|Year : 2016 | Volume
| Issue : 2 | Page : 110-112
Purtscher-like retinopathy in acute alcoholic pancreatitis
Nitin Nema1, Saba Ishrat1, Abha Verma1, Manoj Kela2
1 Department of Ophthalmology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
2 Department of Surgery, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
|Date of Web Publication||23-Jun-2016|
Department of Ophthalmology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A 23-year-old man with a history of alcoholism presented with vomiting, fever, and sharp epigastric pain radiating to the back and flanks. He was diagnosed as a case of acute alcoholic pancreatitis on the basis of clinical findings and investigations. On the next day of presentation, he developed sudden bilateral visual loss. His best-corrected visual acuity was finger counting at one-foot distance in both eyes. He had diffuse whitening in the circumpapillary area, haloes around the retinal vessels (Purtscher flecken) and intra-retinal hemorrhages on ophthalmoscopic examination. Optical coherence tomography revealed bilateral macular edema. These findings were characteristic of Purtscher-like retinopathy. The patient showed systemic and visual improvement at 8 weeks follow-up after receiving the conventional treatment for acute alcoholic pancreatitis. This case emphasizes the importance of fundus examination by an ophthalmologist in the diagnosis of this rare under-diagnosed entity.
Keywords: Cotton-wool spots, optical coherence tomography, Purtscher flecken
|How to cite this article:|
Nema N, Ishrat S, Verma A, Kela M. Purtscher-like retinopathy in acute alcoholic pancreatitis. Oman J Ophthalmol 2016;9:110-2
| Introduction|| |
Purtscher's retinopathy, an occlusive microvasculopathy, was first described in 1910 after intracranial trauma.  It is characterized by sudden visual loss with multiple areas of retinal whitening in the posterior pole. Similar findings are seen in patients with no history of antecedent trauma. This nontraumatic form has been termed as Purtscher-like retinopathy (PuR). It can occur in patients with acute pancreatitis, connective tissue disorders, renal failure, childbirth, bone marrow transplantation, and Valsalva maneuver. ,
So far about fifty such cases have been reported in the literature  and to the best of our knowledge, this is the first case report of PuR associated with acute pancreatitis from India.
| Case Report|| |
A 23-year-old man with a history of heavy alcohol intake for past 1 year reported with complaints of vomiting, fever, and sharp epigastric pain radiating to the back and flanks. The symptoms started 3 h back and were nonremitting. The patient was cooperative and oriented to the surroundings. He had tachycardia but his blood pressure was within normal range. He had rebound tenderness in the epigastric and left hypochondriac regions.
The laboratory investigations showed raised total leukocyte count with polymorphonucleocytosis (hemoglobin: 12 g/dL, total lymphocyte count: 25,800 cells/cu mm, and differential leukocytic count: P85 L7M4E3B1). His blood urea was 74 mg/dL and serum creatinine 1.8 mg/dL. The serum levels of lipase (1015 IU/L, ×20 the normal) and amylase (396 IU/L, ×4 the normal) were elevated. Enzyme-linked immunoassay test for HIV was negative.
Contrast enhanced computerized tomography axial scans of the abdomen revealed localized collection of fluid around the pancreas with peripancreatic inflammation [Figure 1].
|Figure 1: Contrast-enhanced computerized tomography abdomen axial scan showing collection of fluid around pancreas along with inflammation (green arrows)|
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A diagnosis of acute pancreatitis caused by alcohol was made on the basis of clinical features, laboratory tests, and radiological findings. The patient was admitted in the Intensive Care Unit for monitoring and treatment.
On the 2 nd day of illness, the patient complained of sudden loss of vision in both eyes. On ocular examination, the best-corrected visual acuity was finger counting at one feet distance in both eyes. The slit-lamp examination showed an unremarkable anterior segment. Ophthalmoscopic examination was performed after adequate pupillary dilatation. The fundus showed diffuse whitening in the circumpapillary area mainly in the posterior pole surrounded by a halo of clear zone on either side of the retinal vessels in both eyes. This appearance was characteristic of Purtscher flecken. A few intra-retinal hemorrhages were also present around the disc in the posterior pole [Figure 2]. Optical coherence tomography (OCT) showed edema of the inner layers of the retina in the perifoveal region [Figure 3]. These findings were compatible with the diagnosis of PuR.
|Figure 2: (a and b) Fundus photographs showing Purtscher flecken (arrow) and intraretinal hemorrhages involving the posterior poles of both eyes|
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|Figure 3: (a and b) Optical coherence tomography showing bilateral inner retinal and macular edema|
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The conventional treatment for acute pancreatitis was instituted and patient started improving systemically within a week. At the same time, his vision and ophthalmoscopic findings began to show improvement. At 8 weeks follow-up, the visual acuity improved to 6/18 in the right eye and 6/9 in the left. The ocular fundus examination revealed remarkable resolution of retinal whitening and hemorrhages [Figure 4] and diminution of retinal edema on OCT [Figure 5].
|Figure 4: (a and b) Fundus photographs showing resolving retinal whitening at 8-week follow-up visit|
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|Figure 5: (a and b) Optical coherence tomography at 8-week follow-up showing diminished retinal edema|
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| Discussion|| |
The association between PuR and acute pancreatitis was first described by Inkeles and Walsh.  It is a rare but severe complication of acute pancreatitis. However, it is unrelated to the severity of acute pancreatitis.
PuR is diagnosed if the patient has at least three of the following five features: ,,
- Purtscher flecken
- Retinal hemorrhages, low-to-moderate number (1-10)
- Cotton-wool spots (typically restricted to the posterior pole)
- A probable or plausible explanatory etiology
- Complementary investigation compatible with diagnosis.
