Neurofibromatosis (von Recklinghausen's disease) involves the eye in a great variety of clinical appearances. Secondary glaucoma with congenital ectropion uveae and mild buphthalmos in one eye and non-pigmented nodules in the iris of both eyes were observed in a 9-year-old girl with generalized neurofibromatosis. The patient also exhibited pigmented patches (cafe-au-lait spots) and multiple nodular masses of the skin. Two of the masses were excised and vitrified as plexiform neurofibroma, microscopically. This paper reports clinical, biomicroscopic, ophthalmoscopic, gonioscopic and histological findings in the patient.
Child ; Ectropion* ; Female ; Glaucoma* ; Humans ; Hydrophthalmos* ; Iris* ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Skin ; Uvea*

In this study, 55 patients (104 eyes) were studied in order to assess tear drainage, using a computer assisted gamma camera. Twentynine eyes had DCR, 29 eyes had CDCR with Jones' tube, and remaining 46 eyes were considered controls. We measured T1/2 values, the time in which a given amount of tracer drops to half in the conjunctival sac area. The time values were classified into 4 groups; Group I (3 min or less), Group II (3-6min), Group III (6-9min) and Group IV (9min or longer). In the patients with DCR and CDCR, tear drainage was faster than in the control group. Age was considered as a factor of delayed tear flow in those patients with DCR and controls, however, it was not a relevant factor in those patients with CDCR using Jones' tube.
Dacryocystorhinostomy* ; Drainage ; Gamma Cameras ; Humans ; Radionuclide Imaging* ; Tears

Background: Brentuximab vedotin (BV), a potent antibody-drug conjugate, targets the CD30 antigen.In Korea, BV has been approved for the treatment of relapsed or refractory Hodgkin lymphoma (HL), anaplastic large-cell lymphoma (ALCL), and cutaneous T-cell lymphomas, including mycosis fungoides (MF). However, there are limited data reflecting real-world experiences with BV treatment for HL, ALCL, and MF. Methods: This was a multicenter, non-interventional registry study of the efficacy and safety of BV in patients with relapsed or refractory CD30-positive lymphoma (CISL1803/BRAVO).Outcomes were determined based on the occurrence of relapse or progression and overall survival after BV treatment. Results: A total of 85 patients were enrolled in this study. The median number of BV cycles was 10 (range, 2‒16) in the patients with HL. The objective response rate (ORR) of patients with HL to BV was 85.4% (41/48), comprising 27 complete responses (CRs) and 14 partial responses (PRs). The ORR of ALCL was 88% (22/25), consisting of 17 CRs and five PRs, whereas the ORR of MF was 92% (11/12). At the median follow-up of 44.6 months after BV treatment, the median post-BV progression-free survival of HL, ALCL, and MF patients was 23.6 months, 29.0 months, and 16.7 months, respectively (P =0.641). The most common side effect of BV was peripheral neuropathy; 22 patients (25.9%, 22/85) experienced peripheral neuropathy (all grades). Conclusion The treatment outcomes of patients with relapsed or refractory CD30-positive lymphoma improved with BV treatment, and the safety profile was manageable.