Downloaded from http://bjo.bmj.com/ on April 14, 2016 - Published by group.bmj.com Clinical science Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension Sidney M Gospe III,1 M Tariq Bhatti,1,2 Mays A El-Dairi1

1Department of ABSTRACT Monitoring of severity and early Ophthalmology, Duke Background There is a paucity of literature describing detection of optic atrophy in patients with IIH University Medical Center, Durham, North Carolina USA risk factors for vision loss in paediatric idiopathic have been greatly aided by advances in spectral 2Department of Neurology, intracranial hypertension (IIH). We investigate the final domain optical coherence tomography (SD-OCT). Duke University Medical visual function, spectral domain optical coherence This non-invasive imaging modality is now com- Center, Durham, North tomography (SD-OCT) and enhanced depth imaging monly used to measure the thickness of the peripa- Carolina, USA (EDI)-OCT findings in children with papilledema caused pillary RNFL in IIH, and other optic 5–7 Correspondence to by IIH. neuropathies. Moreover, the high resolution of Dr Mays A El-Dairi, Methods Medical records of 31 patients with SD-OCT has allowed pathology of specific layers of Department of Ophthalmology, paediatric IIH (age ≤17 years) were retrospectively the to be analysed qualitatively and quantita- Duke Eye Center, Duke reviewed. photographs on presentation and tively, and we have recently described examples of university, P.O. Box 3802, Durham, NC 27710, USA; automated perimetry, SD-OCT and EDI-OCT imaging on focal atrophy of retinal layers in the macula in [email protected] final follow-up visit were statistically analysed to identify adult patients with IIH that is distinct from the patient characteristics and anatomic findings associated already-well-described RGC and RNFL changes.8 Received 17 April 2015 with irreversible vision loss. Though less common than in adults, IIH may Revised 13 July 2015 Results Permanent or visual field loss affect the paediatric population.9 The longstanding Accepted 29 July 2015 Published Online First developed in 19% of study eyes. Papilledema of belief that paediatric patients are less prone to poor 12 August 2015 modified Frisén grade ≥3 on presentation was highly visual outcomes has been challenged in recent – predictive of permanent vision loss (p<0.001), while years.10 12 The demographics of paediatric IIH are associations between pubertal status and visual function dictated by patients’ pubertal status: pubertal outcome failed to reach statistical significance. SD-OCT patients tend to be obese females (similar to the revealed optic atrophy in 13% and photoreceptor loss in adult population), while pre-pubertal children show 19% of eyes, with both findings highly associated with no tendency towards a particular sex or body vision loss (p<0.0001). Optic disc drusen was noted in habitus.13 14 48% of study eyes by EDI-OCT but was not found to be In this retrospective longitudinal study, we aimed predictive of visual outcome. to address the relative paucity of information Conclusions Clinical observation of high papilledema regarding visual outcomes in paediatric IIH and to grade on presentation is predictive of poor visual examine potential predictors of visual morbidity. outcomes. Vision loss is associated not only with optic We applied SD-OCT and enhanced depth imaging atrophy but also with photoreceptor damage. (EDI)-OCT to assess anatomic abnormalities of the Interestingly, a high proportion of study eyes had optic retina and that may correlate with disc , which was not associated with vision loss, visual disability following treatment of IIH. but can be a diagnostic challenge in distinguishing true papilledema from pseudopapilledema. METHODS All patients with IIH age 17 and under, presenting to the paediatric neuro-ophthalmology clinic between 1 January 2010 and 31 August 2013, were INTRODUCTION included. Patients were required to meet the Patients with idiopathic intracranial hypertension revised diagnostic criteria for IIH proposed by (IIH) often present with headache and visual Friedman, Liu and Digre: (1) observation of papil- symptoms due to elevated intracranial pressure ledema or , (2) otherwise normal (ICP) not caused by an intracranial mass, venous neurological examination, (3) -enhanced sinus thrombosis or central nervous system inflam- neuroimaging excluding secondary aetiologies of mation.1 A hallmark finding of IIH is papilledema. elevated ICP, (4) normal CSF composition and (5) When profound or chronic, papilledema may lead lumbar puncture opening pressure exceeding 28 cm 1 to irreversible visual field defects, decreased visual H2O (or 25 cm H2O if not sedated). Inclusion acuity and the development of optic atrophy from additionally required optic disc photography per- death of retinal ganglion cells (RGCs) and their formed on presentation and Spectralis (Heidelberg, axons that comprise the retinal nerve fibre layer Carlsbad, California, USA) SD-OCT imaging of the (RNFL).2 To alleviate symptoms and prevent per- macula obtained following treatment. manent visual disability, IIH is typically treated Data collected from the initial patient visit with dietary interventions combined with pharma- included age, gender, pubertal status (as determined fl To cite: Gospe SM, cotherapy to reduce cerebrospinal uid (CSF) pro- by menarche in females and growth of facial hair in Bhatti MT, El-Dairi MA. Br J duction3 or surgical intervention via optic nerve boys), serum haemoglobin (if available), visual Ophthalmol 2016;100: sheath fenestration (ONSF) or CSF diversion acuity and optic disc photos. Type of therapy admi- – 505 509. procedures.4 nistered (eg, diuretics, ONSF, CSF diversion

