Block-building performance test using a virtual reality head-mounted display in children with intermittent exotropia.

PURPOSE: To determine whether childhood intermittent exotropia (IXT) affects distance divergence and performance in block-building tasks within a virtual reality (VR) environment. METHODS: Thirty-nine children with IXT, aged 6-12 years, who underwent muscle surgery and 37 normal controls were enrolled. Children were instructed to watch the target moving away and perform a block-building task while fitted with a VR head-mounted display equipped with eye- and hand-movement tracking systems. The change in inter-ocular distance with binocular distance viewing, time to stack five cube blocks of different sizes in order, and distance disparities between the largest and farthest cubes were assessed. All children were evaluated at baseline and 3-month time points. RESULTS: The patients with IXT exhibited a larger distance divergence than did controls (p = 0.024), which was associated with greater distance angle of deviation and poorer distance control (r = 0.350, p = 0.001 and r = 0.349, p = 0.004). At baseline, the patients with IXT showed larger distance disparities in the block-building task than did controls in terms of the horizontal, vertical, and 3-dimensional (3-D) measurements (all ps < 0.050). Larger horizontal disparity was associated with greater distance angle of deviation (r = 0.383, p = 0.037). Three months after surgery, the horizontal and 3-D disparities in the patients with IXT improved significantly and were not comparably different compared with controls. CONCLUSIONS: These preliminary findings suggest that VR-based block-building task may be useful in testing possible deficits in visuo-motor skills associated with childhood IXT.

Sternocleidomastoid muscle asymmetry in unilateral congenital superior oblique palsy.

BACKGROUND: To determine whether there is an asymmetry in bilateral sternocleidomastoid muscle (SCM) thickness in patients with unilateral congenital superior oblique palsy (SOP) and its association with surgical results. METHODS: The medical records of 186 patients with head tilt secondary to unilateral SOP, who were evaluated for the status of the SCM with neck ultrasound or magnetic resonance imaging, were reviewed. The SCM asymmetry index was calculated as a bilateral difference in the maximal muscle thickness divided by each tilted-side SCM thickness. The presence of SCM asymmetry, defined as an index of >10%, and its relationship to residual torticollis ≥5° after SOP surgery were assessed. RESULTS: Of 186 patients with a median age of 1.2 years, SCM asymmetry was present in 102 (54.8%) patients (6.8 ± 1.9 mm for the SOP side vs. 6.6 ± 2.1 mm for the tilted side). The SCM asymmetry did not differ according to age, amount of head tilt or hypertropia. In the patients with SCM asymmetry, more patients (87.3%) underwent physiotherapy than those without asymmetry (61.9%) (P = 0.021). In 99 patients who underwent surgery for SOP, the resolution of torticollis was not significantly different between patients with and without SCM asymmetry (87.2% vs. 76.9%, P = 0.184). CONCLUSIONS: Nearly half of the patients with congenital SOP had SCM thickness asymmetry that was already determined at a young age. However, the surgical results did not differ significantly with respect to SCM asymmetry when physiotherapy was combined. Thus, SOP surgery can be considered despite preoperative SCM asymmetry.

Anomalous extraocular muscles in Crouzon syndrome with V-pattern exotropia.

Strabismus associated with Crouzon syndrome is common and often complex. V-pattern strabismus is most commonly reported in this condition and is mainly thought to be due to an excyclorotation of the orbits and rectus muscle pulleys. We report two cases of children with Crouzon syndrome and V-pattern exotropia who had rectus muscle heterotopy on orbital imaging and were also found intraoperatively to have anomalous extraocular muscles. At the time of surgery, bifid insertion, misdirection, and fibrosis of extraocular muscles were noted. This highlights the various causes of V-pattern strabismus associated with Crouzon syndrome, including dysmorphic orbits and extraocular muscle anomalies.

Pupil-Involving Oculomotor Nerve Palsy Following Tonsillectomy and Adenoidectomy.

Ocular complications of adenotonsillectomy are rare. The authors describe a 6-year-old boy who developed mydrasis and limitations of supraduction and infraduction after adenotonsillectomy. This was attributed to the hemorrhagic compression of the nerve in the cavernous sinus. This is the first report of pupil-involving oculomotor nerve palsy following adenotonsillectomy. [J Pediatr Ophthalmol Strabismus. 2019;56:e76-e78.].

Use of the Bispectral Index to Predict Eye Position of Children during General Anesthesia.

