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119 Opportunistic ocular infections in non-HIV immunocompromised children. Inez B. Y. Wong, Caroline K. L. Chee, Stephen C. Teoh, Eng-Soon Go, Julian W Tang, Poh-Lin Tan Introduction: Many factors have led to increased prevalence of op- portunistic infections in the pediatric population, and ophthalmo- scopic surveillance of susceptible children forms a significant workload for the ophthalmologist. Methods: Retrospective review of consecutive consultations per- formed by a pediatric ophthalmologist over a 12-month period in im- munocompromised children. Results: Twenty-seven patients (mean age 10.2 years; range, 1.3- 21.5) were screened. Underlying conditions included hematological malignancies in 20, of which 19 underwent blood/ marrow transplan- tation (BMT), steroid-resistant nephrotic syndrome, immunodefi- ciency, metastatic osteosarcoma (OGS), and 4 who received BMT for other conditions. One of 8 patients with CMV DNAemia was found to have ocular involvement. This patient developed progressive CMV retinitis despite pre-emptive ganciclovir treatment, and systemic ci- dofovir/ foscarnet therapy, requiring adjunctive intravitreal injections for disease resolution. One case referred for candidemia was found to have chorioretinitis and treated with voriconazole, but died shortly after from OGS. Another patient with disseminated cryptococcus in- fection involving skin and central nervous system had multifocal cho- roiditis which resolved with intravenous amphotericin/ flucytosine. All 3 cases did not have ocular symptoms. Discussion: The need for ophthalmoscopic examination in asymp- tomatic patients is controversial, but the present series showed a rel- atively high yield of 11% (3/27), or 30% (3/10) if excluding those without positive cultures or nucleic acid tests. Although systemic treatment for positive culture elsewhere might prevent or treat any ocular involvement, our case with CMV retinitis highlights the need for other treatments to prevent blinding complications, and hence the need for timely surveillance. Conclusions: Immunocompromised children should be screened for opportunistic ocular infections in the presence of a positive patho- gen test. 120 Efficacy of marlex mesh implantation in posterior fixation suture. Hee Kyung Yang, Yeon-hee Lee, Jeong-Min Hwang Introduction: To develop an effective method of posterior fixation su- ture with marlex mesh implantation, a nonabsorbable high-density polyethylene plastic used surgically in hernia repair or to replace tis- sue defects. Methods: Forty superior rectus muscles in 20 rabbits were divided into four groups as follows: Conventional posterior fixation suture with non-absorbable suture (conventional group), modified posterior fixation with absorbable suture and application of talc (talc group), modified posterior fixation suture with absorbable suture and marlex mesh implantation (marlex mesh group) or modified posterior fixation suture with absorbable suture with marlex mesh implantation and application of talc (marlex-mesh-with-talc group). Eight weeks post- operatively, the tensile strength, adhesion, and histological findings were evaluated. Results: The average tensile strength of the myoscleral union was greatest in the marlex-mesh-with-talc group, followed by the talc group, marlex mesh group and the conventional group (p \0.001). Minimal adhesion was found in the marlex mesh group and conven- tional group, while significant adhesion was found in groups with talc application. Conclusions: Posterior fixation suture can be effectively reinforced with marlex mesh implantation inducing minimal adhesion. 121 The accuracy of vision screening at detecting treatable ocular conditions in children with Down syndrome. Tammy L. Yanovitch, David K. Wallace, Sharon F. Freedman, Laura B. Enyedi, Priya Kishnani, Terri L. Young Introduction: Children with Down syndrome (DS) have an in- creased incidence of ocular disorders. Guidelines recommend ophthalmologic examinations every 1-2 years for these children. No study has evaluated the accuracy of photoscreening in this population. Purpose: To determine the sensitivity, specificity, and positive and negative predictive values of photoscreening at detecting AAPOS- defined, treatable ocular conditions[1] in children with DS. Methods: Vision screening was performed with photoscreening de- vices from MTI and VR in 3- to 10-year-old children with DS (n 5 50). The children underwent complete ophthalmologic evaluations by pediatric ophthalmologists. Sensitivity, specificity, and positive and negative predictive values were calculated using ophthalmo- logic examination findings as the reference standard. Results: Almost all of the children completed screening with the MTI (n 5 47, 94%) and VR (n 5 45, 90%) systems. Mean age was 6.4 2.5 years (3.1-10.8). 44 (94%) children had an identified diagnosis on oph- thalmologic examination. More than half (n527, 57%) had condi- tion(s) requiring treatment. Sensitivities of both the MTI and VR photoscreening for detecting treatable eye conditions were 0.93 [0.76, 0.99]. Specificities for the MTI and VR photoscreening were 0.35 [0.18, 0.57] and 0.55 [0.34, 0.74], respectively. Positive predictive values for the MTI and VR photoscreening were 0.66 [0.50, 0.79] and 0.69 [0.53, 0.82], respectively. Negative predictive values for the MTI and VR photoscreening were 0.78 [0.44, 0.95] and 0.81 [0.51, 0.96], respectively. Conclusions: Photoscreening is sensitive but less specific at detect- ing treatable ocular conditions in children with DS. The use of photo- screening in the DS population has the potential to save time and expense related to routine eye examinations. 122 The incidence and associated endocrine and neurologic abnormalities of optic nerve hypoplasia: A population-based study. Ryan C. Young, Nancy N. Diehl, Brian G. Mohney Purpose: To describe the incidence of optic nerve hypoplasia (ONH) and the rate of associated endocrine, neurologic, and developmental abnormalities among a population-based cohort of children. Methods: The medical records of all children ( \19 years) who were residents of a geographically-defined Midwestern community when diagnosed with ONH from January 1, 1984, through December 31, 2008, were retrospectively reviewed. Results: A total of 19 children were diagnosed with optic nerve hypo- plasia during the 25-year study period, yielding an incidence of 2.4 (95% confidence interval 1.2-3.5) per 100,00 patients \19 years of age, or 1 in 2287 live births. The mean age at diagnosis was 2.2 years, and 53% were male. Commonly associated perinatal conditions in- cluded primiparity in 8 (47%), premature birth in 6 (35%), and maternal diabetes mellitus in 3 (16%). Of the 19 study patients, 16 (84%) had bi- lateral involvement, 8 (47%) presented with decreased visual acuity, 8 (47%) had strabismus, and 5 (26%) had nystagmus. Systemic con- ditions included developmental delay in 12 (63%), neurologic deficits in 10 (53%), and endocrine dysfunction in 5 (26%). Conclusions: This population-based study demonstrated an inci- dence of optic nerve hypoplasia of approximately 1 in 2287 live births. More than half of the patients had developmental and neu- rologic deficits while one-fourth were diagnosed with endocrine dysfunction. Journal of AAPOS Volume 14 Number 1 / February 2010 e31

