Abstract
Paracentral acute middle maculopathy (PAMM) is a recently identified spectral-domain optical coherence tomography (SD-OCT) finding characterized by a hyper-reflective band spanning the inner nuclear layer (INL), which typically evolves to INL atrophy in later stages. Typical clinical features include the sudden onset of one or multiple paracentral scotomas, normal or mild reduction in visual acuity, and a normal fundus appearance or a fundus with a deep grayish lesion. Although its pathophysiology is not yet fully understood, ischemia at the level of the intermediate and deep capillary plexa has been demonstrated to play a major role. Since its first description, an increasing number of publications on PAMM have been published in ophthalmology scientific journals. The purpose of this study is to provide a review of the current literature on PAMM.
Similar content being viewed by others
References
Sarraf D, Rahimy E, Fawzi AA et al (2011) Paracentral acute middle maculopathy a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA Ophthalmol 131(10):1275–1287. https://doi.org/10.1001/jamaophthalmol.2013.4056
Rahimy E, Kuehlewein L, Sadda SR, Sarraf D (2015) Paracentral acute middle maculopathy: what we knew then and what we know now. Retina. 35:1921–1930
Chen Y, Hu Y (2017) The optical imaging of idiopathic paracentral acute middle maculopathy in a Chinese young man and review of the literature. Photodiagn Photodyn Ther 19:383–387. https://doi.org/10.1016/j.pdpdt.2017.07.004
Nakashima H, Iwama Y, Tanioka K, Emi K (2018) Paracentral acute middle Maculopathy following Vitrectomy for proliferative diabetic retinopathy: incidence, risk factors, and clinical characteristics. Ophthalmology. 125(12):1929–1936. https://doi.org/10.1016/j.ophtha.2018.07.006
Casalino G, Coppola M, Earley O, Contieri F, Bandello F, Chakravarthy U (2019) Paracentral acute middle maculopathy as a cause of unexplained visual loss in central retinal vein occlusion. Saudi J Ophthalmol 33(2):168–171. https://doi.org/10.1016/j.sjopt.2018.07.005
Sarda V, Nakashima K, Wolff B, Sahel JA, Paques M (2011) Topography of patchy retinal whitening during acute perfused retinal vein occlusion by optical coherence tomography and adaptive optics fundus imaging. Eur J Ophthalmol 21(5):653–656. https://doi.org/10.5301/EJO.2011.6374
Chu S, Nesper PL, Soetikno BT, Bakri SJ, Fawzi AA (2018) Projection-resolved OCT angiography of microvascular changes in paracentral acute middle maculopathy and acute macular neuroretinopathy. Investig Ophthalmol Vis Sci 59(7):2913–2922. https://doi.org/10.1167/iovs.18-24112
McLeod D (2019) Misery perfusion, diffusive oxygen shunting and interarterial watershed infarction underlie oxygenation-based hypoperfusion maculopathy. Am J Ophthalmol 205:153–164. https://doi.org/10.1016/j.ajo.2019.03.015
Pichi F, Fragiotta S, Freund KB et al (2019) Cilioretinal artery hypoperfusion and its association with paracentral acute middle maculopathy. Br J Ophthalmol 103(8):1137–1145. https://doi.org/10.1136/bjophthalmol-2018-312774
Christenbury JG, Klufas MA, Sauer TC, Sarraf D (2015) OCT angiography of paracentral acute middle maculopathy associated with central retinal artery occlusion and deep capillary ischemia. Ophthalmic Surg Lasers Imaging Retin 46(5):579–581. https://doi.org/10.3928/23258160-20150521-11
Nakamura M, Katagiri S, Hayashi T et al (2019) Longitudinal follow-up of two patients with isolated paracentral acute middle maculopathy. Int Med Case Rep J 12:143–149
