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The purpose of this study was to evaluate the epidemiology and perioperative complications of different reconstructive strategies to correct cleft nasal deformity, with particular attention paid to type and timing of cartilage grafting.Retrospective cohort study was conducted of cleft rhinoplasty performed between 2012 and 2017 in North America utilizing the American College of Surgeons National Surgical Quality Improvement Program- Pediatric hospital network. Medical/surgical complications, reoperations, and readmissions within 30 days postoperatively were analyzed with appropriate statistics.During the study interval, 3317 pediatric patients underwent cleft rhinoplasty, with 8.0% involving the use of cartilage grafts. Ear cartilage was significantly more commonly used for intermediate repair, whereas rib cartilage was more commonly used for late repair (P=0.006). Overall, rhinoplasties with ear cartilage grafts had shorter procedure durations than those without cartilage grafts (P=0.005), whereas those with rib cartilage grafts had increased procedure duration (P0.001). The use of cartilage grafts was not associated with increased complications in either intermediate or late cleft rhinoplasty. Patients with bilateral clefts were more likely to undergo rhinoplasty with cartilage grafts overall (P=0.047) and with cartilage grafts for late reconstruction (P=0.039).Ear cartilage is most frequently utilized for intermediate repair, whereas rib cartilage is most frequently utilized for late repair during cleft rhinoplasty. Ear cartilage grafts are associated with significantly decreased procedure duration, whereas rib cartilage grafts are associated with significantly increased procedure duration. Not surprisingly, cleft rhinoplasty is relatively safe, with a 2% overall short-term complication rate.

Vertical expandable prosthetic titanium rib (VEPTR) devices were designed to treat childhood scoliosis and thoracic insufficiency syndrome. Although they have drastically reduced patient mortality, they present a profound subcutaneous hardware burden. The authors examined the surgical and nonsurgical factors associated with salvage of VEPTR hardware exposures.Between 2014 and 2020, a prospective institutional database was queried for patients with VEPTR hardware complications who required soft-tissue reconstruction. Hardware salvage was considered successful if reconstruction allowed the hardware to be retained until the next VEPTR expansion.Fifty-eight patients required VEPTR hardware salvage. Hardware complications were successfully salvaged in 62.1 percent of patients at 60.0 percent of operative sites. Neuromuscular scoliosis (p = 0.041) and nonambulatory status (p = 0.018) were factors associated with VEPTR hardware salvage failure, whereas congenital scoliosis was associated with successful hardware salvage (p = 0.012) and preventing need for immediate hardware removal (p = 0.049). Exposed hardware, as compared to threatened exposure, was more likely to require immediate removal (p = 0.045) and result in unsuccessful hardware salvage (p = 0.015).Local and regional muscle flaps were able to prevent VEPTR hardware removal in the majority of patients, even in the setting of infection, immobility, incontinence, and multiple systemic comorbidities. Patients with neuromuscular scoliosis and nonambulatory status were at increased risk for failure, whereas those with incontinence and low body mass index trended toward increased risk of failure. Threatened exposure was associated with higher rates of salvage than exposed hardware, and thus earlier referral to plastic surgeons for soft-tissue salvage may be advised.Risk, III.

Although conventional posterior vault distraction osteogenesis is known to achieve the greatest improvement in intracranial volume, anteroposterior expansion of the cranial vault is not always ideal in certain head shapes. Transverse posterior vault distraction osteogenesis is an alternative reconstructive strategy in patients presenting with recalcitrant multisuture craniosynostosis resulting in scaphocephaly with posterior narrowing. The authors present a multimedia demonstration of placing the cranial hinge points at the superior anterior aspect of the bone segments in order to achieve increased intracranial volume and significant improvement in posterior head shape, while preserving normal anterior skull dimensions.

Previous literature has documented craniometric changes of the bony calvaria, increases in intracranial volume, and resolution of Chiari malformations following posterior vault distraction osteogenesis. No studies have analyzed changes to the soft-tissue envelope after posterior vault distraction osteogenesis. In this study, the authors aimed to provide objective measurements of scalp thickness in patients undergoing posterior vault distraction osteogenesis, utilizing facial soft-tissue thickness as a proxy to control for growth. The authors hypothesized that the soft tissues of the scalp are not made thinner by the distraction process, either in the region of distraction or in neighboring areas. Subjects who underwent posterior vault distraction osteogenesis for a craniosynostosis diagnosis who had high-resolution predistraction and post-distractor removal computed tomographic scans within 100 days of each operation were included. The scans were analyzed on Materialise Mimics version 21 software (Materialise, Ghent, Belgium). Six key craniometric landmarks (glabella, pogonion, zygion, vertex, euryon, and opisthocranium) were identified on the three-dimensional bone masks in the Frankfort horizontal plane. The points were overlaid onto soft-tissue thickness masks and thicknesses were recorded. Percent change in postoperative facial soft-tissue thickness was used to control for growth of the craniofacial soft-tissue envelope during the study interval. The preoperative and postoperative posterior vault distraction osteogenesis cohorts did not differ significantly at the glabella, pogonion, opisthocranium, vertex, and zygion. The corrected median thickness at the euryon was significantly increased in the post-posterior vault distraction osteogenesis cohort [2.67 mm (IQR, 2.49 mm to 4.02 mm) versus 5.26 mm (IQR, 3.83 mm to 7.82 mm), p = 0.002]. This is the first study to quantify changes in soft-tissue thicknesses preoperatively and postoperatively in patients undergoing posterior vault distraction osteogenesis, demonstrating maintenance of scalp thickness. .Therapeutic, IV.

