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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
Slit ventricle syndrome and early-onset secondary craniosynostosis in an infant
Hyun Gee Ryoo1, Seung-Ki Kim1, Jung-Eun Cheon2
1Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Republic of Korea.
Insights
This case study highlights slit ventricle syndrome and secondary craniosynostosis in an infant following shunt surgery for hydrocephalus. Early diagnosis through head circumference monitoring is crucial for timely intervention and preventing increased intracranial pressure.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Medical Case Reports
Background:
- Shunt surgery is a standard treatment for infantile hydrocephalus.
- Slit ventricle syndrome and secondary craniosynostosis are potential late complications of shunt placement, sometimes occurring concurrently.
Purpose of the Study:
- To present a case of early-onset secondary craniosynostosis co-occurring with slit ventricle syndrome in an infant.
- To emphasize the diagnostic challenges and management of increased intracranial pressure in this complex scenario.
Main Methods:
- A case report detailing an infant with Dandy-Walker malformation who underwent ventriculoperitoneal shunt placement.
- Monitoring of head circumference revealed a significant decrease post-surgery.
- Subsequent development of increased intracranial pressure symptoms led to shunt revision and diagnosis of sagittal synostosis and slit ventricle syndrome.
Main Results:
- The infant presented with symptoms of hydrocephalus, followed by decreased head circumference after shunt surgery.
- The patient developed slit ventricle syndrome and secondary craniosynostosis, leading to a critical increase in intracranial pressure.
- Emergent cranial expansion surgery with shunt revision successfully resolved the increased intracranial pressure.
Conclusions:
- Clinical vigilance and consistent head circumference monitoring are essential for diagnosing slit ventricle syndrome and secondary craniosynostosis post-shunt surgery.
- Prompt diagnosis and intervention are critical, even in young children, to manage elevated intracranial pressure effectively.
Patient:
Female, 14 months
Final Diagnosis:
Slit ventricle syndrome Symptoms: Hydrocephalus • lethargy and seizure • vomiting
Medication:
- Clinical Procedure: - Specialty: Pediatrics and Neonatology.
Objective:
Challenging differential diagnosis.
Background:
Shunt surgery is a common solution for hydrocephalus in infancy. Slit ventricle syndrome and secondary craniosynostosis are late-onset complications after shunt placement; these 2 conditions occasionally occur together.
Case Report:
We report a case of early-onset secondary craniosynostosis with slit ventricle syndrome after shunt surgery in an infant, which led to a catastrophic increase in intracranial pressure (ICP). A 4-month-old girl with a Dandy-Walker malformation underwent a ventriculoperitoneal shunt procedure. Her head circumference (HC) gradually decreased to approximately the 5(th) percentile for her age group after shunt surgery. Seven months later, she developed increased ICP symptoms and underwent a shunt revision with a diagnosis of shunt malfunction. Her symptoms were temporarily relieved, but she repeatedly visited the emergency room (ER) for the same symptoms and finally collapsed, with an abrupt increase in ICP, 3 months later. Further evaluation revealed the emergence of sagittal synostosis at 7 months after initial shunt surgery. After reviewing all clinical data, slit ventricle syndrome combined with secondary craniosynostosis was diagnosed. Emergent cranial expansion surgery with shunt revision was performed, and the increased ICP signs subsided in the following days.
Conclusions:
Clinical suspicion and long-term HC monitoring are important in the diagnosis of slit ventricle syndrome and secondary craniosynostosis after shunt surgery, even in infants and young children.
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