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

may be renamed in the future, once the histogenesis is better understood.

28.2.6 Diagnostic Approaches

graphy, ocular ultrasonography, optical coherence tomography, color Doppler imaging, computed tomography, and magnetic resonance imaging assist in confirming the diagnosis.

c

Fig. 28.2.4. c Full venous phase of the fluorescein angiogram demonstrating a nasal juxtapapillary mass with bright fluorescence, consistent with retinal capillary hemangioma. d Optical coherence tomography displaying an elevated dome-shaped retinal mass with bright optical surface and deep shadowing. e Optical coherence tomography of the fovea displaying subretinal fluid and optically dense subretinal debris, consistent with subretinal exudation

28.2.6.1 Fluorescein Angiography

Fluorescein angiography is the most helpful diagnostic test for recognizing retinal capillary hemangioma [43, 44] (Fig. 28.2.4). In the early arterial phase, the dilated retinal feeder arteriole appears prominent. Within seconds the retinal tumor is fluorescent as the fine capillaries that comprise the tumor fill with fluorescein. In the venous phase, the dilated draining vein fills with dye and the tumor maintains its bright fluorescence. In the late phase the tumor generally remains fluorescent and leaks dye into the vitreous. The intrinsic rapid fluorescence of the optic disk hemangioma assists in differentiating these tumors from other optic disk lesions.

28.2.6.2 Indocyanine Green Angiography

Indocyanine green angiography is used most often to visualize choroidal abnormalities as it is ideal for visualizing the choroidal vasculature [37]. It may be helpful in identifying a choroidal communication from the optic disk tumor to the adjacent choroid that is speculated to exist in some optic disk hemangiomas [27].

28.2 Retinal Capillary Hemangioma 753

III 28

d

e

28.2.6.3 Ultrasonography

Ocular ultrasonography can detect small retinal capillary hemangiomas greater than 1 mm in thickness, but its sensitivity is best for those tumors larger than 2 mm in thickness. The A-scan demonstrates an initial high spike at the innermost apex of the tumor and high internal reflectivity throughout the mass. B-scan ultrasonography shows a dense echo at the inner apex of the mass and acoustic solidity throughout the mass with no choroidal component. The subretinal fluid and retinal detachment can be demonstrated on ultrasound.

28.2.6.4 Optical Coherence Tomography

Optical coherence tomography (OCT) is a method of cross sectional retinal imaging with high resolution to 10 μm. It is most useful for identifying subtle subretinal fluid, intraretinal edema, cystoid retinal edema, and retinal atrophy. The retinal layers can be appreciated on OCT, and atrophy or disorganization of the photoreceptor layer implies poor visual acuity. With regard to retinal capillary hemangioma, OCT can image the retinal mass, but it is most useful for monitoring related subretinal fluid and other retinal findings that threaten or cause poor visual acuity [36] (Fig. 28.2.4).

754

28 III

Fig. 28.2.5. Small juxtapapillary retinal capillary hemangioma with chronic macular retinoschisis managed with observation. There was no hope for visual improvement

Fig. 28.2.6. Optic disk capillary hemangioma leading to total retinal detachment, neovascular glaucoma, and need for enucleation

A retrospective review of 68 patients with 174 retinal capillary hemangiomas from the Ocular Oncology Service at Wills Eye Hospital revealed initial management of observation (46 %), laser photocoagulation (25 %), or cryotherapy (23 %) [46]. Small tumors (1.5 mm in size; 63 of 99; 64 %) and those touching the optic disk (14 of 29; 48 %) were more likely to be initially observed. Sixty-three (82 %) of the 77 tumors that were initially observed remained stable

28 III

a

c

Fig. 28.2.7. Laser photocoagulation for juxtapapillary retinal capillary hemangioma in a young man. a Juxtapapillary retinal capillary hemangioma. b Fluorescein angiography confirms the vascular mass. c Optical coherence tomography showing subretinal fluid in the foveal region with optically dense subretinal debris. d Immediately following laser photocoagulation, the mass is surrounded by overlapping white retinal spots. The tumor appears blanched

repeated. Cryotherapy is generally reserved for peripheral retinal capillary hemangiomas, anterior to the equator of the eye and some posterior pole tumors that may be too large for photocoagulation. In some equatorial cases, a conjunctival incision will allow more accurate placement of the cryoprobe. Tumors less than 4 mm in thickness and less than 6 mm in base tend to respond adequately to cryotherapy. Larger tumors may require radiotherapy.

