S E C T I O N

17 Choroid

169 ANGIOID STREAKS 363.43

Robert C. Kwun, MD

Park City, Utah

ETIOLOGY/INCIDENCE

Angioid streaks are irregular, jagged, curvilinear lines that radiate from the optic nerve in all directions. These lines were first described in 1889 by Doyne and later called angioid streaks by Knapp because of their resemblance to the branching pattern of blood vessels. The streaks lie beneath the retina and above the choroidal vasculature and are caused by breaks in the collagenous and elastic portion of Bruch’s membrane. These reddish-brown lesions are almost always bilateral and usually do not go past the equator. The streaks may be wide or narrow and may vary in length and number. Often, these lesions are associated with peripapillary chorioretinal changes.

The clinical significance of angioid streaks is based on the common association with several systemic disorders, such as pseudoxanthoma elasticum (Gronblad–Strandberg syndrome), osteitis deformans (Paget’s disease), sickle cell anemia, senile elastosis of skin, hypertensive cardiovascular disorders and, rarely, fibrodysplasia hyperelastica (Ehlers–Danlos syndrome). In addition, streaks are associated with choroidal neovascularization, retinal pigment epithelial detachment and macular degeneration. Other associated findings include peau d’orange fundus changes, disc drusen, peripheral focal salmon spots and pigmentary changes along the streaks.

●Streaks not seen clinically and associated choroidal neovascularization, retinal pigment epithelium detachments, and serous or hemorrhagic detachments may be demonstrated.

Indocyanine green angiography

●There is early hypofluorescence of the streaks with later hyperfluorescence and staining.

●Tiny hyperfluorescence spots are seen at the borders of the streaks.

●There is high resolution of choroidal neovascularization around the streaks.

Histopathology

●Angioid streaks are discrete linear breaks in Bruch’s membrane that are often characterized by extensive calcific degeneration.

●Fibrovascular ingrowth may occur in some of these breaks.

●Salmon spots are isolated breaks in Bruch’s membrane with fibrovascular ingrowth.

●Optic nerve drusen with streaks is usually associated with pseudoxanthoma elasticum.

Related systemic diagnosis in patients with angioid streaks

●Pseudoxanthoma elasticum (89%).

●Paget’s disease of bone (5%).

●Hemoglobinopathy (2%).

●Ehlers–Danlos syndrome (2%).

●Other (2%).

COURSE/PROGNOSIS

The patient is usually asymptomatic early in the course of the disease. Visual loss with metamorphopsia occurs with time. Exudative macular degeneration develops. Minor trauma may cause subretinal hemorrhage.

DIAGNOSIS

Laboratory findings

Fluorescein angiography

●Usually, early hyperfluorescence of the streaks is revealed with late staining. See Figure 169.1.

●Occasionally, hypofluorescence of the streaks is seen with hyperfluorescence of margins.

Differential diagnosis

●Macular degeneration.

●Choroidal sclerosis.

●Myopia and lacquer cracks.

●Pigment lines of reticular dystrophy of retinal pigment epithelium.

●Traumatic hemorrhage.

●Choroidal rupture.

●Chorioretinal folds.

TREATMENT

The use of safety glasses can prevent direct ocular trauma, and contact sports should be avoided. Low vision aids can be used.

Choroid • 17 SECTION

FIGURE 169.1. Angiography shows irregular hyperfluorescence along the course of the angioid streaks.

There is a possible role for laser photocoagulation in welldefined juxtafoveal choroidal neovascularization associated with angioid streaks.

Verteporfin photodynamic therapy of choroidal neovascularization in angioid streaks may limit visual loss.

REFERENCES

Browning AC, Chung AK, Ghanchi F, et al: Verteporfin photodynamic therapy of choroidal neovascularization in angioid streaks: one year results of a prospective case series. Ophthalmology 112:1227–1231, 2005.

Clarkson JG, Altman RD: Angioid streaks. Surv Ophthalmol 26:235–246, 1982.

Gelisken O, Hendrikse F, Deutmann AF: A long-term follow-up study of laser coagulation of neovascular membranes in angioid streaks. Am J Ophthalmol 195:299, 1988.

Groenblad E: Angioid streaks-pseudoxanthoma elasticum: vorlaeufige Mitteilung. Acta Ophthalmol 7:329, 1929.

Knapp H: On the formation of dark streaks as an unusual metamorphosis of retinal hemorrhage. Arch Ophthalmol 21:289–292, 1982.

Lebwohl M, Phelps R, Yannuzzi L, et al: Diagnosis of pseudoxanthoma elasticum by scar biopsy in patients without characteristic skin lesions. N Engl J Med 317:347–350, 1987.

Lim JI, Bressler NM, March MJ, Bressler SB: Laser treatment of choroidal neovascularization in patients with angioid streaks. Am J Ophthalmol 116:414–423, 1993

Paton D: The relation of angioid streaks to systemic disease. Springfield, Ill, Charles C Thomas, 1972.

Quaranta M, Cohen SY, Krott R, et al: Indocyanine green videoangiography of angioid streaks. Am J Ophthalmol 119:136–142, 1995.

Verhoeff FH: Histological findings in a case of angioid streaks. Br J Ophthalmol 32:531, 1948.

