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Introduction: In an effort to maximize benefit and minimize morbidity when performing fronto-orbital distraction osteogenesis (FODO) for unilateral coronal synostosis (UCS), we have transitioned to an endoscopic-assisted approach (“endo-FODO”). This study compares photogrammetric outcomes of patients who underwent FODO via an endoscopic-assisted versus open approach. Methods: We retrospectively reviewed patients treated for UCS from 2013 to 2023. Photogrammetric outcomes at one to three years postoperatively were compared between patients who underwent endo-FODO and age- and sex-matched controls who underwent open FODO. Differences between pre- and postoperative periorbital symmetry ratios, canthal tilt symmetry, and orbital dystopia angle (ODA) were calculated. Results: Twenty patients (ten per group) underwent surgery at a mean age of 6.1 ± 1.8 and 5.4 ± 1.1 months (p = 0.426) and were photographed at 1.6 ± 0.9 and 1.8 ± 0.9 years (p = 0.597) postoperatively in the endo-FODO and open FODO groups, respectively. Patients who underwent endo-FODO demonstrated significant improvements in margin-reflex distance 1 (MRD1) symmetry ratio (p = 0.004), palpebral height symmetry ratio (p = 0.004), canthal tilt symmetry (p = 0.020), and ODA (p = 0.009). Patients who underwent open FODO likewise demonstrated significant improvements in MRD1 symmetry ratio (p = 0.004), palpebral height symmetry ratio (p = 0.033), and ODA (p = 0.004). All postoperative measurements as well as degrees of improvement were similar between groups (p > 0.05). Conclusions: Endo- and open FODO were associated with significant and comparable improvements in soft tissue periorbital symmetry and orbital dystopia at nearly two years postoperatively. While continued follow-up until cranial maturity is needed to assess the durability of aesthetic results, these data support a minimally invasive, endoscopic alternative to fronto-orbital distraction. [ABSTRACT FROM AUTHOR]

Objective: Measuring disability as a concept of impaired global function enables beneficiaries of treatment, the impact of treatment, and targets of health system investment to be rigorously assessed. Measures of disability are not well established for cleft lip and palate. This study aims to systematically review disability weight (DW) studies pertaining to orofacial clefts (OFCs) and identify methodological strengths and shortcomings of each approach. Design: Systematic literature review of studies that met the following criteria: (1) peer-reviewed publication, (2) focus on disability valuation, (3) mention orofacial clefts, and (4) publication January 2001-December 2021. Setting: None. Patients/Participants: None. Interventions: None. Main Outcome Measure(s): Disability weight method of valuation and the value itself. Results: The final search strategy yielded 1,067 studies. Seven manuscripts were ultimately included for data extraction. The disability weights used in our studies, including those newly generated or taken from the Global Burden of Disease Studies (GBD), ranged widely for isolated cleft lip (0.0–0.100) and cleft palate with or without cleft lip (0.0−0.269). The GBD studies limited their consideration of cleft sequelae informing disability weights to impact on appearance and speech-related concerns, while other studies accounted for comorbidities such as pain and social stigma. Conclusions: Current measures of cleft disability are sparse, inadequately reflect the comprehensive impact of an OFC on function and socialization, and are limited in detail or supporting evidence. Use of a comprehensive health state description in evaluating disability weights offers a realistic means of accurately representing the diverse sequelae of an OFC. [ABSTRACT FROM AUTHOR]