Casalino G, Williams M, McAvoy C, Bandello F, Chakravarthy U (2016) Optical coherence tomography angiography in paracentral acute middle maculopathy secondary to central retinal vein occlusion. Eye. 30(6):888–893. https://doi.org/10.1038/eye.2016.57
Shah A, Rishi P, Chendilnathan C, Kumari S (2019) OCT angiography features of paracentral acute middle maculopathy. Indian J Ophthalmol 67(3):417–419. https://doi.org/10.4103/ijo.IJO_1249_18
Rahimy E, Sarraf D, Dollin ML, Pitcher JD, Ho AC (2014) Paracentral acute middle maculopathy in nonischemic central retinal vein occlusion. Am J Ophthalmol 158(2):372–380. https://doi.org/10.1016/j.ajo.2014.04.024
Hussnain SA, Coady PA, Stoessel KM (2017) Paracentral acute middle maculopathy: precursor to macular thinning in sickle cell retinopathy. BMJ Case Rep. Published online 2017 Apr 26. https://doi.org/10.1136/bcr-2016-216124
McLeod D, Beatty S (2015) Evidence for an enduring ischaemic penumbra following central retinal artery occlusion, with implications for fibrinolytic therapy. Prog Retin Eye Res 49:82–119. https://doi.org/10.1016/j.preteyeres.2015.06.001
Querques G, La Spina C, Miserocchi E, Gagliardi M, Corvi F, Bandello F (2014) Photo essay angiographic evidence of retinal artery transient occlusion in Paracentral acute middle Maculopathy. Retina. 34(10):2158–2160
Bakhoum MF, Freund KB, Dolz-Marco R et al (2018) Paracentral acute middle Maculopathy and the ischemic Cascade associated with retinal vascular occlusion. Am J Ophthalmol 195:143–153. https://doi.org/10.1016/j.ajo.2018.07.031
Kaidonis G, Silva RA, Sanislo SR, Leng T (2017) The superficial and deep retinal capillary plexus in cases of fovea plana imaged by spectral-domain optical coherence tomography angiography. Am J Ophthalmol Case Rep 6:41–44. https://doi.org/10.1016/j.ajoc.2016.09.007
Kulikov AN, Maltsev DS, Leongardt TA (2018) Retinal microvasculature alteration in Paracentral acute middle Maculopathy and acute macular Neuroretinopathy. 0(0):1-9 Retin Cases Brief Rep 1. https://doi.org/10.1097/icb.0000000000000709
Nemiroff J, Kuehlewein L, Rahimy E et al (2016) Assessing deep retinal capillary ischemia in Paracentral acute middle Maculopathy by optical coherence tomography angiography. Am J Ophthalmol 162:121–132.e1. https://doi.org/10.1016/j.ajo.2015.10.026
Sridhar J, Shahlaee A, Rahimy E et al (2015) Optical coherence tomography angiography and en face optical coherence tomography features of Paracentral acute middle Maculopathy. Am J Ophthalmol 160(6):1259–1268.e2. https://doi.org/10.1016/j.ajo.2015.09.016
Chen X, Desai SJ, Baumal CR (2017) Paracentral acute middle Maculopathy in pregnancy. Retin Cases Brief Rep. 0(0):1–3. https://doi.org/10.1097/icb.0000000000000679
Ramtohul P, Freund KB (2019) Central acute middle Maculopathy: a novel variant of Paracentral acute middle Maculopathy in Foveal hypoplasia. Ophthalmol Retin 4(3):344–347. https://doi.org/10.1016/j.oret.2019.10.003
Dolz-Marco R, Phasukkijwatana N, Sarraf DFK (2016) Optical coherence tomography angiography in fovea Plana. Ophthalmic Surg Lasers Imaging Retin 47(7):670–673. https://doi.org/10.3928/23258160-20160707-10
Benouaich X, Mahieuab L, Matontic F, Soler V (2017) Persistence of foveal capillary plexi in a case of fovea plana evident on OCT angiography. J Fr Ophtalmol 40(1):4–7. https://doi.org/10.1016/j.jfo.2016.10.002
