- A 26-year-old Hispanic male presented with sudden monocular vision loss in the left eye. Examination found normal vision in the right eye but reduced vision in the left eye with retinal hemorrhage. - Differential diagnoses included Valsalva retinopathy, papillophlebitis, retinal vasculitis, or frosted branch angiitis. Further testing found no evidence of infection, autoimmune disease, or hypercoagulability. - Over subsequent visits, the retinal hemorrhage resolved but ischemic changes remained nasally. The appearance was consistent with a venous occlusion. Calcium deposits developed nasally resembling Kyrieleis plaques. Vision improved to normal in the left eye

1. Popeye Retinopathy
Dr Giulio Bamonte- Dr.ssa Rossella Melchionda

2. 13-5-2019 Paziente Ispanico di 26 anni maschio, si presenta in PS con
calo del visus da un giorno in OS, prima intermittente con flash e poi
dalla mattina permanente.
L’OD e’ normale.
Visus OD 10/10 nat
Visus OS 1/50 nmcl,
tono 11mmHg OO.
Anamnesi generale, oculare e familiare negativa per patologie degne di
nota.
Va tutti i giorni in Palestra.
DD: Valsalva Retinopathy, viene inviato al Chirurgo VR. Si esegue
Retinografia e OCT.

3. 13-5-2019

4. Valsalva Retinopathy
• Particular form of retinopathy, pre-retinal and hemorrhagic in nature,
secondary to a sudden increase in intrathoracic pressure.
• It was used to describe retinal hemorrhages in association with heavy
lifting, coughing, straining at stool, or vomiting.
• Valsalva retinopathy classically manifests as preretinal hemorrhage
secondary to rupturing of superficial retinal vessels caused by physical
exertion.
• The mechanism of a Valsalva maneuver is characterized by a sudden rise in
intrathoracic or intraabdominal pressure against a closed glottis, which
leads to a rapid rise of intravenous pressure within the eye, causing
retinal capillaries to spontaneously rupture.

5. DD of Valsalva
Retinopathy
Anemic Retinopathy
Diabetic Retinopathy
Hemorrhagic Posterior Vitreous Detachment
Hypertensive Retinopathy
Ocular Parasitic Infection
Purtscher Retinopathy and Purtscher-like
Retinopathy
Retinal Macroaneurysm
Retinal Vein Occlusion (RVO)
Sickle Cell Retinopathy
Terson Syndrome

6. 14-5-2019 Alla visita VR oltre quanto visibile in Retinografia si apprezza
nei quadranti superiori un aspetto vascolare ristretto, con vasi a
salsicciotto.
Il segmento anteriore e’ in quiete. Si invia per FAG, visita con esperto
Uveiti e consulto internistico.
Si esegue FAG.

7. 14-5-2019

8. 15-5-2019 Il paziente avverte un leggero discomfort su OS. Situazione
oculare invariata. Si consiglia solo riposo. L’esperto di uveiti pone la
seguente DD: problema vascolare tipo papilloflebite (Ipertensione,
dislipidemia, coagulopatie), problema infiammatorio (meno probabile,
Sarcoidosi, Hiv, TBC, Sifilide, MS, Bechet, Chron, PAN).

9. Papillophlebitis
• Papillophlebitis is a condition in which the clinical features of central retinal
vein occlusion (CRVO) are present but there is no history of vascular
disease.
• Papillophlebitis is characterized by painless unilateral disc edema and
hyperemia, retinal venous engorgement, and a variable extent of
intraretinal hemorrhage and macular edema in otherwise healthy adults
younger than 50 years.
• Papillophlebitis is often associated with systemic vascular disease (eg,
arterial hypertension, diabetes) or hematologic disorders.
• Corticosteroids, both systemic and periocular, are the mainstay of
treatment and are often coupled with anticoagulants such as heparin
and/or aspirin to reduce inflammation and treat any underlying
coagulopathies

10. Papillophlebitis
Q: When and for whom is thrombophilia testing appropriate?
A: Because papillophlebitis can be the first sign of an underlying
connective tissue disorder (eg, lupus, rheumatoid arthritis), young
women may benefit from a hematologic rheumatologic referral.
Screening for hypercoagulable status may include the following:
• Antiphospholipid antibodies and hyperhomocysteinemia;
• Factor V Leiden and prothrombin G20210A mutations; and
• Additional thrombotic risk factors such as pregnancy.