Objective: Approximately 30% of patients with a history of repaired cleft palate (CP) go on to suffer from velopharyngeal dysfunction (VPD). This study discusses the operative management of VPD and postoperative speech outcomes in a cohort of CP patients. Setting: An academic tertiary pediatric care center. Methods: Retrospective cohort study. Patients: Patients with history of repaired CP (Veau I-IV) who underwent operative management of VPD between January 1st, 2010 and December 31st, 2020. Operative modalities were posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), Furlow palate re-repair, and buccal myomucosal flap palate lengthening (PL). Outcome measures: The primary outcome measure is postoperative speech improvement evaluated by the Pittsburgh Weighted Speech Scale (PWSS). Results: 97 patients met inclusion criteria. 38 patients with previous straight-line primary palatoplasty underwent Furlow re-repair; these patients were significantly younger (7.62 vs 11.14, P <.001) and were more likely to have severe VPD per PWSS (OR 4.28, P <.01, 95% CI 1.46-12.56) when compared to VPD patients with previous Furlow repair. 21.1% of these patients required an additional non-revisional VPD procedure. The remaining patients underwent a non-revision procedure (26 PPF, 22 SPP, 11 PL); all experienced significant (P <.001 on paired t-test) reductions in PWSS total and subgroup VPD severity scores without difference in improvement between operation types. SPP was statistically associated with all-cause complication (OR 2.79, 95% CI 1.03-7.59, P <.05) and hyponasality (OR 3.27, 95% CI 1.112-9.630, P <.05). Conclusion: Furlow re-repair reduced need for additional VPD operations. Speech outcomes between non-revisional operations are comparable, but increased complications were seen in SPP. [ABSTRACT FROM AUTHOR]

Objective: Determine interactions between geospatial and socioeconomic factors influencing cleft lip and/or cleft palate (CL/P) management and outcomes. Design: Retrospective review and outcomes analysis (n = 740). Setting: Urban academic tertiary care center. Patients: 740 patients undergoing primary (CL/P) surgery from 2009 to 2019. Main Outcomes Measures: Prenatal evaluation by plastic surgery, nasoalveolar molding, cleft lip adhesion, and age at CL/P surgery. Results: Prenatal evaluation by plastic surgery was predicted by the interaction between higher patient median block group income and shorter patient distance from the care center (OR = 1.07, p = 0.022). Nasoalveolar molding was also predicted by the interaction between higher patient median block group income and shorter distance from the care center (OR = 1.28, p = 0.016), whereas cleft lip adhesion was predicted by higher patient median block group income alone (OR = 0.41, p < 0.001). Lower patient median block group income predicted later age at cleft lip (β = −67.25, p = 0.011) and cleft palate (β = −46.35, p = 0.050) repair surgery. Conclusions: Distance from the care center and lower median income by block group interacted to significantly predict prenatal evaluation by plastic surgery and nasoalveolar molding for patients with CL/P at a large, urban, tertiary care center. Patients living farthest from the care center who received prenatal evaluation by plastic surgery or who underwent nasoalveolar molding had higher median block group income. Future work will determine mechanisms perpetuating these barriers to care. [ABSTRACT FROM AUTHOR]

Epidermolysis bullosa is a genetic skin disorder characterized by blister formation from mechanical trauma. Dystrophic epidermolysis bullosa (DEB) is caused by mutations in the COL7A1 gene presenting as generalized blisters from birth, which can result in extensive scarring, alopecia, esophageal stenosis, corneal erosions, and nail dystrophy. This disease also often leads to pseudosyndactyly of the digits from the closure of webspaces, progressing to a "mitten hand" deformity. Although traditional and current treatment for DEB is largely supportive with wound care and iterative surgical pseudosyndactyly release, emerging gene therapies and novel skin grafts may offer promising treatment. Studies published in the early 2020s have used HSV-1 vectors expressing missing COL7A1 genes to restore collagen function. One of these treatments, B-VEC, is an HSV-1-based topical gene therapy designed to restore collagen 7 by delivering the COL7A1 gene, leveraging a differentiated HSV-1 vector platform that evades the patient's immune system response. Other work has been performed to retrovirally modify autologous keratinocytes, but limitations of this process include increased labor in harvesting and engineering autologous cells. This article provides an overview of DEB treatment with an emphasis on emerging gene therapies and novel skin grafts, especially as they pertain to pseudosyndactyly treatment. [ABSTRACT FROM AUTHOR]