超声乳化联合IOL植入治疗前圆锥晶状体伴有Alport综合征的视觉效果

目的:观察超声乳化联合IOL植入治疗7眼前圆锥晶状体伴有Alport综合征患者的手术效果。方法:Alport综合征患者4例,7眼因前圆锥晶状体行超声乳化联合IOL植入手术。结果:所有患者的术后视力均有明显提高。结论:我们认为对于前圆锥晶状体伴有Alport综合征患者采用超声乳化联合IOL植入手术治疗,是一种安全的疗法。     

Refractive errors in children and young adults with Down’s syndrome

Purpose: Down’s syndrome (DS) is the most common chromosomal anomaly. Numerous ophthalmic features have been reported. The aim of our study was to investigate the incidence of refractive errors in children and young adults with DS in Macedonia. Methods: Fifty‐six children and young adults with DS, aged 2–28 years, from Macedonia, underwent slit‐lamp examination, ocular motility and refraction. Results: The overall incidence of refractive errors in the Macedonian children and young adults with DS was 96.4%. A total of 17.8% of the subjects had myopia, 23.2% had hypermetropia and 55.3% had astigmatism. Strabismus was seen in 13 (23.2%) of the subjects (nine had esotropia, three had exotropia, one had hypertropia). Conclusions: The incidence of refractive errors in Macedonian children and young adults with DS was similar to that in Asian children. Compared with White (Caucasian) and Asian children with DS, Macedonian children and young adults exhibited lower incidences of hypermetropia and myopia, and a higher incidence of astigmatism, in which oblique astigmatism represented the predominant type.     

Terson’s syndrome as a prognostic factor for mortality of spontaneous subarachnoid haemorrhage

Purpose: To evaluate the prognosis of mortality in patients with spontaneous subarachnoid haemorrhage associated with Terson’s syndrome. Methods: A prospective, consecutive case series study was conducted in patients admitted to the emergency room with a diagnosis of acute subarachnoid haemorrhage. After a complete neurological examination, funduscopic examination using binocular indirect ophthalmoscopy under mydriasis was performed upon admission and at days 3, 7, 30 and 60 after the onset. In all cases, the diagnosis of intracranial bleeding was made by computerized tomography, and the clinical condition was graded according to the Hunt & Hess and Glasgow coma scales. Results: Forty‐seven patients with the diagnosis of subarachnoid haemorrhage were enrolled. Forty‐four cases were associated with a ruptured aneurysm and three cases with arterio‐venous malformation. Fourteen patients (29%) were diagnosed with Terson’s syndrome. Seven patients (50%) with Terson’s syndrome died, whereas death occurred in three patients (9%) without Terson’s syndrome (p = 0.002). Ocular findings in Terson’s syndrome were preretinal, intraretinal, sub‐retinal and vitreous haemorrhage. Associated ocular findings included third‐nerve palsy, papilloedema and subconjunctival haemorrhage. Conclusion: The presence of Terson’s syndrome was associated with an increased mortality rate (50% versus 9%; p < 0.01). Therefore, patients with the diagnosis of intracranial haemorrhage should be submitted to a funduscopic examination, because the presence of intraocular haemorrhage is an important life‐threatening prognostic factor.     

前囊膜抛光预防前囊膜皱缩综合征

目的探讨白内障超声乳化人工晶体植入术中前囊抛光对预防术后前囊膜皱缩综合征的临床意义。方法采用临床对照试验方法，比较白内障超声乳化人工晶体植入术中行前囊膜抛光及未行前囊膜抛光两组病例并发前囊膜皱缩综合征的发生率。结果前囊膜抛光组246例（246眼），发生前囊膜皱缩综合征2眼（发生率0．81％）；前囊膜未抛光组192例（192眼），发生前囊膜皱缩综合征8眼（发生率4．17％）。两组前囊膜皱缩综合征发生率比较差异有显著性（x^2=5．436，P〈0．05）。前囊膜抛光组前囊膜皱缩综合征发生率显著低于未行前囊膜抛光组。结论白内障超声乳化联合人工晶体植人术中行前囊膜抛光是预防前囊膜皱缩综合征的一种行之有效的方法。     

Alport综合征眼部临床表现分析

目的:总结Alport综合征的临床表现,尤其是眼部特征。方法:回顾性分析32例被确诊为Alport综合征患者的内科、耳鼻喉科和眼科检查结果。结果:患者30例(93.7%)有疾病家族史。所有患者均有不同程度的肾脏病变:18例(56.3%)有肾功能衰竭,4例(12.5%)肾功能不全,10例(31.3%)血尿。患者20例(62.5%)有感音神经性耳聋。患者13例(40.6%)有眼部异常表现,其中5例(15.6%)为典型性改变:前圆锥晶体3例,黄斑周围斑点2例。结论:眼部异常不是Alport综合征诊断的必需条件,但因其典型的眼科表现应当引起眼科医师的注意,以便早期诊断治疗。     

