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Wormian bones: expanded differential diagnosis and implications for abnormal head shape in infancy
Noah E Alter1, James L Rogers2, Marcelina Puc3
1Department of Plastic Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.
Insights
Wormian bones (WB), or accessory ossicles, are uncommon in children but can indicate underlying skeletal conditions. Their presence, particularly multiple bones, warrants consideration in diagnosing abnormal head shape.
Area of Science:
- Pediatric Radiology
- Developmental Biology
- Craniomaxillofacial Surgery
Background:
- Wormian bones (WB) are accessory ossicles found within cranial sutures.
- While often asymptomatic, numerous WB can be associated with genetic and developmental disorders.
- Understanding WB prevalence and associations is crucial for pediatric diagnostics.
Purpose of the Study:
- To determine the prevalence and anatomical distribution of WB in pediatric patients.
- To investigate clinical associations, such as genetic disorders and abnormal head shape, with WB presence.
- To evaluate the diagnostic significance of WB in pediatric cranial CT imaging.
Main Methods:
- Retrospective review of cranial CT scans from pediatric patients (0-18 years).
- Radiological confirmation of WB and collection of demographic and clinical data.
- Analysis of WB prevalence, location, number, and associated conditions, including cephalic index.
Main Results:
- Wormian bones were identified in 0.57% of 13,519 pediatric patients, increasing to 2.1% in a 5-year clinic cohort.
- The lambdoid suture was the most frequent site for WB.
- Multiple WB (≥2) were common (67.5%), and 16.8% of affected patients had associated skeletal or craniofacial conditions, notably craniosynostosis.
Conclusions:
- Wormian bones are found in a small percentage of pediatric patients, with higher prevalence in specialized clinics.
- WB can co-exist with conditions like craniosynostosis and osteogenesis imperfecta.
- Clinicians should consider WB in the differential diagnosis of abnormal head shape in infants, especially in subspecialty settings.
Purpose:
Wormian bones (WB) are accessory ossicles that develop within cranial sutures. While typically benign, their presence in large numbers has been associated with various genetic and developmental disorders. This study aims to characterize the prevalence, anatomical distribution, and clinical associations of WB in a pediatric population undergoing cranial CT imaging.
Methods:
A retrospective review was conducted at Monroe Carell Jr. Children's Hospital at Vanderbilt. Pediatric patients aged 0 to 18 years who underwent cranial CT imaging for any clinical indication were included. WB were radiologically confirmed, and demographic data, cephalic index, and comorbidities were collected and analyzed.
Results:
Among the 13,519 patients who underwent cranial CT imaging, 77 (0.57%) had radiologically confirmed WB, totaling 476 ossicles. The prevalence increased to 2.1% when examining our clinic cohort over a 5-year period. The lambdoid suture was the most common site (343/476, 72.1%), followed by the posterior fontanelle (53/476, 11.1%). Multiple WB (≥ 2) represented the most common phenotype (52/77, 67.5% of cases), and 13 patients (16.8%) had at least one associated skeletal or craniofacial condition, most commonly craniosynostosis (10/13, 76.9%). Cephalic index analysis demonstrated a predominance of brachycephaly (54/77, 70.1%).
Conclusion:
This study presents a comprehensive evaluation of WB in a large pediatric cohort. WB may co-exist with craniosynostosis or other skeletal conditions such as osteogenesis imperfecta. Given the greater prevalence of WB compared to craniosynostosis (2.1%: clinic cohort & 0.57%: institutional cohort vs. 0.05%), clinicians should consider WB in the differential diagnosis of abnormal head shape in infancy, particularly in a subspecialist practice.
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