Unusual anterior and posterior segment features of coats disease.

PURPOSE: To report the clinical course and management of unusual anterior and posterior segment features of Coats disease and their relation to the age of the patients to increase the awareness towards these rare clinical features rarely described in the current literature. METHODS: A retrospective descriptive review of 45 eyes of 45 patients affected by Coats disease was conducted at the Retinoblastoma Referral Center and Ophthalmology Unit of the University of Siena in Italy analyzing data from 2000 to 2022. Medical records and images were revised to find some cases presenting unusual anterior and posterior segment features in patients affected by Coats disease.We identified therefore 4 unusual clinical conditions: retinal macrocysts, anterior chamber cholesterolosis, fovea-sparing Coats disease and secondary vasoproliferative tumor. RESULTS: Two patients presented with retinal macrocyst (2/45 = 4.4%), one with anterior chamber cholesterolosis (1/45 = 2.2%), two with fovea sparing Coats disease (2/45 = 4.4%) and one with vasoproliferative tumor associated (1/45 = 2.2%) for a total of six (6/45 = 13.3%) patients manifesting unusual anterior or posterior segment features in Coats disease. CONCLUSION: Unusual anterior and posterior segment features of Coats disease such as retinal macrocyst and anterior chamber cholesterolosis have been more frequently reported in younger children while fovea-sparing and vasoproliferative tumors have been more commonly described in older patients. Age is then a strong prognostic marker which allows to distinguish two different phenotypes of Coats disease: patients younger and older than 3 years old with more aggressive and milder phenotype respectively.

The Humira in Ocular Inflammations Taper (HOT) Study.

PURPOSE: To assess factors that impact the risk of relapse in patients with noninfectious uveitis (NIU) who undergo adalimumab tapering after achieving remission. DESIGN: Retrospective study. METHODS: In this multicenter study, patients with NIU were treated with adalimumab and subsequently tapered. Patient demographics, type of NIU, onset and duration of disease, the period of inactivity before tapering adalimumab, and the tapering schedule were collected. The primary outcome measures were independent predictors of the rate of uveitis recurrence after adalimumab tapering. RESULTS: Three hundred twenty-eight patients were included (54.6% female) with a mean age of 34.3 years. The mean time between disease onset and initiation of adalimumab therapy was 35.2 ± 70.1 weeks. Adalimumab tapering was commenced after a mean of 100.8 ± 69.7 weeks of inactivity. Recurrence was observed in 39.6% of patients at a mean of 44.7 ± 61.7 weeks. Patients who experienced recurrence were significantly younger than those without recurrence (mean 29.4 years vs 37.5 years, P = .0005), and the rate of recurrence was significantly higher in younger subjects (hazard ratio [HR] = 0.88 per decade of increasing age, P = .01). The lowest rate of recurrence was among Asian subjects. A faster adalimumab taper was associated with an increased recurrence rate (HR = 1.23 per unit increase in speed, P < .0005). Conversely, a more extended period of remission before tapering was associated with a lower rate of recurrence (HR = 0.97 per 10-weeks longer period of inactivity, P = .04). CONCLUSIONS: When tapering adalimumab, factors that should be considered include patient age, race, and duration of disease remission on adalimumab. A slow tapering schedule is advisable.

Efficacy and safety of intraoperative use of tropicamide 0.02%/phenylephrine0.31%/lidocaine1% intracameral combination during pediatric cataract surgery.

BACKGROUND: To demonstrate the safety and efficacy of the intracameral use of tropicamide 0.02%/phenylephrine 0.31%/lidocaine 1% in pediatric cataract surgery, a combination widely used in adult patients but still off-label in children. METHODS: Design: two-center, prospective, observational study. SETTING: San Giuseppe Hospital, Milan and Meyer Children's Hospital, Florence. STUDY POPULATION: children from 0 to 4 years of age undergoing cataract surgery with or without intraocular IOL implantation, in the absence of clinically significant systemic conditions, history of ocular surgery, concurrent ocular medication, hypersensitivity to any of the substances and post-traumatic cataracts. During the surgery, patients received the combination drug after the primary access to the anterior chamber. Efficacy was evaluated by achieving an adequate mydriasis in order to perform capsulorhexis, while safety was assessed by recording vital signs (heart rate, blood pressure, respiratory rate, temperature) pre- and post-administration of the substance. RESULTS: This study included 53 surgical procedures of 36 patients: 41 eyes were left aphakic, while 12 eyes received primary IOL implantation. The pupil size was adequate to safely perform capsulorhexis in 52 procedures of 53. The difference in pupil enlargement was significant (6.0 ± 1.14 mm, P = < 0.001). There were no notable changes in vital parameters. CONCLUSIONS: The administration of intracameral tropicamide 0.02%/phenylephrine 0.31%/lidocaine 1% in pediatric cataract surgery is effective for obtaining an adequate mydriasis without any vital parameters changes throughout the procedure.

