Ординатура / Офтальмология / Английские материалы / Clinical Pathways in Glaucoma_Zimmerman, Kooner_2001

120 Glaucoma Associated with Raised Episcleral Venous Pressure: The “Red Eye“ Glaucomas

tion of the orbital veins and soft tissue. Shunting of the blood may produce ocular ischemia, and there may be a pulsatile exophthalmos. Patients with CCSF often (75%) give a history of previous trauma. Many of these patients have a dramatic appearance with pulsatile exophthalmos, chemosis, lid edema, vascular engorgement, and restriction of ocular motility. The conjunctival episcleral veins have a tortuous corkscrew appearance. These findings usually occur on the same side as the fistula, but because of connections between the cavernous sinuses, the findings may be bilateral or alternating.7 Occasionally, the clinical findings may mimic thyroid disease. Typically, the patient is a young man who presents soon after a severe head injury with the above findings. In cases of nontraumatic etiology, the patients are typically postmenopausal women. They generally have no bruit, their proptosis is minimal, and their eyes do not pulsate. Most commonly, patients present because of chronic red eye. The increased IOP in nearly every patient can be severe and may cause blindness.24 These patients often complain of a noise in their ears, and a bruit is often present over the frontal or temporal regions or on the globe. The IOP is elevated because of the increased EVP, although angle closure and neovascular glaucoma have been reported.25,26 Skull films, orbital ultrasonography, or CT or magnetic resonance imaging (MRI) scans confirm the diagnosis along with the initial clinical impression, but arteriography provides the most detailed information about these fistulas. Treatment can be difficult and is usually reserved for individuals who have severe pain, incapacitating bruit, progressive glaucomatous vision loss, or other serious complications.27 A variety of embolization and balloon catheter techniques have been developed with increasing success, but there is still a fairly high morbidity and mortality.

Does Orbital Varix Cause Glaucoma?

Orbital varices are typically found in young persons who have a history of intermittent unilateral proptosis beginning in early childhood. The proptosis may be worsened by increasing the venous pressure in the head and neck such as found in the Valsalva maneuver or bending over. Patients present with dilated veins in the eyelid, anterior orbit, sometimes acute orbital hemorrhage, or thrombophlebitis. Approximately 50% of these patients will have other systemic venous abnormalities involving the scalp, palate, and forehead, and rarely it may be associated with Klippel-Trenaunay-Weber syndrome. The conjunctiva may show multiloculated cysts, which may be misdiagnosed as orbital lymphangioma. Orbital venography will show venous angioma composed of a network of large, dilated, and tortuous veins overlying the frontal bone draining into a dilated angular vein. In a typical patient, as reported by Rathburn et al28 in 1970, the dye flowed into a single inferior ophthalmic varix and then into the cavernous sinus. The superior ophthalmic vein was not involved. There was no evidence of intercranial venous abnormality in the cavernous sinus. Their patient required surgery because of intermittent exophthalmos and an incapacitating headache. Following surgical removal of the varix, the headaches disappeared, as did the cosmetic deformity. The diagnosis of orbital varix can be further delineated using orbital radiography, which may show orbital phleboliths and an enlarged orbit. Ultrasonography and CT scan show a soft tissue mass, and ultrasonography will show the mass to enlarge during

the Valsalva maneuver. Glaucoma is uncommon in this condition because of the transient nature of the malformation, and generally the glaucoma, when present, does not respond to medical therapy.4

Is Glaucoma Apparent in Sturge-Weber Syndrome?