OBJECTIVE Children with multiple prematurely fused cranial sutures and those undergoing surgical correction later in life appear to experience worse neurocognitive outcomes, but it is unclear whether higher intracranial pressure (ICP) is implicated in this process. The purpose of this study was to elucidate the effect of age at intervention and number of involved cranial sutures on ICP, as well as to assess which cranial suture closure may be more associated with elevated ICP. METHODS The prospective craniofacial database at the authors’ institution was queried for patients undergoing initial corrective surgery for craniosynostosis in whom intraoperative measurement of ICP was obtained prior to craniectomy. Age, involved sutures, and syndromic status were analyzed in the context of measured ICP by using multiple linear regression. RESULTS Fifty patients met the inclusion criteria. Age at procedure (p = 0.028, β = +0.060 mm Hg/month) and multiple-suture involvement (p = 0.010, β = +4.175 mm Hg if multisuture) were both significantly implicated in elevated ICP. The actual number of major sutures involved was significantly correlated to ICP (p = 0.001; β = +1.687 mm Hg/suture). Among patients with single-suture involvement, there was an overall significant difference of median ICP across the suture types (p = 0.008), with metopic having the lowest (12.5 mm Hg) and sagittal having the highest (16.0 mm Hg). Patients with multiple-suture involvement had significantly higher ICP (p = 0.003; 18.5 mm Hg). Patients with craniofacial syndromes were 79.3 times more likely to have multiple-suture involvement (p < 0.001). Corrective surgery for craniosynostosis demonstrated significant intraoperative reduction of elevated ICP (all p < 0.050). CONCLUSIONS Syndromic status, older age at intervention for craniosynostosis, and multiple premature fusion of cranial sutures were associated with significantly higher ICP.

Objective To evaluate the timing and safety of cleft palate (CP) repair in patients with Robin sequence (RS) treated with mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA) for airway obstruction. Design Retrospective cohort study. Setting Tertiary Pediatric Hospital during 2004-2020. Patients 148 patients with RS underwent MDO, 66 met inclusion by having MDO and followed by palatoplasty. 26 patients with RS underwent TLA, 14 met inclusion by having TLA and followed by palatoplasty. Main Outcome Measures Patient characteristics, hospital/operative details, postoperative complications, and polysomnographic (PSG) data were compared. Results Groups were well-matched except more patients with syndromes underwent MDO (N = 27, 41%, P ≤ .002). In the MDO and TLA cohorts, mean CP repair age was 12.8 ± 1.9 months and 14.6 ± 1.6 months, respectively ( P ≤ .002). Despite the earlier CP repair in the MDO group, there were no differences in peri-operative complication rates after palatoplasty in either group. All sleep respiratory parameters improved after MDO/TLA prior to palatoplasty P ≤ .050. All PSG parameters remained significantly improved after palatoplasty compared to preoperative values, P ≤ .043. Obstructive apnea hypopnea index and Oxygen saturation nadir further improved after palatoplasty within the MDO group, P ≤ .050, while no changes in the TLA group, P ≥ .500. Conclusions MDO was associated with earlier age at palatoplasty than TLA with a similar perioperative risk profile. In those patients with pre- and post-palatoplasty PSG data, palatoplasty was not associated with a deterioration in PSG parameters, and in fact in the MDO group, PSG data improved.

The aim of this study was to compare midchildhood speech outcomes in patients with nonsyndromic Robin sequence with cleft palate (RSCP) treated with mandibular distraction osteogenesis (MDO) to patients with nonsyndromic Veau class I and Veau class II cleft palate (CP).The authors performed a retrospective review of patients with nonsyndromic Robin sequence from 2000 to 2017, comparing those who underwent MDO to patients with nonsyndromic CP. Demographics, operative details, length of hospital stay, complications, and Pittsburgh Weighted Speech Scale scores were collected.Thirty-three patients met inclusion criteria in the MDO group with 127 patients as controls. Despite similar median age (RSCP, 4.5 years; CP only, 4.6 years) and Veau cleft type at early evaluation, there was a significant increase in composite Pittsburgh Weighted Speech Scale score within the MDO cohort ( P ≤ 0.002); specifically, with worse visible nasal emission ( P ≤ 0.007), hypernasality ( P ≤ 0.001), and compensatory articulation ( P ≤ 0.015). However, these differences were not present at age-matched midchildhood evaluation (median, RSCP, 6.5; CP only, 7.1; P ≥ 0.092). Median age-matched follow-up was 6.4 years in the MDO group and 7.1 years in the control group ( P ≥ 0.136). There was also no difference in the rate of secondary speech surgery at midchildhood evaluation ( P ≥ 0.688).The authors' retrospective comparison of speech outcomes in RSCP versus CP only demonstrates no difference in midchildhood speech, conflicting with recent reports. Although patients with Robin sequence treated with MDO had worse visible nasal emission, hypernasality, and compensatory articulation in early childhood, this appears to have resolved in the interim without additional intervention. Longitudinal follow-up is needed to fully understand the speech ramifications of RSCP.Risk, II.

This study tested the core tenets of how facial scars are perceived by characterizing layperson response to faces with scars. The authors predicted that scars closer to highly viewed structures of the face (i.e., upper lip and lower lid), scars aligned against resting facial tension lines, and scars in the middle of anatomical subunits of the face would be rated less favorably.Volunteers aged 18 years and older from the United States were recruited through Amazon's Mechanical Turk to complete a face rating survey. Scars were digitally added in different locations and orientations for a total of 14 unique scars added to each face. Each participant rated 50 different faces on confidence, friendliness, and attractiveness. Data were analyzed using linear mixed effects models.A total of 88,850 ratings [82,990 scarred (93.4 percent)] for attractiveness, friendliness, and confidence were analyzed. In univariate linear mixed effects models, the presence of a facial scar did not significantly impact attractiveness (β = 0.016, SE = 0.014, z = 1.089, p = 0.276). A second set of linear mixed effects models identified interactions between location, subunit placement, and orientation to facial tension lines. Scars located on the lower lid mid subunit perpendicular to facial tension lines were rated less attractive (β = -0.065, SE = 0.028, z = -2.293, p = 0.022).On average, a single well-healed facial scar does not negatively affect first impressions of attractiveness, confidence, or friendliness. Specific scar location and orientation combinations, however, such as a perpendicular scar at the mid-lower eyelid, may result in lower perceived attractiveness, confidence, and friendliness.Risk, III.

