Strabismus Surgery in Hemophilia: A Novel Surgical Technique and Guideline Recommendations.

Hemophilia is a serious X-linked inheritance coagulation factor deficiency. Clinically, prolonged bleeding or delayed clotting in any area of vascular disturbance is the main manifestation of all hemophilia. We presented a 23-year-old male with a history of left sensory esotropia since the age of three. The patient had not undergone any previous eye surgery and refused to wear glasses. Hematologic studies confirmed a diagnosis of hemophilia A. Upon ophthalmologic examination, the patient's visual acuity was 20/20 in the right eye and 20/120 in the left eye, with deep amblyopia. The patient exhibited left inferior oblique overaction and a V pattern. The ophthalmologic examination otherwise revealed no abnormalities. Preoperative correction of factor VIII was deemed necessary, and the recommended dose was administered to raise the factor VIII level to 52%. The patient underwent bilateral medial rectus recession, left lateral rectus plication, and left inferior oblique myectomy. A new technique utilizing viscodissection with subconjunctival injection of a viscoelastic solution was employed to minimize intraoperative bleeding, resulting in reduced bleeding compared to standard strabismus surgery. No unusual bleeding occurred during the procedure. No postoperative bleeding was observed. The patient was discharged on the fourth postoperative day, having achieved satisfactory cosmetic alignment in the primary position with no complications related to hemophilia. In conclusion, strabismus surgery can be performed safely in strabismic patients with hemophilia. Viscodissection is a helpful novel surgical technique to decrease the risk of bleeding during surgery, and we recommend using this technique in patients using anticoagulants. A multidisciplinary team approach and strict post-operative monitoring are essential in order to achieve optimal results.

Persistent epithelial defect after photorefractive keratectomy in a patient with autism.

Refractive surgery has been performed under general anesthesia on pediatric and neurobehaviorally challenged adults without reported loss of vision or serious complications. Persistent epithelial defect (PED) is a rare complication of photorefractive keratectomy (PRK) in the general refractive surgery population. We report a case of PED following PRK under general anesthesia for high myopia in a man with autism and ocular history of juvenile open-angle glaucoma and dry eye syndrome.

Novel finding of atrophic extraocular muscles in Loeys-Dietz syndrome: a case report and review of the literature.

A 19-year-old man with Loeys-Dietz syndrome and right exotropic Duane syndrome after bilateral lateral rectus recessions at age 22 months presented with recurrent progressive exotropia 17 years after his initial surgery. Surgical correction was aborted intraoperatively when extreme atrophy of the right medial rectus, lateral rectus, and superior rectus muscles was observed, later corroborated by orbital magnetic resonance imaging.

Corneal indices following photorefractive keratectomy in children at least 5 years after surgery.

PURPOSE: To evaluate long-term corneal outcomes in pediatric patients who underwent photorefractive keratotomy (PRK) for the treatment of refractive amblyopia. METHODS: In this prospective interventional case series, children with refractive amblyopia underwent PRK between January 1, 2007, and December 31, 2011, at Texas Children's Hospital's Department of Ophthalmology, a single tertiary eye center, and were followed for at least 5 years after surgery. Main outcome measures were 5+ years postoperative indices of corneal thickness, keratometry, degree of corneal haze, and presence or absence of keratectasia. RESULTS: Twelve eyes of 8 subjects aged 3-9 years who underwent PRK and were followed for at least 5 years were included. The mean PRK treatment dose was 8.46 D for the myopic cohort and 4.49 D for the hyperopic cohort, which removed an average of 72 µm of corneal stromal tissue in addition to the 50 µm of corneal epithelium that was removed prior to laser ablation. The mean corneal thickness was 563 µm preoperatively, which decreased to 441 µm immediately following the PRK. The mean corneal thickness 5+ years after PRK was stable, at 498 µm, because of epithelial regrowth. None of the subjects developed visually significant corneal haze or topographic evidence of keratectasia. CONCLUSIONS: In this study cohort, there were no topographic signs of keratectasia or corneal haze in children treated with PRK for high refractive error 5 years or more after surgery.

A Randomized Trial Comparing Bilateral Lateral Rectus Recession versus Unilateral Recess and Resect for Basic-Type Intermittent Exotropia.

