Purtscher retinopathy (PR) is a rare condition first described in 1910 by Otmar Purtscher in a middle-aged man with severe head trauma who presented with visual loss in both eyes. Purtscher observed multiple areas of retinal whitening (Purtscher flecken) and intraretinal hemorrhages in the posterior pole of both eyes.1
After its original description, similar fundus appearance was observed in patients with numerous diseases including acute pancreatitis, fat embolization, childbirth, renal failure, and connective tissue disorders.2–8 Since PR's original description involved trauma, primarily blunt thoracic trauma, and head trauma, these cases were termed as Purtscher-like retinopathy (PLR).
Although associated with several systemic conditions, PR and PLR are relatively uncommon pathologies. Recently the annual incidence was estimated as 0.24 cases per million.8
Lymphatic extravasations caused by the sudden increase in intracranial pressure was first postulated theory for pathophysiology of PR. Microembolic events, such as fat embolization in long bone fractures and air embolization from compressive chest injuries, were also hypothesized. More recently, studies have shown that leukocyte aggregation by activated complement factor 5 (C5a) may lead to leukocyte emboli that can occlude retinal capillaries in conditions such as acute pancreatitis, trauma, and collagen vascular diseases.9,10 This hypothesis was tested by an animal model that resulted in multiple small retinal arteriolar occlusions.11
The patients with PR and PLR present with unilateral or bilateral visual loss ranging from minimal impairment to light perception. Visual field loss in the form of central, paracentral, or arcuate scotoma may accompany the visual loss or may be the only complaint. Acute fundoscopic findings include multiple, discrete areas of retinal whitening, cotton-wool spots, and intraretinal hemorrhages, primarily located in the posterior pole. Optic disc swelling may be present. Fluorescein angiography (FA) may reveal masked choroidal fluorescence by the retinal whitening, cotton-wool spots or blood, arteriolar or capillary nonperfusion, and leakage from vessels in the areas of infarction and from the optic disc.12 Focal swelling of the retinal nerve fiber layer that correspond to cotton-wool spots, subretinal and intraretinal edema in the posterior pole, and distinct areas of increased hyperreflectivity in the inner retinal layers are previously reported optical coherence tomography (OCT) findings in patients with PR and PLR.13–16
Visual outcome in PR and PLR is variable depending on the location and amount of capillary nonperfusion and involvement of the macula and optic disc. Although there are isolated case reports of successful treatment using high-dose intravenous steroids and hyperbaric oxygen therapy, the majority of patients show variable visual recovery without specific treatment.7
We present the clinical course of three eyes of two cases with PLR that revealed OCT findings not described before.
A 39-year-old male presented with complaint of decreased vision in both eyes, starting a week ago, 2 days after a left femur fracture. His best-corrected visual acuity (BCVA) on presentation was 0.05 in the right eye and 0.1 (Snellen) in the left eye. Fundus examination revealed flame-shaped hemorrhage in the left eye and scattered cotton-wool spots and a whitish-appearance in the parafoveal retina bilaterally. Corresponding FA revealed subtle blocking defects over the focus of the intraretinal hemorrhage and the cotton-wool spots. Macular spectral-domain OCT (Topcon 3D OCT-2000 System; Topcon, Tokyo, Japan) scan demonstrated increased reflectivity in the inner plexiform (IPL), inner nuclear (INL), outer plexiform (OPL), and outer nuclear layers (ONL) in the parafoveal retina more obvious in temporal part. OCT scan of a cotton-wool spot showed a focal hyperreflectivity of the retinal nerve fiber and the ganglion cell layers (Figure 1). Past medical history evaluation of the patient did not reveal any abnormality.
Fundus photographs of a 36-year-old male patient (Case 1) with Purtscher-like retinopathy at presentation show scattered cotton-wool spots, parafoveal whitish-appearing retina bilaterally, and a flame-shaped hemorrhage in the left eye (a, c). Fluorescein angiography pictures show hypofluorescent areas clinically corresponded to the cotton-wool spots and the hemorrhage (b, d). Spectral-domain optical coherence tomography (SD-OCT) demonstrates the increased reflectivity in the inner plexiform, inner nuclear, outer plexiform, and outer nuclear layers in the parafoveal retina more obvious temporally (e, f). OCT scan of a cotton-wool spot shows focal hyperreflectivity of the retinal nerve fiber and the ganglion cell layers (g).
