Surgical treatment outcomes in heavy eye syndrome - a case series.

Results: The study cohort comprised five patients, each presenting with unilateral high axial myopia and classic clinical features of HES, including large angle esotropia, hypotropia, and restricted abduction and supraduction. All patients displayed evident superotemporal globe prolapse on MRI imaging, corresponding to the downward displacement of the LR muscle and medial shift of the SR muscle.Following the surgical procedure, all patients demonstrated significant improvements in both vertical and horizontal deviations, effectively addressing the primary clinical manifestations of HES.Conclusions: In the management of HES, several surgical approaches have been explored, yielding mixed results. Our study, employing the technique of partial muscle splitting and scleral fixation, offers a promising avenue for effectively addressing this challenging condition. By adapting the full loop myopexy technique originally proposed by Yokoyama et al. we achieved satisfactory ocular alignment in all five patients. Notably, this approach mitigates the risk of anterior segment ischemia by preserving the unsecured portions of the SR and LR muscles along with MR retroequatorial myopexy.These findings support the consideration of this surgical technique as a safe and effective option for managing HES, providing both cosmetic and functional improvements to afflicted individuals.

What to expect from primary inferior oblique overaction after esotropia surgery : Primary inferior oblique overaction in esotropia.

BACKGROUND: Overelevation in adduction is common in patients with primary esotropia. This study evaluates the variation in ocular motility pattern in patients with primary inferior oblique (IO) muscle overaction after esotropia surgery. METHODS: The medical records of consecutive patients who underwent surgery for infantile, partially accommodative, and basic esotropia over eleven years and had at least one year of follow-up were reviewed. Patients with primary inferior oblique muscle overaction (IOOA) presented at baseline or during follow-up were selected and divided according to the first surgery performed concurrently with horizontal rectus surgery: without IO recession (NO-recess), with unilateral IO recession (UNIL-recess), and with bilateral IO recession (BIL-recess). The success (version normalisation or at least 2 points upgrade in severity scale [0-4] in the operated eye), recurrence rates, and the evolution of the non-operated IO muscles were evaluated. RESULTS: One hundred and ten patients were included - 53 NO-recess, 26 UNIL-recess, and 31 BIL-recess. Medial rectus muscle posterior fixation sutures surgery (PFS) was performed in 88.2% of patients for esotropia. A recession with graded anterior transposition was the weakening IO procedure. In the NO-recess group, 28 (52.8%) patients normalised their mild IOOA after PFS surgery alone. In the UNI-recess group, the success rate was 88.5%, with 16 (61.5%) patients showing worsened IO muscle of the fellow eye, which prompted additional surgery in 10 patients. In the BIL-recess group, all 31 patients improved the adduction pattern of the operated eye for an 80.6% success rate (6 improved marginally). CONCLUSION: Graded anterior transposition of the inferior oblique muscle effectively normalises versions. However, it's frequent for a contralateral overaction to become manifest after unilateral IO surgery.

Revisiting Posterior Fixation Sutures Surgery: Unveiling Novel Approaches for Primary Management of Diverse Esotropia Cases.

INTRODUCTION: Strabismus, specifically esotropia, presents a significant challenge in ophthalmic surgery, while several treatment options exist. This study aims to evaluate the results of posterior fixation sutures (PFS) on the medial rectus as a primary approach for some types of esotropia. METHODS: The medical records of consecutive patients who underwent surgery for esotropia over 11 years and had at least 1 year of follow-up were reviewed retrospectively. Patients were classified into one of three types of deviation: infantile (IE), partially accommodative (PAE) and basic (BE) esotropias. An alignment within 16 prism diopters (PD) of orthotropia was a successful outcome. RESULTS: A total of 404 patients were included: 67 IE, 180 PAE and 157 BE. Before surgery, a deviation greater than 30 PD was present in 88.1% and 80.1%, and a deviation greater than 50 PD was present in 66.5% and 52.9% of patients (near and distance, respectively). In the BE group, PFS was the baseline surgery in a smaller number of cases (75%) compared to the other two groups (versus 86.6% [IE] and 88.3% [PAE], p = 0.002). The need for an additional procedure was significantly higher in the infantile esotropia group (44.8% vs. 18.9% and 24.8%, p < 0.001). Final surgical success was achieved in 95.3% of all patients. Orthotropia was achieved in 19.4% (IE), 29.6% (PAE) and 25.5% (BE) of cases. CONCLUSION: PFS of the medial rectus without recession proved successful as a first-line procedure for esotropia in the subtypes of patients evaluated in this study.