PURPOSE: To compare long-term outcomes after bilateral lateral rectus recession (BLRc) or unilateral lateral rectus recession combined with medial rectus resection in the same eye (R&R) for primary treatment of childhood intermittent exotropia (IXT). DESIGN: Multicenter, randomized clinical trial. PARTICIPANTS: One hundred ninety-seven children 3 to younger than 11 years of age with basic-type IXT, a largest deviation by prism and alternate cover test at any distance of 15 to 40 prism diopters (PD), and near stereoacuity of at least 400 seconds of arc. METHODS: Random assignment to BLRc or R&R and masked examinations conducted every 6 months after surgery for 3 years. MAIN OUTCOME MEASURES: Proportion of participants meeting suboptimal surgical outcome by 3 years, defined as: (1) exotropia of 10 PD or more at distance or near using simultaneous prism and cover test (SPCT); or (2) constant esotropia of 6 PD or more at distance or near using SPCT; (3) loss of 2 octaves or more of stereoacuity from baseline, at any masked examination; or (4) reoperation without meeting any of these criteria. RESULTS: Cumulative probability of suboptimal surgical outcome by 3 years was 46% (43/101) in the BLRc group versus 37% (33/96) in the R&R group (treatment group difference of BLRc minus R&R, 9%; 95% confidence interval [CI], -6% to 23%). Reoperation by 3 years occurred in 9 participants (10%) in the BLRc group (8 of 9 met suboptimal surgical outcome criteria) and in 4 participants (5%) in the R&R group (3 of 4 met suboptimal surgical outcome criteria; treatment group difference of BLRc minus R&R, 5%; 95% CI, -2% to 13%). Among participants completing the 3-year visit, 29% (25 of 86) in the BLRc group and 17% (13 of 77) in the R&R group underwent reoperation or met suboptimal surgical outcome criteria at 3 years (treatment group difference of BLRc minus R&R, 12%; 95% CI, -1% to 25%). CONCLUSIONS: We did not find a statistically significant difference in suboptimal surgical outcome by 3 years between children with IXT treated with BLRc compared with those treated with R&R. Based on these findings, we are unable to recommend one surgical approach over the other for childhood IXT.

An exome sequencing study of Moebius syndrome including atypical cases reveals an individual with CFEOM3A and a TUBB3 mutation.

Moebius syndrome is characterized by congenital unilateral or bilateral facial and abducens nerve palsies (sixth and seventh cranial nerves) causing facial weakness, feeding difficulties, and restricted ocular movements. Abnormalities of the chest wall such as Poland anomaly and variable limb defects are frequently associated with this syndrome. Most cases are isolated; however, rare families with autosomal dominant transmission with incomplete penetrance and variable expressivity have been described. The genetic basis of this condition remains unknown. In a cohort study of nine individuals suspected to have Moebius syndrome (six typical, three atypical), we performed whole-exome sequencing to try to identify a commonly mutated gene. Although no such gene was identified and we did not find mutations in PLXND1 and REV3L, we found a de novo heterozygous mutation, p.E410K, in the gene encoding tubulin beta 3 class III (TUBB3), in an individual with atypical Moebius syndrome. This individual was diagnosed with near-complete ophthalmoplegia, agenesis of the corpus callosum, and absence of the septum pellucidum. No substantial limb abnormalities were noted. Mutations in TUBB3 have been associated with complex cortical dysplasia and other brain malformations and congenital fibrosis of extraocular muscles type 3A (CFEOM3A). Our report highlights the overlap of genetic etiology and clinical differences between CFEOM and Moebius syndrome and describes our approach to identifying candidate genes for typical and atypical Moebius syndrome.

Pediatric refractive surgery: corneal and intraocular techniques and beyond.

Refractive surgery has now been used successfully to treat severe anisometropia and isoametropia associated with amblyopia in children who cannot wear standard spectacles or contact lenses. Extraocular techniques include photorefractive keratectomy, laser-assisted subepithelial keratomileusis, and laser-assisted in situ keratomileusis. Intraocular techniques include refractive lensectomy and phakic intraocular lenses and are still being investigated in children for refractive errors outside the treatment dose capabilities of the excimer laser. This workshop discusses the various techniques, how and when to use each, and their risks and benefits. Newer techniques currently being used in adults that may someday be used in children are also introduced.

Outcomes of integrating genetics in management of patients with retinoblastoma.