Plaque radiotherapy is a form of localized radiotherapy delivered by the transcleral route. Depending on tumor size, location, and associated features, the tumor is treated with an apex dose of 4,000 cGy over a 4- to 5-day period [16]. Larger tumors up to 8 mm thickness and 15 mm base can be treated with this method. External beam radiotherapy is a method of whole eye radiation and is reserved for very advanced tumors with substantial subretinal fluid or

b

d

those in the juxtapapillary region in which more conservative methods are expected to fail [31, 32]. In some advanced cases, especially those with the vitreoretinal form of retinal capillary hemangioma, vitreoretinal surgery with vitrectomy, repair of retinal detachment, endocryotherapy, and endophotocoagulation is employed.

The response to treatment with all of the above methods is slow and may need to be repeated. It is recommended to wait at least 1 – 2 months to assess the response to treatment and if there is a trend toward resolution, then further observation is indicated. If there remains minimal or no change or the disease worsens, then another session of treatment may be considered. The goal of treatment is to resolve associated subretinal fluid and exudation,

but the residual tumor scar may remain as an elevated sclerosed mass.

Patients with juxtapapillary capillary hemangiomas are the most challenging to treat as the tumor, and related subretinal fluid and exudation can lead to profound loss of visual acuity. However, treatment itself can also lead to visual loss. A collaborative

review of 60 eyes with juxtapapillary capillary hemangioma managed in ocular oncology or retina departments in Miami, Philadelphia, and San Francisco revealed poor long term vision (< 20/200) in 55 % of those treated with laser photocoagulation compared to 33 % poor vision in those not requiring or desiring laser treatment [24].

28.2.8.2 Systemic

Those patients with classic retinal capillary hemangioma, especially those of a young age or those with a family history of von Hippel-Lindau disease, should obtain a systemic evaluation as outlined in Table 28.2.2 [35, 39]. The availability of DNA analysis

28 III for patients with suspected von Hippel-Lindau has greatly increased detection of this disease and directed systemic monitoring.

28.2.9 Prognosis

The visual prognosis is quite variable depending upon tumor size and location as well as associated subretinal fluid, subfoveal gliosis, and preretinal fibrosis [24, 46]. The systemic prognosis is good if there is no associated von Hippel-Lindau disease. In those patients with von Hippel-Lindau disease, the projected median survival is 49 years [22].

28.2.10 Summary

Retinal capillary hemangioma is a benign vascular hamartoma of the retina. It can be associated with the von Hippel-Lindau disease and in those cases it is often the first finding of the disease. There is an exudative and vitreoretinal form of this tumor, leading to different clinical features. Treatment is directed toward early detection and obliteration of the tumors using methods of photocoagulation, photodynamic therapy, cryotherapy, radiotherapy, and others.

References

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Core Messages

Cavernous hemangioma is a rare vascular hamartoma localized in the inner layers of the retina, which may involve the periphery of the retina and the optic nerve, respectively

It is usually observed unilaterally, while bilateral manifestations have been reported

It may be diagnosed more often in adults than in children

The lesions of the retina are usually asymptomatic. Symptoms may rarely be caused due to a localization of the lesion involving the macula, and the optic nerve, and due to vitreous hemorrhages, respectively

Histology detects thin walled, endotheliumlined, dilated blood vessels with non-fenestrat- ed endothelium replacing the normal architecture of the inner retina. Preretinal membranes of glial origin on the surface of the lesion have been observed