PURPOSE: To assess the relationship between eye position and anesthesia depth using the bispectral index (BIS) value, a parameter derived from electroencephalography data. METHODS: We investigated the relationship between BIS value and eye position in 32 children who underwent surgery for epiblepharon under general anesthesia. BIS values were recorded continuously throughout the procedure (from induction to awakening). Eye positions were video-recorded and analyzed after surgery. The vertical position of each eye was scored according to its height in relation to the medial canthus. An eye position in which the upper eyelid covered one-third of the cornea was defined as a significant ocular elevation. RESULTS: The BIS value correlated inversely with the end-tidal concentration of each anesthetic agent, whereas it correlated positively with the eye elevation score (eye position = 0.014 × BIS + 0.699, p = 0.011). The mean eye position score was significantly greater in patients whose BIS values were over 65. Eleven patients (34.4%) had significant ocular elevation; their mean concurrent BIS value was 61.6. Two of these patients had elevation during surgery and 9 had elevation during emergence from anesthesia. CONCLUSIONS: We found that high BIS values were correlated with low levels of anesthetic concentration and high eye position, suggesting that BIS monitoring may be useful for predicting eye position during anesthesia. Particular attention must be given to eye position during ophthalmic surgery. Anesthesia depth can be maintained by assuring that the BIS value remains below 65.

Ophthalmic findings in children with nonsyndromic craniosynostosis treated by expansion cranioplasty.

The ocular and systemic abnormalities of nonsyndromic craniosynostosis are often considered to be less severe than those of syndromic craniosynostosis and are less well described. The purpose of this article was to describe the frequency and nature of ophthalmic abnormalities in children treated for nonsyndromic craniosynostosis by expansion cranioplasty. A retrospective review identified 88 consecutive children with nonsyndromic craniosynostosis who underwent expansion cranioplasty with distraction osteogenesis. Assessment of presence and type of strabismus, refractive error, and amblyopia before and 6 months after surgery was recorded. Children with a mean age of 24.4 months were treated for nonsyndromic craniosynostosis (27 with coronal and 61 with sagittal and/or lambdoid). One-fourth of the patients had a fixation preference. Significant refractive errors were found in 45 (51%) of the 88 patients: hyperopia in 27%, myopia in 5%, and astigmatism in 35%. Anisometropia was present in 20%. Of the 85 patients who completed orthoptic examination, 48 (56%) had strabismus: exodeviation in 26%, esodeviation in 14%, and vertical deviation in 5%. Fourteen patients (16%) had abnormal head posture. Significant refractive error and strabismus were more likely to occur in cases with coronal synostosis. The procedures used for cranial vault expansion improved the abnormal head posture but did not affect the refractive error or ocular misalignment. Of children with nonsyndromic craniosynostosis who need neurosurgical correction, more than half were found to have significant refractive error and strabismus. Our findings support the importance of ophthalmic evaluation in these children.

Pirfenidone attenuates the IL-1ß-induced hyaluronic acid increase in orbital fibroblasts from patients with thyroid-associated ophthalmopathy.

PURPOSE: This study aimed to investigate the effect of pirfenidone on the IL-1ß-induced hyaluronic acid (HA) increase in orbital fibroblasts from patients with thyroid-associated ophthalmopathy (TAO). METHODS: Primary cultured orbital fibroblasts were obtained from patients with TAO, and the excreted levels of HA from IL-1ß-treated cells with or without pirfenidone were measured. The effect of pirfenidone on IL-1ß-induced hyaluronic acid synthase (HAS) expression was evaluated. The relevance of the mitogen-activated protein kinase (MAPK)-mediated signaling pathway in IL-1ß-induced HAS expression was assessed using specific inhibitors to p38, extracellular signal-regulated kinase (ERK), or c-Jun N-terminal kinase (JNK). The phosphorylation level of each MAPK in IL-1ß-treated cells with or without pirfenidone and the level of AP-1 DNA binding were measured. The inhibitory potency of pirfenidone on HA production was evaluated using dexamethasone as a reference agent. RESULTS: Pirfenidone strongly attenuated the IL-1ß-induced HA release in a dose-dependent manner. The IL-1ß-induced HAS expression was decreased significantly following cotreatment with pirfenidone at the mRNA and protein levels. The production of mRNAs was halted by cotreatment with inhibitors of ERK and p38, but not by inhibitors of JNK. The IL-1ß-induced ERK and p38 phosphorylation, and AP-1 DNA binding were attenuated in the presence of pirfenidone. Pirfenidone showed greater potency than dexamethasone in inhibiting increases in IL-1ß-induced HA. CONCLUSIONS: Pirfenidone attenuates the IL-1ß-induced HA production in orbital fibroblasts from patients with TAO, at least in part, through suppression of the MAPK-mediated HAS expression. These results support the potential use of pirfenidone for treatment of patients with TAO.

Abducens Nerve Palsy Following Expansion Cranioplasty with Distraction Osteogenesis.

Childhood abducens nerve palsy can occur as a result of trauma, tumour, vasculopathic disease, elevated intracranial pressure, infection, inflammation, and congenital or idiopathic causes. The authors present two cases of unilateral abduction deficit secondary to a recent trans-sutural distraction osteogenesis (TSuDO) operation for craniosynostosis. After distractor removal, the problem resolved spontaneously over 2-4 months in both cases. This is a first reported case of sixth nerve palsy as a complication of TSuDO operation.

