Enzymatic vitreolysis using reengineered Vibrio mimicus-derived collagenase.

Purpose: Collagen is a key player contributing to vitreoelasticity and vitreoretinal adhesions. Molecular reorganization causes spontaneous weakening of these adhesions with age, resulting in the separation of the posterior hyaloid membrane (PHM) from the retina in what is called complete posterior vitreous detachment (PVD). Incomplete separation of the posterior hyaloid or tight adherence or both can lead to retinal detachment, vitreomacular traction syndrome, or epiretinal membrane formation, which requires surgical intervention. Pharmacological vitrectomy has the potential of avoiding surgical vitrectomy; it is also useful as an adjunct during retinal surgery to induce PVD. Previously studied enzymatic reagents, such as collagenase derived from Clostridium histolyticum, are nonspecific and potentially toxic. We studied a novel collagenase from Vibrio mimicus (VMC) which remains active (VMA), even after deletion of 51 C-terminal amino acids. To limit the activity of VMA to the vitreous cavity, a fusion construct (inhibitor of hyaluronic acid-VMA [iHA-VMA]) was made in which a 12-mer peptide (iHA, which binds to HA) was fused to the N-terminus of VMA. The construct was evaluated in the context of PVD. Methods: VMA and iHA-VMA were expressed in Escherichia coli, purified, and characterized with gelatin zymography, collagen degradation assay, fluorescamine-based assay, and cell-based assays. Two sets of experiments were performed in New Zealand albino rabbits. Group A (n = 10) received iHA-VMA, while group B (n = 5) received the equivalent dose of VMA. In both groups, saline was injected as a control in the contralateral eyes. Animals were monitored with indirect ophthalmoscopy, optical coherence tomography (OCT), and B-scan ultrasonography. Retinal toxicity was assessed with hematoxylin and eosin (H&E) staining of retinal tissue. Results: The activity of iHA-VMA and VMA was comparable and 65-fold lower than that of C. histolyticum collagenase Type IV. In the iHA-VMA group, all the rabbits (n = 10) developed PVD, with complete PVD seen in six animals. No statistically significant histomorphological changes were seen. In the VMA group, four of the five rabbits developed complete PVD; however, retinal morphological changes were seen in two animals. Conclusions: iHA-VMA displays targeted action confined to the vitreous and shows potential for safe pharmacologic vitreolysis.

Foveal configurations with disappearance of the foveal pit in eyes with macular pucker: Presumed role of Müller cells in the formation of foveal herniation.

Many eyes with macular pucker are characterized by a centripetal displacement of the inner foveal layers which may result in a disappearance of the foveal pit. In this retrospective case series of 90 eyes with macular pucker of 90 patients, we describe using spectral-domain optical coherence tomography different foveal configurations with ectopic inner foveal layers, document the relationship between posterior vitreous detachment (PVD) and idiopathic epiretinal membrane (ERM) formation and spontaneous and postoperative morphological alterations of the fovea, and propose an active role of Müller cells in the development of foveal herniation. We found that ERM were formed during or after partial perifoveal PVD, or after foveal deformations caused by tissue edema. The ERM-mediated centripetal displacement of the inner foveal layers and in various eyes anterior hyaloidal traction caused a disappearance of the foveal pit and an anterior stretching of the foveola with a thickening of the central outer nuclear layer (ONL). After the edges of the thickened inner layers of the foveal walls moved together, continuous centripetal displacement of the inner foveal layers generated a bulge of the fovea towards the vitreous (foveal herniation). Macular pseudoholes with a herniation of the inner foveal layers show that the outer layer of the protruding foveal walls is the outer plexiform layer (OPL). If the ERM covered the foveal walls and parafova, but not the foveola, the inner layers of the foveal walls were not fully centripetally displaced and the foveal pit was present. The visual acuity of eyes with ectopic inner foveal layers was inversely correlated with the thickness of the foveal center. Spontaneous morphological alterations after disappearance of the foveal pit may include the development of cystoid macular edema or additional thickening of the foveal tissue and foveal herniation. The foveal configuration with ectopic inner layers of the foveal walls and a thick central ONL persisted over longer postoperative time periods. The data show that the centripetal displacement of the inner foveal layers in eyes with macular pucker, which results in a disappearance of the foveal pit, may also generate foveal herniation which is suggested to be caused by contraction of Müller cell processes in the OPL. The centripetal displacement of the inner foveal layers and the formation of foveal herniation are suggested to reverse the foveal pit formation during development.

