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Natural history of hydrocephalus in children with spinal open neural tube defect
1Neurosurgery Division, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.
Insights
Hydrocephalus is common in Spinal Open Neural Tube Defect (SONTD) patients, often linked to Chiari malformation type II. Early MRI and prompt treatment are crucial for managing SONTD-associated hydrocephalus.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neuroscience
- Clinical Neurology
Background:
- Long-term prognosis for Spinal Open Neural Tube Defect (SONTD)-associated hydrocephalus remains poorly understood.
- This study aimed to determine the incidence and natural history of hydrocephalus in SONTD patients.
Purpose of the Study:
- To investigate the incidence and natural history of hydrocephalus in patients with SONTD.
- To analyze the management and outcomes of hydrocephalus in SONTD patients.
Main Methods:
- Retrospective study of 82 SONTD patients over 1-16 years.
- Patients classified into groups with active, compensated, or no hydrocephalus.
- Analysis of treatment timing, complications, and hydrocephalus status.
Main Results:
- Hydrocephalus affected 72% of SONTD patients, frequently associated with Chiari malformation type II.
- Ventriculoperitoneal shunt (VPS) and endoscopic third ventriculostomy (ETV) were primary treatments; VPS revision/replacement occurred in 19.6% due to complications.
- No treatment was required for patients with compensated or no hydrocephalus during follow-up.
Conclusions:
- Hydrocephalus is prevalent in SONTD patients with Myelomeningocele and Chiari malformation type II, necessitating close monitoring and timely intervention.
- Routine brain and craniocervical junction MRI is recommended for SONTD patients to assess ventricular size and Chiari malformation type II.
Background:
The long-term prognosis of patients with Spinal Open Neural Tube Defect (SONTD)-associated hydrocephalus is not well known. This study was conducted to ascertain the incidence and natural history of hydrocephalus in patients with SONTD.
Methods:
All 82 patients with SONTD referred to Neurosurgery/Spina Bifida Clinics at King Khalid University Hospital, Riyadh, Saudi Arabia (January 1995 - July 2010) were studied and followed for a period of 1-16 years. Patients were divided into three groups: Group "A" with active hydrocephalus treated with ventriculoperitoneal shunt (VPS), or endoscopic third ventriculostomy (ETV); Group "B" with compensated hydrocephalus; and Group "C" with no hydrocephalus. Timing of shunt insertion, complications of treatment and status of hydrocephalus were analyzed.
Results:
The mean age of the 82 patients was 7.4 years (range 1-16 years). Group "A" included 59 (72%) patients, Group "B" 7 (8.5%) patients, and Group "C" 16 (19.5%) patients. Chiari malformation type II was found in 71 (86.6%) patients, 57 of whom (80%) were in Group "A" with active hydrocephalus. They were treated by VPS (51 patients) and ETV (8 patients). The shunts were revised or replaced in 10 (19.6%) patients due to obstruction or infection. Primary ETV failed in 3/8 patients, and treated by VPS. None of those in Groups "B" or "C" required treatment for hydrocephalus during the follow up.
Conclusion:
Hydrocephalus affects the majority of patients with SONTD who have Myelomeningocele (MMC) and CM II and requires close surveillance and prompt management. Children with SONTD should routinely undergo MRI examination of brain and craniocervical junction to clarify ventricular size, and the presence of CM II.
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