**1. Introduction**

#### **1.1. Definition**

Glaucomatocyclitic crisis was initially described in detail by Posner and Schlossman in 1948, so it was also called Posner – Schlossman syndrome (PSS). PSS is a special form of anterior uveitis with glaucoma, mainly seen in young adults, characterized by non-granulomatous iridocyclitis with significant elevation of intraocular pressure. In most cases, the disease took a form of acute, recurrent and monocular onset. [1, 2]

### **1.2. Morbidity**

This disease is often seen in people aged from 20 to 50, and rarely in people above 60 years old. 5% or less of PSS cases were reported in people above 60 years old [3]. The disease is rare in Western countries, it was reported that 19 in one million people have suffered from PSS in Fen‐ land [4]. As PSS is a kind of disease attacks intermittently,It is difficult to diagnose PSS in inter‐ mittent period for the lack of diagnostic signs and investigate the morbidity with epidemiology methods.We used the full text VIP Chinese literature retrieval system and Medeline retrieval system to find PSS reports from 1975 to 2011 in both English and Chinese literature and divid‐ ed into review, case, experimental study and clinical report four categories,then analysed the regional distribution of the authors and cases (Chinese reports were divided into the Yangtze river and the other, English reports were divided into Asia and the other).

Chose the report which contained the most number of cases if there were more than two from the same author. Statistical results shows that 1262 cases were reported by 33 chinese clinical reports in which 991 cases reported by 20 reports from the area near the Yangtze river,and 271

© 2013 Zhou et al.; licensee InTech. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2013 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

cases reported by 13 reports from the other area. 211 cases were reported by 16 English clinical reports in which 144 cases reported by 7 reports from the asia area and 67 cases reported by 9 reports from the other area. The results above suggest that there are much more literature and cases related to PSS in the area near the Yangtze river and infer that the prevalence of PSS in that area may be higher.

CMV DNA positive anterior uveitis cases was consider to be accompanied with endothelial halo,clinical difference was not so significant between CMV DNA positive and negative cases as less aqueous humorin sample and weak sensitivity of detection method. In 2008, aqueous analysis for CMV by PCR was performed in 103 eyes of 102 patients with presumed PSS or FHI at the Singapore National Eye Centre. Their records were reviewed for clinical features and human immunodeficiency virus (HIV) status of the CMV-positive patients. The main parameters were age, gender, maximum intraocular pressure, endothelial cell count, endo‐ thelial changes, PCR results, and presence of uveitic cataract and/or glaucoma. It was found that there was no clinically detectable differences between CMV-positive and negative presumed PSS eyes. CMV-positive presumed FHI patients are more likely to be male, older at

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It was reported in South Korean that there was a significant difference of the positive rate of helicobacter pylori serum antibody between cases with PSS (80%) and cases without PSS(56.2%). In another prospective study, 40 cases with PSS and 73 cases without PSS received serologic analysis for the presence of H. pylori infection by an enzyme-linked immunosorbent assay. Positive rate of serum anti-H. pylori IgG was compared between the two groups. It was proved that H. pylori infection occurred significantly more often in PSS patients. This study

**1.** In most cases, the disease always attacks the identical eye repeatedly, binocularly affected

PSS result in paroxysmal increase of IOP repeatedly,which reaches as high as 40 to 60 mmHg, and lasts for 1 to 14 days generally,1 month occasionally,2 months rarely, interval of onset is

**3.** Eyesight is normal generally, blurred when suffer from corneal edema at onset;

**6.** The anterior chamber angle is open, no matter IOP is normal or elevated;

vascular shadow may occur during an acute onset;

**4.** Pupil becomes bigger slightly with normal reaction to light, and never adheres to lens;

**5.** The KP of PSS appeared in a few days after or before the elevation of IOP with number of 1 to 25, took a form of hoar and suet-shaped and disappeared days to 1 month after the IOP returned normal , distributed mainly in the inferior part of the cornea or concealed in the trabecular meshwork. There were no or at most a few planktonic cells in aqueous while the flare was negative .There is no inflammatory cell in vitreous body ( See Figure 1);

Visual field and fundus of most cases is normal generally, but a reversible expanding of

suggests that exposure to H. pylori infection is associated with PSS in Korea. [10]

diagnosis or have nodular endothelial lesions. [9]

**1.4. Clinical features of typical cases [1, 2, 3, 11, 12, 13]**

**2.** Symptoms are not obvious, just mild discomfort for most cases;

**4.** Helicobacter pylori infection.

cases is not common;

from months to 2 years;


**Table 1.** The regional distribution of the authors

#### **1.3. Possible etiology**


The conclusion that PSS was caused by herpes simplex virus (HKS) was reported by Yaina‐ motos in 1995, and was confirmed by many following researches. A recent report showed that antiviral treatment reduced the frequency of the outbreak of the disease. [6-7]

It was reported that aqueous humor of a binocular PSS case were collected after suffering from herpes viral keratitis for five months with anterior chamber paracentesis, then DNA of cytomegalovirus (CMV) and HKS were measured by means of quantitative polymerase chain reaction( PCR), the results showed CMV was positive but HKS was negative. It was speculated that CMV which belongs to herpes virus genera would also leads to PSS like as wise. It was considered that PSS is not a separate disease, but a kind of anterior uveitis relating to infections of herpes virus.

