Ординатура / Офтальмология / Учебные материалы / Retinal Vascular Disease Joussen Springer

c

Fig. 28.3.2. Early (a) and late phase (b) of fluorescein angiography showing the features of a retinal vascular hemangioma: The telangiectatic lesions are demonstrated with hyperfluorescence of the upper half and hypofluorescence of the lower half without significant exudation in the late phase of fluorescein angiography

However, the presence of feeder vessels and a dilated feeder artery would be more typical for capillary hemangioma than for cavernous hemangioma, respectively [26].

Fluorescein angiography reveals a slow and incomplete filling of the cavernous hemangioma. Typically, separation of the plasma and the erythrocytes is detected during the late phases of fluorescein angiography, presenting hypofluorescence in the lower half and hyperfluorescence of the higher half of the vascular lesion (Fig. 28.3.2). This indicates a relatively isolated blood flow from the main stream of the retina [8]. In addition, extravascular leakage and arteriovenous shunts are typically not found in combination with cavernous hemangioma.

Cavernous hemangiomas involving the optic nerve usually represent similar clinical signs com-

pared to those of the periphery. Fluctuations in the size of the aneurysms have been suggested to be responsible for changes in visual function such as amaurosis [8]. Rarely a progression of the disease has been observed with growth and vitreous hemorrhages [19]. Echographic findings detected a dome shaped lesion with high reflectivity followed by signals with irregular reflectivity [31]. Optical coherence tomography revealed intraretinal cysts particularly of the inner retinal layer and signals with a high reflectivity [1].

The size of the aneurysms and the extent of the lesion have been reported to vary from case to case, and thrombosis with organization of the lesion has been observed during the follow-up [26]. However, the tumor usually only has minimal potential for changes during the follow-up [8].

Cavernous hemangioma of the retina may be associated with angiomatous vascular hamartomas of the brain and the skin, respectively [8, 33]. These cavernomas of the brain, CCMs, are defined as abnormally enlarged capillary cavities without intervening brain parenchyma. Intracranial hemorrhages, seizures, focal neurologic symptoms and sudden deaths may be the first signs of these localizations [10, 11]. In addition, choroidal hemangiomas have been diagnosed in two members of a family with autosomal dominant familial hemangiomas of the brain, skin and eye [33]. However, hereditary cerebral cavernous hemangiomas may vary according to the additional manifestation in the skin and eye [20, 33]. Examinations of 60 patients with a familial cerebral cavernoma revealed no manifestation on the skin in any out of 60 patients, while a retinal cavernous hemangioma was demonstrated in 3 out of 60 patients [20].

The incidence of solitary retinal cavernous hemangiomas is unknown due to the possibility of a fol-

low-up without any symptoms. However, retinal cavernous hemangiomas have been found to be present in 5 % of patients with familial cerebral cavernous malformations [20]. Familial cerebral cavernomas represent up to 10 % of the cerebral cavernous malformations, with a prevalence of 0.5 % in the general population [22]. Based on these findings, the incidence of retinal cavernous hemangioma has been estimated in up to 1 in 40,000 persons in the general population [20].

The age of patients with cerebral cavernous malformations and retinal cavernous hemangioma has been found similar to those without a retinal manifestation, with mean ages of 40.3 years (range 20.2 – 55.3 years) and 42.6 years (range 9.6 – 67.8 years), respectively [20].

Hereditary cases with an autosomal dominant pattern represented as CCMs have been associated with mutations in the CCM gene (OMIM 116860). They are classified as a distinct condition. Several genetic loci (CCM) have been identified. CCM1 has been shown to be associated with mutations in the KRIT1 gene on chromosome 7q11.2-q21 [12, 13, 16, 21, 25]. This encodes a microtubule-associated protein, which may play a role in endothelial cell function and vessel formation during angiogenesis [5, 14, 22]. CCM2 is caused by a mutation in the CCM2 (MGC 4607) gene (7p15-p13) normally encoding the protein malcavernin, while mutations in the PDCD10 (programmed cell death 10) gene (3q26.1) are responsible for CCM3 [4, 38 – 40]. Mutations of CCM2 and CCM3 have been suggested to induce disturbances in the angiogenesis (CCM2) and to be involved in the procedures of apoptosis (CCM3) [3, 6]. There is evidence for additional CCM loci, like the CCM4 locus on chromosome 3q26.3-27.2 [24].

