A Comparison of Surgical Techniques for Macroglossia in Beckwith-Wiedemann Syndrome.

Background: Tongue reduction surgery can improve symptomatic macroglossia in Beckwith-Wiedemann syndrome (BWS), but there is no consensus on the optimal surgical technique. This study assesses trends and outcomes of several tongue reduction techniques for treating macroglossia in BWS. Using perioperative and polysomnographic data, we present our institution's rationale for increasingly adopting the peripheral resection technique.

Clarifying the Relationship Between Orbito-Zygomatic and Mandibular Dysmorphology in Treacher Collins Syndrome.

Introduction: This study aims to clarify the relationship between orbito-zygomatic and mandibular dysmorphology as well as their associations with the most frequently observed craniofacial anomalies in patients with Treacher Collins syndrome (TCS).

Methods: The authors retrospectively reviewed patients with TCS who presented between 2000 and 2023. Patients were included if they had at least one computed tomography scan performed before skeletal reconstruction along with clinical photographs.

Quality of Life in Craniofacial Microsomia: Resilience Through Young Adulthood.

ObjectiveWhile studies have analyzed the quality of life (QOL) in children with craniofacial microsomia (CFM), little is known about QOL in adults with CFM. This study compares QOL in adults with CFM to a comparative group using a validated questionnaire.DesignCross-sectional.

Long-term Outcomes of Infant Ear Molding: A 10-year Single-Surgeon Experience.

Background: This study assesses long-term results of ear molding and factors associated with improved outcomes.

Methods: Consecutive infants treated by a single surgeon from 2012 to 2023 were reviewed. Photographs before device application (time T0) and within 6 months of device removal (time T1) were collected.

Conservative Management of Macroglossia in Beckwith-Wiedemann Syndrome.

Introduction: While severe macroglossia in Beckwith-Wiedemann syndrome (BWS) is often treated with tongue reduction surgery, most patients with mild to moderate macroglossia are nonsurgically managed. Given that outcomes for these conservatively managed patients are not well characterized, this study aims to describe the natural history of patients with macroglossia and BWS who did not undergo early surgery.

Methods: Records of patients with BWS and macroglossia seen between 2004-2024 were reviewed.

Minimally Invasive Versus Open Cranial Vault Remodeling Procedures for Single-Suture Craniosynostosis: What Do We Know?

Craniosynostosis is a congenital condition characterized by the premature fusion of cranial sutures, disrupting normal skull and potentially brain growth. The current gold standard for nonsyndromic single-suture craniosynostosis is open cranial vault remodeling, which was pioneered by surgeons like Tessier and Whitaker in the 1970s. Recent advances have led to minimally invasive techniques, such as endoscopic-assisted strip craniectomy with helmeting, spring-assisted cranioplasty, and distraction osteogenesis.

Primary Rhinoplasty for Unilateral Cleft Lip: A Long-Term Cohort Assessment of Aesthetic and Anthropometric Outcomes.

The decision to undertake rhinoplasty maneuvers during cleft lip repair remains controversial. Little data compare long-term outcomes with and without primary rhinoplasty (PR). This study compared nasolabial outcomes in cohorts with unilateral cleft lip (UCL) treated with and without PR at the Children's Hospital of Philadelphia using standardized aesthetic and anthropometric assessments.

Endoscopic-assisted versus open fronto-orbital distraction for unicoronal craniosynostosis: morphometric and technique considerations.

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.

Long-Term Surgical Outcomes of Intermediate Cleft Rhinoplasty.

Background: The risk-to-benefit ratio of operating on the cleft nasal deformity in the period of mixed dentition remains debated. This study characterizes the authors' 18-year experience with intermediate cleft rhinoplasties to add data and nuance to the discussion.

Methods: The authors performed a retrospective cohort study of patients who underwent intermediate cleft rhinoplasty from 2006 to 2023.

Condylar Volumetric Asymmetry Predicts Need for Surgical Intervention in Patients With Cleft Lip and/or Palate.

Objective: In this study, the authors investigate the associations between the mandibular condyle and facial asymmetry in patients with cleft lip and/or palate (CLP).