A larger volume cranial vault expansion is likely facilitated by a low posterior cranial osteotomy beneath the torcula; however, this may impart an increased risk of venous bleeding. The authors compared the safety of infratorcular versus supratorcular osteotomy in patients undergoing posterior vault reconstruction or posterior vault distraction osteogenesis and analyzed volumetric changes.Patients undergoing initial posterior vault reconstruction or distraction osteogenesis between 2009 and 2021 at the authors' institution were grouped by occipital osteotomy location and analyzed retrospectively. Craniometric analysis was performed if patients had high-resolution computed tomography scans available within 180 days preoperatively and postoperatively.A total of 187 patients were included: 106 (57 percent) who underwent posterior vault distraction osteogenesis and 81 (43 percent) who underwent posterior vault reconstruction. Infratorcular osteotomy was more common in reconstruction [ n = 65 (80 percent)] than in distraction osteogenesis [ n = 61 (58 percent); p0.002]. Blood transfused was similar between low and high osteotomy cohorts in the distraction osteogenesis ( p = 0.285) and reconstruction ( p = 0.342) groups. However, median transfused blood volume per kilogram of patient weight was greater in the low versus high osteotomy distraction osteogenesis ( p = 0.010) and reconstruction ( p = 0.041) cohorts. Intraoperative venous sinus injury was rare. In the distraction osteogenesis cohort, there was increased median intracranial volumetric gain in the low (263 ml) compared with the high osteotomy cohort (127 ml; p = 0.043); however, when controlled for distraction distance, only a trend was observed ( p = 0.221). Patients undergoing distraction osteogenesis showed a larger median intracranial volume increase (168 ml) compared with those undergoing reconstruction (73 ml; p0.001).Infratorcular osteotomy can be performed safely in most patients undergoing cranial vault remodeling and does not appear to be associated with greater hemodynamic instability or sinus injury.Therapeutic, III.

PURPOSE The goal of this study was to investigate patient specific factors and surgical techniques that affect occipital step-off deformity in children undergoing posterior vault distraction osteogenesis (PVDO). METHODS Patients who underwent PVDO were retrospectively reviewed and included if a high resolution three-dimensional computed tomography scan was available 1-year post-distractor removal. Two craniofacial surgeons were blinded to individual subjects and presented with 3 still images of three-dimensional bone reconstructions and asked to rate the degree of step off: none, mild, moderate, or severe. The data were analyzed with multinomial logistic regressions and other appropriate statistics. RESULTS Forty-one patients met inclusion criteria. All patients had multisuture or bicoronal craniosynostosis. Multinomial logistic regression identified age at PVDO (Cox and Snell = 0.487, P

INTRODUCTION Children who require submandibular gland excision for ptyalism often have multiple associated comorbidities, including neurodevelopmental disorders and respiratory risk factors. The purpose of this study is to utilize a large multicenter database to elucidate the perioperative profile of submandibular gland excision in children, with particular focus on children who require submandibular gland excision for ptyalism. METHODS The American College of Surgeons National Surgical Quality Improvement Program Pediatric dataset was queried for submandibular gland excision performed from 2012 through 2018. Indications were subclassified based on International Classification of Disease (ICD)-9 and ICD-10 codes. Complications, readmissions, and reoperations were analyzed with appropriate statistics. RESULTS During the study interval, 304 pediatric patients underwent submandibular gland excision, which was mostly performed for ptyalism (56.9%), followed by inflammatory conditions (20.7%). Patients requiring submandibular gland excision for ptyalism were significantly younger (P

The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

BACKGROUND Orthognathic surgery has traditionally been performed after skeletal maturity. Although these procedures are also being performed in children, the implications of earlier intervention and specific risk factors in this younger population remain unknown. METHODS The American College of Surgeons National Surgical Quality Improvement Program Pediatric dataset was queried for orthognathic procedures performed in 2018. Complications, readmissions, and reoperations were analyzed with appropriate statistics. RESULTS Overall adverse event rate after orthognathic surgery in pediatric patients was 7.8% (n = 22 of 281), which were associated with having any comorbidity (P

Fronto-orbital advancement (FOA) of the anterior skull and orbital bandeau is standard of care for craniosynostosis with anterior morphology. Fronto-orbital retrusion, temporal hollowing, and bony contour irregularities are commonly seen in long-term follow-up. In this study, we report several technical adaptations of a new FOA technique described in Fearon et al that help facilitate adaptation of the single-segment neo-bandeau FOA technique in preparation of use in younger patients, and perform a craniometric analysis of the technique. Five consecutive patients who underwent the single-segment neo-bandeau FOA in 2020 with available pre- and post-operative three-dimensional head computed tomography scans were studied. Using Materialise Mimics (Materialise, Ghent, Belgium), cranial length, cranial height, cranial widths, and intracranial volume were measured. Two (40%) patients were male and all were non-Hispanic White with a median age at surgery of 18.6 months (interquartile range 10.4-45.7). Three patients (60%) had bicoronal or other multi-suture craniosynostosis, and 1 each had metopic and sagittal craniosynostosis. Intraoperatively measured intracranial pressure decreased from 17.8 mmHg (R 13.0-20.0) before craniectomy to 4.8 mmHg (R 2.0-11.0; P = 0.038) after craniectomy. Anterior cranial width increased postoperatively (mean 92.6 mm; R 74.9-111.5 versus 117.6 mm; R 109.8-135.2, P = 0.005). Intracranial volume increased from preoperative (mean 1211 cm3; R 782-1949 cm3) to postoperative (1387 cm3; R 1022-2108 cm3; P = 0.009). The authors find in this small sample that a single-segment neo-bandeau FOA demonstrates volumetric expansion similar to conventional FOA techniques and is feasible in infants under 1 year of age.

