Scientific Abstract Presentations: Craniomaxillofacial Session 1 Thursday, October 9 Thu, Oct 9 1:30-3:00 p.m. CDT

Background: Children with metopic craniosynostosis have distinct orbital dysmorphologies that include perceived hypotelorism and a symmetric, elliptical orbital aperture canted toward the synostosed suture. The extent to which hypotelorism is a true skeletal difference or, rather, perceived hypotelorism in the setting of trigonocephaly has been previously debated (1). Additionally, maneuvers to address the intercanthal distance during traditional fronto-orbital advancement procedures yield inconsistent results on orbital anthropometrics. The purpose of this study is to expand on our previous work by detailing pre- and post-operative orbital anthropometrics among a cohort of metopic synostosis patients undergoing endoscopic strip craniectomy (ESC) compared to age-matched controls. We aim to (1) detail the presence/absence of true skeletal hypotelorism in young infants with metopic synostosis and (2) define the role of ESC in restoring orbital anthropometric parameters to normal values.

Methods: A retrospective, cohort study was performed at a single center over a three-year (2020 – 2023) period. Using Slicer software, a three-dimensional craniometric analysis was performed on preoperative and one-year postoperative CT images of children with metopic craniosynostosis and age-matched peers. Twelve craniometric parameters were obtained. Age-matched children with normocephalic head shapes who underwent CT scan for other reasons than to rule-out craniosynostosis were utilized for comparison.

Results: Nine children were included as subjects in the study and fourteen children were included as controls. The mean age of subjects at pre-operative CT scan was 69.3 days and 72.3 days for controls. The mean age of subjects at post-operative CT scan was 14.8 months and 14.3 months for controls, for a mean follow-up of 11.4 months after ESC. 64 orbits were analyzed. Pre-operative subject Dacryon to Dacryon (Da-Da) measurements showed a significant difference from controls (12.2 ± 0.54 millimeters v. 15.4 ± 0.53 millimeters; p=0.0007), while post-surgery, there was no significant difference (16.11 ± 0.67 millimeters v. 15.10 ± 0.50 mm; p=0.2392). Similarly, Zygomaticofrontal suture to Zygomaticofrontal suture (ZF-ZF) measurements revealed a significant pre-operative difference (61.3 ±1.25 millimeters v. 67.2 ± 2.38 millimeters; p=0.0238) with post-operative normalization (76.0 ± 1.22 millimeters v. 76.0 ± 1.19 millimeters; p=0.9767). The supraorbital notch to anterior nasal spine to supraorbital notch (SON-ANS-SON) angle also indicated significant pre-operative differences (39.9 ± 0.96 degrees v. 60.1 ± 3.06 degrees; p=0.0001), with post-operative measurements showing no significant difference from controls (49.6 ± 1.39 degrees v. 55.3 ± 2.00 degrees; p=0.0674).

Conclusions: Previous work demonstrated improvement in interdacryon distance before and after endoscopic strip craniectomy. The present effort shows that orbital hypotelorism in young infants with metopic synostosis is a true skeletal difference from age-matched controls that corrects after endoscopic strip craniectomy.

References: 1. Patel KB, Skolnick GB, Mulliken JB. Anthropometric Outcomes following Fronto-Orbital Advancement for Metopic Synostosis. Plast Reconstr Surg. 2016 May;137(5):1539-1547. doi: 10.1097/PRS.0000000000002129

Introduction: Positional plagiocephaly is characterized by cranial asymmetry, and can be the result of prolonged, uneven pressure on the developing skull (1). Causes include extended time in the supine position, one-sided feeding, or insufficient tummy time (2,3). Beyond cosmetic concerns, it can affect visual, auditory, and speech development(1,2,4). Following the "Back-to-Sleep Campaign," prevalence rose to 16-22.1% in 6-week-old infants (4). In Massachusetts, 4.3% of newborns receive helmet therapy, which can $1,600 on average (5). Despite its extensive impact on development and finances, many caregivers and parents lack awareness, underscoring the need for improved education and prevention strategies.

Methods: This study, a partially blinded, two-arm randomized control trial, is ongoing at University of Massachusetts Memorial Hospital (June 2022 to January 2025). Newborns are assigned to control or intervention groups, with the latter receiving plagiocephaly prevention education. Cephalic index (CI, a proxy for plagiocephaly) measurements and parent surveys are collected at 2-, 4-, and 6-months of age. Increased CI tracks with cranial deformation. Analyses compared measurements and survey responses between groups using unpaired two-sample t-tests and difference-in-difference analysis.

Results: Interim analysis includes 107 subjects. The average baseline CI for control (cCI) and intervention (iCI) groups was 86.85 (SD 6.72) and 86.41 (SD 6.70), respectively (p = 0.983). At 2 months, cCI and iCI were 87.81 (SD 8.10) and 86.34 (SD 4.48) (p = 0.420). At 4 months, cCI and iCI were 90.34 (SD 4.62) and 86.28 (SD 6.19) (p = 0.014). At 6 months, cCI and iCI were 84.86 (SD 4.48) and 83.81 (SD 5.40) (p = 0.204) (Table 1). There was a statistically significant difference between control and intervention group parent behaviors, with the intervention group scoring more points for better compliance with recommendations (15.87 vs. 13.75) (p = 0.028), suggesting greater adoption of preventative measures by intervention parents.

