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Proliferative retinopathy and retinal detachment in pediatric atypical hemolytic uremic syndrome

Zujaja Tauqeer1, Kandace L Gollomp2, Peter Bracha3

  • 1Scheie Eye Institute, University of Pennsylvania, Philadelphia; Division of Pediatric Ophthalmology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
|November 17, 2021
PubMed

Insights

A pediatric patient with atypical hemolytic uremic syndrome developed severe vision loss due to proliferative retinopathy and retinal detachments. This case highlights the ocular complications associated with this rare condition in children.

Area of Science:

  • Ophthalmology
  • Pediatrics
  • Nephrology

Background:

  • Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy.
  • Ocular manifestations in pediatric aHUS are not well-documented.
  • Previous ocular history included chronic retinal detachment and neovascularization.

Observation:

  • A 14-year-old boy with aHUS presented with decreased vision, flashes, and floaters in his left eye.
  • Examination revealed a new combined tractional-rhegmatogenous retinal detachment in the left eye.
  • Despite surgical intervention, progressive retinal and optic nerve ischemia occurred.

Findings:

  • This is the first reported case of proliferative retinopathy and combined tractional-rhegmatogenous retinal detachments in a pediatric patient with aHUS.
  • The patient experienced irreversible vision loss to light perception in the affected eye.
  • The case underscores the potential for severe, sight-threatening ocular complications in pediatric aHUS.

Implications:

  • Early recognition and management of ocular symptoms in pediatric aHUS are crucial.
  • Further research is needed to understand the pathogenesis of ocular complications in aHUS.
  • This case emphasizes the importance of a multidisciplinary approach involving ophthalmologists, nephrologists, and hematologists.

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