pediatric intraocular tumors: what have i learned over the past 25 years?

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Abstracts Papers Costenbader Lecture 001 Tomorrow's physicians: How will they be educated? Susan Day Relevance: Education is one of the key missions of AAPOS. Changes in how we educate physicians are inevitable, as societal demands change, as the pool of physicians' expectations change, and most importantly, as new policies prevail which govern how physicians in training will be paid and how they are to be educated. Format: this lecture will be given from the author's perspective as a program director, her participation in national educational accred- iting and certifying organizations, and her representational review of literature pertaining to physician education. Content: A review of the evolution of medical education in the post-Flexner era will be incorporated, as well as a review of exter- nal pressures threatening to change the traditional peer-driven process of setting standards. Ophthalmology has been pivotal in such changes, with individuals such as Derrick Vail and Edward Jackson revered as leading this evolutionary process. Inevitably, as knowledge has expanded, the need for setting standards has become more specialized, such as setting standards for pediatric ophthalmology and strabismus. A review of varying perspectives— of patients, of "distributive justice" governing decision makers' concept of health care, of physicians in training and in practice, and of educators will be given. A review of financial support for education, as well as future anticipated trends, will be given. Finally, the speaker will address and suggest ways in which fur- ther changes in medical education might benefit student, educator, and society. Learning Objectives: (1) To identify how existing organizations influence medical education; (2) to identify how peer involvement in setting standards differs from alternative approaches; and (3) to identify strategies for improving from a societal perspective the qualifications of physicians of the future. Apt Lecture 002 Pediatric intraocular tumors: What have I learned over the past 25 years? Carol Shields Purpose: To review the spectrum of intraocular tumors in children and the evolution in management. Methods: Retrospective series. Results: The most common intraocular tumor is the choroidal nevus. Risk factors predictive of growth into melanoma have been identified and include thickness .2 mm, subretinal fluid, symptoms, orange pigment, and margin at the optic disc. More recently 3 more risk fac- tors were added including ultrasound hollow, halo absent, drusen ab- sent. Choroidal nevus grows into melanoma at a rate of 1 in 8,800 cases. Risk factors help identify those at risk for growth. Only 1% of choroidal melanoma occurs in children and their prognosis seems to parallel adults. Retinoblastoma has evolved tremendously in 25 years with im- proved classification, identification, imaging, and therapy. The Inter- national Classification of Retinoblastoma, established in 2004, has more appropriately allowed clinicians to stage an eye and predict if chemotherapy is beneficial. Chemoreduction still dominates conservative therapy and plaque radiotherapy is used for failures. Intraarterial chemotherapy and intravitreal chemotherapy are newer avenues to explore. Early results are encouraging but ophthalmic side effects can be profound. Other tumors in children to consider include choroidal hemangi- oma, choroidal osteoma, and rarely choroidal metastasis. Newer therapies with photodynamic therapy and plaque radiotherapy have changed our approach. Retinal pigment epithelial (RPE) tumors such as congenital hypertrophy of RPE, combined hamartoma, con- genital simple hamartoma, and torpedo maculopathy are all found in children. Conclusions: There are numerous intraocular tumors in children. Our understanding has improved over the past 25 years. Identification with correct diagnosis and proper therapy for each tumor is key for tumor control and patient outcome. 003 The expanding spectrum of retinal vasoproliferative tumors in children. Jerry Shields Background/Purpose: Retinal vasoproliferative tumor (RVPT) was originally described as a rather typical idiopathic fundus lesion that occurs in adults. Subsequently, it was subdivided into primary and secondary types, the latter being seen in children as well as adults. The purpose of this presentation is to elucidate the expanding spectrum of secondary RVPTs that includes several important pedi- atric fundus conditions. Methods: Review of lesions coded as RVPT on the Oncology Service of Wills Eye Institute and literature search for other causes of sec- ondary RVPTs in children. Results: The secondary form of RVPT was found to be associated with a variety of underlying ophthalmic disorders that can occur in children, such as intermediate uveitis (pars planitis), retinitis pigmen- tosa including Usher's syndrome, choroidal coloboma, Coats' disease, retinopathy of prematurity, familial exudative vitreoretinop- athy, aniridia, Waardenburg syndrome, neurofibromatosis type 1, x-linked retinoschisis and other pediatric ocular disorders. Illustra- tive clinical examples will be shown. Conclusions: Secondary RVPTs have now been recognized in an ar- ray of well known pediatric fundus entities. Pediatric ophthalmolo- gists should be aware of these associations and be able to differentiate these lesions from retinoblastoma and other serious oc- ular conditions of childhood. Young Investigator Award Lecture 004 Posterior capsular plaque in bilateral congenital cataracts. David Morrison Introduction: The purpose of this study is to evaluate the incidence of posterior capsular plaque associated with bilateral congenital cataracts. Methods: Children with bilateral congenital cataracts were identified from three surgical practices. Children were included if bilateral len- sectomy occurred prior to 7 months and if the child had no systemic condition associated with progressive cataract formation. Surgical videos were evaluated for cataract morphology. Results: Fourteen children with bilateral cataracts were indentified. Surgical videos were available for 24 eyes. Five eyes (21%) had evidence of persistent hyaloid remnants. Of the remaining 19 eyes, cataracts were defined as nuclear in 10 cases, nuclear with cortical extension in 6 cases, and posterior cortical in 3 cases. Seven cases (37%) had evidence of posterior capsular plaque on video review. 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Page 1: Pediatric intraocular tumors: What have I learned over the past 25 years?

