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Introduction

Purtscher’s retinopathy (PUR) is a rare retinal vascular condition associated with sudden vision loss. Although the cause is usually reported to be head and/or chest trauma1,2, nontraumatic cases are called Purtscher’s-like retinopathy (PLR). PLR has been reported in association with complement activation systemic diseases such as acute pancreatitis.3 as well as many other clinical entities (table 1).

Acute macular neuroretinopathy (AMN) is also a rare retinal condition causing sudden onset vision loss and paracentral scotoma more commonly reported as an idiopathic condition affecting females in their 30s.4,5  The etiology of AMN is also not well understood however, disruption of the deep retinal capillary plexus has been suggested as the cause of vision loss.6,7

The concurrence of these two conditions has been reported only once before.8 This is a report of a patient who demonstrated clinical features of both of these disease entities.

Case Report

A 28-year-old male presents with visual disturbance and vision loss for since he was hospitalized recently for a seizure. At the hospital, he was diagnosed with acute pancreatitis which was attributed to excessive alcohol consumption and a poor diet, also severely high blood pressure. At the hospital, he received the proper care and was discharged and was prescribed hydrocodone-acetaminophen 5-325 mg and levetiracetam 500 mg (anti-seizure) both were listed “as directed”. He is currently under the care of his PCP who has been referred for evaluation based on his visual symptoms.

His examination was remarkable for uncorrected and BCVA OD:20/20(-2) OS: 20/20(-2), EOM, primary gaze, and confrontation fields were all normal. Pupils were equal, round, and reactive to light with negative APD. IOP OD 19 OS 22. The slit lamp examination was unremarkable. His fundus examination was remarkable for cotton wool spots OD and edematous appearance of the papillomacular bundle (PMB) petaloid foveal changes OU (Figure 1).

Figure 1) Color fundus photograph of the right eye shows a few cotton wool spots (Bule Arrows). Patellid shifting of the foveal light reflex (FLR) and faint discoloration of the PMB is subtle but notable (Red Arrows)

Optical coherence tomography (OCT) of the right eye shows nerve fiber layer (NFL) thickening and hyperreflectivity of the inner-retina as well as vascular dropout seen on the OCT-A of the right eye as well as hyperreflective changes of the outer-retina OU (Figure 2).

Figure 2) Line Scan SD-OCT of both eyes shows the hyperreflectivity of the outer retina (Yellow Arrows), while the OCT scan associated with the OCT-A shows the inner retinal hyper-reflectance associated with NFL infarct (CWS) (Red Arrow). On OCT-A lack of capillary detail is seen in the area of the CWS this is either a masking effect or as the result of lack of capillary perfusion.(Green Arrow).

Diagnosis of combined Purtscher’s like retinopathy and acute macular neuroretinopathy associated with acute pancreatitis and the hypertensive crisis was made. Since the patient was being medically controlled no additional treatments were prescribed expecting self-limiting improvement. Patient was scheduled a one-month follow-up. As commonly encountered in these types of situations where the patient experiences spontaneous symptomatic improvement, he also did not return for his follow-up visit.

Discussion

Purtscher’s-like retinopathy (PLR) associated with acute pancreatitis is a rare retinal vascular disorder. PLR is characterized by diffuse and multifocal retinal whitening (Purtscher flecken), exudates, and hemorrhage, as well as nerve fiber layer infarcts (cotton wool spots), sometimes accompanied by macular edema. Clinical symptoms of PLR associated with acute pancreatitis typically include reduced or rapidly progressive vision loss and the presence of central or peripheral scotomas.9 The exact etiology of PLR associated with acute pancreatitis remains unclear; however, it is believed to be a result of a complex interplay of local hypoxia and systemic inflammatory and hypercoagulable states, which triggers arterial occlusion and/or thrombosis following direct trauma, or as a result of a hypercoagulable disorder, often involving hyperlipidemia, acute pancreatitis, among other conditions.9 Several cases of PLR associated with acute pancreatitis associated with excessive alcohol consumption have been reported in the literature.3, 10, 11

Anterior and posterior segment ocular examination is the primary diagnostic modality for PLR. Optical coherence tomography can also be used to identify structural changes in the nerve fiber and retinal layers. Current management of PLR associated with acute pancreatitis focuses on preventing tissue damage and improving visual acuity. Treatment options include observation,9, 12 systemic corticosteroids,13 anticoagulants, and antiplatelet agents in the general context of these agents in coagulative vascular disease as well as PLR associated with systemic embolic disease.14,15 Laser photocoagulation, and vitrectomy in rare cases of conversation to proliferative retinopathy associated with neovascularization and vitreous hemorrhage.16

Acute macular neuroretinopathy (AMN) is also a rare retinal disorder causing transient or permanent vision loss associated by either absence of clinical findings, or the presence of intraretinal lesions and an abnormal foveal reflex.6 Although AMN has been reported as an idiopathic condition affecting females in their 30s.4,6  Other etiologic and risk factors have been implicated in association of as causation of AMN (Table 2).

The pathophysiology of AMN is also not clearly understood but OCT findings usually demonstrates hyperreflectivity of the outer nuclear- (ONL) and outer plexiform layers (OPL), involvement of the ellipsoid zone (EZ) as well as alteration of the choroidal vessels.17 Currently there are no effective treatments for AMN and observation is recommended.18

Conclusion

The concurrence of PLR and AMN is extremely rare and the author was only able to identify one case,8  although that case was associated with a multivehicle accident (MVA) the clinical findings were very similar to the case reported here.

