PMID- 29790285
OWN - NLM
STAT- MEDLINE
DCOM- 20190819
LR  - 20190819
IS  - 1442-9071 (Electronic)
IS  - 1442-6404 (Linking)
VI  - 46
IP  - 9
DP  - 2018 Dec
TI  - Peripapillary vessel density changes in Leber's hereditary optic neuropathy: a 
      new biomarker.
PG  - 1055-1062
LID - 10.1111/ceo.13326 [doi]
AB  - IMPORTANCE: The contribution of the microvascular supply to the pathogenesis of 
      Leber's hereditary optic neuropathy (LHON) is poorly understood. BACKGROUND: We 
      aimed at measuring the peripapillary capillary vessel density (VD) using optical 
      coherence tomography angiography (OCT-A) at different stages of LHON. DESIGN: 
      Prospective, cross-sectional, multicenter, observational study. PARTICIPANTS: 
      Twenty-two LHON patients divided in four groups: unaffected mutation carriers 
      (LHON-u); early sub-acute stage (LHON-e); late sub-acute stage (LHON-l); chronic 
      stage (LHON-ch). METHODS: OCT-A scans centred on the optic disc were obtained by 
      spectral domain OCT system. MAIN OUTCOME MEASURES: VD, retinal nerve fibre layer 
      (RNFL) and ganglion cell-inner plexiform layer (GC-IPL) thickness were compared 
      between groups. RESULTS: Significant VD changes were detected in every sector (P 
      < 0.0001). In LHON-e, the VD was reduced in the temporal sector compared with 
      LHON-u and in the temporal and inferotemporal sectors compared with controls. In 
      LHON-l, VD was reduced in whole, temporal, superotemporal and inferotemporal 
      sectors compared with LHON-u and controls. In LHON-ch, the VD was reduced in all 
      sectors compared to the other groups. An asynchronous pattern emerged in the 
      temporal sector with VD changes occurring earlier than RNFL thickness changes and 
      together with GC-IPL thinning. CONCLUSIONS AND RELEVANCE: Significant 
      peripapillary miscrovascular changes were detected over the different stages of 
      LHON. Studying the vascular network separately from fibres revealed that 
      microvascular changes in the temporal sector preceded the changes of RNFL and 
      mirrored the GC-IPL changes. Measurements of the peripapillary vascular network 
      may become a useful biomarker to monitor the disease process, evaluate 
      therapeutic efficacy and elucidate pathophysiology.
CI  - (c) 2018 Royal Australian and New Zealand College of Ophthalmologists.
FAU - Balducci, Nicole
AU  - Balducci N
AUID- ORCID: 0000-0002-2625-2244
AD  - Studio Oculistico d'Azeglio, Bologna, Italy.
FAU - Cascavilla, Maria Lucia
AU  - Cascavilla ML
AD  - Scientific Institute San Raffaele, Milan, Italy.
FAU - Ciardella, Antonio
AU  - Ciardella A
AD  - Ophthalmology Unit, Policlinco Sant'Orsola-Malpighi, Bologna, Italy.
FAU - La Morgia, Chiara
AU  - La Morgia C
AD  - Unit of Neurology, Department of Biomedical and Neuromotor Sciences (DIBINEM), 
      University of Bologna, Bologna, Italy.
AD  - IRCCS, Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria, 
      Bologna, Italy.
FAU - Triolo, Giacinto
AU  - Triolo G
AD  - Scientific Institute San Raffaele, Milan, Italy.
FAU - Parisi, Vincenzo
AU  - Parisi V
AD  - GB Bietti Foundation IRCCS, Rome, Italy.
FAU - Bandello, Francesco
AU  - Bandello F
AD  - Scientific Institute San Raffaele, Milan, Italy.
FAU - Sadun, Alfredo A
AU  - Sadun AA
AD  - Department of Ophthalmology, Doheny Eye Institute, University of California, Los 
      Angeles, California, USA.
FAU - Carelli, Valerio
AU  - Carelli V
AD  - Unit of Neurology, Department of Biomedical and Neuromotor Sciences (DIBINEM), 
      University of Bologna, Bologna, Italy.
AD  - IRCCS, Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria, 
      Bologna, Italy.
FAU - Barboni, Piero
AU  - Barboni P
AD  - Studio Oculistico d'Azeglio, Bologna, Italy.
AD  - Scientific Institute San Raffaele, Milan, Italy.
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Multicenter Study
PT  - Observational Study
DEP - 20180620
PL  - Australia
TA  - Clin Exp Ophthalmol
JT  - Clinical & experimental ophthalmology
JID - 100896531
MH  - Adult
MH  - Cross-Sectional Studies
MH  - Female
MH  - Fluorescein Angiography/*methods
MH  - Follow-Up Studies
MH  - Fundus Oculi
MH  - Humans
MH  - Male
MH  - Optic Atrophy, Hereditary, Leber/*diagnosis
MH  - Optic Disk/*blood supply
MH  - Prospective Studies
MH  - Retinal Ganglion Cells/*pathology
MH  - Retinal Vessels/*pathology
MH  - Severity of Illness Index
MH  - Tomography, Optical Coherence/*methods
MH  - Young Adult
OTO - NOTNLM
OT  - Leber hereditary optic atrophy
OT  - OCT-A
OT  - optic nerve
OT  - optical coherence tomography angiography
EDAT- 2018/05/24 06:00
MHDA- 2019/08/20 06:00
CRDT- 2018/05/24 06:00
PHST- 2018/01/25 00:00 [received]
PHST- 2018/05/15 00:00 [revised]
PHST- 2018/05/16 00:00 [accepted]
PHST- 2018/05/24 06:00 [pubmed]
PHST- 2019/08/20 06:00 [medline]
PHST- 2018/05/24 06:00 [entrez]
AID - 10.1111/ceo.13326 [doi]
PST - ppublish
SO  - Clin Exp Ophthalmol. 2018 Dec;46(9):1055-1062. doi: 10.1111/ceo.13326. Epub 2018 
      Jun 20.