PURPOSE The purpose of this study is to quantitatively establish the volumetric changes observed with posterior vault distraction osteogenesis in the anterior, middle, and posterior thirds of the cranial vault; characterize change in cranial length, width, and height, correlating these changes to demographic variables that may help identify why younger kids gain more volume; and describe the short-term position of the transport segment. METHODS Multisuture craniosynostosis patients who underwent posterior vault distraction osteogenesis were retrospectively reviewed. Pediatric, dose-reduced, thin cut helically acquired head computed tomography scans were analyzed on Materialise Mimics v22 (Materialise, Ghent, Belgium). Pre and post-PVDO and "old" and "young" cohort were compared. RESULTS Twenty-one patients met inclusion criteria. The change in cranial length was significantly less at a median of 14.9 mm [Interquartile range (IQR) 11.8, 31.6] compared to the X-ray measured AP distraction distance (30 mm [IQR 24, 33]; P

Anterior cranial vault fronto-orbital distraction osteogenesis is a promising treatment modality for children with unicoronal craniosynostosis. A minimally invasive, endoscope-assisted approach offers the additional potential benefits of less scalp scarring, decreased blood loss, and decreased scalp dissection. In this article, the authors present a novel technique for minimally invasive, endoscope-assisted fronto-orbital distraction osteogenesis.

Previous literature has documented changes in nasal obstruction after acute LeFort I osteotomy. However, there is a paucity of studies that evaluate distraction-mediated LeFort I (DO-LFI) without concomitant intranasal interventions using the nasal obstruction symptom evaluation (NOSE) scale in Class III patients. The purpose of this study is to objectively evaluate nasal obstruction quality of life through the NOSE scale in patients undergoing DO-LFI. Inclusion into the study required both a preoperative (1 year ≤ date of service) and postoperative (≥6 months and ≤2 years) NOSE scale administration. Nasal obstruction symptom evaluation scales were compared using Wilcoxon signed rank test. There was a significant difference in composite NOSE scales, x̃ = 8.0 (interquartile range: 4.0-11.0), x̃ = 1.0 (interquartile range: 1.0-3.0), P 0.017, preoperatively and postoperatively respectfully. Additionally, when looking at individual components of the NOSE scale, nasal congestion or stuffiness, and trouble breathing through nose were significantly improved after DO-LFI (P 0.017). Nasal blockage or obstruction (P 0.084) and trouble breathing when exercising (P 0.076) trended towards significant improvement, as well. Trouble sleeping did not differ, P 0.611. We elucidate, in this pilot study, that there is an association between DO-LFI and patient reported nasal obstructive symptoms. Future prospective studies utilizing the NOSE scale are needed to determine causality.

To analyze whether the choice of intraoperative local anesthetic for cleft lip repair is associated with the amount of perioperative narcotic utilization. Retrospective cohort study. Hospitals participating in the Pediatric Health Information System. Primary cleft lip repairs performed in the United States from 2010 to 2020. Local anesthesia injected—treatment with lidocaine alone, bupivacaine alone, or treatment with both agents. Perioperative narcotic administration. During the study interval, 8954 patients underwent primary cleft lip repair. Narcotic utilization for unilateral ( P Conclusions In children undergoing cleft lip repair, local anesthetic combination of lidocaine and bupivacaine is associated with decreased perioperative narcotic use compared to lidocaine or bupivacaine alone.

The purpose of this study was to review our institution's experience using helmet molding therapy in children with isolated non-syndromic sagittal craniosynostosis before placement of cranial springs and provide objective measurements of craniometric changes to help determine its role in treatment.Patients who underwent preoperative helmet molding therapy for sagittal craniosynostosis were retrospectively reviewed. Three-dimensional surface tomography scans were used to measure head circumference, cranial width, cranial length, cranial index (CI), and cranial vault asymmetry.Seventeen patients underwent orthotic helmeting therapy before spring mediated cranial vault expansion. Patients spent a median of 48 days (interquartile range [IQR] 32, 57) in preoperative orthotic helmeting therapy. There were increases in both cranial width and length post-helmeting (median: 107.5 mm [IQR 104.8, 110.4] versus 115.6 mm [IQR 114.5, 119.3]; P 0.001) (median: 152.8 mm [IQR 149.2, 154.9] versus 156.8 mm [IQR 155.0, 161.5]; P 0.001), respectively. There was a greater increase in cranial width (P = 0.015). Consequently, patients' CI improved after preoperative helmeting (median: 0.702 [IQR 0.693, 0.717] versus 0.739 [0.711, 0.752]; P 0.001). There was no evidence of growth restriction from helmeting (pre-helmeting Head circumference [HC]: median 96.8 percentile [IQR 90.6, 99.9] versus post-helmeting HC: 98.7 percentile [IQR 94.7, 99.8]; P = 0.109).Preoperative helmeting in patients with non-syndromic isolated sagittal craniosynostosis can be used to improve CI before surgical correction. Significant benefits can be achieved in shorter preoperative helmeting durations than previously reported with no evidence of cranial growth restriction, which supports its feasibility and utility in children undergoing spring mediated cranial vault expansion.