A KRIT1/CCM1 truncating mutation [22] and a KRIT1/CCM1 splice-site mutation [17] have been found in a patient with cerebral and retinal angiomas. Recently, mutations in any of the three cerebral cavernous malformation genes, CCM/KRIT1, CCM2/MGC 4607, and PDCD10, have been detected in patients with cavernous hemangiomas of the retina and the brain [20].

ies, fluorescein angiography reveals a normal filling of the capillaries together with an atypical formation and dilatation of the capillaries. During the followup, leakage from the atypical capillaries is usually observed particularly in patients with symptoms and a decrease in visual acuity. In contrast, fluorescein angiography of cavernous hemangioma presents

the typical separation of the plasma and erythro- III 28 cytes with a low filling rate and without an exuda-

tion. As summarized by Gass [8], retinal telangiectasis represents a progressive disease which affects the retinal structure and the intrinsic retinal vasculature, while cavernous hemangioma is at least in part isolated from the normal capillary vessels.

Retinal capillary hemangioma may be differentiated typically from cavernous hemangioma due to the presence of feeder vessels, the supplying artery and the draining vein, the tortuosity of these vessels, the subretinal and intraretinal exudation, which may be associated with exudative retinal detachment, and the leakage during the fluorescein angiography. However, small retinal capillary hemangiomas may be difficult to detect and the feeder vessels may not be visible. In addition, retinal lesions of the retina may be associated with cerebral angiomas (von Hip- pel-Lindau syndrome) [34, 35, 37].

Racemous angioma may be associated with arteriovenous anomalies of the central nervous system (Wyborn-Mason syndrome). It represents dilated tortuous retinal vessels with a direct communication between the retinal veins and arteries, and typically without a capillary bed between the communicating vessels [34, 43].

28.3.6 Treatment

Cavernous hemangioma of the retina usually does not require any treatment. Rarely, pars plana vitrectomy may be indicated following vitreous hemorrhages [15]. However, usually the retinal cavernous hemangioma does not develop any progression. In addition, laser therapy and cryotherapy have not shown any significant benefits for the follow-up of cavernous hemangiomas [8, 18].

The following diseases may play a role in differential diagnosis of the retinal cavernous hemangioma:

Parafoveal telangiectasis may present a similar picture compared to cavernous hemangiomas particularly in early stages of the disease. However, intraretinal and subretinal exudation and an increase in vascular dilatation may be observed during the follow-up [9]. In addition, due to its direct visualization of the structure of the retinal capillar-

Cavernous hemangioma of the retina represents a benign vascular hamartoma. The typical clinical findings may help to differentiate this type of hamartoma from other retinal diseases. A significant progression of the disease and complications which may irreversibly threaten the visual function during the follow-up have been observed in only a few cases. Treatment is usually not required. Rarely pars plana vitrectomy may be taken into consideration if a

severe vitreous hemorrhage does occur without any signs of regression. Follow-up examinations may help to exclude complications within the eye due to the cavernous hemangioma of the retina.

In addition, cavernous retinal hemangioma may be associated with vascular hamartomas of the brain and the skin, representing a sporadic or hereditary

28 III CCM, which may induce intracranial hemorrhages, seizures, focal neurologic symptoms and may be responsible for life threatening complications. Therefore, clinical examinations of the patient and the family members are recommended to exclude this condition with extraocular manifestations in the skin and central nervous system.