Although AMD and PLR are rare conditions, optometrist should be familiar with the presentation and risk factors of these conditions and consider the differential diagnosis of several other retinal vascular disease that have similar presentations.

References

  1. Roncone DP. Purtscher’s retinopathy. Optometry. 2002 Mar;73(3):166-72
  2. Demazure S, Gogneaux L, Ansquin M. Post-traumatic Purtscher’s retinopathy caused by chest compression following a motor vehicle accident: A case report. J Fr Ophtalmol. 2021 Nov;44(9): 563-65
  3. Hamp AM, Chu E, Slagle WS, Hamp RC, Joy JT, Morris RW. Purtscher’s retinopathy associated with acute pancreatitis. Optom Vis Sci. 2014 Feb;91(2):43-51
  4. Bos PJ, Deutman AF. Acute macular neuroretinopathy. Am J Ophthalmol. 1975; 80(4): 573-84
  5. Bhavsar KV, Lin S, Rahimy E, Joseph A, Freund KB, Sarraf D, Cunningham ET. Acute macular neuroretinopathy: A comprehensive review of literature. Surv Ophthalmol. 2016; 61(5):583-65
  6. Rahimy E, Sarraf D. Paracentral acute middle maculopathy spectral-domain optical coherence tomography features of deep capillary ischemia. Curr Opin Ophthalmol. 2014; 25(3):207-12
  7. Casalino G, Arrigo A, Romano F, Munk MR, Bandello F, Parodi MB. Acute macular neuroretinopathy: pathogenetic insights from optical coherence tomography angiography. Br J Ophthalmol. 2019; 103:410–14
  8. Şekeryapan Gediz B. Acute Macular Neuroretinopathy in Purtscher Retinopathy. Turk J Ophthalmol. 2020 Apr 29;50(2):123-26
  9. Agrawal A, McKibbin MA. Purtscher’s and Purtscher-like retinopathies: a review. Surv Ophthalmol. 2006 Mar-Apr. 51(2):129-36
  10. Gahn GM, Khanani AM, Khan M, Aziz AA, Siddiqui FA, London NJ, Mukkamala LK, Morse LS. Purtscher’s-like retinopathy associated with acute pancreatitis. Am J Ophthalmol Case Rep. 2020 Aug 20;20:100892. doi: 10.1016/j.ajoc.2020.100892. PMID: 32913924; PMCID: PMC7471597
  11. Massa R, Vale C, Macedo M, et al. Putscher-like Retinopathy, Hindawi Case Reports in Ophthalmol Medicine, 2015  https://doi.org/10.1155/2015/421329
  12. Kincaid MC, Green WR, Knox DL, Mohler C. A clinicopathological case report of retinopathy of pancreatitis. Br J Ophthalmol. 1982; 66(4): 219-26
  13. Wang A, Yen M, Liu J. Pathogenesis and Neuroprotective Treatment in Purtscher’s Retinopathy. Japanese J Ophthalmol. 1998; 42:318-22
  14. Frayser R, Hickam JB. Effect of Vasodilator Drugs on Retinal Blood Flow in Man. Arch Opththalmol. 1965; 73(5):640-2
  15. Elwood KF, Dieu AC, Kuranz CV, Mititelu M. Purtscher-Like Retinopathy with Cardioembolic Stroke: Case Report and Literature Review. Case Rep Ophthalmol. 2022; 17;13(3):943-48
  16. Lee DH, Hwang CK, Cukras CA, Wiley HE, Malik FF, Chew EY, Keenan TD. Two cases of severe Purtscher-like retinopathy demonstrating recurrence and progression to neovascularization and vitreous hemorrhage. Am J Ophthalmol Case Rep. 2020;  12;18:100664. doi: 10.1016/j.ajoc.2020.100664. PMID: 32211564
  17. Lee SY, Cheng JL, Gehrs KM, et al. Choroidal features of acute macular neuroretinopathy via optical coherence tomography angiography and correlation with serial multimodal imaging. JAMA Ophthalmol. 2017; 135:1177-83
  18. Rush, James A. Acute macular neuroretinopathy. Am J of Ophthalmol. 1977; 83(4): 490-94
Mohammad Rafieetary
Mohammad Rafieetary
Dr. Mohammad Rafieetary is a consultative optometric physician who has been in practice at the Charles Retina Institute (CRI) since 1996. Dr. Rafieetary is a graduate of Boston University and has received his Doctor of Optometry from the University of Missouri-St. Louis. He has completed a residency in Primary Care Optometry and Ocular Disease at the Southern College of Optometry (SCO) and the Memphis Health Center. Before joining CRI, Dr. Rafieetary was on faculty and served as an Associate Professor and the Chief of Advanced Ocular Disease Service at the Southern College of Optometry where he currently is an Adjunct Faculty. Dr. Rafieetary has received several awards including the SCO Faculty of the Year, the American Diabetic Association’s National Achievement of Distinction Award, and the Tennessee Associations of Optometric Physician’s Optometrist of the Year. Dr. Rafieetary is a Fellow of the American Academy of Optometry where he serves as the Chair of the Retina Interest Group, and Fellow of the Optometric Retina Society where he serves as the President and Chair of the Education Committee. He is board certified by the American Board of Optometry (ABO) as well as the American Board of Certification in Medical Optometry. He also serves on the Board of Directors of ABO. Dr. Rafieetary is published in numerous professional publications and lectures extensively about diseases of the eye with an emphasis on conditions of the retinal and vitreous. He also serves on the Advisory and Editorial Teams of serval professional companies and publications.

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