Mandibular distraction osteogenesis (MDO) and continuous positive airway pressure (CPAP) may each have a role in effectively treating tongue-based airway obstruction (TBAO) in Robin sequence (RS). This study describes longitudinal outcomes after treatment of TBAO with CPAP and/or MDO. Retrospective cohort study. Tertiary Pediatric Hospital. A total of 129 patients with RS treated with CPAP and/or MDO from 2009 to 2019 were reviewed. Subjects receiving baseline and at least one follow-up polysomnogram were included. 55 who underwent MDO ± CPAP and 9 who received CPAP-only treatment were included. Patient characteristics, feeding, and polysomnographic data were compared and generalized linear mixed modeling performed. Baseline obstructive apnea–hypopnea index (OAHI) was greater in the MDO-treated group (median x˜ = 33.7 [interquartile range: 26.5-54.5] than the CPAP-treated group (x˜ = 20.3[13.3-36.7], P ≤ .033). There was significant reduction in OAHI following treatment with CPAP and MDO modalities, P ≤ .001. SpO2 nadir after MDO was lower in syndromic (x˜ = 85.0[81.0-87.9] compared to nonsyndromic patients (x˜ = 88.4[86.8-90.5], P ≤ .005.) CPAP was utilized following MDO in 2/24 (8.3%) of nonsyndromic and 16/31 (51.6%) of syndromic subjects ( P ≤ .001,) for a median duration of 414 days. Three patients (5%) underwent tracheostomy, all had MDO. Nasogastric tube feeding at hospital discharge was more common following MDO (44, 80%) than CPAP-only (4, 44.4%, P ≤ .036), but did not differ at 6-month follow-up ( P ≥ .376). CPAP appears to effectively reduce obstructive apnea in patients with RS and moderate TBAO and be a useful adjunct in syndromic patients following MDO with improved but persistent obstruction.

Background Craniosynostosis is the premature fusion of cranial sutures in pediatric patients, which may lead to elevated intracranial pressure due to cerebro-cephalic disproportion between a growing brain and constricted skull. It is unknown whether this increased pressure is distributed equally throughout the cranial vault, or whether certain areas of the brain experience greater pressure at these regions of premature osseous fusion. Methods Optical coherence tomography (OCT) is a noninvasive modality for detecting elevated intracranial pressure. Optical coherence tomography was utilized to measure the peripapillary retinal nerve fiber layer (RNFL) thickness in patients undergoing surgical correction of craniosynostosis. Retinal nerve fiber layer in the eye ipsilateral to the unicoronal suture fusion was compared to the RNFL in the eye contralateral to the unicoronal suture fusion. Results During the study interval, 21 patients met inclusion criteria. Median age at operative intervention was 8.0 months, and 28.6% patients presented with left-sided unicoronal craniosynostosis, whereas 71.4% of patients presented with right-sided unicoronal craniosynostosis. Rather than universal increase on the affected side of coronal suture fusion, retinal nerve fiber layer thickness parameters showed a rotation phenomenon, such that the patterns of elevation had a 45° circumferential rotation in the direction of intorsion. Conclusions The explanation for these results remains elusive, but they likely indicate either intracranial changes transmitted differentially to the peripapillary retina, or differing retinal morphology, between the ipsilateral and contralateral eyes in unicoronal craniosynostosis.

Maxillary hypoplasia is common in patients with cleft lip and palate (CL/P), and its etiology is incompletely understood. The purpose of this study is to evaluate facial suture patency in patients with CL/P and maxillary hypoplasia. The authors hypothesize that patients with CL/P will demonstrate higher rates of premature midfacial suture fusion in comparison to unaffected controls. Skeletally mature patients with CL/P and midface hypoplasia were identified, along with a cohort of unaffected age- and sex-matched controls. High-resolution facial computed tomography scans were evaluated for the presence of facial suture fusion. Utilizing a previously published suture fusion grading scale, the facial sutures were classified as open, partially open, closed, or pathologically absent. Thirty-one CL/P patients with midface hypoplasia were identified, with age and sex-matched controls. The frequency of intermaxillary suture fusion did not differ between patients with CL/P and unaffected controls (P 0.05.) Pathologic absence of the midpalatal suture was more commonly present in patients with CL/ P and midface hypoplasia in comparison to unaffected controls (P 0.05.) The role of midfacial sutures in the development of midfacial hypoplasia seen in CLP has not previously been studied or described. Our data show that the midpalatal suture is frequently pathologically absent in patients with CL/P and maxillary hypoplasia. The authors did not identify statistically significant differences in other midfacial sutures between patients with CL/P and controls, leading us to conclude that midfacial sutures may not play a key role in the development of midfacial hypoplasia.

BACKGROUND Patients with hemifacial microsomia (HFM) may undergo unilateral mandibular distraction osteogenesis (MDO) before skeletal maturity in an effort to improve facial symmetry. Mandibular distraction osteogenesis's effect on airway volumes have been studied in the past, though to our knowledge, none have accounted for the effect of head and neck posture on airway morphology. This study aimed to tackle this shortcoming, using imaging to analyze the upper airway of patients with HFM before and after surgical intervention with MDO. METHODS/DESCRIPTION The authors retrospectively reviewed patients with a diagnosis of unilateral HFM whom underwent unilateral MDO with an oblique vector at age 4 to 14 years at a single institution from 2004 to 2019. Patients with pre- and post-MDO three-dimensional computed tomography scans of the upper airway within 12 months of distractor placement and removal, respectively, were included. Head and neck postures were determined by craniocervical, pitch, roll, and yaw angles. Pre- and post-operative pharyngeal airway volumes, pharyngeal surface area, minimum retropalatal cross-sectional areas (RP CSA) and retroglossal (RG) CSA and associated anteroposterior distances were measured using Mimics 22.0 (Materialise; Leuven, Belgium). Comparison was done using Kruskal-Wallis tests and linear mixed-effects models controlling for head and neck postures. RESULTS Ten patients met inclusion criteria. Mean age at pre-distractor placement computed tomography scan was 99 ± 35 months, and mean duration between pre- and post-surgery scans was 220 ± 90 days. Head and neck posture were found to be significant predictors of all airway dimensions. After controlling for significant factors with fixed effects linear modeling, surface area was found to be significantly smaller in patients after MDO by 189.48 mm2 (F[10.8] = -3.47, P = 0.0053), compared to their preoperative measurements. Surgery was not a significant predictor of changes in airway volume (F[11.6] = 0.52, P = 0.61), minimum RP CSA (F[12.2] = -0.64, P = 0.53), minimum RG CSA (F[12.6] = -1.64, P = 0.13), RP anteroposterior distance (F[14.0] = 0.30, P = 0.77), or RG anteroposterior distance (F[20.0] = -0.04, P = 0.97). CONCLUSIONS Oblique vector MDO in patients with HFM is associated only with statistically significant changes in the surface area of the upper airway, and is not associated with statistically significant changes in dimensions like volume, CSA, or anteroposterior dimension. This is an important finding, as it may guide discussions surrounding risk/benefit ratio for MDO in childhood.

