NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the Retina: A New Phenotype and its Differential Diagnosis
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NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the Retina: A New Phenotype and its Differential Diagnosis. / Sirks, Marc J; van Dijk, Elon H C; Pauleikhoff, Laurenz J B; Diederen, Roselie M H; Boon, Camiel J F.
in: ASIA-PAC J OPHTHALMO, Jahrgang 12, Nr. 6, 17.11.2023, S. 614-621.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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TY - JOUR
T1 - NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the Retina: A New Phenotype and its Differential Diagnosis
AU - Sirks, Marc J
AU - van Dijk, Elon H C
AU - Pauleikhoff, Laurenz J B
AU - Diederen, Roselie M H
AU - Boon, Camiel J F
N1 - Copyright © 2023 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.
PY - 2023/11/17
Y1 - 2023/11/17
N2 - PURPOSE: The presence of peripapillary intraretinal fluid (IRF) has a broad differential diagnosis, including several types of neovascular and pachychoroid-related diseases. However, the clinician may encounter cases without signs of neovascular or pachychoroid disease, or any other previously described diagnosis. For these patients, we propose the term NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the retina, and we discuss the differential diagnosis.DESIGN: A retrospective chart study set in a tertiary referral center for retinal diseases in Amsterdam, the Netherlands.METHODS: Using multimodal imaging, cases suspected of peripapillary pachychoroid syndrome were reviewed. Cases without signs of neovascular or pachychoroid disease were included in this study. These cases were discussed in a group of senior retinal specialists to establish a diagnosis, and if there was no evidence for any previously described diagnostic entity, these cases were categorized as NOPPES.RESULTS: Four cases of NOPPES were identified, 3 female patients and 1 male patient, aged between 58 and 75 years. Two patients were myopic, and 1 patient had a mild hyperopia. Three out of 4 cases showed unilateral peripapillary IRF, and 1 case had bilateral IRF. No improvement was seen after intravitreal bevacizumab or aflibercept, nepafenac eye drops, oral acetazolamide, vitrectomy with internal limiting membrane peeling, or surgery for carotid stenosis. One case showed a reduction in IRF after starting prednisolone eye drops.CONCLUSIONS: We describe NOPPES, a new form of peripapillary schisis-like IRF. NOPPES seems relatively therapy-resistant. More research is needed to delineate the clinical spectrum of NOPPES and its pathogenesis and treatment.
AB - PURPOSE: The presence of peripapillary intraretinal fluid (IRF) has a broad differential diagnosis, including several types of neovascular and pachychoroid-related diseases. However, the clinician may encounter cases without signs of neovascular or pachychoroid disease, or any other previously described diagnosis. For these patients, we propose the term NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the retina, and we discuss the differential diagnosis.DESIGN: A retrospective chart study set in a tertiary referral center for retinal diseases in Amsterdam, the Netherlands.METHODS: Using multimodal imaging, cases suspected of peripapillary pachychoroid syndrome were reviewed. Cases without signs of neovascular or pachychoroid disease were included in this study. These cases were discussed in a group of senior retinal specialists to establish a diagnosis, and if there was no evidence for any previously described diagnostic entity, these cases were categorized as NOPPES.RESULTS: Four cases of NOPPES were identified, 3 female patients and 1 male patient, aged between 58 and 75 years. Two patients were myopic, and 1 patient had a mild hyperopia. Three out of 4 cases showed unilateral peripapillary IRF, and 1 case had bilateral IRF. No improvement was seen after intravitreal bevacizumab or aflibercept, nepafenac eye drops, oral acetazolamide, vitrectomy with internal limiting membrane peeling, or surgery for carotid stenosis. One case showed a reduction in IRF after starting prednisolone eye drops.CONCLUSIONS: We describe NOPPES, a new form of peripapillary schisis-like IRF. NOPPES seems relatively therapy-resistant. More research is needed to delineate the clinical spectrum of NOPPES and its pathogenesis and treatment.
KW - Humans
KW - Male
KW - Female
KW - Middle Aged
KW - Aged
KW - Retrospective Studies
KW - Diagnosis, Differential
KW - Tomography, Optical Coherence/methods
KW - Retina
KW - Ophthalmic Solutions
KW - Fluorescein Angiography/methods
U2 - 10.1097/APO.0000000000000645
DO - 10.1097/APO.0000000000000645
M3 - SCORING: Review article
C2 - 37974318
VL - 12
SP - 614
EP - 621
JO - ASIA-PAC J OPHTHALMO
JF - ASIA-PAC J OPHTHALMO
SN - 2162-0989
IS - 6
ER -