Advancement of the muscle insertion toward the limbus as an alternative or adjunct to resection.

PURPOSE: To describe the surgical outcomes of patients that underwent strabismus surgery involving advancement of the muscular insertion line toward the limbus. METHODS: The medical records of patients who underwent rectus muscle advancement surgery between January 2009 and June 2019 and had at least 1 year of follow-up were reviewed retrospectively. Patients were classified into one of four types of deviation: (1) convergence insufficiency, without distance deviation; (2) small-angle distance esotropia, with diplopia; (3) residual strabismus after previous surgery; and (4) large-angle exotropia. The first group underwent 2 mm advancement of the medial rectus insertion bilaterally; the second, 2-3 mm advancement of the lateral rectus muscle bilaterally; the third, 2-3 mm advancement of the lateral or medial rectus muscle, with or without simultaneous resection; and the fourth, 3 mm advancement of the medial rectus with simultaneous resection, with or after maximum recession of the lateral rectus, bilaterally or unilaterally. RESULTS: A total of 25 patients were included (mean age, 28.76 ± 22.75 years): 4 with convergence insufficiency, 3 with small-angle distance esotropia and diplopia, 16 with residual strabismus, and 2 with large-angle exotropia. Mean postoperative follow-up was 4.76 ± 2.03 years. Surgery was successful in all patients at 1 year (residual deviation ≤10Δ), and all but 1 at 5 years; 1 patient required reoperation. CONCLUSIONS: Advancement of the muscle insertion proved successful as an alternative or adjunctive procedure to other forms of muscle tightening in the subtypes of patients evaluated in this study.

Macular Changes in a Mucopolysaccharidosis Type I Patient with Earlier Systemic Therapies.

PURPOSE: To describe retinal findings in a patient with mucopolysaccharidosis type I (MPS I) that underwent an early treatment with hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). Case Report. We describe a case of a 12-year-old female with a biochemical and genetic diagnosis of MPS I. She underwent HSCT and ERT on the first year of life. The visual acuity was 5/10 in both eyes and she had bilateral grade 2 corneal haze. Spectral domain optical coherence tomography (SD-OCT) revealed thickening of the external limiting membrane (ELM) at the fovea. In the parafoveal and perifoveal regions, SD-OCT displayed a loss of the interdigitation, ellipsoid, and myoid zones and of the ELM accompanied by progressive thinning of the outer nuclear layer. Fundus infrared imaging revealed a hyperreflective ring centred on the fovea and hyporeflective areas in temporal parafoveal regions in both eyes. En face OCT imaging revealed two hyperreflective rings on the outer retinal level. CONCLUSION: This patient developed macular changes with foveal deposition of hyperreflective material and parafoveal thinning, despite early systemic treatment. Systemic therapies can provide an increase in life expectancy and stabilize visual acuity and corneal clouding, although their effect on retinal degeneration is unknown.

Child with a mild phenotype of Incontinentia Pigmenti and inner retinal dysfunction.

PURPOSE: To describe a case of a child with mild phenotype of Incontinentia Pigmenti (IP), with changes in Spectral-Domain Optical Coherence Tomography (SD-OCT) and Optical Coherence Tomography Angiography (OCT-A) and an electronegative dark-adapted (DA) 3.0 electroretinogram (ERG), suggestive of inner retinal dysfunction. CASE REPORT: We described a 7-year-old female child with IP. Her best corrected acuity was 8/10 in the right eye and 6/10 in the left eye. Biomicroscopy, intraocular pressure and fundoscopy were normal. The electroretinography findings showed an electronegative DA 3.0 ERG with a normal a-wave but a b-wave that did not elevate above baseline. SD-OCT identified irregularities in the outer plexiform layer in both eyes, and OCT-A assessment revealed at the superficial capillary plexus, areas of decrease in the flow in parafoveal and perifoveal regions. CONCLUSION: Classically, IP affects the peripheral retina; however, vascular and structural changes in macula can occur as well. To our knowledge, we report the first electronegative electroretinogram in a patient with IP.

Adequacy of the Fogging Test in the Detection of Clinically Significant Hyperopia in School-Aged Children.