It is unknown if craniofacial trauma services are inequitably distributed throughout the US. The authors aimed to describe the geographical distribution of craniofacial trauma, surgeons, and training positions nationwide. State-level data were obtained on craniofacial trauma admissions, surgeons, training positions, population, and income for 2016 to 2017. Normalized densities (per million population [PMP]) were ascertained. State/regional-level densities were compared between highest/lowest. Risk-adjusted generalized linear models were used to determine independent associations. There were 790,415 craniofacial trauma admissions (x[Combining Tilde] = 2330.6 PMP), 28,004 surgeons (x[Combining Tilde] = 83.5 PMP), and 746 training positions (x[Combining Tilde] = 1.9 PMP) nationwide. There was significant state-level variation in the density PMP of trauma (median 1999.5 versus 2983.5, P

The purpose of this study was to investigate the financial implications of demographic and socioeconomic factors upon the cost of surgical procedures for craniosynostosis.A retrospective cohort study was conducted of admissions for craniosynostosis surgery in the United States from 2015 through 2020 using the Pediatric Health Information System. Patient demographics, case volume, and surgical approach were analyzed in context of hospital charges.During the study interval, 3869 patients were admitted for surgery for craniosynostosis. In multivariate regression accounting for demographic and socioeconomic factors, hospital admission charges were significantly higher in patients with longer hospital length of stay ( P0.001), longer ICU length of stay ( P0.001), living in an underserved area ( P = 0.046), preoperative risk factors ( P = 0.016), and those undergoing open procedures ( P0.001); hospital admission charges were significantly lower in patients with White race ( P = 0.020) and those treated at high-volume centers ( P0.001). In multivariate regression, ICU length of stay was significantly higher in patients with preoperative risk factors ( P0.001), undergoing open procedures ( P0.001), government insurance ( P = 0.018), and not treated at high-volume centers ( P = 0.005). There were significant differences in admission charges ( P0.001), charge-to-cost ratios ( P0.001), and likelihood of being treated at high-volume craniofacial centers ( P0.001) across geographic regions of the country.In the United States, there is significant sociodemographic variability in charges for craniosynostosis care, with increased hospital charges independently associated with non-White race, preoperative risk factors, and living in an underserved area.

BACKGROUND Resorbable hardware allows adequate strength for maintaining the relative position of the craniofacial skeleton during osseous healing, and allowing subsequent growth in pediatric patients. The purpose of this study was to determine the complication profile of the latest copolymer of resorbable plates for craniofacial reconstruction in pediatric patients. METHODS Retrospective query of the operative billing record was performed for use of the DePuy Synthes Rapidsorb Fixation System at the authors' tertiary children's hospital from 2015 to 2019. Three hundred twenty-five patients were randomly selected for analysis of complications within the first postoperative year. RESULTS Median age at craniofacial reconstruction was 3.0 years, with 127 procedures (39.9 percent) performed for an intracranial mass and 141 procedures (44.3 percent) performed for craniosynostosis. Overall, 7.9 percent patients had a postoperative wound complication. The most common complications were dehiscence (3.8 percent), hematoma/seroma (3.5 percent), and infection (2.5 percent). There were no instances of extrusion, plate fracture, or screw loosening. Hardware complications in the temporoparietal region were more likely to occur at incision sites (p = 0.001), whereas wound complications at the frontal region were more likely to occur away from incision sites (p < 0.001). There were no differences in complications between plates secured with resorbable screws or with the resorbable injectable polymer system (p ≥ 0.161 for all). Radiotherapy was significantly implicated in development of clinical infection (p = 0.001), culture-positive infection (p < 0.001), readmission (p = 0.007), reoperation (p = 0.003), and plate removal (p = 0.007). CONCLUSION Resorbable cranial hardware has an overall favorable complication profile for craniofacial reconstruction in pediatric patients undergoing surgical intervention for craniosynostosis or intracranial mass resection.

The goal of this study was to investigate patient specific factors and surgical techniques that affect occipital step-off deformity in children undergoing posterior vault distraction osteogenesis (PVDO).Patients who underwent PVDO were retrospectively reviewed and included if a high resolution three-dimensional computed tomography scan was available 1-year post-distractor removal. Two craniofacial surgeons were blinded to individual subjects and presented with 3 still images of three-dimensional bone reconstructions and asked to rate the degree of step off: none, mild, moderate, or severe. The data were analyzed with multinomial logistic regressions and other appropriate statistics.Forty-one patients met inclusion criteria. All patients had multisuture or bicoronal craniosynostosis. Multinomial logistic regression identified age at PVDO (Cox and Snell = 0.487, P 0.001) as a predisposing factor and one potentially protective factor, height of osteotomy (Cox and Snell = 0.394, P = 0.004) in univariate models. Post-hoc pairwise Mann-Whitney U test revealed there was decreased step-off rating when comparing osteotomy below torcula (median 0, [IQR 0, 3]) and above torcula (median 3 [IQR 1, 4]; P = 0.036) as well as a decreased step-off when below inion (median 0 [IQR 0, 1]; P = 0.001) was compared to above the torcula.Older age at PVDO predict increased risk of developing an occipital step-off deformity after PVDO, while design of the occipital osteotomy below the inion is protective. Barrel staves with greenstick outfracture did not lower the risk of occipital step-off in this sample, the reasons for which are unclear.

