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Objective: To establish 3-D visible human model of the rectus EOM Pulley., Methods: The pictures of Masson stained adjacent coronal sections of a whole orbit were imaged by digital camera, which were entered into computer continuously after removing spottiness through PhotoShop 7.0 software. 3D-Doctor software was used to register and split the image. Finally three-dimensional computerized reconstructions of the rectus EOM pulley and eye ball were achieved. At the same time pulley system structure was analyzed using the 3D-Doctor software., Results: Three-dimensional model of the rectus EOM pulley displayed directly the three-dimensional relationship of pulley and the eye ball, extraocular muscles and orbit. This three-dimensional model could be rotated randomly, providing convenience for multi-angle observation. The demonstration of the three-dimensional model selectively provides a approach to get rid of disturbance and lock the aim for observation. The length of pulley was about 4.6 to 5.8 mm. The origin of the four rectus pulleys was 13.8 to 18.0 mm from the corneoscleral limbus. Media rectus pulley was thickest among four rectus pulley. The ratio between media rectus pulley thickness and corresponding media rectus thickness could reach 1.11., Conclusions: Three-dimensional model of the rectus EOM pulley provided direct picture for clinic doctors to understand the structure of pulley, which also provided three-dimensional anatomic basis for iconography research of the rectus EOM pulley. The data of pulley could be helpful to establish Chinese rectus EOM pulleys data bank.

Objective: To analyze the clinical manifestations of affected individuals in a family of congenital fibrosis of the extraocular muscles (CFEOM) with juvenile canities., Methods: All affected and unaffected individuals were retrospectively analyzed in this study. The clinical features include genetic aspects, sex, age, ptosis, restriction of eye movement, aberrant innervation and surgical procedures, were evaluated., Results: This pedigree was inherited as autosomal dominant. There were 14 cases suffering from congenital fibrosis of extraocular muscles in four generations. They had congenital blepharoptosis, head-tilt, chin lift and primary gaze fixed in a hypotrophic position. But vertical and horizontal positions of the eye and restriction of eye movement were different among affected individuals. Some of them also had pupillary abnormally, aberrant innervation and juvenile canities. Inferior rectus recession improved hypotropia in patients with infraducted eyes and chin elevation. Horizontal muscle recession corrected horizontal strabismus satisfactorily in most cases. Ptosis was repaired by frontalis sling or levator resection., Conclusions: This is the first report of CFEOM associated with juvenile canities. There was phenotypic heterogeneity in this CFEOM pedigree. So the phenotype alone is not sufficient to distinguish among the 3 genotypically distinct CFEOM syndromes. The combination of clinical characteristics and genetic analysis are the basis for the establishment of diagnosis.

Congenital cranial dysinnervation disorders (CCDDs) are congenital non-progressive, sporadic or familial abnormalities of cranial musculature that result from developmental abnormalities of, or the complete absence of, one or more cranial nerves with primary or secondary muscle dysinnervation. These disorders include vertical disorders of ocular motility, horizontal disorders of ocular motility and disorders with abnormalities of facial motility that were previously referred to as "congenital fibrosis syndromes". The advances in clinical and molecular genetics of congenital cranial dysinnervation disorders are reviewed.

Objective: To clone and eukaryotic express wild type and truncated mouse ciliary neurotrophic factor (CNTF) gene, and to observe the biological effect of two types of CNTF gene expressing in ARPE-19 cells., Methods: RT-PCR was used to amplify the cDNA of CNTF gene, and truncated CNTF cDNA was obtained by site-directed mutagenesis. The two types of CNTF gene were cloned into plasmid pTracer-CMV and transfected to ARPE-19 cells. Dot blotting was used to detect the expression of CNTF. MTT and flow cytometry apoptosis assay were performed to observe the biological effect of CNTF expressing in ARPE-19 cells., Results: Wild type and truncated CNTF gene were amplified by RT-PCR, and their eukaryotic expression plasmids were successfully constructed. After ARPE-19 cells transfected with two types of recombinant plasmids, the CNTF were detected in the supernatant of cells culture. MTT result shows that two types of CNTF have no proliferation promoting effect to ARPE-19 cells, and quantitive apoptosis assay implicated that CNTF could partially suppress the apoptosis that induced by the cells culturing with serum free culture., Conclusion: Expression of two types of CNTF in ARPE-19 cells gets prepared for gene therapy research of retinitis pigmentosa.

