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Prevalence and Clinical Associations of Peripapillary Hyperreflective Ovoid Mass-like Structures in Craniosynostosis.
J Neuroophthalmol
January 2025
Department of Ophthalmology (JGJ-C, TE, Y-HC, LRD, RAG), Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Frank H. Netter Medical School (JGJ-C), North Haven, Connecticut; and Department of Anesthesiology (DZ), Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Background: Patients with craniosynostosis are at high risk of developing elevated intracranial pressure (ICP) causing papilledema and secondary optic atrophy. Diagnosing and monitoring optic neuropathy is challenging because of multiple causes of vision loss including exposure keratopathy, amblyopia, and cognitive delays that limit examination. Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are an optical coherence tomography (OCT) finding reported in association with papilledema and optic neuropathy.
View Article and Find Full Text PDFFrontal Sinus Volume in Normocephalic Nonsyndromic Sagittal Craniosynostosis: A Comparative Study.
J Craniofac Surg
January 2025
Division of Plastic and Reconstructive Surgery, University of Mississippi Medical Center.
Purpose: This study aimed to investigate frontal sinus volume as a potential indicator of cranial compensatory growth in unoperated normocephalic nonsyndromic sagittal craniosynostosis (NNSC) patients compared with age-matched and sex-matched controls. Previous studies have suggested that frontal sinus volume is suppressed in unoperated craniosynostosis and may be an intracranial space conservation phenomenon.
Methods: Head computed tomographies (CTs) from 22 unoperated NNSC patients at our institution were utilized in this study and matched with age-matched and sex-matched control subjects.
Long-Term Outcomes of Regional Morphology in Spring-Assisted Surgery and Cranial Vault Remodeling.
Plast Reconstr Surg
February 2025
From the Departments of Plastic and Reconstructive Surgery.
Background: Spring-assisted surgery (SAS) and cranial vault remodeling (CVR) are widely used surgical techniques to correct sagittal craniosynostosis (SC). The authors evaluated changes in regional morphology of patients with SC who had undergone SAS or CVR, using the frontal bossing index (FBI), occipital bulleting index, vertex narrowing index (VNI), and scaphocephalic severity index (SCI) to capture differences in anterior protrusion, posterior protrusion, width restriction, and global dysmorphology, respectively.
Methods: Indices were measured on computed tomography and 3-dimensional photographs (n = 788) of 257 patients with SC from 2001 through 2022 who underwent SAS (n = 177) or CVR (n = 80).
Two-Year Progressive Cranial Changes Following the Melbourne Technique for Sagittal Craniosynostosis.
Cleft Palate Craniofac J
January 2025
Department of Plastic Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Objective: The Melbourne technique for total cranial vault remodeling aims to address all aspects of scaphocephaly in sagittal craniosynostosis. These features include anterior-posterior excessive length, anteriorly displaced vertex position, frontal bossing, vertex narrowing, and occipital bulleting. This study aimed to determine the progressive cranial changes that occur following the Melbourne technique for sagittal craniosynostosis.
View Article and Find Full Text PDFJansen metaphyseal chondrodysplasia: analysis of craniofacial manifestations.
JBMR Plus
February 2025
Radiology and Imaging Sciences, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD 20892, United States.
Jansen metaphyseal chondrodysplasia (JMC) is an ultra-rare disorder caused by constitutive activation of parathyroid hormone type 1 receptor (PTH1R). We sought to characterize the craniofacial phenotype of patients with the disease. Six patients with genetically confirmed JMC underwent comprehensive craniofacial phenotyping revealing a distinct facial appearance that prompted a cephalometric analysis demonstrating a pattern of mandibular retrognathia.
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