Despite contradictions regarding the treatment of PLR and the late presentation of the patient, we decided to refer him to hyperbaric oxygen therapy (HOT) in order to help to increase retinal oxygenation, since parafoveal retina was involved. HOT sessions at 2.5 atm for 120 minutes were given once a day for 20 courses.
At the 1-month examination performed after 20 sessions of HOT, BCVA was 0.33 in the right and 0.4 in the left eye, the number and size of cotton-wool spots had decreased, hyperreflectivity of the outer retinal layers had disappeared, and increased reflectivity of the inner layers had decreased significantly bilaterally. At the 3-month examination, BCVA was 0.33 at the right and 0.5 at the left eye. Fundus examination revealed a few number of the cotton-wool spots residues. Hyperreflectivity areas on the macular OCT completely disappeared except an area in the inner plexiform layer in the right eye. Temporal retina was thinner than nasal retina on the horizontal OCT scan of the macula (Figure 2).
At 3 months, fundus examination of Case 1 revealed a few number of the cotton-wool spots residues (a, b). Hyperreflectivity areas on macular optical coherence tomography (OCT) completely disappeared. Note that the parafoveal temporal retina is thinner than the nasal retina on macular OCT (c, d, e, f).
A 63-year-old male presented with decreased vision in the right eye of 2 days duration. His BCVA was 0.05 in the symptomatic right eye and 0.8 in the left eye. Fundus examination of the right eye revealed scattered cotton-wool spots and a faint whitish-appearance in the parafoveal retina. The left eye fundus examination was unremarkable. FA showed subtle hypofluorescent areas that corresponded to the cotton-wool spots seen clinically in the right eye. SD-OCT performed at the initial presentation revealed increased reflectivity in the IPL, INL, OPL, and ONL in the right parafoveal retina (Figure 3). Medical history evaluation and systematic physical assessment of the patient did not reveal any abnormality. Mild increase of erythrocyte sedimentation rate (20 mm/hour) was detected in laboratory tests. The patient was referred to HOT. HOT sessions at 2.5 atm for 120 minutes were given once a day for 20 courses. At the 1-month examination, BCVA increased to 0.6 in the right eye, the number and size of cotton-wool spots decreased, hyperreflectivity of the outer retinal layers disappeared, and increased reflectivity of the inner layers decreased significantly. At the last examination of the patient performed at 12 months after presentation, BCVA was 0.8 in the right eye. Fundus examination was unremarkable. Hyperreflectivity areas on OCT completely disappeared. Macular OCT showed parafoveal retinal atrophy (Figure 3).
Fundus photograph of the right eye of 63-year-old male patient (Case 2) with Purtscher-like retinopathy at presentation shows scattered cotton-wool spots and parafoveal mild, whitish-appearing retina (a). Spectral-domain optical coherence tomography (SD-OCT) scan demonstrates increased reflectivity in the inner plexiform, inner nuclear, outer plexiform, and outer nuclear layers in the parafoveal retina (c). Fundus examination was unremarkable (b), and SD-OCT macular scan shows controlled retinal atrophy at 12 months (d).