Sturge-Weber syndrome, also known as encephalofacial angiomatosis or encephalotrigeminal angiomatosis, is seen with a flat facial hemangioma that follows the distribution of the fifth cranial nerve. A meningohemangioma, which may produce a seizure disorder in the child, may also be present. The meningohemangioma may be associated with calcification easily revealed by skull x-rays occurring as early as age 1 year. The association of facial hemangiomatous nevi with neurologic impairment was first clinically delineated by Sturge in 1879 in a young girl, as cited in Bodensteiner and Roach.29 In 1922, Weber wrote the first report of the radiologic features of the syndrome. In this condition, the classic manifestations include the facial port-wine stain, a contralateral hemiparesis, hemiatrophy of the brain, and mental retardation with a homonymous hemianopsia. Other features include glaucoma, dental abnormalities, and skeletal lesions. There is no clear genetic pattern, and two affected individuals almost never arise in the same family. The syndrome presents in all races and with equal frequency in both sexes. A variant of the condition is called Klippel-Trenaunay-Weber syndrome, which is seen in children who have extensive involvement of the limbs and trunk. The glaucoma seen in Sturge-Weber syndrome occurs in anywhere from 30 to 70% of patients. It may be associated with outflow obstruction by congenital malformation of the anterior chamber angle. It may be related to hypersecretion from an associated angioma of the choroid and most probably is related to the EVP of the episcleral hemangioma. The prominent neurologic features include seizures, focal neurologic defects, and mental retardation. Port-wine stains are progressive lesions, and the location of the port-wine stain predicts its response to pulse dye laser treatment. Concerning the ocular manifestations, the glaucoma is usually unilateral when the cutaneous lesion affects one side, but bilateral cases do occur. The management of the glaucoma is difficult and typically requires surgery.7

The visual field defects may occur as a manifestation of involvement of the cerebral cortex. A choroidal hemangioma is present in about 40% of the cases and is sometimes very subtle and difficult to identify. It typically is described as the “tomato catsup fundus.” Glaucoma is especially common if there is involvement of the upper eyelids, and presents in early infancy 60% of the time. Some researchers advocate goniotomy as the best surgical option in younger patients because of its low complication rate and its reasonable success.30 Because of the multiple etiology, other authors believe a combined trabeculotomy/trabeculectomy is best for early onset glaucoma.31 When either of these procedures fail, the Ahmed glaucoma valve implant has been studied in a fairly large group of Sturge-Weber patients and has an acceptable success rate.32 No matter what surgery is done, approximately 25% will experience intraoperative or early postoperative choroidal detachment resulting from a rapid expansion of the choroidal hemangioma with effusion of fluid into the suprachoroidal and subretinal spaces. Maintaining a normal to high IOP throughout the surgery through the injection of a viscoelastic material into the anterior chamber may

122 Glaucoma Associated with Raised Episcleral Venous Pressure: The “Red Eye“ Glaucomas

help prevent some of these intraoperative complications. Posterior sclerostomy, followed by anterior chamber reformation, should be performed in the event that an expulsive suprachoroidal hemorrhage occurs. Extreme caution is advised to prevent penetration of the choroid, because penetration would lead to a disastrous hemorrhage.

Is Glaucoma Caused by Orbital-Meningeal Shunts— the Dural Shunt Syndrome (DSS)?

Dural fistulas are communications between the cavernous sinus and an extradural branch of the external or interior carotid artery. The fistulas generally have lower blood flow and lower mean pressure. The clinical appearance in these patients is far less dramatic than the appearance of those with CCSF. These patients lack bruits, and have a variable exophthalmos and variable limitations of motility. However, the conjunctival episcleral vessels have the same corkscrew, arterialized appearance and the IOP is elevated. This condition is commonly seen in elderly women with no precedent history of trauma. Dural fistulas can close spontaneously and may not require treatment. All attempts should be made to control the elevated IOP with medications until the dural shunt resolves. In most of these cases, the elevated IOP is secondary to EVP and is a well-recognized feature of DSS. However, shallowing of the anterior chamber is a rare finding, as is ocular ischemia with rubeosis. Fiori et al33 presented three cases with elevation of IOP and angle shallowing, while one case also exhibited rubeotic glaucoma. Abnormalities of vision can also occur with vascular disorders involving the occipital lobe. Arteriole and venous disease may cause loss of visual field, distortion of vision, visual hallucinations, and palinopsia. Seven patients have been so described by Kupersmith et al,34 although there is no mention of glaucoma in these patients. These dural shunts frequently have been misdiagnosed early in the course of the disease, and conditions to be ruled out include migraines, cluster headaches, endocrine ophthalmopathy, chronic conjunctivitis, episcleritis, iritis, and orbital tumor.35 An unusual carotid artery fistula was reported by Nagaki et al36 in a 77-year-old woman following routine cataract surgery. At the 1-month follow-up visit, a choroidal detachment was noted in the eye and a CT scan showed enlargement of the superior ophthalmic vein. Furthermore, cerebral angiography revealed fistulas between the meningeal branch of both the internal and external carotid arteries and the cavernous sinus. Neurosurgical treatment was performed and the symptoms disappeared.