PURPOSE: To evaluate the efficacy of the "fogging test," performed with a +2 diopters (D) lens, in the exclusion of clinically significant hyperopia in school-aged children. METHODS: We studied 54 children between 5 and 11 years of age, with 10/10 best-corrected bilateral visual acuity (VA) without significant degree of correction. VA was assessed in each eye with a "bilateral" +2 D sphere over-refraction followed by cycloplegic retinoscopy. The capacity of the test to detect hyperopia of ≥+2 D and ≥+1.5 D was evaluated by examining the respective receiver operating characteristic (ROC) curves and sensitivity and specificity values for different cutoff values of VA. RESULTS: For the detection of hyperopia ≥+2 D, the area under the ROC curve (AUC) was 0.955 (p ≤ 0.001). The VA cutoff with best discriminative capacity was ≥5/10, with a sensitivity of 100%, specificity of 79%, positive predictive value (PPV) of 57%, and negative predictive value (NPV) of 100%. In respect of ≥+1.5 D hyperopia, the test capacity was lower (AUC = 0.832; p ≤ 0.001). The best VA cutoff was also of ≥5/10, with a PPV of 81% and a NPV of 85%. CONCLUSION: The accuracy of the test was high for the evaluation of ≥+2 D hyperopia but lower for ≥+1.5 D hyperopia. For the detection of ≥+2 D hyperopia, the VA cutoff of <5/10 may permit the exclusion of clinically significant hyperopia in selected children, without the need for cycloplegia. For the same cutoff, the PPV was low, meaning that in children with ≥5/10 VA cycloplegic refraction remains obligatory.

Fundoscopic Changes in Maroteaux-Lamy Syndrome.

PURPOSE: To describe a clinical case of mucopolysaccharidosis type VI (MPS VI), or Maroteaux-Lamy syndrome, with fundoscopic alterations that may correspond to scleral deposits of glycosaminoglycans. MATERIALS AND METHODS: Clinical case report. RESULTS: A 16-year-old girl with MPS VI was examined at the Ophthalmology Department for poor vision due to opacified corneas. Treatment consisted of bilateral penetrating keratoplasty. Retinographies and enhanced depth imaging optical coherence tomography (EDI-OCT) were performed after surgery, suggesting the presence of scleral glycosaminoglycan deposits. The patient evolved with stable corneal and fundoscopic findings. CONCLUSIONS: To our knowledge, this is the first case of MPS VI described in vivo with suspected deposits of glycosaminoglycans in the sclera. Fundoscopic alterations are not usually included in the ocular pathological spectrum of MPS VI. However, with improved control of systemic comorbidities, survival rates of these patients have increased, which in turn has made it possible to observe other changes besides the ones that were classically described. Despite being particularly challenging to manage, efforts should be made to maximizing the visual acuity of these patients, in order to provide them the best possible quality of life.

Factors Predictive of Success in Probing for Congenital Nasolacrimal Duct Obstruction.

PURPOSE: To report the success rate of children undergoing probing for congenital nasolacrimal duct obstruction (CNLDO) and the factors relating to the failure of the procedure. METHODS: This retrospective case series included 88 eyes of 62 patients, aged 1 to 138 months, who underwent probing between January 2008 and December 2014 in the Pediatric Ophthalmology Unit of Centro Hospitalar São João. The procedure was performed in the operating room under general anesthesia. Surgical success was defined as successful lacrimal irrigation in-traoperatively and resolution of epiphora at the follow-up visit 1 month after surgery. RESULTS: The overall success rate after first probing was 77.3% (68 of 88 eyes). No differences were found regarding age (P = .546), gender (P = .740), surgical experience (P = .611), or laterality (P = .328) between children who were cured and not cured. The surgical success rate decreased in children older than 4 years, although not to a statistically significant degree (P = .190). Surgical success after second probing was 85.7% (12 of 14 eyes), and the median interval between the two procedures was 3 months (range: 2 to 54 months). In 30% (7 of 20 eyes, 4 of 13 patients) of children with persistent obstruction, otorhinolaryngology evaluation evinced an adenoid hypertrophy requiring surgical correction. CONCLUSIONS: The success rate of nasolacrimal probing for CNLDO was not related to age, gender, laterality, or the surgeon's experience. Otorhinolaryngology evaluation is recommended for unresponsive patients. [J Pediatr Ophthalmol Strabismus. 2017;54(2):123-127.].

