A 22-year-old woman with alcoholic pancreatitis and bilateral central scotomata

History

A 22-year-old woman presented emergently at Kelowna General Hospital, British Columbia, with a 3-day history of epigastric pain with radiation to her back. This was assessed to be alcoholic pancreatitis, and she was admitted to the hospital for supportive care. Approximately 2 hours into her admission, she reported new-onset, binocular, blurry vision but no pain, flashes, floaters, or scotomata. On the third day of admission, the patient reported new-onset, bilateral, patchy central vision loss. Given the evolution of her vision loss, the ophthalmology service was consulted.

Examination

Visual acuity in the right eye was counting fingers; in the left eye, 20/20 (uncorrected). Her pupils were round and reactive to light bilaterally. There was no relative afferent pupillary defect. The anterior segments were normal, with no cells. Imaging (Figure 1) revealed areas of superficial retinal whitening, with greater involvement in the right than the left eye.

Figure 1.

Color fundus photographs obtained at initial presentation showing presence of multiple cotton wool spots and areas of retinal whitening in the right eye (A) and left eye (B).

Ancillary Testing

Optical coherence tomography (OCT) of both maculae at initial presentation (Figure 2) revealed hyper-reflectance, retinal thickening, disorganization of inner retinal layers, and macular edema corresponding to the areas of superficial retinal whitening, greater in the right than in the left eye.

Figure 2.

Optical coherence tomography (OCT) images providing a cross-sectional view of the macula of the right eye (A) and left eye (B), showing inner retinal hyper-reflectance corresponding to areas of superficial retinal whitening, and macular edema, greater in the right eye than in the left. A small collection of subfoveal fluid was also noted in the left eye.

Visual field testing performed 2 weeks after her initial visit revealed scotomata corresponding to areas of retinal whitening in the right eye; the left eye visual field was essentially normal (Figure 3).

Figure 3.

30-2 Humphrey visual field testing of the left eye (A) and right eye (B) 2 weeks after presentation. The physiologic blind spot is visible 15°-20° temporal to fixation in both eyes. There are scattered scotomata in an inferior arcuate distribution of the right eye corresponding to the superior Purtscher flecken. The left visual field is essentially normal.

OCT angiography revealed flow voids corresponding to areas of superficial retinal whitening (Figure 4). Impaired flow was most prominent within areas supplied by the superior arcades bilaterally but also within watershed regions along the horizontal raphe.

Figure 4.

OCT angiography slab through the deep capillary plexus obtained at initial presentation showing multiple hypodense areas corresponding to areas of reduced blood flow in the right eye (A) and left eye (B) demarcated by dotted lines.

Widefield fluorescein angiography—both early stage and late stage (Figure 5—revealed occlusions within the small arteriolar branches of the superior temporal vascular arcades, greater in the right eye than in the left. Again, these corresponded to areas of superficial retinal whitening. No other retinopathy, vasculitis, or disc edema were noted.

Figure 5.

A–B, Widefield fluorescein angiography obtained at initial presentation showing small arteriolar filling defects, more prominent in the arterial phase image in the right eye (A) than venous phase of left eye (B); defects are demarcated by red arrows and the area enclosed within the dotted red line. C–D, Widefield angiography obtained at initial presentation showing no emboli, cystoid macular edema, disc leak, or vasculitis was seen (C, late phase, right eye; D, late phase, left eye).

Treatment

The patient was started on oral prednisone 1 mg/kg daily with instructions to taper by 5 mg every week. The patient was also asked to follow-up in clinic for repeat imaging and assessment of her vision.

Differential Diagnosis

The differential diagnosis included diabetic retinopathy, hypertensive retinopathy, central retinal artery occlusion, central retinal vein occlusion, white dot syndromes, HIV retinopathy, and collagen vascular disorders. Although these may present with similar retinal examination characteristics, there are variable differences between them on history and physical examination.

Classically, diabetic maculopathy presents as retinal hemorrhages, microaneurysms, macular edema and hard exudates. In hypertensive retinopathy, retinal findings include retinal and nerve fiber layer hemorrhages, arteriolar remodeling with arteriovenous nicking, narrowing, and sclerosis, with hard exudates, cotton wool spots, retinal edema, and tortuous appearing arterioles. Both conditions develop over a more chronic time scale and lead to progressive, painless vision loss. They both also lack the characteristic polygonal patches of superficial retinal whitening, also known as Purtscher flecken, that was seen in our case.

In patients with central retinal artery occlusion, the classic findings include panretinal whitening, a cherry red spot over the fovea, and a boxcar-like appearance of the affected blood vessel due to sluggish blood flow. In a central retinal vein occlusion, findings include significant retinal hemorrhages, cotton wool spots, and macular edema, typically associated with an area of venous tortuosity and dilation. Patients often present with painless, monocular vision loss. Both conditions lack the characteristic patches of Purtscher flecken.