Opportunistic ocular infections in non-HIV immunocompromised children

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Volume 14 Number 1 / February 2010 e31

119 Opportunistic ocular infections in non-HIVimmunocompromised children. Inez B. Y. Wong, Caroline K. L.Chee, Stephen C. Teoh, Eng-Soon Go, Julian W Tang, Poh-Lin TanIntroduction: Many factors have led to increased prevalence of op-portunistic infections in the pediatric population, and ophthalmo-scopic surveillance of susceptible children forms a significantworkload for the ophthalmologist.Methods: Retrospective review of consecutive consultations per-formed by a pediatric ophthalmologist over a 12-month period in im-munocompromised children.Results: Twenty-seven patients (mean age 10.2 years; range, 1.3-21.5) were screened. Underlying conditions included hematologicalmalignancies in 20, of which 19 underwent blood/ marrow transplan-tation (BMT), steroid-resistant nephrotic syndrome, immunodefi-ciency, metastatic osteosarcoma (OGS), and 4 who received BMTfor other conditions. One of 8 patients with CMV DNAemia was foundto have ocular involvement. This patient developed progressive CMVretinitis despite pre-emptive ganciclovir treatment, and systemic ci-dofovir/ foscarnet therapy, requiring adjunctive intravitreal injectionsfor disease resolution. One case referred for candidemia was foundto have chorioretinitis and treated with voriconazole, but died shortlyafter from OGS. Another patient with disseminated cryptococcus in-fection involving skin and central nervous system had multifocal cho-roiditis which resolved with intravenous amphotericin/ flucytosine.All 3 cases did not have ocular symptoms.Discussion: The need for ophthalmoscopic examination in asymp-tomatic patients is controversial, but the present series showed a rel-atively high yield of 11% (3/27), or 30% (3/10) if excluding thosewithout positive cultures or nucleic acid tests. Although systemictreatment for positive culture elsewhere might prevent or treat anyocular involvement, our case with CMV retinitis highlights the needfor other treatments to prevent blinding complications, and hencethe need for timely surveillance.Conclusions: Immunocompromised children should be screened foropportunistic ocular infections in the presence of a positive patho-gen test.