Evaluation of macular vessel density changes after strabismus surgery using optical coherence tomography angiography.

PURPOSE: To compare retinal vessel density before and after strabismus surgery using swept-source optical coherence tomography angiography (SS-OCTA). METHODS: In this longitudinal, single-arm pilot study, vascular density in the deep capillary plexus (DCP), superficial retinal capillary plexus (SCP) and choriocapillaris layer (CCL) in consecutive subjects undergoing strabismus surgery was assessed using SS-OCTA preoperatively (T0), on the first day postoperatively (POD1), and 30 days postoperatively (POD30). RESULTS: A total of 92 eyes of 56 patients (54% males) were included. Mean patient age was 41.1 ± 22.7. OCT vascular density of the DCP was 50.20 ± 5.57 at T0, 52.74 ± 4.77 at POD1, and 50.92 ± 4.58 at POD30. The differences were statistically significant for T0 versus POD1 (P < 0.05). Vascular density of the CCL was 50.72 ± 4.80 at T0, 53.59 ± 3.65 at POD1, and 51.39 ± 4.64 at POD30. The differences were statistically significant for T0 versus POD1 (P < 0.05). No significant differences were found in SCP (P > 0.05). CONCLUSIONS: Transitory hemodynamic changes can occur in the DCP and in the CCL following muscle recession procedures.

Inferior rectus pulled-in-two syndrome: Surgical results after anterior and nasal transposition of the inferior oblique muscle.

Pulled-in-two syndrome (PITS) is a serious intraoperative complication of strabismus surgery in which an extraocular muscle manipulated during the procedure is ruptured and potentially lost. Usually, there is a systemic or local condition that determines muscle weakness when put under tension. If the proximal portion of the broken muscle can be found, it can be reattached to the ocular globe or remaining muscle. If this is not possible, there are multiple varying approaches. We present three cases of PITS of the inferior rectus muscle, treated with good results with anterior and nasal transposition of the inferior oblique muscle. We propose this surgery as another potential technique if the muscle can not be retrieved.

The Role of Hypertropia in the Surgical Management of Bilateral Inferior Oblique Muscle Overaction.

PURPOSE: Inferior oblique muscle overaction (IOOA) is an ocular motility anomaly consisting of overelevation in adduction, often associated with ipsilateral hypertropia. The weakening procedure of IO muscle is the most widely used procedure in IOOA. Usually, surgical planning is based on the degree of overaction of the IO muscle. MATERIALS AND METHODS: We have retrospectively analyzed patients with bilateral IOOA with and without hypertropia in primary position, who underwent a bilateral IO weakening procedure. Both the amount of IOOA and the presence of a hypertropia in primary position were taken into consideration for the surgical plan. RESULTS: Nineteen patients met the entry criteria for this study. In 12 patients, a hypertropia in primary position was present at baseline, and it was significantly lower after the asymmetrical IO weakening: 11 had an asymmetric IOOA at baseline, and one had symmetric IOOA. None of the remaining seven patients had a vertical deviation in primary position before surgery, despite having asymmetric IOOA. None of them developed a hypertropia in primary position after symmetric IO weakening. CONCLUSIONS: Our findings outline the utility of considering both the presence of a vertical deviation in primary position and the magnitude of IOOA in this set of patients. Asymmetric inferior oblique weakening procedure is effective in treating a hypertropia in the primary position and bilateral IOOA.

Unilateral Surgery in Bilateral Asymmetrical Dissociated Vertical Deviation With Strong Preferential Fixation.

PURPOSE: To determine the best treatment for dissociated vertical deviation (DVD). METHODS: The authors investigated the postoperative results of unilateral surgery for DVD in 14 patients when the visual acuity difference between the two eyes did not result in a postoperative dominance switch. All patients underwent bilateral medial rectus recession for congenital esotropia. RESULTS: With this unilateral surgical procedure, all patients showed a significant improvement of the DVD in the primary position. None of the patients developed hypotropia, anti-elevation syndrome, or inferior oblique overaction in the contralateral (dominant) eye, and no changes were observed in the contralateral eye. CONCLUSIONS: Unilateral surgery for DVD in the non-dominant eye in case of strong fixation preference does not create a postoperative manifest DVD in the unoperated dominant eye. [J Pediatr Ophthalmol Strabismus. 2022;59(1):60-64.].