Conclusion: Our interim analysis demonstrates a positive trend, with the intervention group demonstrating less cranial deformation at each time interval and reaching statistical significance at 4 months. This aligns with parent-reported behaviors at 4 months, where intervention parents demonstrated greater implementation of preventative measures. The correlation between parental engagement and CI supports the intervention's potential efficacy. As we enroll more newborns, we anticipate enhanced statistical power, leading to more definitive conclusions at 6 months. Preliminary data suggests that early education may help prevent positional plagiocephaly.

Average CI Intervention Group Control Group P Mean (SD) Baseline CI 86.41 (6.70) 86.85 (6.72) 0.98 Mean (SD) CI at 2 mo 86.34 (4.48) 87.81 (8.10) 0.42 Mean (SD) CI at 4 mo 86.28 (6.19) 90.34 (4.62) 0.014 Mean (SD) CI at 6 mo 83.81 (5.40) 84.86 (4.48) 0.2 Table 1 – Average CI of the intervention and control groups at the 2-, 4-, and 6-month time points.

References 1. Aarnivala H, Vuollo V, Harila V, Heikkinen T, Pirttiniemi P, Valkama AM. Preventing deformational plagiocephaly through parent guidance: a randomized, controlled trial. Eur J Pediatr. Sep 2015;174(9):1197-208. doi:10.1007/s00431-015-2520-x 2. Marshall JM, Shahzad F. Safe Sleep, Plagiocephaly, and Brachycephaly: Assessment, Risks, Treatment, and When to Refer. Pediatr Ann. Oct 1 2020;49(10):e440-e447. doi:10.3928/19382359-20200922-02 3. Biggs WS. Diagnosis and management of positional head deformity. Am Fam Physician. May 1 2003;67(9):1953-6. 4. Cavalier A, Picot MC, Artiaga C, et al. Prevention of deformational plagiocephaly in neonates. Early Hum Dev. Aug 2011;87(8):537-43. doi:10.1016/j.earlhumdev.2011.04.007 5. Wood JJ, Phan QA, Phan J, et al. Treatment of Plagiocephaly With Orthotic Helmets: Prevalence, Costs, and Inequities in Massachusetts. J Craniofac Surg. Jan-Feb 01 2025;36(1):74-77. doi:10.1097/SCS.0000000000010655

Background: Velopharyngeal insufficiency (VPI) is common in the cleft palate (CP) population. While some studies indicate that earlier age of CP repair may reduce VPI rates, the optimal timing for CP repair and its relation to the need for subsequent VPI surgery remains unclear. This study aims to evaluate how the timing of cleft palate repair impacts the rate of VPI surgery at a single institution.

Methods: A retrospective review of patients who underwent cleft palate repair before the age of 18 months at a tertiary children's hospital between 1990 and 2023 was conducted. Patients with craniofacial syndromes and a follow-up time of less than five years from CP repair were excluded. Data on patient demographics and cleft repair were collected. The primary outcome measure was the rate of VPI surgery. Fisher's exact and Wilcoxon rank-sum tests were performed both before and after propensity score matching for sex, age, insurance payor, cleft phenotype, surgical technique, and surgeon experience.

Results: A total of 393 patients met the inclusion criteria, with 108 patients remaining post-matching. The median age at cleft palate repair was 11.2 months (Range: 3.3-17.8). The cleft types were categorized as cleft lip and palate (81.1%), isolated cleft palate (17.9%), and submucosal cleft palate (1.0%). According to the Veau classification, the clefts were further categorized as Class I (isolated soft palate) (5.6%), Class II (hard/soft palate) (9.3%), Class III (complete unilateral cleft palate involving the alveolar ridge) (42.6%), and Class IV (complete bilateral cleft palate involving the alveolar ridge) (40.7%). Out of these patients, 67 (17.0%) underwent VPI surgery. Pre- and post-matching analysis indicated that CP age was not significantly different between patients who required VPI surgery and those who did not (median age post-matching: 11.5 months vs 11.1 months, p = 0.6).

Conclusion: This study reveals that within the first 3 years of life, the timing of cleft palate repair does not significantly affect the risk of subsequent VPI surgery. These findings suggest that factors other than the timing of initial CP repair may be more influential in determining the risk of future VPI. While the timing of CP may not impact VPI surgery rates, further research is warranted to explore its effects on VPI diagnosis and the need for speech therapy.

Background Mandibular distraction osteogenesis (MDO) has emerged as a powerful technique for airway management in patients with Robin Sequence (RS). The optimal age to perform MDO in RS patients has yet to be rigorously investigated. In this study, we evaluate the role of surgical timing in optimizing respiratory outcomes after MDO over a 20-year period.

Methods A retrospective review of patients with RS who underwent MDO at a major children's hospital between 2004 and 2023 was conducted. Patients without both pre-and post-operative polysomnography were excluded. The primary outcome of interest was the Obstructive Apnea-Hypopnea Index (OAHI), classified as mild for OAHI 1-5, moderate for OAHI 6-10, and severe for OAHI >10. Secondary outcomes included major postoperative complications requiring reoperation or readmission, supplemental oxygen requirement at discharge, and post-MDO tracheostomy. Statistical analyses were performed using piecewise regression, Pearson's chi-square, and Student's T-tests.