Gospe SM, et al. Br J Ophthalmol 2016;100:505–509. doi:10.1136/bjophthalmol-2015-307043 505 Downloaded from http://bjo.bmj.com/ on April 14, 2016 - Published by group.bmj.com Clinical science

procedures) and assessments of visual acuity and visual field (using the automated Humphrey Field Analyzer/HFA II-i; Carl Zeiss Meditec, Dublin, California, USA) were documented. Using optic disc photographs, the severity of papilledema on presentation for both eyes of each patient was graded by the modified Frisén scale15 by a masked reader (MAE-D), and the eye with more severe disc swelling (or the right eye if the papil- ledema was symmetric) was selected for further analysis. Humphrey visual fields were categorised as normal or demon- strating an enlarged blindspot, single or double arcuate , central scotoma or paracentral scotoma; the latter two classifica- tions required depressions within 10° of fixation, with a foveal threshold <30 dB being denoted a central scotoma and a foveal Figure 1 Visual acuity pre-treatment and post-treatment. Scatterplot threshold of ≥30 dB denoted a paracentral scotoma. Finally, raw of visual acuity measurements by the logarithm of the minimum angle of Spectralis SD-OCT and (when available) EDI-OCT data were resolution (LogMAR) in the study eye for each subject. The visual acuity inspected in a masked fashion for each patient, and the presence by Snellen equivalent is marked on the right axis. Pre-pubertal subjects of retinal or optic nerve pathology was documented. are displayed on the left and pubertal subjects on the right. Visual acuity Data analysis was performed through the construction of con- on presentation is plotted as blue diamonds, and visual acuity at most 2 recent follow-up visit is plotted as red squares. Patients undergoing optic tingency tables and χ testing in order to test the predictive nerve sheath fenestration in the study eye are marked with orange value for adverse visual outcomes of various patient character- arrows, and those undergoing cerebrospinal fluid diversion procedures fi istics on presentation. Poor visual outcomes were classi ed as are marked with green arrows. HM, hand-motion; LP, light perception. permanent central vision loss (visual acuity of 20/40 or worse or a central/paracentral scotoma on visual field testing) or per- manent peripheral vision loss (single or double arcuate scotoma with normal visual acuity maintained normal acuity, while all on visual field testing). Correlation between anatomic abnormal- eight presenting with abnormal visual acuity demonstrated some ities on final SD-OCT imaging and adverse visual outcomes degree of improvement over the course of treatment (figure 1). were similarly tested. Ultimately, 28 study eyes had normal visual acuity, 2 had moder- ately diminished visual acuity (one pubertal, one pre-pubertal) RESULTS and 1 had profound loss of visual acuity (pubertal). Automated Thirty-one patients met criteria for inclusion in the study perimetry data were available for 23 patients (11 of 12 pubertal (table 1), with an average follow-up time of 30.7±16.2 months and 12 of 19 pre-pubertal). Five patients (four pubertal, one (mean±SD). There were 12 pubertal (all female) and 19 pre- pre-pubertal) demonstrated permanent peripheral visual field pubertal (12 male, 7 female) children. The mean age on presen- loss in the study eye, and four patients (three pubertal, one pre- tation was 15.4±1.4 years for pubertal children and 7.8 pubertal) demonstrated permanent central or paracentral scot- ±3.4 years for pre-pubertal children. The mean grade of papil- omas. A total of seven patients had at least one form of perman- ledema on presentation was 2.6±1.4 in the study eyes overall, ent visual field loss in the study eye. with no significant difference between pubertal and pre-pubertal Final SD-OCT images of the optic nerve head and macula group (p=0.26). On presentation, 23 patients had normal were reviewed for the study eye of each patient. RNFL OCT visual acuity (20/20–20/30), 5 had moderately diminished visual demonstrated a final average RNFL thickness of 106.9 acuity (20/40–20/80) and 3 had profound visual acuity loss (20/ ±29.1 mm. Four eyes (all pubertal) demonstrated optic atrophy, 100—light perception) in the study eyes. defined as average RNFL thickness <80 mm. Only two of these Twenty-four patients were successfully managed with acetazo- patients underwent RNFL OCT during the early phase of their lamide alone, while seven required surgical intervention: three disease and were observed to develop optic atrophy at 2 and underwent unilateral ONSF (all in the study eyes) and four 4 months, respectively, after initial presentation. Buried optic underwent CSF diversion procedures. All study eyes presenting disc drusen were noted on EDI-OCT of the optic nerve head in

