Ab interno Schlemm's Canal Surgery.

In primary open-angle glaucoma, the site of greatest resistance to aqueous outflow is thought to be the trabecular meshwork (TM) and inner wall of Schlemm's canal. Augmentation of the conventional (trabecular) outflow pathway can facilitate physiologic outflow and subsequently lower intraocular pressure. The most recent approach to enhancing the conventional outflow pathway is via an internal approach to the TM and Schlemm's canal. Ab interno Schlemm's canal surgery includes 4 novel surgical approaches: (1) removal of the TM and inner wall of Schlemm's canal by an internal approach (ab interno trabeculectomy), (2) implantation of a microstent to bypass the TM, (3) disruption of the TM and inner wall of Schlemm's canal via an internal approach (ab interno trabeculotomy), and (4) dilation of Schlemm's canal via an internal approach (ab interno canaloplasty). The first category includes the Trabectome (Neomedix, Tustin, CA, USA), and Kahook Dual Blade (New World Medical, Rancho Cucamonga, CA, USA). The second category includes the iStent (Glaukos, Laguna Hills, CA, USA), as well as the investigational Hydrus Microstent implant (Ivantis, Irvine, CA, USA). The third category includes gonioscopic-assisted transluminal trabeculotomy (iSciences catheter; Ellex, Adelaide, Australia), and 360° suture trabeculotomy (TRAB360, Sight Sciences, Menlo Park, CA, USA). The fourth category includes ab interno canaloplasty or AbiC (Ellex), and Visco360 (Sight Sciences). In contrast to external filtration surgeries, such as trabeculectomy and aqueous tube shunt, these procedures are categorized as internal filtration surgeries and are performed from an internal approach via gonioscopic guidance. Published results suggest that these surgical procedures are both safe and efficacious for the treatment of open-angle glaucoma.

Spheroidal degeneration in H626R TGFBI variant lattice dystrophy: a multimodality analysis.

PURPOSE: The aim of this study was to describe clinical, imaging, molecular genetic, histopathologic, immunohistochemical, and ultrastructural characteristics of coexistent amyloid and spheroidal degeneration-type deposits in a family with histidine-626-arginine transforming growth factor beta-induced (H626R TGFBI) variant lattice dystrophy. METHODS: This is a retrospective clinical-pathological and genetic analysis of one family with H626R variant lattice dystrophy. RESULTS: Pedigree analysis showed an autosomal dominant inheritance pattern of the disease. Examination of 3 affected family members revealed asymmetric, thick, branching lattice-like deposits associated with corneal haze. Sequencing of the TGFBI gene revealed a high-penetrance disease-causing sequence variation (H626R CAT>CGT heterozygous). Optical coherence tomography demonstrated fusiform, poorly demarcated hyperechoic stromal deposits with focal hypoechoic central regions. Histology of the corneal discs from 2 affected family members showed stromal deposits consistent with TGFBI amyloid. Some amyloid deposits contained a central nidus of spheroidal degeneration-type material that demonstrated autofluorescence, stained with elastic and Masson trichrome stains, did not stain with periodic acid-Schiff or Congo red stains, was nonbirefringent, and did not immunoreact with keratoepithelin antibodies. Transmission electron microscopy confirmed the presence of amyloid fibrils with central, electrodense, homogeneous, discrete, spheroidal degeneration-type deposits. CONCLUSIONS: The presence of spheroidal deposits in a subset of affected patients, variability in presentation within an individual and between family members, predominant anterior corneal stromal location and nonimmunoreactivity of deposits for keratoepithelin suggest that these deposits are degenerative in nature. The deposits may arise from ultraviolet light-altered proteins diffused from the limbus, which form a nidus for keratoepithelin deposition.