Results A total of 63 patients with RS met the inclusion criteria. Patients had a median age of 5.85 weeks at the time of MDO. After MDO, patients demonstrated an average OAHI reduction of 24.7 events per hour, corresponding to a mean post-operative OAHI of 8.7 ± 3.9. Piecewise linear regression identified a temporal threshold of 4 weeks, above which the relationship between age at surgery and OAHI reduction changed significantly. The mean OAHI reduction for those who underwent MDO before the age of 4 weeks was 19.6 ± 4.6, while that for those who underwent MDO after 4 weeks was 26.8 ± 3.6 (p<0.05). The early cohort had significantly lower postoperative average highest-end tidal CO2 volume (44.3 ± 0.7 mmHg vs 47.9 ± 0.6 mmHg, p<0.05). Postoperatively, the early cohort also had a higher proportion of patients in the mild OAHI category (48.3% vs 23.5%, p<0.05). Major complications, supplemental oxygen at discharge, post-MDO tracheostomy, syndromic conditions, cleft palate, and cardiopulmonary diagnoses did not vary between cohorts.

Conclusion Patients who underwent MDO before four weeks of age were significantly more likely to have mild OSA postoperatively, and lower postoperative highest-end tidal CO2 volumes. These findings suggest early intervention with MDO may be optimal for improving OSA in patients with RS. Neonatal OSA can lead to impaired growth and development, cardiovascular morbidity, and can even be life-threatening. Therefore, MDO at 4 weeks or before is a suitable option for RS patients to help mitigate these severe risks.

Background: Assessment for elevated intracranial pressure (ICP) helps guide interventional decision-making to treat craniosynostosis. However, noninvasive techniques for measuring ICP are limited. This study assessed whether optic nerve sheath diameter (ONSD) on low-dose computed tomographic (CT) scans is associated with ICP in patients with craniosynostosis.

Methods: Pediatric patients treated between 2014 and 2023 with craniosynostosis, intraoperative ICP measurements by direct subdural catheterization, and spectral domain-optical coherent tomographic (SD-OCT) data were included. ONSD was retrospectively assessed on preoperative CT scans by a masked neuroradiologist and compared with measures and proxies of ICP.

Results: Among the 132 patients included (median age, 6.9 years; interquartile range, 4.7 to 9.5 years), 41 (31.1%) had a syndromic diagnosis. Maximal ONSD was increased in patients with an ICP of 15 mmHg or greater (6.1 mm versus 5.5 mm; P < 0.01) and 20 mmHg or greater (6.3 mm versus 5.6 mm; P < 0.01). Maximal (r = 0.32; P < 0.001), minimum (r = 0.26; P = 0.003), and average (r = 0.29; P < 0.001) ONSD correlated with direct ICP measurements. ONSD and SD-OCT measurements were also correlated (maximum retinal nerve fiber layer, r = 0.21, P = 0.04; maximum retinal thickness, r = 0.24, P = 0.02). An ONSDmax threshold of 5.75 mm demonstrated 65% sensitivity and 64% specificity for detecting ICP of 15 mmHg or greater on optimized receiver operating characteristic curve analysis. Multivariable logistic regression generated an algorithm incorporating ONSDmax and age to detect an ICP of 20 mmHg or greater with 64% sensitivity and 80% specificity.

Conclusions: ONSD measured on low-dose CT scans detected elevated ICP with moderate accuracy. Precision increased when patient age was taken into consideration. Given the ease of accessing CT scan data, this may be a helpful ICP proxy for clinical decision-making.

Purpose: Craniosynostosis (CS) disrupts normal skull and facial development and is associated with an increased risk of neurocognitive impairment. Surgical intervention in infancy is aimed at correcting skull morphology and mitigating potential neurodevelopmental delays. However, the impact of surgical correction on brain development and long-term cognitive outcomes remains unclear. This study establishes a standardized, multi-institutional neuroimaging protocol to assess pre- and post-operative brain changes in infants with CS, using advanced neuroimaging techniques to evaluate white matter integrity and functional connectivity. Neuroimaging findings are compared to healthy controls from the Baby Connectome Project (BCP) to elucidate the effects of cranial surgery on brain development and neurocognitive outcomes.

Methods: This prospective, multi-institutional study was conducted across three craniofacial centers: Vanderbilt University Medical Center, Yale University, and Arkansas Children's Hospital. Twenty-two infants diagnosed with CS were enrolled and underwent non-sedated neuroimaging using Diffusion Tensor Imaging (DTI) and functional MRI (fMRI) at two time points: pre-operatively and 2–4 months post-operatively. Imaging parameters including Fractional Anisotropy (FA), Mean Diffusivity (MD), Axial Diffusivity (AD), and Radial Diffusivity (RD) were analyzed to assess white matter integrity and functional connectivity in key brain regions. Neurocognitive outcomes were measured using the Bayley Scales of Infant Development (BSID-4) to evaluate cognitive, motor, and language skills. Normative neuroimaging trend lines were constructed from 911 healthy infants at 1,010 time points in the BCP dataset, enabling direct comparisons with age- and sex-matched controls.

Results: This study successfully implemented a standardized, multi-site MRI protocol for non-sedated neuroimaging in infants with CS. Of the 22 enrolled infants, 21 completed imaging and neuropsychological testing, yielding a total of 42 MRI scans. The cohort had a mean pre-operative age of 5.6 ± 1.4 months and post-operative age of 10.3 ± 1.7 months. Sagittal synostosis was the most prevalent subtype (55%), followed by metopic (23%), unicoronal (14%), and bicoronal (9%). Surgical interventions included cranial vault reconstruction (64%) and strip craniectomy (36%).