Incidence of posterior vitreous detachment after congenital cataract surgery: an ultrasound evaluation.

BACKGROUND: To investigate the incidence of developing posterior vitreous detachment (PVD) in children after congenital cataract surgery. METHODS: This is a prospective study which recruited 131 children with congenital cataracts who underwent cataract surgery between June 1, 2015, and September 1, 2018. The patients were divided into two groups depending on their post-operation phakic status (with or without IOL implantation). Infants aged from 6 to 12 months from two groups were analyzed as subgroups, respectively. B-scan ultrasonography was performed before the procedure and at 1, 3, 6, 9, and 12-month follow-ups, respectively, after the operation. RESULTS: Of the 131 eyes included in the analyses, 74 were aphakic, and 57 were pseudophakic after surgery. The postoperative rate of PVD in all analyzed eyes was 6.9% (9 of 131 eyes). After 12 months, PVD was significantly more prevalent in the eyes that underwent cataract surgery with IOL implantation (10.5%, 6 of 57 eyes) compared to the eyes without IOL implantation (4.1%, 1 of 74 eyes, P < 0.05); however, the eyes in the aphakic group were significantly younger than the eyes in the pseudophakic group, while the mean axial length (AL) of the pseudophakic eyes (21.11 ± 2.07 mm) was significantly higher than that of the aphakic eyes (18.93 ± 1.86 mm) (P < 0.01). In patients between the ages of 6 and 12 months of age from the two groups, the AL of patients with IOL implantation continued to be significantly increased compared to the group without IOL implantation (20.44 ± 1.68 mm vs. 19.78 ± 1.52 mm, P < 0.01). At the follow-up appointments, two patients with PVD were observed among the 14 eyes that had undergone cataract surgery with IOL implantation, while one eye was observed to have developed PVD among the 15 eyes without IOL implantation. CONCLUSIONS: PVD occurs with greater frequency after congenital cataract surgery, particularly in eyes that have undergone IOL implantation. We suggest that PVD should be carefully monitored in children after congenital cataract surgery to avoid subsequent ocular pathologies such as retinal detachment. Future studies are needed to determine other potential risk factors that have not been as thoroughly explored, as opposed to better-known factors such as older age, longer axial length, and IOL implantation.

Continued smoking and posterior vitreous adhesion in the elderly evaluated on swept-source optical coherence tomography.

In this cross-sectional study including 1150 eyes of 681 volunteers ≧ 65 years old without retinal pathology, factors affecting the progression of posterior vitreous detachment (PVD) were investigated. PVD stages were diagnosed based on swept-source optical coherence tomography (SS-OCT). Linear mixed effect model was used to determine whether age, gender, diabetes mellitus (DM), hypertension (HT), dyslipidemia (DL), and smoking status were associated with various stages of PVD. As a result, the multivariable analysis disclosed that the associations between older age and higher PVD stages (estimate [95% CI], 0.031 [0.020 to 0.042]; P < 0.0001), and current smokers and lower PVD stages (estimate [95% CI], - 0.24 [- 0.43 to - 0.056]; P = 0.011) were statistically significant. In contrast, female gender was not an independent factor affecting PVD stages in the elderly. Our analysis indicated that higher PVD stages observed in female eyes may be due to confounding effect, in which current smokers were predominantly males (i.e., 12.6% among males vs. 3.9% among females, P < 0.0001). In conclusion, our findings suggest that continuous smoking is associated with an adherent vitreoretinal interface in the elderly.

Effect of posterior vitreous detachment on treat-and-extend versus monthly ranibizumab for neovascular age-related macular degeneration.

