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

Randolf A. Widder, Thomas Dietlein, Sven Dinslage … (2018)

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Natural history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study.

Robert Listernick, Joel Charrow, Mark Greenwald … (1994)

To assess the natural history of optic pathway tumors (OPT) in children with neurofibromatosis type 1 (NF-1), from January 1985 through May 1993 we performed a prospective, longitudinal study of OPT in an unselected population of children with NF-1. Of 227 children with NF-1 seen in a specialty clinic, 176 (77%) underwent neuroimaging. Children in whom tumors were identified were followed closely by both repeated neuroimaging and ophthalmologic examinations to detect tumor growth or visual deterioration. Thirty-three children (19%) were found to have OPT at a median age of 4.2 years. The median age of children who had ophthalmologic complaints was significantly lower than that of children who had no such complaints (1.9 vs 5.3 years; p < 0.001). Although eight tumors were discovered because of ophthalmologic complaints or evidence of precocious puberty, 25 children (76%) were free of symptoms at the time of diagnosis. Twenty-one children (64%) had normal ophthalmologic findings at diagnosis; six children, all with chiasmal tumors, had previously unrecognized decreased visual acuity. Only three children (9%) had evidence of either tumor growth or deteriorating vision after diagnosis; the median duration of neuroimaging follow-up was 2.4 years (range, 0.2 to 7.2 years) and of ophthalmologic examinations 3.4 years (range, 0.2 to 8.1 years). All symptomatic OPT were diagnosed before 6 years of age. We conclude that OPT rarely progress during the next few years in children with NF-1 once the OPT have been discovered. The utility of screening neuroimaging for OPT in symptom-free children with NF-1 appears very limited.