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Shall we treat hydrocephalus associated to brain stem glioma in children?
Thomas Roujeau1, Federico Di Rocco, Christelle Dufour
1Department of Paediatric Neurosurgery, Hôpital Necker Enfants Malades, APHP, 149, rue de sèvres, 75015 Paris, France. thomas.roujeau@nck.aphp.fr
Insights
Brain stem glioma in children can cause hydrocephalus, a condition affecting 22% of patients. Ventriculoperitoneal shunts are a viable treatment option for this complication, without impacting survival rates.
Area of Science:
- Pediatric Neurosurgery
- Pediatric Oncology
- Neuro-oncology
Background:
- Brain stem glioma represents 6-9% of pediatric brain tumors.
- Tumor progression can obstruct cerebrospinal fluid (CSF) pathways, leading to hydrocephalus.
- Diffuse intrinsic pontine glioma (DIPG) is a specific type of brain stem glioma not amenable to surgery.
Purpose of the Study:
- To assess the incidence of hydrocephalus in children with diffuse intrinsic pontine glioma.
- To evaluate the management strategies for hydrocephalus in this patient population.
- To determine the impact of hydrocephalus on the overall survival of children with brain stem glioma.
Main Methods:
- Retrospective review of patient charts from a single institution (Necker Enfants Malades, Paris).
- Inclusion criteria: children treated for brain stem glioma between January 2005 and December 2010, not amenable to surgery, and excluding exophytic gliomas.
- Data collected included hydrocephalus incidence, treatment methods (ventriculoperitoneal shunt, endoscopic third ventriculostomy), and survival outcomes.
Main Results:
- Hydrocephalus occurred in 11 out of 51 children (22%) with brain stem glioma.
- The median time from diagnosis to hydrocephalus onset was 3.2 months, and from hydrocephalus onset to death was 2.8 months.
- Nine patients were treated with ventriculoperitoneal (VP) shunts, and two with endoscopic third ventriculostomy; one patient required a repeat VP shunt due to early failure.
Conclusions:
- The overall 1-year survival rate for brain stem glioma patients was 33%.
- The development of obstructive hydrocephalus did not significantly alter survival rates compared to patients without hydrocephalus.
- Hydrocephalus was not found to be directly related to terminal tumor progression, and VP shunt placement is a recommended first-line treatment option.
Purpose/Method:
Brain stem glioma accounts for 6-9% of brain tumors in children. Tumor progression may lead to CSF pathway obstruction and development of hydrocephalus. We retrospectively reviewed charts of patients consecutively treated in our institution with diffuse intrinsic pontine glioma in order to assess incidence of hydrocephalus, its management, and its impact on overall survival. All patients had brain stem glioma not amenable to surgery. Cases with exophytic brain stem glioma were excluded.
Results:
Fifty-one children were treated from January 2005 to December 2010 for brain stem glioma in the Pediatric Neurosurgery Department of Necker Enfants Malades, Paris, France. Hydrocephalus occurred in 11 of them (22%). They were six boys and five girls; the average and median time from tumor diagnosis to onset of hydrocephalus were 5.3 and 3.2 months, respectively, while average and median time from onset of hydrocephalus to death were 5.3 and 2.8 months, respectively. Hydrocephalus was treated in nine patients by a ventriculoperitoneal (VP) shunt and in two patients by an endoscopic third ventriculostomy. Because of early failure, a VP shunt was implanted in one child.
Conclusion:
The overall 1-year survival rate was 33%. Survival rate of patients with such obstructive hydrocephalus was not significantly different from patients harboring brain stem glioma who did not develop hydrocephalus. Furthermore, hydrocephalus was not related to terminal tumor progression. Considering both risks and benefit of treatment, VP shunt could be proposed, on the base of our experience, as the first option in spite of the apparently obstructive nature of the hydrocephalus associated to a brain stem tumor.
