacial conditions. The mainstays for surgical correction include palate repair and/or pharyngeal surgery. However, primary pharyngoplasty has a failure rate of 15% to 20%. Although revision pharyngoplasty may be necessary in those with persistent VPI, little is known regarding the indications for and outcomes after such procedures. The purpose of this study is to describe the authors' experience with indications for and outcomes after revision pharyngoplasty. Methods A single-center retrospective review was performed of all patients undergoing revision pharyngoplasty between 2002 and 2019. Demographic data and Pittsburgh Weighted Speech Scores, diagnoses, comorbidities, and complications were tabulated. Two-tailed Student t test was used, and a P value of 0.05 or less was considered statistically significant. Results Thirty-two patients (65.6% male) met inclusion criteria for this study. The most common diagnoses included cleft palate (68.8%), submucous cleft palate (SMCP, 18.8%), and congenital VPI (6.3%, likely occult SMCP). Most patients (84.4%) underwent palatoplasty before their initial pharyngoplasty. The primary indication for initial pharyngoplasty was VPI (mean age 7.1 ± 4.6 years). The most common indication for revision pharyngoplasty (mean age 11.2 ± 5.1 years) included persistent VPI (n = 22), followed by obstructive sleep apnea (OSA) (n = 11). Persistent VPI (n = 8) and OSA (n = 6) were the most common complications after secondary pharyngoplasty. Thirteen patients (40.6%) within the revision pharyngoplasty cohort required additional surgical intervention: 4 underwent tertiary pharyngoplasty, 4 underwent takedown for OSA (n = 3) or persistent VPI (n = 1), 3 underwent takedown and conversion Furlow for persistent VPI (n = 2), OSA (n = 2) and/or flap dehiscence (n = 1), and 2 underwent palatal lengthening with buccal myomucosal flaps for persistent VPI. Of the 4 patients who required a tertiary pharyngoplasty, the mean age at repair was 6.6 ± 1.1 years and their speech scores improved from 13.5 to 2.3 after tertiary pharyngoplasty (P = 0.11). The overall speech score after completion of all procedures improved significantly from 19 to 3.3. Conclusion Patients who fail primary pharyngoplasty represent a challenging population. Of patients who underwent secondary pharyngoplasty, nearly half required a tertiary procedure to achieve acceptable speech scores or resolve complications.

Objective: The endoscopic endonasal approach (EEA) is commonly employed in skull base surgery for neoplasm resection. While nasal deformity following EEA is described, this study aimed to perform a detailed qualitative and quantitative assessment of the associated saddle nose deformity (SND) in particular. Setting/Participants: This is a retrospective review of 20 adult patients with SND after endoscopic endonasal approach (EEA) for resection of skull base tumors over a 5-year period at the University of Pittsburgh Medical Center. Outcome Measures: Fifteen measurements related to SND were obtained on pre-and post-operative imaging. Statistical analyses were performed to evaluate differences between pre- and post-operative anatomy. Results: The most common EEA was transsellar. Reconstruction techniques included 9 free mucosal grafts alone, 8 vascularized nasoseptal flaps (NSF), 1 combined free mucosal graft/abdominal fat graft, and 1 combined NSF/fascia lata graft. Imaging analysis showed a trend toward loss of mean nasal height, nasal tip projection and nasolabial angle post-operatively. Sub-group analysis showed that patients with NSF reconstruction had a significantly decreased nasal tip projection (1.2mm, p=0.039) and increased alar base width (1.2mm, p=0.046) post operatively. Patients without functional pituitary microadenomas demonstrated significantly increased nasofrontal angle and decreased nasal tip projection on post-operative imaging, in contrast to those with functional adenomas who had no measurable significant changes. Conclusions: Clinically evident SND does not always lead to significant radiographic changes. This analysis suggests that patients who undergo surgery for indications other than functional pituitary microadenomas or who receive NSF reconstruction develop more marked SND on standard imaging tests.