316

170 CHOROIDAL DETACHMENT 363.70

(Ciliochoroidal Detachment)

James W. Hung, MD

Boston, Massachusetts

A. Robert Bellows, MD

Boston, Massachusetts

ETIOLOGY/INCIDENCE

Choroidal detachment is a clinical term used to describe the separation of the choroid and sclera created by a fluid accumulation between the two layers. Anatomically the suprachoroidal space is a potential space, normally containing less than 10 microliters of fluid and is limited anteriorly by the scleral spur and posteriorly by the vortex veins. When fluid accumulates in this space, anterior displacement of the ciliary body and lens iris diaphragm often occurs and is associated with anterior chamber shallowing. The term ciliochoroidal detachment can be used interchangeably, since it implicates both the ciliary body and the choroid.

Although accumulation of any type of fluid can produce a choroidal detachment, the two most common forms are associated with the accumulation of serous and/or hemorrhagic fluids. The exact mechanism of any form of choroidal detachment is not fully understood, but intraocular hypotony is thought to be a primary contributor. When sufficient lowering of the intraocular pressure below episcleral venous pressure creates a pressure differential across the capillary bed, this gradient promotes the transudation of serous fluid from the choriocapillaris into the suprachoroidal space creating a detachment of the choroid and often retina from the sclera. Small and medium sized protein molecules move through intact cells with leaky junctions and accompanying the fluid into the suprachoroidal space. The drop in intraocular pressure creates a hydrostatic pressure gradient that can also produce mechanical traction on the arteries, veins and choroid when they emerge from the intrascleral canals. The rupture of these vessels is thought to be the etiology of hemorrhagic choroidal detachment referred to as a suprachoroidal hemorrhage. While there are many causes of hypotony it is most often found in the setting of ocular trauma, intraocular surgery, particularly glaucoma filtering surgery or tube shunt surgery, infrequently in cataract surgery and rarely in circumstances of aqueous suppressants or other pharmacologic agents.

Other specific entities associated with the development of choroidal detachment include inflammation, vascular abnormalities and malignancy. Inflammation as manifested in scleritis, choroiditis, Vogt–Koyanagi–Harada syndrome, Wegener’s granulomatosis, orbital pseudotumor, and extensive panretinal photocoagulation frequently lead to the accumulation of serous fluid in the suprachoroidal space. Vascular malformations such as carotid-cavernous fistulas and Sturge Weber syndrome, as well as a unique ophthalmic syndrome associated with prominent episcleral vessels and elevated episcleral venous pressure. These vascular entities are associated with an increase in resistance of blood flow through the episcleral vessels and result in accumulation of fluid in the suprachoroidal space. Additional unusual anatomic variants such as nanophthalmos and the uveal effusion syndromes are associated with choroidal detachment as are ocular tumors, primary and secondary, that can cause a choroidal detachment.

The risk factors of choroidal detachment include: advanced age, preoperative elevated intraocular pressure, high myopia, elevated episcleral venous pressure, severe hypertension, Valsalva maneuvers and aphakia. Choroidal detachments have also been documented to form spontaneously or related to medication. The risk factors of developing a suprachoroidal hemorrhage are many of the mentioned entities and, in addition, blood dyscrasias, anticoagulants, increased intraocular pressure that is lowered suddenly, filtering surgery and tube shunt procedures.

The reported incidence of choroidal detachments vary widely depending on the etiology and clinical setting. It must be emphasized that the incidence of choroidal detachment is far less since the advent of sutured flap closure of glaucoma filtering procedures and tight wound maintenance and closure following cataract surgery. The use of antimetabolites has resulted in more successful lowering of intraocular pressure and surprisingly enough has not been associated with an increase in the incidence of choroidal detachment. The entity of hypotony maculopathy is more prevalent with hypotony following antimetabolites than is the development of a choroidal detachment.

Serous choroidal detachments are more common following the postoperative periods of hypotony after glaucoma surgery. With the advent of phacoemulsification surgery the maintenance of intraocular pressure during surgery and tight wound closure following surgery has minimized postoperative hypotony with only the rare complication of choroidal detachment following cataract surgery. The more frequent use of glaucoma drainage implant surgery (tube shunt), both valve and nonvalve, has been associated with a higher incidence of choroidal detachment, particularly hemorrhagic choroidal detachments. The entity of an expulsive suprachoroidal hemorrhage during surgery is now rare because of the small incision tight wound closure associated with most cataract and glaucoma surgery. The incidence of choroidal detachment has also diminished in penetrating keratoplasty and vitreoretinal procedures.

DIAGNOSIS

Clinical signs and symptoms

A serous choroidal detachment is usually painless unless associated with underlying inflammatory cause such as scleritis. However, a hemorrhagic choroidal detachment is often heralded by severe sudden pain that is often diagnostic.

Choroidal detachments appear as large, brown, quadratic intraocular dome-shaped balloons that often exist circumferentially, most prominent nasal and temporal.

Minimally elevated anterior choroidal detachments are often difficult to identify with an ophthalmoscope and are more often associated with shallowing of the anterior chamber. B-scan ultrasonography is helpful in documenting a posterior choroidal detachment but ultrasound biomicroscopy (UBM) is frequently necessary to identify anterior choroidal detachments. A recent study has utilized color Doppler imaging to identify choroidal detachments and distinguish them from retinal detachments. This distinction is often more accurately made during the clinical examination.

Large choroidal detachments can lead to direct apposition of opposing retinal surfaces frequently referred to as ‘kissing’ choroidals and often require drainage. In an effort to distinguish a choroidal detachment from an intraocular tumor, transilluminating (Hagen’s sign) results in a transillumination of a

choroidal detachment with no transillumination in solid tumors.