Initial neuroimaging analyses revealed significant pre-operative reductions in FA and increased MD, AD, and RD across all synostosis subtypes compared to age- and sex- matched controls, indicating compromised white matter integrity prior to surgical intervention. Post-operatively, infants demonstrated robust improvements in FA and reductions in MD, AD, and RD, suggesting a trend toward normalization of white matter microstructure. Notably, the magnitude of white matter recovery varied by suture type, anatomical location, and surgical approach.

Conclusion: This proof-of-concept study demonstrates the feasibility of a multi-site, non-sedated MRI protocol for evaluating neurodevelopmental outcomes in infants with CS. The observed pre-operative deficits in white matter integrity and subsequent post-operative improvements underscore the importance of early surgical intervention in mitigating neurodevelopmental delays. Future analyses will explore post-surgical neuroimaging changes and their neurocognitive correlates to guide surgical decision-making and optimize patient outcomes.

Background:

Despite limitations such as donor-site pain, resorption, and limited size and shape of bone stock, autologous bone grafts are the gold standard for bone replacement (1,2). Although beta-tricalcium phosphate (β-TCP) is an established bone replacement material, wide clinical applications have been limited by lack of bone infiltration within its bulk form. Lithography-based ceramic manufacturing (LCM), a novel form of 3D printing, leverages photopolymerization to create β-TCP structures with higher feature resolution and surface quality than traditional techniques. This fabrication modality allows for increased control of the microarchitecture of the construct creating a porous design that facilitates bony infiltration and enables engineers to manufacture implant sizes and shapes that are not possible with traditional ceramic processing (3). While the technology is promising, limited research is available on LCM fabricated scaffolds in animal models.

Purpose:

This study explores the bone regenerative capacity of LCM fabricated 100% β-TCP scaffolds for the repair of critically-sized mandibular defects using a skeletally mature rabbit model.

Methods:

Unilateral critical-sized bony defects (10mm × 10mm, full thickness) were created in mandibular rami of New Zealand White rabbits (N=10). Seven rabbits underwent bony defect repair using LCM scaffolds made of 100% β-TCP and three rabbit defects remained unfilled as negative controls. Animals were euthanized after 8 weeks, and the mandibles were harvested, cleaned and scanned using micro–computed tomography (micro-CT) (μCT 40; SCANCO Medical, Bassersdorf, Switzerland) with 18 μm resolution. Imaging data was processed using Amira Software for three-dimensional (3D) reconstruction and volumetric analysis. Histological analysis was performed using Stevenel blue and van Gieson red picrofuchsin staining. Two-dimensional (2D) histomorphometric quantification was also performed.

Results:

There was no evidence of scaffold fracture during initial bony defect repair or at necroscopy. No bridging bone was seen in unfilled defects. 3D volumetric analysis revealed bridging bone within LCM β-TCP implant sites yielding 8.62±3.47% in fill of bone. Remaining scaffold volume was 32.95±3.21% of the defect. 2D histologic analysis revealed that mean regenerated bone and remaining scaffold was 14.09±6.7% and 34.14±2.5% of the defect respectively. Bone regeneration and remaining scaffold obtained via 2D histologic analysis were consistent with results from 3D volumetric analysis (p<0.05). Qualitative histological analysis of defect sites repaired with scaffolds revealed areas of vascularized woven and lamellar bone, with no evidence of ectopic bone, excess inflammation, or fracture.

Conclusion:

Bone regeneration in a short-term rabbit model following critical-sized mandibular defect repaired with LCM 100% β –TCP scaffolds demonstrate analogous radiographic and histologic properties as native bone.

References: 1. Younger EM, Chapman MW. Morbidity at bone graft donor sites. J Orthop Trauma. 1989;3(3):192-5. doi:10.1097/00005131-198909000-00002 2. Ahlmann E, Patzakis M, Roidis N, Shepherd L, Holtom P. Comparison of anterior and posterior iliac crest bone grafts in terms of harvest-site morbidity and functional outcomes. J Bone Joint Surg Am. May 2002;84(5):716-20. doi:10.2106/00004623-200205000-00003 3. Ryan E, Yin S. Compressive strength of β-TCP scaffolds fabricated via lithography-based manufacturing for bone tissue engineering. Ceramics International. 2022/06/01/ 2022;48(11):15516-15524. doi:https://doi.org/10.1016/j.ceramint.2022.02.085

Abstract Background: Craniosynostosis of the lambdoid suture is a rare malformation, accounting for 1-5% of all non-syndromic craniosynostoses, and may present as unilateral, bilateral, or in complex forms.1 Insufficient surgical correction may render patients with permanent mental deficits from intracranial hypertension and psychological distress from cosmetic deformity. Surgical techniques to address craniosynostoses have evolved, with common options being cranial vault remodeling (CVR), suturectomy, and more recently, spring-assisted suturectomy.2 There is a paucity of literature surrounding comparison between different techniques for treatment of lambdoid craniosynostosis. We present outcomes of patients with lambdoid craniosynostosis who underwent surgical intervention at a level one pediatric institution. Methods: A retrospective analysis of 26 patients who were surgically treated from January 2011 to February 2025 for lambdoid craniosynostosis was conducted. Patients with bilateral lambdoid craniosynostosis and those with three or more affected sutures were excluded from the study.

