Visual Outcomes of Optic Pathway Glioma Treated With Chemotherapy in Neurofibromatosis Type 1.

PURPOSE: To analyze the visual outcomes following chemotherapy for optic pathway glioma in children with neurofibromatosis type 1. METHODS: This was a retrospective cohort study of 58 children with optic pathway glioma between 2003 and 2019. Best corrected visual acuity (BCVA) at diagnosis and at the last follow-up visit were analyzed. Correlation tests were performed to determine whether the final BCVA was predicted by the initial BCVA, initial Dodge stage, or retinal nerve fiber layer (RNFL) thickness stability. The first-line chemotherapy regimen included vincristine plus carboplatin (2003 to 2016) and vinblastine (since 2017). RESULTS: Twenty-four children (41%) received chemotherapy. The median age at diagnosis was 3 years, and 61% of the children were female. From the first visit to the last follow-up visit, there was a statistically significant difference in mean BCVA in eyes in the no treatment group (P = .034) but not in eyes in the chemotherapy group (P = .824). A moderate and weak positive correlation was found (r = 0.58 and r = 0.29, respectively). At the last follow-up visit, BCVA remained stable in 73% and improved in 27% of eyes in the no treatment group, and BCVA worsened in 25%, remained stable in 62%, and improved in 13% of eyes in the chemotherapy group. At the last follow-up visit, BCVA and Dodge stage had a weak negative correlation (r = -0.06 and r = -0.17, respectively). A negative moderate correlation was identified between RNFL thickness and BCVA at the last follow-up visit (r = -0.48 in the no treatment group and r = -0.46 in the chemotherapy group). CONCLUSIONS: Children treated with chemotherapy had worse BCVA, although the treatment arrested the decline or improved BCVA in 75% of the patients with no major side effects. [J Pediatr Ophthalmol Strabismus. 2022;59(2):128-135.].

Optic disc drusen in children: morphologic features using EDI-OCT.

AIMS: This study aimed to investigate morphologic features of optic disc drusen (ODD) and peripapillary hyperreflective ovoid mass-like structures (PHOMS) in children, using enhanced depth imaging optical coherence tomography (EDI-OCT). It also assessed if the presence of these features were associated with decreased peripapillary retinal nerve fibre layer (RNFL) thickness. METHODS: Retrospective observational study of children with ODD. All subjects underwent complete ophthalmic examination and multimodal imaging. ODD were identified on EDI-OCT as circumscribed hyporeflective spheroidal elements located in front of lamina cribrosa, fully or partially surrounded by a hyperreflective border. PHOMS were identified as hyperreflective ovoid structures located in the peripapillary circumference. Both associations between ODD and RNFL loss and PHOMS and RNFL loss were tested using chi-squared test. RESULTS: In total, 38 eyes of 20 children were analysed. PHOMS were present in 90% of patients. ODD and PHOMS were predominantly found in the nasal, superonasal and inferonasal sectors. A significant positive association was found between ODD and decreased RNFL thickness in the nasal (p = 0.02), superonasal (p = 0.05) and inferotemporal (p = 0.04) sectors. There was no significant association found with the presence of PHOMS. CONCLUSION: EDI-OCT allowed morphological analysis of ODD and PHOMS in children. Drusen were found to be distinct from PHOMS both in their appearance and impact on the RNFL. ODD are hyporeflective and appear on the ONH above the lamina cribrosa and were associated with decreased thickness of the RNFL. On the contrary, PHOMS are hyperreflective structures located around the ONH and were not associated with RNFL loss.

Non-Hodgkin lymphoma with relapses in the lacrimal glands.

OBJECTIVE: To report an unusual case of systemic non-Hodgkin lymphoma (NHL) with repeated relapse in the lacrimal glands, in spite of complete remission for several years after treatment. METHODS: A 78-year-old male with small lymphocytic B cell NHL, stage IV disease (lung invasion), was submitted to surgery and chemotherapy in 2001, with complete remission of the disease. In 2003 he developed a nodular lesion in the right lacrimal fossa. Pathology results revealed a local relapse of NHL. Radiation and chemotherapy were initiated and complete remission was again achieved. In 2012 the patient developed a new nodular lesion located in the left lacrimal fossa, resulting in diplopia, ptosis and proptosis of the left eye. Orbital computerized tomography (CT), ocular ultrasound and incisional biopsy were performed. RESULTS: Orbital CT revealed a lesion infiltrating the left lacrimal gland and encircling the globe. Biopsy results confirmed a local relapse of B cell NHL. The patient was submitted to local radiation therapy with progressive resolution of ptosis, proptosis and diplopia. Response to treatment was monitored with ocular ultrasound. CONCLUSIONS: Patients with NHL diagnosis should be immediately investigated if ophthalmic or orbital symptoms develop. NHL extension to the orbit and adnexa is infrequent (5% of NHL cases) but may occur at any stage of the disease, including as a relapse site. In such cases, radiation and chemotherapy achieve good results, inducing long periods of remission.

Lacrimal gland carcinoma ex pleomorphic adenoma with chronic lymphocytic leukemia infiltration.

PURPOSE: To report a rare case of lacrimal gland carcinoma ex pleomorphic adenoma (Ca ex PA) with chronic B-cell lymphocytic leukemia (B-CLL) infiltration in a patient without a previous diagnosis of B-CLL. PATIENT AND METHODS: We report a 66-year-old woman who presented with recent worsening of a long-standing right eye proptosis. Sequential orbital computed tomography imaging was performed over the course of 2 years, and biopsy specimens were analyzed. RESULTS: Initial computed tomography scans revealed a lacrimal gland lesion with stable dimensions for more than 1 year and no malignancy features on incisional biopsy. Subsequently, lesion volume growth and bone erosion were documented on orbital computed tomography. Lateral orbitectomy and lacrimal gland resection were performed. Pathology and immunohistochemistry detected Ca ex PA with B-CLL infiltration. CONCLUSION: This case highlights the importance of persistent investigation of clinically suspicious orbital lesions. To our knowledge, this is the first description of a case of lacrimal gland Ca ex PA with B-CLL infiltration.