Objective: To elucidate the difference in the pathogenesis between strabismic amblyopia and anisometropic amblyopia., Methods: Using an EPI gradient echo sequence in 1.5 T MRI and visual electrophysiological system, the pattern visual evoked potentials and calcarine activation by monocular viewing of check-board pattern with reversal were examined in 18 strabismic amblyopes and 15 anisometropic amblyopes. The calcarine activation in terms of binocular voxels index and dependence on the spatial frequency of the stimuli were compared. The correlation between PVEP P(100) latency and activation intensity was also analyzed., Results: The binocular voxels index was lower in the strabismic amblyope than that in the anisometropic amblyope. The calcarine activation from amblyopic eyes in strabismic amblyopes was more suppressed at higher spatial frequencies (1, 2 c/d); while that from amblyopic eyes in anisometropic amblyopes was more suppressed at all spatial frequencies (0.25, 0.5, 1 and 2 c/d). Inverse correlations existed between PVEP P(100) latency and activation intensity in two types amblyopia (P < 0.05)., Conclusions: The binocular interaction is not equal in these two different types of amblyopia. Meanwhile, the amblyopia-related deficits in the low and high-spatial-frequency pathway of visual system are not similar between these two different types of amblyopia.

Objective: To investigate a high molecular weight fluorescein angiography in the application of retinal neovascularization model in mouse., Methods: retinal neovascularization model was induced by exposure mouse to an environment containing high concentration of oxygen. High molecular weight fluorescein isothiocyanate dextran were perfused through the left ventricle directly, then the mouse eyes were enucleated and fixed with 4% paraformaldehyde. The retina was separated from the eyecup and flat mounting was performed on a gelatin coated slide. The vasculature was examined under fluorescent microscope., Result: The whole retinal vasculature was clearly visualized under fluorescent microscope. By focusing on different layer of the tissue, superficial, deep vascular plexus and connecting vessels also could be distinguished. The neovascular response occurred at the junction between the vascular and the avascular retina., Conclusion: High molecular weight fluorescein angiography can be applied for retinal neovascularization evaluation.

Objective: To study the structure and three-dimensional distribution of extraocular muscle Pulley in orbit., Methods: 5 fresh cadaver orbits were fixed in situ and serially sectioned at 4 microm thickness. Every nearby sections were stained using Masson's trichrome, Van Gieson'stain and immunohistochemical staining, the stained tissue was examined by gross anatomy and light microscopy., Results: Near the equator of the globe, extraocular muscle Pulleys were at the points where rectus penetrate posterior Tenon's fascia, encircling the rectus orbit side by fiber ring structure. There was Pulley Band among extraocular muscle Pulleys. The main composition of the Pulley and Pulley Band were collagen (I and III type), elastic and smooth muscle., Conclusion: Constitution of Pulley provides dissection basis as the functional origins of the extraocular muscle.

Objective: To analyze the clinical manifestations and the methods and effects of surgical management on Duane's Retraction Syndrome (DRS)., Methods: 67 cases with DRS were summarized retrospectively. The data were recorded and analysed, including sex, age, type, clinical features, surgical methods and the results., Results: 63 cases had only monocular involvement. The number of Type I DRS was 47 cases (35 cases with esotropia, 12 cases with exotropia). 8 cases were Type II DRS with exotropia and 12 cases were Type III DRS (11 of them with exotropia). In 37 cases with upshoot and/or downshoot phenomenon, 31 of them were associated with exotropia. Ipsilateral medial rectus or lateral rectus recession, or bilateral medial rectus recession, improved the head compensation position, narrow palpebral fissure and globe retraction, with improvement or elimination of the deviation. After the operation, 60 cases (90%) showed horizontal tropia less than 10 prism diopters. Upshoot and/or downshoot were improved in all of the 37 cases after recessing medial or/and lateral recti, or simultaneously weakening of the inferior oblique muscle., Conclusion: Weakening medial and/or lateral recti can improve the clinical manifestations of DRS. Upshoot and downshoot are more common in eyes with exotropia. Lateral rectus recession is the main treatment method for relieving upshoot and downshoot in DRS with exotropia. We infer that the upshoot and downshoot phenomenon mainly related to the "bridle" effect. Recession of both horizontal recti can improve the serious clinical manifestations.

