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Year : 2008  |  Volume : 1  |  Issue : 1  |  Page : 2 Table of Contents   

Psuedotumour cerebri syndrome in children

Department of Ophthalmology and Vision Science, The Hospital for Sick Children, Toronto, Ontario, Canada

Correspondence Address:
J Raymond Buncic
Department of Ophthalmology, University of Toronto, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-620X.43312

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How to cite this article:
Buncic J R. Psuedotumour cerebri syndrome in children. Oman J Ophthalmol 2008;1:2

How to cite this URL:
Buncic J R. Psuedotumour cerebri syndrome in children. Oman J Ophthalmol [serial online] 2008 [cited 2023 Jan 30];1:2. Available from: https://www.ojoonline.org/text.asp?2008/1/1/2/43312

The possibility of severe visual loss due to high and persistent intracranial hypertension with papilledema in the Pseudotumour Cerebri Syndrome (PTC) is outlined in the excellent paper of Al-Senawi et al. in this issue of the Oman Journal of Ophthalmology. It is now better appreciated that the rare visual loss in this unusual syndrome in children must be guarded against, as it is in adults, with early diagnosis and brisk management. Visual acuity is generally not affected till late stages in PTC, unless macular edema or hemorrhage supervene, as a relatively acute complication of the disc pathology. Arteriolar occlusions at the disc with acute symptoms occur rarely. Visual degradation due to gradual field loss may not be apparent till it becomes advanced.

Visual loss in PTC in children is rare: in a recent retrospective survey of 300 cases of papilledema due to PTC in children reported at the Canadian Ophthalmological Society in 2008 by Mallipatna et al. of the Hospital for Sick Children (HSC) in Toronto, 25 children with idiopathic intracranial hypertension, a specific variant of PTC were detailed. In this subset, only one case had visual acuity loss, and this normalized with aggressive medical treatment.

Careful assessment of the optic nerve swelling in children assumes a special importance because of the clinician's inability to rely on visual field results as a guide to visual loss, and the urgency for success of treatment of the intracranial hypertension. The latter is due to many reasons, which include the young age, the learning curve for performing reliable perimetry, and inability to carry out reliable perimetry when the older child is too ill to be a reliable witness. The characteristics of the papilledema and the health of the peripapillary retinal nerve fiber layer (RNFL) will serve as indicators of vision loss and urgency of the need for treatment or its escalation, as well as a means of following and eventually decreasing and ceasing medical therapy. The presence of axonal microinfarcts ("soft exudates") on the swollen disc surface or in the peripapillary region indicate that there has been axonal damage and perhaps death, and that the stage is set for the development of secondary optic atrophy and visual loss. The appearance of the peripapillary RNFL attenuation indicates that atrophy has begun and that some urgency is needed in the management. The use of the friendly red-free filter in examining children as well as good quality fundus photos, including red-free views as well, provide guides for management from beginning to end. Optical Coherence Tomography (OCT) is useful but not always as definitive in the practical decision making process as direct observation of the disc and its surrounding.

Aggressive medical management of the raised intracranial pressure involves a dose of a diuretic, preferably acetazolamide up to 2 grams per day depending on the patient's weight. Follow-up frequency is determined by the observable danger to vision and should be tailored to the acuteness of the situation. Sedated examination of the child may be necessary at some point to study and photograph the discs and distinguish types of pseudopapilledema from true swelling (keeping in mind that both can coexist in some cases). Differentiation must be made between apparent decrease in papilledema from true decrease in edema or an apparent decrease in edema due to atrophy of axons on the disc. In the latter case, the peripapillary RNFL attenuation can be used as a guide.

We have found that aggressive medical management with diuretics and steroids as outlined by Al-Senawi et al. will result in resolution of the problem although follow-up examinations remain important because of recurrences that occur in a few cases. Surgical intervention in severe cases or when there is no response to even maximal therapy is rarely necessary in our experience at The Hospital for Sick Children: in such cases, we have found ventriculperitoneal shunts by our neurosurgeons to be effective.


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