Outcomes after surgical removal of anterior epi-capsular plaque associated with persistent pupillary membranes in children.

INTRODUCTION: Anteriorepi-capsular plaque (ACP) is usually eccentric and is associated with persistent iris strands arising from the collarette. Outcomes of lens preserving ACP peeling along with removal of persistent pupillary membrane (PPM) strands have seldom been reported with only a few cases within some case series appearing in the literature. Herein we aim to report outcome after surgical removal of ACP associated with PPM in children. METHODS: Research was done in three different Institutes. Charts were reviewed for all children who had undergone ACP removal surgery from 2010. Age at surgery, gender, preoperative best corrected visual acuity (BCVA), preoperative refraction, morphologic details of ACP, intraoperative complications, ophthalmic viscosurgical device use, age at last follow-up, postoperative complications, and postoperative BCVA were reviewed. RESULTS: A total of 16 patients, 10 male and 6 female, were included in study. Age at surgery ranged from 2 to 86 months old. Age at last follow up visit ranged from 2 to 14 years old. Postoperative complications were not observed in any of the 16 patients. Specifically, in all patients the crystalline lens remained clear at the last follow up visit. CONCLUSIONS: Congenital ACP of the lens associated with PPM and clear lens can be safely surgically removed with a very low risk of iatrogenic cataract in the early period after surgery.

Validation of the postnatal growth and retinopathy of prematurity screening criteria: A retrospective Italian analysis.

PURPOSE: Retinopathy of prematurity (ROP) is the leading cause of childhood blindness. The aim of our study is to validate the new screening criteria elaborated by the Postnatal Growth and Retinopathy of Prematurity (G-ROP) study group in a monocentric cohort of Italian preterm infants. METHODS: We retrospectively applied the G-ROP screening criteria to a cohort of preterm infants born between May 2015 and July 2020 with known birth weight, gestational age, serial weight measurement, and known ROP outcome. Primary outcomes were sensitivity and specificity of ROP detection, especially of treatment requiring ROP. Secondary outcomes were reduction of ophthalmologic examinations and of infants requiring screening. RESULTS: We retrospectively evaluated 595 children and 475 were included in our study. Of them, 119 developed any type ROP, 39 developed type 1 ROP, and 28 underwent treatment. G-ROP criteria predicted 39 of 39 cases of type 1 ROP (100% sensitivity and specificity). Sensitivity and specificity for detection of treated ROP were 100%. Considering any type ROP detection, sensitivity was 87.4% and specificity was 100%. Our analysis showed that screening could be avoided in 50% of patients, resulting in a 29% reduction of the number of examinations. CONCLUSIONS: Our study validates the new G-ROP screening protocol in a monocentric cohort of premature infants. We demonstrate that all Type 1 ROP and requiring treatment ROP could be found even with a reduction of eye examinations.

Definition of successful outcomes after surgery for each type of strabismus: a Delphi study.

BACKGROUND: The Delphi process has been widely used to delineate guidelines for the treatment of disorders for which there is little or no evidence in the published literature. The purpose of this study was to use the Delphi process to identify areas of consensus and disagreement on the definition of success after surgery for each type of strabismus. METHODS: Two rounds of electronic questionnaires were sent to 28 members of the Strabismus Success Definition Delphi Study Group. For the first round, responses to 70 questions were captured as agree (= 1) and disagree (= 2). For round 2, a total of 89 questions were captured on a Likert scale ranging from 1 (strongly disagree) to 7 (strongly agree). Consensus was determined a priori at 85%. RESULTS: In both the first and second rounds, inter-rater agreement of 85% consensus was reached for only 20% of questions. Intra-rater agreement per question was low, with κ values ranging from -0.11 to 0.62. Intra-rater agreement was also low among themes, ranging from poor to fair agreement: κ = 0.25 for motor, κ = 0.28 for sensory, and κ = 0.35 for follow-up. CONCLUSIONS: This study highlights consensus areas that could be considered by researchers in designing studies and identifies areas where lack of consensus indicates that further research is needed.

Outcome of surgery for near angle in patient with incomitant distance/near esotropia and without manifest deviation at distance.

