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PAPILLEDEMA VS. PSEUDOPAPILLEDEMAIN CHILDREN

CASE PRESENTATIONPresented by

Maha HassanOphthalmology resident, MIOR

MSc

12 years old child came to outpatient

clinic complaining about headache and

blurring of vision of 3 weeks duration.

Headache is intermittent and associated with

nausea.

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◦Examination:

OD OS

BCVA 1.0 1.0

Iop 14 16

External eye exam Normal Normal

Pupils RRR RRR

Color test 11/11 11/11

Motility Free motility Free motility

Fundus examination

Differential diagnosis:

◦Papilledema.

◦Pseudopapilledema.

◦Papillitis.

◦Optic neuropathy.

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◦Papilledema is optic nerve head swelling secondary

to elevated intracranial tension.

◦Stages:

Idiopathic intracranial hypertension:

Neurological syndrome of raised intracranial

pressure (>250mm H2o) in abscence of the triad:

◦Space occupying lesion.

◦Abnormal CSF.

◦Hydrocephalus.

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Pediatric IIH

◦ IIH mainly occurs among obese women of childbearing

age. Although its prevalence among the pediatric population is

not known, it is not uncommon among the young. In children

younger than 6 years, a specific cause of intracranial

hypertension can usually be identified. Primary or idiopathic

cases of intracranial hypertension are usually seen after age 11

years.

Papilledema versus Pseudopapilledema:

◦History.

◦ Fundus examination.

◦Autoflourescence.

◦B-scan.

◦CT Scan /MRI

◦OCT.

◦Visual field.

◦ Fluorescein angiography.

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Fundus examination :

1.Retinal haemorrhage:

◦More common in papilledema.

◦May be present in disc drusen due to vascular stress by the drusen.

Fundus examination

1.Retinal haemorrhage :

◦More common in papilledema.

◦May be present in disc drusen due to vascular stress by the drusen.

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Spontaneous venous pulsation :

◦ The presence of spontaneous venous pulsations suggests the absence of papilledema.

Visual field:

◦Papilledema : enlarged blind spot.

◦Pseudopapilledema :

◦ Early asymptomatic.

◦ 75% will develop peripheral visual field defect.

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B scan

◦Calcified drusen.

◦Hyperechoic defect with posterior shadowing.

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◦ B scan ultrasonography can be used to aid in distinguishing

between papilledema and pseudopapilledema with 80-90 %

sensitivity and specificity by measuring the external diameter of

the optic nerve sheath.

◦ In papilledema, the external diameter of the optic nerve sheath

is substantially distended (5.0-5.7 mm or greater).

◦ The “crescent sign” refers to an echolucent area in the anterior

intraorbital nerve.

◦ Lateral gaze (thirty degree test) commonly leads to a 10%

reduction in diameter on A scan measurement in the presence of

excess fluid, but not if normal or if increased ONSD is due to

infiltration.

◦ Fluid-related ONSD can also be caused by other pathology, such

as inflammation and trauma.

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◦ The nerve must be scanned axially for the measurement to be accurate, and there is a degree of operator dependance.

Autoflourescence

◦Only in superficial drusen.

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CT scan

MRI◦Evaluate the presence of intracranial mass lesions,cerebral venous sinus thrombosis and nonspecificindicators of increased intracranial pressure.

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OCTSpectral domain OCT, and more specifically the enhanced depth

imaging (EDI) technology, represents a turning point in directly

visualise drusen, to quantify their size and to recognise their impact

on neighbouring structures inside the optic nerve head.

◦ Top: internal contour of the hypo-reflective space between the

retina and the RPE in horizontal V shape in the papilloedema.

Bottom: fluted contour of said space, ripple-like margins in

pseudoedema due to buried drusen.

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Differentiation is based on differences in the morphology of the hypo-

reflective space between the neurosensory retina and the pigment

epithelium, which is present in both entities. In papilloedema, said

space is defined by an internal smooth contour that gradually narrows

down and towards the periphery describing a horizontal “V”, whereas

in the case of drusen the contour is less smooth, undulating and with a

brusque thickness reduction towards the periphery. In contrast with

papilloedema, where said space comprises liquid, OND exhibit a

nodular elevation and shade effect due to the high reflectance of

drusen that marks optically empty spaces.

Fluorescence angiography :

◦Papilledema :Hyperflourescence with leakage.

◦Pseudopapilledema : Hyperflourescence but without leakage.

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◦ CONCLUSIONS: The best imaging technique for correctly

classifying pediatric eyes as PPE or PE is FA. Other imaging

modalities, if used in isolation, are more likely to lead to

misinterpretation of PE as PPE, which could potentially result in

failure to identify a life-threatening disorder causing elevated

intracranial pressure and papilledema.

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◦Back to our Patient:

◦Male 12 years old child.

◦ Systemically free.

◦Hyperflourescence of optic nerve.

Thank you