Are Carotid-Jugular Venous Shunts

Associated with Glaucoma?

Atypical causes of “red eye shunt syndrome” were reported in 1961.4 The researchers studied 14 cases of glaucoma caused by increased EVP with an extraocular origin. They believed that the fistula was from the carotid artery directly into the jugular complex, and was a low-flow fistula. They did tonographic studies in the patients and concluded that there was a relatively normal outflow facility. As cited by others, Nordman is credited by Weinreb and Karwatowski4 with reporting intraocular vascular shunts concerning arteriole

venous fistulas, increased EVP, and glaucoma. Further detailed analysis on this subject is unavailable at this time due to a lack of translation.

Do Intraorbital Vascular Shunts Cause Glaucoma?

Arterial venous shunts in the orbit are quite rare, and most are a part of more extensive intracranial or facial arterial venous malformations (AVMs). Of over 600 orbital tumors studied by Wright,37 only three were AVMs. These lesions were congenital with numerous large feeding arteries, a central nidus, and numerous dilated draining veins. In contrast to the AVMs, arterial venous fistulas are characterized by a single arteriovenous connection within the vascular mass. Most of these fistulas in the orbit occur after an injury to an ethmoidal artery caused by fracture of the ethmoid bone and rupture of the artery into the ophthalmic venous system. Recently, it has been reported that a spontaneous arterial venous fistula occurred in the orbit of a 73-year-old woman with a 1-year history of mild proptosis.38 She did in fact have a glaucoma, and the hemodynamic characteristics of this fistula were quite complicated. The conclusion was that a complete analysis of the hemodynamics by means of selective cerebral angiography was needed for differential diagnosis between these conditions.

Lacey et al39 have undertaken a systematic review of these lesions as part of a continuum of research on the subject of vascular lesions of the orbit. They presented a large collection of patients seen over a 20-year period with orbital vascular malformations studied in a variety of ways. They were specifically interested in the distensible venous malformations. What was unique about their study was that a selected group of patients underwent intraoperative venography and embolization of their malformations. Direct intralesional venography identified the extent of the lesion and the drainage pathway. Pressure was used to achieve control of outflow, which upon angiography confirmation was followed by injection of a cyanoacrylic glue mixture into the lesion to form a cast. The vascular lesion and its cast were excised in a relatively blood-free procedure. This may represent the current state of the art for dealing with these lesions. The authors comment that it is particularly important that the hemodyamics of the venous malformation be understood before embolization is performed. Occlusion of the drainage pathway of a lesion will lead to expansion or stasis within any remaining portion if it is not completely excised. Such altered hemodyamics may favor postoperative thrombosis or hemorrhage and this may be the cause of some of the more serious complications that have been seen in the past.

Do Idiopathic or Sporadic Cases of Increased EVP Occur and Cause Glaucoma?

Patients with dilated episcleral vessels with elevated IOP frequently occur with exophthalmos. These are typically caused in the conditions of carotid cavernous fistula, dural arteriovenous shunts, orbital varices, and pulsatile exophthalmos. Dilated episcleral veins without exophthalmos have been described in patients with dural arteriovenous fistulas, in familial cases, in idiopathic cases,40 sometimes in patients with Sturge-Weber, in patients with advanced glaucoma, and in those with extraocular venous obstruction. Talsman et al41

