Biomedical subjects
Publications and source records attributed to D H Abramson.
Although neurofibroma is a rare orbital tumor, ophthalmic nurses may have the first opportunity to observe patients with this tumor. These patients may first be seen with normal visual acuity, proptosis, or affected ocular motility. An interesting patient who was diagnosed with an orbital neurofibroma is presented.
A patient had inadvertently mistaken her son's diflorasone diacetate ointment for gentamicin sulfate ophthalmic ointment and applied it to her eye in error. Although no serious consequences resulted from the instillation of the topical corticosteroid, this incident provides another example of mistaken medication tubes.
PURPOSE: The anatomic location of intraocular retinoblastoma foci was investigated in patients with bilateral retinoblastoma. The study was designed to evaluate the retinal topography of intraocular tumors and their time course of presentation. METHODS: A retrospective study of 565 eyes with bilateral retinoblastoma was conducted. Data included patient age at detection of each tumor and tumor location within the retina. Intraocular location was characterized in three ways: 1) superior versus inferior retina; 2) nasal versus temporal retina; and 3) macular to peripheral retina, defined as four circular zones. RESULTS: There was a direct correlation between patient age at tumor detection and retinal topography. This relationship followed a central-to-peripheral distribution, with macular tumors presenting earliest and anterior retinal tumors presenting last. Twenty-nine (100%) macular tumors were detected at the time of initial diagnosis, and none presented after 15.5 months of age. When controlled for surface area tumors were located equally in all circular zones. CONCLUSION: There is a time course in which tumors in different parts of the retina come to clinical presentation. All macular tumors in this study and the majority of tumors in the posterior pole were detected before age 24 months. The authors provide possible explanations for these findings and their implications for treatment.
BACKGROUND: A retrospective analysis of all patients with retinoblastoma on file at the Ophthalmic Oncology Center of the New York Hospital, Cornell University Medical Center was performed to examine the characteristics of retinoblastoma in children with a unilaterally affected parent. METHODS: Data were analysed for laterality of disease, age at initial diagnosis, number of tumour foci at diagnosis, ocular outcome, and patient survival. RESULTS: There were 54 patients, including nine pairs of siblings, diagnosed with retinoblastoma, each of whom had one unilaterally affected parent. The retinoblastoma was bilateral in 49 (91%) patients and unilateral in the remaining five (9%). The mean age at diagnosis was 10.5 months (range 1-36 months) and 35 patients (65%) were diagnosed within the first year of life. The mean number of initial tumour foci was 1.7 per eye; 1.6 foci per eye in bilaterally affected patients, and 3.7 foci per eye in unilaterally affected patients (p = 0.36). Retention of the eye was possible for 51 of 103 eyes affected with retinoblastoma. The mean follow up for patients was 9.0 years, with a range of 1 month to 30.5 years. There were a total of 12 deaths (22%): from retinoblastoma (two), sarcoma (five), cutaneous malignant melanoma (one), cancer type unknown (two), and cause unknown (two). CONCLUSION: Overall, the characteristics of retinoblastoma patients with a unilaterally affected parent, were similar when compared with all retinoblastoma patients with a positive family history.