AIMS: To investigate the impact of posterior vitreous detachment (PVD) on the efficacy of treat-and-extend (T&E) ranibizumab in neovascular age-related macular degeneration. METHODS: In a post hoc analysis of a randomised controlled clinical trial, spectral-domain optical coherence tomography images of treatment-naïve patients randomised to receive T&E (n=265) or monthly (n=264) ranibizumab for 12 months were included. Certified, masked graders diagnosed the presence or the absence of complete PVD. The main outcome measures were the mean change in best-corrected visual acuity (BCVA) and central retinal thickness (CRT) at month 12, the number of administered ranibizumab injections and the proportion of patients extended to more than 8 weeks. RESULTS: At baseline, complete PVD was present in 51% and 56% of patients in the monthly and T&E arms, respectively. Mean change in BCVA at month 12 was +9.0 (PVD) vs +9.5 letters (no PVD, p=0.78) in monthly treated eyes, and +6.0 (PVD) vs +7.5 letters (no PVD, p=0.42) in T&E treated eyes. Conversely, mean change in CRT at month 12 was -174 (PVD) vs -173 µm (no PVD, p=0.98) in the monthly arm, and -175 (PVD) vs -164 µm (no PVD, p=0.58) in the T&E arm. In T&E treated patients, the median number of injections was eight vs nine (p=0.035). 71% of PVD eyes were extended successfully, compared with 55% of eyes without PVD (p=0.005). CONCLUSION: PVD was not found to impact functional and anatomical outcomes of T&E ranibizumab therapy. However, patients without a complete PVD required more retreatments and were significantly less likely to be successfully extended. TRIAL REGISTRATION NUMBER: NCT01948830.

Characteristics and pathologies of the vitreo-macular interface-results from the Gutenberg Health Study.

PURPOSE: We aimed to determine the prevalence of characteristics and pathologies of the vitreo-macular interface within the general population. METHODS: The Gutenberg Health Study is a population-based study in Germany, including an ophthalmological examination with refraction, biometry and optical coherence tomography (OCT) imaging. Characteristics of the vitreo-macular interface were graded on volume scans including visibility of an epiretinal membrane, full-thickness macular hole, lamellar hole and pseudohole. Overall and age-specific prevalences including 95% confidence intervals [95%-CI] were calculated. Association analyses were conducted to determine systemic and ocular factors that are associated with epiretinal membranes (the most common pathology) using multivariable logistic regression. RESULTS: A total of 1890 people aged 40-80 years were included in the study. Of these, 4.7% (95%-CI: 3.8%-5.8%) had an epiretinal membrane in at least one eye, 0.1% a full-thickness macular hole, 0.6% a lamellar hole and 0.6% a pseudohole. The presence of an epiretinal membrane was associated with higher age, myopic refractive error and prior retinal laser therapy, but not with gender, body height, body weight, smoking, prior cataract surgery or intraocular pressure. CONCLUSIONS: Epiretinal membranes are more frequent in older and myopic subjects and in those with prior retinal laser therapy.

Spontaneous disappearance and recurrence of impending macular hole: a case report.

BACKGROUND: There have been several reports of spontaneous closure and reopening of a macular hole, however, in most of those cases, it was observed in eyes post vitrectomy. Here, we report a case of multiple episodes of spontaneous disappearance and recurrence of impending macular hole (stage 1B macular hole) with no history of previous surgery. CASE PRESENTATION: A 76-year-old Japanese man presented with a primary complaint of reduced visual acuity in his right eye. On initial examination, the visual acuity in his right and left eye was 0.4 and 0.01, respectively. He had previously been diagnosed as having macular degeneration of unknown origin in his left eye. Optical coherence tomography imaging confirmed vitreomacular traction and impending macular hole in his right eye. After a 1-week follow-up period, posterior vitreous detachment was detected, and the impending macular hole appeared to be resolved. Two months later, the impending macular hole had completely disappeared and his visual acuity had improved to 0.9. Six months later, he again noticed decreased vision in his right eye. An examination revealed that his visual acuity had dropped to 0.4, and there was a recurrence of impending macular hole. An optical coherence tomography examination showed no definitive findings of vitreous traction, and, 1 month later, spontaneous disappearance was observed again and his visual acuity improved to 0.7. CONCLUSIONS: In this case, both the initial onset and the recurrence involved impending macular hole, however, the optical coherence tomography findings differed at each examination. These findings suggest that some causes other than vitreous traction were responsible for both the spontaneous disappearance and recurrence of the impending macular hole in this present case.