Dansingani KK, Inoue M, Engelbert M, Freund KB (2015) Optical coherence tomographic angiography shows reduced deep capillary flow in paracentral acute middle maculopathy. Eye. 29(12):1620–1624. https://doi.org/10.1038/eye.2015.180
Phasukkijwatana N, Rahimi M, Iafe N, Sarraf D (2016) Central retinal vein occlusion and paracentral acute middle maculopathy diagnosed with en face optical coherence tomography. Ophthalmic Surg Lasers Imaging Retin 47(9):862–864. https://doi.org/10.3928/23258160-20160901-10
Iafe NA, Onclinx T, Tsui I, Sarraf D (2017) Paracentral acute middle maculopathy and deep retinal capillary plexus infarction secondary to reperfused central retinal artery occlusion. Retin Cases Br Rep 11(1):S90–S93. https://doi.org/10.1097/ICB.0000000000000424
Sridhar J, Shahlaee A, Shieh WS, Rahimy E (2017) Paracentral acute middle maculopathy associated with retinal artery occlusion after cosmetic filler injection. Retin Cases Br Rep 11(1):S216–S218. https://doi.org/10.1097/ICB.0000000000000466
Garrity ST, Tseng VL, Sarraf D (2018) Paracentral acute middle Maculopathy in a Perivenular Fern-like distribution with en face optical coherence tomography. Retin Cases Brief Rep 12:S25–S28. https://doi.org/10.1097/ICB.0000000000000657
da Fonseca MLG, Souza A, Pereira MB, Vianna RNG, Cravo LM, Demori E. (2019) Paracentral acute middle maculopathy associated with hypoperfusion of the cilioretinal artery and impending central retinal vein occlusion. Eur J Ophthalmol 0(0):1–3. https://doi.org/10.1177/1120672119885787
Maltsev DS, Kulikov AN, Burnasheva MA, Chhablani J (2020) Prevalence of resolved paracentral acute middle maculopathy lesions in fellow eyes of patients with unilateral retinal vein occlusion. Acta Ophthalmol 98(1):e22–e28. https://doi.org/10.1111/aos.14196
Rivera-De La Parra D, Fromow-Guerra J (2017) Paracentral acute middle Maculopathy in Purtscher retinopathy. Retin Cases Brief Rep 0(0):1–3. https://doi.org/10.1097/icb.0000000000000696
Tokimitsu M, Murata M, Toriyama Y, Hirano T, Iesato Y, Murata T (2016) Delineation of capillary dropout in the deep retinal capillary plexus using optical coherence tomography angiography in a patient with Purtscher’s retinopathy exhibiting normal fluorescein angiography findings: a case report. BMC Ophthalmol 16(1):1–5. https://doi.org/10.1186/s12886-016-0298-x
Khatibi A (2018) Brazilian booty retinopathy: Purtscher-like retinopathy with Paracentral acute middle Maculopathy associated with PMMA injection into buttocks. Retin Cases Br Rep 12(1):17–20. https://doi.org/10.1097/ICB.0000000000000380
Ilginis T, Keane PA, Tufail A (2015) Paracentral acute middle maculopathy in sickle cell disease. JAMA Ophthalmol 133(5):614–616. https://doi.org/10.1001/jamaophthalmol.2014.6098
Chen X, Rahimy E, Sergott RC et al (2015) Spectrum of retinal vascular diseases associated with paracentral acute middle maculopathy. Am J Ophthalmol 160(1):26–34.e1. https://doi.org/10.1016/j.ajo.2015.04.004
de Oliveira BMR, Moraes de Souza L, Andrade GC, Lobos CZ, Cunha de Souza E, Bueno de Moraes NS (2020) Purtscher-like retinopathy and paracentral acute middle maculopathy caused by industrial silicone embolism. Retin Cases Br Rep 00(00):1–3. https://doi.org/10.1097/ICB.0000000000000986
Rahimy E, Sarraf D (2014) Paracentral acute middle maculopathy spectral-domain optical coherence tomography feature of deep capillary ischemia. Curr Opin Ophthalmol 25(3):207–212. https://doi.org/10.1097/ICU.0000000000000045
Burnasheva MA, Maltsev DS, Kulikov AN, Sherbakova KA, Barsukov AV (2020) Association of Chronic Paracentral Acute Middle Maculopathy Lesions with hypertension. Ophthalmol Retin 4(5):504–509. https://doi.org/10.1016/j.oret.2019.12.001
