Predictive value of OCT and MRI for postoperative visual recovery in patients with chiasmal compressive lesions

Purpose We aimed to investigate the predictive value of retinal thickness measured by optical coherence tomography (OCT) and mass biometrics measured using magnetic resonance image (MRI) for visual recovery after surgery for removal of a mass compressing the optic chiasm. Methods Consecutive patients who showed typical temporal visual field defect (VFD) with respect to the vertical meridian due to a chiasmal compressive mass and who underwent mass removal surgery were recruited. Ophthalmic examination was performed preoperatively and postoperatively. Retinal thickness was measured by the Cirrus OCT. The height and size of the mass and suprasellar extension (SSE) in both the sagittal and coronal planes were evaluated. Patients were divided into two groups based on the improvement in VFD (mean deviation [MD] change ≥ 5 dB: group R; others: group NR) and clinical characteristics were compared. Results Fifteen patients were included in the study. Eight (53.3%) patients were allocated into group R and others (7 patients, 46.7%) into group NR. Age, sex, initial visual acuity, initial MD was not different between the two groups. The retinal thicknesses were not different while tumor height, volume, and both sagittal and coronal SSE were significantly different between the two groups. (p = 0.029, 0.014, <0.001, and <0.001, respectively) All MRI parameters showed significant predictive value for the degree of MD recovery. Conclusion MRI showed better predictive value than OCT in predicting postoperative VFD recovery in patients with temporal VFDs due to chiasmal compressive disorder.

Rapidly Progressive Pituitary Apoplexy in a Patient with COVID-19 Disease Treated with Endoscopic Endonasal Surgery

This report describes a case of pituitary apoplexy with rapidly evolving hemorrhage in a 74-year-old female with coronavirus disease 2019 (COVID-19) disease. The patient presented with severe headache and mild respiratory symptoms, with laboratories concerning for pituitary hypofunction. Brain imaging demonstrated a sellar mass concerning for a pituitary adenoma with ischemic apoplexy. She subsequently developed visual deficits within 24 hours of presentation, and repeat imaging demonstrated evolving hemorrhage and new mass effect on the optic chiasm. She was successfully managed with urgent endoscopic endonasal surgery despite her COVID-19 positive status by taking special intraoperative precautions to mitigate SARS-CoV2 transmission risk. Only a handful of cases of pituitary apoplexy have been reported in association with COVID-19 disease, and even fewer reports exist of endonasal procedures in such cases. We discuss the potential implication of COVID-19 in the occurrence of pituitary apoplexy, in addition to the safety and success of endonasal surgery in this population.

The change of microglia numbers within the mice retina, optic nerve and chiasm following intravitreal AAV2-GFP injection

Purpose To explore the optimized concentration of AAV2-GFP for sparse transfection of retinal ganglion cells (RGCs) and optic nerve (ON), and to examine the changes of microglial morphology and distribution in the retina, optic nerve and chiasm after injection. Methods We defined the optimal concentration of AAV2-GFP for sparse labeling of RGCs and axons in WT mice. We further explored the changes of microglial morphology and distribution in the retina, optic nerve and chiasm after intravitreal injection in CX3CR1+/GFP mice. Results 14 days after intravitreal injection of AAV2-GFP, live imaging of the retina showed that fundus fluorescence was very strong and dense at 2.16 × 1011 VG/retina, 2.16 × 1010 VG/retina, 2.16 × 109 VG/retina. RGCs were sparsely marked at a concentration 1:1000 (2.16 × 108 VG/retina) and fundus fluorescence was weak. The transfected RGCs and axons were unevenly distributed in the retina and significantly more RGCs were transfected near the injection site of AAV2-GFP compared to the other sites of the flat-mounted retina. Microglia density increased significantly in the retina and part of optic nerve, but not in the optic chiasm. The morphology of microglia was largely unchanged. Conclusions AAV2-GFP was highly efficient and the optimal concentration of sparsely labeled RGCs was 1:1000 (2.16 × 108 VG/retina). After intravitreal injection of AAV2-GFP, the number of microglia increased partly. The morphology of microglia was comparable.

Visual disturbance as primary symptom of pituitary apoplexy in pregnancy

Pituitary apoplexy in pregnancy is a potentially fatal condition caused by acute ischaemic infarction or haemorrhage of pre-existing pituitary adenoma or within a physiologically enlarged pituitary gland. It has a wide spectrum of clinical presentations ranging from a mild headache to sudden collapsed. Here, we report a life-threatening case of pituitary apoplexy in a non-functioning pituitary macroadenoma occurring during pregnancy that presented with bilateral blurring of vision. Visual field showed bitemporal superior quadrantanopia. Urgent non-contrast brain MRI revealed an acute expansion of a hemorrhagic pituitary lesion complicated with local compression to the optic chiasm. The patient underwent an uneventful right supraorbital craniotomy and excision of the tumour under general anaesthesia with no foetal loss. The repeated visual field at 2 weeks after surgery showed recovering visual field defect. Hence, early neurosurgical intervention is advisable to prevent mortality and morbidity due to permanent visual field loss.