Table 1 Patient characteristics All subjects Pre-pubertal Pubertal p Value

N (male/female) 31 (12M/19F) 19 (12M/7F) 12 (0M/12F) Age (years, mean±SD) 7.8±3.4 15.4±1.4 Follow-up (months) 30.7±16.1 34.6±16.0 24.4±15.0 0.087 Papilledema grade 2.6±1.4 2.4±1.0 3.0±1.8 0.26 Treatment Acetazolamide 24 (77%) 14 (73%) 10 (83%) Optic nerve sheath fenestration 3 (10%) 2 (11%) 1 (8%) Cerebrospinal fluid Diversion 4 (13%) 3 (16%) 1 (8%) Visual acuity on presentation Normal (20/20–20/30) 23 (74%) 15 (79%) 8 (67%) Moderately diminished (20/40–20/80) 5 (16%) 4 (21%) 1 (8%) Profoundly diminished (20/100 or worse) 3 (10%) 0 (0%) 3 (25%)

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13 of the 27 eyes in which this was performed (figure 2A). in one eye (figure 2E,F). SD-OCT on initial presentation was B-scan ultrasonography had been performed on five of these available for four of these eyes, and all four demonstrated evi- eyes and confirmed the presence of drusen (figure 2B). Review dence of photoreceptor damage during the acute phase of of optic disc photos from presentation and after treatment papilledema. revealed that cases of optic disc drusen did not represent mis- Patient characteristics on presentation were analysed to deter- diagnosis of IIH due to pseudopapilledema as the disc swelling mine which factors may be predictive of poor visual outcomes improved following treatment of elevated ICP (figure 2C,D). (table 2). Grade of papilledema was classified as mild (grade 0–2) Photoreceptor ellipsoid zone abnormalities were seen on or moderate-to-severe (grade 3–5). Eyes that ultimately developed macular SD-OCT in six eyes (19%), including frank focal loss permanent peripheral , central/paracentral scotomas or of the ellipsoid zone in five eyes and ellipsoid zone irregularity any visual loss were invariably those with moderate-to-severe

