Acute pancreatitis with Purtscher's retinopathy: case report and review of the literature

doi:10.1016/S1590-8658(00)80338-2

Digestive and Liver Disease

Volume 32, Issue 8, November 2000, Pages 729-732

https://doi.org/10.1016/S1590-8658(00)80338-2 Get rights and content

Abstract

The case is described of a 32-year-old man suffering from alcoholism who came to the Emergency Unit with vomiting, fever, and sharp epigastric pain irradiating to the chest and upper abdomen. A diagnosis of acute pancreatitis was made after high amylase and lipase levels were observed and the results of computed tomography scan revealed images typical of acute pancreatitis. Findings upon admission and after the initial 48 hours did not correlate with a severe or complicated course according to Panson's criteria. On the third day after admission he suddenly developed decreased vision. A fluorescein angiogram showed arteriolar occlusion, retinal and choriocapillary ischaemia. Purtscher's retinopathy was suspected. After 4 weeks, the patient had recovered from acute pancreatitis, ophthalmoscopic examination showed normal results, and visual acuity had almost returned to normal. Activation of complement in acute pancreatitis could account for many haematologic acute disorders due to leucocyte emboli or other complement-mediated aggregates. Coagulation abnormalities may range from isolated intravascular thrombosis to severe disseminated intravascular coagulation. Purtscher's retinopathy, due to microembolizations in the choroidal and retinal arterioles, should be included among the various systemic effects of acute pancreatitis. This visual disorder is a rare systemic manifestation of acute pancreatitis which was not correlated to a severe or complicated clinical course. Treatment of these ocular complications remains to be established and outcome, therefore, depends upon resolution of the pancreatic disease.

References (17)

G. Slater et al.
Renal cortical necrosis and Purtscher's retinopathy in hemorrhagic pancreatitis
Am J Med
(1984)
V.D. Stoumbos et al.
Purscher's-like retinopathy in chronic renal failure
Ophthalmology
(1992)
B.A. Blodi et al.
Purtscher-like retinopathy after uncomplicated administration of retrobulbar anesthesia
Am J Ophthalmol
(1997)
D.M. Inkeles et al.
Retinal fat emboli as sequela to acute pancreatitis
Am J Ophthalmol
(1975)
N. Agarwal et al.
Acute pancreatitis: a multisystem disease
Gastroenterologist
(1993)
J.C. Lai et al.
Complement-induced retinal arteriolar occlusions in the cat
Retina
(1997)
O. Purtscher
Angiopathia retinae traumatica. Lymphrragien des Augengrundes
Albrecht von Graefe Arch Ophth
(1912)
M.C. Kincaid et al.
A clinicopathological case report of retinopathy of pancreatitis
Br J Ophth
(1982)

There are more references available in the full text version of this article.

Cited by (30)