A study from Singapore showed that the CMV DNA of aqueous humor was positive for 24 of 104 anterior uveitis cases with monocular high IOP, in which 18 cases were PSS, 5 cases were Fuchs heterochromic iridocyclitis (FHI). [8]Another study showed CMV DNA was positive in 35 of 67 PSS cases (52.2%), 15 of 35 FHI cases (41.7%). Although the kerato-precipitates (KP) in CMV DNA positive anterior uveitis cases was consider to be accompanied with endothelial halo,clinical difference was not so significant between CMV DNA positive and negative cases as less aqueous humorin sample and weak sensitivity of detection method. In 2008, aqueous analysis for CMV by PCR was performed in 103 eyes of 102 patients with presumed PSS or FHI at the Singapore National Eye Centre. Their records were reviewed for clinical features and human immunodeficiency virus (HIV) status of the CMV-positive patients. The main parameters were age, gender, maximum intraocular pressure, endothelial cell count, endo‐ thelial changes, PCR results, and presence of uveitic cataract and/or glaucoma. It was found that there was no clinically detectable differences between CMV-positive and negative presumed PSS eyes. CMV-positive presumed FHI patients are more likely to be male, older at diagnosis or have nodular endothelial lesions. [9]

**4.** Helicobacter pylori infection.

cases reported by 13 reports from the other area. 211 cases were reported by 16 English clinical reports in which 144 cases reported by 7 reports from the asia area and 67 cases reported by 9 reports from the other area. The results above suggest that there are much more literature and cases related to PSS in the area near the Yangtze river and infer that the prevalence of PSS in

**research**

0 11 1 20 32

Other areas 2 11 0 13 26

Other areas 3 12 0 9 24

**Clinical report**

**Aggregate**

**Region Review Case Experimental**

English Asia 2 0 2 7 11

and abnormal development in angle of anterior chamber.[1]

Aggregate 7 34 3 49 93(total)

**1.** Many factors were considered to be related to the onset of PSS, such as allergy, fatigue, mental fatigue, mental stress, decreased body resistance, infection, hypothalamic disor‐ ders, autonomic dysfunction, abnormal reactions of ciliary vascular and nervous system

**2.** Recent research had confirmed that concentration of prostaglandins,(PGs) in the anterior chamber aqueous increased obviously in the PSS cases, especially that of PGE. [5]

The conclusion that PSS was caused by herpes simplex virus (HKS) was reported by Yaina‐ motos in 1995, and was confirmed by many following researches. A recent report showed that

It was reported that aqueous humor of a binocular PSS case were collected after suffering from herpes viral keratitis for five months with anterior chamber paracentesis, then DNA of cytomegalovirus (CMV) and HKS were measured by means of quantitative polymerase chain reaction( PCR), the results showed CMV was positive but HKS was negative. It was speculated that CMV which belongs to herpes virus genera would also leads to PSS like as wise. It was considered that PSS is not a separate disease, but a kind of anterior uveitis relating to infections

A study from Singapore showed that the CMV DNA of aqueous humor was positive for 24 of 104 anterior uveitis cases with monocular high IOP, in which 18 cases were PSS, 5 cases were Fuchs heterochromic iridocyclitis (FHI). [8]Another study showed CMV DNA was positive in 35 of 67 PSS cases (52.2%), 15 of 35 FHI cases (41.7%). Although the kerato-precipitates (KP) in

antiviral treatment reduced the frequency of the outbreak of the disease. [6-7]

that area may be higher.

380 Glaucoma - Basic and Clinical Aspects

Chinese Near the

**1.3. Possible etiology**

**3.** Infection by herpes virus.

of herpes virus.

Yangtze river basin

**Table 1.** The regional distribution of the authors

**Literature types**

> It was reported in South Korean that there was a significant difference of the positive rate of helicobacter pylori serum antibody between cases with PSS (80%) and cases without PSS(56.2%). In another prospective study, 40 cases with PSS and 73 cases without PSS received serologic analysis for the presence of H. pylori infection by an enzyme-linked immunosorbent assay. Positive rate of serum anti-H. pylori IgG was compared between the two groups. It was proved that H. pylori infection occurred significantly more often in PSS patients. This study suggests that exposure to H. pylori infection is associated with PSS in Korea. [10]

#### **1.4. Clinical features of typical cases [1, 2, 3, 11, 12, 13]**

**1.** In most cases, the disease always attacks the identical eye repeatedly, binocularly affected cases is not common;

PSS result in paroxysmal increase of IOP repeatedly,which reaches as high as 40 to 60 mmHg, and lasts for 1 to 14 days generally,1 month occasionally,2 months rarely, interval of onset is from months to 2 years;


Visual field and fundus of most cases is normal generally, but a reversible expanding of vascular shadow may occur during an acute onset;

**7.** Coefficient of outflow facility (C value) descends in episodes and recovery as IOP in intermission; various stimulation tests for glaucoma are negative in intermission.

