Sensitivity of papilledema as a sign of shunt failure in children

doi:10.1016/j.jaapos.2008.08.003

Journal of American Association for Pediatric Ophthalmology and Strabismus

Volume 13, Issue 1, February 2009, Pages 63-66

https://doi.org/10.1016/j.jaapos.2008.08.003 Get rights and content

Purpose

Papilledema is considered one of the cardinal ophthalmologic signs of shunt failure. However, the prevalence of papilledema in children with shunt malfunction has not been systematically investigated. The purpose of this study is to determine the sensitivity of papilledema as a sign of shunt failure in children.

Methods

A prospective evaluation was undertaken of all children (n = 29; <19 years of age) with surgically confirmed shunt malfunction who were treated at Arkansas Children's Hospital between July 2007 and April 2008. Each child had a dilated fundus examination and/or RetCam photograph while under anesthesia immediately before shunt repair. The optic disk was evaluated for the presence of papilledema and pallor. Intracranial pressure was measured during surgical shunt repair at the discretion of the neurosurgeon.

Results

Twenty-nine patients had surgically confirmed shunt failure. Patient's age ranged from 36 days to 18 years, 8 months. Four patients (14%) had papilledema, and 1 patient had severe optic disk pallor. The remaining 24 patients had flat optic disks. Five patients had flat optic disks despite an intracranial pressure ≥300 mm H₂O.

Conclusions

Papilledema is not a sensitive sign of shunt failure. Even children with severe elevations in intracranial pressure from shunt malfunction may have flat optic disks. Therefore, physicians that evaluate children with shunts should be aware that a normal optic disk does not preclude shunt malfunction.

Section snippets

Methods

This study was approved by the Institutional Review Board at the University of Arkansas for Medical Sciences and conformed to the requirements of the United States Health Insurance Portability and Privacy Act.

All children with surgically confirmed shunt malfunction at Arkansas Children's Hospital, Little Rock, Arkansas, between July 2007 and April 2008 were prospectively analyzed. Although there was no specific upper age limit for inclusion in this study, patients at Arkansas Children's

Results

There were a total of 35 patients with surgically confirmed shunt failure. Two patients were excluded from analysis because of the presence of an intracranial tumor (1 patient) and microphthalmos (1 patient). Of the remaining 33 patients, 4 patients did not receive an eye examination because a study physician was not available at the time of surgical shunt repair.

Data from the remaining 29 patients was then compiled for analysis. The patients ranged in age from 36 days to 18 years. There were

Discussion

Our study shows that papilledema is not a sensitive sign of shunt failure. Even children with elevated intracranial pressure from shunt malfunction often have flat optic disks. We are not suggesting that papilledema is not an important finding in patients with shunts. Indeed, several investigators have described patients in whom papilledema was the only sign of shunt failure.3, 4 These studies show that children with shunts and papilledema should be considered to have shunt malfunction, even in

