utilization of mrv to evaluate pediatric patients with papilledema

2
showed significant correlation (x 2 5 9.57). All patients (n 5 7) with GVHD and .10 ophthalmology visits are now deceased. Discussion: There is a statistically significant association between ocular GVHD and mortality in BMT patients with acute GVHD. This is consistent with a 1989 study of adult patients demonstrating increased mortality in those with ocular GVHD-associated pseudo- membranous conjunctivitis. Conclusion: Screening for ocular disease in patients with GVHD is beneficial. A prospective study will provide ophthalmology evalua- tions to allogeneic BMT patients with Grade .2 GVHD, including an evaluation and management template providing a more uniform approach to care. References 1. Jabs DA, Wingard J, Green WR, et al. The eye in bone marrow transplan- tation. Conjunctival graft-vs-host disease. Arch Ophthalmol 1989;107: 1343-8. 053 Intravitreal bevacizumab for retinopathy of prematurity (ROP): the Akron Childrens Hospital (ACH) experience. Nancy N. Hanna, Richard W. Hertle, William Lawhon, Deborah Vogel, Anand Kantak Introduction: There are multiple reports regarding the use of bevaci- zumab for the treatment of ROP. This report summarizes the ACH experience regarding indications, treatment technique, and patient outcomes after use of intravitreal bevacizumab (IVB) for ROP. Methods: With institutional review board approval, we are prospec- tively collecting data in neonates treated with IVB at ACH. The indi- cation for treatment included high risk prethreshold and threshold ROP. Parental knowledge and consent were obtained after discus- sion regarding alternative intervention with standard laser, and fundus videography was performed prior to, immediately after and one week after injection. The neonates were treated with topical antibiotics then re-examined every 1-4 weeks until 70 weeks post- conceptual age. A neonatologist reviewed all patient records to evaluate for possible side effects of treatment. Results: 34 eyes in 17 neonates were treated between 3/2011 and 9/2013 with one injection of 0.625 mg bevacizumab in 0.025 ml solu- tion. The mean gestational age was 24.2 wks, birth weight 616 gms and age at treatment 36.1 weeks. There were no unfavorable outcomes, 32 eyes in 16 patients had uncomplicated regression of ROP. Both eyes in one patient had subsequent vitreous hemorrhage requiring monocular laser and vitrectomy. 2 neonates developed strabismus, 30% of eyes with regression had remaining avascular retina. No systemic complications could be attributed to IVB. Discussion: IVB is an alternative, and possibly advantageous treatment for ROP. Conclusion: Further experience, research and data collection, as was done for laser treatment, needs to be accomplished for IVB to approach a standard of care therapy References 1. Mintz-Hittner HA, Kennedy KA, Chuang AZ; BEAT-ROP Cooperative Group. Efficacy of intravitreal bevacizumab for stage 3+ retinopathy of prematurity. N Engl J Med 2011;17(364):603-15. 2. Wu WC, Kuo HK, Yeh PT, Yang CM, Lai CC, Chen SN. An updated study of the use of bevacizumab in the treatment of patients with prethreshold retinop- athy of prematurity in Taiwan. Am J Ophthalmol 2013;155:150-58. 3. Castellanos MA, Schwartz S, Garc ıa-Aguirre G, Quiroz-Mercado H. Short- term outcome after intravitreal ranibizumab injections for the treatment of retinopathy of prematurity. Br J Ophthalmol 2013;97:816-19. 