Vitreous & Retina

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Hereditary / Congenital
X-linked juvenile retinoschisis is characterized by splitting of the superficial layers of the sensory retina. Most cases demonstrate a stellate or spoke wheel-like maculopathy. Bilateral microcystic-like changes of the macula are shown in OCT studies.

  • Affects only males
  • Female carriers have normal vision and are normal on ophthalmic examination
  • Age of onset is in the first decade
  • Histopathologically, there is a split between the nerve fiber and ganglion cell layer
  • Clinical features:
    • Symptoms: decreased central vision (20/25 - 20/50) and slowly progressive
    • Signs:
      • Presence of perifoveal microcystic changes with spoke wheel-like plications of the overlying ILM, often referred to as stellate maculopathy which are better seen on red-free fundus photography
      • Peripheral retinal schisis occurs in 50% of cases
      • The most common location for this peripheral schisis is the inferotemporal quadrant
      • Potential findings at later ages as the condition progresses may include RPE changes. macular hole, retinal dragging with vitreous strands, complicated with vitreous hemorrhage and rhegmatogenous retinal detachment
  • Ancillary Test:
    • Fluorescein angiographic reveals similar pattern to that of cystoid macular edema, but without leakage of dye in the late phase
    • Visual field testing shows an absolute scotoma that corresponds to the areas of peripheral schisis
    • ERG shows refuced photopic and scotopic b-wave
  • Management:
    • Observation, since this is a slowly progressive condition where patients maintain their visual acuity for a long period of time
    • Surgical management is necessary if complications such as retinal detachment, vitreous hemorrhage or macular hole should occur

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