incidence of pediatric horner syndrome and the risk of neuroblastoma: a population-based study

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consisted of 2 hours of daily patching plus randomization to either 0.51 mg/kg/tid levodopa with carbidopa 0.17 mg/kg/tid OR 0.76 mg/ kg/tid levodopa with carbidopa 0.17 mg/kg/tid for 8 to 10 weeks. Vi- sual acuity was measured with E-ETDRS testing after stopping the medication and again 8-12 weeks later after a period of patching alone. Adverse events were elicited at each contact. Results: The mean improvement after 8-10 weeks of treatment was 4 letters in the 16 subjects in the low-dose group and 6 letters in the 17 subjects in the high dose group. Ten or more letters of improvement was noted in 2 (13%) of the low-dose group and 5 (29%) in the high- dose group. No subject stopped the study medication. Headaches were reported by 6 subjects. After discontinuing levodopa, the mean change 8-12 weeks later was 2 letters in the low-dose group and 3 letters in the high-dose group. Conclusions: Improvement in amblyopic eye acuity was observed in both groups. Levodopa/carbidopa was well tolerated. A placebo con- trolled trial is necessary to determine whether levodopa can aug- ment occlusion therapy. 026 Analysis of clinical misdiagnoses in children treated with enucleation. Tina Rutar, Susan Hung, Michele Bloomer, J. Brooks Crawford Introduction: The purpose of the retrospective case series was to evaluate discordant clinical and pathological diagnoses that have led to pediatric enucleations over time. Methods: All pathology reports of pediatric enucleation specimens (subject ages 0 to 18 years) from one ocular pathology laboratory from 1960 to 2008 were reviewed. Specimens with discordant clinical and pathologic diagnoses were further analyzed. Formalin-fixed, par- affin-embedded sections of enucleated eyes of any misdiagnoses were reevaluated. Results: Out of 729 pediatric patients (746 eyes) who underwent enu- cleation during the study period, there were 29 patients (4.0%) and 30 eyes (4.0%) with discordant clinical and pathological diagnoses. Of the 362 patients enucleated for clinically-suspected malignancies, 21 (5.8%) were misdiagnosed in that no evidence of malignancy was found on histopathological examination. Of the 367 patients enucleated for suspected benign clinical indications, 7 (1.9%) had intraocular malignancies by histopathology. The misdiagnosis enu- cleation rate decreased with each respective decade studied, with the highest rate of 6.5% (18/276 eyes) in the 1960s and no misdiag- noses occurring from 1990 to 2008. Conclusions: In children, benign and malignant intraocular condi- tions can simulate each other, especially retinoblastoma, Coats dis- ease, nematode, and bacterial endophthalmitis, panuveitis, and persistent hyperplastic primary vitreous. Clinical misdiagnoses in pe- diatric enucleation specimens have decreased throughout the past five decades, likely due to improved diagnostic techniques. However, we recommend that in uncertain situations, the ophthalmologist dis- cuss the remote possibility of clinical misdiagnosis during the pedi- atric enucleation consent process. 027 Incidence of pediatric Horner syndrome and the risk of neuroblastoma: A population-based study. Stephen J. Smith, Nancy N. Diehl, Jacqueline A. Leavitt, Brian G. Mohney Purpose: There are no known population-based studies on the inci- dence of pediatric Horner syndrome, and the current recommended work-up is based on the frequency of underlying etiologies, including neuroblastoma, reported at major referral centers. The purpose of this study is to describe the incidence of pediatric Horner syndrome and the risk of occult malignancy in a population-based cohort. Methods: The medical records of all pediatric patients ( \19 years) residing in a defined population who were diagnosed with Horner syndrome from January 1, 1969, through December 31, 2008, were retrospectively reviewed. Results: Twenty pediatric patients were diagnosed with Horner Syn- drome during the 40-year period, yielding an age- and gender-ad- justed incidence of 1.42 (95% CI, 0.80-2.04) per 100,000 patients \ 19 years of age. Eleven (55%) of the 20 had a congenital onset, for a birth prevalence of 1 in 6250 (95% CI, 3333-10000), while the remain- ing 9 (45%) were acquired. Seven (63.6%) of the 11 congenital cases had a history of birth trauma while the remaining 4 (36.4%) had no identifiable cause for their syndrome. Six (66%) of the 9 acquired cases occurred following surgery or trauma while the remaining 3 (33%) had no known etiology. None (95% CI, 0.0%-16.8%) of the 20 pa- tients were found to have a neuroblastoma or other malignancy dur- ing a mean following of 56.5 months (range, 0.0-256.9 months). Conclusions: The incidence of pediatric Horner syndrome in this population was 1.42 per 100,000 patients \19 years, with a birth prev- alence of 1 in 6,250 for those with congenital onset. Birth, surgical or other trauma accounted for 13 (65%) of the patients while none of the 20 patients were found to have an underlying mass lesion. 028 Trabeculotomy without scleral flap for the treatment of pediatric glaucoma. Erin D. Stahl, Scott E. Olitsky Introduction: The purpose of this report is to describe a modification of the conventional trabeculotomy procedure. This retrospective, case series review examines 14 cases of trabeculotomy surgery per- formed without a scleral flap. In these cases, the radial scleral inci- sion was initiated into full-thickness sclera and dissected posteriorly until Schlemm's canal was located. The scleral incision was then se- cured with 10-0 vicryl suture. Methods: Our patient database was searched for trabeculotomy sur- geries performed since September 2006. The operative reports were reviewed and surgical data was collected on those cases done with- out a scleral flap. Data on age, gender, etiology of elevated intraoc- ular pressure, corneal diameter, pre-op intraocular pressure, location of trabeculotomy site, evidence of canal, ability to cannulate in both directions, and 1 day postoperative examination of wound and anterior chamber were recorded. Results: Of the 14 cases there was no incidence of postoperative wound leak or flat anterior chamber. In all cases, Schlemm's canal was located and cannulated with the trabeculotome in both direc- tions. Etiology of glaucoma treated in this study included infantile glaucoma, juvenile glaucoma, and aphakic glaucoma. Conslusions: This case series shows that trabeculotomy can be suc- cessfully completed without the use of a scleral flap. Abandoning the need for the scleral flap in trabeculotomy surgery may be beneficial in cases of advanced glaucoma in which the sclera has become stretched and thinned. Primary cases have also been found to be satisfactorily completed without the creation of a scleral flap. 029 Anterior transposition of the inferior oblique for unilateral superior oblique palsy. Mitchell B. Strominger, Nikhil N. Batra, Noopur N. Batra Purpose: Anterior transposition of the inferior oblique muscle for the treatment of unilateral superior oblique palsy remains controversial due to reports of surgical overcorrection resulting in postoperative hypotropia. This study was to determine our incidence of postopera- tive hypotropia in primary position and the effectiveness of anterior transposition of the inferior oblique muscle in the treatment of unilat- eral superior oblique palsy with inferior oblique overaction. Journal of AAPOS Volume 14 Number 1 / February 2010 e7