054 Noninvasive assessment of intracranial pressure using otoacoustic emissions in pediatric patients with idiopathic intracranial hypertension (IIH). Gena Heidary, Jeffrey N. Hollander, Carly E. Milliren, Guang-Wei Zhou, Michel Fayad, Susan E. Voss Introduction: The ability to follow response to treatment with elevated intracranial pressure (ICP) and papilledema are crucial factors in the preservation of visual function in IIH. Current methods of ICP monitoring are invasive. Distortion-product otoacoustic emissions (DPOAEs), measured non-invasively through the ear canal, appear to be sensitive to ICP changes in healthy subjects. 1 We evaluated whether changes in DPOAEs correlate with changes in ICP in pediatric patients. Methods: Prospective study of pediatric patients undergoing a medically necessary lumbar puncture (LP). DPOAE magnitudes for 13 frequencies (range, 516-3984 Hz) were measured. Normal mid- dle-ear function was confirmed by otoscopy and a tympanogram. Measurements were obtained using software from Mimosa Acous- tics (HearID v3.1). Data including age, opening and closing ICPs were recorded. Results: Eighteen subjects were evaluated (14 female patients) with a median age of 13.0 years (4.0 interquartile range, IQR). Among the five patients with elevated ICP, median opening pressure was 32.6 cm H 2 O (2.2 IQR) compared with 18.0 cm H 2 O (3.4 IQR) for control pa- tients. Cases and controls differed significantly on change in ICP (P 5 0.01). Strong negative correlations were found between the change in ICP and resulting change in DPOAE magnitude at two fre- quencies, 703 Hz and 984 Hz. Spearman correlation coefficients were 0.68 and 0.62, respectively. Discussion: Changes in DPOAEs appear to reflect elevation in ICP. More extensive work is needed to determine the utility of DPOAE measurements in longitudinal monitoring of patients with IIH. Conclusion: DPOAEs may offer a promising noninvasive alternative for monitoring ICP in normal hearing patients. References 1. Voss SE, Horton NJ, Tabucchi TH, Folowosele FO, Shera CA. Posture- induced changes in distortion-product otoacoustic emissions and the po- tential for non-invasive monitoring of changes in intracranial pressure. Neurocrit Care 2006;4:251-7. 055 Utilization of MRV to evaluate pediatric patients with papilledema. Jeffrey N. Hollander, Sanjay Prabhu, Gena Heidary Introduction: In pediatric patients presenting with papilledema, there is little standardization of the diagnostic work up with respect to initial neuroimaging studies. 1 Specifically, the role of magnetic reso- nance venography (MRV) has not been fully investigated in those patients without risk factors in the clinical history that would warrant a MRV. We sought to investigate the utility of MRV in identifying pe- diatric patients with dural sinus thrombosis (DST) who present with papilledema. Methods: Ten-year (2000-2010) retrospective chart review of all pa- tients 18 years or younger with a diagnosis of idiopathic intracranial hypertension (IIH, ICD-9 348.2) or papilledema (ICD-9 377.0) at a sin- gle, tertiary care center. Data including clinical characteristics and radiographic findings at time of presentation were recorded. Results: Of the 360 patients who fulfilled the inclusion criteria, 20% (72/360) of patients had a MRV, in addition to MRI, as part of an initial evaluation. Positive findings suggestive of DST were identified in 14% (10/72) of subjects, of whom 80% were male. In addition to papille- dema, presenting symptoms and signs included headache (80%) and 6th nerve palsy (50%). Only four patients had a predisposing Journal of AAPOS Volume 18 Number 4 / August 2014 e17

