Abstracts
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Abstracts Edited by Ronald V. Keech, MD Retinoblastoma treated with primary chemotherapy alone: the significance of tumour size, location, and age. Gombos DS, Kelly A, Coen PG, Kingston JE, *Hungerford JL. British Journal of Ophthal- mology 2002;86:80-3. The authors’ goal was to determine whether there is any corre- lation between tumor size, tumor location, or patient’s age at diag- nosis of retinoblastoma and response to chemotherapy. The medical records from the ocular oncology service of 2 hospitals were reviewed for children with intraocular retinoblastoma who were treated only with chemotherapy and followed for at least a year. It is not stated whether this was a selected group or whether it included all children who met these criteria. In general, the chemo- therapy protocal consisted of carboplatin, vincristine, and etoposide given at 21-day intervals for either 6 or 8 cycles. Patient data included sex, age at presentation, eye involvement, and genetic presentation (familial versus sporadic). Each eye with a tumor was staged by using the Reese-Ellsworth classification. Each tumor was coded by size and location. The basal diameter was estimated by using indirect ophthalmoscopy. In some cases, ultrasonography was used to verify the measurement. Each tumor was classified into 1 of 3 different locations—macula, macula to equator, equator to ora serrata. Tumors were divided into those that responded or failed to respond to chemotherapy. Failure was defined as a lack of response or continued growth of the tumor requiring additional treatment. Thirty-six patients met the inclusion criteria; 18 (50%) were boys, 10 (28%) had a positive family history, and 8 (22%) were unilateral sporadic cases. The median age at diagnosis was 8.8 months with a range of 2 weeks to 5 years. A total of 78 tumors were identified in 42 eyes. By using the Reese-Ellsworth classification, 8 tumors were Ia or Ib, 40 were IIa or IIb, 20 were IIIa or IIIb, 5 were IVa or IVb, and 19 were Va or Vb. The basal diameters of the tumors were 0.25 to 17 mm, with a mean diameter of 6.6 mm. Thirty-one (40%) of the tumors were located in the macula, 39 (50%) between the macula and equator, and 8 (10%) between the equator and ora serrata. Fifty six (72%) of the tumors responded successfully to chemotherapy alone, but 22 (28%) required additional therapy. Twenty-six (84%) of the macular tumors responded to chemotherapy compared with 30 (64%) of the extramacular tumors; this was statistically significant (P .060). Based on the Reese-Ellsworth classification, 22 (69%) of the 32 eyes with staging from Ia (solitary tumor 4 dd behind the equator) to IIIb (solitary tumors 10 dd behind the equator) and 2 (20%) of the 10 eyes with staging from IVa (multiple tumors 10 dd) to Vb (vitreous seeding) were successfully treated with chemotherapy alone. The mean basal diameter was 6.7 mm for tumors that responded to chemotherapy and 6.2 mm for those that required additional therapy. Forty-six (77%) of the tumors with a basal diameter greater than 2 mm were successfully treated with chemotherapy, but only 10 (56%) of the tumors less than or equal to 2 mm responded (P .088). Five (63%) of the 8 tumors diagnosed in children less than 2 months of age failed to respond to chemotherapy compared with 17 (24%) of the 70 children older than 2 months of age at diagnosis (P .032). The time to failure to respond to chemotherapy ranged from 2 to 25 months (mean, 10.8 months). Eighty-two percent of the tumors that did not respond were evident by 12 months after treatment. Visual acuity outcomes were not reported. The authors concluded that retinoblastoma is more likely to respond to primary chemotherapy if it is a sporadic presentation and located in the macula, and the patient is older than 2 months at presentation. They suggest that tumors less than 2 mm in basal diameter might be less responsive to this treatment. Visual results in children treated for macular retinoblastoma †Watts P, Westall C, Colpa L, MacKeen L, Abdolell M, Gallie B, Heon E. Eye 2002;16:75-80. Previous studies have shown that the only remaining eyes of children with retinoblastoma involving the macula might retain good vision with treatment. In this study, the authors’ goal was to eval- uate the visual outcome of the eyes of children with retinoblastoma involving the macula in at least 1 eye who underwent patching therapy of the fellow eye. It was assumed that the patients in these cases had vision impairment from the tumor, the treatment, or amblyopia. Medical records from 1 institution were reviewed for children with retinoblastoma who had macular involvement in at least 1 eye and did not undergo an enucleation in either eye. Demographic data, age at presentation, treatment received, patching therapy, and visual acuity in both eyes before patching and at the final exami- nation were recorded. Visual acuity was recorded with Cardiff vanishing optotypes, Teller Acuity Cards, or linear Snellen acuity. The extent of macular involvement was determined by using retina photographs of each patient. A circle with the fovea as the center and the radius equal to the disc-to-fovea distance was drawn on the photograph. The area within the circle occupied by the