The typical clinical findings are represented by:

– Clusters of saccular aneurysms within the

inner retinal layers presenting a grape-like formation

–A separation of the plasma and erythrocytic layers detected by fluorescein angiography. Usually there is no exudation observed

–Thrombosis of the lesion, small hemorrhages and membranes on the surface may occur

Therapy of the cavernous hemangioma is usually not necessary. Rarely vitrectomy may be considered in the case of persistent and severe vitreous hemorrhages

Cavernous hemangioma of the retina may present a manifestation of cerebral cavernous malformations (CCM) with cavernomas of the central nervous system and the skin. Therefore an exclusion of this entity, involving the brain and the skin, is recommended

Choroidal hemangiomas have been observed in patients of a family with autosomal dominant familial cavernous hemangiomas of the brain

28.3.1 Introduction

Cavernous hemangioma represents a rare vascular hamartoma of the retina involving the periphery of the retina and the optic nerve, respectively. The manifestation of this capillarovenous lesion is usually observed unilaterally. However, bilateral cases with a manifestation in both eyes have been reported [2]. The majority of patients are adult, while it is diagnosed rarely in children [28, 29, 44]. There are typical clinical findings of the tumor consisting of clusters of saccular aneurysms within the inner retinal layers, and presenting a grape-like formation [8]. Typical features with a separation of the plasma and erythrocytic layers may be detected by fluorescein angiography [8, 26]. Usually the lesions are diagnosed during a routine examination. Symptoms may rarely be caused by the tumor in the case of a localization involving the macula and the optic

nerve and inducing hemorrhages, respectively [8, 19, 29].

In addition, cavernous hemangioma of the retina may present a manifestation of “neuro-oculocutaneo- us syndrome” with cavernous hemangiomas of the central nervous system and the skin [5, 11]. Due to their clinical and genetic characterization, cerebral hemangiomas have been defined as cerebral cavernous malformations (CCMs) and cavernomas [7, 16, 22].

This chapter reports on the clinical findings, characteristics, and pathological features of the retinal cavernous hemangioma, and the co-segregation which may at least in part present with cavernomas of the skin and the brain.

28.3.2 History

Cavernous hemangioma of the retina was first convincingly observed and published by Niccol and

Moore (1934) [30]. It was subsequently observed by others [32, 42]. However, it has been suggested that some reports published before 1971 described the clinical signs of cavernous hemangioma as telangiectasis, Coats’ disease, congenital retinal angioma, angiomatosis retinae and a secondary vascular reaction to a previous exudative process [8]. Gass [8] compared the clinical findings observed in some of his patients with those of the literature and clearly defined the typical characteristics of this lesion. In addition, the term “neuro-oculocutaneous syndrome” has been used for cavernous hemangiomas of the retina associated with angiomatous lesions of the brain and the skin [8, 36]. The combination of clinical findings was first published by Weskamp and Cotlier (1940) [41]. They reported on a young female with a retinal vascular tumor. Vascular lesions of the skin and the brain suggested the presence of a cavernous hemangioma due to histological examinations.

28.3.3 Pathological Features

Thin walled, endothelium-lined and dilated blood vessels replacing the normal architecture of the inner retina have been detected histologically [8, 27]. The vascular lumens were at least in part connected to each other [8]. In addition, the telangiectatic retinal vessels were similar to normally encountered retinal vessels with a thin layer of non-fenestrated endothelial cells and a basement membrane [27]. The inner limiting membrane could not be separated over the entire area of the tumor [8]. However, preretinal

membranes have been observed in some cases. They consisted of spindle-shaped cells with glial filaments in the cytoplasm, the presence of glial-fibrillary acidic protein, and suggested a glial origin of the membranes [27].

(Fig. 28.3.1). It is localized within the inner layers of the retina [8, 26]. The vascular hamartoma may be associated with hemorrhages and fibrous (fibroglial) tissue on the surface of the tumor. The vascular pattern of the normal retina is not usually affected.