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Page 1: Utilization of MRV to evaluate pediatric patients with papilledema

Volume 18 Number 4 / August 2014 e17

showed significant correlation (x2 5 9.57). All patients (n 5 7) withGVHD and .10 ophthalmology visits are now deceased.Discussion: There is a statistically significant association betweenocular GVHD and mortality in BMT patients with acute GVHD. Thisis consistent with a 1989 study of adult patients demonstratingincreased mortality in those with ocular GVHD-associated pseudo-membranous conjunctivitis.Conclusion: Screening for ocular disease in patients with GVHD isbeneficial. A prospective study will provide ophthalmology evalua-tions to allogeneic BMT patients with Grade .2 GVHD, includingan evaluation and management template providing a more uniformapproach to care.

References

1. Jabs DA, Wingard J, Green WR, et al. The eye in bone marrow transplan-tation. Conjunctival graft-vs-host disease. Arch Ophthalmol 1989;107:1343-8.

053 Intravitreal bevacizumab for retinopathy of prematurity (ROP):the Akron Childrens Hospital (ACH) experience. Nancy N. Hanna,Richard W. Hertle, William Lawhon, Deborah Vogel, Anand KantakIntroduction: There are multiple reports regarding the use of bevaci-zumab for the treatment of ROP. This report summarizes the ACHexperience regarding indications, treatment technique, and patientoutcomes after use of intravitreal bevacizumab (IVB) for ROP.Methods: With institutional review board approval, we are prospec-tively collecting data in neonates treated with IVB at ACH. The indi-cation for treatment included high risk prethreshold and thresholdROP. Parental knowledge and consent were obtained after discus-sion regarding alternative intervention with standard laser, andfundus videography was performed prior to, immediately after andone week after injection. The neonates were treated with topicalantibiotics then re-examined every 1-4 weeks until 70 weeks post-conceptual age. A neonatologist reviewed all patient records toevaluate for possible side effects of treatment.Results: 34 eyes in 17 neonates were treated between 3/2011 and9/2013 with one injection of 0.625 mg bevacizumab in 0.025 ml solu-tion. The mean gestational age was 24.2 wks, birth weight 616 gmsand age at treatment 36.1 weeks. There were no unfavorableoutcomes, 32 eyes in 16 patients had uncomplicated regression ofROP. Both eyes in one patient had subsequent vitreous hemorrhagerequiring monocular laser and vitrectomy. 2 neonates developedstrabismus, 30% of eyes with regression had remaining avascularretina. No systemic complications could be attributed to IVB.Discussion: IVB is an alternative, and possibly advantageoustreatment for ROP.Conclusion: Further experience, research and data collection, aswas done for laser treatment, needs to be accomplished for IVB toapproach a standard of care therapy

References

1. Mintz-Hittner HA, Kennedy KA, Chuang AZ; BEAT-ROP Cooperative Group.Efficacy of intravitreal bevacizumab for stage 3+ retinopathy of prematurity.N Engl J Med 2011;17(364):603-15.

2. WuWC, Kuo HK, Yeh PT, Yang CM, Lai CC, Chen SN. An updated study of theuse of bevacizumab in the treatment of patients with prethreshold retinop-athy of prematurity in Taiwan. Am J Ophthalmol 2013;155:150-58.

3. Castellanos MA, Schwartz S, Garc�ıa-Aguirre G, Quiroz-Mercado H. Short-term outcome after intravitreal ranibizumab injections for the treatment ofretinopathy of prematurity. Br J Ophthalmol 2013;97:816-19.

Journal of AAPOS

054 Noninvasive assessment of intracranial pressure usingotoacoustic emissions in pediatric patients with idiopathicintracranial hypertension (IIH). Gena Heidary, Jeffrey N. Hollander,Carly E. Milliren, Guang-Wei Zhou, Michel Fayad, Susan E. VossIntroduction: The ability to follow response to treatment with elevatedintracranial pressure (ICP) and papilledema are crucial factors in thepreservationof visual function in IIH. Currentmethodsof ICPmonitoringare invasive. Distortion-product otoacoustic emissions (DPOAEs),measured non-invasively through the ear canal, appear to be sensitiveto ICP changes in healthy subjects.1We evaluatedwhether changes inDPOAEs correlate with changes in ICP in pediatric patients.Methods: Prospective study of pediatric patients undergoing amedically necessary lumbar puncture (LP). DPOAE magnitudes for13 frequencies (range, 516-3984 Hz) were measured. Normal mid-dle-ear function was confirmed by otoscopy and a tympanogram.Measurements were obtained using software from Mimosa Acous-tics (HearID v3.1). Data including age, opening and closing ICPswere recorded.Results: Eighteen subjects were evaluated (14 female patients) witha median age of 13.0 years (4.0 interquartile range, IQR). Among thefive patients with elevated ICP, median opening pressure was 32.6 cmH2O (2.2 IQR) compared with 18.0 cm H2O (3.4 IQR) for control pa-tients. Cases and controls differed significantly on change in ICP(P 5 0.01). Strong negative correlations were found between thechange in ICP and resulting change in DPOAE magnitude at two fre-quencies, 703 Hz and 984 Hz. Spearman correlation coefficients were�0.68 and �0.62, respectively.Discussion: Changes in DPOAEs appear to reflect elevation in ICP.More extensive work is needed to determine the utility of DPOAEmeasurements in longitudinal monitoring of patients with IIH.Conclusion: DPOAEs may offer a promising noninvasive alternativefor monitoring ICP in normal hearing patients.