Results: Of the 26 patients, the average age at diagnosis by CT scan was 7.7 months (Range: 0.8-23.5 months). Sixty-five percent of the patients were male, and 77% of the patients were white. Isolated lambdoid craniosynostosis (77%) was the most common presentation, followed by lambdoid craniosynostosis with sagittal suture involvement (19%), and lambdoid craniosynostosis with metopic suture involvement (4%). The right lambdoid suture was involved in 54% of cases, and all patients were non-syndromic. Cranial vault remodeling (CVR) was completed in 65% of patients, suturectomy with molding helmet therapy (23%), and suturectomy with springs (12%). Of patients who underwent CVR, the median age at surgery was 10.7 months (Range: 7.2-26.7 months), median operative time was 340 minutes (Range: 240-528), 11.8% had additional suture involvement, 35% experienced postoperative complications, and no patients required additional surgery. Of patients who underwent suturectomy with molding helmet therapy, the median age at surgery was 3.7 months (Range: 2.2-4.6 months), median operative time was 259 minutes (Range: 210-407), 50% had additional suture involvement, 67% experienced postoperative complications, and 33% required secondary CVR due to refusion of sutures in one patient and residual head deformity in another. Of patients who underwent suturectomy with springs, the median age at surgery was 6.5 months (Range: 5-8.4), median operative time was 207 minutes (Range: 113-260), and no patients required additional surgery.

Conclusion: Suturectomy and molding helmet therapy has limited utility to address lambdoid craniosynostosis with 33% of cases in our study requiring additional vault remodeling surgery. While CVR is the current standard of care for craniosynostosis, cosmetic results are modest at best. Suturectomy with spring assisted-cranioplasty demonstrates promising correction of the primary deformity with low morbidity and may become more commonplace for lambdoid craniosynostosis.

References

1. Rhodes JL, Tye GW, Fearon JA. Craniosynostosis of the Lambdoid Suture. Semin Plast Surg. 2014;28(3):138-143. doi:10.1055/s-0034-1384809
2. Sameer Shakir, Melissa Roy, Amy Lee, Craig B. Birgfeld. Management of Sagittal and Lambdoid Craniosynostosis - ClinicalKey. Oral Maxillofac Surg Clin. 2022;34(3):421-433.

PURPOSE: Since 2005, 50 face transplants have been performed globally with a 10-year transplant survival of 74%, defined as either transplant loss or patient death (1). Facial vascularized composite allotransplants (fVCA) are a reconstructive option for patients with severe facial disfigurements due to trauma, disease, or malformation meant to improve psychosocial wellbeing and restore facial function and appearance. While data on graft survival has been reported, there is limited data on patient-reported outcome measures (PROMs) following fVCA. FACE-Q is a series of rigorously developed PROMs that can be used to measure appearance, facial function, health-related quality of life, and adverse effects in patients with facial conditions (2). This study utilizes FACE-Q concepts to investigate health-related quality of life and experience of care in individuals who underwent fVCA.

METHODS: Semi-structured interviews were conducted with patients recruited from the Plastic Surgery Transplantation Program at Brigham and Women's Hospital in Boston, MA. FACE-Q concepts informed the interview guide. Interviews were audio-recorded and transcribed. Data were coded using line-by-line coding and content analysis. Constant comparison was used to create themes and subthemes.

RESULTS: Interviews were conducted with the recipients of seven fVCAs with a mean age of 54.6 years old at the time of surgery (SD 10.9, range 31-68). A majority were White (85.7%, n=6), one was Black (14.3%), and 57.1% were men (n=4). Time between injury and fVCA varied from 2-10 years and follow-up time varied from 3-10 years. After fVCA, 85.7% (n=6) expressed overall satisfaction with their facial appearance; one expressed that appearance was unimportant. 71.4% (n=5) felt their postoperative faces were proportionate, one felt the donor's face was too large, and one felt the teeth and palate were too large.

A majority had an improved ability to eat postoperatively compared to preoperatively (85.7%, n=6); however, despite notable improvement, 57.1% (n=4) still experienced functional challenges with eating such as food falling out of their mouth or difficulty swallowing some solids. For drinking, 71.4% (n=5) experienced an improvement, but 42.9% (n=3) still experienced functional challenges such as difficulty drinking from a cup. Of those who had difficulty breathing preoperatively, 60.0% (n=3) experienced improved breathing postoperatively and 40.0% (n=2) experienced continued difficulty breathing postoperatively. Of those who discussed financial impact, 33.3% (n=2) experienced financial strain related to the fVCA. Overall, 57.1% (n=4) described decreased distress in social settings, for example, feeling like they fit in more with others, were stared at less, and were more comfortable going out in public and meeting new people after the fVCA. 85.7% (n=6) described a desire to help other fVCA recipients by sharing their experiences or a benefit to hearing the experiences of other fVCA recipients before their own. All patients expressed strong overall satisfaction with how fVCA impacted their lives. One recipient noted, "I'm so happy I got a second chance at life."

CONCLUSIONS: fVCA can impact the quality of life of patients with severe facial disfigurements by improving functional and psychosocial outcomes such as the ability to eat, drink, breathe, and exist comfortably in public spaces.