Background: Pilonidal sinus disease (PSD) is a chronic inflammatory disease affecting the soft tissue of the sacrococcygeal region and remains a challenging disease for clinicians to treat. The optimal treatment for PSD remains controversial and recent reports describe several different surgical approaches offering different benefits. Approximately 40% of initial incision and drainage cases require subsequent surgery. Due to high recurrence rates and postoperative complications, a more complex revision surgery involving a flap reconstruction may be required. We hypothesised that the combination of an extracellular matrix (ECM) graft with tissue flap reconstruction may decrease the postoperative complications and recurrence rates for PSD. Method: We report a retrospective case series using a surgical flap reconstruction with concomitant implantation of an ovine forestomach ECM graft under a fasciocutaneous flap with an off-midline closure for recurrent PSD, where previously surgical intervention had failed due to wound dehiscence and/or recurrent disease. Results: The case series included six patients. After three weeks, all patients except one were fully healed, and the sixth was fully healed by week 4; all wounds remained fully healed at 12 weeks. All patients achieved good cosmesis and were able to return to normal function without any residual symptoms. Conclusion: This pilot case series explored augmenting a flap reconstruction for complex PSD with advanced ECM graft materials, demonstrating that it may improve outcomes and minimise typical complications seen in flap closure, such as inflammation, infection, haematoma/seroma and hypoperfusion. Although the study had a limited number of participants, long-term outcomes were promising and suggest that further studies are warranted.

Purpose Mandibular distraction osteogenesis (MDO) effectively treats tongue-based airway obstruction (TBAO) in micrognathic patients with Robin Sequence. Mandibular distraction osteogenesis may also address TBAO in certain nonmicrognathic patients who have severe obstructive apnea, although there is no current literature to guide MDO use in these atypical patients. This study describes outcomes of MDO in a series of patients with TBAO without micrognathia. Methods Patients who underwent MDO for TBAO from 2013-20 were reviewed, and patients with micrognathia were excluded. Study subjects received baseline/follow up polysomnography. Polysomnography variables, including Obstructive Apnea Hypopnea Index, oxyhemoglobin saturation nadir (SpO2 nadir), percent sleep time end tidal CO2 greater than 50 mm Hg (%ETCO2 > 50), and respiratory-related arousals were compared before and after MDO. Demographics, syndromic/cleft palate status, airway anomalies, respiratory support, and feeding outcomes were collected. Results One hundred and twenty-four patients underwent MDO during this study period; 5 were nonmicrognathic and included in analysis. Sixty percent (n = 3) of the cohort was syndromic: 1 patient each had Trisomy 9, Beckwith Wiedemann syndrome, and duplicated pituitary gland plus syndrome. Forty percent (n = 2) of patients had a cleft palate, 60% (n = 3) had laryngomalacia, and 40% had tracheomalacia. Median (range) age at MDO was 53 days (47-167 days), and median length of distraction was 16 mm (14-20 mm). After MDO, median Obstructive Apnea Hypopnea Index decreased from x[Combining Tilde] = 60.7/h (11.6-109.4) to x[Combining Tilde] = 5.3/h (3.5-19.3) (P = 0.034). SpO2 nadir increased (69% [58-74] to 85% [80-88], P = 0.011), and median %ETCO2 > 50 mm Hg decreased (5.8% [5.2-30.1] to 0.0% [0.0-1.3], P ≤ 0.043). Continuous positive airway pressure was used by all patients immediately after MDO, and at 6 months postoperatively, 1 patient remained on continuous positive airway pressure and 1 patient required supplemental oxygen. At last follow up, no patients had significant residual airway obstruction or required a tracheostomy. Conclusions Mandibular distraction osteogenesis can effectively treat severe TBAO in some patients without micrognathia that would otherwise be candidates for tracheostomy. When used in select patients, MDO significantly improves obstructive sleep apnea and reduces need for ventilatory support, although feeding support is still needed in most patients at 6 months. Further study in a larger cohort will help identify appropriate candidates for MDO and characterize outcomes of unique patient populations.

Objective: Ideal timing of palatoplasty continues to be debated given that early repair is thought to improve speech and hearing, whereas delayed repair is associated with less midface growth disruption. The purpose of this study is to elucidate optimal timing of palatoplasty in patients with comorbidities to mitigate perioperative complications. Design: Retrospective cohort study. Setting: Hospitals participating in the American College of Surgeons National Surgical Quality Improvement Program Pediatric. Patients: Palatoplasty performed for children younger than the age of 2 years with comorbidities. Outcomes: Medical/surgical complications, reoperations, readmissions within 30 days postoperatively. Results: Patients with comorbidities having Veau I or II cleft palate were associated with significantly decreased risk of adverse events when performing palatoplasty after 125 days of age ( P < .001). Patients with comorbidities having Veau III cleft palate were associated with significantly decreased risk of adverse events when performing palatoplasty after 225 days of age ( P = .010). Patients with comorbidities having Veau IV cleft palate were associated with significantly decreased risk of adverse events when performing palatoplasty after 250 days of age ( P = .045). Conclusions: Infants with comorbidities having progressively increasing Veau classification demonstrate unique age-dependent perioperative thresholds, such that more extensive phenotypes are associated with better perioperative outcomes with older age at time of cleft palate repair.