Aribas YK, Aktas Z, Bayrakceken K, Atalay T, Ozdek S. (2017) Paracentral acute middle Maculopathy in primary congenital Glaucoma. Retin Cases Brief Rep (figure 1) 0(0):1–3. https://doi.org/10.1097/icb.0000000000000652
Carey A, Cohen S (2016) Paracentral acute middle Maculopathy in birdshot Chorioretinopathy: a novel association. Retin Cases Br Rep 10(2):151–153. https://doi.org/10.1097/ICB.0000000000000212
Choi RY, Swan RJ, Hersh A, Vitale AT (2018) Retinal manifestations of juvenile Dermatomyositis: case report of bilateral diffuse chorioretinopathy with paracentral acute middle maculopathy and review of the literature. Ocul Immunol Inflamm 26(6):929–933. https://doi.org/10.1080/09273948.2017.1305421
Starr MR, Softing Hataye AL, Bakri SJ (2019) Asymptomatic multifocal paracentral acute middle maculopathy associated with juvenile dermatomyositis: optical coherence angiography findings. Retin Cases Br Rep 00(00):1–5. https://doi.org/10.1097/ICB.0000000000000849
Pham C, Boo A, Chew SKH, Okada M (2019) Paracentral acute middle maculopathy in a young patient following routine phacoemulsification surgery. Clin Exp Ophthalmol 47(9):1206–1209. https://doi.org/10.1111/ceo.13597
Creese K, Ong D, Sandhu SS et al (2017) Paracentral acute middle maculopathy as a finding in patients with severe vision loss following phacoemulsification cataract surgery. Clin Exp Ophthalmol 45(6):598–605. https://doi.org/10.1111/ceo.12945
Hooshmand J, Moore P, McKay D, Vote BJ (2018) Paracentral acute middle maculopathy associated with severe vision loss following vitrectomy for vitreous haemorrhage. Clin Exp Ophthalmol 46(6):706–708. https://doi.org/10.1111/ceo.13151
Ang MJ, Chen JJ, McDonald HR (2019) Paracentral acute middle maculopathy after epiretinal membrane removal. Retin Cases Brief Rep 00(00):1–4. https://doi.org/10.1097/icb.0000000000000883
O’Day R, Harper CA, Wickremasinghe SS (2019) Central retinal artery occlusion showing features of paracentral acute middle maculopathy following uncomplicated pterygium surgery. Clin Exp Ophthalmol 47(1):141–143. https://doi.org/10.1111/ceo.13353
Fanzca JC (2018) Anaesthetic perspective to 10 cases of paracentral acute middle maculopathy following cataract surgery. Clin Exp Ophthalmol 46(4):449–450. https://doi.org/10.1111/ceo.13098
Denny MR, Kalevar A, Chen JJ, Johnson RN (2019) Paracentral acute middle maculopathy associated with idiopathic intracranial hypertension. Retin Cases Br Rep 00(00):1–3. https://doi.org/10.1097/ICB.0000000000000846
Zhao Z, Faith P, Pakzad-Vaezi K et al (2017) Paracentral acute middle maculopathy associated with bilateral optic disk swelling and meningitis. Retin Cases Brief Rep 0(0):1–6. https://doi.org/10.1097/icb.0000000000000671
Moura-Coelho N, Cunha JP, Maleita D et al (2019) Paracentral acute middle maculopathy (PAMM) in neuro-ophthalmology – a case series. 4th EUNOS Congr Neuro-Ophthalmology 43sup1:1–121. https://doi.org/10.1152/ajpheart.00221.2007
Ho CYD, Yan B, Symons RCA, Hardy TG (2020) Paracentral acute middle maculopathy in a case of high-flow direct carotid cavernous fistula. Retin Cases Brief Rep 00(00):1–3. https://doi.org/10.1097/ICB.0000000000000996
Nakamura M, Katagiri S, Hayashi T, Yoshioka M, Nakano T (2018) Paracentral acute middle maculopathy after endovascular coil embolization. Retin Cases Brief Rep 0(0):1–5. https://doi.org/10.1097/ICB:0000000000000784