Citations: 1. Homsy P, Huelsboemer L, Barret JP, Blondeel P, Borsuk DE, Bula D, Gelb B, Infante-Cossio P, Lantieri L, Mardini S, Morelon E, Nasir S, Papay F, Petruzzo P, Rodriguez E, Özkan Ö, Özmen S, Pomahac B, Lassus P. An Update on the Survival of the First 50 Face Transplants Worldwide. JAMA Surg. 2024 Dec 1;159(12):1339-1345. doi: 10.1001/jamasurg.2024.3748. PMID: 39292472; PMCID: PMC11411447. 2. Klassen AF, Rae C, Riff W, Denadai R, Murray DJ, Bracken S, Courtemanche DJ, Bulstrode N, O'Hara J, Butler D, Goldstein J, Tassi A, Hol ML, Johnson D, Ganske IM, Kölby L, Benitez S, Breuning EE, Malic CC, Allen GC, Pusic AL, Cano S. FACE-Q craniofacial module: Part 2 Psychometric properties of newly developed scales for children and young adults with facial conditions. J Plast Reconstr Aesthet Surg. 2021 Sep;74(9):2330-2340. doi: 10.1016/j.bjps.2021.03.009. Epub 2021 Mar 25. PMID: 34172403.

Background: Primary rhinoplasty (PR) at the time of unilateral cleft lip repair intervenes directly on the stigmata of the cleft nasal deformity, correcting nasal tip under-projection, alar rim collapse, columellar deviation, alar base retraction, nasal sill widening, and nasal floor disruption with the goal of improved nasal symmetry. This study aims to assess that goal while comparing the anthropometric changes seen in the immediate and extended post-operative period to evaluate efficacy of PR techniques and their longevity with continued facial growth.

Methods: Retrospective review was utilized to identify all patients who underwent primary rhinoplasty by a single surgeon from 2018-2024. Anthropometric measurements were conducted on standardized 2D photographs pre-operatively, immediately post-operatively, and at 1 year post-operatively. Patients with standardized 3D photographs were treated as a subgroup, with measurements taken pre-operatively and immediately post-operatively. Anthropometrics were expressed as angles or ratios, with a ratio closer to 1 indicating improved symmetry. Analysis was conducted using paired t-tests and Wilcoxon signed-rank tests.

Results: 29 patients met inclusion criteria, of which 22 had standardized 2D photographs and 8 had 3D photographs. 1 patient had both 2D and 3D photographs and was included in both subgroup analyses. Within the 2D cohort comparing pre-op to immediate post-op measurements, nostril width (1.95±0.63 vs. 0.96±0.15), nasal base width (1.75±0.50 vs. 0.99±0.14), alar height (0.82±0.17 vs. 1.02±0.16), lateral lip height (LLH) (0.74±0.19 vs. 0.96±0.15), and philtral height (0.52±0.17 vs. 0.97±0.16) were significantly more symmetrical post-operation (p<0.05). Columellar deviation angle was improved post-operatively as well (13.96º±7.55º vs. 6.3º ±2.28º, p<0.05). Average columellar height was significantly increased post-operatively (0.77±0.20 vs. 1.13±0.27, p<0.001), however absolute difference from ideal (0.25±0.16 vs. 0.19±0.23, p=0.092) approached but did not reach significance. There was noted to be significant relapse in columellar height (1.11±0.27 vs. 0.90±0.15), nostril width (1.15±0.23 vs. 0.96±0.15), nasal base width (1.16±0.22 vs. 0.99±0.14), and alar height (0.88±0.07 vs. 1.02±0.16) when comparing immediate post-op to 1-year measurements (p<0.05). 3D anthropometrics corroborated 2D findings in the pre- to immediate post-operative period.

Conclusions: Thoughtful primary rhinoplasty at the time of unilateral cleft lip repair results in improved symmetry in multiple domains as assessed by anthropometric measurement in the immediate post-operative period and at one year follow up. However, some degree of nasal relapse should be anticipated in the long-term. Overcorrection at the time of PR may therefore be beneficial to achieve the best long-term nasal symmetry.

Background: Surgical treatment of fibrous dysplasia (FD) in the pediatric patients is an area that has not been fully studied. In addition, the literature lacks data on outcomes of different surgical treatment modalities. We examine our experience with surgical treatment of fibrous dysplasia in the pediatric population treated at Mayo Clinic.

Methods and Materials: A retrospective review of 20 patients who had at least one surgery for craniofacial fibrous dysplasia before 18 years of age.Diagnosis of fibrous dysplasia was determined by pathologic confirmation, or if there was no record of pathology findings, by presence of imaging features characteristic of fibrous dysplasia. Surgeries were categorized into three groups: (1) bone contouring only, (2) removal of the involved bone, contouring, and replacement of the contoured bone, or (3) en bloc resection of the involved bone and reconstruction with an alloplastic material, bone allograft, or bone autograft obtained from non-involved sites. Recurrence, need for secondary surgeries, and occurrence of post operative complications were the main primary outcomes.