In the last 5 years, we witnessed the progress in the basic and clinical research of strabismus and amblyopia in China, which should be attributed to the effective leadership of the Chinese Ophthalmological Society and the efforts of all members of the professional team. We are glad to see the work of our researchers and clinical scientists in the studies on the molecular and neuronal mechanisms of amblyopia, visual developmental plasticity and functional brain imaging of amblyopia, in the studies on the extraocular muscle's pulley and in the studies on the diagnoses and therapies of strabismus and amblyopia, and furthermore, in epidemiological studies of strabismus and amblyopia. We expect more fruits and creative findings in this field in China in the next decade.

Objective: To investigate the stereoacuity before and after superior oblique sagittal transposition in patients with acquired excyclotorsion., Methods: 14 cases of acquired excyclotorsion who had complaints of forward and downward torsional diplopia were treated with superior oblique sagittal transposition. All patients were examined with Titmus test prior and after surgery., Results: Surgical treatment greatly improved torsional diplopia. The stereoacuity improved gradually from 800 to 60 seconds of arc, peripheral to macular stereoacuity, and even foveal stereoacuity., Conclusion: The superior oblique sagittal transposition is an effective method for primary and down gaze acquired excyclotorsion. The operation not only alleviated diplopia in the practical fixation field, but also improved stereoacuity.

Objective: To describe the clinical phenotype in a Chinese family with congenital fibrosis of extraocular muscles and to identify the location of candidate gene of the disease in chromosome., Methods: The clinical feature of all affected members in this family were examined. A genome-wide linkage screening was conducted. Direct genomic sequencing was used to evaluate the candidate gene KIf21A., Results: Four affected members in the pedigree were born with classic phenotype of CFEOM. By linkage analysis the disease gene was mapped to chromosomal region 12p11.2-q12 defined by microsatellite markers D12S1648 and D12S1668. The maximum Lod Score was 2.12 (D12S1090). Direct sequence showed no mutation in all exons and exon-intron boundaries of the candidate gene KIF21A, a polymorphism substitution occurred in the exon 21., Conclusions: The disorder in this family should be referred as CFEOM1 which was inherited as an autosomal dominant trait. The candidate gene was linked to CFEOM1 locus on chromosome 12p11.2-q12, between marker D12S1648 and D12S1668. It's more likely that KIf21A is not the disease causing gene in this family.

Objective: To assess the effects of 20% ethanol used in LASEK on corneal wound healing., Methods: Forty-eight eyes from 24 rabbits were deepithelialized by two techniques. The epithelium were detached with either 20% ethanol (applied for 30 seconds) or mechanical scraping, then ablated was performed. The number of superficial stromal keratocytes was counted and the morphologic changes were observed. Expression of TGF-beta1 and bFGF mRNAs was detected and analyzed by reverse transcription-polymerase chain reaction and immunocytochemical methods at 1, 7, 30 and 90 days after the surgery., Results: One day after the surgery, the expression of TGF-beta1 and bFGF in the keratocytes in both treated groups was lower than that of the normal controls. Seven days after the surgery, the expression of TGF-beta1 and bFGF in both treated groups was greater than that of the normal controls (P < 0.01). The expression in the alcohol-treated group was greater than that of the surgical-treated group (P < 0.01). The expression of TGF-beta1 and bFGF reached the peak 30 days after the surgery, no significant difference was detected between the alcohol-treated and surgical-treated groups. There was no significant difference in expression level between the alcohol-treated and surgical-treated groups 3 months after the surgery; as well as between treated groups and normal group. The amount of TGF-beta1 and bFGF mRNA was positively correlated with the number of keratocytes. The correlation coefficient between TGF-beta1 and bFGF mRNA and the number of the keratocytes was 0.744 (P < 0.01) and 0.738 (P < 0.01) in the alcohol-treated group; and was 0.664 (P < 0.01) and 0.785 (P < 0.01) in the surgical-treated group, respectively., Conclusions: The expression of TGF-beta1 and bFGF mRNA undergo a dynamic process of "decrease-increase-normal". Although ethanol has a slight toxic effect on rabbit epithelial cells, but the effects do not persist over time, therefore, it is relatively safe to use the alcohol treatment in the LASEK.

