• Users Online: 37
  • Print this page
  • Email this page
CASE REPORT
Year : 2020  |  Volume : 58  |  Issue : 2  |  Page : 109-111

Phakomatosis pigmentovascularis with sturge–Weber syndrome


Department of Glaucoma, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India

Correspondence Address:
Dr. Premanand Chandran
Aravind Eye Hospital, Avinashi Road, Coimbatore - 641 014, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_122_19

Get Permissions

An 11-year-old boy presented with port-wine stain on the right side of the face and pigmentation of the sclera and iris in the left eye (LE). Fundus examination revealed inferior rim excavation of disc with orangish red tomato ketchup appearance of background retina suggestive of diffuse choroidal hemangioma in the right eye (RE) and normal retina in the LE. Ultrasonography showed increased thickness of retinochoroidal scleral complex, and swept-source optical coherence tomography showed choroidal thickening in the RE. He was diagnosed to have phakomatosis pigmentovascularis (PPV) with Sturge–Weber syndrome and glaucoma. PPV is a rare congenital disorder characterized by the presence of capillary malformation and pigmentary nevi. PPV can present with or without systemic involvement. Those without systemic involvement need to be followed up closely as they can manifest later in life.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed68    
    Printed5    
    Emailed0    
    PDF Downloaded27    
    Comments [Add]    

Recommend this journal