Ultrasonographic characteristics of active ocular toxoplasmosis / Achados ultrassonográficos em toxoplasmose ocular ativa

ABSTRACT Purpose: To evaluate ophthalmic ultrasonographic findings associated with active ocular toxoplasmosis. Methods: Forty-seven eyes with active ocular toxoplasmosis in 47 patients were subjected to ocular ultrasonography using the transpalpebral technique (10-MHz transducer) and fundus photography. Patient medical records were retrospectively reviewed. Results: Ocular ultrasonography revealed vitritis, posterior vitreous detachment, retinal wall thickening, and non-rhegmatogenous retinal detachment in 47 (100%), 36 [76.6%; partial in 12 (25.5%) and total in 23 (48.9%)], 12 (25.5%), and 5 eyes (10.6%). Thirty-five of the 36 eyes with posterior vitreous detachment (97.2%) exhibited posterior hyaloid thickening; moreover, adhesion to the exudative lesion and vitreoschisis were observed in 4 (11.1%) and 12 eyes (25.5%), respectively. Ultrasonography detected the location of the exudative focus in 12 eyes (25.5%). Conclusion: Ultrasonography is helpful for detecting important intraocular findings of acute ocular toxoplasmosis that can be hindered by medial opacity or posterior synechiae.

RESUMO Objetivo: Avaliar os achados da ultrassonografia na toxoplasmose ocular ativa. Métodos: Quarenta e sete olhos com toxoplasmose ocular ativa em 47 pacientes foram submetidos à ultrassonografia ocular pela técnica transpalpebral (transdutor de 10 MHz) e fundo de olho. Os prontuários médicos foram revistos retrospectivamente. Resultados: A ultrassonografia ocular revelou vitreíte, descolamento vítreo posterior, espessamento da parede da retina e descolamento de retina não regmatogênico em 47 (100%), 36 [76,6%; parcial em 12 (25,5%) e total em 23 (48,9%)], 12 (25,5%) e 5 olhos (10,6%). Trinta e cinco dos 36 olhos com descolamento vítreo posterior (97,2%) exibiram espessamento hialoide posterior; além disso, a adesão à lesão exsudativa e vitreosquise foi observada em 4 (11,1%) e 12 (25,5%), respectivamente. A ultrassonografia detectou a localização do foco exsudativo em 12 olhos (25,5%). Conclusão: A ultrassonografia é útil na detecção de importantes achados intra-oculares de toxoplasmose ocular aguda que podem ser prejudicados pela opacidade medial ou sinéquia posterior.

The role of posterior vitreous detachment on the efficacy of anti-vascular endothelial growth factor intravitreal injection for treatment of neovascular age-related macular degeneration.

PURPOSE: A prospective cohort study investigating the effect of posterior vitreous detachment (PVD) on the efficacy of intravitreal bevacizumab for exudative age-related macular degeneration (AMD), in view of evidence that the vitreoretinal interface impacts the severity of the disease. METHODS: Treatment-naïve AMD eyes with (+) complete PVD and without (-) PVD on ultrasonography received three monthly and then pro re nata bevacizumab injections. Best-corrected visual acuity (BCVA) on Snellen charts and optical coherence tomography (OCT) findings were recorded for 12 months. Secondary analysis included PVD definition and group allocation according to OCT baseline scan. RESULTS: Forty-one eyes of 34 patients met the inclusion criteria. At 12 months, median BCVA improved by 0.12 logMAR in the PVD+ group [interquartile range (IQR) -0.52, 0.03, P = 0.140] and remained the same in the PVD- group (IQR -0.12, 0.15, P = 0.643). Median central retinal thickness improved by 43.5 µm and 43 µm in the PVD+ (IQR -143, 3, P = 0.016) and PVD- group (IQR -90, -14, P = 0.008), respectively. All parameters were similar in the two groups at final follow up (P > 0.05). The secondary analysis included 32 eyes of 26 patients and showed no significant differences between the groups at the 12 months endpoint (P > 0.05). CONCLUSION: Our findings show no significant impact of PVD as assessed by ultrasound or by OCT on visual and anatomical outcomes in exudative AMD treated with bevacizumab.