Suprachoroidal hemorrhages may develop suddenly and present as large, very dark balloon-like elevations of the choroid. Occasionally B-scan ultrasonography can distinguish blood in the suprachoroidal space.

Development of an intraocular hemorrhagic choroidal detachment is heralded by the patient expressing severe pain, often ‘breaking through’ apparent adequate local anesthesia. The surgeon may notice an alteration in the red reflex, often associated with shallowing of the anterior chamber and positive vitreous pressure in the presence of a large wound, extrusion of retina and the intraocular contents. Principal effort is directed to closing the wound to prevent egress of tissue.

Differential diagnosis

●Serous choroidal detachment.

●Hemorrhagic choroidal detachment.

●Retinal detachment.

●Primary metastatic tumors.

PROPHYLAXIS

The development of a serous or hemorrhagic choroidal detachment is difficult to predict and to prevent. However, meticulous preoperative evaluation of the patient as well as intraoperative efforts to minimize the degree of length of time of ocular hypotony can diminish the attendant risk. Preoperative systemic hypertension should be medically controlled. Administration of anticoagulants, aspirin containing medications or anything altering the blood clotting mechanism should be avoided if possible. Intraocular pressures should be lowered slowly with digital pressure or osmotic agents. The choice of anesthetic, general or local, is not usually associated with the development of a choroidal detachment. However, in patients with elevated venous pressure, alteration of the venous drainage system from the head during general anesthesia can cause elevation of pressure in the venous drainage system of the eye.

Intraoperative

A paracentesis should be created to very slowly lower the intraocular pressure in order to slowly reduce the pressure gradient. Microsurgical techniques are necessary for visualization and appropriate closure of ocular tissues with fine suture material. The tight suture closure of trabeculectomy flaps or corneal incision and ligature sutures in non-valve tube shunt procedures can be utilized. Antimetabolite agents are always used with caution.

Postoperative

The need to avoid prolonged hypotony is possible.

Attempts are made to minimize coughing, straining, vomiting, heavy lifting and aggressive physical activity. Stool softeners, laxatives and anti-emetic agents are frequently recommended.

TREATMENT

Ocular

Serous or small hemorrhagic suprachoroidal detachment

Serous and small hemorrhagic suprachoroidal detachments are usually self-limited. When the intraocular architecture is not

170 CHAPTERDetachment Choroidal •

Choroid • 17 SECTION

171 CHAPTERFolds Choroidal •

Choroid • 17 SECTION

●Traction on the optic nerve in the absence of scleral contact.

●Orbital tumors (hemangioma, meningioma).

●Thyroid orbitopathy.

●Orbital cellulitis/inflammation.

●Dural sinus fistula.

●Optic nerve edema/intracranial hypertension.

●Ethmoid sinus mucocele.

●Idiopathic.

COURSE/PROGNOSIS

●Can occur in normal eyes.

●May not affect visual function.

●Possible decreased visual acuity and/or metamorphopsia due to macular involvement.

●Possible permanent visual loss due to long-term macular involvement.

●Variable location of folds not predictive of the underlying pathology.

●Characteristic pigmentation patterns due to persistent or recurrent folds: typically bead-like pigmentation lines on the slope of the fold.

DIAGNOSIS

Laboratory findings

●Fluorescein angiogram.

●Early phase: alternating lines of hyperfluorescence and hypofluorescence corresponding to the density of the pigment epithelium at the peaks and valleys of the folds, respectively.

●Late phase: no leakage.

●Folds more visible angiographically than clinically.

●Computed tomography scanning or magnetic resonance imaging when orbital tumor or inflammation is suspected.

●Ultrasonography when posterior scleritis is suspected.

Differential diagnosis

●Retinal folds.

●Entirely separate entity rarely coinciding with choroidal folds.

●Appear finer on ophthalmoscopy.

●Associated with vitreoretinal disease and usually radiate from visible pathology in the retina.

●Fluorescein angiogram is normal (relative to the folds).

TREATMENT

When possible, treatment is reversal of the underlying cause.

REFERENCES

Bullock JD, Egbert PR: Experimental choroidal folds. Am J Ophthalmol 78:618–623, 1974.

Bullock JD, Egbert PR: The origin of choroidal folds: a clinical, histopathological, and experimental study. Documenta Ophthalmol 37:261–293, 1974.

Bullock JD, Waller RB: Choroidal folding in orbital disease. Proceedings of the Third International Symposium on Orbital Disorders, Amsterdam. 1977:483–488.

Cangemi FE, Trempe CL, Walsh JB: Choroidal folds. Am J Ophthalmol 86:380–387, 1978.

Friberg TR: The etiology of choroidal folds: a biomechanical explanation. Graefes Arch Clin Exp Ophthalmol 227:459–464, 1989.

Greibel SR, Kosmorsky GS: Choroidal folds associated with increased intracranial pressure. Am J Ophthalmol 129:513–516, 2000.

Newell FW: Choroidal folds. Am J Ophthalmol 75:930–939, 1973.

Newell FW: Fundus changes in persistent and recurrent choroidal folds. Br J Ophthalmol 68:32–35, 1984.

Norton EWD: A characteristic fluorescein angiographic pattern in choroidal folds. Proc R Soc Med 62:119, 1969.

Steuhl KP, Richard G, Weidle EG: Clinical observations concerning choroidal folds. Ophthalmologica 190:219–224, 1985.