OBJECTIVE: To present the outcome of a comprehensive team approach to provide genetic evaluation and testing for a large cohort of children diagnosed with retinoblastoma. METHODS: The multidisciplinary team included pediatric oncologists, an ophthalmologist, an ophthalmic pathologist, a geneticist, and genetic counselors. Retrospective data from 8 years included 90 initial evaluations, of which 81 probands were diagnosed with retinoblastoma (34 bilateral and 47 unilateral) and 9 were evaluated because of a positive family history. RESULTS: Genetic testing was accomplished equivalently in bilateral and unilateral cases in 51 of 81 patients (63%). In 5 of 30 patients (17%), with unilateral disease an RB1 mutation was identified in peripheral blood samples. In another 7 of 30 patients (23%), mutation analysis confirmed the occurrence of sporadic retinoblastoma. Overall, genetic testing of 48 at-risk family members from 21 families revealed 6 individuals positive and 42 negative for the familial mutation. CONCLUSIONS: Our study emphasizes that genetics can be incorporated into the management plan of all retinoblastoma patients using a team approach to ensure timely evaluations and appropriate counseling. Genetic evaluations improved risk prediction for patients and family members as well as prevented overutilization of clinical screening tests, which had potential morbidity for relatives documented to not carry an RB1 mutation.

A data-driven approach to the management of accommodative esotropia.

PURPOSE: To determine if key findings on initial examination are predictive of ability to achieve and maintain functional alignment (residual esotropia of less than 8 prism diopters) with single-vision spectacles, bifocals, or surgery in children with accommodative esotropia. DESIGN: Retrospective, observational case series. METHODS: Institutional review of 68 consecutive accommodative esotropia patients. Exclusion criteria included previous spectacles use, other ocular pathologic features or surgery, or follow-up of less than 2 years. The main outcome measure was the ability to achieve functional alignment. RESULTS: Mean age at the time of single-vision spectacle prescription (P = .02), mean cycloplegic refractive error (P = .016), amblyopia (P = .02), uncorrected near deviation (P < .001), and uncorrected distance deviation (P < .001) differed significantly between children who achieved functional alignment with single-vision lenses and those who did not. The most parsimonious prediction model revealed that presence of amblyopia (P = .113; odds ratio [OR], 0.138; 95% confidence interval [CI], 0.012 to 1.59), uncorrected distance deviation (P = .004; OR, 1.156; 95% CI, 1.049 to 1.274), mean cycloplegic refractive error (P = .008; OR, 0.300; 95% CI, 0.123 to 0.732), and age at time of single-vision lens prescription (P = .007; OR, 0.259; 95% CI, 0.097 to 0.690) were the best predictors of ability to achieve orthotropia with single-vision spectacles with a sensitivity of 94% (95% CI, 71% to 99%) and specificity of 91% (95% CI, 75% to 98%). CONCLUSIONS: Significant differences exist in the clinical presentations of children who achieve functional orthotropia with single-vision spectacles and those who require bifocals or surgery. An evidence-based algorithm may help practitioners predict which intervention is most likely to benefit an individual child.

Retinoblastoma: review of current management.

The most common ocular cancer in children is retinoblastoma. It affects approximately 300 children in the U.S. every year. It can affect one or both eyes and the disease can be inherited. Altered discoloration of the pupil and strabismus are the usual symptoms that lead to medical attention. Subsequent appropriate diagnostic studies and care provided by a multidisciplinary team, including an ophthalmologist, a pediatric oncologist, a radiation oncologist, and a geneticist, among others, often result in optimal short-term and long-term care. The best initial and subsequent treatments are based on whether the child has unilateral or bilateral disease, the stage of the disease, and the age of the child. Enucleation, chemotherapy, and various forms of radiation therapy along with local ophthalmic therapies can be used in the treatment of retinoblastoma. Cure rates are high in children when the tumor is confined to the eye and has not spread systemically or into the orbit or brain. Children with the heritable form of retinoblastoma are at high risk for developing subsequent malignancies, most commonly sarcomas. This risk is greater for those children with the heritable form of the disease who were exposed to ionizing radiation at age <1 year. Exciting discoveries using animal models are providing new insights into the development of this disease and opening new avenues for targeted therapies that may lead to high cure rates with minimal toxicities.

Ultrasound biomicroscopy (UBM) characteristics of scleral tunnels created with suture needles commonly used during strabismus surgery.