Our patient met all the five diagnostic features. The most striking finding was the presence of Purtscher flecken which is considered as a pathognomonic sign and found in 50% of the cases.  The fleckens presumably result from the occlusion of the precapillary arterioles. They appear as intraretinal whitening with a halo of clear zone on either side of retinal arterioles, venules, and precapillary arterioles in the posterior pole. The fleckens must be differentiated from the cotton-wool spots. The latter has ill-defined feathery edges and is located superficially over the major retinal vessels.  The fleckens are predominantly distributed in the posterior pole because this region of the retina is supplied by the end vessels and therefore, more susceptible to ischemic insults. On the other hand, there is a plexus of capillaries in the peripheral region of the retina that protects it from ischemia. 
The patient of PuR presents with sudden visual loss of variable degree after few hours or days of the culprit event. The visual impairment is usually self-limiting, but sometimes can be permanent.  In our patient, the visual acuity improved significantly after a longitudinal follow-up of 8 weeks.
There are many postulations for the pathogenesis of PuR. The strongest argument is in the favor of an embolic phenomenon where air, fat, fibrin clots, or leukocyte aggregates clog the retinal arteriole. In acute pancreatitis, leukocyte aggregates (leukoemboli), which are formed as a result of complement activation, are released in the circulation and occlude the retinal vessels. 
The diagnosis of PuR mainly relies on history and clinical findings. However, OCT confirms the macular and inner retinal edema and helps in monitoring the patient. Fluorescein angiography shows hypofluorescence in the early phase due to masking by overlying retinal whitening and blood. Moreover, there can be areas of retinal nonperfusion. 
PuR should be considered in the differential diagnosis of bilateral visual loss in young adults. It should be differentiated from hypertensive retinopathy, central retinal artery occlusion, diabetic retinopathy, commotio retinae, HIV retinopathy, alcohol-induced toxic amblyopia, and Leber's hereditary optic neuropathy.
There is no specific treatment available for PuR. Observation and treatment of the underlying etiology may be the most reasonable therapeutic option.  Studies have suggested the use of systemic corticosteroids and nonsteroidal anti-inflammatory drugs with variable results. , Corticosteroids work by stabilizing the injured neuronal membrane and microvascular channels as well as curtail the complement cascade.  Nesmith et al. have recently described the beneficial effect of intravitreal bevacizumab for the management of macular edema associated with PuR. 
A surge in alcohol abuse, especially in young adults in India, has been reported with an increase in the prevalence of alcohol-related diseases.  Therefore, the clinicians must be familiar with this rare entity as a cause of visual loss. We recommend that ocular fundus examination should be done routinely in acute pancreatitis, particularly in those patients who have associated visual obscurations.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Purtscher O. Noch unbekannte befunde nach schadeltrauma. Ber Dtsch Ophthalmol Ges 1910;36:294-310.
Behrens-Baumann W, Scheurer G, Schroer H. Pathogenesis of purtscher′s retinopathy. An experimental study. Graefes Arch Clin Exp Ophthalmol 1992;230:286-91.
Nor-Masniwati S, Azhany Y, Zunaina E. Purtscher-like retinopathy following valsalva maneuver effect: Case report. J Med Case Rep 2011;5:338.
Carrera CR, Pierre LM, Medina FM, Pierre-Filho Pde T. Purtscher-like retinopathy associated with acute pancreatitis. Sao Paulo Med J 2005;123:289-91.
Inkeles DM, Walsh JB. Retinal fat emboli as sequela to acute pancreatitis. Am J Ophthalmol 1975;80:935-8.
Proença Pina J, Ssi-Yan-Kai K, de Monchy I, Charpentier B, Offret H, Labetoulle M. Purtscher-like retinopathy: Case report and review of the literature. J Fr Ophtalmol 2008;31(6 Pt 1):609-13.
Agrawal A, McKibbin MA. Purtscher′s and Purtscher-like retinopathies: A review. Surv Ophthalmol 2006;51:129-36.
Higgins JP, Green S, editors. Cochrane Handbook for Systematic Reviews of Interventions. Vol. 26, Ver. 5.0.0. Chichester (UK): John Wiley & Sons; 2008. p. 434-49.
Miguel AI, Henriques F, Azevedo LF, Loureiro AJ, Maberley DA. Systematic review of Purtscher′s and Purtscher-like retinopathies. Eye (Lond) 2013;27:1-13.
Jacob HS, Goldstein IM, Shapiro I, Craddock PR, Hammerschmidt DE, Weissmann G. Sudden blindness in acute pancreatitis. Possible role of complement-induced retinal leukoembolization. Arch Intern Med 1981;141:134-6.
Shibata K, Furuki I, Saitoh Y, Mochizuki K, Tanabe J. A case of Purtscher′s retinopathy. Am J Emerg Med 1994;12:661-3.
Beckingsale AB, Rosenthal AR. Early fundus fluorescein angiographic findings and sequelae in traumatic retinopathy: Case report. Br J Ophthalmol 1983;67:119-23.
Hammerschmidt DE, White JG, Craddock PR, Jacob HS. Corticosteroids inhibit complement-induced granulocyte aggregation. A possible mechanism for their efficacy in shock states. J Clin Invest 1979;63:798-803.
Nesmith BL, Bitar MS, Schaal S. The anatomical and functional benefit of bevacizumab in the treatment of macular edema associated with Purtscher-like retinopathy. Eye (Lond) 2014;28:1038-40.
Prasad R. Alcohol use on the rise in India. Lancet 2009;373:17-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]