References

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2.Bell D, Yang HK, O’Brien C (1997) A case of bilateral cavernous hemangioma associated with intracerebral hemangioma. Arch Ophthalmol 115:818 – 819

3.Bergametti F, Denier C, Labauge P, Arnoult M, Boetto S, Clanet M, Coubes P, Echenne B, Ibrahim R, Irthum B, Jacquet G, Lonjon M, Moreau JJ, Neau JP, Parker F, Tremoulet M, Tournier-Lasserve E (2005) Mutations within the programmed cell death 10 gene cause cerebral cavernous malformations. Am J Hum Genet 76:42 – 51

4.Craig HD, Gunel M, Cepeda O, Johnson EW, Ptacek L, Steinberg GK, Ogilvy CS, Berg MJ, Crawford SC, Scott RM, Stei- chen-Gersdorf E, Saboroe R, Kennedy CTC, Mettler G, Beis MJ, Fryer A, Award IA, Lifton RP (1998) Multilocus linkage identifies two new loci for mendelian form of stroke, cerebral cavernous malformation at 7p15 – 13 and 3q25.2 – 27. Hum Mol Genet 7:1851 – 1858

5.Denier C, Gase JM, Chapon F, Domenga V, Lescoat C, Joutel A, Tournier-Lasserve E (2002) Ktit1/cerebral cavernous malformation 1 mRNA is preferentially expressed in neurons and epithelial cells in embryo and adult. Mech Dev 117:363 – 367

6.Denier C, Goutagny S, Labauge P, Krivosic V, Arnoult M, Cousin A, Benabid AL, Cornoy J, Frerebeau P, Gilbert B, Houtteville JP, Jan M, Lapierre F, Loiseau H, Menei P, Mercier P, Moreau JJ, Nivelon-Chevallier A, Parker F, Redondo AM, Scarabin JM, Tremoulet M, Zerah M, Maciazek J, Tour- nier-Lasserve E (2004) Mutations within the MGC4607 gene cause cerebral cavernous malformations. Am J Hum Genet 74:326 – 337

7.Dobyns WB, Michels VV, Groover RV, Mokri B, Trautmann JC, Forbes GS, Laws ER Jr (1987) Familial cavernous malformations of the central nervous system and retina. Ann Neurol 21:578 – 583

8.Gass JDM (1971) Cavernous hemangioma of the retina. Am J Ophthalmol, 71:799 – 814

9.Gass JDM, Blodi BA (1993) Idiopathic juxtafoveolar retinal teleangiectasis. Update of classification and follow-up study. Ophthalmology 100:1536 – 1546

10.Gislason I, Stenkulla S, Alm A, Wold E, Walinder PE (1979) Cavernous hemangioma of the retina. Acta Ophthalmol (Copenh) 57:709 – 717

11.Goldberg RE, Pheasant TR, Shields JA (1979) Cavernous hemangioma of the retina. A four generation pedigree with neurocutaneous manifestations and an example of bilateral retinal involvement. Arch Ophthalmol 97:2321 – 2324

12.Gunel M, Award IA, Finberg K, Anson J, Lifton RP (1995) Mapping a gene causing cerebral cavernous malformations. Proc Nat Acad Sci USA 92:6620 – 6624

13.Gunel M, Laurans MS, Shin D et al (2002) KRIT1 a gene mutated in cerebral cavernous malformation, encodes a microtubule-associated protein. Proc Natl Acad Sci USA 99:10677 – 10682

14.Guzeloglu-Kayisli O, Amankulor NM, Voorhees J, Luleci G, Lifton RP, Gunel M (2004) KRIT1/cerebral cavernous malformation 1 protein localizes to vascular endothelium, astrocytes and pyramidal cells of the adult human cerebral cortex. Neurosurgery 54:943 – 949

15.Haller JA, Knox DL (1993) Vitrectomy for persistent vitreous hemorrhage from cavernous hemangioma of the optic disc. Am J Ophthalmol 116:106 – 107

16.Johnson EW, Iyer LM, Rich SS, Ott HT, Gil-Nagel A, Kurth JH, Zabramski JM, Marchuk DA, Weissenbach J, Clericuzio CL, Davis LE, Hart BL, Gusella JF, Kosofsky BE, Louis DN, Morrison LA, Green DE, Weber JL (1995) Refined localization of the cerebral cavernous malformation gene (CCM1) to a 4 cM interval of chromosome 7q contained in a well defines YAC contig. Genome Res 5:368 – 380