11. 20-5-2019 Al controllo si apprezzano 1+ cell in CA + depositi edoteliali. Il
vitreo appare limpido. Per il resto invariato.
Si instaura tp locale con Prednisolone collirio 6 al di e unguento prima di
dormire + cyclolux 2 al di.
22-5-2019 1+ cell in CA e depositi endoteliali, cellule in CV (globuli rossi,
spill over?).
Visus OS 2/10 con miglioramento dell’emorragia, che sta liberando il
centro della macula.
I vasi sembrano obliterati sia nasalmente che temporalmente, possibile
vasculite. Le arteriole sono ristrette, sembra aspetto a vasculite venosa.

12. Visita Internistica: Pz Ecuadoregno. Ultimo viaggio un anno prima. Un
anno prima negativo STD. No contatti TB. Anamnesi prossima negativa.
Anamnesi remota positiva per polmonite a 5 mesi di vita. Nega
assuzione di droghe e alcol (sociale). In un relazione fissa da 6 anni.
Esame fisico e di Laboratorio negativo

13. Visita internistica 20-5-2019
• Controles: RR 130/93, pols 62/min, Lengte: 162cm, gewicht 72kg (met kleding), BMI 27, H/H: geen lymfadenopathie in de
hals of supraclaviculair. Inspectie keel: geen roodheid, geen zwelling. Cor: S1S2, geen souffle Pulm: beiderzijds VAG zonder
bijgeluiden Abd: soepel, NP, WT, geen drukpijn, geen hepatosplemomegalie Extr: soepele kuiten, geen oedemen Geen
huidafwijkingen, behoudens enkele puistjes op de thorax. Geen afwijkingen aan de nagels.
• Laboratorium 16-06 Ureum 4.1, Kreatinine 82, natrium 142, kalium 4.3, ASAT 26, ALAT 30, LD 161, Bilirubine 9, Alk Fos 81,
Hb 10.0, Leukocyten 7.5, Trombocyten 238, Bezinking 2, CRP <1, Ferritine 131, Vit B12 323, Foliumzuur 11.4
• Conclusie: Retinabloeding links met atypisch beeld bij oogheelkundig onderzoek mogelijk passend bij vasculitis dan wel
veneus occlusie beeld DD - Cardiovasculair (HT, dyslipidemie) - Trombus bij hypercoagulabiliteit (factor V Leiden,
homocysteinemie, antifosfolipiden) - Papillophlebitis t.g.v. inflammatie, echter inflammatieparamters laag (vasculitis
ihkv M. Behcet, Crohn, PAN, sarcoïdose, MS, SLE; infectieus ihkv HIV, CMV, Syfilis, toxoplasmose, TB (Eales disease),
virale hepatitis)
• Beleid: Aanvullend lab: lipidenspectrum, HbA1c, nuchter glucose - USED + totaal eiwit, kreatinine, microalbumine -
Controle 1-2 weken met Dynamap vooraf - Te bespreken: ANA/ANCA, factor V Leiden, homocysteine, antifosfolipiden -
PM: TB, HIV, CMV, toxoplasmose screening
• 22-5-2019 Lijkt een veneus probleem (DD trombotisch danwel inflammatoir), overige is er secundair aan. - Vandaag nog
prikken: antifosfolipidensyndroom (Lupus anticoagulans, anti cardiolipine, beta-2 glycoproteine), complement (C1q, C3,
C4), ANA screening, HIV, Lues serologie - X-thorax bij nog sarcoïdose in de DD - Controle a.s. maandag reeds gepland -->
dan 24-uurs bloeddrukmeting regelen ter uitsluiting nachtelijke hypertensie - Geen antistolling, geen reden voor prikken
stollingsfactoren