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Introduction The concept of “overcorrection” for trigonocephaly has been reported to achieve both anterior cranial fossa expansion and normalization of craniofacial form. The purpose of this study is to describe in detail a standardized technique to fronto-orbital advancement utilizing the concept of “overcorrection” and objectively evaluate intermediate results. Methods This retrospective study included patients with isolated metopic synostosis who underwent surgery via the proposed surgical technique and age and sex-matched unaffected controls. Craniofacial morphometric analysis was performed on pre-, immediate post-, and intermediate postoperative (>2 years) three-dimensional (3D)-rendered computed tomographic (CT) scans and photographs. Key CT-based measurements included interzygomaticofrontal suture distance (IZFS), endocranial bifrontal angle (ECA), and temporal expansion. 3D photogrammetry was performed using established measurements and associated Z-scores converted. A Paired t-test and analysis of variance were performed when appropriate. Results Forty-one patients were included. A comparison of pre- and immediate postoperative CT scans demonstrated statistically significant increases in all measurements. Subset analysis of 12 patients with intermediate follow-up (age: 39.6 ± 3.6 months) demonstrated significant differences from preoperative values except for IZFS, which decreased from immediate postoperative values and was smaller than age- and sex-matched controls. 3D photogrammetry demonstrated a mean Z-score above the norm for frontal breath. 3D photogrammetry is also positively correlated with CT-based measurements. Conclusions This standardized “overcorrection” approach for trigonocephaly can provide the appropriate changes to maintain a normal ECA despite a reduction in bifrontal width over time. 3D photogrammetry positively correlated with CT-based measurements and may provide useful information when following patients clinically. Long-term follow-up assessment to determine the necessary degree of overcorrection at skeletal mature is needed.

Metopic craniosynostosis (MCS) refers to the premature fusion of the metopic cranial suture resulting in trigonocephaly, characterized by a keel-shaped forehead, suture ridging, orbital hypotelorism, retrusion and upsloping of the lateral supraorbital rim, and bitemporal narrowing. 1 Although severe trigonocephaly is pathognomonic for MCS, mild to moderate phenotypes are more difficult to diagnose and classify by severity. There is currently no standard for classification of severity, and variability in management protocols exists. Surgical intervention for mild to moderate phenotypes remains controversial as objective methods to delineate which patients require surgery are lacking. 1 Although operative intervention effectively preserves normal neurocognition, studies have demonstrated a worsening aesthetic result with Background: Quantifying the severity of head shape deformity and establishing a threshold for operative intervention remains challenging in patients with metopic craniosynostosis (MCS). This study combines three-dimensional skull shape analysis with an unsupervised machine-learning algorithm to generate a quantitative shape severity score (cranial morphology deviation) and provide an operative threshold score. Methods: Head computed tomography scans from subjects with MCS and normal controls (5 to 15 months of age) were used for objective three-dimensional shape analysis using ShapeWorks software and in a survey for craniofacial surgeons to rate head-shape deformity and report whether they would offer surgical correction based on head shape alone. An unsupervised machine-learning algorithm was developed to quantify the degree of shape abnormality of MCS skulls compared to controls. Results: One hundred twenty-four computed tomography scans were used to develop the model; 50 (24% MCS, 76% controls) were rated by 36 craniofacial surgeons, with an average of 20.8 ratings per skull. The interrater reliability was high (intraclass correlation coefficient, 0.988). The algorithm performed accurately and correlates closely with the surgeons assigned severity ratings (Spearman correlation coefficient, r = 0.817). The median cranial morphology deviation for affected skulls was 155.0 (interquartile range, 136.4 to 194.6; maximum, 231.3). Skulls with ratings of 150.2 or higher were very likely to be offered surgery by the experts in this study. Conclusions: This study describes a novel metric to quantify the head shape deformity associated with MCS and contextualizes the results using clinical assessments of head shapes by craniofacial experts. This metric may be useful in supporting clinical decision making around operative intervention and in describing outcomes and comparing patient population across centers. (Plast.