Clinical characteristics and surgical treatment of idiopathic uveal effusion syndrome

INTRODUCTION

Uveal effusion refers to the leakage of fluid from the choriocapillaris, and may cause exudative detachment of the choroid and retina without retinal breaks. Uveal effusion may be idiopathic or secondary to choroidal tumors,extensive chorioretinitis, and surgery, among others.Macular involvement in exudative retinal detachment leads to significant visual acuity impairment.

The term “uveal effusion syndrome” (UES) was first introduced by Schepens and Brockhurstin 1963; the disease is diagnosed by exclusion when the other causes of uveal effusion have been ruled out. UES is generally divided into nanophthalmic UES and idiopathic UES (IUES). Nanophthalmic UES is easily diagnosed because of the characteristic clinical manifestation,short eyeball axis; IUES is rarely reported in clinical practice and may be misdiagnosed as another exudative choroidal or retinal detachment disease. The causes of IUES remain unknown, and the disease predominantly occurs in otherwise healthy middle-aged people. Gass and Jallowhypothesized that UES is primarily caused by congenital scleral abnormalities that act as barriers to the transscleral outflow of protein from the suprachoroidal space,and secondarily predisposes one to vortex vein obstruction.According to this theory, Gassproposed a surgical management method for the treatment of UES using scleral sclerectomy and sclerostomy; subsequent studies further confirmed the effectiveness of these surgical modalities.In this study, we reviewed the records of patients with an IUES diagnosis treated using surgery, in the past ten years, and analyzed the clinical features and surgical outcomes associated with the disease.

SUBJECTS AND METHODS

This study was approved by Beijing Tongren Hospital Ethical Committee and informed consent was signed by all participants before surgery.

Ophthalmic ultrasound and UBM,among others, can be helpful in the diagnosis of IUES.Sclerectomy and sclerostomy are surgical modalities that can successfully treat the disease. Some patients may experience recurrence after surgery; reoperation remains safe and effective for them. Long-term follow-up is essential in such settings.

All patients underwent routine eye examinations, including binocular indirect ophthalmoscopy, ultrasound examination,optical coherence tomography (OCT), and ultrasound biomicroscopy (UBM), and some underwent fluorescein angiography and indocyanine green angiography. A small number of patients did not undergo angiography examinations due to allergies or poor general conditions. Patients were tested for syphilis, human immunodeficiency virus infection and hepatitis B and C infection and underwent routine blood and urine examinations. The exclusion criteria were as follows:the presence of a short eye axis (eye axis ＜20.5 mm); uveitis;panretinal photocoagulation; intraocular surgery; trauma,and a known cause of ciliochoroidal effusion. Patients with rhegmatogenous retinal detachment and choroidal detachment as a complication were also excluded.

(3) 當逆作法施工采用盆式開挖法挖土時，最大位移出現(xiàn)在地下連續(xù)墻墻頂處，此時應在確保將墻頂位移控制在合理范圍的前提下，基于首層土的開挖深度，確定盆式挖土預留土體的經(jīng)濟寬度。針對逆作法盆式開挖不同坡肩寬度下地下連續(xù)墻的位移變化情況，采用有限元法進行了研究。以往的工程經(jīng)驗表明，當軟土區(qū)首層土盆式開挖深度取6.5 m、坡肩寬度取6.0 m時，即可實現(xiàn)將地下連續(xù)墻的變形控制在合理范圍內的目的。

The causes of UES remain unknown. In 1983, Gasshypothesized that the initial cause of UES is congenital scleral dysplasia, which acts as a barrier leading to the obstruction of the transscleral flow of the suprachoroidal protein and secondary venous occlusion. Retinal detachment and choroidal detachment are resolved by sclerectomy,indirectly confirming that scleral dysplasia causes UES.In 2011, a study that used enhanced depth imaging spectral domain OCT found that the thickness of the choroid was significantly increased in patients with IUES, adding to the theory of Gass pertaining to the scleral barrier to transscleral flow in the disease. In our study, we observed abnormal scleral thickness values on surgery, as well as irregular collagen fiber arrangement and abnormal deposition,indicating the presence of proteoglycans-like in the scleral matrix, as observed on pathological and electro-microscopic examination, further confirming Gass' hypothesis. Previous studies have shown the presence of a complex relationship between collagen fibrils and proteoglycans in the extracellular matrix, leading to transscleral fluid outflow obstruction through these scleral abnormalities. The obstruction of transscleral outflow caused by abnormal sclera first leads to ciliochoroidal detachment; during this period, patients have no clinical symptoms. Our study confirmed, using UBM,that all the participants had binocular leakage and ciliary body detachment.