Lando L, Isaac DLC, Avila MP (2019) Paracentral acute middle maculopathy after aortic aneurysm repair. Retin Cases Brief Rep 00(00):1–3 5. https://doi.org/10.1097/ICB.0000000000000927
Michalak SM, Mukherjee N, Gospe SM (2020) Bilateral paracental acute middle maculophaty after cardiopulmonary bypass. Retin Cases Br Rep 00(00):1–4. https://doi.org/10.1097/ICB.0000000000000982
Al-khersan H, Russell JF, Shi Y et al (2020) Wide field swept source OCT angiography of multifocal retinal and choroidal occlusions from embolic triamcinolone acetonide. Am J Ophthalmol Case Rep 18:100704. https://doi.org/10.1016/j.ajoc.2020.100704
Haider AS, Viswanathan D, Williams D, Davies P. (2017) Paracentral acute middle Maculopathy in Susac syndrome. Retin Cases Brief Rep 0(0):1–7. https://doi.org/10.1097/icb.0000000000000633
Baciu P, Nofar CM, Spaulding J, Gao H (2017) Branch retinal artery occlusion associated with paracentral acute middle maculopathy in a patient with livedo reticularis. Retin Cases Br Rep 11(4):356–360. https://doi.org/10.1097/ICB.0000000000000370
Arf S, Sayman Muslubas I, Hocaoglu M, Karacorlu M (2018) Retinal deep capillary plexus ischemia in a case with antiphospholipid syndrome. Retin Cases Br Rep 12(2):106–110. https://doi.org/10.1097/ICB.0000000000000436
Schofield JR, Palestine AG, Pelak V, Mathias MT (2019) Deep capillary retinal ischemia and high-titer prothrombin-associated antiphospholipid antibodies: a case series of three patients. Am J Ophthalmol Case Rep 14:105–109. https://doi.org/10.1016/j.ajoc.2019.03.010
Haskes C, Santapaola S, Zinn J (2017) An atypical case of paracentral acute middle maculopathy. Optom Vis Sci 94(8):845–850. https://doi.org/10.1097/OPX.0000000000001087
Sebastiani S, Pellegrini M, Giannaccare G, Sarraf D (2018) Paracentral acute middle maculopathy associated with Phosphodiesterase-5 inhibitor therapy. Retin Cases Brief Rep 00(00):1–4. https://doi.org/10.1097/icb.0000000000000839
Zonnevylle K, Jacob J, Luyten S, Stanescu-Segall D (2019) Paracentral acute middle maculopathy following oral intake of sumatriptan. Retin Cases Br Rep 00(00):1–3. https://doi.org/10.1097/ICB.0000000000000914
Elwood KF, Wong RW (2018) Paracentral acute middle Maculopathy after Preworkout Havok ingestion. Retin Cases Brief Rep 0(0):1–4. https://doi.org/10.1097/icb.0000000000000739
Ghasemi Falavarjani K, Phasukkijwatana N, Freund KB et al (2017) En face optical coherence tomography analysis to assess the Spectrum of Perivenular ischemia and Paracentral acute middle Maculopathy in retinal vein occlusion. Am J Ophthalmol 177:131–138. https://doi.org/10.1016/j.ajo.2017.02.015
Fawwaz A, Maha L, Harara M (2018) Swept source Oct (en-face) imaging in conjunction with (retinal flow) Oct Angio map “marking out” deep capillary plexus ischemia in paracentral acute middle maculopath. J Clin Exp Ophthalmol 09(02):1–5. https://doi.org/10.4172/2155-9570.1000718
Jung JJ, Chen MH, Frambach CR, Rofagha S, Lee SS (2018) Spectral domain versus swept source optical coherence tomography angiography of the retinal capillary plexuses in sickle cell maculopathy. Retin Cases Br Rep 12(2):87–92. https://doi.org/10.1097/ICB.0000000000000448
Campbell JP, Zhang M, Hwang TS et al (2017) Detailed vascular anatomy of the human retina by projection-resolved optical coherence tomography angiography. Sci Rep 7:42201. https://doi.org/10.1038/srep42201