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Influence of sex and disease etiology on the development of papilledema and optic nerve sheath extension in the setting of intracranial pressure elevation in children
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Dilatation of the optic nerve sheath diameter and swelling of the optic disc are known phenomena associated with intracranial pressure elevation.
Do sex and disease etiology have an impact on the development of optic disc elevation and optic nerve sheath extension in children in the setting of ICP elevation? Fundoscopic papilledema and point-of-care-ultrasound techniques-optic nerve sheath diameter (US-ONSD) and optic disc elevation (US-ODE) - were compared in this regard.
72 children were included in this prospective study; 50 with proven pathology (e.g. pseudotumor cerebri, tumor), 22 with pathology excluded. Bilateral US-ONSD and US-ODE were quantified by US using a 12-MHz-linear-array-transducer. This was compared with fundoscopic optic disc findings and in 28 patients with invasive ICP values, stratified for sex and etiology.
In patients with proven disease, significant more girls (69%) had fundoscopic papilledema compared with boys (37%, p < 0.05). Girls had also larger US-ODE values (0.86 ± 0.36 mm vs. 0.65 ± 0.40 mm in boys). 80% of tumor patients had initial papilledema (100% girls, 79% boys), compared with 50% in pseudotumor cerebri (PTC) (83% girls, 30% boys). US-ONSD had no sex- and no etiology-specificity.
Presence of papilledema appears to be influenced by sex and etiology, whereas US-ONSD is not. Girls seem more likely to develop papilledema under similar conditions. Male sex and PTC appear as risk factors for being undetected by fundoscopic findings. US-ONSD and US-ODE seem useful tools to identify pathologies with potentially increased ICP requiring treatment in children regardless of sex and etiology.
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Citation Excerpt :
The demonstrated lack of correlation between opening pressure on lumbar puncture and ICP monitoring emphasizes the need for direct ICP monitoring in patients suspected to have elevated ICP despite normal lumbar punctures.16 Because papilledema takes time to develop, it is a relatively insensitive sign of acute shunt malfunction.12 In our case the clinical symptomatology and MRI finding of flattening of the posterior sclera providing clues to the underlying diagnosis of elevated ICP due to shunt malfunction.2
A 14-year-old boy with a history of shunted congenital hydrocephalus began having headaches with nausea and vomiting after transcontinental flights. He gradually developed horizontal diplopia indicative of mild bilateral sixth nerve palsy, without papilledema or ventriculomegaly. Intracranial pressure monitoring showed no signs of elevation. After he subsequently developed papilledema, surgical exploration showed shunt malfunction, and shunt replacement produced rapid resolution of symptoms. This case demonstrates the importance of relying on clinical history and neuro-ophthalmologic examination in patients with hydrocephalus and suspected shunt failure, even when objective confirmatory evidence of intracranial pressure elevation is lacking.
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A study in humans examining acute rises in ICP after spontaneous hemorrhage or craniocerebral trauma showed that patients with mild increases in ICP (20-30 mm Hg) did not develop optic disc edema and that the development of papilledema was rare even in patients with significant ICP elevations (30-70 mm Hg) for at least 3 consecutive days.35 Even in children with surgically confirmed shunt failure and severe elevations in ICP, only 14% had developed papilledema in one study, again underscoring its inadequacy as a sign of shunt failure.29 Resolution of papilledema after normalization of ICP may also take weeks to months.26
Cerebrospinal fluid (CSF) diversion or shunting procedures are the most commonly performed surgery for the treatment of hydrocephalus and are often employed in the management of elevated intracranial pressure due to a variety of diseases. Despite their popularity however, approximately 50% of shunts fail within the first two years, and several revisions are required within the first decade after placement. Ophthalmologists may encounter patients with a CSF shunt to evaluate for concerns of vision loss or diplopia and to determine if papilledema is present. We discuss the neuro-ophthalmic manifestations and evaluation of possible CSF shunt malfunction.
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Completion of PVDO results in an improvement or resolution of elevated ICP.39,59 Outcomes used to determine resolution (eg, fundoscopy), however, have a low sensitivity (11%–40%) compared with the gold standard direct ICP measurements.60–62 Optical coherence tomography was recently validated for detecting elevated ICP in patients with CS, with greater specificity (89%).63
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Transorbital point-of-care ultrasound versus fundoscopic papilledema to support treatment indication for potentially elevated intracranial pressure in children
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: Supported in part by an unrestricted grant from Research to Prevent Blindness (New York, NY) and the Pat & Willard Walker Eye Research Center, Jones Eye Institute, University of Arkansas for Medical Sciences (Little Rock, AR).

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Major articleSensitivity of papilledema as a sign of shunt failure in children

Purpose

Methods

Results

Conclusions

Section snippets

Methods

Results

Discussion

Surv Ophthalmol

Am J Ophthalmol

Ophthalmology

Mechanical complications in shunts

Paediatr Neurosurg

Permanent visual loss after shunt malfunction

Neurology

Shunt failure without ventriculomegaly proclaimed by ophthalmic findings

J Neurosurg

The diagnosis of blocked cerebrospinal fluid shunts: A prospective study of referral to a paediatric neurosurgical unit

Child's Nerv Syst

Major article
Sensitivity of papilledema as a sign of shunt failure in children