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Cilioretinal artery hypoperfusion and its association with paracentral acute middle maculopathy
  1. Francesco Pichi1,2,
  2. Serena Fragiotta3,
  3. K Bailey Freund3,
  4. Adrian Au4,5,
  5. Andrea Lembo6,
  6. Paolo Nucci6,
  7. Stefano Sebastiani7,
  8. Juan Carlos Gutierrez Hernandez8,
  9. Emanuela Interlandi9,
  10. Francesco Pellegrini9,
  11. Rosa Dolz-Marco10,
  12. Roberto Gallego-Pinazo10,
  13. Jorge Orellana-Rios11,12,
  14. Feisal A Adatia13,
  15. Monique Munro13,
  16. Emad B Abboud1,
  17. Nicola Ghazi14,
  18. Eduardo Cunha Souza15,
  19. Radgonde Amer16,
  20. Piergiorgio Neri1,
  21. David Sarraf4,5
  1. 1 Cleveland Clinic Abu Dhabi, Eye Institute, Abu Dhabi, United Arab Emirates
  2. 2 Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, Cleveland, Ohio, USA
  3. 3 Vitreous Retina Macula Consultants of New York, New York City, New York, USA
  4. 4 Stein Eye Institute, University of California, Los Angeles, California, USA
  5. 5 Greater Los Angeles VA Healthcare Center, Los Angeles, California, USA
  6. 6 San Giuseppe Hospital, University Eye Clinic, Milan, Italy
  7. 7 University Eye Clinic of Bologna, Policlinico Sant’Orsola-Malpighi, Bologna, Italy
  8. 8 Clinica Oftalmologica, Caja Costarricense del Seguro Social, San Rafael de Escazú, Costa Rica
  9. 9 Unità Operativa di Oculistica, Ospedale di Conegliano, Conegliano, Italy
  10. 10 Unit of Macula, Oftalvist Clinic, Valencia, Spain
  11. 11 New York Eye and Ear Infirmary of Mount Sinai, New York City, New York, USA
  12. 12 University of Antofagasta, Antofagasta, Chile
  13. 13 Mitchell Eye Center and Rockyview, Calgary, Alberta, Canada
  14. 14 Lebanese American University, Medical Center, Rizk Hospital, Beirut, Lebanon
  15. 15 Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto, Brazil
  16. 16 Hadassah Medical Center, Jerusalem, Israel
  1. Correspondence to Dr Francesco Pichi, Cleveland Clinic Abu Dhabi, Abu Dhabi 112412, United Arab Emirates; ilmiticopicchio{at}


Background/aims To study the multimodal imaging findings of a large series of eyes with cilioretinal artery obstruction (CILRAO) and describe the systemic associations.

Methods Multicentre, retrospective chart review from 12 different retina clinics worldwide of eyes with CILRAO, defined as acute retinal whitening in the distribution of the cilioretinal artery, were identified. The clinical, systemic information and multimodal retinal imaging findings were collected and analysed.

Results A total of 53 eyes of 53 patients with CILRAO were included in the study. In 100% of eyes, fundus photography illustrated deep retinal whitening corresponding to the course of the cilioretinal artery. Twenty-eight patients (52.8%) presented with isolated CILRAO (baseline best-corrected visual acuity (BCVA) 20/50, final BCVA 20/25) associated with nocturnal hypotension, 23 patients (43.4%) with CILRAO secondary to central retinal vein occlusion (CRVO) (baseline BCVA 20/40, final BCVA 20/20) and two patients with CILRAO due to biopsy-proven giant cell arteritis (GCA) (baseline BCVA 20/175, final BCVA 20/75). With spectral domain optical coherence tomography (SD-OCT), a hyper-reflective band involving the inner nuclear layer (ie, paracentral acute middle maculopathy or PAMM) was noted in 51 eyes (28/28 eyes with isolated CILRAO and 23/23 eyes with CILRAO+CRVO) corresponding to the retinal whitening. In the two eyes with CILRAO+GCA, SD-OCT illustrated hyper-reflective ischaemia of both the middle and inner retina.

Conclusions Isolated CILRAO and CILRAO secondary to CRVO are the result of hypoperfusion or insufficiency, rather than occlusion, of the cilioretinal artery and are associated with PAMM or selective infarction of the the inner nuclear layer. With GCA, there is complete occlusion of the cilioretinal artery producing ischaemia involving both the middle and inner retina associated with worse visual outcomes.

  • retina
  • imaging
  • macula

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  • Contributors Substantial contributions to the conception or design of the work; or the acquisition, analysis or interpretation of data for the work: FP, SF, KBF, AA, AL, PN, SS, JCGH, EI, FP, RD-M, RG-P, JO-R, FAA, MM, EBA, NG, ECS, RA, PN, DS. Drafting the work or revising it critically for important intellectual content: FP, SF, KBF, AA, AL, PN, SS, JCGH, EI, FP, RD-M, RG-P, JO-R, FAA, MM, EBA, NG, ECS, RA, PN, DS. Final approval of the version to be published: FP, SF, KBF, AA, AL, PN, SS, JCGH, EI, FP, RD-M, RG-P, JO-R, FAA, MM, EBA, NG, ECS, RA, PN, DS. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: FP, SF, KBF, AA, AL, PN, SS, JCGH, EI, FP, RD-M, RG-P, JO-R, FAA, MM, EBA, NG, ECS, RA, PN, DS.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval Cleveland Clinic Abu Dhabi Institutional Review Board.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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