Glaucoma in Axenfeld–Rieger Syndrome. A Clinical Case

Abstract

Background: AxenfeldRieger syndrome is a genetically heterogeneous group of morphogenesis disorders associated with abnormal development of the anterior segment of the eye, teeth, the organ of hearing, and abdominal region. Сongenital glaucoma, which is refractory to standard methods of treatment due to pronounced changes in the structures of the anterior segment of the eyeball and other comorbidities, is frequent manifestation of this syndrome. There are several methods for treating glaucoma in patients with AxenfeldRieger syndrome: sinus trabeculectomy, implantation of drainage devices and transscleral cyclocoagulation. Сlinical case description: A patient with congenital glaucoma associated with AxenfeldRieger syndrome underwent sinus trabeculectomy with posterior scleral trepanation in the right eye and valve drainage implantation in the left eye at the S. Fyodorov Eye Microsurgery Federal State Institution, Moscow due to the intraocular pressure decompensation. In the postoperative period, an encapsulated cyst around the body of the drainage was detected, and then a revision of the operation area was performed. After the anti-glaucoma operations, the intraocular pressure compensation was achieved in the follow-up period up to 9 months. Conclusion: Depending on the degree of the changes in the anterior chamber angle structures, sinus trabeculectomy or valve drainage implantation are the methods of choice for the surgical treatment aimed at the intraocular pressure compensation and visual function preservation in patients with Axenfeld-Rieger syndrome.