Optical coherence tomography in the evaluation of neurofibromatosis type 1 subjects with optic pathway gliomas

Abstract

Introduction: This is a retrospective study of adult strabismus patients to determine their pre-and postoperative binocular status and possible effects of surgery. Methods: A list of all consecutive adult patients who underwent surgery (one surgeon) for strabismus between June 1990 and September 2009 was compiled and their medical charts were reviewed. Patients who had stereo acuity, measured by the Titmus test, recorded both preoperatively and postoperatively, were included. A total of 179 patients underwent surgery for strabismus. Of these, 120 patients, aged 16 to 80 years, were included; 30 were excluded as their charts did not contain information on stereo acuity at appropriate dates, and 29 charts were unavailable. Prism management was incorporated pre-and/or postoperatively in some of the patients. Results: Overall, 63 of the 120 patients improved in binocular function (52.5%), 56 remained the same (46.67%), and 1 decreased (0.83%). If you exclude those patients with 40 seconds of stereo acuity preoperatively, whose stereopsis, by definition, could not improve further (N 5 17), and look only at the 103 surgical patients who could improve, then 62% improved. Conclusions: The benefits of surgical correction of strabismus in adults include improvement in binocular function as seen in 62% of the patients in the study.

Optical Coherence Tomography in the Diagnosis of Optic Pathway Gliomas

“…At least two high-speed peripapillary RNFL circle scans (circle scan size, 3.5 mm) were obtained. 4 Depending on the quality of the scan and correct scan position around the optic nerve, a single RNFL image was chosen for analysis. Peripapillary RNFL thickness (lm) measurements were automatically calculated by SD-OCT software, providing a global average (G) and average thickness for each of six sectors: temporal (T), temporal-superior (TS), temporalinferior (TI), nasal (N), nasal-superior (NS), and nasal-inferior (NI) (Figs.…”

“…1 Approximately 65% of these cases are detected in young children (less than 5 years of age), and one-third to one-half of these patients develop progressive disease. 4,5 OPGs show a highly variable and unpredictable growth pattern, ranging from indolent to rapidly progressive tumors, and may lead to visual loss, neurologic sequelae (hemiparesis, ataxia), hydrocephalus, macrocephaly, systemic signs (developmental delay, failure to thrive, diencephalic syndrome), and death. 1,4 Children with symptomatic OPG may have relevant ophthalmological abnormalities at the time of diagnosis (including marked visual impairment, abnormal pupillary function, optic nerve atrophy, and/or proptosis) due to early onset of the disease and related diagnostic difficulties.…”

“…4,5 OPGs show a highly variable and unpredictable growth pattern, ranging from indolent to rapidly progressive tumors, and may lead to visual loss, neurologic sequelae (hemiparesis, ataxia), hydrocephalus, macrocephaly, systemic signs (developmental delay, failure to thrive, diencephalic syndrome), and death. 1,4 Children with symptomatic OPG may have relevant ophthalmological abnormalities at the time of diagnosis (including marked visual impairment, abnormal pupillary function, optic nerve atrophy, and/or proptosis) due to early onset of the disease and related diagnostic difficulties. 1,4 Decreased visual acuity is considered the most clinically relevant indicator of OPGs, whereas serial visual acuity measurement is the best method of follow-up of affected patients.…”

“…1,4 Children with symptomatic OPG may have relevant ophthalmological abnormalities at the time of diagnosis (including marked visual impairment, abnormal pupillary function, optic nerve atrophy, and/or proptosis) due to early onset of the disease and related diagnostic difficulties. 1,4 Decreased visual acuity is considered the most clinically relevant indicator of OPGs, whereas serial visual acuity measurement is the best method of follow-up of affected patients. 1 Unfortunately, a large proportion of NF-1 children have moderate to severe impairment in one or more areas of cognitive function, mainly sustained attention difficulties, reducing the positive predictive value of a visual function examination (as well as any other functional test needing patients' collaboration).…”

“…1 Unfortunately, a large proportion of NF-1 children have moderate to severe impairment in one or more areas of cognitive function, mainly sustained attention difficulties, reducing the positive predictive value of a visual function examination (as well as any other functional test needing patients' collaboration). 4,6 Moreover, tests that do not require patients' collaboration (e.g., pupillary reflex or optic disc evaluation [ODE]) allow only advanced cases to be theoretically detected because of the low sensitivity of the tests themselves. 1,[4][5][6] This study compared visual function assessment (VFA), ODE by indirect ophthalmoscopy, and retinal nerve fiber layer (RNFL) analysis by optical coherence tomography (OCT) as the diagnostic tools for OPG in pediatric patients (2-15 years of age) affected by NF-1.…”