17.Kitzmann AS, Pulido JS, Ferber MJ, Highsmith WE, Babo- vic-Vuksanovic D (2006) A splice-site mutation in CCM1/ KRIT1 is associated with retinal and cerebral cavernous hemangioma. Ophthalmic Genet 27:157 – 159

18.Klein M, Goldberg MF, Cotlier E (1975) Cavernous hemangioma of the retina. Report of four cases. Ann Ophthalmol 7:1213 – 1221

19.Kushner MA, Jampol LM, Haller JA (1994) Cavernous hemangioma of the optic nerve. Retina 14:59 – 361

20.Labauge P, Krivosic V, Denier C, Tournier-Lasserve E, Gaudric A (2006) Frequency of retinal cavernomas in 60 patients with familial cerebral cavernomas. A clinical and genetic study. Arch Ophthalmol 124:885 – 886

21.Laberge-le Coteulx S, Jung HH, Labauge P, Houtteville J-P, Lescoat C, Cecillon M, Marechal E, Joutel A, Tournier-Las- serve E (1999) Truncating mutations in CCM1 encoding KRIT1 cause hereditary cavernous angiomas. Nature Genet 23:189 – 1993

22.Laberge-Le Couteulx S, Brezin AP, Fontaine B, TournierLasserve E, Labauge P (2002) A novel KRIT/CCM1 truncating mutation in a patient with cerebral and retinal cavernous angiomas. Arch Ophthalmol 120:217 – 118

23.Lewis RA, Cohen MH, Wise GN (1975) Cavernous hemangioma of the retina and optic disc. A report of three cases an a review of the literature. Br J Ophthalmol 59:422 – 434

24.Liquori CL, Berg MJ, Squiteri F, Ottenbacher M, Sorlie M, Leedom TP, Cannella M, Maglione V, Ptacek L, Johnson EW, Marchuk DA (2006) Low frequency of PDCD10 mutations in a panel of CCM3 probands: potential for a fourth CCM locus. Hum Mutat 27:118

25.Marchuk Da, Gallione CJ, Morrison LA, Clericuzio CL, Hart BL, Kosofsky BE, Louis DN, Gusella JF, Davis LE, Prenger VL (1995) A locus for cerebral cavernous malformations maps to chromosome 7q in families. Genomics 28:311 – 314

26.Messmer E, Laqua H, Wessing A, Spitznas M, Weidle E, Ruprecht K, Naumann GO (1983) Nine cases of cavernous hemangioma of the retina. Am J Ophthalmol 95:383 – 390

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(1984) Cavernous hemangioma of the retina. Immunohistochemical and ultrastructural observations. Arch Ophthalmol 102:413 – 418

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Core Messages

A nodular gliovascular proliferation causing exudation and fibrosis

Inferotemporal, pre-equatorial, pink-yellow mass or masses

Idiopathic or secondary to other ocular disease, such as uveitis

Treatment with cryotherapy, brachytherapy photodynamic therapy are angiogenic agents Visual loss in most patients, as a result of maculopathy

Enucleation may be necessary because of neovascular glaucoma

28.4.1 History

In 1982, Baines and associates described eight patients with inferotemporal, peripheral, retinal, telangiectatic nodules, associated with subretinal, exudates, macular edema, and epiretinal membranes [1]. Shields and colleagues initially called this condition “presumed acquired retinal hemangioma” but later renamed it “vasoproliferative retinal tumor” [7, 8]. Numerous single case reports and several case series have been published, with the disease described using a variety of terms [10].

28.4.2 Histological Features

Essentials

Spindle-shaped glial cells with eosinophilic cytoplasm and small nucleus

Dense capillary network and large, hyalinized blood vessels

Foreign body giant cells, macrophages and cholesterol clefts

Vasoproliferative tumor consists mostly of glial cells, which are spindle shaped and eosinophilic, with no nuclear pleomorphism, and which stain positively for glial fibrillary acidic protein (GFAP, Dako) (Fig. 28.4.1) [3, 4, 11]. There is a fine capillary network throughout the lesion, with these blood vessels staining positively with CD31 antibody. There are also dilated and hyalinized blood vessels, some of which are occluded. Histology also shows exudates, macrophages and foreign body giant cells.

