title: Peripheral hypertrophic subepithelial corneal degeneration: nomenclature, phenotypes, and long-term outcomes.
abstract: OBJECTIVE
  To describe the long-term outcomes of peripheral hypertrophic subepithelial corneal degeneration.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Twenty-two patients under the care of the External Disease Service, Moorfields Eye Hospital.
  
  
  METHODS
  All patients matching clinical diagnostic criteria were included. Symptomatic patients were managed either conservatively or were offered superficial keratectomy in progressive cases where symptom control was inadequate. All excised tissue was examined histologically.
  
  
  METHODS
  Clinical phenotype, symptoms, recurrence rate after surgery, and histopathologic results.
  
  
  RESULTS
  Twenty-two white patients (20 women and 2 men; age range, 27-88 years; median age, 42 years) had peripheral, usually bilateral (20/22 [91%]), elevated circumferential peripheral subepithelial corneal opacities and adjacent abnormal limbal vasculature, with or without pseudopterygia in 9 patients (41%), among whom 7 (32%) patients had bilateral disease. Apart from the abnormal vasculature, there were no signs of chronic ocular surface inflammation. Symptoms were ocular surface discomfort in 10 patients (45%), reduced vision in 4 patients (18%), and both of these in 5 patients (23%). Three patients (14%) were asymptomatic. There was no treatment in 6 patients (27%), topical lubricants in 8 patients (36%), spectacles in 1 patient (5%), and superficial keratectomy in 7 patients (32%; 5 bilateral), which was repeated for incomplete primary excision in 1 eye of 2 patients (9%) and for a bilateral recurrence in 1 patient (5%). Median follow-up after excision was 5 years (range, 1-11 years). Histopathologic changes were similar to those observed in pterygia (vascular component) and Salzmann's nodular degeneration (corneal component).
  
  
  CONCLUSIONS
  Peripheral hypertrophic subepithelial corneal degeneration is an uncommon, usually bilateral, idiopathic disorder, occurring mostly in white women with a distinct phenotype. The condition was first described in 2003. Further cases among some patients have been described in more recent case series of Salzmann's nodular degeneration and diffuse keratoconjunctival proliferation, as well as in the previously described familial pterygoid corneal degeneration. Surgical excision was required in 30% of patients in this series, with infrequent short-term recurrences. The cause is uncertain.
  
  
  BACKGROUND
  The author(s) have no proprietary or commercial interest in any materials discussed in this article.
authors.affiliation: Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom.
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Gore
authors.forename: Alfonso
authors.initials: A
authors.lastname: Iovieno
authors.forename: Benjamin J
authors.initials: BJ
authors.lastname: Connell
authors.forename: Robert
authors.initials: R
authors.lastname: Alexander
authors.forename: George
authors.initials: G
authors.lastname: Meligonis
authors.forename: John K
authors.initials: JK
authors.lastname: Dart
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003316:Corneal Diseases / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D006801:Humans; D006984:Hypertrophy / Q000175:diagnosis / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012008:Recurrence; D013285:Strabismus / Q000175:diagnosis / Q000473:pathology*; D009626:Terminology as Topic