Keywords
papilledema - optic nerve edema - ophthalmology consultations - screening
Papilledema refers to optic disc edema due to elevated intracranial pressure (ICP) and was first described in 1853.[1] The proposed pathogenesis is that elevated ICP causes elevated pressure on the optic nerve, which decreases axoplasmic flow, leading to congestion and edema in the optic nerve head. It is thought that this typically takes 1 to 5 days to develop but depends on the rate of elevation in pressure.[2] Early papilledema can present without any symptoms, although it may be associated with symptoms common of increased ICP including headaches which are worse in the morning, nausea and vomiting, pulsatile tinnitus, transient visual obscuration, and/or sixth nerve palsy. Long-standing papilledema can result in visual field defects and loss of vision. The possibility for serious underlying conditions as a cause of papilledema such as central nervous system (CNS) tumors or CNS inflammation makes it an ominous finding on fundus examination. When patients are suspected to have elevated ICP, ophthalmologists are often consulted to examine patients to identify papilledema which can help rule-in that possibility.
Ophthalmology consultation is common in both the emergency room and inpatient setting.[3] One frequent reason for consultation is to rule out papilledema in cases of headache, head trauma, suspected intracranial mass, or prior to lumbar puncture and has been estimated to account for 3.3% of ophthalmology consults.[4]
Nonetheless, the actual diagnosis of papilledema in such consults is rare. In a study of consultations to rule out papilledema in a pediatric emergency room, papilledema was diagnosed in only 1.2 to 6% of all consultations.[5]
[6] The only clinical factor significantly associated with a positive diagnosis of papilledema was visual complaints associated with headache.
Additionally, the appropriate timing of such consultations has not been well established. It is known that papilledema is typically not present in cases of acute rise in ICP (< 7 days), and its absence does not eliminate the possibility of elevated ICP.[7] In 479 emergency room consults to rule out papilledema, Segev-Becker et al found that 60% of the patients had been symptomatic for < 24 hours, of which all had normal eye exams.[5]
To date there have been no studies evaluating the clinical factors predictive of making the diagnosis of papilledema in a population of children and adults. The purpose of this study is to evaluate the incidence of ophthalmology consultations to screen for papilledema in an acute inpatient and emergency room setting and determine the frequency and factors associated with a positive diagnosis of bilateral optic nerve swelling.
Methods
This retrospective case series was approved by the Northwell Health Institutional Review Board. Medical records were reviewed for all consecutive patients who had a hospital-based ophthalmology consult requested for suspected optic disc swelling between September 1, 2016 and December 31, 2016. Consults conducted in the emergency room and inpatient units at North Shore University Hospital, Long Island Jewish Medical Center, and Cohen Children's Medical Center for both adults and children were included in this study. All patients underwent a complete ophthalmologic exam. Examinations included assessment of visual acuity, color vision, pupils, confrontation visual fields, intraocular pressure, extraocular motility, anterior segment, and dilated fundus. Demographics, reason for consult, requesting service, medical and ocular history, associated symptoms, and final diagnosis were recorded.
Evaluation of the optic nerve and diagnosis of optic disc swelling was made based on either direct ophthalmoscopy, indirect ophthalmoscopy, and/or slit lamp biomicroscopy at the patient's bedside or in the hospital eye examination room if the patient's medical and physical condition allowed. The examination was performed by the ophthalmology residents on the consult service and all diagnoses were confirmed by an attending comprehensive ophthalmology hospitalist.
Results
A total of 36 consults—13 males and 23 females—with a mean age of 19.8 (range: 3–71) years were called to screen for papilledema. The most common service to request the consult was pediatrics (44%), followed by neurosurgery (42%), medicine (8%), and neurology (6%). The most frequent reason for consultation was headache (61%), followed by visual changes (30.5%) and nausea/vomiting (25%) ([Table 1]).
Table 1
Demographics and characteristics of consults
|
Number
|
|
Total
|
36
|
|
Average age
|
19.8 y (range: 3–71)
|
|
Gender
|
13/36 (36%) male
|
|
Consult requested by:
|
|
|
Pediatrics
|
16 (44%)
|
|
Medicine
|
3 (8%)
|
|
Neurosurgery
|
15 (42%)
|
|
Neurology
|
2 (6%)
|
|
Presenting symptoms:
|
|
|
Visual changes
|
11 (30.5%)
|
|
Headache
|
22 (61.1%)
|
|
Nausea or vomiting
|
9 (25%)
|
|
Bilateral optic nerve swelling
|
5 (14%)
|
|
Referred by outside ophthalmology
|
4 (11%)
|
A positive diagnosis of bilateral optic nerve swelling occurred in 14% (5/36) of consults ([Table 2]). Of these five consults, four of them were sent into the hospital by an outpatient ophthalmologist who noted the bilateral optic nerve swelling and one was noted by an emergency room pediatrician. Two out of the five patients with papilledema (40%) presented with changes in vision, two (40%) with headache, one (20%) had nausea and vomiting, one (20%) had pulsatile tinnitus, and one patient was asymptomatic.
