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[Paediatric pseudotumour cerebri]
Y M Betancourt-Fursow de Jiménez1, C S Jiménez-Betancourt, J C Jiménez-Leon
1Instituto Neurológico, Universidad de Carabobo, Valencia, Venezuela. neuroval@telcel.net.ve
Insights
Paediatric pseudotumour cerebri, or idiopathic intracranial hypertension, is a rare but serious condition in children causing headaches and vision loss. Treatment requires a personalized, multidisciplinary approach to prevent permanent visual impairment.
Area of Science:
- Neurology
- Ophthalmology
- Pediatrics
Context:
- Idiopathic intracranial hypertension (IIH) in children, also known as pseudotumour cerebri, presents differently than in adults.
- This condition is not benign and requires careful management to prevent long-term consequences.
Purpose:
- To review the clinical characteristics and treatment outcomes of pediatric patients diagnosed with pseudotumour cerebri.
- To highlight the importance of individualized and multidisciplinary treatment strategies.
Summary:
- A 10-year review identified 23 pediatric patients (ages 3-15) diagnosed with pseudotumour cerebri.
- Treatment approaches included acetazolamide, diet, steroids, optic nerve sheath fenestration, and lumboperitoneal shunts for refractory cases.
Impact:
- Emphasizes the need for tailored, ongoing care to avoid permanent visual damage in children with IIH.
- Highlights surgical interventions for severe cases, indicating the complexity of managing this condition.
Introduction:
Paediatric pseudotumour cerebri or idiopathic intracranial hypertension syndrome is rare in the paediatric age and presents with clinical features that differ from the clinical picture seen in adults. It is not a benign condition in children and, although sometimes used to identify this syndrome, the term 'benign intracranial hypertension' must therefore be avoided. It is characterised by an increase in the intracranial pressure with analytically normal cerebrospinal fluid and the absence of expansive injuries detected by means of neuroimaging.
Development:
It is a complex syndrome that causes intense headaches in children with acute loss of vision and, on occasions, optic atrophy. Its treatment has to be tailored to each patient and the use of different pharmacological or neurosurgical treatments must be considered to avoid permanent visual damage. We reviewed the case mix at our Institute over the last 10 years (1995-2005) and found 23 children (between 3 and 15 years of age) who fulfilled Dandy criteria confirming a diagnosis as suffering from paediatric pseudotumour cerebri, with no predominance of sex or associated obesity.
Conclusions:
Its treatment must be established on an individualised basis, as well as ongoing and multidisciplinary; fenestration of the optic nerve sheath or the implantation of lumboperitoneal shunts correct refractory syndromes, which in our case accounted for 35% of the total number. The rest were treated with acetazolamide, diet and steroids.
