A 26-year-old African American male presented to the eye clinic with blurry vision, his left eye was worse than his right eye. The patient had been hospitalized eight days previous for the treatment of acute pancreatitis secondary to alcohol abuse and noted blurry vision while in the hospital. On examination, he was found to have best corrected visual acuity of 20/30 OD and 20/70 OS. All other entrance tests and anterior segment findings were within normal limits. Dilated fundus exam revealed extensive peripapillary polygonal plaque-like white lesions (Purtscher’s flecken), cotton wool spots and a few flame shaped intraretinal hemorrhages in both eyes (Figure 1A,B). Optical coherence tomography (OCT) of the macula showed a serous macular detachment OU (Figure 2A,B). Given the case history and fundus appearance, the patient was diagnosed with Purtscher’s-like retinopathy following acute pancreatitis. Since he had previously left the hospital against medical advice, he was sent to his primary care doctor to continue treatment of his pancreatitis. Same day laboratory results for serum lipase, a diagnostic measure for pancreatitis, was severely elevated: 1721 U/L (Ref 11-82 U/L), 20 times the upper limits of normal. Therefore, he was admitted for further treatment. The patient was to return to the eye clinic but has since been lost to follow up.

DISCUSSION

Purtscher’s Retinopathy was first described in 1910 by Otmar Purtscher, an Austrian ophthalmologist, who discovered retinal findings in a patient that fell from a tree and sustained cranial trauma. Classically, it has been associated with severe head injuries, but since then, it has been associated with many other systemic issues such as long bone fractures, renal failure, preeclampsia related to childbirth, shaken baby syndrome, acute pancreatitis and more. In general, the term Purtscher’s refers to patients with retinopathy with a history of trauma, and Purtscher’s-like if related to other systemic issues, although there is some inconsistency in the existing literature.  

Purtscher’s and Purtscher’s-like retinopathies are rare, with an estimated incidence of 0.24 per million per year. Patients with Purtscher’s-like retinopathy often report an acute, painless decrease in vision, typically at the time of the event, or within 24-48 hours. It is bilateral approximately 60% of the time and is virtually always bilateral if associated with acute pancreatitis. Visual field loss, such as central, paracentral or arcuate defects, may also be noted. 

Classic retinal findings include cotton wools spots (93%), retinal hemorrhages (65%) and Purtscher Flecken (63%). The retinal findings typically present 24 to 48 hours after onset of the associated systemic condition and persist for 1-3 months. Purtscher flecken are the pathognomonic finding for Purtsher’s retinopathy. Purtscher’s flecken are polygonal plaque-like lesions and are the result of precapillary arteriole occlusions in the inner nuclear layer that look similar to cotton wool spots. However, unlike cotton wool spots, they do not obscure the retinal vessels. Additionally, Purtscher’s flecken have a unique characteristic: a clear demarcation zone between the white lesion and the adjacent retinal vessel. (Fig 3) This area of normal retina represents the perivascular capillary-free or avascularized zone, which extends about 50 μm on either side of a vessel. The retinal hemorrhages present are most frequently flame shaped, but dot/blot hemorrhages can also be found. The amount of hemorrhage is typically minimal. Other associated findings can include optic nerve edema, macular edema, macular ischemia and serous macular detachment.

In the acute stages of Purtscher’s-like retinopathy, OCT will show hyper-reflectivity of the inner retina corresponding to cotton wool spots and Purtscher’s flecken, indicating ischemia in each respective retinal layer. Further, edema and subretinal fluid may occur at the macula. Following resolution of acute retinal findings, long-term thinning and atrophy may occur, particularly in those with ocular sequelae. 

Most cases of Purtscher’s and Purtscher’s-like retinopathy are left untreated and typically resolve on their own. Treatment of the underlying condition, such as pancreatitis, is also important. Other treatments, such as high dose intravenous steroids, have proven unsuccessful. Approximately 40% of patients resolve to normal or near-normal vision, with no sequelae. Long term sequelae of Purtscher’s and Purtscher’s-like retinopathy may include optic atrophy (64%), retinal pigment epithelial atrophy (23%), retinal nerve fiber layer thinning and foveal thinning (14%), and retinal vessel attenuation or sheathing (4%).

Differential diagnoses for Purtscher’s-like retinopathy may include central retinal artery occlusion, central retinal vein occlusion and commotio retinae.

 A link between Purtscher’s-like retinopathy and acute pancreatitis was first suggested in 1975. Acute pancreatitis results from an inability of the pancreas to release the enzymes and hormones it produces due to an obstruction to outflow. After gallstones, alcohol abuse is the second most common cause of acute pancreatitis and the most common cause of chronic pancreatitis. Symptoms of acute pancreatitis include nausea, intractable vomiting and epigastric pain. High serum and urine amylase and lipase levels in addition to epigastric tenderness are typical of acute pancreatitis. The diagnosis can be confirmed with abdominal ultrasound or computed tomography scan. Treatment of acute pancreatitis includes intravenous fluids, pain management and nutritional support. Patients are also instructed to abstain from alcohol use as more frequent consumption after the initial episode is related to higher risk of developing chronic pancreatitis and later pancreatic cancer.

CONCLUSION

While Purtscher’s retinopathy is classically associated with severe trauma, a similar presentation can be seen in association with various other conditions, such as acute pancreatitis. Patients often present with reduced vision and classic retinal findings. Following resolution of the acute retinal changes, patients can have residual decreased visual acuity and visual field loss. There is currently no accepted treatment for Purtscher’s and Purtscher’s-like retinopathy; therefore the best course of action is to treat the underlying condition and monitor for ocular resolution. 

Figure 1A,B: Fundus photos OD, OS, demonstrating extensive peripapillary intraretinal whitening (Purtscher’s flecken and cotton wool spots) with areas of intraretinal hemorrhages. Images taken with Eidon by I-care.

Figure 2A,B: Macular OCT OD, OS demonstrating serous macular detachment OU and  hyper-reflectivity of inner retinal layers, corresponding to the cotton wool spots and Purtscher’s flecken observed on clinical exam. Images taken with Heidelberg Spectralis.

Figure 3. Image of Purtscher’s flecken highlighting its polygonal plaque-like appearance and a clear demarcation zone between the white lesion and the adjacent retinal vessel (indicated by arrows). Images taken with Eidon by I-care