Combination of intravitreal ranibizumab and laser photocoagulation for aggressive posterior retinopathy of prematurity.

PURPOSE: To report on 2 cases of aggressive posterior retinopathy of prematurity (ROP) treated with intravitreal ranibizumab (Lucentis(®)) and laser photocoagulation. METHODS: Two premature females, born at 25 and 26 weeks' gestation with a birth weight of 530 and 550 g, respectively, with aggressive posterior ROP received combined treatment with laser photocoagulation and intravitreal ranibizumab (0.3 mg [30 µl]) to each eye. Structural outcomes were evaluated by indirect ophthalmoscopy and documented by retinography. RESULTS: An intravitreal injection was made at 34 weeks of postmenstrual age in the first case, followed by laser photocoagulation 1 week later. There was a partial regression of ROP with treatment. Five weeks later, neovascularization regrowth with bleeding in both eyes (intraretinal and subhyaloid) occurred and retreatment with combined therapy was performed. In the second case, single therapy with laser photocoagulation was made at 34 weeks of postmenstrual age. In spite of the confluent photocoagulation in the avascular area, progression to 4A ROP stage occurred 1 week later. Both eyes were retreated 1 week later with intravitreal ranibizumab and laser photocoagulation. Treatment resulted in ROP regression in both cases. There were no signs of systemic or ocular adverse side effects. CONCLUSION: The cases presented show that combination therapy of indirect laser photocoagulation and intravitreal ranibizumab can be effective in the management of aggressive posterior ROP. Further investigation on anti-VEGF safety in premature infants is necessary. Additional studies are needed to define the role of anti-VEGF in ROP treatment.

Development of periradicular lesions in normal and diabetic rats%Desenvolvimento de lesões perirradiculares em ratos normais e diabéticos

Evidências indicam que pacientes diabéticos são mais significativamente afetados por problemas de origem endodôntica. Este estudo avaliou radiográfica e histologicamente o desenvolvimento de inflamação perirradicular em ratos controle e diabéticos depois da indução de infecção pulpar. As polpas dos primeiros molares inferiores de ratos normais ou diabéticos foram expostas e deixadas em contato com a cavidade oral por 21 e 40 dias. Em seguida, os animais foram sacrificados, as mandíbulas removidas cirurgicamente, fixadas em formalina e depois radiografadas em posição padronizada. As imagens radiográficas das lesões perirradiculares foram escaneadas e as imagens computadorizadas avaliadas quanto à área total das lesões usando software específico. Espécimes representativos foram preparados também para análise histológica. A análise radiográfica revelou que os ratos diabéticos apresentaram lesões periradiculares significativamente maiores quando comparados com os ratos normais, independentemente do período experimental (p<0.05). O exame histológico de espécimes representativos revelou lesões perirradiculares mais extensas e com exsudato inlflamatório severo no grupo de ratos diabéticos quando comparado com o grupo controle. Os dados deste estudo indicam que ratos diabéticos podem ser mais propensos a desenvolver lesões perirradiculares extensas, possivelmente devido à diminuição da capacidade de defesa contra patógenos microbianos.

Development of periradicular lesions in normal and diabetic rats.

Evidence suggests that diabetic patients are more significantly affected by problems of endodontic origin. This study sought to radiographically and histologically examine the development of periradicular inflammation in control and in diabetic rats after induction of pulpal infection. The pulps of the mandibular first molars of normal and streptozotocin-induced diabetic rats were exposed and left in contact with their oral cavities for 21 and 40 days. Afterwards, the animals were sacrificed, the mandibles were surgically removed, fixed in formalin and then radiographed in a standardized position. The radiographic images of the periradicular lesions were scanned and computerized images were evaluated for the total area of the lesions using a specific software. Representative specimens were also prepared for histopathological analysis. Radiographic analysis revealed that diabetic rats presented significantly larger periradicular lesions when compared with control rats, regardless of the experimental period (p<0.05). Histopathological examination of representative specimens revealed larger periradicular lesions and more severe inflammatory exudate in the group of diabetic rats when compared with the control group. Data from the present study indicated that diabetic rats can be more prone to develop large periradicular lesions, possibly due to reduction in the defense ability against microbial pathogens.