Figure 2 Anatomic findings on optical coherence tomography (OCT). (A) Enhanced depth imaging (EDI) of an optic nerve head following treatment and resolution of papilledema, revealing the presence of a buried druse (arrow). (B) B-scan ultrasonography confirming the presence of a hyper-reflective optic disc druse (arrow). (C and D) Optic disc photography on presentation (C) and after treatment (D) of a patient with evidence of buried optic disc drusen by EDI-OCT, demonstrating improvement in optic disc swelling over time. OCT of the peripapillary retinal nerve fibre layer at each time point (insets) confirms reduced swelling over time. (E and F) Macular SD-OCT imaging of a patient with focal loss of photoreceptor ellipsoid zone (E) and a patient with focal irregularity of ellipsoid zone (F), both marked with arrows.

Gospe SM, et al. Br J Ophthalmol 2016;100:505–509. doi:10.1136/bjophthalmol-2015-307043 507 Downloaded from http://bjo.bmj.com/ on April 14, 2016 - Published by group.bmj.com Clinical science

Table 2 Contingency analysis of risk factors for poor visual outcomes Adverse outcome (OR (p value))

Risk factor Any vision loss Peripheral field loss Central/paracentral scotoma Optic atrophy Photoreceptor loss

Pubertal 4.3 (0.12) 7.0 (0.071) 6.0 (0.11) Infinite (0.003) 12.9 (0.012) Papilledema grade ≥3 Infinite (0.0008) Infinite (0.005) Infinite (0.008) Infinite (0.008) Infinite (0.0008) Optic disc drusen NS (0.94) NS (0.54) NS (0.96) NS (0.58) NS (0.30) Infinite OR due to 100% or 0% occurrence in one subpopulation. NS, not significant.

papilledema (all p≤0.008). Moderate-to-severe papilledema was vision loss, whereas 50% of eyes with grade 3 or worse papille- also highly predictive of optic atrophy (p=0.008) and photorecep- dema suffered permanent vision loss. While patients with paedi- tor damage (ellipsoid zone irregularity/loss; p=0.0008) on final atric IIH are known to be at risk for permanent visual SD-OCT. The study eyes of pubertal patients were significantly disability,9 ours is the first study to demonstrate more likely to demonstrate optic atrophy (p=0.003) or photo- moderate-to-severe papilledema on presentation as a risk factor receptor damage (p=0.012); however, differences in peripheral for this feared outcome in the paediatric population. field loss, paracentral/central scotomas and any vision loss by One unexpected finding was the high rate of buried optic disc pubertal status did not reach statistical significance. Severe anaemia drusen identified by EDI-OCT. While the prevalence of optic (serum haemoglobin <10 g/100 mL) on presentation was only disc drusen in the general population has been estimated at present in 1 of 25 patients for which this value was available; this 2.0% in autopsy studies,16 we uncovered evidence of buried patient developed photoreceptor loss and a paracentral scotoma in drusen in 48% of study eyes. Optic disc drusen and papilledema the study eye. The presence of optic disc drusen was found to have have been reported to coexist in patients with paediatric IIH,17 no correlation with final visual outcomes, optic atrophy or photo- and it is interesting to speculate that optic disc drusen may play receptor loss. a role in the development of papilledema in IIH. Drusen may The clinical significance of anatomic abnormalities on final form as a result of axoplasmic stasis due to small foraminal OCT imaging was assessed through correlation to final visual openings as the optic nerve pierces the ’s lamina cribrosa, function (table 3). Both photoreceptor damage and optic causing calcifications to accumulate in axonal mitochondria, atrophy were significantly associated with peripheral vision loss, which are subsequently extruded extracellularly.18 It could be central/paracentral scotoma and any vision loss (all p<0.04). It that eyes with small foraminal openings for optic nerve axons should be noted, however, that there is a very high correlation are particularly prone to the compressive effects of elevated ICP between photoreceptor damage and optic atrophy. Four of six and would therefore be more likely to manifest with papille- eyes with photoreceptor damage also demonstrated evidence of dema. Of note, the presence of optic disc drusen did not correl- coexisting optic atrophy. Conversely, each of the four eyes with ate with vision loss in our patients, including, surprisingly, visual optic atrophy had evidence of photoreceptor damage. field defects. Of particular interest, nearly one-fifth of our patients demon- DISCUSSION strated evidence of permanent photoreceptor damage by OCT. To our knowledge, this is the first study to correlate anatomic While thinning of the RNFL and RGC layer on OCT is a hall- OCT findings to final visual function outcomes in a cohort of mark of optic atrophy and not unexpected in severe IIH, it is patients with paediatric IIH. Supporting the prevailing view in not obvious how retinal photoreceptors would suffer damage in the field, our pubertal patients were overwhelmingly female this disease. Notably, all four eyes that developed optic atrophy (100%); in contrast, a majority of our pre-pubertal patients also demonstrated photoreceptor damage on OCT. (63%) were male. While medical or surgical treatment of ele- There have been prior reports of photoreceptor damage (par- vated ICP was effective in stabilising or improving visual acuity, ticularly to cones) in patients with optic neuropathies, including a sizeable portion of study eyes (23%) were left with permanent glaucoma, , ischaemic and compressive optic neu- visual dysfunction. Pubertal children were significantly more ropathies, and IIH.19 20 One proposed explanation is that retro- likely to develop optic atrophy or photoreceptor damage by grade trans-synaptic degeneration may occur from the inner to OCT, but any increased propensity to develop adverse visual the outer retina. For this to occur, retrograde degeneration outcomes did not meet statistical significance. Clinical grading would have to proceed across two series of synapses: the RGC/ of papilledema by the modified Frisén scale on presentation was bipolar cell synapse in the inner plexiform layer and the bipolar the strongest predictor of visual morbidity as none of the eyes cell/photoreceptor synapse in the outer plexiform layer. One with a papilledema grade of 2 or less developed permanent recent primate study in which half of the retina’s RGCs were