Electron microscopic study of anterior lens capsule allotransplants in chronic corneal ulcers

PURPOSE: This study provides ultrastructural morphology and quantitative analysis of allotransplants of anterior lens capsule in the treatment of recurrent corneal ulcers. METHODS: Mechanical ulcers of uniform size were created with a 6-mm corneal trephine in 9 eyes at one-third corneal depth in 6 white New Zealand male rabbits. Following initial epithelial regrowth, an identical injury was created in the same area of each cornea a second time. In 6 eyes (treated group), an anterior lens capsule from a healthy donor rabbit was sutured into the ulcer bed, followed by antibiotic/steroid drops 3 times daily for 1 week. The remaining 3 control eyes were allowed to heal without surgical intervention using the same antibiotic/steroid drops only. Slit-lamp examination, histopathology, and electron microscopic findings with hemidesmosome counts and size were recorded over a 3-month follow-up period. RESULTS: The control injured group had discontinuous epithelial basement membrane with significant reduction in hemidesmosome count compared with noninjured controls (P<0.0001). The treated subgroup 7 days after surgery showed linear basement membrane with identifiable lamina lucida and densa and newly-formed hemidesmosomes, which were significantly more numerous than in the injured but nontreated group (P<0.0001). Numerous hemidesmosomes and firm adhesion of epithelium to underlying stroma were seen in this group with lens capsule allografts 3 months after surgery. The number and size of hemidesmosomes did not significantly differ between groups 7 days and 3 months after surgery. CONCLUSIONS: On the ultrastructural level, anterior lens capsule allotransplants in mechanically induced corneal ulcer heal with continuously formed epithelial basement membrane and numerous hemidesmosomes. This enables significant reformation of hemidesmosomes that are identical in size to hemidesmosomes in healthy corneas and helps forming a stronger apposition for epithelial cells to underlying structures in eyes where epithelial basement membrane has been destroyed from chronic ulceration.     

Ocular manifestations of Alport syndrome

AIM： To analyze the clinical manifestation of Alport syndrome, especially the ocular features. METHODS: The physical, ophthalmologic and audiologic examination results of thirty-two patients with Alport syndrome were analyzed retrospectively. RESULTS: Thirty (93.7%) patients had some family history. All patients had renal disease: eighteen (56.3%) patients with chronic renal failure, four (12.5%) patients with renal insufficiency, and the other ten (31.3%) patients with hematuria. Twenty (62.5%) patients had sensorineural deafness. Thirteen (40.6%) patients had ocular deformity, five (15.7%) patients had typical ocular changes: three patients with anterior lenticonus, and two patients with macular flecks. CONCLUSION: Ocular anomalies are not requisite for the diagnosis of Alport syndrome. But its typical ocular features should be recognized by the ophthalmologists which supports the diagnosis.     

Lattice corneal dystrophy,gelsolin type (Meretoja’s syndrome)

Purpose: This paper reviews current knowledge about the pathogenesis, clinical manifestations and treatment of lattice corneal dystrophy, gelsolin type (LCD2, Meretoja’s syndrome). Methods: Material is derived from literature searches, a case study of a Finnish patient living in Sweden, and interviews in Helsinki with Professor Ahti Tarkkanen and Dr Sari Kiuru‐Enari, both of whom have extensive first‐hand experience in treating patients with the disease. Results: The disease is now reported from several countries in Europe, as well as Japan, the USA and Iran. Treatment is symptomatic and is based on eye lubrication combined with rigorous monitoring of intraocular pressure to reduce corneal haze and postpone the need for keratoplasty. When systemic symptoms occur, the ophthalmologist should consult other specialists. Conclusions: The disease is probably under‐reported and is almost certainly to be found in more countries, including Sweden. Every ophthalmologist should be vigilant and consider this diagnosis when discovering a corneal lattice dystrophy, especially because the disease is an inherited, lifelong chronic condition with systemic symptoms.     

Electron microscopic investigation of anterior lens capsule in an individual with true exfoliation

AIM: To determine the changes which occur in the anterior capsule in true exfoliation which is a very rare condition.METHODS: The anterior capsule from a 93 year-old patient and 6 other patients with age-related cataract during capsulorhexis was examined via transmission electron microscopy (TEM).RESULTS: TEM revealed apoptosis of lens epithelial cells in both two groups. Moreover, we observed lamellar delamination, granular belts in the anterior capsular zone and loss of the subcapsular epithelium cells in the posterior capsular zone, as well as abnormal fibrils located in the central capsular layer only in the sample from the patient with true exfoliation.CONCLUSION:We suggest that loss of lens epithelial cells and appearance of abnormal fibrils is important in disease developing, and our study supported age-related degeneration is one of causative factors in true exfoliation.     