Abstracts

Papers

Costenbader Lecture

001 Tomorrow's physicians: How will they be educated? SusanDayRelevance: Education is one of the key missions of AAPOS. Changesin how we educate physicians are inevitable, as societal demandschange, as the pool of physicians' expectations change, and mostimportantly, as new policies prevail which govern how physiciansin training will be paid and how they are to be educated.Format: this lecture will be given from the author's perspective asa program director, her participation in national educational accred-iting and certifying organizations, and her representational review ofliterature pertaining to physician education.Content: A review of the evolution of medical education in thepost-Flexner era will be incorporated, as well as a review of exter-nal pressures threatening to change the traditional peer-drivenprocess of setting standards. Ophthalmology has been pivotal insuch changes, with individuals such as Derrick Vail and EdwardJackson revered as leading this evolutionary process. Inevitably,as knowledge has expanded, the need for setting standards hasbecome more specialized, such as setting standards for pediatricophthalmology and strabismus. A review of varying perspectives—of patients, of "distributive justice" governing decision makers'concept of health care, of physicians in training and in practice,and of educators will be given. A review of financial support foreducation, as well as future anticipated trends, will be given.Finally, the speaker will address and suggest ways in which fur-ther changes in medical education might benefit student, educator,and society.Learning Objectives: (1) To identify how existing organizationsinfluence medical education; (2) to identify how peer involvementin setting standards differs from alternative approaches; and (3) toidentify strategies for improving from a societal perspective thequalifications of physicians of the future.

Apt Lecture

002 Pediatric intraocular tumors: What have I learned over thepast 25 years? Carol ShieldsPurpose: To review the spectrum of intraocular tumors in childrenand the evolution in management.Methods: Retrospective series.Results: The most common intraocular tumor is the choroidal nevus.Risk factors predictive of growth into melanoma have been identifiedand include thickness .2 mm, subretinal fluid, symptoms, orangepigment, and margin at the optic disc. More recently 3 more risk fac-tors were added including ultrasound hollow, halo absent, drusen ab-sent. Choroidal nevus grows into melanoma at a rate of 1 in 8,800cases. Risk factors help identify those at risk for growth. Only 1%of choroidal melanoma occurs in children and their prognosis seemsto parallel adults.Retinoblastoma has evolved tremendously in 25 years with im-

proved classification, identification, imaging, and therapy. The Inter-national Classification of Retinoblastoma, established in 2004, hasmore appropriately allowed clinicians to stage an eye and predictif chemotherapy is beneficial. Chemoreduction still dominatesconservative therapy and plaque radiotherapy is used for failures.

Journal of AAPOS

Intraarterial chemotherapy and intravitreal chemotherapy are neweravenues to explore. Early results are encouraging but ophthalmicside effects can be profound.Other tumors in children to consider include choroidal hemangi-

oma, choroidal osteoma, and rarely choroidal metastasis. Newertherapies with photodynamic therapy and plaque radiotherapyhave changed our approach. Retinal pigment epithelial (RPE) tumorssuch as congenital hypertrophy of RPE, combined hamartoma, con-genital simple hamartoma, and torpedo maculopathy are all found inchildren.Conclusions: There are numerous intraocular tumors in children. Ourunderstanding has improved over the past 25 years. Identificationwith correct diagnosis and proper therapy for each tumor is keyfor tumor control and patient outcome.

003 The expanding spectrum of retinal vasoproliferative tumors inchildren. Jerry ShieldsBackground/Purpose: Retinal vasoproliferative tumor (RVPT) wasoriginally described as a rather typical idiopathic fundus lesionthat occurs in adults. Subsequently, it was subdivided into primaryand secondary types, the latter being seen in children as well asadults. The purpose of this presentation is to elucidate the expandingspectrum of secondary RVPTs that includes several important pedi-atric fundus conditions.Methods: Review of lesions coded as RVPTon the Oncology Serviceof Wills Eye Institute and literature search for other causes of sec-ondary RVPTs in children.Results: The secondary form of RVPT was found to be associatedwith a variety of underlying ophthalmic disorders that can occur inchildren, such as intermediate uveitis (pars planitis), retinitis pigmen-tosa including Usher's syndrome, choroidal coloboma, Coats'disease, retinopathy of prematurity, familial exudative vitreoretinop-athy, aniridia, Waardenburg syndrome, neurofibromatosis type 1,x-linked retinoschisis and other pediatric ocular disorders. Illustra-tive clinical examples will be shown.Conclusions: Secondary RVPTs have now been recognized in an ar-ray of well known pediatric fundus entities. Pediatric ophthalmolo-gists should be aware of these associations and be able todifferentiate these lesions from retinoblastoma and other serious oc-ular conditions of childhood.

Young Investigator Award Lecture

004 Posterior capsular plaque in bilateral congenital cataracts.David MorrisonIntroduction: The purpose of this study is to evaluate the incidence ofposterior capsular plaque associated with bilateral congenitalcataracts.Methods: Children with bilateral congenital cataracts were identifiedfrom three surgical practices. Children were included if bilateral len-sectomy occurred prior to 7 months and if the child had no systemiccondition associated with progressive cataract formation. Surgicalvideos were evaluated for cataract morphology.Results: Fourteen children with bilateral cataracts were indentified.Surgical videos were available for 24 eyes. Five eyes (21%) hadevidence of persistent hyaloid remnants. Of the remaining 19 eyes,cataracts were defined as nuclear in 10 cases, nuclear with corticalextension in 6 cases, and posterior cortical in 3 cases. Seven cases(37%) had evidence of posterior capsular plaque on video review.

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