Objective: To identify mutations in a four-generation Chinese family with retinitis pigmentosa and to investigate its clinical phenotype., Methods: Ophthalmic and electrophysiological examinations of patients with RP were performed. A genome-wide scan and linkage analysis were conducted in this family. Direct genomic sequencing was used to evaluate the candidate gene. A restriction assay was used to confirm the mutation status in this family, and to exclude it as a polymorphism in a reference population., Results: The mutation gene was mapped close to RP11 in chromosomal region 19q13.4 by linkage analysis. A novel single heterozygous base substitution (G > C) was detected at the beginning of intron 8 in the PRPF31 gene whereby the consensus GT dinucleotide of the intron 8 splice donor site was changed to CT. The mutation was co-segregated completely with the disease phenotype, but was absent in the unaffected relatives and in 100 reference subjects, thus supporting its pathogenic nature in this family. The clinical findings of RP patients were consistent with a relatively severe and diffuse type of RP., Conclusion: A novel splice site mutation (IVS8 + 1G > C) in the PRPF31 gene caused retinitis pigmentosa in the four-generation Chinese RP family studied.

Objective: To evaluate the surgical technique of pituitary adenomas by naso-sphenoid way and the prevention of complication., Method: The pituitary adenomas in 19 cases were removed under endoscope and microscope by the transnasosphenoided way. Sphenoid-saddle base was restructed with lip-tendon-membrane and the nasal separate bone., Result: Seventeen cases had entire resection in the 19 patients. Two cases had subtotal resection. The symptoms were improved with no sever complications in 19 cases during the period of 3-36 months follow-up and no recovery in 17 cases., Conclusion: lt is a effective surgical technique that the pituitary neoplasms are removed by nasosphenoidal way under the endoscope and microscope with the alcohol for preventing the tumor recovery and with the lip-tendon-membrane and nasal separate bone for restructing the saddle base.

Objective: To investigate the characteristic changes of the retina, the visual pathway and the visual cortex in patients with anisometropic amblyopia by simultaneously recording multifocal VEP and multifocal ERG., Methods: Pattern and flash simultaneously recording of the multifocal VEP and ERG of both eyes of 24 anisometropic amblyopes were recorded and the results were compared with those of 30 normal subjects., Results: In different retinal areas the amplitudes of multifocal pattern VEP and ERG and second order kernel of multifocal flash VEP and ERG of the amblyopic eyes were reduced. In amblyopic eyes the latencies of mVEP were markedly prolonged while no changes of the mERG latencies were found. Amplitudes of first order kernel of multifocal flash VEP and ERG in amblyopic eyes were reduced while no changes in latencies. The abnormalities of multifocal pattern VEP waveforms in amblyopia were larger in central area than in peripheral and were correlated with the damage of visual acuity of the amblyopic eyes. Retinocortical time of multifocal pattern and the second order kernel of multifocal flash responses were prolonged in amblyopic eyes, while no changes were found for the first order kernel responses., Conclusion: The mVEP and mERG results revealed characteristic suggesting a malfunction of the retina, the visual pathway and the visual cortex in amblyopic patients.

Objective: To investigate the changes of the multifocal electroretinogram (mfERG) in amblyopic eyes. Methods The mfERG of 28 anisometropic amblyopic cases (28 eyes), 25 strabismic amblyopic cases (25 eyes), 14 isometropic amblyopic cases (28 eyes) and 54 normal control eyes were tested., Results: The N1 wave and P1 wave amplitude densities of mfERG first order kernel and the P1 wave amplitude densities of second order kernel in amblyopic eyes were significantly attenuated in the central region of the visual field (P < 0.05), and these phenomena gradually reduced with the increase of the eccentricity. The N1 wave and P1 wave amplitude densities of mfERG first order kernel at the temporal visual field were smaller than that of the nasal visual field, and the latencies were delayed at the temporal visual field in all groups (P < 0.05). The P1 wave amplitude densities of mfERG second order kernel at the upper visual field were smaller than that of the lower visual field in all groups (P < 0.05), and no significant differences could be detected in other indexes between the upper visual field and the lower visual field (P > 0.05). There was no significant difference in latencies of mfERG between the amblyopic eyes and control eyes (P > 0.05). No significant difference of mfERG was found among the three types of amblyopic eyes. In the amblyopic eyes, there was no linear correlation between visual acuity eyes and various indexes of mfERG (P > 0.05). There was significant difference in mfERG between the amblyopic eyes and contralateral eyes (P < 0.05), but there was no significant difference between the contralateral eyes and the control eyes (P > 0.05)., Conclusions: The X ganglion cells in the retina of amblyopic eyes are abnormal, but the visual information transmitting time in the retina was normal in the amblyopic eyes.