124 Glaucoma Associated with Raised Episcleral Venous Pressure: The “Red Eye“ Glaucomas

reported six unilateral cases and one bilateral case of dilated episcleral veins with elevated IOP without exophthalmos. They were careful to rule out the common causes of this syndrome. Their workup included orbital venography and carotid arteriography. Inflammatory stenosis of the jugular veins and superior vena cava obstruction were ruled out on clinical grounds. Intraocular tumors were also excluded because there were no sentinel vessels. They believe that this condition is common. Moreover, the authors stressed that mistakes often occur in working up patients who have a chronic red eye and have been erroneously described as having a chronic conjunctivitis. Absence of diffuse congestion of the conjunctival vessels with elevation of IOP and accompanying glaucomatous optic disc cupping and pallor should alert the clinician to initiate a serious workup.

Are There Familial Causes of Increased EVP

and Glaucoma?

Glaucoma associated with idiopathic elevated EVP was found in two members of a family and reported by Minas and Podos42 in 1968. Both were affected unilaterally with large episcleral veins, significantly elevated EVP, raised IOP, cupping of the disc, open angles with budding shunt vessels, and glaucomatous field loss. One of the patient’s mother had a similar picture in both eyes. Neither case was found to have any of the entities known to produce elevated EVP.

Does Chronic Use of Systemic and/or Topical Medications

Affect the EVP and Glaucoma?

The effects of various pharmacological agents on EVP have been evaluated in numerous studies. One report on pilocarpine demonstrates decreased EVP; however, these measurements were reported relatively high and were probably unreliable.43 Another investigator found that pilocarpine decreased EVP,44 and a third study found that there was no effect from pilocarpine and acetazolamide on EVP.45 Similarly, studies evaluating epinephrine’s effect had equivocal results.46,47 Clondine seems to decrease EVP, but the changes are too small to account for any change in the IOP.48 Also, in rabbits exposed to oxygen, there was a decrease in IOP and EVP.49 Some experiments have shown that exposure of the eye to cold decreases EVP.50 Recently, Netland et al51 has used calcium channel blockers in the management of low tension and open angle glaucoma. Sawada et al52 reported prevention of visual field defect progression with brovincamine in eyes with normal tension glaucoma. An interesting study analyzing topical verapamil and decreased EVP was reported by Abreu et al.53 They studied 20 normal human subjects in a prospective, double-masked, randomized, crossover placebo study. The authors reported that calcium channel blockers are frequently used for the treatment of various cardiac disorders including angina pectoris, cardiac arrhythmia, systemic hypertension, and Raynaud’s phenomenon. The calcium channel blockers reduce vascular resistance and help prevent vasospasm by blocking the entry of calcium into the cells. The ocular effects of calcium channel blockers were studied a few years ago with a flurry of activity, and their potential clinical role became apparent, especially in treating patients with normal tension glaucoma. There is typically an arterial vasodilation with reduction of arterial blood pressure. The reduc-

tion in IOP caused by calcium channel blockers may be due at least in part to increased outflow facility, with resultant effect on EVP.

Treatment and Management

It is apparent that the treatment and management of the 17 causes of increased EVP and glaucoma is challenging and individualized. In general, management of the glaucomas would involve medical treatment first, using combinations of drug classes, advancing to laser trabeculoplasty if the angle structures are normal and accessible. Filtration surgery, generally of the guarded variety with or without antimetabolites, would be the next choice. In advanced cases, implantation of glaucoma valves with or without antimetabolites or possibly cyclodestructive procedures should be considered. There are no large studies comparing surgical procedures because these conditions are relatively uncommon and present sporadically to a variety of practitioners. We should, however, be reminded that the red eye glaucomas may be a more common cause of glaucoma than is universally recognized. A careful and thorough evaluation and treatment of the underlying cause is required.

