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      Failure of XEN Gel Stent Implantation as a Stand-Alone Procedure in Congenital Glaucoma: Case Report of Secondary Congenital Glaucoma in Neurofibromatosis Type 1

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          Abstract

          A XEN gel stent implant procedure was performed in a one-year-old child with severe unilateral congenital glaucoma. At the age of 6 weeks, an uncomplicated 360° trabeculotomy had been performed, which resulted in intraocular pressure (IOP) control for only 4 months. The gel stent implantation was performed ab interno without complications. However, 1 month later, the stent was repelled into the anterior chamber due to the elasticity of Tenon's layer. A first revision surgery was performed, with excision of Tenon's layer and implantation of a new gel stent under sight. At the age of 18 months, a second revision surgery was performed because of an encapsulated Tenon cyst with insufficient IOP control, again with the implantation of a new stent. At that time, a progressive upper eyelid swelling was apparent. Eyelid biopsy led to the diagnosis of neurofibromatosis type 1, presenting with an orbital plexiform neurofibroma. Further insufficient IOP control resulted in a cyclodestructive procedure and loss of light perception during follow-up. XEN gel stent implantation in congenital glaucoma in infants is more challenging than that in adult patients. Gel stent implantation ab interno may be difficult due to the thickness and elasticity of Tenon's layer. Gel stent dislocation may occur, even months after surgery. Trabeculectomy might be a better approach after failed trabeculotomy in congenital glaucoma. An underlying systemic disease might become apparent late during follow-up.

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          Most cited references 14

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          The XEN45 Gel Stent as a minimally invasive procedure in glaucoma surgery: success rates, risk profile, and rates of re-surgery after 261 surgeries

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            von Recklinghausen Neurofibromatosis

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              Options in pediatric glaucoma after angle surgery has failed.

              Congenital glaucoma is primarily a surgical disease with medical management serving as a temporizing measure before surgery or as postoperative adjunctive treatment. First-line surgery for congenital glaucoma consists of incisional procedures on the anterior chamber angle: goniotomy and trabeculotomy. Angle surgery has a high success rate with few complications. Despite the high initial success rate, almost 20% of angle procedures eventually fail, and surgeons are confronted with a choice of what procedure to do next: a trabeculectomy with or without adjunctive antifibrosis therapy, glaucoma drainage surgery, or cyclodestructive procedures. This review will discuss and compare these procedures as reported in recent studies and how variables such as age, number of prior procedures, and type of glaucoma have clarified the order in which these procedures might be performed after failed angle surgery. Clinical reports in refractory pediatric glaucoma consist solely of retrospective studies of varying size and quality. Recent studies of trabeculectomy in this population suggest mitomycin C is associated with increased risk of late infectious complications. Trabeculectomy has worse outcome among younger patients Glaucoma drainage devices have a success rate approaching 80% at 1 year, but less with longer follow-up. Cyclodestructive procedures are generally reserved for advanced cases, but low-dose cyclodiode therapy and endocyclophotocoagulation may prove useful earlier in the disease (< 2 years). Refractory pediatric glaucoma remains a challenge. Glaucoma drainage devices appear to be the most predictable and possibly safest procedure to consider after failed conventional angle surgery.
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                Author and article information

                Contributors
                Journal
                Case Rep Ophthalmol Med
                Case Rep Ophthalmol Med
                CRIOPM
                Case Reports in Ophthalmological Medicine
                Hindawi
                2090-6722
                2090-6730
                2021
                23 July 2021
                : 2021
                Affiliations
                Department of Ophthalmology, Universitätsklinikum Aachen, Pauwelsstr. 30, 52057 Aachen, Germany
                Author notes

                Academic Editor: Pradeep Venkatesh

                Article
                10.1155/2021/9947167
                8325593
                34341693
                Copyright © 2021 Hannah Schellhase et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report

                Ophthalmology & Optometry

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