14. Visita internistica 27-5-2019
Dynamap: RR 115/65 mmHg Lab: Ur 4.1 Kreat 88
Leverchemie nl Hb 10 MCV 87 T 238 L 7.5 BSE 2 INR 1.0 APTT 26 Hba1c 35
Lipiden: Cholesterol 4.6 TG 1.0 LDL 3.6
APS: beta 2 glycoproteine, cardiolipine IgM/IgG negatief. Lupus anticoagulans volgt
Complement: volgt ANA screening: < 0.5 Sarcoidose: ACE, S-IL2R volgt
Used: leuko's, eiwit, ery's negatief. MMB
HIV: negatief T.Pallidum negatief. TB quantiferon negatief.
X-Thorax: Normale thorax. Geen aanwijzing voor sarcoïdose.
Conclusie: - Cardiovasculair DD Hypertensie. Geen dislypidemie (LDL 3.6) > 24u BD meting volgt
DD Trombus bij hypercoagulabiliteit ikv factor V Leiden, homocysteinemie, antifosfolipiden >cardiolipine, betaglycoproteine neg, lupus anticoagulans volgt
- Retinale vasculitis, echter BSE laag (2) DD auto-immuun mgk ihkv M. Behcet, Crohn, PAN, sarcoïdose, MS, SLE, GCA
> ANA negatief > s-IL2R/ACE volgt > complement volgt
DD infectieus, mgk ihkv CMV, Toxoplasmose, HSV/VZV, CMV). > HIV/Lues/ TB serologie negatief
Beleid - 24u bloeddrukmeting ter uitsluiting nachtelijke hypertensie PM volgende keer dieetadvies (LDL 3.6)
PM nog FVL en homocysteine bepalen? --> iom Soonwala, niet nodig, gezien geen behandelconsequenties (geen zin om antistolling te starten)

15. 27-5-2019 Visita Oculistica con FAG: nasalmente si apprezza ischemia,
ma l’area e’ ancora coperta dal sangue. Aspetto a Frosted Branch
Arteritis (che pero’prende sia vene che arterie)? In CA le cellule sono
diminuite. Tp steroidea locale a scalare.

16. 22-5-2019
27-5-2019

17. Frosted Branch Angiitis
• Usually bilateral
• Specific Syndrome (primary form): rare, children or young adults
• Common immune pathway in responde to multiple infective agents:
CMV retinitis.
• Oher conditions suchs glomerulonefritis and CRVO

18. Frosted Branch Angiitis
Visual Acuity usually very poor
Florid translucent retinal perivascular
sheating of both arteriooles and venules
Anterior uveitis, vitritis and retinal oedema
Papillitis, hard exudates, retinal hemorrhages
and venous occulsion
TP is with steroid.

19. 28-5-2019 Consulto con collega dell’Universita’ di Leiden: sospetta
Valsalva in un caso di Retinopatia Purtscher che tuttavia da’ una
problematica di solito a livello del polo posteriore, qui il problema e’
nasale (papillo flebite/crvo).

20. Purtscher Retinopathy
• Purtscher retinopathy is a hemorrhagic and vasoocclusive
vasculopathy, which, was first described as a syndrome of sudden
blindness associated with severe head trauma. These patients had
findings of multiple white retinal patches and retinal hemorrhages
that were associated with severe vision loss.
• Purtscher (and Purtscher like) retinopathy has been associated with
traumatic injury, primarily blunt thoracic trauma and head trauma,
and numerous nontraumatic diseases.

21. DD of Purtscher
Retinopathy
• Acute Pancreatitis;
• fat embolization;
• amniotic fluid embolization;
• preeclampsia;
• hemolysis, elevated liver enzymes, and
low platelets (HELLP) syndrome;
• and vasculitic diseases, such as lupus.
Patients with known vasculitic disease
(eg, systemic lupus erythematosus,
scleroderma, dermatomyositis) are at risk
for developing a Purtscher-like
retinopathy with microvascular
occlusion

22. Pathophysiology of Purtsher Retinopathy
• These lesions are known as Purtscher flecken (larger infarcts of the
retinal capillary bed) and cotton-wool spots (small retinal
microinfarcts at the level of the nerve fiber layer).
• Fluorescein leakage in Purtscher retinopathy suggests that an acute
endothelial cell injury is caused by trauma, possibly predisposing
the retinal vessels to occlusion

23. Pathophysiology of Purtsher Retinopathy
• The condition has been associated with various vasculopathies.
• The most accepted mechanism is leukoembolization that causes
arterial occlusion and infarction of the microvascular bed.
• Other possible sources of emboli include fat emboli, amniotic fluid,
air emboli, and granulocyte aggregation resulting from complement
activation.