INTRODUCTION: To investigate how a subset of patients with partially accommodative esotropia, with a manifest deviation <10 Prism Diopters (PD) at distance and an angle of strabismus at near wider than 10 PD, could respond to a surgical approach based on the misalignment at near. METHODS: Six patients examined from 2015 to 2019 met criteria for inclusion: partially accommodative esotropia, patients compliant to full correction for at least 6 months, angle of esotropia for near larger at least 10 PD, angle of misalignment obtained with the alternate prism cover test and simultaneous prism cover test at distance <10 PD. All patients underwent bilateral medial recti recession. They were examined at 3 weeks and at 6 months. RESULTS: No significant difference in the angle of esotropia at distance was found at any follow-up (Baseline: 7.17 ± 1.33 PD vs 3 weeks: 7.0 ± 1.01 PD vs 6 months: 7.33 ± 1.03 PD, p = 0.65, and p = 0.36, respectively). No case of consecutive exotropia was described. Considering the angle at near, there was a significant reduction at 3 weeks (Baseline: 41.7 ± 6.83 PD vs 3 weeks: 9.33 ± 1.63 PD, p = 0.027), that remained stable at the final follow-up (9.0 ± 1.1 PD, p = 0.32). All patients were suppressors and had nil stereopsis pre-operatively, all of them developed fusion and a different degree of stereopsis [276 ± 284 arcseconds (Range 80-800)]. CONCLUSIONS: A surgical approach based on the angle of strabismus for near could obtain satisfactory ocular alignment without consecutive exotropia in children with partially accommodative esotropia wearing full refractive correction, with a manifest deviation for distance <10 PD and a higher misalignment for near, at least for 6 months after surgery.

Optical coherence tomography angiography for the measurement of optic disc: Macular relationship.

INTRODUCTION: Fundus photography is the gold standard for assessing ocular torsion over the last 30 years. However, it is not a precise and reproducible tool during clinical practice. Optical coherence tomography angiography is characterized by precise identification of the macula and the optic disc, and it could be an effective method to easily calculate the angle of ocular torsion, compared to fundus photography. The aim of this study was to show whether any difference in the measurement and the accuracy of the angle of torsion between the head of the optic nerve and the fovea was present. METHODS: This is a prospective single-, referral-center study conducted at the San Giuseppe Hospital in Milan on 80 eyes of 40 adult patients, included in a random-sample way. Exclusion criteria were non-cooperation, higher refractive errors of ±3 diopters, retinal and optic disc pathologies, and ocular movement disorders. RESULTS: Patients' mean age was 54.3 ± 16.3 (range: 22-83) years. The angle measured by the fundus camera was 7.78° ± 3.04°, while the angle measured by the angiography was 7.09° ± 3.08° (p = .035). The mean interocular difference was 1.54° ± 3.42° for fundus photography and 0.5° ± 4.71° for angiography (p = .013). CONCLUSION: Optical coherence tomography angiography is a very useful, fast, precise, reproducible, and reliable technique in cooperative subjects, not inferior to the fundus camera and less prone to human error.

Asymmetric superior oblique split Z-tendon lengthening for face turn in bilateral asymmetric superior oblique overaction and exotropia.

INTRODUCTION: The aim was to evaluate the effectiveness of the procedure of the superior oblique split Z-tendon lengthening to collapse A pattern and treat face turn in a cohort of patients with both incomitant intermittent exotropia and a vertical deviation associated with a bilateral asymmetric superior oblique overaction. METHODS: We retrospectively reviewed the clinical records of patients with facial turn associated with intermittent exotropia and bilateral asymmetric superior oblique overaction, who underwent superior oblique muscle split tendon lengthening and concurrent horizontal surgery between 2009 and 2017. RESULTS: A total of eight patients met the inclusion criteria. The preoperative average face turn was 26.8° (range, 20°-5°), and it significantly improved to 5.4° (range, 2°-8°) (p < 0.05). All the patients showed an improvement in the face turn with neutralization of the vertical deviation. The vertical deviation in the right gaze for the patient with a right turn and the left gaze in patients with left turn improved significantly (values of p < 0.05). CONCLUSION: The superior oblique split Z-tendon lengthening was an effective procedure to collapse the A pattern and the treatment of the face turn in a cohort of patients with both incomitant intermittent exotropia and a vertical deviation associated with a bilateral asymmetric superior oblique muscle overaction with less complications, and less varying results.

Augmented vertical rectus transpositions: Intraoperative measurement of torsion following sequential muscle detachment.