tumor scar was used to calculate the percentage of macular involvement. Fifteen children were included in the study; 12 children had bilateral tumors with macular involvement in at least 1 eye, and 3 had unilateral tumors. It appears that some bilateral cases had macular involvement in both eyes, although it was apparently asym- metric. The median age at diagnosis was 5 months (range, 2 to 30 months). Median follow-up was 25 months (range, 10 months to 7 years). Most patients were treated with a combination of chemo- therapy and focal therapy. The median age at start of patching therapy was 15 months (range, 4 to 36 months). Patching recom- mendations consisted of 4 to 8 hours a day of occlusion. The median duration of occlusion per day was 4 hours (range, 0.5 to 8 hours). The median percentage of posterior pole involvement was 34% (range, 11% to 100%). Eighty percent of the children had some improvement in their visual acuity. The final visual acuity of the 14 eyes tested ranged from 20/25 to hand motions (mean between 20/100 to 20/200). Of the children in whom final acuity was recorded, 73% had an acuity of 20/200 or better, and 21% had 20/40 or better after patching. No association was found between the age of the patient, sex, duration of patching, and percentage of posterior pole involvement and the improvement in visual acuity. * Department of Ophthamology, Ocular Oncology Service, St. Bar- tholomew’s Hospital, West Smithfield, London EC1A 7BE, United Kingdom. J AAPOS 2002;6:268-9. Copyright © 2002 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2002/$35.00 0 75/3/127428 doi:10.1067/mpa.2002.127428 †49 St. Agatha Road, Cardiff CF14 4EA, United Kingdom. Journal of AAPOS 268 August 2002
Transcript of Abstracts
AbstractsEdited by Ronald V. Keech, MD
Retinoblastoma treated with primary chemotherapy alone: thesignificance of tumour size, location, and age. Gombos DS, Kelly A,Coen PG, Kingston JE, *Hungerford JL. British Journal of Ophthal-mology 2002;86:80-3.
The authors’ goal was to determine whether there is any corre-lation between tumor size, tumor location, or patient’s age at diag-nosis of retinoblastoma and response to chemotherapy. The medicalrecords from the ocular oncology service of 2 hospitals werereviewed for children with intraocular retinoblastoma who weretreated only with chemotherapy and followed for at least a year. Itis not stated whether this was a selected group or whether itincluded all children who met these criteria. In general, the chemo-therapy protocal consisted of carboplatin, vincristine, and etoposidegiven at 21-day intervals for either 6 or 8 cycles. Patient dataincluded sex, age at presentation, eye involvement, and geneticpresentation (familial versus sporadic). Each eye with a tumor wasstaged by using the Reese-Ellsworth classification. Each tumor wascoded by size and location. The basal diameter was estimated byusing indirect ophthalmoscopy. In some cases, ultrasonographywas used to verify the measurement. Each tumor was classified into1 of 3 different locations—macula, macula to equator, equator toora serrata. Tumors were divided into those that responded or failedto respond to chemotherapy. Failure was defined as a lack ofresponse or continued growth of the tumor requiring additionaltreatment.
Thirty-six patients met the inclusion criteria; 18 (50%) were boys,10 (28%) had a positive family history, and 8 (22%) were unilateralsporadic cases. The median age at diagnosis was 8.8 months witha range of 2 weeks to 5 years. A total of 78 tumors were identifiedin 42 eyes. By using the Reese-Ellsworth classification, 8 tumorswere Ia or Ib, 40 were IIa or IIb, 20 were IIIa or IIIb, 5 were IVa orIVb, and 19 were Va or Vb. The basal diameters of the tumors were0.25 to 17 mm, with a mean diameter of 6.6 mm. Thirty-one (40%) ofthe tumors were located in the macula, 39 (50%) between themacula and equator, and 8 (10%) between the equator and oraserrata. Fifty six (72%) of the tumors responded successfully tochemotherapy alone, but 22 (28%) required additional therapy.Twenty-six (84%) of the macular tumors responded to chemotherapycompared with 30 (64%) of the extramacular tumors; this wasstatistically significant (P � .060). Based on the Reese-Ellsworthclassification, 22 (69%) of the 32 eyes with staging from Ia (solitarytumor � 4 dd behind the equator) to IIIb (solitary tumors � 10 ddbehind the equator) and 2 (20%) of the 10 eyes with staging from IVa(multiple tumors � 10 dd) to Vb (vitreous seeding) were successfullytreated with chemotherapy alone. The mean basal diameter was 6.7mm for tumors that responded to chemotherapy and 6.2 mm forthose that required additional therapy. Forty-six (77%) of the tumorswith a basal diameter greater than 2 mm were successfully treatedwith chemotherapy, but only 10 (56%) of the tumors less than orequal to 2 mm responded (P � .088). Five (63%) of the 8 tumors
diagnosed in children less than 2 months of age failed to respond tochemotherapy compared with 17 (24%) of the 70 children older than2 months of age at diagnosis (P � .032). The time to failure torespond to chemotherapy ranged from 2 to 25 months (mean, 10.8months). Eighty-two percent of the tumors that did not respond wereevident by 12 months after treatment. Visual acuity outcomes werenot reported.