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Page 1: Incidence of pediatric Horner syndrome and the risk of neuroblastoma: A population-based study

Volume 14 Number 1 / February 2010 e7

consisted of 2 hours of daily patching plus randomization to either0.51 mg/kg/tid levodopa with carbidopa 0.17 mg/kg/tid OR 0.76 mg/kg/tid levodopa with carbidopa 0.17 mg/kg/tid for 8 to 10 weeks. Vi-sual acuity was measured with E-ETDRS testing after stopping themedication and again 8-12 weeks later after a period of patchingalone. Adverse events were elicited at each contact.Results: The mean improvement after 8-10 weeks of treatment was 4letters in the 16 subjects in the low-dose group and 6 letters in the 17subjects in the high dose group. Ten or more letters of improvementwas noted in 2 (13%) of the low-dose group and 5 (29%) in the high-dose group. No subject stopped the study medication. Headacheswere reported by 6 subjects. After discontinuing levodopa, themean change 8-12 weeks later was 2 letters in the low-dose groupand �3 letters in the high-dose group.Conclusions: Improvement in amblyopic eye acuity was observed inboth groups. Levodopa/carbidopa was well tolerated. A placebo con-trolled trial is necessary to determine whether levodopa can aug-ment occlusion therapy.

026 Analysis of clinical misdiagnoses in children treated withenucleation. Tina Rutar, Susan Hung, Michele Bloomer, J. BrooksCrawfordIntroduction: The purpose of the retrospective case series was toevaluate discordant clinical and pathological diagnoses that haveled to pediatric enucleations over time.Methods: All pathology reports of pediatric enucleation specimens(subject ages 0 to 18 years) from one ocular pathology laboratoryfrom 1960 to 2008 were reviewed. Specimens with discordant clinicaland pathologic diagnoses were further analyzed. Formalin-fixed, par-affin-embedded sections of enucleated eyes of any misdiagnoseswere reevaluated.Results: Out of 729 pediatric patients (746 eyes) who underwent enu-cleation during the study period, there were 29 patients (4.0%) and 30eyes (4.0%) with discordant clinical and pathological diagnoses. Ofthe 362 patients enucleated for clinically-suspected malignancies,21 (5.8%) were misdiagnosed in that no evidence of malignancywas found on histopathological examination. Of the 367 patientsenucleated for suspected benign clinical indications, 7 (1.9%) hadintraocular malignancies by histopathology. The misdiagnosis enu-cleation rate decreased with each respective decade studied, withthe highest rate of 6.5% (18/276 eyes) in the 1960s and no misdiag-noses occurring from 1990 to 2008.Conclusions: In children, benign and malignant intraocular condi-tions can simulate each other, especially retinoblastoma, Coats dis-ease, nematode, and bacterial endophthalmitis, panuveitis, andpersistent hyperplastic primary vitreous. Clinical misdiagnoses in pe-diatric enucleation specimens have decreased throughout the pastfive decades, likely due to improved diagnostic techniques. However,we recommend that in uncertain situations, the ophthalmologist dis-cuss the remote possibility of clinical misdiagnosis during the pedi-atric enucleation consent process.