RESULTS

The baseline characteristics of the 26 patients included in this study are summarized in Table 1. Seventeen (65.4%) of the 26 patients were men, and 9 (34.6%) were women. None of the patients had a family history of the disease. The average age of symptom onset was 46.8y (22-64y). Of the 26 patients,7 (26.9%) had binocular retinal detachment at presentation.The mean duration from symptom onset to surgery was 8.5mo. Three eyes underwent reoperation for recurrent retinal detachment. The time to recurrence in these three eyes was 1,5, and 9y, after surgery, respectively.

Ultrasound examination of the eyes showed that 24 patients had monocular or binocular retinal detachment (Figure 1A).OCT examination showed only macular exudative retinal detachment in two eyes of two patients (Figure 2A). UBM showed effusion of the ciliary body in the binocular eyes among all patients (Figures 3A-3D). Surgery was performed on the eyes showing retinal detachment, including two in which the subretinal fluid was only confined to the macular region, simulating idiopathic central serous chorioretinopathy.The axial length of the surgical eyeballs ranged from 20.9 to 23.7 mm (average 22.5 mm). The thickness of the sclera near the temporal scleral process was examined by UBM and was found to be an average of 0.76 mm. Fluorescence angiography showed mottled high and low fluorescence in the fundus,which was a leopard-like change.

The average preoperative intraocular pressure (IOP) was 14.8(9-27) mm Hg, and the postoperative IOP (the first day after surgery) averaged 13.9 (7-24) mm Hg.

Surgery was performed on all 33 eyes with retinal detachment.Retinal detachment was characterized by exudative retinal detachment and subretinal fluid shifts with changes in the eye or head position; no break or tear was seen on fundus examination, and no viable proliferations were observed in the vitreous (Figure 4A). Macular retinal detachment was predominantly characterized by neuroepithelial detachment,and more often accompanied by retinal retinoschisis.

During the surgery, we observed the spontaneous release of a pale-yellow suprachoroidal effusion fluid as the choroid was exposed. However, the choroidal effusion fluid was intentionally not drained out. The sclera was abnormally rigid and thick, and scleral texture disorder was noted; therefore, the razor blade knife had to be changed frequently. No complications occurred during the surgery. Pathological examination of the surgically excised scleral slice confirmed the presence of scleral collagen fiber arrangement disorder. Electron microscopy of the scleral slice also showed an irregular scleral fiber arrangement and marked deposits of matrix between the fiber bundles (Figure 5).

Three patients with recurrent retinal detachment underwent a second surgery—four-quadrant lamellar sclerectomy—combined with partial full-thickness sclerostomy. The scar tissue was removed during surgery and abnormal scleral thickness was observed again. Retinal detachment was resolved within 4mo after the second surgery.

All patients were followed-up for at least 6mo after surgery.Retinal detachment was gradually resolved from 1wk to 5mo after surgery (Figures 2B and 4B). Pigmentation was observed in the retinal detachment zone. Choroidal detachment resolution was achieved earlier than retinal detachment resolution (Figure 1B), and ciliary body effusion was gradually resolved (Figures 3E-3H). The visual acuity of 30 eyes increased to varying degrees, and there was no significant change in the visual acuity in three eyes.

DISCUSSION

In this study, we only reviewed the clinical data of hospitalized patients who undergone surgery in the past ten years. Some outpatients who were relieved by medical treatment were not included in the analysis. We found that ophthalmic ultrasound and UBM, among others, aided the diagnosis of IUES.Sclerectomy and sclerostomy were associated with treatment success. However, some patients experienced recurrence after surgery; they underwent successful reoperation.

高校學生管理是一項較為復雜的工作，管理者要順應時代的發(fā)展趨勢，堅持以人為本，與時俱進。人本理念在高校學生管理中的運用，最根本的是要解決觀念問題，正確指導學生進行管理體制和方法的創(chuàng)新，要對理論知識進行深入的研討，還要在實踐中不斷的完善管理經(jīng)驗，讓學生們轉變思想觀念，強化服務意識，逐步實現(xiàn)民主交流、平等溝通、和諧統(tǒng)一的校園生活環(huán)境。

UES was first reported in 1963 by Schepens and Brockhurst;however, only a few cases of the disease have been reported to date. The incidence of IUES is even lower in the existing literature. To the best of our knowledge, the current study, which included 33 eyes from 26 patients, presents the largest number of IUES cases to date. We reviewed the records of IUES patients with an axial surgical eyeball length of≥20.5 mm, consistent with the criteria employed by Johnson and Gass.

Uyamaclassified 19 eyes from 16 patients with IUES into three groups: type 1 included nanophthalmic eyes with an axial eyeball length shorter than 19.0 mm; type 2 included nonnanophthalmic eyes with a rigid and thick sclera and an axial length averaging 21.0 mm; type 3 included non-nanophthalmic eyes with a normal size and scleral thickness and an axial eyeball length ranging from 25.0 to 22.9 mm. They concluded that sclerectomy was effective in types 1 and 2 only; in type 3 eyes, this treatment modality was ineffective. However, in the type 3 group, only two eyes from two patients were treated.Johnson and Gassinvestigated 23 eyes of 20 patients with IUES who underwent quadrantic partial-thickness sclerectomy with an axial length of ≥20.5 mm (average 23.1 mm),and found that subretinal and/or supraciliochoroidal fluid resolution occurred within 6mo in 19 eyes (83%) after one procedure and in 22 eyes (96%) after one or two procedures.