INTRODUCTION: After vertical recti transposition surgery, a torsional change may occur. We hypothesized that step-by-step monitoring of the intraoperative torsional position of the eye can avoid any unexpected complications and identify critical moments of surgery that may have adverse effects. METHODS: Nine patients with sixth nerve palsy that underwent complete transposition of the superior (SR) and inferior rectus (IR) muscles to the lateral rectus muscle by means of the technique of augmented vertical recti transposition were prospectively included. The torsional position of the eye was recorded at eight stages of the procedure. RESULTS: We observed two distinct patterns of induced torsional deviation during partial disinsertion of a vertical rectus muscle depending on whether disinsertion began medially or laterally, after cutting about 3/4 of the muscle fibers: ¾ nasal disinsertion of the SR and ¾ temporal disinserton of the IR caused intorsion, ¾ temporal disinsertion of the SR and ¾ nasal disinsertion of the IR caused extorsion. Torsion improved after the vertical rectus was attached temporally to the sclera along the spiral of Tillaux with tying of the augmentation suture to the LR. The greatest change in torsion was from ¾ temporal disinsertion of the SR (5°extorsion), to reattachment along the spiral of Tillaux temporally (5°intorsion). CONCLUSION: The SR and IR have different torsional effects, which only appears when more than half of the fibers are detached. Augmented transposition does not cause any additional torsional effects. This intraoperative monitoring system can be used to detect unintended torsional complications, especially during transposition surgery.

Unusual presentation of early-onset X-linked retinoschisis: Report after 1 year of multimodal follow-up.

PURPOSE: To describe the unusual presentation, diagnosis, and clinical course of an early-onset X-linked infantile retinoschisis. CASE REPORT: A 6-month-old infant presented with strabismus and poor fixation. After the detection of bilateral intraretinal hemorrhage and diffuse dystrophic retinal pattern at indirect ophthalmoscopy, the patient received a complete evaluation under anesthesia. Retinal wide-field imaging, spectral domain optical coherence tomography, and electroretinogram were performed and revealed a retinoschisis involving the posterior pole and the inferior periphery in the right eye. In the left eye, an inferior retinal detachment extending to the macula was detected. Blood sample and genetic counseling were required in the strong suspicion of an inherited retinal dystrophy. Genetic tests confirmed the diagnosis of X-linked retinoschisis (RS1 gene mutation). After consultation with a pediatric vitreoretinal surgeon, a wait and see strategy was chosen. The follow up visits showed a surprisingly good natural course of the disease. CONCLUSION: X-linked retinoschisis is a well-known inherited retinal disease potentially affecting young children as early as 3 months old. In this case, the stunning presentation (diffuse retinal pigment epithelium dystrophic changes resembling a macular dystrophy) and the positive course of the disease (resolution of macular retinal detachment in the left eye and stability of schisis in the right eye) arise some interesting considerations about the necessity of an early surgical treatment.

Four petal evisceration versus standard technique: A retrospective study.

PURPOSE: To compare the four-petal evisceration technique and the standard evisceration technique. METHODS: In this retrospective, comparative case series study, sixteen charts of patients fulfilled the inclusion criteria and were were reviewed. Orbital implant exposition and complication rates between the two surgical techniques were evaluated. RESULTS: A total of 16 eye of 16 patients fulfilled the inclusion criteria and were included in the analysis (eight in the four petal group and eight in the standard evisceration group).During postoperative follow-up there were no cases of implant exposure or migration in the four-petal group and two cases (25%) of implant exposure in the standard evisceration group.Exposed orbital implants dimension were both 16 mm and patients eviscerated for phtisis bulbiAll surgeries were uneventful, and there were no serious complications during the study. CONCLUSION: Four petal evisceration technique seems particularly useful in patients with phtisis bulbi because it allowed the placement of an implant significantly larger than the standard technique with low rate of esposition.

Latest evidences on meibomian gland dysfunction diagnosis and management.

Meibomian gland dysfunction (MGD) can be considered the leading cause of dry eye disease (DED) and one of the most common ophthalmic disorders found in clinical practice. The growing body of literature provides a substantial amount of information on this condition, but more efforts are needed to better interpret research data and to properly apply them to daily clinical practice., In this article, we reviewed the most recent publications on MGD diagnosis and management, focusing on the highest available level of evidence, provided by well-designed and well-reported studies on humans., Latest evidences on MGD diagnosis are mainly focused on imaging techniques, including meibography, optical coherence tomography (OCT), and in vivo confocal microscopy. Meibographic parameters, such as drop-out and glands' distortion, show great diagnostic accuracy, which accounts for their widespread use in clinical practice and research., Recent randomized controlled clinical trials on MGD treatment provided data on the role of antibiotics, steroids, essential fatty acids, intraductal meibomian gland probing, electronic heating devices and intense pulsed light therapy.