Predicting the early visual outcomes in sellar-suprasellar lesions compressing the chiasm: the role of SD-OCT series of 20 patients operated via endoscopic endonasal approach.

BACKGROUND: Tumors involving the sellar area often cause visual disorders due to optic nerves/chiasm compression, so that surgery is required. However, the likelihood of visual improvement/restoration is variable. Optical coherence tomography (OCT) is a valid diagnostic tool for defining pathological conditions of the optic apparatus. We herein investigated the role of the SD-OCT and the best corrected visual acuity in predicting postoperative visual recovery, in patients complaining of chiasm compression due to sellar-suprasellar lesions. METHODS: We retrospectively analyzed 20 patients (7 females and 13 males; mean age 50.8±17.87 years, range 11-83), with optic chiasm/nerve compression that underwent endoscopic endonasal approach at the Division of Neurosurgery of the University of Naples Federico II, Naples, Italy, between June 2014 and February 2015. Preoperative ophthalmological assessment focused on BCVA Snellen charts, standard automated perimetry test and SD-OCT. Test were repeated at two weeks, one and three months after surgery. RESULTS: A significant thickness reduction in all the patients, as compared to the control group was noted: GCC thickness reduction remained mostly unchanged postoperatively: 42.9% of the patients had altered visual acuity (VA), while the Perimetry revealed 90% of altered preoperative MD values (MD>-2.00): perimetry improvement was noted in 93% of cases and visual acuity in 91.3% of cases. A direct correlation between preoperative retinal status and functional recovery was identified considering the values of pRNFL and GCC. CONCLUSIONS: OCT provides valuable information regarding the entity of compression also in patients without any visual field defects. Although several factors should be taken into consideration, we retain that preoperative RNFL and GCC thickness values can be claimed as a reliable predictor of visual recovery in those patients presenting chiasm compression.

Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: assessing cardiac effects of long-term enzyme replacement therapy.

PURPOSE: Anderson-Fabry disease is a multisystemic disorder of lipid metabolism secondary to X-chromosome alterations and is frequently associated with cardiac manifestations such as left ventricular (LV) hypertrophy, gradually leading to an alteration in cardiac performance. The purpose of this study was to monitor, using magnetic resonance imaging (MRI), any changes produced by enzyme replacement therapy with agalsidase beta at the cardiac level in patients with Anderson-Fabry disease. MATERIALS AND METHODS: Sixteen (ten men, six women) patients with genetically confirmed Anderson-Fabry disease underwent cardiac MRI before starting enzyme replacement therapy (baseline study) and after 48 months of treatment with agalsidase beta at the dose of 1 mg/kg (follow-up study). RESULTS: After 48 months of treatment, a significant reduction in LV mass and wall thickness was observed: 187±59 g vs. 149±44 g, and 16±3 mm vs. 13±3 mm, respectively. A significant reduction in T2 relaxation time was noted at the level of the interventricular septum (81±3 ms vs. 67±7 ms), at the apical level (80±8 ms vs. 63±6 ms) and at the level of the lateral wall (82±8 ms vs. 63±10 ms) (p<0.05). No significant variation was observed in ejection fraction between the two studies (65±3% vs. 64±2%; p>0.05) (mean bias 1.0); however, an improvement was noted in the New York Heart Association (NYHA) class of the majority of patients (12/16) (p<0.05). CONCLUSIONS: In patients with Anderson-Fabry disease undergoing enzyme replacement therapy with agalsidase beta, MRI documented a significant reduction in myocardial T2 relaxation time, a significant decrease in maximal myocardial thickness and in total LV mass. MRI did not reveal significant improvements in LV global systolic function; however, improvement in NYHA functional class was noted, consistent with improved diastolic function.