027 Incidence of pediatric Horner syndrome and the risk ofneuroblastoma: A population-based study. Stephen J. Smith,Nancy N. Diehl, Jacqueline A. Leavitt, Brian G. MohneyPurpose: There are no known population-based studies on the inci-dence of pediatric Horner syndrome, and the current recommendedwork-up is based on the frequency of underlying etiologies, includingneuroblastoma, reported at major referral centers. The purpose ofthis study is to describe the incidence of pediatric Horner syndromeand the risk of occult malignancy in a population-based cohort.

Journal of AAPOS

Methods: The medical records of all pediatric patients (\19 years)residing in a defined population who were diagnosed with Hornersyndrome from January 1, 1969, through December 31, 2008, wereretrospectively reviewed.Results: Twenty pediatric patients were diagnosed with Horner Syn-drome during the 40-year period, yielding an age- and gender-ad-justed incidence of 1.42 (95% CI, 0.80-2.04) per 100,000 patients \19 years of age. Eleven (55%) of the 20 had a congenital onset, fora birth prevalence of 1 in 6250 (95% CI, 3333-10000), while the remain-ing 9 (45%) were acquired. Seven (63.6%) of the 11 congenital caseshad a history of birth trauma while the remaining 4 (36.4%) had noidentifiable cause for their syndrome. Six (66%) of the 9 acquiredcases occurred following surgery or trauma while the remaining 3(33%) had no known etiology. None (95% CI, 0.0%-16.8%) of the 20 pa-tients were found to have a neuroblastoma or other malignancy dur-ing a mean following of 56.5 months (range, 0.0-256.9 months).Conclusions: The incidence of pediatric Horner syndrome in thispopulation was 1.42 per 100,000 patients\19 years, with a birth prev-alence of 1 in 6,250 for those with congenital onset. Birth, surgical orother trauma accounted for 13 (65%) of the patients while none of the20 patients were found to have an underlying mass lesion.

028 Trabeculotomy without scleral flap for the treatment ofpediatric glaucoma. Erin D. Stahl, Scott E. OlitskyIntroduction: The purpose of this report is to describe a modificationof the conventional trabeculotomy procedure. This retrospective,case series review examines 14 cases of trabeculotomy surgery per-formed without a scleral flap. In these cases, the radial scleral inci-sion was initiated into full-thickness sclera and dissected posteriorlyuntil Schlemm's canal was located. The scleral incision was then se-cured with 10-0 vicryl suture.Methods: Our patient database was searched for trabeculotomy sur-geries performed since September 2006. The operative reports werereviewed and surgical data was collected on those cases done with-out a scleral flap. Data on age, gender, etiology of elevated intraoc-ular pressure, corneal diameter, pre-op intraocular pressure,location of trabeculotomy site, evidence of canal, ability to cannulatein both directions, and 1 day postoperative examination of wound andanterior chamber were recorded.Results: Of the 14 cases there was no incidence of postoperativewound leak or flat anterior chamber. In all cases, Schlemm's canalwas located and cannulated with the trabeculotome in both direc-tions. Etiology of glaucoma treated in this study included infantileglaucoma, juvenile glaucoma, and aphakic glaucoma.Conslusions: This case series shows that trabeculotomy can be suc-cessfully completed without the use of a scleral flap. Abandoning theneed for the scleral flap in trabeculotomy surgery may be beneficialin cases of advanced glaucoma in which the sclera has becomestretched and thinned. Primary cases have also been found to besatisfactorily completed without the creation of a scleral flap.

029 Anterior transposition of the inferior oblique for unilateralsuperior oblique palsy. Mitchell B. Strominger, Nikhil N. Batra,Noopur N. BatraPurpose: Anterior transposition of the inferior oblique muscle for thetreatment of unilateral superior oblique palsy remains controversialdue to reports of surgical overcorrection resulting in postoperativehypotropia. This study was to determine our incidence of postopera-tive hypotropia in primary position and the effectiveness of anteriortransposition of the inferior oblique muscle in the treatment of unilat-eral superior oblique palsy with inferior oblique overaction.