AIMS/BACKGROUND: Recent work using computerised perimetry has documented predictable visual field defects in patients with choroidal melanoma. Despite the higher frequency of occurrence of choroidal metastases, relatively little is known about their exact effects on visual performance, specifically with respect to visual field. This study is a pilot retrospective analysis of the results of automated perimetry testing in patients with choroidal metastatic disease. METHODS: The Humphrey field analyser was used to perform visual field tests on 15 eyes in 11 patients diagnosed with choroidal metastases. All 11 primary tumours were carcinomas originating in the breast (seven), lung (one), kidney (one), stomach (one), and prostate (one). All patients had either central 30 and/or peripheral 30-60 threshold strategies. RESULTS: Each of the 15 eyes tested had a demonstrable visual field defect. Eight eyes (53%) had absolute scotomas and seven (43%) had relative scotomas; the defects did not consistently correspond in size or location to the tumour as depicted by ophthalmoscopy or ultrasonography. This is in contradistinction to automated perimetry results in patients with choroidal melanoma. Three of the four eyes (75%) which were retested after treatment of the metastases showed improvement of the visual field. Seven of nine eyes (77%) that initially had reduced visual acuity had improvement after treatment. This clinical improvement corresponded well to tumour shrinkage and resolution of subretinal fluid. CONCLUSION: These findings may be useful in the decision to initiate treatment for symptomatic choroidal metastatic disease and in explaining to patients and their referring physicians what improvement could be expected from such treatment. A prospective evaluation of patients with choroidal metastases seems appropriate based on this pilot experience and may elucidate if the differences noted between visual field performance of these patients and those with choroidal melanoma may be useful in some diagnostically difficult cases.
PURPOSE: To study the attenuation and scattering effects of ocular implants, made from different materials, on the dose distributions of a 6 MV photon beam, and 6, 9, and 12 MeV electron beams used in orbital radiotherapy. METHODS AND MATERIALS: Central axis depth-dose measurements were performed in a polystyrene phantom with embedded spherical ocular implants using film dosimetry of a 6 MV photon beam and electron beams of 6, 9, and 12 MeV energy. The isodose distributions were also calculated by a computerized treatment planning system using computerized tomography (CT) scans of a polystyrene phantom that had silicone, acrylic, and hydroxyapatite ocular implants placed into it. RESULTS: Electron beam dose distributions display distortions both on the measured and calculated data. This effect is most accentuated for the hydroxyapatite implants, for which the transmissions through ocular implants are on the order of 93% for the 6 MV photon beam, and range from 60% for 6 MeV electrons to 90% for 12 MeV electrons. CONCLUSION: We studied the effect of ocular implants of various materials, embedded in a polystyrene phantom, on the dose distributions of a 6 MV photon beam, and 6, 9, and 12 MeV electron beams. Our investigations show that while 6 MV photons experience only a few percent attenuation, lower energy electron beam with 60% transmission is not a suitable choice of treating tumors behind the ocular implants.
In 1972, the Intergroup Rhabdomyosarcoma Study standardized the classification and treatment of orbital rhabdomyosarcoma. It established chemotherapy with external beam radiotherapy as the standard of treatment, yet there was no comparison to treatment with external beam radiotherapy alone. We retrospectively examined the charts of patients with orbital rhabdomyosarcoma treated solely with external beam radiotherapy for the following data: age at diagnosis, gender, tumor recurrence, and tumor-related mortality. We followed 24 patients (mean follow-up 7.9 years) treated with external beam radiotherapy alone. The average age at diagnosis was 5.5 years; there were 15 males and 9 females. Three patients (12.5%) suffered tumor recurrence and there was one (4.2%) tumor-related death. When compared to the Intergroup Rhabdomyosarcoma Study, results suggest that chemotherapy with external beam radiotherapy may offer no advantage to external beam radiotherapy alone for the treatment of orbital rhabdomyosarcoma.
The interpretation of intraocular masses on magnetic resonance imaging (MRI) scan is not always straightforward. A case presentation of a 92 year old woman who was thought to have a neoplasm is presented.
A case presentation of a ten year old girl diagnosed with acute lymphoblastic leukemia is described. Following her combination chemotherapy regimen with steroids and asparaginase, a most interesting development of hyperlipidemia of the blood plasma and lipemia retinalis was noted.
Anterior ischemic optic neuropathy (AION) occurs when there is compromised blood supply to the optic nerve and can result in visual loss. Ophthalmic nurses are key health professionals to recognize this entity and triage the patient accordingly. A case study of a patient with non-arteritic AION is presented.