Fig. 28.4.1. Light micrograph of vasoproliferative retinal tumor showing eosinophilic glial cells, capillary network, hyalinized blood vessels, macrophages and giant cells. (Courtesy of S.E. Coupland, Berlin)

28.4.3 Pathogenesis

The pathogenesis is not known. Approximately 75 % of cases are idiopathic and 25 % are secondary to other ocular diseases, such as retinitis pigmentosa, uveitis, retinal detachment, congenital toxoplasmosis and Coats’ disease (Fig. 28.4.2) [7, 9]. When such disease affects both eyes, multiple vasoproliferative lesions are more likely to develop. Some patients have bilateral vasoproliferative lesions in the absence of any apparent underlying ocular disease. Bilateral vasoproliferative tumors have been reported in a pair of monozygotic twins [12]. There is no sex preponderance. The condition can present at any age but is usually detected between the ages of 40 and 60 years. Diffuse tumors are rare, tending to arise in

young females and associated with more severe visual loss as well as neovascular glaucoma [7].

28.4.4 Clinical Features

Essentials

Yellow-pink retinal mass

Inferotemporal location in most cases

Pre-equatorial or at equator

Solitary, diffuse or multiple

Small retinal feeder vessels

Hard exudates, extending to macula

Macular edema

Exudative retinal detachment

Vitreous hemorrhage

Epiretinal membranes involving macula and causing retinal traction

Anterior vitreous cells

Retinal pigment epithelial hyperplasia Rubeosis and neovascular glaucoma

Fig. 28.4.3. Fundus photograph showing an inferotemporal vasoproliferative tumor associated with hard exudates. (Courtesy of C. Mosci, Genoa)

Patients tend to present with visual loss, floaters, and/or photopsia.

On ophthalmoscopy, the tumor has the appearance of a yellow or pink, intraretinal mass associated with adjacent hard exudates and occasionally retinal hemorrhages (Fig. 28.4.3). The hard exudates tend to extend posteriorly, eventually involving the fovea (Fig. 28.4.4). There may also be macular edema and exudative retinal detachment. In advanced stages, epiretinal membranes may develop, which can cause retinal distortion (Fig. 28.4.5). Solitary lesions predominate. Multiple lesions are more common in females when the disease is idiopathic and increase in incidence from about 6 % to 41 % in the presence of preexisting ocular disease [7]. A small minority of

patients have a diffuse vasoproliferative lesion and these tend to be relatively young, with an average age of 19 years [7].

Fluorescein angiography shows a rich capillary network and/or telangiectatic blood vessels within the tumor (Fig. 28.4.6). These leak profusely so that the entire lesion is hyperfluorescent in the late stages of the angiogram.

On ultrasonography, vasoproliferative tumors vary in size from 1.0 to more than 5 mm, averaging about 3 mm. The internal acoustic reflectivity varies from patient to patient and can be low, medium or high.

Tumor biopsy may be needed in some patients.

28 III

Fig. 28.4.4. Macular exudates from a peripheral vasoproliferative tumor. (Courtesy of H. Heimann, Berlin)

lesions are always associated with large retinal feeder vessels. There may also be extraocular manifestations of von Hippel-Lindau disease, a positive family history, or both.

Amelanotic melanomas can show dilated tumor vessels, particularly if the tumor has a collar-stud or mushroom shape. This tumor rarely causes exudates and hemorrhages.

Choroidal hemangiomas are pink, indistinct, nearly always located close to optic disk or fovea, and rarely associated with hemorrhages or hard exudates.

Coats’ disease consists of intraretinal telangiectasia with intraretinal or subretinal hard exudates. It tends to occur in children, mostly males, and causes exudative retinal detachment without the formation of a mass. Rarely, it is associated with a vasoproliferative tumor.