172 CHOROIDAL RUPTURES 363.63

Scott D. Pendergast, MD

Beachwood, Ohio

ETIOLOGY/INCIDENCE

Choroidal rupture is characterized by tearing of Bruch’s membrane and the closely associated choriocapillaris and retinal pigment epithelium (RPE) after contusive ocular injury. Direct choroidal rupture occurs at the site of injury and typically is located anterior to the equator and is oriented parallel to the ora serrata. Indirect choroidal ruptures are frequently crescentshaped tears of the RPE-Bruch’s membrane-choriocapillaris complex that are concentric to the optic disk and occur in the posterior pole remote from the site of impact. In the acute stages, choroidal ruptures often are associated with hemorrhagic detachment of the RPE, subretinal or intraretinal hemorrhage, and retinal edema, which may obscure the presence and extent of choroidal rupture. As the retina edema and hemorrhagic components resolve, a subretinal yellow-white scar develops that is frequently associated with hyperplasia of the RPE.

●Direct choroidal ruptures probably result from compression necrosis of ocular tissues at the site of contusive injury.

●Indirect choroidal ruptures have been attributed to contrecoup forces that result in rapid anteroposterior compression and horizontal expansion of the globe. Bruch’s membrane is relatively inelastic and susceptible to rupture, whereas the relatively distensible retina and rigid sclera are less prone to rupture.

●The concentric configuration of most indirect choroidal ruptures may be due to a tethering effect of the optic nerve.

●Approximately 1% of patients evaluated at large eye clinics have choroidal rupture.

●The incidence of indirect choroidal rupture after blunt trauma is 5% to 10%.

●Multiple ruptures occur in approximately 25% of eyes.

●Rupture occurs temporal to the optic nerve in 80% of cases, with macular involvement in 66% of eyes.

●Indirect choroidal ruptures are more common than direct choroidal ruptures (80% versus 20%).

COURSE/PROGNOSIS

●Based on histologic studies, direct and indirect choroidal ruptures heal 14 to 21 days after injury.

●Retinal edema resolves in 2 to 3 weeks, whereas hemorrhage may persist for 2 to 3 months.

●A yellow-white subretinal scar develops that may be associated with hypopigmentation or hyperpigmentation of the surrounding RPE.

●Presenting visual acuity is frequently reduced to 20/200 or worse and ranges from 20/20 to light perception depending on the location of the choroidal rupture.

●Visual acuity improves in approximately 60% to 70% of cases, with mean final visual acuity ranging from 20/40 to 20/70 in recent series.

●Eyes with choroidal ruptures involving the fovea, the presence of dense subfoveal hemorrhage, and the development of extensive pigmentary changes in the macula portend a worse prognosis. However, a recent retrospective series found that eyes with foveal choroidal ruptures could maintain good central vision after 4 years of follow-up.

DIAGNOSIS

Laboratory findings

Diagnosis is based on the characteristic clinical appearance as well as a history of preceding ocular trauma.

●If retinal edema, hemorrhage, or both are present and obscure findings, serial examinations may be necessary to establish a diagnosis of choroidal rupture.

●Fluorescein angiography (FA) often is helpful in confirming the presence, location, and extent of choroidal ruptures and demonstrates a hypofluorescent curvilinear streak in the early transit phase followed by hyperfluorescence in the late transit phase with variable hyperfluorescence in the recirculation phase.

●Indocyanine green angiography (ICGA) is useful in localizing major and minor choroidal ruptures and is particularly useful in identifying ruptures obscured by dense hemorrhage. Initial ICGA typically shows hypofluorescent streaks that become more apparent over time and are often more numerous that hyperfluorescent streaks identified on FA.

PROPHYLAXIS

●Appropriate protective eyewear (e.g. polycarbonate lenses) worn while engaging in contact sports is the most effective prophylaxis.

●When Bruch’s membrane is abnormal, as in angioid streaks, pathologic myopia, or pre-existing choroidal rupture, relatively minor trauma can result in extensive choroidal ruptures. High-risk patients should be advised of the hazards associated with blunt ocular trauma related to contact sports and other activities.

TREATMENT

●There is no treatment for choroidal ruptures, but a careful examination is important to exclude other ocular injuries associated with blunt ocular trauma, such as commotio retinae, retinal dialysis, hyphema, angle recession and traumatic iritis.

●Inflammation of the anterior segment can be treated with topical steroids and cycloplegics.

●Patients should be followed closely (e.g. monthly) for the first 6 months to detect complications such as retinal detachment and choroidal neovascularization (CNV).

●Retinal detachments that are progressive are usually repaired using standard scleral buckling techniques. In some instances, scar formation limits the detachment and may obviate the need for surgery.

●Choroidal neovascularization:

●The incidence is unknown, but it appears to be relatively common.

●This occurs 1 month to 4 years or more after injury and necessitates Amsler grid testing and long-term follow-up.

●Patients may note decreased central visual acuity, metamorphopsia or scotoma.

●Clinical signs include the presence of subretinal hemorrhage, fluid, or exudate or a pigmented subretinal lesion.

●FA and/or ICGA should be performed to confirm the diagnosis and determine the extent and location of CNV relative to the fovea.

●No controlled studies are available and treatment remains somewhat controversial.

●Because choroidal rupture represents a focal abnormality of Bruch’s membrane as in presumed ocular histoplasmosis syndrome (POHS), results of photocoagulation for CNV associated with POHS may provide useful treatment guidelines for CNV associated with choroidal ruptures.

●Laser photocoagulation is frequently used to treat CNV, particularly when the center of the fovea is not involved.