PURPOSE/METHODS: We reviewed follow-up data on 32 patients with orbital rhabdomyosarcoma, who were treated with external beam radiation with and without adjunctive chemotherapy between 1960 and 1992. RESULTS/CONCLUSIONS: Patients were followed up from five to eight years. Of the 32 patients, six (19%) had best-corrected visual acuity of 20/70 or better; five patients (15%) had best-corrected visual acuity of light perception to 20/300; 21 patients (66%) had no light perception. The reported long-term visual acuities should be factored in the equation for selecting treatment for patients with orbital rhabdomyosarcoma.
PURPOSE: This report examines the incidence and outcome of trilateral retinoblastoma in children treated for retinoblastoma. A group of patients who are at highest risk for the development of trilateral retinoblastoma is defined. METHODS AND MATERIALS: Between 1979 and 1990, 117 children were treated with external beam radiation therapy for retinoblastoma, (97/117, bilateral). Median follow-up time was 68 months. The median age at diagnosis was 7 months. RESULTS: Six cases of trilateral retinoblastoma were identified. The incidence of trilateral retinoblastoma in children with bilateral retinoblastoma was 6% (6/97) and 10% in those with a family history of retinoblastoma. The median age at diagnosis of RB in the children with trilateral retinoblastoma, was 3 months, younger than the median age of the entire retinoblastoma group. In all cases, the pineal region was excluded from the radiotherapy fields. Treatment for the trilateral retinoblastoma consisted of craniospinal axis radiation therapy and chemotherapy in three patients, chemotherapy alone in two, and no treatment in one. All patients died from this disease. Overall, of the 117 children treated at our institution for retinoblastoma with a median follow-up of 68 months, 12 have died. Trilateral retinoblastoma was the major cause of death, accounting for 50% (6/12) of deaths. CONCLUSION: Trilateral retinoblastoma is a major and under-appreciated cause of mortality in the first 5 years after the diagnosis of bilateral retinoblastoma. A more aggressive approach toward screening a defined population of childhood retinoblastoma survivors may be warranted.
A retrospective chart review of 427 eyes diagnosed with unilateral retinoblastoma was performed to determine which eyes, which patients, and when new intraocular tumours would develop after treatment. Mean follow up was 8.16 years. Twenty five (6%) of 427 unilateral retinoblastoma patients developed new intraocular tumours after treatment. Five (1%) unilateral patients who were previously treated with enucleation developed new tumours (in the fellow eye). Fifteen (24%) unilateral patients who were previously treated with external beam radiation developed new tumours (equally in either eye). New tumours did not develop in the macula of either eye. The relative risk of developing new intraocular tumours after treatment was 16% in patients diagnosed before 1 year old and 2.2% for patients diagnosed after 1 year old (p < 0.001). The mean time to onset for the development of new tumours after treatment was 0.74 years; no new tumours appeared after 7.5 years of age. Those patients who are diagnosed with unilateral retinoblastoma in the first 6 months of life and have a family history of the disease are at greatest risk of developing new intraocular tumours.
Retinoblastoma is the most common primary intraocular tumor in children. Retinoblastoma can be hereditary (familial) or nonhereditary (nonfamilial). Nurses who have an understanding of the genetic patterns for retinoblastoma can participate in the counseling of these patients. A chart is provided as a tool for teaching patients about family patterns of retinoblastoma.