Familial exudative vitreoretinopathy complicated with full thickness macular hole: A case report.

RATIONALE: To report a case of familial exudative vitreoretinopathy (FEVR) complicated with full-thickness macular hole (FTMH). PATIENT CONCERNS: A 39-year-old male presented after becoming aware of metamorphopsia in his left eye. DIAGNOSES: Fundus examination showed a retinal avascular area, retinal vascular abnormality, and yellow exudation at the peripheral retina in both eyes. Optical coherence tomography findings revealed impending MH (IMH) due to posterior vitreous detachment (PVD) in his left eye. Despite of the occurrence of spontaneous complete PVD, an FTMH developed at 4 months after the onset of IMH. INTERVENTIONS: To treat the FTMH, vitreous surgery was performed. Intraoperative findings revealed that the thick posterior vitreous membrane (PVM) had no adhesions with the edge of the FTMH. However, a thin epiretinal membrane (ERM) was observed around the MH. OUTCOMES: Postoperatively, the FTMH was closed, and the patient's corrected visual acuity improved from (0.4) to (0.8). LESSONS: In this present case, an IMH developed via traction by a thick PVM, characteristic of FEVR, with FTMH then developing via traction by a thin ERM. Our findings reveal that it is vital to fully understand these anatomical features before performing vitreous surgery for FTMH complicated with FEVR.

Idiopathic macular hole with asteroid hyalosis: Two case reports.

INTRODUCTION: Asteroid hyalosis (AH) is characterized by mild vitreous-body liquefaction and a reduced likelihood of posterior vitreous detachment (PVD). Here, we report the clinical features of 2 cases of macular hole (MH) in eyes with AH. The extent of retinal vitreous adhesion at the time of vitreous surgery, the presence or absence of iatrogenic retinal breaks, and the postoperative course was examined in regard to the association with AH. CASE PRESENTATION: Case 1 involved a 67-year-old female with decreased visual acuity in her left eye. Although preoperative optical coherence tomography examination revealed complete PVD with operculum around the fovea central region, vitreoretinal adhesion was quite strong starting at the midperiphery of the fundus, and an iatrogenic retinal break was formed at the inferior site during vitreous surgery. Endophotocoagulation was performed, and no onset of retinal detachment (RD) postsurgery was observed. Case 2 involved a 74-year-old male with metamorphopsia in his right eye. Preoperative perifoveal PVD was observed, yet vitreoretinal adhesion was strong starting at the midperiphery of the fundus and multiple iatrogenic retinal breaks had formed on the inferior, superior, and temporal sites. Moreover, RD occurred during surgery. Endophotocoagulation and gas tamponade was performed; however, postoperative RD requiring multiple reoperations occurred. CONCLUSION: As with normal MH, MH with AH is thought to be caused by perifoveal PVD, yet as the vitreoretinal adhesion was found to be quite strong starting at the midperiphery of the fundus, we decided to keep the artificial PVD within a range where adhesion was loose.

Retinal detachment after acute posterior vitreous detachment resulting from posterior chamber phakic intraocular lens implantation.

A 39-year-old woman presented with an early posterior vitreous detachment (PVD) after the implantation of an Implantable Collamer Lens phakic intraocular lens (pIOL). The patient detected PVD, noticing metamorphopsia early after surgery; it was confirmed with optical coherence tomography 5 days postoperatively. She was examined by a retinal expert, and no retinal tears were found. However, 15 days postoperatively, the patient had a retinal tear; 38 days later, a retinal detachment (RD) occurred despite treatment with argon laser photocoagulation. Prompt treatment and adequate patient cooperation achieved a final good uncorrected distance visual acuity (20/20). To our knowledge, this is the first description of the whole sequence involving early PVD leading to RD after pIOL implantation. This case highlights the importance of a fundus examination before and after surgery and of informing patients about the small chance of RD and visual symptoms.

A Study of the Natural History of Vitreomacular Traction Syndrome by OCT.