The antiviral treatment systemically or implanting long-acting agents maybe helpful to reduce the frequency of attack, but it has worrying and serious side effects and cost too

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383

It was considered in the early years that PSS have a favorable prognosis without glau‐ comatous damage of optic disk and visual field, however, a number of authors have con‐ firmed that part of the PSS cases suffered from glaucomatous damage similar to that in primary glaucoma patients in recent years. A lot of questions remained vague such as monocular or binocular, age of onset, the detailed features of its IOP and KP, the inci‐ dence and degree and relating factors of glaucomatous damage, especially the clinical approaches via which the damage occurred and disease complicated with PSS. These brought about to two undesirable consequences: first, PSS patients were misdiagnosed as primary glaucoma and received incorrect treatment even led to serious adverse conse‐ quences due to the lack of knowledge on the clinical characteristics of PSS.On the other hand, most cases of PSS combined with primary glaucoma patients especially these with primary angle-closed glaucoma were failed to be diagnosed correctly without delay, thus

In order to solve the problems mentioned above, we have made a long-term systematic clinical

The main results of our clinical research included 4 fields as following: the clinical character‐ istics of PSS; the glaucomatous optic nerve damage in PSS patients; the clinical approach of

The research about clinical characteristics of the PSS included four aspects: clinical observation and analyzation of monocular primary open-angle glaucoma(POAG) and binocular PSS; clinical features of elderly PSS patients; characteristics and clinical value of the intraocular pressure and the C- value in PSS patients ; the characteristic of postural intraocular pressure

*3.1.1. Clinical observation and analyzation of monocular primary open-angle glaucoma and binocular*

**Background:** As we knew, most of POAG patients are binocularly involved, while monocular attack is one of typical features of PSS. However, clinically suspected monocular POAG patient

**2. The dispute and problems about prognosis**

the best opportunity of treatment lost ; severe damage resulted in.

optic nerve damage in PSS patients; other diseases concomitant with PSS.

study about PSS for more than 20 years persistently.

**3. The main results of our clinical research**

**3.1. Study on the clinical characteristics of the PSS**

change in PSS patients.

*PSS*

much. [12]

**8.** The forms of onset of PSS could be divided into three kinds: KP, high IOP and intermediate type, according to relationship between KP and IOP.

**Figure 1.** Anterior segment of a case with PSS in episodes. Arrows indicate the typical hoar and suet-shaped KP.

#### **Typical case**

The patient complained of her blurred vision two months ago, examination in other hospital showed: conjunctiva of her left eye wasn't congestive and the cornea was edematous mildly, IOP: 34/ 18(R/L) mmHg; there were some round lipid-like KP in the left cornea, aqueous flare (-). She came to our hospital on June 7, 2012, ophthalmologic examination: vision was 0.5/ 1.5(R/ L), best corrected vision of left eye was 1.2(-1.25DS), IOP: 18/ 13 (R/L) mmHg. Her right eye was normal, conjunctiva of her left eye wasn't congestive and there were five rounds lipid-like KP in the left cornea, binocular C/D was 0.4. Her KP faded away after the treatment of chloromethyl and pranopulin (three times a day) for three weeks. Examination of FFA, ICGA and Virus screening were normal on July 10.The measurement of her 24 hours IOP performed two weeks after she ceased the drugs was 20-14mmHg(R), 15-12 mmHg (L). The result of her visual field and the OCT for glaucoma was normal. She was diagnosed as PSS in left eye and suggested to be observed and treated timely.

#### **1.5. Treatment of typical cases in episodes**

**1.** Anti-inflammation: Corticosteroid drugs is needed in most cases, but it should not be used too long a time, so as not to cause the corticosteroid glaucoma.

It is a better select in some cases to apply non-steroidal anti-inflammatory drugs (NSAIDs) such as eye drops of pranoprofen, indomethacin and flufenamic acid.

Reducing IOP: Eye drops of epinephrine, timolol,or clonidine was needed singly or jointly for common patient, carbonic anhydrase inhibitor orally when the IOP is higher than 30mmHg and mannitol of intravenous drip when the IOP is higher than 40mmHg.

The antiviral treatment systemically or implanting long-acting agents maybe helpful to reduce the frequency of attack, but it has worrying and serious side effects and cost too much. [12]