BACKGROUND: Children diagnosed with retinoblastoma, a rare cancer of the eye, tend to develop and die of second primary cancers in childhood and adolescence, but few investigations have followed patients into adulthood. Retinoblastoma is frequently caused by inherited mutations of the RB1 tumor suppressor gene. Most patients with germline (hereditary) mutations have bilateral disease. PURPOSE: We sought to quantify the mortality from second malignancies among long-term survivors of retinoblastoma and to identify factors that predispose to these deaths. METHODS: A retrospective cohort study examined mortality among 1603 patients enrolled at 1 year after diagnosis of retinoblastoma during the period 1914-1984. Data on demography, family history, and retinoblastoma treatment were collected by medical chart review and questionnaire interview. Number of deaths, by cause, was compared with the corresponding expected figure based on U.S. mortality data for the general population for 1925-1990. RESULTS: Follow-up was complete for 1458 patients (91%) for a median of 17 years after retinoblastoma diagnosis. A total of 305 deaths occurred, 167 of them from retinoblastoma. There were 96 deaths from second primary tumors (relative risk [RR] = 30), 21 from other known causes (RR = 1.0), and 21 from ill-defined or unknown causes. Statistically significant excess mortality was found for second primary cancers of bone, connective tissue, and malignant melanoma and benign and malignant neoplasms of brain and meninges. Among 919 children with bilateral retinoblastoma, 90 deaths from second primary tumors occurred (RR = 60). Deaths from second tumors were more frequent among females (RR = 39) than males (RR = 22) (P = .007). The cumulative probability of death from second primary neoplasms was 26% at 40 years after bilateral retinoblastoma diagnosis, and additional cancer deaths occurred thereafter. Radiotherapy for retinoblastoma further increased the risk of mortality from second neoplasms. An excess of mortality from a second cancer, not seen in prior studies, was found among the 684 children with unilateral disease (RR = 3.1; 95% confidence interval = 1.0-7.3). CONCLUSIONS: These findings implicate germinal mutations in the retinoblastoma gene in second cancer mortality. Radiotherapy treatment for retinoblastoma appears to further enhance the inborn susceptibility to development of a second cancer. IMPLICATIONS: Patients with retinoblastoma, particularly bilateral retinoblastoma, should have careful follow-up, and interventions should be developed to reduce mortality from a second cancer.
BACKGROUND: The increase in intraocular pressure in the human eye that is associated with the use of intravenous succinylcholine has long been ascribed to contraction of the extraocular muscles leading to compression of the globe. This created concern that such contraction would extrude global contents in the patient with an open globe, and led clinicians to avoid the use of succinylcholine in these patients. METHODS: The authors studied 15 patients undergoing elective enucleation, and compared the intraocular pressure change after the administration of succinylcholine in the diseased eye after all the extraocular muscles had been detached to that of the normal eye that had the extraocular muscles attached. RESULTS: The authors found no difference in baseline intraocular pressure (mmHg) between eyes (15.1 vs. 16.1) or at peak intraocular pressure (25.2 vs. 24.7), but did observe a significant difference in pressure in both eyes when baseline was compared with peak pressure. CONCLUSIONS: The authors concluded that extraocular muscle contraction does not contribute to the increase in intraocular pressure after succinylcholine.
Aqueous humor from children with retinoblastoma obtained at enucleation and from eyes with adult cataracts were assayed with electrochemical liquid chromatography (Model 5500 Coulochem electrode array system) for metabolites of tyrosine, tryptophan metabolic pathways, catecholamine degradation pathways and ascorbate. More than 20 metabolites were identified in human aqueous for the first time. High levels of ascorbate were found in aqueous of eyes with adult cataracts (254, 336 ng/ml). Tyrosine metabolism in both sets of eyes was through dopamine. Vandylmandelic acid (VMA), homovanillic acid (HVA), and 3-methoxy, 4-hydroxyphenylglycol (MHPG) were all detected in retinoblastoma eyes. Although eyes with either adult cataracts or childhood retinoblastoma convert tryptophan through the serotonin pathway, retinoblastoma eyes metabolize tryptophan through the kynurenine pathway to a greater degree than adult cataract eyes.
Retinoblastoma is the most common primary intraocular tumor of childhood. The most common presenting sign of retinoblastoma is leukocoria; the second most common presenting sign is strabismus. The optometrist's ability to recognize the presenting signs and symptoms of retinoblastoma can lead to early diagnosis and expeditious treatment. Treatment modalities include enucleation, radiotherapy, cryotherapy, photocoagulation, and chemotherapy. Goals of treatment include patient survival and preservation of vision. Cooperation of the optometrist, the ophthalmologist, the ophthalmic nurse, the pediatric oncologist, and the radiation therapist plays an important role in the long-term survival of these children.