PURPOSE: To enhance the safety and efficacy of surgical treatment of strabismus, we sought to measure and determine the ultrasound biomicroscopy (UBM) profile of scleral tunnels created with needles commonly used during strabismus surgery, to determine which needles are less likely to create the complication of scleral perforation. METHODS: Adult cadaver eyes were secured in a styrofoam head. Intraocular pressure was maintained between 15 and 21 mm Hg. Then S14, S24, S28 and TG100 needles were used to create scleral tunnels simulating those created during routine strabismus surgery. Ten scleral tunnels were created with each needle type at 3 different sites on the globe, for a total of 120 passes. The thickness of the sclera and the maximum depth and length of each scleral tunnel were measured using UBM. RESULTS: The mean tunnel depth below the scleral surface (+/- SD) was 0.43 +/-0.11 mm, 0.37 +/-0.09, 0.40 +/-0.08 and 0.34 +/-0.07 mm, for the S14, S24, S28 and TG100 needles, respectively (P=0.002, One way ANOVA). For both the S14 and S28 needles, there was a "statistically significant" P 0.05) linear trend of an increase in the depth of the pass as the length of the pass increased (P=0.01 for the S14 and P=0.02 for the S28 {Pearson Correlation 2 tailed test}). A similar trend was found with the S24 needle but the trend was not "statistically significant" (P=0.35). No such trend was found with the TG100 needle. CONCLUSIONS: Needle design had a definite impact on the characteristics of scleral tunnels created to simulate those made during strabismus surgery and may influence needle selection by the surgeon for different or various surgical circumstances, but the differences were not such as to predicate for or against the general use of any of these four needles for strabismus surgery.

Refractive surgery in children: is it ready for prime time?

INTRODUCTION: Potential indications for excimer laser procedures in children include accommodative esotropia, bilateral high ametropia, and severe anisometropia. Treatment of these conditions has traditionally included spectacle or contact lens use. This treatment, however, is often ineffective in children with severe anisometropia or bilateral ametropia, especially those with neuropsychological disorders. Refractive surgery may be a viable treatment option for these conditions. METHODS: The visual and refractive results from our studies on photorefractive keratectomy for pediatric anisometropic amblyopia are discussed. Also, I will present a review of the world literature on excimer laser procedures for accommodative esotropia, pediatric high anisometropia, and pediatric bilateral high ametropia. RESULTS: In our study, at 36 months follow-up, 7 of 9 patients who were able to perform psychophysical acuity testing preoperatively had improvement of two or more lines of uncorrected visual acuity and 6 of 9 had improvement of two or more lines of best corrected visual acuity. Fifty percent of the myopic patients and 100% of the hyperopic patients were within 2 D of refractive target at the 36-month follow-up visit. Refractive error stability has been good and corneal haze has been minimal. CONCLUSIONS: Refractive surgery in children to reduce amblyopiogenic levels of refractive error is proving to be relatively stable. Best corrected and uncorrected visual acuity has also been shown to improve following the excimer laser procedures. Refractive surgery also appears to be effective for pure accommodative esotropia. Randomized clinical trials are needed to fully establish safety and efficacy. Other refractive procedures, such as clear lens extraction and phakic intraocular lenses, may also prove to be valid treatment options for these conditions in the future.

Long-term outcomes of photorefractive keratectomy for anisometropic amblyopia in children.

PURPOSE: To evaluate the long-term visual acuity (VA) and refractive error responses to excimer laser photorefractive keratectomy (PRK) for treatment of anisometropic amblyopia in children. DESIGN: Prospective interventional case-control study. PARTICIPANTS: Eleven children, 2 to 11 years old, with anisometropic amblyopia who were noncompliant with conventional therapy with glasses or contact lenses and occlusion therapy were treated with PRK. A cohort derived retrospectively of 13 compliant and 10 noncompliant children with refractive errors similar to those of the PRK group who were treated with traditional anisometropic amblyopia therapy served as control groups. INTERVENTION: Photorefractive keratectomy for the eye with the higher refractive error. MAIN OUTCOME MEASURES: (1) Refractive error reduction and stability in the treated eye, (2) cycloplegic refraction, (3) VA, (4) stereoacuity, and (5) corneal haze up to 3 years after PRK. Compliant and noncompliant children with anisometropia amblyopia were analyzed as controls for refractive error and VA. RESULTS: Preoperative refractive errors were -13.70 diopters (D) (+/-3.77) for the myopic group and +4.75 D (+/-0.50) for the hyperopic group. Mean postoperative refractive errors at last follow-up (mean, 31 months) were -3.55 D (+/-2.2.5) and +1.41 D (+/-1.07) for the myopic and hyperopic groups, respectively. At last follow-up, cycloplegic refractions in 4 (50%) of 8 myopes and all hyperopes (100%) were within 3 D of that of the fellow eye. Five (63%) of 8 myopic children achieved a refraction within 2 D of the target refraction. Two (67%) of 3 hyperopic patients maintained their refractions within 2 D of the target. Refractive regressions (from 1 year after surgery to last follow-up) were 0.50+/-1.41 D (myopes) and 0.60+/-0.57 D (hyperopes). Seven children (77%) were able to perform psychophysical VA testing preoperatively and postoperatively. Five (71%) of the 7 children had uncorrected VA improvement of at least 2 lines, and 4 (57%) of 7 had best spectacle-corrected VA improvement of at least 2 lines, with 1 improving 7 lines. Five (55%) of 9 children had improvement of their stereoacuity at last follow-up. Subepithelial corneal haze remained negligible. The mean final VA of the PRK group was significantly better than that of the noncompliant control group (P = 0.003). The mean final refractive error for both myopic and hyperopic groups was also significantly better that that of the control groups (P = 0.007 and P<0.0001, respectively). CONCLUSIONS: Photorefractive keratectomy for severe anisometropic amblyopia in children resulted in long-term stable reduction in refractive error and improvement in VA and stereopsis, with negligible persistent corneal haze.