●Photodynamic therapy has been used successfully to treat subfoveal CNV associated with traumatic choroidal rupture.

●Submacular surgery with excision of subfoveal CNV improved visual acuity to 20/30 or better in 3 patients in a small series.

●Because CNV has been reported to spontaneously resolve, some investigators advocate observation of CNV.

COMPLICATIONS

●Choroidal neovascularization is the most frequent late complication of choroidal ruptures.

●Retinal detachment is occasionally observed in cases of anterior choroidal ruptures.

●A variety of visual field defects have been observed.

●Chorioretinal vascular anastomosis is a rare complication of choroidal ruptures.

REFERENCES

Bressler SB, Bressler NM: Traumatic maculopathies. In: Shingleton BJ, Hersh PS, Kenyon KR, eds: Eye trauma. St Louis, Mosby-Year Book, 1991:187–194.

Conrath J, Forzano O, Ridings B: Phtodynamic therapy for subfoveal CNV complicating traumatic choroidal rupture. Eye 18:946–947, 2004.

Gross JG, King LP, de Juan E, Jr, et al. Subfoveal neovascular membrane removal in patients with traumatic choroidal rupture. Ophthalmology 103:579–585, 1996.

Kohno T, Miki T, Shiraki K, et al: Indocyanine green angiographic features of choroidal rupture and choroidal vascular injury after contusion ocular injury. Am J ophthalmol 129:38–46, 2000.

Raman VR, Desai UR, Anderson S, et al: Visual prognosis in patients with traumatic choroidal rupture. Can J Ophthalmol 39:260–266, 2004.

172 CHAPTERRuptures Choroidal •

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173 EXPULSIVE HEMORRHAGE 363.62

(Suprachoroidal Expulsive Hemorrhage)

Fumiki Okamoto, MD, PhD

Ibaraki, Japan

Sachiko Hommura, MD, PhD

Ibaraki, Japan

Expulsive hemorrhage is one of the most serious and catastrophic complications of intraocular surgery. It occurs suddenly, as a localized or sometimes generalized suprachoroidal hemorrhage, breaking into the vitreous and then extruding the intraocular contents through the surgical wound. Early detection of the problem and prompt, aggressive treatment are essential to save the patient’s vision; however, in many cases, even rapid remedial measures may fail to preserve useful vision. Prevention of the emergency by understanding the factors that may predispose a patient to experience an intraoperative expulsive hemorrhage is much preferable to attempting to deal with the after-effects.

ETIOLOGY/INCIDENCE

Expulsive hemorrhage is fortunately rare; according to Ling, it occurs in approximately 0.04% of cataract surgery. Among other ocular surgical procedures, expulsive hemorrhage occurs in 0.15% of glaucoma operations, 0.12% of pars plana vitrectomy, and 0.56% of penetrating keratoplasty. The incidence of this complication is higher in elderly patients who have arteriosclerosis or hypertension and glaucoma.

The exact cause of expulsive hemorrhage is unknown, but the immediate event occurs as a result of sudden drop in intraocular pressure gradient when the eye is opened. This sudden change may induce rupture of intraocular vessels if the vessel walls are fragile. The presence of necrotic artifacts in the walls of the short and long ciliary arteries is most important and contributes significantly to occurrence of expulsive hemorrhage.

Risk factors for expulsive hemorrhage fall into two categories: vascular factors and intraoperative risks. Vascular factors include:

●Older age;

●Elevated arterial pressure before surgery;

●Chronic systemic hypertension;

●Generalized arteriosclerosis;

●Hyperlipidemia;

●Diabetes;

●Use of anticoagulant agents;

●High myopia;

●Glaucoma (elevated preoperative intraocular pressure).

Many patients demonstrate combinations of two or more of these vascular factors; in our surgical experience, among 500 successful cataract procedures (controls) versus 5 cataract procedures that involved expulsive hemorrhage, the latter patients had higher numbers of vascular risk factors (four or five factors) compared with controls (only 3 in 500 had four risk factors and none had five factors).

Intraoperative risk factors include:

●Intraoperative systolic hypertension;

●Ocular pain;

●Urge to urinate;

●Cough reflex;

●Vitreous loss;

●Idiopathic (unknown) factors.

Among these factors, poorly controlled intraoperative pain, unsuppressed cough, or the urge to urinate indicates elevation of venous return pressure, which may be a contributing cause of expulsive hemorrhage.

Consideration also must be given to the type of surgical procedure; the procedure most commonly associated with expulsive hemorrhage is the penetrating keratoplasty, with vitreoretinal procedures, cataract extractions and glaucoma operations carrying somewhat lesser risk of expulsive hemorrhage. Penetrating keratoplasty is an ‘open sky’ procedure that induces prolonged hypotony, scleral collapse, and relative forward displacement of the intraocular contents; in retinal/vitreous operations, direct trauma to the choroid or the vortex vein may be responsible for precipitating expulsive hemorrhage. Glaucoma procedures carry a relatively low incidence of expulsive hemorrhage comparable with that of cataract surgery; in such cases, prolonged ocular hypotony probably results in rupture of the ciliary artery and subsequent massive bleeding.

