PMID- 38471086
OWN - NLM
STAT- Publisher
LR  - 20240312
IS  - 1937-1578 (Electronic)
IS  - 1935-1089 (Linking)
DP  - 2024 Mar 1
TI  - CONCENTRIC MACULAR RINGS SIGN IN COMBINED HAMARTOMA OF RETINA AND RETINAL PIGMENT 
      EPITHELIUM.
LID - 10.1097/ICB.0000000000001563 [doi]
AB  - PURPOSE: To describe two cases of concentric macular rings (CMR) sign in patients 
      with combined hamartoma of retina and retinal pigment epithelium (CHRRPE). 
      METHODS: History and clinical examination, spectral-domain optical coherence 
      tomography (SD-OCT), and optic coherence tomography angiography (OCTA). RESULTS: 
      The first patient was a 26-year-old woman with clinical diagnosis of 
      Neurofibromatosis type 2. The Best-Corrected Visual Acuity (BCVA) was 20/25 in 
      the right eye (OD) and 20/20 in the left eye (OS). She presented a subtle grayish 
      lesion in the macular region of OD. On macular SD-OCT, there was a thickened and 
      disorganized retina, suggestive of CHRRPE. In addition, at the parafoveal region, 
      there was a dentate pattern in the outer plexiform layer (OPL) and Henle fiber 
      layer (HFL) on cross-sectional SD-OCT. OCTA showed no foveal avascular zone (FAZ) 
      in OD and Optos ultra-wide-field revealed CMR sign in this eye. The second 
      patient was a 14-year-old boy, with diabetes mellitus type 1. His BCVA was 20/25 
      in OD and 20/20 in OS. He had a subtle whitish lesion in the macular region of 
      OD, with SD-OCT findings suggestive of CHRRPE. Similar to the first case, there 
      was a dentate pattern in the OPL and HFL, on SD-OCT. In OD, blue reflectance 
      images exhibited the CMR sign, and OCTA showed absent FAZ. CONCLUSION: CHRRPE may 
      be related to changes in the OPL and HFL interface and may present the CMR sign.
FAU - da Silva Conci, Livia
AU  - da Silva Conci L
AUID- ORCID: 0000-0002-8460-8421
AD  - Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.
FAU - Maia, Renata Martins
AU  - Maia RM
AD  - Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.
FAU - Cavalcanti Lira, Rodrigo Pessoa
AU  - Cavalcanti Lira RP
AD  - Department of Surgery, Medical School, Federal University of Pernambuco, Recife, 
      Brazil.
FAU - Zacharias, Leandro Cabral
AU  - Zacharias LC
AD  - Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.
FAU - Cunha de Souza, Eduardo
AU  - Cunha de Souza E
AD  - Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.
FAU - Gianotti Pimentel, Sergio Luis
AU  - Gianotti Pimentel SL
AD  - Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.
FAU - Shields, Carol
AU  - Shields C
AD  - Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
LA  - eng
PT  - Journal Article
DEP - 20240301
PL  - United States
TA  - Retin Cases Brief Rep
JT  - Retinal cases & brief reports
JID - 101298744
SB  - IM
EDAT- 2024/03/12 18:42
MHDA- 2024/03/12 18:42
CRDT- 2024/03/12 16:43
PHST- 2023/05/28 00:00 [received]
PHST- 2024/02/13 00:00 [accepted]
PHST- 2024/03/12 18:42 [medline]
PHST- 2024/03/12 18:42 [pubmed]
PHST- 2024/03/12 16:43 [entrez]
AID - 01271216-990000000-00293 [pii]
AID - 10.1097/ICB.0000000000001563 [doi]
PST - aheadofprint
SO  - Retin Cases Brief Rep. 2024 Mar 1. doi: 10.1097/ICB.0000000000001563.