Intraoperative Rupture of an Intracranial, Extradural Hydatid Cyst: Case Report and Treatment Options

A 23-year-old woman was presented to the Emergency Unit with intracranial hypertension syndrome and blindness in her left eye which had started recently. A cranial native computed tomography scan and a magnetic resonance imaging (MRI) with contrast examinations revealed a giant intracranial cystic lesion, extending into the left frontal lobe, which was compressing the optic chiasm and eroding the internal plate of the left frontal bone. Surgical craniotomy was performed for evacuation and decompression, but during the craniotomy the cyst ruptured. After assessing the degree of erosion of the internal bone plate, we concluded that the primary origin of the cyst was intraosseous. With the dura mater being intact, abundant lavage with H2O2 was applied and the bone flap was replaced after rigorous bone scraping. Imaging control at six and twelve months identified no recurrence of the cyst. In the literature, hydatid cysts located in the skull bone are very rare and most of them rupture intraoperatively. Given their extremely low incidence in developed countries, any neurosurgeons’ experience with such pathology is limited and in some cases surgery cannot be delayed. In the case of intracerebral hydatid cysts, a neurosurgeon usually has only one shot at surgery, so simple and quick-to-access therapeutic guidelines must be developed in order to inform the choice of surgical technique. We conclude that the most successful surgical approach could be double concentric craniotomy. This surgical technique is used in intracerebral tumors, which also have an important bone invasion.

CTNI-53. RADIATION TREATMENT VOLUMES BEFORE AND AFTER BRAF/MEK THERAPY IN NEWLY DIAGNOSED PAPILLARY CRANIOPHARYNGIOMAS: A CORRELATIVE ANALYSIS OF THE ALLIANCE A071601 PHASE II TRIAL

PURPOSE Standard of care for craniopharyngiomas is surgery with or without radiotherapy (RT). Cohort A of Alliance A071601 evaluated the efficacy of BRAF/MEK inhibition with vemurafenib/cobimetinib in patients with previously untreated papillary craniopharyngiomas (PCP), which carry the BRAF V600E mutation. Cohort B is currently enrolling patients with recurrence after RT. In a correlative analysis, we examined changes in RT volumes after BRAF/MEK therapy in Cohort A. METHODS Previously unirradiated patients with BRAF-mutated PCP were treated with vemurafenib/cobimetinib. Sixteen patients had scans available before starting vemurafenib/cobimetinib (“pre-therapy”) and after completing therapy (“post-therapy”). Two patients went off study treatment after 8 and 9 days due to side-effects and were excluded for this analysis. Gross target volumes (GTV) were contoured on pre-therapy and post-therapy scans. On post-therapy scans, an additional target comprising gross disease and at-risk regions for microscopic residual disease (GTV-micro) was defined and considered the treatment volume. Clinical target volume (CTV) was a 5-mm uniform expansion on pre-therapy GTV and post-therapy GTV-micro. Volumes were independently reviewed by two radiation oncologists. Changes in volumes from pre- versus post-therapy were compared using the Wilcoxon signed rank test. RESULTS In 14 patients evaluated, 57% were female and median age at enrollment was 49.5 years (range 33-83). Median time on treatment was 8.9 months (range 4.0-18.0). Median GTV pre-therapy was 3.8 mL (range 0.2-23.4) versus 0.3 mL (range 0.0-3.2) post-therapy (p=0.0001) and 1.7 mL (range 0.1-8.0) post-therapy GTV-micro (p=0.0001). Median CTV pre-therapy was 13.7 mL (range 2.8-51.8) versus 9.1 mL (range 2.2-27.5) post-therapy (p=0.0001). All tumors abutted the optic chiasm pre-therapy, only 6 did post-therapy. CONCLUSIONS Vemurafenib/cobimetinib resulted in smaller RT volumes. BRAF/MEK inhibitors could reduce RT volumes and spare dose to surrounding normal structures. Enrollment to Cohort B of Alliance A071601 should be considered for patients with recurrent tumors after RT. SUPPORT https://acknowledgments.alliancefound.org

Machine Learning-Based Radiomics of the Optic Chiasm Predict Visual Outcome Following Pituitary Adenoma Surgery

Preoperative prediction of visual recovery after pituitary adenoma surgery remains a challenge. We aimed to investigate the value of MRI-based radiomics of the optic chiasm in predicting postoperative visual field outcome using machine learning technology. A total of 131 pituitary adenoma patients were retrospectively enrolled and divided into the recovery group (N = 79) and the non-recovery group (N = 52) according to visual field outcome following surgical chiasmal decompression. Radiomic features were extracted from the optic chiasm on preoperative coronal T2-weighted imaging. Least absolute shrinkage and selection operator regression were first used to select optimal features. Then, three machine learning algorithms were employed to develop radiomic models to predict visual recovery, including support vector machine (SVM), random forest and linear discriminant analysis. The prognostic performances of models were evaluated via five-fold cross-validation. The results showed that radiomic models using different machine learning algorithms all achieved area under the curve (AUC) over 0.750. The SVM-based model represented the best predictive performance for visual field recovery, with the highest AUC of 0.824. In conclusion, machine learning-based radiomics of the optic chiasm on routine MR imaging could potentially serve as a novel approach to preoperatively predict visual recovery and allow personalized counseling for individual pituitary adenoma patients.

Molecular Profile of a Pituitary Rhabdomyosarcoma Arising From a Pituitary Macroadenoma: A Case Report

Pituitary sarcoma arising in association with pituitary adenoma is an uncommon finding. Most cases of secondary sarcoma have been noted to arise with a median interval of 10.5 years post radiation. In this case report, we describe a 77-year-old man with an incidental discovery of a pituitary macroadenoma on magnetic resonance imaging (MRI) and underwent radiotherapy. Three years after radiation treatment, there was an acute change in clinical symptoms and increase in tumor size and mass effect on the optic chiasm which prompted surgical resection. A pituitary adenoma along with a separate spindle-cell sarcomatous component was identified in histology. Immunohistochemical stain for muscle markers confirmed a development of pituitary rhabdomyosarcoma (RMS). Molecular profiling of the tumor identified mutations in TP53, ATRX, LZTR1, and NF1. Despite its rarity, characterization of pituitary RMS with immunohistochemistry and molecular studies may provide an insight to its pathophysiological relationship with pituitary adenoma.