Fig. 28.4.5. Epiretinal membrane causing distortion of the retina. (Courtesy of H. Heimann, Berlin)

Fig. 28.4.6. Fluorescein angiogram showing patchy hyperfluorescence. (Courtesy of H. Heimann, Berlin)

28.4.5 Differential Diagnosis

Eccentric disciform lesions are usually associated with exudates and hemorrhages but are subretinal and tend to arise in older patients.

Retinal hemangioblastomas cause hard exudates, retinal hemorrhages, and fibrosis; however, these

28.4.6 Treatment

Essentials

Observation for asymptomatic patients

Cryotherapy for lesions up to 2 mm in thickness

Brachytherapy for large lesions

Photodynamic therapy for posterior lesions

Vitrectomy for vitreous hemorrhage or epiretinal membranes

Intravitreal antiangiogenic agents

Observation is indicated if a small, peripheral lesion is not causing much exudation so that there seems to be no threat to vision [6].

Cryotherapy using the triple free-thaw technique can conveniently be applied transconjunctivally in most cases, because these lesions are usually located anteriorly [7]. A single treatment session usually induces atrophy of the tumor and resorption of the hard exudates (Fig. 28.4.7). Some patients require several treatments, particularly if the tumor is large (i.e., > 2 mm thick).

Plaque radiotherapy can be selected for tumors that do not respond to cryotherapy or for large lesions (Fig. 28.4.8) [3]. A dose sufficient to induce vascular closure is administered (e.g., more than 300 Gy to base of lesion). A ruthenium plaque may be preferable to an iodine applicator if it delivers a high dose of radiation to the tumor without excessive radiation to optic disk, macula and lens.

Photodynamic therapy has recently been shown to be effective (Fig. 28.4.9) [2]. With posterior lesions it may cause less visual loss than other methods. It may also be useful if it is important to avoid

Fig. 28.4.7. Vasoproliferative tumor in the left eye of a 23-year-old female a at presentation, and b 1 year after cryotherapy. The tumor has become fibrotic and the exudates have regressed. (Courtesy of J. Elizalde, Barcelona)

III 28

a

b

Fig. 28.4.8. Regressed vasoproliferative tumor surrounded by choroidal atrophy after ruthenium plaque radiotherapy. (Courtesy of H. Heimann, Berlin)

inflammation, for example, if there is an epiretinal membrane or fibrosis with retinal traction.

Vitreoretinal surgery may be required to treat vitreous hemorrhage and epiretinal membranes. Hard exudates threatening the fovea have also been removed successfully, although this treatment requires further evaluation (Fig. 28.4.10).

In view of the fact that epiretinal membrane formation commonly causes visual loss, there is scope

a

Fig. 28.4.9. Vasoproliferative tumor a at presentation and b after photodynamic therapy. (Courtesy of E. Balestrazzi and A. Tiberti, Rome)

for investigating the scope of intravitreal triamcinolone injection as a means of preventing this complication. Intravitreal antiangiogenic agents such as bevacizumab (Avastin) may be useful [5].

28 III

Fig. 28.4.9b

a

b

Fig. 28.4.10. Left fundus of a 23-year-old female with an inferior vasoproliferative tumor in the left eye showing a hard exudates threatening macula at presentation, and b conservation of macula after ruthenium brachytherapy of the tumor and surgical removal of the hard exudates. The retina was folded and the exudates were brushed away from its outer surface. (Courtesy of C. Groenewald, Liverpool)

28.4.7 Prognosis

The natural course of this disease varies from patient to patient, progressing slowly or not at all in some patients and causing severe exudation, retinal fibrosis, traction retinal detachment and/or rubeotic glaucoma in others. Visual loss often persists even when treatment successfully induces tumor atrophy and resorption of the hard exudates.

References

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12.Wachtlin J, Heimann H, Jandeck C, Kreusel KM, Bechrakis NE, Kellner U, et al. (2002) Bilateral vasoproliferative retinal tumors with identical localization in a pair of monozygotic twins. 21:893 – 4