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Pseudotumor cerebri in children
P H Phillips1, M X Repka, S R Lambert
1Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287-9009, USA.
Insights
Pseudotumor cerebri in children can lead to permanent vision loss, with optic nerve atrophy affecting one-third of patients. Early detection and monitoring are crucial for managing this condition.
Area of Science:
- Pediatric Neurology
- Ophthalmology
- Neuroimaging
Background:
- Pseudotumor cerebri (PTC), or idiopathic intracranial hypertension, requires updated demographic and outcome data in the era of advanced neuroimaging.
- Understanding PTC in pediatric populations is essential for effective diagnosis and management.
Purpose of the Study:
- To describe the demographic characteristics and outcomes of children diagnosed with pseudotumor cerebri.
- To analyze associated etiologic factors and long-term visual prognosis in pediatric PTC cases.
Main Methods:
- Retrospective review of medical records for children under 18 diagnosed with PTC between 1977 and 1997.
- Defined PTC by increased intracranial pressure, normal/small ventricles, and normal cerebrospinal fluid composition.
- Excluded cases later diagnosed with central nervous system malignancy.
Main Results:
- 35 pediatric patients with PTC were analyzed (10 idiopathic, 25 associated with other disorders).
- Mean age was 10.6 years; 57% were female, 37% obese.
- Visual deficits (visual acuity <20/40, visual field deficits, cranial nerve deficits) were present at diagnosis, with most cranial nerve deficits resolving.
- Optic nerve atrophy occurred in 33% of patients; residual visual field deficits were noted in some at follow-up.
Conclusions:
- Associated etiologic factors are common in pediatric PTC; gender predominance was not observed.
- Permanent visual loss can occur, highlighting the importance of sensitive visual assessment methods.
- Quantitative perimetry and optic nerve examination are more effective than visual acuity for detecting visual system damage.
- Rare cases of PTC may mask intracranial neoplasms, necessitating extended follow-up.
Purpose:
Demographic and outcome data in the era of modern neuroimaging are needed to describe pseudotumor cerebri in children.
Methods:
We reviewed the medical records of children less than 18 years old who were diagnosed with pseudotumor cerebri between 1977 and 1997. We defined pseudotumor cerebri as (1) increased intracranial pressure, (2) normal or small ventricles, and (3) normal cerebrospinal fluid composition. The condition might be idiopathic or the result of a nontumor etiology.
Results:
Thirty-seven patients had an initial diagnosis of pseudotumor cerebri. Two patients were subsequently diagnosed with a central nervous system malignancy and were excluded from further analysis. The remaining 35 patients included 10 patients with idiopathic pseudotumor cerebri and 25 patients with disorders reported to be associated with pseudotumor cerebri. The mean age was 10.6 years with a range of 3 to 17 years. Twenty patients (57%) were female and 13 patients (37%) were obese. At presentation 4 patients had a visual acuity less than 20/40 in the best eye and 10 patients had visual field deficits. Seventeen patients (49%) had cranial nerve deficits, all of which resolved with normalization of the intracranial pressure. Follow-up data were obtained on 30 patients. Only one patient had a final visual acuity less than 20/40 in the best eye, whereas six patients had residual visual field deficits. Ten patients (33%) had optic nerve atrophy.
Conclusions:
There was no gender predominance, and associated etiologic factors were common in these children with pseudotumor cerebri. Permanent visual loss occurs in some children with pseudotumor cerebri. Quantitative perimetry and optic nerve examination were more sensitive than visual acuity determination in detecting damage to the visual sensory system. In rare instances the patient diagnosed with pseudotumor cerebri will be found after extended follow-up to harbor an intracranial neoplasm.