All patients underwent four-quadrant lamellar sclerectomy combined with partial full-thickness sclerostomy. The extent of each quadrant slice was 5×7 mm, about half to twothirds of the scleral thickness. A 1×2 mmsclerostomy was made in the center of each sclerectomy site. All the excised scleral pieces underwent pathological examination. The pieces obtained from one patient underwent electron microscopy.

顆粒復合肥按國標生產，一般只含氮磷鉀，養(yǎng)分不平衡。在生產方面，液體肥同樣具有優(yōu)勢，液體復混肥生產過程無污染，無排放，更加環(huán)保和節(jié)能；顆粒復合肥生產過程是能耗過程，存在污染和排放。

總之，加強企業(yè)青年人才隊伍建設，更好地發(fā)揮青年人才在企業(yè)生產建設中的主力軍作用，是提升企業(yè)基層工作水平的關鍵所在。應把培養(yǎng)造就青年人才作為企業(yè)人才隊伍建設的一項重要戰(zhàn)略任務，開展青年人才職業(yè)生涯規(guī)劃，開展青年人才責任教育，加強青年人才管理制度建設，加大青年人才選任力度，采取及早選苗、重點扶持、跟蹤培養(yǎng)等特殊措施，使大批青年人才持續(xù)不斷涌現(xiàn)出來。為不斷提升基層工作水平，推動企業(yè)和諧、健康發(fā)展，實現(xiàn)“打造世界一流，實現(xiàn)率先發(fā)展”目標，提供堅實的人才保障。

In some patients, the degree of UES may show spontaneous improvement; however, the disease course is long and may result in serious visual acuity damage. In previous reports,including 20 patients with a natural disease course and a follow-up period of up to 8y, 9 patients showed spontaneous choroidal reattachment within 4mo to 6y, while the remaining 11 did not show improvements at the end of the observation period. Therefore, timely surgery is required for IUES patients with retinal detachment.

Inconsistent with the findings of previous research, our study found that the sclera of IUES patients with an axis greater than 20.5 mm is still thickened, and the blade needs to be replaced repeatedly during surgery. Ultrasound examination could not accurately measure scleral thickness. While we attempted to measure the thickness of the sclera near the scleral process by UBM, we did not measure the normal scleral thickness using the same method. We found that the mean thickness of the sclera near the scleral process was 0.76 mm by UBM. A previous study showed that UBM may be a more accurate and precise method for the measurement of scleral thickness than magnetic resonance imaging; the study found mean thicknesses values of 0.65±0.08 and 0.55±0.05 mm for eyes with UES and control eyes, respectively, by UBM.

Other clinical features observed in our patients were: a predominance of young and middle-aged patients; male predominance; chronic disease course; asymptomatic initial stage; normal intraocular pressure, slightly higher intraocular pressure in some cases; and absence of inflammatory reaction in the anterior segment and vitreous cavity, consistent with previous studies.

根據(jù)萊考夫的闡述，算術是沿路線運動的隱喻與算術是量尺隱喻也同樣有著密切的關系。他指出，我們運動的路線形成了一條物理線段—一一條沿著我們的軌跡的想像的線。運動的路線和物理線段之間有著簡單的關系。運動的起點與物理線段的一端相一致，運動的終點與物理線段的另一端相一致，運動的路線與物理線段的剩余部分相一致。正是因為有了路線與物理線段的這種一致性，所以有了與量尺隱喻相關的“算術是沿路線運動”的隱喻。[2]71-72

This study has some limitations. First, there was no control group. Some patients with exudative retinal detachment experience spontaneous recovery, but this may take a long time. Previous studies have shown that prolonged retinal detachment durations lead to gradual vision impairments.Therefore, surgery is required in cases with exudative retinal detachment, especially when the macula is involved. Second,most of the patients lived far away from the hospital and were spread across the country; therefore, data on the patients' final vision values could not be obtained because patients tended not to attend the follow-up if retinal reattachment was achieved.Our study did not statistically detect the relationship between patients' visual acuity and preoperative visual acuity as well as the preoperative lesion time. Previous studies have shown that there is no statistical correlation between postoperative visual acuity and preoperative lesion time.

In conclusion, we found that four-quadrant scleral lamellar sclerectomy combined with partial full-thickness sclerostomy was safe and effective for patients with IUES. In patients with relapse, reoperation is still safe and effective. Among IUES patients, the axial axis is normal, but the thickness and composition of the sclera are abnormal. Further research is needed to determine the causes of scleral thickening and component changes; genetics or metabolism may play a role in the disease course.

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