From the Guidelines
Hydrocephalus is a common complication after meningomyelocele repair, with approximately 80% of children with spina bifida developing hydrocephalus. This is based on the most recent and highest quality study available, which found that spinal dysraphism, including meningomyelocele, is a significant cause of hydrocephalus 1. The study highlights the importance of preventive measures, such as folic acid supplementation for women of childbearing age, to reduce the incidence of spina bifida and subsequent hydrocephalus.
Key Findings
- The study found a high incidence of hydrocephalus in children with spina bifida, with approximately 80% developing hydrocephalus 1.
- Spinal dysraphism, including meningomyelocele, is a significant cause of hydrocephalus, and the provision of folic acid for all women of childbearing age is an important preventive measure 1.
- The diagnosis of hydrocephalus can be challenging, particularly in resource-limited settings, and the study highlights the need for improved diagnostic facilities and standardized classification methods 1.
Clinical Implications
- Close neurological monitoring is essential following meningomyelocele repair, with regular head circumference measurements in infants and attention to symptoms such as headache, vomiting, irritability, or developmental delays that might indicate developing hydrocephalus.
- Early recognition and prompt treatment of hydrocephalus are crucial to prevent neurological damage from increased intracranial pressure.
- Management of hydrocephalus typically involves CSF diversion procedures, most commonly ventriculoperitoneal shunting, though endoscopic third ventriculostomy may be an option in selected cases.
Limitations and Future Directions
- The study acknowledges several limitations, including the heterogeneity of the non-postinfectious hydrocephalus group and the lack of standardized classification methods 1.
- Future research should aim to improve our understanding of the causes of hydrocephalus, particularly postinfectious hydrocephalus, and to develop context-relevant and feasible clinical guidelines for the management of pediatric hydrocephalus 1.
From the Research
Hydrocephalus as a Complication of Meningomyelocele Repair
- Hydrocephalus is a common complication associated with myelomeningocele, occurring in 35% to 90% of cases 2.
- The condition is typically treated with the insertion of a ventriculoperitoneal shunt, although endoscopic third ventriculostomy with choroid plexus cauterization is emerging as an alternative treatment option 2, 3.
Treatment Options for Hydrocephalus
- A prospective cohort study found that endoscopic third ventriculostomy with choroid plexus cauterization had a similar success rate to ventriculoperitoneal shunt placement at 6 months, with lower morbidity 2.
- A retrospective cohort study comparing endoscopic third ventriculostomy with choroid plexus cauterization to ventricular shunting found that patients treated with the former remained shunt-free, while a majority of those receiving an upfront shunt required revision 3.
Management of Hydrocephalus
- The management of hydrocephalus in patients with myelomeningocele can be challenging, with cerebrospinal fluid diversion often required for the remainder of their lives 4.
- A systematic review and meta-analysis found no statistically significant differences in shunt-related complications between simultaneous and delayed ventriculoperitoneal shunt placement in neonates undergoing myelomeningocele closure 5.
Incidence and Presentation of Hydrocephalus
- Hydrocephalus can present in various ways, including as a pseudomeningocele, and should be considered as a potential cause of postsurgical complications in patients with myelomeningocele 6.
- Approximately 70%-80% of children born with myelomeningocele develop hydrocephalus and require cerebrospinal fluid diversion 5.