24. Pathophysiology of Purtscher Retinopathy
• Other proposed mechanisms of vascular occlusion include angiospasm
resulting from an acute rise in venous pressure from compressive chest
injuries or possibly acute head injuries and endothelial cell damage
resulting from acutely increased intraluminal pressure.
• Valsalva Retinopathy Pathophysiology:
1. a sudden increase in intrathoracic pressure decreases venous return to the right
side of the heart.
2. diminished cardiac filling lowers the mean arterial pressure, slowing the pulse,
leading to reflex tachycardia and peripheral vasoconstriction.
3. release of the strain causes a prompt reduction in the intrathoracic pressure,
further lowering the blood pressure and simultaneously increasing the cardiac
pressure.
4. an abrupt increase in blood pressure occurs as venous blood surges back to the
heart, inducing reflex bradycardia.

25. Related Conditions
Related Conditions and Diseases
• Multifocal Chororidopathy Syndromes ;
• Retinal Artery Occlusion;
• Neuroophthalmic manifestations of
vascular eye diseases;
• Hemoglobinopathy Retinopathy
• Eales Disease
• Hiv Retinopathy
• Drug Induced
• Hypertension…

26. 6-6-2019
OD aspetto retinico normale, le vene sembrano un poco congeste.
OS Il sangue continua a riassorbirsi. Nasalmente si apprezza ischemia,
l’aspetto e’ quello di una occlusione venosa di branca. Aspetto a Frosted
Branch Arteritis.
Tp steroidea locale sospesa.
29-6-2019
OS l’emorragia maculare si é completamente riassorbita, il visus
e’risalito a 10/10. Nasalmente si apprezza ischemia.

27. 11-08-2019
Va tutto bene, i sintomi sono completamente rientrati, il visus e’ 10/10.
Al fondo l’ OD e’ normale con leggera congestione venosa.
In OS il polo posteriore e’ praticamente normale, al livello della papilla
un piccolo tuft emorragico e vasi fantasma. Nasalmente si apprezzano
emorragie preretiniche.
I vasi nasalmente presentano come delle calcificazioni biancastre al loro
interno tipo: Kyrieleis Plaques

28. 14-08-2019

29. Kyrieleis Plaques
Kyrieleis plaques are segmental
periarteriolar inflammatory plaques that
occur in various diseases such as toxoplasma
retinochoroiditis, cytomegalovirus retinitis,
and Susac syndrome
Although first described as of part of a
Tubercolosis related retinitis by Dr Werner
Kyrieleis, calcific plaques on the walls of
blood vessels, have become associated with
toxoplasmosis retinitis.
Labo in our patients shows however IgG + for
Toxo but not IgM
DD SLE, PAN, Lues, HSV, VZV, IRVAN, Churg
Strauss syndr, Sarcoidose, relapsing
polychrondritis, Wegener, Crohn, Frosted
branch angiitis

30. 30-08-2019
Continuiamo a pensare a una Toxo, l’unica lesione che ce lo fa supporre
e’una area depigmentata sotto la papilla correlata tra l’altro a un difetto
superiore del CV.
Pensiamo di fare un puntura in CA per PCR toxo e Herpes ma ci viene
sconsigliata dai colleghi piu’ esperti dell’universita’ di Leiden.

32. 9-9-2019

33. 20-09-2019
Il paziente non ha piu’ sintomi, e’ contento ma non abbiamo una
diagnosi.
IL 6 Novembre e’ programmato per un argon laser della zona ischemica
paranasale.
L’ipotesi piu’ probabile e’ una Periflebite su base infiammatoria o
vascolare (es Eales Disease in assenza di TB, Toxo, Herpes, Occlusione
venosa) oppure danno microvascolare da rialzo acuto di pressione
dovuto al sollevamento pesi, tipo Retinopatia di Purtscher, complicato
da una Maculopatia Valsalva.

34. 3-10-2019
O piu’ probabilmente …..non ci abbiamo capito niente!
Grazie per l’attenzione!!