The authors concluded that retinoblastoma is more likely torespond to primary chemotherapy if it is a sporadic presentation andlocated in the macula, and the patient is older than 2 months atpresentation. They suggest that tumors less than 2 mm in basaldiameter might be less responsive to this treatment.
Visual results in children treated for macular retinoblastoma†Watts P, Westall C, Colpa L, MacKeen L, Abdolell M, Gallie B, HeonE. Eye 2002;16:75-80.
Previous studies have shown that the only remaining eyes ofchildren with retinoblastoma involving the macula might retain goodvision with treatment. In this study, the authors’ goal was to eval-uate the visual outcome of the eyes of children with retinoblastomainvolving the macula in at least 1 eye who underwent patchingtherapy of the fellow eye. It was assumed that the patients in thesecases had vision impairment from the tumor, the treatment, oramblyopia.
Medical records from 1 institution were reviewed for childrenwith retinoblastoma who had macular involvement in at least 1 eyeand did not undergo an enucleation in either eye. Demographicdata, age at presentation, treatment received, patching therapy, andvisual acuity in both eyes before patching and at the final exami-nation were recorded. Visual acuity was recorded with Cardiffvanishing optotypes, Teller Acuity Cards, or linear Snellen acuity.The extent of macular involvement was determined by using retinaphotographs of each patient. A circle with the fovea as the centerand the radius equal to the disc-to-fovea distance was drawn on thephotograph. The area within the circle occupied by the tumor scarwas used to calculate the percentage of macular involvement.
Fifteen children were included in the study; 12 children hadbilateral tumors with macular involvement in at least 1 eye, and 3had unilateral tumors. It appears that some bilateral cases hadmacular involvement in both eyes, although it was apparently asym-metric. The median age at diagnosis was 5 months (range, 2 to 30months). Median follow-up was 25 months (range, 10 months to 7years). Most patients were treated with a combination of chemo-therapy and focal therapy. The median age at start of patchingtherapy was 15 months (range, 4 to 36 months). Patching recom-mendations consisted of 4 to 8 hours a day of occlusion. The medianduration of occlusion per day was 4 hours (range, 0.5 to 8 hours).The median percentage of posterior pole involvement was 34%(range, 11% to 100%). Eighty percent of the children had someimprovement in their visual acuity. The final visual acuity of the 14eyes tested ranged from 20/25 to hand motions (mean between20/100 to 20/200). Of the children in whom final acuity was recorded,73% had an acuity of 20/200 or better, and 21% had 20/40 or betterafter patching. No association was found between the age of thepatient, sex, duration of patching, and percentage of posterior poleinvolvement and the improvement in visual acuity.
*Department of Ophthamology, Ocular Oncology Service, St. Bar-tholomew’s Hospital, West Smithfield, London EC1A 7BE, UnitedKingdom.J AAPOS 2002;6:268-9.Copyright © 2002 by the American Association for Pediatric Ophthalmology andStrabismus.1091-8531/2002/$35.00 � 0 75/3/127428doi:10.1067/mpa.2002.127428 †49 St. Agatha Road, Cardiff CF14 4EA, United Kingdom.
Journal of AAPOS268 August 2002
The authors concluded that vision can improve in patients withretinoblastoma even if the macula is involved. They recommend atrial of patching therapy in this situation if amblyopia is likely. Thisstudy did not address the optimum amount of patching necessary.
Reliability of binocular vision measurements used in the clas-sification of convergence insufficiency. ‡Rouse MW, Borsting E,Deland PN, and The Convergence Insufficiency and Reading Study(CIRS) Group. Optometry and Visual Science 2002;79:254-64.