There also may be unknown factors in the patient’s medical background that increase the risk for expulsive hemorrhage during intraocular surgery. Wohlrab and associates report a recent case in which surgery was being performed for acute glaucoma and a mature cataract when expulsive hemorrhage occurred; after the operation, laboratory analysis of blood smears demonstrated a myelocytic proliferation, indicating the previously unsuspected presence of myelodysplastic syndrome. The operated eye was eventually enucleated due to extremely high intraocular pressure unresponsive to intensive antiglaucoma therapy; histopathologic examination revealed the retina and choroid were swollen due to leukemic cell infiltration and choroidal hemorrhages.

A second case, reported by Weissgold and coworkers, involved a penetrating keratoplasty being performed for delayed-onset fungal keratitis after fungal endophthalmitis; 3 months earlier, the patient had received aggressive therapy (vitrectomy and intraocular injection of antifungal drugs) for the endophthalmitis. Although the existing prosthetic intraocular lens was left in place after the vitrectomy, a posterior capsulectomy was performed. At the time of the penetrating keratoplasty and complicating expulsive hemorrhage, the eye could not be saved; histopathologic analysis of the eviscerated vitreous and the ulcerated cornea disclosed fungal elements of Acremonium kiliense, the causative organism of the original endophthalmitis. The authors conclude that despite aggressive therapy, fungal infections are particularly difficult to eradicate and may have exacerbated choroidal inflammation in this patient, perhaps increasing the risk of catastrophic expulsive hemorrhage.

COURSE/PROGNOSIS

Unfortunately, visual outcome after expulsive hemorrhage often is severely compromised, despite the most up-to-date vitreoretinal surgery. Welch and coworkers assessed visual outcome in 30 patients after massive suprachoroidal hemorrhage and reported that more than half (18) of the patients had a final visual acuity of 20/200 or better; in 1 patient, visual acuity returned to 20/20. The remaining 12 patients experi-

173 CHAPTERHemorrhage Expulsive •

Choroid • 17 SECTION

●Tumor thickness of more than 2 mm;

●Tumor touching the optic disk;

●Presence of symptoms;

●Orange pigment;

●Subretinal fluid.

Clinical risk factors for metastasis of small uveal melanoma (3 mm thick or smaller) include:

●Tumor thickness of more than 2 mm;

●Documented growth;

●Tumor touching the optic disk;

●Presence of symptoms.

DIAGNOSIS

Clinical signs and symptoms

Uveal melanomas may give rise to symptoms (blurred vision, photopsia, metamorphopsia) or be detected during a routine eye examination. Melanomas tend to be dome-shaped but may appear flat and diffuse. Rupture of Bruch’s membrane produces a ‘collar-stud’ appearance (Figure 174.1). Pigmentation is variable although surface orange-pigment (lipofuschin) is characteristic. The diagnosis is largely made on clinical appearance on indirect ophthalmoscopy or slit-lamp examination.

Laboratory findings

●Ultrasonography: acoustically hollow uveal mass on B-scan and medium to low internal reflectivity within the mass on A-scan.

●Intravenous fluorescein angiography: patchy early fluorescence and diffuse late staining of the choroidal mass, often with a double circulation pattern.

●Indocyanine green angiography: gradual hyperfluorescence over 20 minutes, but patterns can vary.

●Computed tomography: moderately dense, noncalcified intraocular mass.

●Magnetic resonance imaging: an intraocular mass that is hyperintense to vitreous on T1-weighted images, hypointense to vitreous on T2-weighted images, and moderate enhancement with gadolinium contrast.

FIGURE 174.1. Typical ‘collar-stud’ melanoma. Note the amelanotic portion of the tumor that has breached Bruch’s membrane.

●Microscopic histopathology: spindle or epithelioid cells according to the Callender classification.

Differential diagnosis

Common differential diagnoses include:

●Uveal neoplasms:

●Nevus;

●Metastasis;

●Hemangioma;

●Lymphoid tumor;

●Osteoma.

●Retinal neoplasms:

●Retinal astrocytic hamartoma;

●Retinal capillary hemangioma;

●Combined hamartoma retina and retinal pigment epithelium;

●Vasoproliferative tumor of the fundus;

●Retinoblastoma.

●Pigment epithelial neoplasms:

●Pigment epithelial hypertrophy (CHRPE)/hyperplasia;

●Pigment epithelial adenoma;

●Medulloepithelioma.

●Non-neoplastic diseases:

●Subretinal hemorrhage secondary to age-related macular/ extramacular degeneration;

●Retinal arterial macroaneurysm;

●Choroidal hemorrhage/detachment;

●Posterior scleritis;

●Choroidal granuloma;

●Retinal detachment.

TREATMENT

Systemic

A number of treatment options currently exist for metastatic melanoma, however, response rates are uniformly poor. Whilst patients with metastases to certain sites such as the skin may survive for several years, the majority of those with diffuse liver metastases die within 6 months. Owing to poor response rates intravenous therapy is often reserved for palliation of symptoms. Intrahepatic chemoembolization has been used for isolated liver metastases and similarly, focal metastases (in many organs) may be amenable to local surgical resection. Various groups have tried immunotherapy however a successful melanoma vaccine remains elusive.

Local

The aims of treatment of uveal melanoma are:

●To eradicate the tumor before metastasis occurs;

●Preserve the eye;

●Preserve vision.

The best form of treatment for any melanoma depends on many different variables including the size and apparent activity of the tumor, the health of the affected eye and it’s fellow and the age and general health of the patient. What might be ideal for a 35 year old airline pilot might be wholly inappropriate for a 90 year old in residential care.