Methods: Condylar volume was calculated by measuring the bony volume of the posterior mandible superior to the sigmoid notch in skeletally mature patients with CLP and controls. Relationships between condylar asymmetry, facial deviation, and malocclusion were compared using t-tests, correlation analysis, and receiver operating characteristic curves.

The Evolution of Unicoronal Synostosis Correction: Long-Term Aesthetics of Fronto-Orbital Distraction versus Advancement.

Background: This study compares the long-term aesthetic outcomes of patients with unicoronal synostosis (UCS) who underwent fronto-orbital distraction osteogenesis (FODO) versus traditional fronto-orbital advancement and remodeling (FOAR).

Methods: Patients treated for nonsyndromic UCS from 2009 to 2023 were retrospectively reviewed. Perioperative and complication characteristics were compared between all patients who underwent FOAR, open FODO, and endoscope-assisted FODO (endo-FODO).

Surgical Management of 2350 Pediatric Dermoid Cysts.

Background: The authors examined operative and pathologic findings of a large series of dermoid cysts at a high-volume pediatric hospital over 23 years.

Methods: A retrospective review was performed of all dermoid cysts excised from 2000 to 2023 at the Children's Hospital of Philadelphia. Lesions were classified by location.

Long-term photogrammetric outcomes of midface advancement in Apert syndrome: are we nearing normal?

Background: The aesthetic goals of midface surgery in Apert syndrome are to correct the multi-planar midface deficiency and normalize facial ratios. This study characterizes the long-term photogrammetric outcomes of midface advancement in Apert syndrome.

Methods: Patients with Apert syndrome who underwent midface distraction from 2000 to 2023 were retrospectively reviewed.

Long-Term Surgical Outcomes of Primary Rhinoplasty.

ObjectiveTo examine the impact of primary rhinoplasty on subsequent rhinoplasties for patients with cleft lip nasal deformity.DesignRetrospective cohort study.SettingTertiary care pediatric hospital.

The Peripheral Reduction With Keyhole Tongue Reduction Technique for Macroglossia in Beckwith-Wiedemann Syndrome.

Objective: Patients with Beckwith-Wiedemann syndrome commonly have macroglossia, which can negatively affect dentoskeletal development, breathing, speaking, and eating. Tongue reduction surgery can improve symptoms, but there is no standardized surgical approach.

Methods: A video and observational commentary highlighting the effectiveness of a tongue reduction technique for BWS were presented.

Optic Nerve Sheath Diameter on Computed Tomography Scans Reflects Elevated Intracranial Pressure in Patients with Craniosynostosis.

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.

A coddling of the sagittal suture: inequality in spring-assisted expansion.

Purpose: We examined differences in long-term morphometric outcomes of spring-mediated cranioplasty (SMC) for various forms of isolated nonsyndromic sagittal craniosynostosis.

Methods: A retrospective review was performed of children who underwent SMC from 2011 to 2020 at the Children's Hospital of Philadelphia. Cephalic indices (CI), Whitaker grades, parietal bone thickness, and degree of suture fusion were assessed.

Genetic Heterogeneity, Craniofacial Surgical Burden, and Surgical Techniques in Patients With Saethre-Chotzen Syndrome.

Objective: While genotype correlates with phenotype in patients with many forms of syndromic craniosynostosis, the relationship between molecular diagnosis and craniofacial surgical history in patients with Saethre-Chotzen syndrome (SCS) is more variable. This manuscript characterizes that relationship and evaluates operative trends in these patients over the past 3 decades.

Methods: Demographic information, molecular diagnosis, and craniofacial surgical history in patients born with SCS between 1989 and 2023 were compared with appropriate statistics, including t tests and analysis of variance.

Early posterior vault distraction osteogenesis changes the syndromic craniosynostosis treatment paradigm: long-term outcomes of a 23-year cohort study.

Background: Early surgical management of syndromic craniosynostosis varies widely between centers, with patients typically undergoing multiple intracranial procedures through childhood. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures.

Methods: We retrospectively analyzed the longitudinal operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up.