Table 2
Characteristics of patients with bilateral optic nerve swelling
|
Case
|
Age (y), Sex
|
Symptoms
|
Ophthalmology referred
|
Visual acuity
|
Color (Ishihara)
|
APD
|
Final diagnosis
|
|
1
|
13, F
|
Dilated pupil, restricted extraocular motility
|
No
|
20/20 OU
|
12/12 OU
|
No
|
Pineal germinoma
|
|
2
|
13, F
|
Headache, nausea, vomiting
|
Yes
|
20/20 OU
|
12/12 OU
|
No
|
Viral meningitis
|
|
3
|
28, F
|
Headache, blurry vision
|
Yes
|
20/400 OD, 20/20 OS
|
N/A[a]
|
N/A[a]
|
Atypical meningioma
|
|
4
|
10, M
|
Craniosynostosis screening exam
|
Yes
|
20/30 OU
|
N/A[a]
|
No
|
Elevated intracranial pressure
|
|
5
|
25, F
|
Blurry vision, tinnitus
|
Yes
|
20/20 OU
|
12/12 OU
|
No
|
Idiopathic intracranial hypertension
|
Abbreviations: APD, afferent pupillary defect; F, female; M, male.
a Not able to be performed.
All of the patients with papilledema except one had corrected near-visual acuity of 20/30 or better and none had an afferent papillary defect. Color vision was full in three of the patients, not available in two patients.
The final diagnoses in the patients with papilledema included two with intracranial masses (one pineal germinoma and one atypical meningioma), one with viral meningitis, one with idiopathic intracranial hypertension, and one case of elevated ICP secondary to craniosynostosis.
Discussion and Conclusion
Discussion and Conclusion
Ophthalmologists are commonly consulted when there is a concern for elevated ICP to “rule out papilledema,” which is potentially vision threatening and more importantly carries the possibility of a life-threatening etiology. Papilledema can develop anywhere from hours to weeks after a rise in ICP.[8] In our study, 36 consults were called during a 4-month period. The consulting ophthalmologist made no new diagnoses of papilledema, but confirmed five cases that were previously known to the consulting service.
A common cause of elevated ICP in children is ventriculoperitoneal (VP) shunt failure in patients with a history of hydrocephalus. The overall prevalence of papilledema in cases of VP shunt failure was found to be 14%, and 24% in those with documented ICP > 200 mm H2O.[9] Another study found the sensitivity of papilledema in predicting elevated ICP in cases of VP shunt failure to be 23%.[5] While there have been isolated case reports of VP shunt failure where papilledema was the only presenting sign, it must be noted that this is the exception to the rule.[10]
[11] It has also been shown that younger children are less likely to present with papilledema in the setting of elevated ICP.[12]
One limitation of our study was that the duration of symptoms was inconsistently reported and unable to be evaluated. Numerous studies have documented that ICP must be elevated for a sufficient duration to be transmitted to the optic nerve and manifest as papilledema.[7]
[13]
[14]
[15] Steffen et al studied 37 patients with acute elevations in ICP who had daily fundus examinations for 7 days after the acute elevation and found that only one patient developed papilledema within 7 days.[7] The absence of papilledema in the acute setting of raised ICP has also been demonstrated in patients with acute intracranial hemorrhage.[15] Thus, evaluation for papilledema may be unreliable in the setting of acute onset of symptoms or sudden rise in ICP.
In our study two out of five patients with papilledema were ultimately diagnosed with a CNS mass. Fear of missing an intracranial tumor is the driving force for many consults; however, it appears that papilledema at time of CNS tumor diagnosis is relatively uncommon, being found in 13% of patients in a meta-analysis of 4,000 pediatric tumors.[16] Furthermore, a large retrospective review found that the vast majority (96.5% of 479 patients) of pediatric patients with headaches had normal ophthalmological exams with no evidence of optic disc swelling.[5]
The most common presenting symptom in patients suspected of having papilledema in our study was headache (61%). Yet, only two of the five patients with papilledema reported headaches. In children evaluated for headache the most common causes are usually extracranial, including upper respiratory infections, sinusitis, migraine, or other infections. Headaches in the absence of other neurologic symptoms are rarely related to serious intracranial causes.[17]
[18]
Not to be excluded in the assessment of the utility of ophthalmology consults is the cost—both the financial cost as well as the allocation of limited resources—in addition to the time it takes to get and complete an ophthalmology consult. Furthermore, an eye exam can be difficult and upsetting to a child. In addition, not all hospitals have ophthalmologists on staff, which could require a costly and time-consuming transfer to another institution. With the rise of telemedicine, recent studies have assessed the quality of optic nerve photography for diagnostics purposes. In a recent study of 103 children aged 5 to 12 years old, a nonmydriatic digital fundus camera was used to produce clinically satisfactory images with an average reviewer rating 4.6 out of 5 (1 = unsatisfactory and 5 = satisfactory). Additionally, the average length of time to obtain the image was 1.8 minutes and the average patient cooperation was rated as 4.4 out of 5 (1 = uncooperative, no images obtained and 5 = very cooperative).[19] Cost, patient satisfaction, and length of stay could all potentially be improved by utilization of such devices.
In conclusion, no new cases of bilateral optic nerve swelling were found in screening consults to “rule out papilledema” in our study. All of the positive diagnoses in our study had been previously identified by another physician and known to the primary team prior to ophthalmology consultation. There were no significant associations of subjective complaints or objective findings in patients with optic nerve swelling. A review of the literature suggests that dilated fundus examinations may not be a good screening tool to “rule out” elevated ICP, especially in the acute setting. A negative examination for optic nerve swelling does not definitively exclude elevated ICP. Further larger studies must assess the cost effectiveness and utility of screening inpatient eye exams for papilledema.