Observation

Periodic observation may be recommended to initially manage selected small melanomas that have dormant characteristics on ophthalmoscopic examination. Such a lesion should be care-

Uvea Posterior the174ofCHAPTERMelanoma Malignant •

Choroid • 17 SECTION

fully examined two or three times a year, and some form of active treatment should be instituted if growth of the tumor is subsequently documented.

The diagnostic tests and the frequency of follow-up examinations depend on the size and the apparent activity of the tumor. If a patient has a tumor that is classified as a suspicious nevus or dormant melanoma, then a repeat examination should be performed in 3 to 4 months, with fundus photography and ultrasonography for documentation. If no growth is detected, examination should be repeated every 4 to 6 months thereafter. If the lesion remains quiescent then these intervals may be increased. If however growth is documented, or the lesion shows increasing orange pigment, subretinal fluid or symptoms active treatment is advisable.

For those lesions that require treatment, the main therapeutic options consist of:

●Diode laser transpupillary thermotherapy;

●Brachytherapy (radioactive plaques);

●Charged particles (proton beam or Helium ion);

●Stereotactic radiosurgery;

●Local resection;

●Enucleation;

●Exenteration.

Transpupillary thermotherapy

Over the last decade transpupillary thermotherapy (TTT) has supplanted argon laser photocoagulation as the treatment for small uveal melanomas (<3.5mm in thickness) at the posterior pole. The technique utilizes an infrared diode laser to heat the tumour to a level that is cytotoxic without causing photocoagulation. Initial animal studies and subsequent clinical studies on enucleated eyes performed by Oosterhuis et al suggested that the technique could produce necrosis to a depth of 4 mm, ideal therefore for small melanomas. TTT is usually delivered via a slit-lamp-mounted diode laser and is performed under retrobulbar or sub-Tenons anesthesia. Laser is applied via a contact lens through a dilated pupil. Traditionally, a 3mm spot size has been used with a 1-minute application time per spot. Power levels vary from as little as 250 mW for deeply pigmented tumors to 1500 mW for amelanotic lesions. The aim of treatment is to produce a light gray burn at the end of the 1-minute application. A heavier burn results in photocoagulation which may impede the heating of the deeper portions of the tumor. Adjacent spots are applied to cover the entire surface of the tumor. The majority of melanomas require 3 (or more) treatments usually at 2 to 3 month intervals before ultimate regression to a (hopefully) flat chorioretinal scar.

Initial reports suggested excellent rates of local control as well as preservation of vision in patients treated with TTT. However as might be expected as follow-up continues, increasing numbers of local recurrences including cases of extraocular extension are now being reported. For this reason many authorities reserve TTT as an adjunct to plaque therapy (sandwich technique) rather than as a primary therapy.

Plaque radiotherapy

Plaque radiotherapy is the most widely used method in the management of melanoma of the choroid and ciliary body. Various isotopes have been used however the two main alternatives are 125I (most popular in the USA) and 106Ru (Europe). Each has its own advantages and disadvantages. Iodine is a potent gamma-emitter that may be used to treat tumors of essentially any size. Each plaque is tailor-made for the patient allowing unique flexibility. Half-life is short however at approximately

4 weeks. Ruthenium on the other hand is a low energy B- emitter with a half-life of 1 year, therefore Ruthenium plaques may be reused many times making them more economical. The main limitation of Ruthenium however is that it is only suitable for tumors up to 6mm in thickness. Regardless of the isotope used the majority of plaques are circular however notched plaques are available for the treatment of peripapillary lesions.

The current indications for treatment of a posterior uveal melanoma with an episcleral plaque are:

●Selected small melanomas that are documented to be growing;

●Most medium-sized and large choroidal and ciliary body melanomas that are less than 10 mm in thickness in an eye that has useful or salvageable vision; and

●Most medium-sized and large melanomas that occur in the patient’s only useful eye, regardless of the visual acuity.

In the past extraocular extension of the melanoma has been considered a contraindication to plaque therapy, however small deposits can be easily covered by a plaque and respond as readily as the intraocular tumor.

Plaques may be inserted under general or local anesthesia. A peritomy is performed and the extraocular muscles are hooked with traction sutures to allow exposure of the tumor site. Extraocular muscles occasionally need to be disinserted to allow accurate placement of the plaque. Trans-scleral transillumination is performed to localize the tumor. The shadow of the tumor is outlined on the sclera with a sterile marking pencil, and a dummy plaque is used to align the scleral sutures. The dummy plaque is then removed, and the radioactive plaque is inserted and tied securely in position. The plaque is left in position long enough to deliver 80 to 100 Gy to the tumor apex, after which it is removed and the patient is discharged.

In general, brachytherapy offers excellent local control rates and preservation of the globe. Visual prognosis depends largely on the location of the tumor as some form of radiation retinopathy is inevitable.

Local resection

Theoretically, an ideal approach to the management of a melanoma of the ciliary body or choroid is to perform local resection to remove the tumor and salvage the eye, particularly if this can be achieved without worsening the patient’s prognosis for life. In recent years, a technique of partial lamellar sclerouvectomy has been popular. This technique involves removing the tumor and inner sclera while leaving intact the retina and the outer sclera. A radioactive plaque may also be applied to the resection site for a brief period as a safeguard against local recurrence. The technique is lengthy and technically demanding requiring hypotensive anesthesia making case selection particularly important.

The relative indications for local resection of a posterior uveal melanoma are:

●A growing ciliary body melanoma or a ciliochoroidal melanoma that does not cover more than one third of the pars plicata; and

●A choroidal melanoma that is no greater than 15 mm in diameter, centered near the equator.