References

1. Voss SE, Horton NJ, Tabucchi TH, Folowosele FO, Shera CA. Posture-induced changes in distortion-product otoacoustic emissions and the po-tential for non-invasive monitoring of changes in intracranial pressure.Neurocrit Care 2006;4:251-7.

055 Utilization of MRV to evaluate pediatric patients withpapilledema. Jeffrey N. Hollander, Sanjay Prabhu, Gena HeidaryIntroduction: In pediatric patients presenting with papilledema, thereis little standardization of the diagnostic work up with respect toinitial neuroimaging studies.1 Specifically, the role of magnetic reso-nance venography (MRV) has not been fully investigated in thosepatients without risk factors in the clinical history that would warranta MRV. We sought to investigate the utility of MRV in identifying pe-diatric patients with dural sinus thrombosis (DST) who present withpapilledema.Methods: Ten-year (2000-2010) retrospective chart review of all pa-tients 18 years or younger with a diagnosis of idiopathic intracranialhypertension (IIH, ICD-9 348.2) or papilledema (ICD-9 377.0) at a sin-gle, tertiary care center. Data including clinical characteristics andradiographic findings at time of presentation were recorded.Results: Of the 360 patients who fulfilled the inclusion criteria, 20%(72/360) of patients had a MRV, in addition to MRI, as part of an initialevaluation. Positive findings suggestive of DSTwere identified in 14%(10/72) of subjects, of whom 80% were male. In addition to papille-dema, presenting symptoms and signs included headache (80%)and 6th nerve palsy (50%). Only four patients had a predisposing

Page 2: Utilization of MRV to evaluate pediatric patients with papilledema

e18 Volume 18 Number 4 / August 2014

risk factor of otitis media. The 6 remaining patients presented withoccult DST.Discussion: Utilization of MRV appeared to identify occult dural sinusthrombosis in pediatric patients with papilledema who presentedwith otherwise non-specific symptoms typically associated with idio-pathic intracranial hypertension.Conclusion: MRV should be considered in the diagnostic work up ofpediatric papilledema.

References

1. Standridge SM, O'Brien SH. Idiopathic intracranial hypertension in a pedi-atric population: a retrospective analysis of the initial imaging evaluation. JChild Neurology 2008;23:1308-11.

056 Is ophthalmic follow-up of premature infants necessary?Saurabh Jain, Charles Hennings, Melissa ChiuIntroduction: There is no universally accepted guidance on thefollow-up of babies who fulfil the criteria for retinopathy of prematu-rity (ROP) screening yet are negative for pathology.

To guide practice, we aim to investigate rates of ophthalmic disor-ders and interventions in this cohort.Methods: We investigated our current cohort of preterm infants be-ing followed up in the ophthalmic clinics. These were identified by aword search for the phrase prem through the clinic letters of threelarge London hospitals gave a base sample.

We included all children with at least two years follow up and an-alysed their charts.

We also carried out a UK wide survey of all paediatric ophthalmol-ogists to investigate current practice of follow up of preterm babies.Results: A total of 214 babies met the inclusion criteria. Of these,22.4% (48) were found to have ROP. In this group 50% (24) hadsome other form of ocular pathology. 50% (24) also required someform of intervention. 77.6% (166) of babies were found to have noROP. 34% (56) of these have some form of ocular pathology and33% (55) required some form of intervention in the form of strabismussurgery, glasses or occlusion therapy.

We found significant variation in the rate of follow up of these ba-bies in the sample of physicians surveyed.Discussion: There is significantly higher rates of ophthalmic dis-orders and intervention in our cohort of preterm children thanin the normal population. There seems to be no consensus aboutthe best way to follow up these children and when to dischargethem.Conclusion: In view of the high rate of ophthalmic disorders identi-fied in our cohort we recommend close follow up till at least 2 yearsof age or until uniocular visual acuities can be obtained.

