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Various Syndromes with Benign Intraocular Tumors

  • Mahesh Shanmugam Palanivelu
  • Pradeep Sagar
Chapter

Abstract

A selected group of benign intraocular tumors manifest as a part of neurocutaneous syndromes which are termed as phakomatoses. Most of the syndromes are caused by an underlying genetic defect that is responsible for multiple lesions involving various organ systems. Some ocular tumors are benign, asymptomatic, and serve as an indicator to the underlying disease such as astrocytic hamartoma associated with tuberous sclerosis complex. Some tumors can be locally destructive resulting in loss of vision as in von-Hippel Lindau and Sturge-Weber syndrome. Few ocular lesions may need observation alone and a few require active treatment with various modalities such as laser photocoagulation, photodynamic therapy, radiotherapy or vitreo-retinal surgery. Identification of the ocular disease would prompt periodic screening for systemic involvement to decrease morbidity and mortality associated with these syndromes.

Keywords

Phakomatoses von-Hippel Lindau syndrome Wyburn-Mason syndrome Eye Sturge Weber syndrome Tuberous sclerosis Neurofibromatosis CHRPE CHRRPE 

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Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  • Mahesh Shanmugam Palanivelu
    • 1
  • Pradeep Sagar
    • 1
  1. 1.Department of Vitreo-Retina and Ocular OncologySankara Eye HospitalBangaloreIndia

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