PMID- 27984356
OWN - NLM
STAT- MEDLINE
DCOM- 20180921
LR  - 20180921
IS  - 1937-1578 (Electronic)
IS  - 1935-1089 (Linking)
VI  - 12
IP  - 4
DP  - 2018 Fall
TI  - STRUCTURAL AND FUNCTIONAL MONITORING OF EXTRAMACULAR CYSTOID SPACES IN A CASE OF 
      X-LINKED RETINOSCHISIS TREATED WITH ACETAZOLAMIDE.
PG  - 318-321
LID - 10.1097/ICB.0000000000000495 [doi]
AB  - BACKGROUND: Carbonic anhydrase inhibitors (CAIs) have been shown to have a 
      beneficial effect on cystoid macular edema in X-linked retinoschisis (XLRS) and 
      other inherited retinal conditions. The effect of CAIs outside the macula has 
      been less well studied. METHODS: Snellen visual acuity, spectral-domain optical 
      coherence tomography (SD-OCT), kinetic visual field, and dark-adapted 
      single-flash full-field electroretinogram (ERG) testing were all done at baseline 
      and at least one follow-up visit. A 55-year-old male diagnosed with XLRS 
      exhibited extensive macular and extramacular cystoid splitting in the right eye 
      and was treated with oral extended-release acetazolamide 500 mg/day. RESULTS: By 
      6 months of follow-up on acetazolamide treatment, SD-OCT demonstrated resolution 
      of cystoid spaces both within the macula and out to the midperiphery. Visual 
      acuity improved from 20/70 to 20/30. The full-field ERG was distinctly 
      electronegative at both baseline and at a follow-up visit, with oscillatory 
      potentials becoming more apparent at the follow-up visit. Peripheral visual field 
      boundaries did not change significantly. CONCLUSION: This report demonstrates 
      structural resolution of cystoid spaces throughout much of the retina in a 
      patient with XLRS, adding to a published case report in which we first noted that 
      extramacular cystoid spaces observed in XLRS may respond to CAI treatment. To our 
      knowledge, this is the first reported study of follow-up functional studies (ERG 
      and perimetry) in a CAI treatment responder with XLRS.
FAU - Collison, Frederick T
AU  - Collison FT
AD  - The Pangere Center for Inherited Retinal Diseases, The Chicago Lighthouse, 
      Chicago, Illinois.
FAU - Fishman, Gerald A
AU  - Fishman GA
AD  - The Pangere Center for Inherited Retinal Diseases, The Chicago Lighthouse, 
      Chicago, Illinois.
AD  - Department of Ophthalmology and Visual Sciences, University of Illinois at 
      Chicago College of Medicine, Chicago, Illinois.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Retin Cases Brief Rep
JT  - Retinal cases & brief reports
JID - 101298744
RN  - 0 (Carbonic Anhydrase Inhibitors)
RN  - O3FX965V0I (Acetazolamide)
SB  - IM
MH  - Acetazolamide/*therapeutic use
MH  - Carbonic Anhydrase Inhibitors/*therapeutic use
MH  - Genetic Diseases, X-Linked/*drug therapy
MH  - Humans
MH  - Macular Edema/*drug therapy
MH  - Male
MH  - Middle Aged
MH  - Retinoschisis/*drug therapy
EDAT- 2016/12/17 06:00
MHDA- 2018/09/22 06:00
CRDT- 2016/12/17 06:00
PHST- 2016/12/17 06:00 [pubmed]
PHST- 2018/09/22 06:00 [medline]
PHST- 2016/12/17 06:00 [entrez]
AID - 10.1097/ICB.0000000000000495 [doi]
PST - ppublish
SO  - Retin Cases Brief Rep. 2018 Fall;12(4):318-321. doi: 
      10.1097/ICB.0000000000000495.