Abstract:The authors report a case of asymptomatic leukemic optic neuropathy as the first sign of acute lymphoblastic leukemia relapse in a 4-year-old boy. Routine ophthalmologic examination showed normal visual acuity and pupillary function in the presence of a tumoral mass covering the left optic disc. The mass resolved with preservation of vision after intrathecal chemotherapy. A routine ophthalmological examination is recommended for all patients with a history of acute lymphoblastic leukemia to exclude optic nerve… Show more
“…It is well established that the optic nerve can be the site of CNS relapse in leukemia and lymphoma, despite presumed systemic and medullary remission (5,(10)(11)(12). Although most optic neuropathy cases occur in the setting of preexisting CNS disease, there are cases in which leukemic optic neuropathy is the first manifestation of CNS involvement (2)(3)(4)(13)(14)(15)(16). Leukemic infiltration into the optic nerve is postulated to occur through the leptomeninges through infiltration of the perivascular spaces within the pial septa of the nerve (15).…”
Background: Optic neuropathy in the context of leukemia and lymphoma raises concern for central nervous system involvement or relapse and warrants prompt evaluation and treatment. To date, a gold standard for the diagnosis and management of leukemic optic neuropathy has yet to be established. Methods: Case series and review of the literature. Two illustrative cases were selected to discuss their treatment course and outcome. Results: We report 7 cases of patients with leukemia or lymphoma presenting with optic nerve infiltration. All patients received steroid therapy for presumed infiltrative optic neuropathy, and 4 patients underwent radiation therapy. Along with systemic chemotherapy, all patients received intrathecal chemotherapy except one. Three patients received chimeric antigen receptor T-cell therapy. Conclusions: Leukemic and lymphomatous optic neuropathy is difficult to diagnose and treat, and there is no gold standard for diagnosis or treatment in the current literature. We help clarify how this disease should be approached in a multidisciplinary fashion and on an individual basis to correctly diagnose and treat the vision loss, while considering the patient's long-term prognosis based on their systemic disease.
“…It is well established that the optic nerve can be the site of CNS relapse in leukemia and lymphoma, despite presumed systemic and medullary remission (5,(10)(11)(12). Although most optic neuropathy cases occur in the setting of preexisting CNS disease, there are cases in which leukemic optic neuropathy is the first manifestation of CNS involvement (2)(3)(4)(13)(14)(15)(16). Leukemic infiltration into the optic nerve is postulated to occur through the leptomeninges through infiltration of the perivascular spaces within the pial septa of the nerve (15).…”
Background: Optic neuropathy in the context of leukemia and lymphoma raises concern for central nervous system involvement or relapse and warrants prompt evaluation and treatment. To date, a gold standard for the diagnosis and management of leukemic optic neuropathy has yet to be established. Methods: Case series and review of the literature. Two illustrative cases were selected to discuss their treatment course and outcome. Results: We report 7 cases of patients with leukemia or lymphoma presenting with optic nerve infiltration. All patients received steroid therapy for presumed infiltrative optic neuropathy, and 4 patients underwent radiation therapy. Along with systemic chemotherapy, all patients received intrathecal chemotherapy except one. Three patients received chimeric antigen receptor T-cell therapy. Conclusions: Leukemic and lymphomatous optic neuropathy is difficult to diagnose and treat, and there is no gold standard for diagnosis or treatment in the current literature. We help clarify how this disease should be approached in a multidisciplinary fashion and on an individual basis to correctly diagnose and treat the vision loss, while considering the patient's long-term prognosis based on their systemic disease.
“…There are numerous other causes of disc edema and uveitis in the pediatric population. Bilateral disc edema can be caused by intracranial hypertension (primary and secondary causes), 12 , 13 , 14 optic neuritis, 15 leukemic infiltration of the optic nerve, 16 , 17 , 18 and compression by tumors at the sella. 19 Pseudo-papilledema caused by anomalous optic nerves, optic nerve drusen, and retinal nerve fiber layer myelination represents another diagnostic consideration.…”
Purpose
To report an unusual case of incomplete Vogt-Koyanagi-Harada (VKH) in a 14-Year-Old African American female.
Observations
Here we present a 14-Year-Old African American Female with incomplete VKH who presented to the emergency department with a one-month history of malaise, fever, bilateral decreased vision and temporal headaches. At the time of presentation, she was found to have bilateral anterior uveitis and disc edema. The brain and orbit MRI were unremarkable, CSF analysis showed lymphocytic pleocytosis with negative cultures and gram stain. An extensive lab workup was unrevealing.
Conclusion
This is an unusual case of incomplete VKH in a young African American female presenting with anterior uveitis and disc edema without serous retinal detachments. Initiation of high dose oral prednisone promptly after diagnosis resulted in rapid improvement of symptoms and improvement in visual acuity with the resolution of the disc edema. This case highlights the importance of considering VKH in the differential diagnosis of disc edema associated with uveitis, even in the absence of serous retinal detachment.
“…However, leukemic infiltration should be the working diagnosis until proven otherwise. Isolated leukemic optic nerve infiltration has rarely been reported as the initial presentation of disease recurrence 1,2 . Because the optic nerve is a pharmacologic sanctuary site, systemic chemotherapy penetration may be inadequate, making the optic nerve susceptible to recurrence 3 …”
Section: Figurementioning
confidence: 99%
“…Isolated leukemic optic nerve infiltration has rarely been reported as the initial presentation of disease recurrence. 1,2 Because the optic nerve is a pharmacologic sanctuary site, systemic chemotherapy penetration may be inadequate, making the optic nerve susceptible to recurrence. 3 In this case, optic disc infiltration was evident only on ophthalmic examination with no evidence of CNS disease or optic nerve enhancement on MRI.…”
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