Purpose Pupillary light reflex (PLR) is driven by outer retinal photoreceptors and by melanopsin-expressing intrinsically photosensitive retinal ganglion cells of the inner retina. To isolate the melanopic component, we studied patients with severe vision loss due to Leber congenital amaurosis (LCA) caused by gene mutations acting on the outer retina. Methods Direct PLR was recorded in LCA patients (n = 21) with known molecular causation and severe vision loss. Standard stimuli (2.5 log scot-cd.m-2; ∼13 log quanta.cm-2.s-1; achromatic full-field) with 0.1- or 5-second duration were used in all patients. Additional recordings were performed with higher luminance (3.9 log scot-cd.m-2) in a subset of patients. Results The LCA patients showed no detectable PLR to the standard stimulus with short duration. With longer-duration stimuli, a PLR was detectable in the majority (18/21) of patients. The latency of the PLR was 2.8 ± 1.3 seconds, whereas normal latency was 0.19 ± 0.02 seconds. Peak contraction amplitude in patients was 1.1 ± 0.9 mm at 6.2 ± 2.3 seconds, considerably different from normal amplitude of 4.2 ± 0.4 mm at 3.0 ± 0.4 seconds. Recordings with higher luminance demonstrated that PLRs in severe LCA could also be evoked with short-duration stimuli. Conclusions The PLR in severe LCA patients likely represents the activation of the melanopic circuit in isolation from rod and cone input. Knowledge of the properties of the human melanopic PLR allows not only comparison to those in animal models but also serves to define the fidelity of postretinal transmission in clinical trials targeting patients with no outer retinal function.

X-linked retinitis pigmentosa (XLRP) caused by mutations in the RPGR gene is an early onset and severe cause of blindness. Successful proof-of-concept studies in a canine model have recently shown that development of a corrective gene therapy for RPGR-XLRP may now be an attainable goal. In preparation for a future clinical trial, we have here optimized the therapeutic AAV vector construct by showing that GRK1 (rather than IRBP) is a more efficient promoter for targeting gene expression to both rods and cones in non-human primates. Two transgenes were used in RPGR mutant (XLPRA2) dogs under the control of the GRK1 promoter. First was the previously developed stabilized human RPGR (hRPGRstb). Second was a new full-length stabilized and codon-optimized human RPGR (hRPGRco). Long-term (>2 years) studies with an AAV2/5 vector carrying hRPGRstb under control of the GRK1 promoter showed rescue of rods and cones from degeneration and retention of vision. Shorter term (3 months) studies demonstrated comparable preservation of photoreceptors in canine eyes treated with an AAV2/5 vector carrying either transgene under the control of the GRK1 promoter. These results provide the critical molecular components (GRK1 promoter, hRPGRco transgene) to now construct a therapeutic viral vector optimized for RPGR-XLRP patients.

Purpose To determine efficacy outcome measures for clinical trials of Leber congenital amaurosis (LCA) associated with a common intronic mutation in the CEP290 gene. Methods CEP290-LCA patients (ages 5-48) with the intronic mutation (c.2991+1655A>G) were studied as a retrospective observational case series using clinical methods and with full-field sensitivity testing (FST), optical coherence tomography (OCT), autofluorescence imaging (NIR-RAFI), transient pupillary light reflex (TPLR), oculomotor control and instability (OCI), a mobility course, and a questionnaire (NEI-VFQ). Patients were investigated cross-sectionally but a subset was able to be followed longitudinally. Results With FST, there was no rod function; cone sensitivities had a wide range from not detectable to near normal. OCT analyses indicated retained central photoreceptors with abnormal distal laminae. Based on OCT and FST, most patients had dissociation of structure and function. TPLR was nondetectable in the majority of patients, with responders demonstrating severe losses in light sensitivity. OCI was abnormal in most patients. NEI-VFQ scores had a similar range to those of other severe retinopathies. Mobility scores were consistent with FST sensitivities. In patients examined with FST, OCT, and NIR-RAFI over long-term intervals (7-10 years), there was limited but detectable disease progression. Conclusions Efficacy would be a quantitative change in foveal cone function and possibly distal laminar structure. FST provides a subjective photoreceptor-based outcome; OCT and NIR-RAFI can assess photoreceptor and RPE structure. TPLR and OCI can provide objective measures of postretinal transmission. Minimal change over a decade indicates that there is no practical value in natural history studies.

Inherited retinal degenerations cause progressive loss of photoreceptor neurons with eventual blindness. Corrective or neuroprotective gene therapies under development could be delivered at a predegeneration stage to prevent the onset of disease, as well as at intermediate-degeneration stages to slow the rate of progression. Most preclinical gene therapy successes to date have been as predegeneration interventions. In many animal models, as well as in human studies, to date, retinal gene therapy administered well after the onset of degeneration was not able to modify the rate of progression even when successfully reversing dysfunction. We evaluated consequences of gene therapy delivered at intermediate stages of disease in a canine model of X-linked retinitis pigmentosa (XLRP) caused by a mutation in the Retinitis Pigmentosa GTPase Regulator (RPGR) gene. Spatiotemporal natural history of disease was defined and therapeutic dose selected based on predegeneration results. Then interventions were timed at earlier and later phases of intermediate-stage disease, and photoreceptor degeneration monitored with noninvasive imaging, electrophysiological function, and visual behavior for more than 2 y. All parameters showed substantial and significant arrest of the progressive time course of disease with treatment, which resulted in long-term improved retinal function and visual behavior compared with control eyes. Histology confirmed that the human RPGR transgene was stably expressed in photoreceptors and associated with improved structural preservation of rods, cones, and ON bipolar cells together with correction of opsin mislocalization. These findings in a clinically relevant large animal model demonstrate the long-term efficacy of RPGR gene augmentation and substantially broaden the therapeutic window for intervention in patients with RPGR-XLRP.