Histopathological examination on a patient with PLR revealed fibrin-positive occluding material in retinal and choroidal vessels and focal areas of retinal edema, cystoid degeneration, loss of photoreceptors, and loss of architecture in the inner retinal layers with abrupt transition to normal retina.19 OCT findings regarding the involvement of these layers have been previously reported. Alasil et al. described focal retinal nerve fiber layer swelling that clinically correspond to the cotton-wool spots and central subretinal fluid in a Purtscher-like retinopathy case. The authors hypothesized that microinfarction involving retinal capillary supplying retinal nerve fiber layer and choriocapillaris were the causes of this findings.14
Increased hyperreflectivity in the IPL, INL, and OPL that correspond to a parafoveal polygonal retinal whitening area were described in a patient with pancreatic cancer who presented with PLR. The authors claimed that this lesion was resulted from relative ischemia in the deep capillary bed that was supplied by an embolized arteriole.15 Similar OCT findings were reported by Chen et al. in a 29-year-old patient with PR. They hypothesized that these findings were resulted from ischemia of the intermediate and deep capillary plexuses. They postulated that the lesion is paracentral acute middle maculopathy (PAMM),20 a recently characterized presentation of deep retinal capillary ischemia, manifesting as hyperreflective bands within the middle retina on spectral-domain OCT imaging.21,22
OCT scans of our patients showed all previously described OCT findings except intraretinal and subretinal fluid. Despite probable common pathophysiology (deep retinal capillary ischemia), we did not evaluate our patients as PAMM patients since the presence of hyperreflectivity in the outer nuclear layers on OCT.
Based on previous reports, our findings, and the histopathology studies, it is possible to claim that clinical findings in PLR cases may vary depend on involved retinal or choroidal capillary vessels.
Histological studies identified that the macular retina have three capillary plexuses that lie at the level of the retinal nerve fibers (superficial capillary plexus), at the inner boundary of the INL (middle capillary plexus), and at the deep boundary between the INL and OPL (deeper capillary plexus).23,24 Separate or simultaneous involvement of these plexuses and choriocapillaris might lead to observation of different combinations of the OCT findings discussed above.
Although well-known theory that the metabolic needs of the outer retina, extending from the outer portion of the INL through the retinal pigment epithelium (RPE) are met by the choriocapillaris,25 the ONL involvement without subretinal fluid in our patients give rise to reasonable grounds for suspicion about probable role of the deeper capillary plexus in the blood supply of the outer nuclear layer.
Dysfunction of the overlying RPE due to affected choriocapillaris was proposed theory for subretinal fluid in patients with PLR. The lack of subretinal fluid in our patients might also be explain with differences of RPE capacity or extent of involved area.
It is interesting that no parafoveal nerve fiber layer or ganglion layer involvement, both supplied by superficial capillary plexus, has been reported yet. Possible different structure of parafoveal superficial capillary plexus might be the reason for this exemption. OCT angiography (OCTA), a recently developed, noninvasive, dye-less imaging modality, might answer this question in the future. Since current OCTA software have separated the retinal capillaries into two plexuses — a superficial capillary plexus (SCP) and a deep capillary plexus (DCP) — and incorporates segments of the middle capillary plexus into either the SCP or DCP.
At present, there are no definite guidelines to the treatment of PR or PLR, and there are only isolated case reports of successful treatment with steroids and HOT. Because of possible role of capillary ischemia in the pathophysiology of PLR we decided to refer our patients to HOT. HOT is breathing 100% oxygen while under increased atmospheric pressure. When a patient is given 100% oxygen under pressure, hemoglobin is saturated, but the blood can be hyperoxygenated by dissolving oxygen within the plasma. This state of serum hyperoxia causes an increase of oxygen delivery to ischemic tissues. It was demonstrated that HOT at 2 atm markedly increased the preretinal oxygen tension26 and that a hyperoxygenated choroid can supply the oxygen requirements of the whole retina in spite of the hyperoxic retinal vasoconstriction.27 Visual function improvement after HOT in our elder patient with earlier presentation and intervention was better. However, since his findings was milder than the young patient and the possibility of spontaneous recovery, it is difficult to draw conclusion that HOT has beneficial effect in early presented cases or patients with PLR at all.
The potentially weakness of this study is that we could not identify the reason for PLR in the elder patient. However, because of similar findings of both patients in fundus and OCT examination at presentation and follow-up, we decided that the second patient is also a PLR case.
To our best knowledge, hyperreflectivity of the ONL on OCT was not previously described in patients with PLR. These findings are with accordance of histopathological findings and support the microvascular embolization theory in pathophysiology of PLR.
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