[Management of Radius-Maumenee syndrome : Treatment with deep sclerectomy, viscocanalostomy and collagen matrix implantation]

Ophthalmologe. 2016 Sep;113(9):775-8. doi: 10.1007/s00347-015-0213-5.
[Article in German]

Abstract

We describe the case of an 18-year-old female patient who presented with vasodilation of the episcleral vessels in both eyes. The dilated vessels were more prominent in the right eye and, furthermore, examination of the fundus oculi showed a glaucomatous excavation of the right optic disc. No underlying eye or systemic disease was found as the cause for the vasodilation, therefore, Radius-Maumenee syndrome (idiopathic dilated episcleral vessels with secondary open angle glaucoma) was diagnosed. Radius-Maumenee syndrome is a diagnosis by exclusion. If no underlying disease can be detected primary therapy of the glaucoma is required. Carotid cavernous fistulas as the most common cause for dilated episcleral vessels and elevated episcleral venous pressure should be ruled out. Due to the progression of the excavation and the pathologically elevated intraocular pressure in the right eye of our patient we decided to perform a deep sclerectomy in combination with viscocanalostomy and implantation of a collagen matrix. The operation and postoperative period were free from complications. Two months after the surgical procedure the right eye showed a normalized intraocular pressure of 7 mmHg but no morphological changes in terms of reduction of the vasodilation. In summary, deep sclerectomy in combination with viscocanalostomy and implantation of a collagen matrix showed good results in the reduction of intraocular eye pressure in Radius-Maumenee syndrome.

Keywords: Episcleral venous pressure; Glaucoma; Intraocular pressure; Open angle glaucoma; Vasodilation.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Collagen / administration & dosage*
  • Combined Modality Therapy / methods
  • Female
  • Glaucoma, Open-Angle / diagnosis
  • Glaucoma, Open-Angle / therapy*
  • Humans
  • Sclera / surgery*
  • Scleral Diseases / diagnosis
  • Scleral Diseases / therapy*
  • Sclerostomy / methods*
  • Syndrome
  • Treatment Outcome
  • Viscoelastic Substances / administration & dosage*

Substances

  • Viscoelastic Substances
  • Collagen