The purpose of this study was to determine the reliability ofbinocular vision measurements used in the classification of conver-gence insufficiency. Subjects were selected from a group of fifthand sixth grade students who had at least 20/30 best correctedvisual acuity in each eye and no strabismus by a unilateral covertest with fixation at 3 m or 30 cm. The first 20 students who met thecriteria were used as subjects. Intraexaminer and interexaminerreliability was evaluated with a standard protocal for the followingmeasurements: (1) heterophoria test (NH) (using a phoropter with arotary prism), at 30 cm with a single line 20/30 Snellen target, (2)positive and negative fusional vergences (PFV) (using the phoropterwith the rotary prisms) at 30 cm with a 20/30 Snellen target, (3) nearpoint of convergence (NPC) with a single line 20/30 Snellen targetusing an accommodative rule, and (4) monocular accommodativeamplitude (AA) of the right eye only using a single line 20/30 Snellentarget with an accommodative rule. Two masked examiners inde-pendently performed 3 consecutive sets of measurements for eachtest on every subject and repeated the tests about a week later.Intraexaminer and interexaminer agreement was assessed by usingintraclass correlation coefficients (ICC), median absolute difference(MAD), and coefficient of repeatability (COR). The ICC is an overallindex of reliability ranging between 0 and 1. It is generally acceptedthat an ICC of less than 0.4 indicates poor reliability, and an ICC ofmore than 0.75 indicates good-to-excellent reliability.
The study group consisted of 12 girls and 8 boys with an overallmean age at testing of 10.8 years (range, 10.2 to 11.5 years). For asingle examination session, the intraexaminer reliability based onICC was good (0.88 to 0.99) for the NH, NPC, and AA, and lessreliable (0.71 to 0.94) for the PFV. The reliability for the sameexaminer between 2 different examination sessions (intraexaminerbetween-session reliability) was good for the NPC (0.92 to 0.89), NH(0.81, 0.81), and AA (0.89, 0.69), and fair for the PFV (0.59, 0.53). Thereliability between 2 different examiners for a single examinationsession was good for NH (0.91, 0.72), NPC break (0.92, 0.89), and AA(0.81, 0.85), and fair for PFV (0.57, 0.65). The authors estimated that95% of all patients would have a maximum or “worst case” differ-ence at a single test session of 7 to 8 PD for NH, 12 to 14 PD for PFVto break, 5 cm for NPC, and 5 to 10 D for AA.
The authors noted that 3 common measures (NH, NPC, and AA)used in to assess and classify convergence insufficiency have good
reliability when repeated at the same session or during differentsessions. The PFV to a break had only fair reliability, with clinicallysignificant differences between sessions. They concluded that thelarge potential test-retest difference found could complicate clinicaldecision making in regard to diagnosis and treatment.
Effects of the duration of early strabismus on the binocularresponses of neurons in the monkey visual cortex (V1). MatsuuraK, Zhang B, Smith EL, §Chino YM. Investigative Ophthalmology &Visual Science 2002;43:1262-9.
Purpose: To determine how the duration of early strabismusinfluences the severity of loss of disparity sensitivity in V1 neuronsand the effects of extensive poststrabismus visual experience onthe maintenance of functional binocular connections.
Methods: Concomitant strabismus was optically simulated in 10rhesus monkeys with a prism-rearing procedure. The onset ofstrabismus was kept constant at 4 weeks of age, and the durationwas maintained for 2, 4, or 8 weeks. In 1 group of monkeys (infants),the neurophysiological experiments were conducted immediatelyafter the period of rearing with prisms. In another group (adults),after the termination of the prism-rearing regimen at either 8 or 12weeks of age, the monkeys were kept in a normal housing environ-ment until maturity, and behavioral testing was conducted beforethe recording experiments to determine their monocular and binoc-ular visual capacities. Extracellular single-unit recording methodswere used in anesthetized and paralyzed monkeys, and dichopticsine-wave gratings were used as stimuli to assess the effects of theperiod of early strabismus on binocular interactions in V1.
Result: In all strabismic monkeys, the sensitivity of V1 units tointerocular spatial phase disparity (disparity sensitivity) was signif-icantly reduced, and the prevalence of binocular suppression washigher than that found in age-matched control animals. Although 8weeks of strabismus resulted in a slightly larger loss of disparitysensitivity, the overall effects of the duration of strabismus weresurprisingly small in infant strabismic monkeys. After poststrabis-mus visual experience, a small but significantly higher degree ofdisparity sensitivity was noted in V1 if prism-rearing was terminatedafter 4 weeks of strabismus (ie, at 8 weeks of age), but not after 8weeks of strabismus (ie, at 12 weeks of age).
Conclusions: A brief period (2 weeks) of misalignment after theemergence of stereopsis is sufficient to drastically reduce func-tional binocular connections in V1, and longer periods of strabismusresult in little additional loss in disparity sensitivity. Clinically, theseresults suggest that taking corrective measures for infantile esotro-pes before the known onset age for stereopsis might be importantfor maintaining better binocular sensory function and better intero-cular alignment at later stages of development.
‡Southern California College of Optometry, 2575 Yorba Linda Blvd,Fullerton, CA 92831.
§University of Houston, College of Optometry, 505 J. Davis ArmisteadBuilding, Houston, TX 77204-2020.
Journal of AAPOSVolume 6 Number 4 August 2002 Keech 269
