title: Microcornea, posterior megalolenticonus, persistent fetal vasculature, and coloboma: a new syndrome.
abstract: OBJECTIVE
  To report a newly identified syndrome of bilateral microcornea, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma (MPPC).
  
  
  METHODS
  Noncomparative case series.
  
  
  METHODS
  Eight patients with MPPC syndrome.
  
  
  METHODS
  Clinical data collected retrospectively included visual acuity, findings on office examination as well as examination under anesthesia, and, in some cases, fluorescein angiography. Intraoperative findings and postoperative visual acuity and clinical findings were recorded when surgical intervention was performed.
  
  
  METHODS
  Clinical description, intraoperative findings, and surgical outcomes.
  
  
  RESULTS
  All patients were found to have microcornea with corneal diameters of less than 8 mm. In all cases, the crystalline lens was found to be retrodisplaced with massive enlargement and a dramatic posterior lenticonus (posterior megalolenticonus), and the ciliary processes frequently were drawn to the lens capsule. A stalk of persistent fetal vascular tissue extended from the posterior pole of the lens to the optic disc. Posterior chorioretinal coloboma was present in all cases. Some cases also exhibited grossly dysplastic retina. Presentation frequently was asymmetric. Eight eyes of 6 patients underwent lensectomy, vitrectomy, membrane peeling, and fluid-Healon exchange (Healon OVD [sodium hyaluronate]; Advanced Medical Optics, Santa Ana, CA) with functional vision in at least 4 of the 5 patients with postsurgical follow-up.
  
  
  CONCLUSIONS
  Bilateral microcornea, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma syndrome is a distinct syndrome previously unreported, to the authors' knowledge, and appropriate surgical intervention may result in significantly improved visual function.
authors.affiliation: Associated Retinal Consultants, Royal Oak, Michigan 48073, USA. tranchod@yahoo.com
authors.forename: Tushar M
authors.initials: TM
authors.lastname: Ranchod
authors.forename: Polly A
authors.initials: PA
authors.lastname: Quiram
authors.forename: Nolan
authors.initials: N
authors.lastname: Hathaway
authors.forename: Lawrence Y
authors.initials: LY
authors.lastname: Ho
authors.forename: Ben J
authors.initials: BJ
authors.lastname: Glasgow
authors.forename: Michael T
authors.initials: MT
authors.lastname: Trese
mesh_terms: D002648:Child; D002675:Child, Preschool; D002829:Choroid / Q000002:abnormalities* / Q000601:surgery; D003103:Coloboma / Q000209:etiology* / Q000601:surgery; D003315:Cornea / Q000002:abnormalities*; D005260:Female; D007839:Functional Laterality; D006801:Humans; D007223:Infant; D007908:Lens, Crystalline / Q000002:abnormalities* / Q000601:surgery; D008297:Male; D054514:Persistent Hyperplastic Primary Vitreous / Q000209:etiology* / Q000601:surgery; D012160:Retina / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies; D013577:Syndrome; D014792:Visual Acuity; D014821:Vitrectomy