Posterior uveitis in Hodgkin’s disease

Purpose: To describe the features of posterior uveitis in patients who either developed or were known to have, biopsy-proven Hodgkin’s disease (HD). Methods: Four patients were identified who were attending the uveitis clinic. Their charts were reviewed retrospectively and information on their HD was obtained from other hospitals where necessary. Their case histories are presented. Results: Two patients presented with uveitis prior to the diagnosis of HD and in the other two, the HD was thought to be in remission. The commonest ocular signs were of vitritis and discrete, white, chorioretinal lesions. No difference in the ocular findings were apparent between those who had a known diagnosis of HD and the those that did not. Conclusions: HD can occur in the eye and can mimic posterior uveitis. In two of the patients, the uveitis preceded the diagnosis of HD and at the time of presentation all investigations were normal. No specific diagnostic features were apparent, though three of the patients had vitritis and chorioretinal lesions.     

Sclerosing Wegener’s granulomatosis in the orbit

Purpose: To report three cases of sclerosing Wegener’s granulomatosis in the orbit and to compare the histopathological morphology with those of other types of sclerosing orbital inflammation. Methods: We analysed the clinical data along with histopathological specimens from orbital biopsies and from two enucleated eyes. Results: All three patients had longstanding inflammation in the orbit and involvement of the paranasal sinuses. Proptosis, impaired ocular motility and reduced visual acuity dominated the clinical picture. All histopathological specimens featured granulomatous inflammation, tissue necrosis, vasculitis and widespread dense fibrosis. In certain areas of all specimens the fibrous tissue was arranged in characteristic concentric, onionskin‐like whorls of collagen around obliterated small blood vessels. Conclusions: Wegener’s granulomatosis may lead to orbital fibrosis and sclerosis. The stromal changes and cellular infiltrate resemble the non‐specific orbital inflammation previously termed orbital pseudotumour. We present the first ‘onionskin’ lesions in association with Wegener’s granulomatosis in the orbit. This latter morphology may represent an abnormal fibrotic reaction in inflammatory tissue.     

Bimanual anterior segment revision surgery for anterior capsule contraction syndrome associated with anterior flexion of intraocular lens haptics

Purpose

To report the incidence of anterior capsule contraction syndrome (ACCS) and to present a novel minimally invasive bimanual technique for anterior segment revision surgery associated with ACCS with anterior flexion of the intraocular lens haptics.

Methods

A consecutive cohort of 268 eyes of 161 patients undergoing phacoemulsification and implantation of the same type of hydrophilic acrylic aspheric intraocular lens cohort were analysed and a novel technique of minimally invasive bimanual technique for anterior segment revision surgery is described.

Results

We identified four eyes (1.5%) of three patients with advanced ACCS. Successful restoration of a clear visual axis with minimal induction of astigmatism and rapid visual rehabilitation was achieved in all four cases.

Conclusion

This technique is a safe and minimally invasive alternative to laser or vitrector-cut capsulotomy to restore a clear visual axis. In cases of advanced ACCS, it offers the option for haptic reposition or amputation.     

A new autosomal dominant Peters’ anomaly phenotype expanding the anterior segment dysgenesis spectrum

Purpose: To test the association of genes involved in anterior segment development in a family with autosomal dominantly inherited Peters’ anomaly (PA) with a unique ocular phenotype. Methods: Six members of a five‐generation family with PA were extensively phenotyped and linkage analysis of candidate genes, namely, PAX6, PITX2, FOXC1, CYP1B1 and MAF, was performed. Results: The complete pedigree consisted of 38 members, 19 of whom were affected. The six probands examined had bilateral microcornea, corneal opacity, iridocorneal adhesions, nystagmus and strabismus, but cataract, keratolenticular adhesions, glaucoma and posterior embryotoxon were absent. PAX6 gene mutations had been previously excluded in one of the affected members. DNA markers for candidate genes CYP1B1 on 2p22, PITX2 on 4q25, PAX6 on 11p13, MAF on 16q23 and FOXC1 on 6p25 were genotyped. Highly negative lod scores were obtained for all markers. Conclusions: The exclusion of these genes as likely candidates supports the hypothesis that the ocular phenotype associated with PA segregating in this family is a distinct, new, autosomal dominant entity in the anterior segment dysgenesis spectrum.     