Objective: To evaluate the significance of Orbit 1.8 Computer Biomechanics Simulation in the diagnosis and treatment of congenital superior oblique paresis., Methods: Fifteen patients with congenital superior oblique paresis were enrolled in the present study. Hess test and other strabismus tests were performed before and after the surgery. Parameters such as contractile force, elasticity, innervation of extraocular muscles and the secondary changes in other muscles were treated by the Orbit 1.8 to simulate the preoperative situation. A biomechanical strabismus model was created. Surgical manipulations were tested on the model to predict postoperative outcomes. After surgery, simulated and actual outcomes were compared., Results: The predicted results matched with the actual results quite well. Before the surgery, the mean difference between these two data was 1.66 degrees +/- 0.44 degrees in horizontal and 1.97 degrees +/- 0.51 degrees in vertical gaze. After surgery, mean difference in horizontal and vertical gaze of these two data was 1.21 degrees +/- 0.34 degrees and 1.59 degrees +/- 0.41 degrees., Conclusions: Orbit 1.8 Computer Biomechanics Simulation offers a biomechanical approach to understand all of the forces that combine to control eye movement and how these forces interact in normal eyes and in eyes with motility problems. It can be used to reflect the causes of strabismus, to design the surgical procedure, to verify the diagnosis and to predict the outcome of surgery. It is a helpful tool in the diagnosis and treatment of ocular motility disorders.

Objective: To investigate the results of using shifting fixation eye to manage monocular or asymmetrical binocular dissociated vertical deviation (DVD)., Methods: Twenty-three DVD children whose corrected vision was better than 0.5 in the amblyopic eye were studied. The goal of the shifting fixation eye was gained by decreasing the vision of the primary fixation eye to 2 or 3 lines (Snellen Visual Acuity) lower than that of the fellow fixation eye by occlusion or optical methods. The vision, alignment and retinal correspondence were evaluated before and after the treatment., Results: After the treatment, in 10 patients, the vision of the primary fixation eye did not decrease significantly and the vision of the fellow eye increased 2 lines. In 19 patients, a cosmetic result was achieved; the primary fixation eye was shifted in 4 patients that had similar hepertropia in two eyes (

Objective: To investigate the mutation spectrum of phosphodiesterase beta subunit (PDE6B) gene and incidence in Chinese retinitis pigmentosa (RP) patients., Methods: Genomic DNA was extracted from the blood samples of 38 patients from 35 autosomal recessive retinitis pigmentosa (ARRP) families and 55 sporadic cases. The mutation of the PDE6B gene was detected using PCR-SSCP, and the amplified PCR product of abnormal bands was sequenced., Results: Within intron 10 of PDE6B gene, a mutation was found in an ARRP family, a G --> A transition at 19th base upstream 5'-end of exon 11. A novel complex heterozygous variant of PDE6B gene in a sporadic case, a T to C transition in codon 323 resulting in the substitution of Gly by Ser and two bp(TG) inserted between the 27th-28th bp upstream of the 5'-end of exon 10 were both present in the same isolate RP. But they were not found in 100 unrelated normal individuals. A sporadic RP was found carrying a sequence variant of PDE6B gene, a G to C transversion in intron 18, the 15th base adjacent to the 3'end of exon 18. Another isolate RP was found to have 2 bp inserted between 31st and 32nd base upstream 5'end of exon 4 (in intron 3) of PDE6B gene., Conclusion: There is a complex heterozygous mutation of PDE6B gene responsible for a sporadic RP patient in China. Several DNA variants were found in intron of PDE6B gene in the national population.