Future Considerations

Epstein9 in 1997 was the first to point out that there are some basic aspects concerning increased EVP that need investigation. First, his measurements have shown that reducing the IOP by filtering operations in the syndrome of openangle glaucoma associated with elevated EVP does not change EVP. He concluded that the pressure in these vessels must be determined by something other than the rate of outflow of aqueous humor. After successful filtration surgery, aqueous humor is no longer restricted by the regular outflow system to enter the episcleral veins against the venous back pressure but is free to bypass the system and escape into the extravascular tissue at low IOP. Second, Epstein reports that in certain cases where there is increased EVP of idiopathic origins, no blood has been seen gonioscopically in Schlemm’s canal either before or after surgery. This despite the fact that filtration operations have made the IOP considerably lower than the pressure in the episcleral veins on the surface of the globe. It was suggested that obstruction may lie within or beyond Schlemm’s canal. Similar phenomena may be involved in the pathogenesis of reduced aqueous humor outflow, as is commonly seen with chronic elevation of EVP.

In light of the current trends to perform nonpenetrating filtering surgery,54,55 it would be interesting to know what the EVP changes are once this “lake of aqueous” has been created in the midscleral space. Once identified, pharmacologic modulation may enhance filtration and long-term success.

Pharmacologic manipulations of the EVP in unoperated eyes are another area of active research. Abreu et al53 did a prospective, randomized, doublemasked, crossover, placebo-controlled study testing one eye of 20 normal subjects with either the calcium channel blocker verapamil or placebo. Their results indicate that a single drop and a 2-week administration of topical verapamil decreased IOP and EVP significantly, with more pronounced reduction

126 Glaucoma Associated with Raised Episcleral Venous Pressure: The “Red Eye“ Glaucomas

at the 2-week treatment than after a single dose treatment. The mechanism of the calcium channel blockers has been discussed by Netland et al51 and Sawada et al.52 There may be a future in this area of investigation, especially concerning the treatment of low-tension glaucoma patients. Because the calcium flux can affect the aqueous humor dynamics, there may be a hydrostatic component caused by an efferent arterial blood pressure and ciliary body perfusion and an osmotic component caused by an effect on the active secretion of sodium, calcium, and other ions by the ciliary epithelium. The mechanism may be modulation of aqueous outflow by decreasing EVP.

The complexity of the control mechanism for vascular plexus involved in episcleral venous drainage has recently been examined by Selbach et al,5 using scanning electron microscopy of vascular resin casts of the rat and rabbit eye. They also did fluorescein immunohistochemistry to investigate the multiple neuropeptides in these areas. They discovered that the episcleral arteriovenous anastomosis connects arterioles directly with the episcleral venous plexus, which also drains the aqueous humor. The nerve fiber layer plexus around the episcleral arteriovenous anastomosis is far more dense than around the arteriovenous connections at the limbal arcade. They concluded that there is an elaborate innervation in this area that may be involved in subtle modulations of the blood flow and possibly of the aqueous humor outflow dynamics. Basic anatomic research would be helpful in humans, as well as possible pharmacologic manipulation of these various neuropeptides.

References

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2.Hieshima GB, Higashida RI, Halback UV: Advances in the diagnosis and treatment of carotid cavernous fistula. Ophthalmology 1986;93 (clinical progress suppl):69.

3.Keltner JL, Satterfield D, Dublin AB, et al: Dural and carotid cavernous sinus fistulas. Ophthalmology 1987;94:1585–1600.

4.Weinreb RN, Karwatowski WSS: Glaucoma association with elevated episcleral venous pressure. In: Ritch R, Shields MB, Krupin T (eds): The Glaucomas, 2d ed. St. Louis: CV Mosby, 1996:1143.

5.Selbach JM, Scholfelder U, Funk RHW: Arteriovenous anastomoses of the episcleral vasculature in the rabbit and rat eye. J Glaucoma 1998;7:50–57.

6.Warwick R: Eugene Wolff’s anatomy of the eye and orbit, 7th ed. Philadelphia: WB Saunders, 1976.

7.Stamper RL, Lieberman MF, Drake MV: Becker-Shaffer’s diagnosis and therapy of the Glaucomas, 7th ed. St. Louis: CV Mosby, 1999: chapters 4, 18, and 20.

8.Kupfer C, Sanderson P: Determination of pseudofacility of the eye in man. Arch Ophthalmol 1968;80:194.