Results: Monostotic FD was present in 55% of patients, while the remaining patients had polystotic bone involvement. The median age of presentation was 10 years old, with the most common presentations being visible growth in 50% and pain in 22%. Median age at first surgery was 12 (range 8 - 17). A total of 36 distinct surgical procedures were performed in 20 patients with a mean number of 1.9 surgeries/patient (range 1-5). Median follow up duration was 10.6 years (IQR 6.9 - 15.8 years). The maxilla was both the bone that was most commonly affected and most commonly operated on, being affected in 10 patients, with 7 of them undergoing 14 surgeries involving the bone. Other commonly involved regions included the zygoma (n = 6) and mandible (n = 6). The most common indication was visible or palpable growth in 63.9% of cases followed by confirmation of diagnosis (13.9%) and pain or headache (8.3%). The oldest age at which a pediatric patient needed a subsequent surgery was 23 years. Bone contouring only was the initial surgery in 15 patients; removal of the involved bone, contouring, and replacement of the contoured bone was the initial surgery in 1 patient; and en bloc resection of the involved bone and reconstruction was performed in 4 patients. In the group of patients who had undergone contouring procedures, 9 (55.6%) had recurrence requiring subsequent surgeries. Two of the nine needed multiple contouring and debulking surgeries, and one was treated with a subsequent complete resection and reconstruction. Patients with subsequent surgeries had a mean time to reoperation of 3.7 years (range 0.24 – 9.8). Four patients, all with monostotic calvarial disease, initially had a resection and reconstruction. None of the patients who had an en bloc resection and reconstruction, either as an initial or subsequent surgery, had recurrence. The remaining one patient had removal, contouring, and replacement of the involved bone, with no later recurrence of disease. Three of the contouring and debulking procedures, all involving the maxilla, resulted in a complication – two of which required surgical repair, while one (CN V2 numbness) did not. The two complications managed surgically were a lateral canthus distraction requiring one surgery to repair and a lower lid ectropion requiring two. The en bloc resection and reconstruction done to treat a recurrence was the only en bloc resection and reconstruction with a complication. It resulted in malocclusion that did not require further operations.

Conclusions: Surgical treatment of fibrous dysplasia has an acceptable risk profile, with surgical complications occurring in 10% of patients. Recurrence rate and need for secondary surgical procedures was relatively high in patients who had bone contouring only. This could be indicative of the natural continued progression of the disease in this age group.

Purpose Classical teaching suggests that the sagittal, coronal, and lambdoid sutures normally begin to close during the third decade of life. Therefore, in pediatric patients, the incidental finding of a closed suture is often alarming and raises concerns for potential craniosynostosis. Distinguishing craniosynostosis from normocephalic cranial suture closure can be challenging, particularly when a closed suture is discovered in an otherwise asymptomatic patient. This study investigates the prevalence of incidentally discovered cranial suture closure in healthy pediatric patients and its effects on cranial vault morphology.

Methods A retrospective review was performed of patients aged 0 to 18 who underwent head CT imaging in our institution's pediatric emergency department from May 2014 to April 2024. Patients with a diagnosis of any known craniofacial disorder, genetic syndrome, skull fracture, prior skull surgery, intracranial pathology such as ventriculoperitoneal shunt or hydrocephalus, and CT slice thickness > 1mm were excluded. All scans were reviewed on axial images by one of three attending neuroradiologists for the presence of open, partially fused, or completely fused metopic, sagittal, coronal, and lambdoid sutures. Cephalic index was computed as the quotient of greatest biparietal diameter, measured on the widest axial slices, and occipitofrontal diameter, measured on lateral scout radiographs. Patient demographics, age at time of scan, and indication for imaging were also recorded and compared with appropriate statistics.

Results A total of 1,119 scans met inclusion criteria, with a mean patient age of 8.35 years. Most patients were male (62.5%), and trauma was the most common indication for imaging (91.2%). Overall, complete sagittal suture closure was observed in 5.8% of patients, and 5.3% had partial closure. In patients aged 15 to 18, 17.3% of patients had either a partially (8.9%) or completely (8.4%) closed sagittal suture. Partial coronal and lambdoid suture closures were observed in 2.4% and 1.3% of patients, respectively, with complete closure being rare (≤0.4%). The metopic suture was closed in 83% of patients, with only 6.8% remaining open, consistent with its known closure early in life. Most coronal and lambdoid sutures exhibited symmetrical closure patterns, with strong correlations between the right and left coronal (r=0.90, p<0.0001) and right and left lambdoid sutures (r=0.94, p<0.0001). Sagittal suture closure showed a weaker but significant correlation with coronal and lambdoid sutures (r≈0.3, p<0.0001), indicating that while some individuals may have a predisposition for multi-suture closure, many experience isolated sagittal fusion. In multivariable regression analysis adjusting for age, sex, race, and involvement of other sutures, sagittal suture closure was associated with a decrease in cephalic index (β=-0.8, p=0.026). Among patients with a CI <70, 86.4% exhibited partial or complete sagittal suture closure, though most patients with complete sagittal suture closure remained normocephalic (CI>70, 83.1%).

Conclusions Our study found that up to 17% of pediatric patients may experience normocephalic partial (8.9%) or complete (8.4%) sagittal suture closure by age 18. While sagittal suture closure was linked to a decreased cephalic index, most patients with incidental closure remained normocephalic.

Background: Aplasia cutis congenita (ACC) is a rare condition characterized by the focal absence of skin and soft tissue, often seen on the scalp. (1) While most ACC lesions affect superficial tissues such as the epidermis or dermis and heal with minimal intervention, more severe disease can lead to complications such as exposed bone or dura, cerebrospinal fluid leaks, hemorrhage, or infection, which may require surgical intervention.( 1–4) Herein, we present all patients with ACC who were treated at a large academic pediatric hospital.