120 Efficacy of marlex mesh implantation in posterior fixationsuture. Hee Kyung Yang, Yeon-hee Lee, Jeong-Min HwangIntroduction: To develop an effective method of posterior fixation su-ture with marlex mesh implantation, a nonabsorbable high-densitypolyethylene plastic used surgically in hernia repair or to replace tis-sue defects.Methods: Forty superior rectus muscles in 20 rabbits were dividedinto four groups as follows: Conventional posterior fixation suturewith non-absorbable suture (conventional group), modified posteriorfixation with absorbable suture and application of talc (talc group),modified posterior fixation suture with absorbable suture and marlexmesh implantation (marlex mesh group) or modified posterior fixationsuture with absorbable suture with marlex mesh implantation andapplication of talc (marlex-mesh-with-talc group). Eight weeks post-operatively, the tensile strength, adhesion, and histological findingswere evaluated.Results: The average tensile strength of the myoscleral union wasgreatest in the marlex-mesh-with-talc group, followed by the talcgroup, marlex mesh group and the conventional group (p \0.001).Minimal adhesion was found in the marlex mesh group and conven-tional group, while significant adhesion was found in groups with talcapplication.Conclusions: Posterior fixation suture can be effectively reinforcedwith marlex mesh implantation inducing minimal adhesion.

Journal of AAPOS

121 The accuracy of vision screening at detecting treatableocular conditions in children with Down syndrome. Tammy L.Yanovitch, David K. Wallace, Sharon F. Freedman, Laura B. Enyedi,Priya Kishnani, Terri L. YoungIntroduction: Children with Down syndrome (DS) have an in-creased incidence of ocular disorders. Guidelines recommendophthalmologic examinations every 1-2 years for these children.No study has evaluated the accuracy of photoscreening in thispopulation.Purpose: To determine the sensitivity, specificity, and positive andnegative predictive values of photoscreening at detecting AAPOS-defined, treatable ocular conditions[1] in children with DS.Methods: Vision screening was performed with photoscreening de-vices from MTI and VR in 3- to 10-year-old children with DS (n 5

50). The children underwent complete ophthalmologic evaluationsby pediatric ophthalmologists. Sensitivity, specificity, and positiveand negative predictive values were calculated using ophthalmo-logic examination findings as the reference standard.Results: Almost all of the children completed screening with the MTI(n 5 47, 94%) and VR (n 5 45, 90%) systems. Mean age was 6.4� 2.5years (3.1-10.8). 44 (94%) children had an identified diagnosis on oph-thalmologic examination. More than half (n527, 57%) had condi-tion(s) requiring treatment. Sensitivities of both the MTI and VRphotoscreening for detecting treatable eye conditions were 0.93[0.76, 0.99]. Specificities for the MTI and VR photoscreening were0.35 [0.18, 0.57] and 0.55 [0.34, 0.74], respectively. Positive predictivevalues for the MTI and VR photoscreening were 0.66 [0.50, 0.79] and0.69 [0.53, 0.82], respectively. Negative predictive values for the MTIand VR photoscreening were 0.78 [0.44, 0.95] and 0.81 [0.51, 0.96],respectively.Conclusions: Photoscreening is sensitive but less specific at detect-ing treatable ocular conditions in children with DS. The use of photo-screening in the DS population has the potential to save time andexpense related to routine eye examinations.

122 The incidence and associated endocrine and neurologicabnormalities of optic nerve hypoplasia: A population-basedstudy. Ryan C. Young, Nancy N. Diehl, Brian G. MohneyPurpose: To describe the incidence of optic nerve hypoplasia (ONH)and the rate of associated endocrine, neurologic, and developmentalabnormalities among a population-based cohort of children.Methods: The medical records of all children (\19 years) who wereresidents of a geographically-defined Midwestern community whendiagnosed with ONH from January 1, 1984, through December 31,2008, were retrospectively reviewed.Results: A total of 19 children were diagnosed with optic nerve hypo-plasia during the 25-year study period, yielding an incidence of 2.4(95% confidence interval 1.2-3.5) per 100,00 patients \19 years ofage, or 1 in 2287 live births. The mean age at diagnosis was 2.2 years,and 53% were male. Commonly associated perinatal conditions in-cluded primiparity in 8 (47%), premature birth in 6 (35%), and maternaldiabetes mellitus in 3 (16%). Of the 19 study patients, 16 (84%) had bi-lateral involvement, 8 (47%) presented with decreased visual acuity,8 (47%) had strabismus, and 5 (26%) had nystagmus. Systemic con-ditions included developmental delay in 12 (63%), neurologic deficitsin 10 (53%), and endocrine dysfunction in 5 (26%).Conclusions: This population-based study demonstrated an inci-dence of optic nerve hypoplasia of approximately 1 in 2287 livebirths. More than half of the patients had developmental and neu-rologic deficits while one-fourth were diagnosed with endocrinedysfunction.