Retino-choroidal changes in patients with acute pancreatitis: A prospective analysis of a novel biomarker
2020, Pancreatology
Citation Excerpt :
This entity is a rare feature of AP presenting with sudden loss of vision and occurs due to complement activation resulting from activated pancreatic enzymes. The activated complement system causes granulocyte aggregation and subsequent retinal micro-embolization leading to retinal vasculopathy [6–8]. However, besides Purtscher’s retinopathy, the fundus changes in AP have been rarely studied in details.
There is paucity of data on ocular changes in acute Pancreatitis (AP). Moreover, subclinical alterations in retina & choroid have not been studied in AP.
To prospectively study retino-choroidal changes in AP.
Sixty patients (mean age 39.07 years; 41 males) with AP were followed up till recovery/death. Baseline slit-lamp examination, choroidal thickness (CT), retinal thickness (RT), choroidal vascularity index (CVI), retinal capillary density index (CDI) and arteriovenous ratio (AVR) were recorded. The patients were divided into two groups – mild (Group A; 5 patients) and moderately severe/severe (Group B; 55 patients) as per revised Atlanta classification.
Fundus examination showed mild optic disc edema with retinal hemorrhages in 6 (10%) patients in group B as compared to none in group A (p = 1.00). None of the patients had Purtscher retinopathy. Mean CT (317 ± 56.29 μm) was increased as compared to normal subjects (278.90 ± 57.84 μm, p = 0.003). The mean CVI (0.62 ± 0.04) was decreased as compared to normal (0.66 ± 0.01, p < 0.0001) as was the mean AVR (0.67 ± 0.03 vs. 0.7 ± 0.02, p < 0.0001). However, the mean RT of subjects with AP (239.68 ± 33.76 μm) was not significantly different compared to the normal subjects 253.17 ± 33.67 µm (p=NS). The mean CDI of superficial and deep plexus were comparable between normal and patients with AP. CT, RT, CVI, AVR and CDI were comparable between group A and group B as well as survivors and non-survivors.
Clinically significant ocular changes are seen infrequently in AP. However, subclinical changes in CT, CVI and AVR are observed in patients with AP compared to normal individuals.
Acute Pancreatitis
2010, Sleisenger and Fordtran’s Gastrointestinal and Liver Disease- 2 Volume Set: Pathophysiology, Diagnosis, Management, Expert Consult Premium Edition - Enhanced Online Features and Print
Purtscher-like retinopathy: Case report and review of the literature
2008, Journal Francais d'Ophtalmologie
J. Proença Pina, K. Ssi-Yan-Kai, I. de Monchy, B. Charpentier, H. Offret, M. Labetoulle
Purtscher’s retinopathy is a rare condition, seen in patients with a history of trauma. Purtscher-like retinopathy includes the nontraumatic causes of this retinal disease, which is often misdiagnosed. The diagnosis is based on clinical settings and is supported by intravenous fluorescein angiography. We describe a case of acute, severe retinopathy in a patient experiencing a scleroderma renal crisis. We discuss the pathogeny, prognosis and treatment, which is still widely controversial.
La rétinopathie de Purtscher est une atteinte rare survenant dans un contexte traumatique. La pseudo-rétinopathie de Purtscher regroupe l’ensemble des atteintes rétiniennes sémiologiquement similaires au syndrome initialement décrit en 1910 par Purtscher, mais survenant dans un contexte non traumatique. Cette rétinopathie est largement méconnue. Son diagnostic est clinique et angiographique. Nous rapportons le cas d’une pseudo-rétinopathie de Purtscher bilatérale survenue dans le cadre d’une crise rénale sclérodermique chez une femme atteinte d’une sclérodermie généralisée. Sur la base d’une revue de la littérature, nous discutons la physiopathogénie de ce syndrome ainsi que sa signification pronostique. Le traitement quant à lui ne fait pour l’instant pas l’objet d’un consensus.
Proteolytic cleavage of carboxypeptidase N markedly increases its antifibrinolytic activity
2008, Journal of Thrombosis and Haemostasis
Background: Carboxypeptidase N (CPN) is a constitutively active basic carboxypeptidase sharing specificity with activated thrombin‐activable fibrinolysis inhibitor (TAFIa). Generally, CPN is regarded as being non‐antifibrinolytic. However, this assumption has not been thoroughly investigated, particularly with respect to long‐term antifibrinolysis. In addition, a recent report has shown that plasmin cleavage increases the catalytic activity of CPN. Therefore, we investigated the antifibrinolytic properties of CPN and plasmin‐cleaved CPN (CPNc). Methods: CPN was incubated with plasmin for various periods of time and the prolongation of clot lysis at various concentrations of CPN/CPNc mixture was investigated in TAFI‐depleted plasma. CPN cleavage was analyzed by electrophoresis and catalytic activity was determined by monitoring cleavage of the small