It should be stressed that melanomas that meet these criteria also can be managed with other conservative techniques such as radiotherapy in most instances. The preferred method of therapy is unresolved, and each case must be evaluated individually.

Proton beam

This technique utilizes protons produced by a cyclotron to kill the tumor. It is therefore limited to a handful of centers around the world. Tumors are localized with tantalum markers inserted in the operating theater. In theory, melanomas of any size may be treated with charged particles. Rates of local control and preservation of vision are similar to those with episcleral plaques however minor anterior segment complications are probably more frequent.

Stereotactic radiosurgery

This is a form of external radiotherapy delivered by means of a Leksell gamma knife. This unit consists of 201 individual cobalt sources that may be focused onto the tissue requiring treatment. It may therefore be used to treat inaccessible or complex lesions in the brain or for our purposes, the eye. The technique requires the application of a stereotactic frame to the skull and the use of MRI to localize the tumor with reference to the frame. Owing to the limited availability of the gamma knife unit, relatively few uveal melanoma patients have been treated with stereotactic radiosurgery.

Enucleation

Although a number of conservative therapeutic options now exist, enucleation remains a mainstay of treatment of uveal melanoma. There are definite indications for enucleation, although the indications for this procedure are fewer than they were in the past. Enucleation tends to be reserved for those melanomas that are too large to be managed with radiotherapy or local resection particularly if the vision has already been lost. Occasionally however patients request enucleation as they are unable to cope with the thought of a cancer in their eye and request that it be treated ‘once and for all.’

The technique of enucleation for melanoma is no different than that for other conditions and a primary implant may almost always be inserted. The Collaborative Ocular Melanoma Study has shown that the use of pre-enucleation radiotherapy offers no survival advantage.

Orbital exenteration

Orbital exenteration is seldom necessary for uveal melanoma and is largely reserved for those with massive extraocular spread with no evidence of systemic metastases. With improved diagnostic techniques and earlier recognition of uveal melanomas, it has become uncommon for patients to present initially with extensive extraocular involvement.

COMPLICATIONS

There appears to be little or no danger in the close observation of small melanomas that show dormant features. Most of these tumors have little, if any, tendency to grow and a low potential to metastasize. Lesions located within 2 mm of the optic disk or foveola should be followed more frequently. If growth is documented, then plaque radiotherapy or transpupillary thermotherapy should be considered.

The complications of transpupillary thermotherapy appear to be fewer than those of photocoagulation and include retinal vascular obstruction, cystoid macular edema, preretinal membrane formation with retinal traction, choroidovitreal or retinal neovascularization, vitreous hemorrhage, and retinal detachment. Treatment with these methods is most often successful

if the tumor is small, being less than 3 mm in thickness as measured by ultrasonography.

There are very early complications of episcleral plaque radiotherapy, including ocular irritation and diplopia. Diplopia can occur if a rectus muscle is disinserted to properly position the plaque. Later potential complications of episcleral plaque radiotherapy include radiation retinopathy, radiation papillopathy, neovascular glaucoma, vitreous hemorrhage, radiation cataract, keratoconjunctivitis sicca, radiation anterior uveitis, sclera necrosis, and persistent diplopia. Tumors treated with episcleral plaque radiotherapy may show a rather dramatic response to treatment however the majority show either stabilization or a gradual decrease in size during a follow-up period of 2 to 6 years. Depending on tumor location, visual results have been satisfactory, and complications relatively few.

The early complications of local tumor resection, however, are greater than those of plaque radiotherapy. The most important potential early surgical complications of local resection are hypotony, wound leak, vitreous bleeding, and retinal detachment. Potential late complications of local resection include vitreous fibrosis, cataract, and ischemic inflammation in the anterior segment. The vitreous fibrosis can lead to chronic traction on the retina and a delayed retinal detachment. In many cases, removal of a ciliochoroidal tumor necessitates removal of a large portion of the zonular support to the lens. This can lead to postoperative shifting of the lens, with inflammation, corneal edema, or glaucoma.

COMMENTS

The management of malignant melanoma of the posterior uvea is controversial. There is now little doubt that for comparable tumors the chances of survival are similar regardless of whether the eye is removed or another form of conservative therapy is used. Current management can range from periodic observation and fundus photography of selected small lesions that appear dormant to transpupillary thermotherapy, radiotherapy, or local resection for growing tumors in eyes with useful or salvageable vision. In cases in which the tumor is far advanced and there is no hope of useful vision, enucleation generally is advisable.

The choice of therapy is a complex issue, and each case must be considered individually. In selecting a therapeutic approach, certain factors must be carefully weighed; these include the size of the melanoma, its extent and location, its apparent activity, the status of the opposite eye, and the age, general health, and psychologic status of the patient.

Periodic observation can be cautiously used for selected small choroidal melanomas that have dormant characteristics. If such lesions are documented to grow or if they show substantial ophthalmoscopic risks for growth on the initial examination, then active treatment can be instituted. Transpupillary thermotherapy may be an option if the tumor is no more than 12 mm in diameter or 3.5 mm in thickness. For medium-sized or large tumors that are growing, the patient can be managed with either episcleral plaque radiotherapy or local resection of the tumor. Because radiotherapy has fewer immediate visual complications than local resection, more patients are managed with radiotherapy, most commonly in the form of a radioactive plaque.

Patients with large tumors that have produced severe visual loss are managed by enucleation. If there is significant extrascleral extension on initial examination or orbital recurrence

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