Objective: To identify the mutation spectrum of phosphodiesterase beta subunit (PDE6B) gene, the incidence in Chinese patients with retinitis pigmentosa (RP) and their clinical phenotypic characteristics., Methods: Screening of mutations within PDE6B gene was performed using polymerase chain reaction-heteroduplex-single strand conformation polymorphism (PCR-SSCP) and DNA sequence in 35 autosomal recessive (AR) RP and 55 sporadic RP cases. The phenotypes of the patients with the gene mutation were examined and analyzed., Results: Novel complex heterozygous variants of PDE6B gene in a sporadic case, a T to C transversion in codon 323 resulting in the substitution of Gly by Ser and 2 base pairs (bp: G and T) insert between the 27th-28th bp upstream of the 5'-end of exon 10 were both present in a same isolate RP. But they are not found in 100 unrelated healthy individuals. Ocular findings showed diffuse pigmentary retinal degeneration in the midperipheral and peripheral fundi, optic atrophy and vessel attenuation. Multi-focal ERG indicated that the rod function was more severely deteriorated. A mutation was found in a case with RP in a ARRP family, a G to A transversion at 19th base upstream 5'-end of exon 11 (within intron 10) of PDE6B gene. A sporadic RP carried a sequence variant of PDE6B gene, a G to C transition, at the 15th base adjacent to the 3'-end of exon l8. In another isolate case with RP was found 2 bp (GT) insert between 31st and 32nd base upstream 5'-end of exon 4 (in intron 3) of PDE6B gene., Conclusions: There are novel complex heterozygous mutations of PDE6B gene responsible for a sporadic RP patient in China. This gene mutation associated with rod deterioration and RP. Several DNA variants were found in introns of PDE6B gene in national population.

Objective: To investigate the relationship between angiotensin-1 converting enzyme (ACE) genotype and the incidence of in-stent restenosis (ISR)., Methods: One hundred and twenty eight patients, native of northeast China and of Han nationality, underwent coronary stenting consecutively from 1997 to 2000 and were followed up for 3 months to 2 years. They were divided into two groups according to the degree of stenosis of arterial lumen diagnosed during the second coronary angiography: ISR group (n = 43, with the lumen of stenting area stenosed by more than half of the diameter of the original lumen) and non-ISR group (n = 85, with the stenosis less than half of the diameter of original lumen). DNA of peripheral white blood cells was collected and ACE genotype was analyzed by PCR method. The demographic data, medical history, and body mass index of the patients were recorded. The cholesterol, triglycerin, blood sugar, systolic pressure, and diastolic pressure were examined 24 hours before stenting., Results: More patients with type II diabetes were found in the ISR group than in the NISR group (P < 0.025). No difference in other clinical and biochemical indexes was found between these two groups (P > 0.05). The frequencies of DD, ID and II were 0.651, 0.163, and 0.186 respectively in ISR patients, and were 0.412, 0.282, and 0.305 respectively in NISR group. The restenosis rate was significantly higher in patients with ACD DD genotype than in patients with ID or II genotypes (P < 0.025). The frequency of D allele (73.26%) was significantly higher than that of I allele (26.74%) (P < 0.01)., Conclusion: ACE polymorphisms are associated with ISR. The D alleles might be a risk factor for ISR.

Objective: To explore the ways of repairing the hypopharyngeal and cervial esophageal breach postoperatively., Method: 6 kinds of operations in 29 patients with hypopharyngeal and cervical esophageal repairing were analyzed., Result: In 9 patients laryngeal function reserved, 8 cases recovered their function of swallow and speech postoperatively. One received a laryngectomy in II stage for wrong swallow. In 20 cases without laryngeal function, 17 cases can swallow, but 3 cases had fistula. The 1, 3 and 5 year survival rates were 92%, 64.7% and 46.2% respectively., Conclusion: The firsthand suture and pectoralis major myocutaneous flap were better for the hypopharyngeal repairing of the patients with laryngeal function. The glossus flap, laryngeal tissue flap, pectoralis major myocutaneous flap and gastric-pharyngeal tally are useful for the digestive canal reconstruction of the patients with laryngectomy.