Anterior capsule contraction and intraocular lens dislocation in eyes with pseudoexfoliation syndrome

AIMS—To examine the extent of anterior capsule contraction as well as intraocular lens (IOL) decentration and tilt following implant surgery in eyes with pseudoexfoliation syndrome (PE).
METHODS—53 eyes from 53 patients with PE and 53 control eyes from 53 age matched patients, undergoing phacoemulsification and implant surgery, were recruited. The anterior capsule opening area and the amounts of IOL decentration and tilt after undergoing continuous curvilinear capsulorhexis were measured using the Scheimpflug videophotography system at 1 week and 1, 3, 6, 9, and 12 months postoperatively.
RESULTS—The mean area of the anterior capsule opening in the PE group was significantly smaller than that in the control group at 1 month postoperatively and later. The percentage reductions in the PE group were approximately 25%, while they were less than 10% in the control group. The degree of IOL tilt was also larger in the PE group than in the control group. Five eyes (9.4%) in the PE group underwent a neodymium:YAG laser anterior capsulotomy, but none in the control group underwent a capsulotomy.
CONCLUSIONS—The contraction of the anterior capsule opening was more extensive in the PE eyes than in the control eyes, thus resulting in a high Nd:YAG laser anterior capsulotomy rate. The IOL tilt was also greater in the PE eyes than in the control eyes.

Keywords: anterior capsule contraction; intraocular lens dislocation; pseudoexfoliation syndrome; continuous curvilinear capsulorhexis     

青年尸眼前囊膜弹性的研究

目的 研究青年人晶状体前囊膜弹性,以例提高白内障摘出和人工晶状体植入术的成功率。方法 测量53人共90只尸眼（平均年龄26.8a)晶状体前囊膜连续环形撕开口的弹性。结果 前囊膜撕开口弹性为1.38-2.74,前囊膜撕开口直径越大,弹性越大;前嗝 膜弹性与年龄无关。结论 本年龄段内（19-40a)前囊膜弹性与撕开口直径呈直线正相关,青壮年期白内障术中前囊膜撕开口直径5.0-5.5mm为宜。     

玻璃体切除术中切除晶状体保留前囊膜同时植入人工晶状体的疗效观察

目的:探讨玻璃体切除联合晶状体切除保留前囊膜同时植入人工晶状体的临床疗效。方法:玻璃体视网膜病变同时伴有晶状体混浊46例46眼,术中采用经睫状体平坦部切口切除晶状体,保留前囊膜,并行玻璃体切除,Ⅰ期植入人工晶状体。结果:所有患者视力均有不同程度提高,部分患者复查时前囊膜出现不同程度混浊,行YAG激光切开。结论:玻璃体切除联合晶状体切除保留前囊膜Ⅰ期植入人工晶状体,保留了眼内正常解剖结构,减少了术中及术后并发症,视力恢复良好。     

Rupture of the anterior lens capsule in Alport syndrome

Alport syndrome is an inherited disorder of type IV collagen, a major constituent of basement membranes. Eighty-five percent of cases are transmitted through X-linked dominant inheritance, although autosomal dominant and autosomal recessive inheritance has also been reported. Clinical manifestations of Alport syndrome include progressive glomerulopathy, sensorineural deafness, anterior lenticonus, posterior corneal dystrophy, and abnormal retinal pigmentation. Anterior lenticonus may lead to loss of vision because of progressive myopia or cataract formation. We report 2 cases of unusual cataract formation in adolescent boys who had a rupture of the anterior lens capsule. One rupture was spontaneous, and the other was traumatic.     

Ultrastructure of anterior lens capsule of intumescent white cataract

Purpose: To analyse the anterior lens capsule thickness and ultrastructure changes of intumescent white cataracts in comparison with nuclear cataracts to prove possible structural reasons for surgical difficulties with the intumescent white cataract. Methods: Anterior lens capsules from 35 eyes with intumescent white and 35 eyes with nuclear cataracts were analysed for their thickness by semithin sections technique and for morphological characteristics by transmission electron microscopy. Results: Capsule thickness was not significantly different in intumescent white compared to nuclear cataracts (mean values 17.5 and 18.5 μm, respectively, p = 0.369). The main morphological features of capsules were extrusions of capsule at the basement membrane‐epithelial border embedding cellular material which were significantly more frequent in intumescent cataracts. Filaments in the basement membrane as well as rarefication of its structure and lamellae were often concomitant with the extrusions. Conclusions: Anterior capsules of white intumescent cataracts do not differ in thickness but have different ultrastructure morphology compared to nuclear cataracts. The extrusions of basement membrane at the basement membrane‐epithelial border towards epithelium, the filamentary inclusions within basement membrane and its rarefication could be the structural causes of tensile weakness and hence additional reason to surgical problems.