Patients and Methods: A retrospective chart review and analysis of patients diagnosed with ACC who were treated from January 2012 to October 2023 was conducted. Variables that were collected included patient demographics, birth history, co-morbidities, surgical intervention, types of conservative therapy, sites affected by ACC, depth of tissue involvement, and lesion outcomes.

Results: 162 patients with ACC were identified. Complete medical records detailing the management of ACC could be obtained on 144. Four patients were included in the initial cohort, but excluded from the final analysis since they died prior to resolution of the ACC. Of the 144 patients, 55% (79/144) were female and 47% (67/144) had other comorbidities including 12% (17/144) with internal organ abnormalities. The average area affected across the entire cohort was 7.05 cm2 (Range: 0.12 to 96 cm2). Patients who were typically managed with local wound care (21%) and those who received surgical treatment (26%) had average lesion sizes of 7.14 cm2 and 16.19 cm2 (range: 0.70 to 96 cm2), respectively. Incidentally, patients, managed with education and close follow up only, were noted to have smaller lesion sizes (1.87 cm2). Twelve percent of patients did not follow up.

Conclusions: ACC most commonly involves the superficial tissues of the scalp and is often seen with concomitant limb anomalies or comorbid conditions such as cardiac or neurologic defects. Conservative treatment and reassurance are often suitable for most lesions; however, surgical intervention may be necessary to mitigate risk of life-threatening complications. Further research should pursue indications for surgery, and the best practice for local wound care to achieve the fastest time to closure.

References 1. Alexandros B, Dimitrios G, Elias A, et al. Aplasia cutis congenita: Two case reports and discussion of the literature. Surg Neurol Int. 2017;8:273. doi:10.4103/sni.sni18817 2. Beekmans SJA, Don Griot JPW, Mulder JW. Split rib cranioplasty for aplasia cutis congenita and traumatic skull defects: more than 30 years of follow-up. J Craniofac Surg. 2007;18(3):594-597. doi:10.1097/scs.0b013e3180576f44 3. Santos de Oliveira R, Barros Jucá CE, Lopes Lins-Neto A, Aparecida do Carmo Rego M, Farina J, Machado HR. Aplasia cutis congenita of the scalp: is there a better treatment strategy? Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2006;22(9):1072-1079. doi:10.1007/s00381-006-0074-y 4. Harvey G, Solanki NS, Anderson PJ, Carney B, Snell BJ. Management of aplasia cutis congenita of the scalp. J Craniofac Surg. 2012;23(6):1662-1664. doi:10.1097/SCS.0b013e31826542de

Background: Palatal fistulae remain the most challenging complication following cleft palate repair, with incidence rates ranging from 2.4% to 35%. They pose significant morbidity for patients, including oronasal regurgitation, speech impairment, and failure to thrive. While extensively reported in pediatric populations, palatal fistula repair in adults remains poorly documented, with adult patients presenting with significant challenges due to chronic defects, excessive scarring, and limited tissue elasticity, necessitating the use of various techniques for successful closure. This study aims to evaluate the surgical management of adult palatal fistulas, highlighting the complexities, recurrence rates, and post-operative speech outcomes, providing insight into the most effective surgical techniques for fistula closure in adult patients. Methods: A retrospective review was conducted of adult patients aged 18 and above presenting with palatal fistulas at CLAPP Hospital, Lahore, Pakistan, over nine years (2015–2023). Data were collected on basic demographic characteristics, fistula size and location, surgical techniques, recurrence rates, and speech outcomes. Various surgical techniques, including Secondary Bardach Repair, Secondary Langenbeck Repair, Buccal Sulcus Flap, Buccinator Myomucosal Flap, and Tongue Flap, were employed based on the size and location of defects. Speech assessment was performed pre- and post-operatively using a modified Ann Kummer Speech Evaluation Protocol. Statistical analysis was conducted using Pearson's Chi-squared test, Fisher's Exact Test, and Welch's t-test, with significance set at p < 0.05 using R Statistical Software (Version 4.3.2; R Core Team 2024). Results: A total of 245 adult patients (152 males, 93 females; mean age 24.7 ± 6.47 years) were included. Most fistulas were located in the anterior (26.5%) and posterior (8.6%) hard palate, with a substantial proportion extending through multiple regions (26.9%). Fistula sizes varied, with 29.8% classified as S1 (1–5mm), 26.5% as S2 (6–10mm), 17.6% as S3 (11–15mm), and 20.4% as S4 (>15mm). The most commonly utilized surgical technique was the Secondary Bardach Repair (59.2%), followed by Buccal Sulcus Flap (13.5%), Secondary Langenbeck Repair (8.6%), and Buccinator Myomucosal Flap (5.3%). Post-operatively, fistula recurrence was observed in 21.6% of cases, with significantly higher recurrence in patients presenting with recurrent fistulae (p = 0.0209). Speech assessment revealed significant improvement after surgical repair. Pre-operatively, 81.2% of patients exhibited severe hypernasality (G3), while post-operatively, this reduced to 18.4%. The mean composite speech score improved from 14.0 to 11.4 (p < 0.001). Patients experiencing a recurrence of fistula had significantly worse speech outcomes compared to those who did not (p < 0.001). Conclusion: Palatal fistula repair in adults remains a challenging but essential intervention to improve speech and overall quality of life. This study provides valuable insights into the surgical management, recurrence risks, and functional outcomes of this condition, emphasizing the need for future multi-center studies with longer follow-up periods to refine treatment protocols for this underrepresented patient population.