9.Epstein KD: Glaucoma associated with extraocular venous congestion (increased episcleral venous pressure). In: Epstein DL, Allingham RR, Schuman TS (eds): Chandler and Grant’s Glaucoma, 4th ed. Baltimore: Williams and Wilkins, 1997: chapter 46.

10.Kupfer C: Clinical significance of pseudofacility. Am J Ophthalmol 1973;75:193–204.

11.Bill A: Physiology of the outflow mechanism. In: Drance SM, Neufeld AH (eds): Glaucoma: Applied Pharmacology in Medical Treatment. New York: Grune and Stratton, 1984.

12.Jones L, Reeh M, Wirtschefter J: Ophthalmic anatomy: a manual with some clinical applications. AAO continuing education program. Rochester, MN: AAO, 1970.

13.Zeimer RC, Gieser DK, Wilensky JT, et al: A practical venomanometer. Arch Ophthalmol 1983;101:1447–1449.

14.Talusan ED, Schwartz B: Episcleral venous pressure. Arch Ophthalmol 1981;99:824-828.

15.Harris GJ: Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Am J Ophthalmol 1999;127:453–455.

16.Jorgensen JS, Guthoff R: The role of episcleral venous pressure in the development of secondary glaucoma. Klin Monatsbl Augenheilkd 1988;193:471–475.

17.Alfano JE: Glaucoma following ligation of the superior venacava. Am J Ophthalmol 1956;42:412–414.

18.Lokich JJ, Goodman R: Superior vena cava syndrome. JAMA 1975;231:58–61.

19.Bettelheim H: Episcleral venous pressure in pulmonary hypertension. A contribution to the problem of “cardiogeneous” glaucoma. Graefes Arch Klin Exp Ophthalmol 1969;177:108–115.

20.Fu E: Bilateral corkscrew episcleral veins from tricuspid incompetence. Am J Ophthalmol 1996;122:577–578.

21.Brismar G, Brismar J: Aseptic thrombosis of orbital veins and cavernous sinus. Acta Ophthalmol 1977;55:9–21.

22.Meyer O: Inflammatory jugular phlebostenosis as the cause of glaucoma exogenicum. Br J Ophthalmol 1946;30:682–688.

23.Friberg TR, Sanborn G, Weinreb RN: Intraocular and episcleral venous pressure increase during inverted posture. Am J Ophthalmol 1987;103:523–526.

24.Phelps CD, Thompson HS, Ossoinig K: The diagnosis and prognosis of atypical carotidcavernous fistula (red-eyed shunt syndrome). Am J Ophthalmol 1982;93:423–436.

25.Weekers R, Delmarcelle Y: Pathogenesis of intraocular hypertension in cases of arteriovenous aneurysm. Arch Ophthalmol 1952;48:338–343.

26.Buus D, Tse D, Parrish R: Spontaneous carotid cavernous fistula presenting with acute angle closure glaucoma. Arch Ophthalmol 1989;107:596–597.

27.Kupersmith MJ, Berenstein A, Choi IS, et al: Management of nontraumatic vascular shunts involving the cavernous sinus. Ophthalmology 1988;95:121–130.

28.Rathburn JE, Hoyt WF, Beard C: Surgical management of orbitofrontal varix in Klippel- Trenaunay-Weber syndrome. Am J Ophthalmol 1970;70:109–12.

29.Bodensteiner JB, Roach ES: Sturge-Weber syndrome. Mt. Freedom, NJ: Sturge-Weber Foundation, 1999.

30.Iwach AG, Hoskins Jr HD, Heatherington Jr J, et al: Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology 1990;97:904–909.

31.Mandal AK: Primary combined trabeculotomy-trabeculectomy evaluation for early onset glaucoma in Sturge-Weber syndrome. Ophthalmology 1999;106:1621–1627.

32.Hamush NG, Coleman AC, Wilson MR: Ahmed glaucoma valve implant for management of glaucoma in Sturge-Weber syndrome. Am J Ophthalmol 1999;128:758–760.

33.Fiore PM, Latina MA, Singleton BJ, et al: The dural shunt syndrome. Ophthalmology 1990;97:56–62.

