Pseudotumor cerebri as an important differential diagnosis of papilledema in children
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Published on: June 28, 2024
Felix Distelmaier1, Ulrike Sengler, Martina Messing-Juenger
1Department of General Pediatrics, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, D-40225 Düsseldorf, Germany. distelmaier@med.uni-duesseldorf.de
Insights
Childhood pseudotumor cerebri (PTC) requires careful management to prevent vision loss. Acetazolamide is effective, but younger children may need different treatments, including surgery if medical options fail.
Area of Science:
- Pediatric Neurology
- Ophthalmology
Background:
- Primary pseudotumor cerebri (PTC) is a rare condition in children characterized by elevated cerebrospinal fluid (CSF) pressure.
- It can lead to serious visual complications if not managed promptly.
Purpose of the Study:
- To investigate the clinical features of pediatric PTC.
- To highlight treatment options for normalizing intracranial pressure in children with PTC.
Main Methods:
- Retrospective chart review of 12 pediatric patients diagnosed with primary PTC.
- Analysis of clinical presentations, diagnostic findings, and treatment outcomes.
Main Results:
- Headache was the most common presenting symptom; four patients were asymptomatic.
- Acetazolamide was the primary treatment, with furosemide and prednisone as second-line options.
- Younger children were more difficult to treat, and one required surgical intervention (ventricular-peritoneal shunting).
Conclusions:
- Treatment aims to relieve symptoms and preserve vision.
- Acetazolamide is an effective first-line therapy for reducing intracranial pressure.
- Younger children may present unique challenges in PTC management, potentially indicating age-related etiological differences, and surgical intervention should be considered for refractory cases.
Introduction:
Primary pseudotumor cerebri (PTC) in childhood is a rare but important differential diagnosis in children presenting with papilledema. It is defined as elevated cerebrospinal fluid (CSF) pressure of more than 20 cm H(2)O, normal CSF composition, and exclusion of underlying structural or systemic causes. Visual loss is a serious complication, which requires careful monitoring and management.
Patients And Methods:
We conducted a retrospective chart review of 12 patients with primary PTC. The mean age at presentation was 8212 years, and there was a male-to-female ratio of 7:5. The aim of this study was to investigate the clinical features of primary PTC in children, and to highlight the different treatment options in normalizing intracranial pressure in these patients.
Results:
In the majority of cases, children presented with headache. Four patients had no obvious symptoms and papilledema was found on routine eye examination. Obesity was uncommon and there was no distinct sex predilection. Acetazolamide was our drug of choice for the initial treatment. Furosemide and prednisone were used as second-line agents. Treatment was gradually decreased after resolution of the papilledema with exception of the two youngest children, who remained symptomatic. One child underwent ventricular-peritoneal shunting.
Discussion:
The treatment goals of PTC are the relief of symptoms, and preservation of visual function. Acetazolamide is an effective first-line method of lowering raised intracranial pressure. In our study group especially the young children were difficult to treat. This might indicate an age-related difference in the etiology of PTC. When medical treatment remains ineffective and visual function deteriorates, surgical treatment should be considered.
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