title: Combined hamartoma of the retina and retinal pigment epithelium in 77 consecutive patients visual outcome based on macular versus extramacular tumor location.
abstract: OBJECTIVE
  To evaluate visual outcome of eyes with combined hamartoma of the retina and retinal pigment epithelium (RPE).
  
  
  METHODS
  Noncomparative case series.
  
  
  METHODS
  Seventy-nine eyes of 77 patients.
  
  
  METHODS
  Retrospective chart review.
  
  
  METHODS
  Visual outcome.
  
  
  RESULTS
  The presenting symptoms were decreased vision (n = 32; 40%), strabismus (n = 22; 28%), both (n = 3; 4%), irritation (n = 4; 5%), and none (n = 18; 23%). The tumors had the following characteristics: a mean diameter of 7.6 mm, a mean thickness of 1.9 mm, round (n = 52; 66%) or curvilinear (n = 27; 34%) configuration, and other features including intralesional corkscrew vessels (n = 51; 65%), feeding straight vessels (n = 50; 63%), retinal traction (n = 64; 81%), fibrosis/gliosis (n = 36; 46%), and exudation (n = 10; 13%). Referring diagnosis was unknown (n = 40; 51%) or incorrect (n = 19; 24%) as retinoblastoma (n = 4), astrocytoma (n = 1), toxocariasis (n = 2), choroidal nevus (n = 5), melanoma (n = 6), and hemangioma (n = 1). The mean initial visual acuity by logarithm of the minimum angle of resolution (Snellen) for macular (n = 29) versus extramacular (n = 28) tumors was 1.2 (20/320) versus 0.61 (20/80) and at 4 years was 1.72 (20/800) versus 0.79 (20/125). Visual acuity loss of >or=3 Snellen lines was 60% versus 13%. By univariate analysis, the most important factors predictive of poor visual acuity included macular location and clock hour meridian of the tumor.
  
  
  CONCLUSIONS
  Combined hamartoma of the retina and RPE can cause profound visual acuity loss, particularly with macular tumors.
  
  
  BACKGROUND
  The authors have no proprietary or commercial interest in any materials discussed in this article.
authors.affiliation: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. carol.shields@shieldsoncology.com
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Archana
authors.initials: A
authors.lastname: Thangappan
authors.forename: Kimberly
authors.initials: K
authors.lastname: Hartzell
authors.forename: Paola
authors.initials: P
authors.lastname: Valente
authors.forename: Cesare
authors.initials: C
authors.lastname: Pirondini
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D002675:Child, Preschool; D005260:Female; D005451:Fluorescein Angiography; D006222:Hamartoma / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008266:Macula Lutea; D008297:Male; D011379:Prognosis; D012164:Retinal Diseases / Q000175:diagnosis / Q000503:physiopathology*; D055213:Retinal Pigment Epithelium / Q000473:pathology / Q000503:physiopathology*; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000175:diagnosis / Q000503:physiopathology*; D014792:Visual Acuity / Q000502:physiology*