PURPOSE: To examine the natural history of vitreomacular traction syndrome (VMTS) in the absence of other ocular comorbidities. DESIGN: Retrospective clinical case series. PARTICIPANTS: A total of 183 eyes of 159 patients diagnosed with VMTS with no other ocular comorbidity. METHODS: Patients with VMTS were identified from an OCT database at Moorfields Eye Hospital, London. Sequential OCT scans and patient notes were reviewed over a minimum period of 6 months. Data collected included patient demographics, best-corrected visual acuity, and OCT features of vitreomacular adhesion. Contingency tests and binary logistic modeling were used to identify baseline predictors of stability and progression. MAIN OUTCOME MEASURES: The rates of spontaneous resolution (defined by release of traction), progression to full-thickness macular hole, and surgical intervention were analyzed. RESULTS: Presenting visual acuity was 0.3±0.3 logMAR units. The mean length of follow-up was 17.4±12.1 months. During this period, VMTS persisted in 60% and resolved in 20% (occurring on average at 15 months). Of the remainder, 12% developed a macular hole and 8% elected to proceed with surgery for symptoms. Focal adhesion <1500 µm was present in 87%. A premacular membrane with macular pucker (PMM) was present in 20%. With persistent VMTS, vision and central foveal thickness remained unchanged. The relative risk of resolution increased in those cases with better presenting visual acuities, lesser foveal thicknesses, and no associated PMMs; vision significantly improved in those cases with resolution. CONCLUSIONS: VMTS persists in the majority of patients but despite this, visual acuities did not deteriorate significantly over the study period unless patients developed a full-thickness macular hole or required surgical intervention for symptoms. Resolution spontaneously occurred in 20%, with an improvement in vision.

Evaluation of posterior vitreous detachment after uneventful phacoemulsification surgery by optical coherence tomography and ultrasonography.

BACKGROUND: The aim was to investigate the effect of uneventful phacoemulsification on vitreoretinal interface evaluated by optical coherence tomography (OCT) and ultrasound. METHODS: One hundred and fifty eyes were evaluated for the presence or absence of posterior vitreous detachment by ultrasound and OCT prior to cataract surgery. Only those eyes with intact vitreoretinal adhesion were included in the study. All patients underwent cataract surgery. At one month, three months and six months, all patients were assessed with OCT and ultrasound. The data were analysed for statistical significance. RESULTS: The study was carried out on 30 of 31 patients with no posterior vitreous detachment. The mean age of patients was 62.63 ± 7.15 years (range 49 to 75 years) and 15 were female. Posterior vitreous detachment developed in 17 patients (56.66 per cent) at one month, 21 patients (70 per cent) at three months and 22 patients (73.33 per cent) at six months following the surgery. No complications or acute symptoms were detected associated with posterior vitreous detachment. CONCLUSION: Vitreoretinal interface changes and vitreoretinal separations and progression of separation can be easily evaluated by OCT. The current study confirms that posterior vitreous detachment occurs commonly soon after cataract surgery.

Comparison of macular findings due to vitreomacular traction alone or in association with epiretinal membrane.

PURPOSE: To evaluate the anatomical and functional findings in patients with vitreomacular traction (VMT) alone or in combination with epiretinal membrane (ERM). METHODS: In this retrospective, cross-sectional study, we studied 102 patients with VMT, either alone (n = 37) or combined with ERM (n = 65). All patients were examined with spectral-domain optical coherence tomography (SD-OCT). We recorded the vitreofoveal angle of VMT nasally and temporally, the horizontal diameter of VMT, macular thickness, the presence, type, and location of macular edema, the ellipsoid zone (ΕΖ)/external limiting membrane (ELM) status, and the visual acuity. RESULTS: Patients with VMT combined with ERM presented smaller vitreofoveal angle nasally and temporally, broader adhesion diameter, and a greater extent of EZ defect compared to patients with VMT alone, although there was no statistically significant difference in EZ and ELM condition regarding the number of affected cases. There is also no statistically significant difference between the 2 groups concerning the visual acuity. In the majority of patients with VMT alone, cystoid macular edema was present mainly at the foveal area. In cases where VMT coexisted with ERM, macular edema was mostly found to be diffuse, while cystoid or mixed type, extending to the whole macular area, was also present. CONCLUSIONS: Patients with VMT in association with ERM have different characteristics in SD-OCT compared to those with VMT alone regarding the type and location of macular edema, the extent of EZ defect, as well as the vitreofoveal angle and the VMT diameter. Moreover, they presented worse visual acuity compared to those with VMT alone, although the difference did not reach statistical significance.