In white dot syndromes, patients may complain of painless vision loss. On retinal examination, multiple discrete, white lesions are seen, as in our case, but the depth at which these lesions occur is distinctive. In white dot syndromes, lesions are deep within the retina and rarely present as areas of superficial retinal whitening. Furthermore, characteristic angiographic findings of white dot syndromes, including early dye blockage, late staining, cystoid macular edema, disc leakage, and vasculitis, were not seen in our case.

Diagnosis and Discussion

Purtscher retinopathy was first described in 1910 by Otmar Purtscher as a characteristic chorioretinopathy associated with trauma.1 Retinal findings include the presence of polygonal white retinal patches (called Purtscher flecken), cotton wool spots, retinal hemorrhages, and sometimes papilledema.2 Patients with Purtscher retinopathy commonly present with a chief complaint of painless and sudden bilateral vision loss, with or without associated scotomata. These symptoms can arise at the time of trauma/injury or be delayed by up to 24–48 hours after the event.3 When the primary insult leading to this pathology is traumatic in nature (eg, head trauma, chest compression, long bone trauma), it is referred to as Purtscher retinopathy. When characteristic retinal findings are seen in the absence of trauma (eg, pancreatitis, HELLP syndrome), it is referred to as Purtscher-like retinopathy.

The current hypothesis for how this retinopathy develops is through an upregulation of the complement immune system. High levels of complement system proteins cause aggregation of leukocytes, which embolize into the retinal arterioles and lead to microvascular infarcts within the superficial retina.4 In the setting of acute pancreatitis, the initiation of this process is thought to be due to the elevated pancreatic enzymes in the blood, which cause the initial up-regulation of complement system proteins. In keeping with this pathogenesis, elevations in complement C5a protein levels are often noted.

Given the rarity of this pathology, establishing a diagnostic criterion has been difficult. In 2013, Miguel et al5 proposed an updated criterion which stated that patients must display three or more of the following features for a diagnosis: Purtscher flecken, retinal hemorrhages in low-to-moderate numbers, cotton-wool spots confined to the posterior pole, probable explanatory etiology, and complementary investigation compatible with diagnosis. Making this diagnosis requires a complete ophthalmic examination. Multimodal imaging modalities are commonly used, including color photography, fundus autofluorescence, OCT, OCT angiography, and fluorescein angiography.

Currently, there is no standard of treatment for this condition. Previous case studies have shown some derived benefit from using high-dose corticosteroids while other studies have also shown that observation alone is non-inferior.3,5,6 In the case of Purtscher-like retinopathy, treating the underlying condition (acute pancreatitis, COVID-19, HELLP syndrome) is just as important. Patients are to be managed per clinical decision making, but follow-up and repeat assessments are paramount.

In our case, the patient was treated with oral prednisone 1mg/kg tapered slowly by 5 mg weekly, and was also asked to follow-up in clinic for reassessment of her vision. On her 1-month follow-up, her uncorrected visual acuity had improved to 20/25 in the right eye and 20/20 in the left eye. There was considerable improvement in the number of cotton wool spots and in the size of Purtscher flecken bilaterally (Figure 6). Findings of inner retinal thickening and macular edema in both eyes, along with the subretinal fluid accumulation seen in the left eye macula had resolved (Figure 7). There were signs of thinning of the inner retinal layers that corresponded to the previously noted areas of Purtscher flecken, suggesting inner retinal atrophy.

Figure 6.

Resolving areas of superficial retinal whitening in the right eye (A) and left eye (B).

Figure 7.

OCT of the macula of the right eye (A) and left eye (B). Marked improvement is observed of the hyperreflective inner and middle retinal lesions and macular edema, but inner retinal thinning remains, indicating tissue atrophy.

On repeat OCT angiography, flow voids were once again noted suggesting irreversible retinal vascular compromise, particularly within watershed areas along the horizontal raphe (Figure 8). Some improvement was noted along the superior arcades bilaterally. For further assessment of her vision and retinal function, repeat visual field testing and imaging was arranged, but unfortunately, this patient was lost to follow-up.

Figure 8.

OCT angiography slab through the deep capillary plexus obtained at 1-month follow-up showing persistence of flow voids as noted by the hypodense areas in the right eye (A) and left eye (B) and demarcated with the dotted line. Some improvement is observed along the superior arcades bilaterally.

Our case demonstrates that, although rare, Purtscher retinopathy should be considered on the differential diagnosis of patchy vision loss in the setting of acute pancreatitis. The prognosis and visual recovery is variable for patients and there is a lack of strong evidence to help guide prognostication and treatment. The utility of high-dose corticosteroids has been supported by some authors, and this treatment may have been beneficial in this case.