34.Kupersmith MJ, Berenstein A, Nelson PK, et al: Visual symptoms with dural arteriovenous malformations decreasing draining into occipital veins. Neurology 1999;52:156–162.

35.Harbison JW, Guerry D, Wiesinger H: Dural arteriovenous fistula and spontaneous choroidal detachment: new cause of an old disease. Br J Ophthalmol 1978;62:483–490.

36.Nagaki Y, Hayasaka S, Kadoi C, et al: Carotid artery fistula after cataract surgery. Ophthalmic Surg Laser 1999;30:160–162.

37.Wright JE: Orbital vascular anomalies. Trans Am Acad Ophthalmol Otolaryngol 1974;78: 606–616.

38.Ohtsuka K, Hashimot M: Clinical findings in a patient with spontaneous arteriovenous fistulas of the orbit. Am J Ophthalmol 1999;127:736–737.

39.Lacey B, Rootwan J, Marotta TR: Distensible venous malformations of the orbit. Ophthalmology 1999;106:1197–1209.

40.Radius RL, Maumenee AE: Dilated episcleral vessels and open-angle glaucoma. Am J Ophthalmol 1978;86:31–35.

41.Talusan ED, Fishbein SL, Schwartz B: Increased pressure of dilated episcleral veins with openangle glaucoma without exophthalmos. Ophthalmology 1983;90:257–265.

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43.Thomasson TL: The venous tension in eyes suffering from simple glaucoma. Acta Ophthalmologica 1947;25:221.

44.Bain WES: Variations in the episcleral venous pressure in relation to glaucoma. Br J Ophthalmol 1954;38:129.

45.Linner E: Further studies of the episcleral venous pressure in glaucoma. Am J Ophthalmol 1956;41:646.

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128 Glaucoma Associated with Raised Episcleral Venous Pressure: The “Red Eye“ Glaucomas

48.Krieglstein GK, Langham ME, Leydhecker W: The peripheral and central neural actions of clonidine in normal and glaucomatous eyes. Invest Ophthalmol Vis Sci 1978;17:149.

49.Yablonski ME, Gallin P, Shapiro D: Effect of oxygen on aqueous humor dynamics in rabbits. Invest Ophthalmol Vis Sci 1985;26:1781.

50.Ortiz GJ, et al: Effect of cold air on aqueous humor dynamics in humans. Invest Ophthalmol Vis Sci 1988;29:138.

51.Netland PA, Chaturvedi N, Dreyer EB: Calcium channel blockers in the management of lowtension and open-angle glaucoma. Am J Ophthalmol 1993;115:608–613.

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7

Glaucoma Associated with Primary Disorders of the Corneal Endothelium

Sylvia L. Hargrave

Two conditions with disorders of the corneal endothelium are discussed in this chapter. Both iridocorneal endothelial (ICE) syndrome and posterior polymorphous dystrophy are conditions in which there is strong clinical and histopatholigic evidence that the corneal endothelial disorder is directly associated with the changes causing the glaucoma.

Definition

The ICE syndrome includes several related disorders: essential iris atrophy or progressive iris atrophy, iris nevus (Cogan-Reese) syndrome, and Chandler’s syndrome. Glaucoma is an important feature of this syndrome, and the related disorders may represent a continuum of the same process. Scheie and Yanoff1 and Yanoff2 proposed the term ICE to group essential iris atrophy, Chandler’s syndrome, and iris nevus syndrome as varying clinical manifestations of a single disease entity.

What Is Progressive (Essential) Iris Atrophy?

A form of secondary glaucoma associated with atrophy of the iris and hole formation was described by Harms3 in 1903. The condition was termed essential iris atrophy or progressive essential iris atrophy. Progressive iris atrophy is characterized by prominent atrophy of the iris, with iris stromal and/or fullthickness iris holes. Peripheral anterior synechiae develop early, and progress both circumferentially and onto the cornea.4 Pupillary distortion and ectropion uveae occur typically toward the most prominent anterior synechiae.4,5 More