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Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
Published on: November 4, 2025
A Treatment Algorithm for Patients Presenting with Sagittal Craniosynostosis after the Age of 1 Year
Edward J Ruane1,2, Catharine B Garland1,2, Liliana Camison1,2
1Pittsburgh, Pa.; and Madison, Wis.
Insights
Delayed diagnosis of sagittal craniosynostosis in older children has a low risk of intracranial hypertension. Most patients can be managed non-surgically, avoiding invasive monitoring.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Ophthalmology
Background:
- Sagittal craniosynostosis, a birth defect causing an elongated head shape, is typically treated surgically soon after birth.
- This study focuses on a unique group: patients with mild, missed, or late-developing sagittal craniosynostosis.
Purpose of the Study:
- To evaluate the authors' treatment protocol for delayed sagittal craniosynostosis diagnosis.
- To assess the incidence of intracranial hypertension in this patient population.
Main Methods:
- A prospective cohort study included 52 patients over 1 year old with isolated sagittal craniosynostosis.
- Evaluations involved craniofacial surgery, neurosurgery, ophthalmology, fundus examinations, and visual evoked potentials.
Main Results:
- Only 17.3% of patients required surgical intervention.
- Intracranial hypertension was identified in 25% of patients undergoing monitoring after inconclusive ophthalmologic evaluations.
- The majority of patients presented without signs of increased intracranial pressure and are managed conservatively.
Conclusions:
- A non-surgical management approach appears safe for many patients with delayed sagittal craniosynostosis diagnosis.
- The risk of intracranial hypertension is low in this specific patient cohort.
- Serial evaluation is crucial for monitoring patients managed non-surgically.
Background:
Sagittal craniosynostosis typically presents shortly after birth, with a scaphocephalic head shape, and is addressed surgically for functional and aesthetic concerns. This study highlights the authors' experience with a challenging patient population: those with phenotypically mild, missed, and late-developing sagittal craniosynostosis.
Methods:
A prospective cohort study was conducted for all cases of sagittal craniosynostosis presenting to the authors' institution between July of 2013 and December of 2015. Patients older than 1 year with isolated sagittal craniosynostosis were included. All children were evaluated by craniofacial surgery, neurosurgery, and ophthalmology departments. All patients had dilated fundus examinations and visual evoked potentials.
Results:
Fifty-two patients met inclusion criteria. Only nine patients have been treated surgically (17.3 percent). Two patients underwent operative correction for obvious scaphocephaly. Four patients who presented with concerning ophthalmologic evaluations and another patient with classic intracranial hypertension-related headaches underwent cranial vault expansion. Eight patients presented with inconclusive ophthalmologic evaluations. These patients were admitted for intracranial pressure monitoring, of which two were found to have elevated levels (25 percent) and underwent operative intervention. Thirty-seven other patients presented with isolated sagittal craniosynostosis in the setting of overall normocephaly without any signs concerning for intracranial hypertension. These patients continue to undergo serial evaluation.
Conclusions:
The authors describe their treatment protocol for a large series of patients presenting with the delayed diagnosis of sagittal craniosynostosis. Based on the early experience of the authors' center with this protocol, the risk of intracranial hypertension appears to be low in this population. The majority of patients to date have been managed nonsurgically without invasive monitoring.
Clinical Question/Level Of Evidence:
Therapeutic, IV.