Reasons for delay of surgical intervention in adult strabismus.

BACKGROUND: It has been our impression that adult patients with strabismus frequently delay surgical intervention. OBJECTIVES: To determine the length of time adult patients waited before undergoing strabismus surgery and to determine the reasons why these delays occurred. DESIGN, SETTING, AND PARTICIPANTS: Prospective survey of consecutive patients who delayed strabismus surgery for more than 1 year. INTERVENTION: Preoperative survey. MAIN OUTCOME MEASURES: The primary outcome measure was time between the onset of strabismus and surgery and the reason why surgery had not been previously sought. RESULTS: The mean age among 128 study participants was 45.7 years (age range, 18-86 years). The mean time between the onset of current strabismus and surgery was 19.9 years (range, 1-72 years). The major reasons for delay in seeking surgical treatment included the following: surgery was never offered by eye care specialist (35 patients [27%]), surgery was offered but declined by the patient (29 patients [23%]), the patient had received prior satisfactory nonsurgical care (17 patients [13%]), the patient had never sought care (14 patients [11%]), the patient had a previous poor surgical experience (8 patients [6%]), and the patient had been told by their eye specialist that nothing could be done or that surgery could make them worse (8 patients [6%]). CONCLUSIONS: Strabismus surgery is often delayed for many years in adult patients who could potentially benefit from it. Almost half of such delays could be avoided by better education of the lay public and the medical community.

Successful implementation of a protocol for photorefractive keratectomy in children requiring anesthesia.

PURPOSE: To describe a protocol for treating children with photorefractive keratectomy (PRK) under general anesthesia and to review intraoperative and postoperative complications. SETTING: Institutional academic practice. METHODS: Nine patients between 3 years and 9 years of age were treated with PRK under general anesthesia for anisometropia with unilateral high myopia or high hyperopia and amblyopia of the affected eye. Induction of anesthesia and the surgical procedure were carried out in separate rooms. The laser beam was centered on the entrance pupil, and eye position was monitored throughout the procedure. Specific precautions were taken before and during the procedure to prevent unwanted effects of inhalational anesthetic agents on laser performance. RESULTS: All children did well, with no anesthesia-related or treatment-related complications. CONCLUSIONS: Our protocol for PRK under general anesthesia was effective and efficient in children who were unable to cooperate for the procedure using local anesthesia. It can be adapted for laser in situ keratomileusis and other refractive surgical procedures in children and uncooperative adults.

Recurrent conjunctivitis as a presentation of munchausen syndrome by proxy.

PURPOSE: To report a case of Munchausen syndrome by proxy, which manifested as recurrent bilateral keratoconjunctivitis in an infant. DESIGN: Interventional case report. INTERVENTION: The patient underwent numerous diagnostic studies, including two endoscopies, skin biopsy, conjunctival pH measurement, and a skeletal survey. She underwent daily eye examinations until the corneal and conjunctival epithelial defects resolved. MAIN OUTCOME MEASURE: Resolution of cutaneous, mucosal, corneal, and conjunctival epithelial defects. RESULTS: A punch biopsy of the right postauricular area was performed, and pathology subsequently determined that the findings seemed to be the result of an exogenous injury. The conjunctival pH was 8.0, consistent with exposure to an exogenous, caustic agent. The acute ocular lesions resolved. CONCLUSIONS: Munchausen syndrome by proxy can be seen with ophthalmic manifestations and should be considered in the differential diagnosis when ocular abnormalities cannot be explained after a thorough and methodical evaluation.