Late development of a lamellar macular hole after the spontaneous separation of vitreoretinal traction: case report.

PURPOSE: To report a case of epiretinal macular membrane and a lamellar macular hole developing after the spontaneous separation of an idiopathic vitreoretinal traction. METHODS: Retrospective, observational, long follow-up of a case evaluated and documented with optical coherence tomography and managed with current therapies including pars plana vitrectomy. RESULTS: Good anatomic and functional results with stable 20/30 best-corrected visual acuity. CONCLUSIONS: The natural course of idiopathic vitreomacular traction has been documented during prolonged observation after its spontaneous separation from the fovea leading to the development of an epiretinal membrane and a lamellar macular hole.

Cephalometrics in Stickler syndrome: Objectification of the typical facial appearance.

INTRODUCTION: Stickler syndrome is a connective tissue disorder characterized by orofacial, ocular, skeletal and auditory symptoms. The orofacial phenotype mainly consists of midfacial hypoplasia, micrognathia and cleft palate. Large phenotypic variability is evident though. Few studies have tried to substantiate the typical facial appearance in Stickler syndrome patients. METHODS: Molecularly confirmed Stickler patients were invited to undergo cephalometric analysis based on a lateral radiograph in standardized conditions. Angular and linear measurements were performed according to Steiner's and Sassouni's analysis and compared with age- and gender-matched reference values. RESULTS: Thirteen patients aged 10-62y were included, twelve of whom had type 1 Stickler syndrome (COL2A1 mutation) and one type 2 Stickler syndrome (COL11A1 mutation). The position of maxilla and mandible relative to the cranial base was not significantly different from the reference population (S-N-A: p = 0.73, S-N-B: p = 0.43). The mandibular plane and y-axis showed an elevated angle with the cranial base in most patients, although not significant for the total group (S-N to Go-Me: p = 0.20, S-N to S-Gn: p = 0.18). Dental analysis was normal, except for a higher overjet value (p = 0.006) and a higher angle between occlusal plane and Frankfort plane (p = 0.022). CONCLUSION: Cephalometric analysis was not able to thoroughly prove the abnormal facial appearance in Stickler syndrome. The majority of patients had normal dentofacial proportions. The most frequently observed anomaly in our series is a rather short and posteriorly rotated mandible, but clinical variability is high.

PHOTORECEPTOR OUTER SEGMENT LENGTH AND OUTER FOVEAL THICKNESS AS FACTORS ASSOCIATED WITH VISUAL OUTCOME AFTER VITRECTOMY FOR VITREOMACULAR TRACTION SYNDROME.

PURPOSE: To investigate the predictive factors for postoperative best-corrected visual acuity (BCVA) in patients with vitreomacular traction syndrome treated with vitrectomy. METHODS: The records of 21 patients with 21 eyes that underwent vitrectomy for vitreomacular traction syndrome and followed for at least 12 months were retrospectively reviewed. The BCVA and spectral domain optical coherence tomography findings were investigated preoperatively and at 1, 3, 6, and 12 months postoperatively. Axial length was measured preoperatively. The correlations between 12-month postoperative BCVA and preoperative parameters, including BCVA, age, axial length, central foveal thickness, outer foveal thickness, and photoreceptor outer segment length, were evaluated. RESULTS: Twelve-month postoperative BCVA was significantly negatively correlated with preoperative outer foveal thickness and photoreceptor outer segment length (outer foveal thickness: P = 0.029, r = -0.501; photoreceptor outer segment length: P = 0.022, r = -0.523, respectively) but not correlated with age, axial length, preoperative BCVA, and preoperative central foveal thickness (age: P = 0.346, r = 0.216; axial length: P = 0.333, r = 0.242; BCVA: P = 0.202, r = 0.290; central foveal thickness: P = 0.065, r = -0.410, respectively). CONCLUSION: Preoperative outer foveal thickness and photoreceptor outer segment length could be good predictive factors of postoperative BCVA in patients with vitreomacular traction syndrome.