057 Superior rectus transposition versus medial rectus recessionfor esotropic Duane retraction syndrome (DRS). Ramesh Kekunnaya,Shailja Tibrewala, Virender SachdevaIntroduction: Medial rectus recession (MRc)1 or vertical rectustranspositions (VRT)2 are the commonly described procedures foresotropic DRS. Recently superior rectus transposition (SRT) andMR recession3 have been shown to align the eyes and improveabduction. The purpose of this study was to compare the results ofSRT (with or without MRc) with MRc alone for treatment of esotropicDRS.Methods: A comparative interventional case series involving eso-tropic DRS patients who underwent surgery between May 2007 to

February 2013 was conducted. The main outcome measures werechange in the amount of esotropia and improvement in the abduction.Results: There were 8 and 16 subjects in the SRT � MRc and MRcgroup respectively. Mean age of the children was 12 � 7.1 and 12.5� 8.4 years in SRT�MRc andMRc groups respectively. Mean preop-erative esotropia reduced from 20D� 13D and 24D� 9D to 3D� 3D and4D � 6D in the SRT�MRc and MRc group respectively. Success rate(alignment within 8D of orthotropia and abnormal head posture of lessthan 5�) was 87% and 69% in the SRT�MRc and MRc group respec-tively (P5 0.62). Abduction limitation improved from�3.6� 0.7 to�2.4� 0.5 (1.2 units) in the SRT�MRc group as compared to no improve-ment in the MRc group (P 5\0.001).Discussion: The current study shows that SRT � MRc group hadadditional improvement in abduction which was comparable to pre-vious studies.2,3

Conclusion: Though both the procedures are successful in correct-ing esotropia in DRS, SRT � MRc has additional advantage ofimproving abduction.

References

1. Pressman SH, ScottWE. Surgical treatment of Duane's syndrome. Ophthal-mology 1986;93:29-38.

2. Rosenbaum AL. Costenbader lecture: the efficacy of rectus muscle trans-position surgery in esotropic Duane syndrome and VI nerve palsy. J AAPOS2004;8:409-19.

3. Mehendale RA, Dagi LR, Wu C, Ledoux D, Johnston S, Hunter DG. Superiorrectus transposition and medial rectus recession for Duane syndrome andsixth nerve palsy. Arch Ophthalmol 2012;130:195-201.

058 Compartmentalized innervation of the superior oblique (SO)muscle: double nerves for double duty? Alan Le, Joseph L. DemerIntroduction: Innervation of human horizontal rectus muscles isbifurcated into distinct superior and inferior divisions that innervatenon-overlapping compartments of muscle fibers. We suspect thatthis pattern of compartmentalized innervation may be found in otherextraocular muscles. The SO, innervated by the trochlear nerve(CN4), is a prime candidate due to the muscles important torsionaland vertical actions. We sought neuroanatomical evidence forcompartmental SO innervation.Methods:Whole orbits were obtained from four humans: two adults,a 17-month-old toddler, and a stillborn 33-week-old fetus. Whole or-bits were fixed in formalin, embedded in paraffin, coronally sectionedat 10 microns thickness, stained with Masson trichrome, and micro-photographed at 4� to 10�. Trochlear nerve branches were tracedin serial sections and reconstructed in 3-D.Results: CN4 in all four orbits divided external to the muscle. Allpost-natal CN4 bifurcated into two minimally overlapping divisionsinnervating separate muscle zones: superomedial and inferolateral.The fetal CN4 displayed four branches with more overlap.Discussion: Compartmentalized innervation of the SOmuscle may laya physiological foundation for selective activation of separatemuscle compartments analogous to those in the rectus muscles.Segregation of the CN4 into divisions innervating non-overlappingmuscle fiber compartments is probably refined during postnatalmaturation. Initial bifurcation of the main CN4 trunk external to themuscle might permit independent contraction of the two compart-ments, perhaps separately influencing torsional versus vertical duc-tions.Conclusion: The human SO muscle exhibits a compartmentalizedpattern of innervation by CN4 that might subserve separate controlof torsional and vertical actions.

Journal of AAPOS