Table 3 Contingency analysis of visual significance of optical coherence tomography (OCT) abnormalities Adverse outcome (OR (p value))

OCT finding Any vision loss Peripheral Field loss Central/paracentral scotoma Optic atrophy Photoreceptor loss

Optic atrophy Infinite (<0.0001) 31.5 (0.005) 78 (0.001) Infinite (<0.0001) Photoreceptor loss 120 (<0.0001) 9.5 (0.037) Infinite (<0.0001) Infinite (<0.0001) Infinite OR due to 100% or 0% occurrence in one subpopulation.

508 Gospe SM, et al. Br J Ophthalmol 2016;100:505–509. doi:10.1136/bjophthalmol-2015-307043 Downloaded from http://bjo.bmj.com/ on April 14, 2016 - Published by group.bmj.com Clinical science experimentally ablated without any subsequent photoreceptor Contributors SMG and MAE-D: design of the study, conduct of the study and loss casts significant doubt on retrograde trans-synaptic degener- manuscript preparation. SMG, MTB, and MAE-D: data analysis and interpretation, fi ation as a mechanism for photoreceptor damage in optic manuscript review and nal approval of the manuscript. neuropathies.21 Competing interests None declared. Another potential cause of photoreceptor pathology in IIH is Ethics approval Duke University Medical Center Institutional Review Board. a mechanical insult due to choroidal folds in the macula that Provenance and peer review Not commissioned; externally peer reviewed. commonly develop in papilledema. 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Gospe SM, et al. Br J Ophthalmol 2016;100:505–509. doi:10.1136/bjophthalmol-2015-307043 509 Downloaded from http://bjo.bmj.com/ on April 14, 2016 - Published by group.bmj.com

Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension Sidney M Gospe III, M Tariq Bhatti and Mays A El-Dairi

Br J Ophthalmol 2016 100: 505-509 originally published online August 12, 2015 doi: 10.1136/bjophthalmol-2015-307043

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