substrate, FA‐Ala‐Lys. Results: CPN exhibited antifibrinolytic properties in plasma clot lysis assays when present at supraphysiological concentrations. Depletion of CPN from plasma decreased the lysis time of clots formed from minimally diluted plasma at low tissue‐type plasminogen activator (t‐PA) concentrations. Plasmin cleavage of CPN markedly increased the antifibrinolytic properties. CPN and CPNc prolonged lysis in a non‐saturable, dose‐dependent, and t‐PA‐dependent manner. At sufficient concentration, CPN and CPNc prolonged lysis at least forty‐fivefold. CPNc was 700% more antifibrinolytic than CPN but only 7% more active toward FA‐Ala‐Lys. The active site inhibitor GEMSA eliminated the antifibrinolytic effects of CPN and CPNc. Antifibrinolytic activity correlated with cleavage of active and/or regulatory subunits, presumably generating heterodimeric CPNc. Conclusions: Limited proteolysis of CPN by plasmin generates an enzyme with greatly increased antifibrinolytic properties. We speculate that (patho)physiological proteolysis of CPN may generate a long‐term antifibrinolytic enzyme.
Purtscher's and Purtscher-like retinopathies: A review
2006, Survey of Ophthalmology
Citation Excerpt :
Despite initial visual loss, the acuity recovered without treatment. Since then, in addition to various types of trauma, a similar retinal appearance has also been described in a variety of conditions including acute pancreatitis, fat embolism syndrome, renal failure, childbirth, and connective tissue disorders (Table 1).2,3,5,7–10,14,15,17,21,23–25,28,30,31,35,36,42,44–46,48 The term “Purtscher-like retinopathy” is sometimes used to describe the retinopathy seen in conditions other than trauma.
Purtscher's retinopathy is a rare condition, seen in patients with a history of trauma or other systemic disease. The clinical appearance would suggest embolic occlusion of the pre-capillary arterioles as the most likely pathogenesis, although other factors leading to blood-vessel damage may also contribute. The diagnosis is made on clinical grounds and supported by intravenous fluorescein angiography. Without treatment, the majority of patients recover some of their visual function. Treatment with systemic steroids may improve visual outcome in some patients but at present there is little evidence to support such treatment routinely.
Purtscher's retinopathy: A rare complication of acute non-alcoholic pancreatitis
2003, Gastroenterologia y Hepatologia
La retinopatía de Purtscher es una complicación infrecuente de la pancreatitis aguda alcohólica que se caracteriza por la aparición de exudados algodonosos y hemorragias localizadas en torno al disco óptico. Su aparición responde a fenómenos isquémicos en el polo posterior retiniano por microembolias en las arteriolas y capilares retinianos. Esta complicación no se relaciona con un peor pronóstico evolutivo de la pancreatitis aguda y la evolución de la retinopatía es habitualmente favorable, con desaparición de las lesiones oftalmológicas y normalización de la agudeza visual a las 4-6 semanas en la mayoría de los casos. Sin embargo, en algunos casos pueden quedar lesiones residuales, especialmente si se produce atrofia óptica. Presentamos el caso de una paciente de 40 años con pérdida de agudeza visual en el curso de una pancreatitis aguda leve idiopática por retinopatía de Purtscher.
Purtscher's retinopathy is a rare complication of acute alcoholic pancreatitis that is characterized by the development of cotton-wool exudates and retinal hemorrhages located around the optic disk. Its development is due to ischemic phenomena in the posterior pole of the retina due to microemboli in the retinal arterioles and capillaries. This complication is not related to a poorer prognosis of acute pancreatitis and the retinopathy usually has a favorable outcome with disappearance of the ophthalmic lesions and restoration of visual acuity after 4-6 weeks in most cases. However, some patients may show residual lesions, especially if optical atrophy occurs. We present a 40-year-old woman with loss of visual acuity due to Purtscher's retinopathy during the course of mild acute idiopathic pancreatitis.

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^∗: Dr. Campo's current position is at Gastroenterology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00139, Rome, Italy.

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Acute pancreatitis with Purtscher's retinopathy: case report and review of the literature

Abstract

Am J Med

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Acute pancreatitis: a multisystem disease

Gastroenterologist

Complement-induced retinal arteriolar occlusions in the cat

Retina

Angiopathia retinae traumatica. Lymphrragien des Augengrundes

Albrecht von Graefe Arch Ophth

A clinicopathological case report of retinopathy of pancreatitis

Br J Ophth