Pecen PE, Smith AG, Ehlers J (2015) Optical coherence tomography angiography and retinal microvascular ramification in acute macular neuroretinopathy and paracentral acute middle maculopathy. JAMA Ophthalmol 133(12):1478–1480. https://doi.org/10.1016/j.survophthal.2016.12.001
Yu S, Wang F, Pang CE, Yannuzzi LA, Freund KB (2014) Multimodal imaging findings in retinal deep capillary ischemia. Retina. 34(4):636–646. https://doi.org/10.1097/IAE.0000000000000048
Scarinci F, Varano M, Parravano M (2019) Retinal sensitivity loss correlates with deep capillary plexus impairment in diabetic macular ischemia. J Ophthalmol 7589841. https://doi.org/10.1155/2019/7589841
Pereira F, Godoy BR, Maia M, Regatieri CV (2019) Microperimetry and OCT angiography evaluation of patients with ischemic diabetic macular edema treated with monthly intravitreal bevacizumab: a pilot study. Int J Retin Vitr 5(1):1–7. https://doi.org/10.1186/s40942-019-0176-9
Balk LJ, Coric D, Knier B et al (2019) Retinal inner nuclear layer volume reflects inflammatory disease activity in multiple sclerosis; a longitudinal OCT study. Mult Scler J - Exp Transl Clin 5(3):205521731987158. https://doi.org/10.1177/2055217319871582
Saidha S, Al-Louzi O, Ratchford JN et al (2015) Optical coherence tomography reflects brain atrophy in multiple sclerosis: a four-year study. Ann Neurol 78(5):801–813. https://doi.org/10.1002/ana.24487
Cunningham ET, Levinson RD, Jampol LM, Engstrom RE, Lewis H, Holland GN (2001) Ischemic maculopathy in patients with acquired immunodeficiency syndrome. Am J Ophthalmol 132(5):727–733. https://doi.org/10.1016/S0002-9394(01)01217-X
Arevalo J, Garcia R, Arevalo F, Fernandez C (2015) Unilateral ischemic maculopathy associated with cytomegalovirus retinitis in patients with AIDS: optical coherence tomography findings. J Ophthalmic Vis Res 10(4):487–490. https://doi.org/10.4103/2008-322X.176903
Turbeville SD, Cowan LD, Gass JD (2003) Acute Macular Neuroretinopathy: A Review of the Literature. Surv Ophtalmol 48(1):1–11. https://doi.org/10.1055/a-0799-9630
Dansingani KK, Freund KB (2015) Paracentral acute middle maculopathy and acute macular neuroretinopathy: Related and distinct entities. Am J Ophthalmol 160(1):1–3.e2. https://doi.org/10.1016/j.ajo.2015.05.001
Bhavsar KV, Lin S, Rahimy E et al (2016) Acute macular neuroretinopathy: a comprehensive review of the literature. Surv Ophthalmol 61(5):538–565. https://doi.org/10.1016/j.survophthal.2016.03.003
Fawzi AA, Pappuru RR, Sarraf D et al (2012) Acute macular neuroretinopathy: long-term insights revealed by multimodal imaging. Retina. 32(8):1500–1513. https://doi.org/10.1097/IAE.0b013e318263d0c3
Literature research
We searched the PubMed and Google Scholar databases to identify articles published up to 30 April 2020, using the following key terms: paracentral acute middle maculopathy, PAMM, and macular ischemia. In addition, we manually searched the reference lists of most primary articles.
Author information
Authors and Affiliations
Corresponding authors
Ethics declarations
Conflict of interest
All authors declare that they have no conflicts of interest related to this paper.
Ethical approval
This article does not contain any studies with humans or animals performed by any of the authors.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Moura-Coelho, N., Gaspar, T., Ferreira, J.T. et al. Paracentral acute middle maculopathy—review of the literature. Graefes Arch Clin Exp Ophthalmol 258, 2583–2596 (2020). https://doi.org/10.1007/s00417-020-04826-1
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00417-020-04826-1