Photophobia measurement in intermittent exotropia using the contrast sensitivity test.

BACKGROUND: To assess the presence and extent of photophobia in children with intermittent exotropia (X[T]) using the contrast sensitivity test. METHODS: Fifty-eight children with X(T) and 34 normal controls were studied with the functional acuity contrast test. Each participant viewed the stimuli of contrast monocularly and binocularly under photopic and mesopic conditions, performed with and without glare. Photophobia was defined as a reduction of contrast sensitivity caused by glare light. We compared the photophobia of children with X(T) to that of normal controls, and to the photophobia 3 months after muscle surgery. RESULTS: With stimuli of glare, the contrast sensitivity of children with X(T) was suppressed at intermediate spatial frequencies under mesopic condition (p = 0.006 for 6 cycles per degree [cpd], p = 0.027 for 12 cpd), whereas that of normal controls showed no difference. It occurred when X(T) patients viewed targets binocularly, and significantly improved after strabismus surgery (p = 0.003 at 6 cpd). The measured photophobia of X(T) was strongly correlated to the photophobia symptoms reported by parents (p = 0.002). CONCLUSIONS: The mesopic contrast sensitivity with glare can represent the photophobia of children with X(T). Contrast sensitivity may be a useful measure for monitoring symptoms related to X(T).

Parent-reported symptoms of attention deficit hyperactivity disorder in children with intermittent exotropia before and after strabismus surgery.

PURPOSE: To investigate the symptoms of attention deficit hyperactivity disorder (ADHD) as reported by parents in children with intermittent exotropia [X(T)] and to determine whether strabismus surgery for X(T) affects ADHD symptoms. MATERIALS AND METHODS: Fifty-one consecutive children undergoing muscle surgery for X(T) were prospectively recruited. One parent of each child completed the ADHD rating scale IV (ADHD RS-IV) assessment consecutively before and one year after surgery. Patients whose preoperative scores were above the cut-off point, the 90th percentile based on a Korean sample, were regarded as demonstrating the ADHD trait. The impact of muscle surgery on ADHD symptoms was assessed by comparing the preoperative scores with the post-operative scores. RESULTS: Eight (15.7%) of the 51 patients demonstrated the ADHD trait. ADHD RS-IV scores following strabismus surgery significantly decreased in patients with the ADHD trait (p=0.014), while they did not differ in patients without the ADHD trait. Seven (87.5%) of the 8 patients with the ADHD trait showed improvement in their ADHD RS-IV scores after surgery. There was no difference in surgical success rates between X(T) patients with and without the ADHD trait. CONCLUSION: The ADHD trait was relatively common in children with X(T), and the parent-reported symptoms of the children with the ADHD trait improved after strabismus surgery. These results suggest that childhood X(T) may be one contributing factor to ADHD- related symptoms.

Changes in the interpupillary distance following general anesthesia in children with intermittent exotropia: a predictor of surgical outcomes.

PURPOSE: To determine whether changes in ocular alignment following general anesthesia, as measured by interpupillary distance, can be used as a predictor for surgical outcomes in children with intermittent exotropia. METHODS: The authors obtained digital photographs of 40 children with exotropia and 20 children with epiblepharon (control group) before and immediately after induction of general anesthesia in the primary supine position. Differences in the anatomic interpupillary distance (aIPD) for each patient were measured and compared with the preoperative angles of deviation. They were also correlated with surgical outcomes on the first day and 1 year after surgery. RESULTS: All 60 patients demonstrated an increase in aIPD following general anesthesia. The mean change in aIPD in patients with exotropia was similar to that in patients with epiblepharon (3.78% ± 2.53% and 3.15% ± 1.05%, respectively). In patients with exotropia, there was a significant positive linear correlation between the preoperative distance deviation (P) and the change in eye position (A): A = 0.362 P - 4.488, r(2) = 0.476 (P < .001). Five (71%) of seven patients whose changes were outside the 80% confidence interval for expected values had poor surgical outcomes on the first postoperative day and four had unsatisfactory results 1 year after surgery. CONCLUSIONS: Changes in aIPD following general anesthesia were strongly correlated with preoperative angle of deviation. Therefore, they may be a useful predictor of surgical outcomes in children with exotropia.

Transient increase of higher-order aberrations after lateral rectus recession in children.

The changes of higher-order aberrations (HOAs) after bilateral lateral rectus muscle recession were evaluated. Forty eyes of 20 children were enrolled and their wavefront information was assessed until postoperative 3 months. Even though the root mean square (RMS) of total aberration was not changed, the RMS of HOA was transiently increased at postoperative 1 week and returned to baseline level after 1 month. Among individual Zernike coefficient, secondary astigmatism, quadrafoil, secondary coma, secondary trefoil, and pentafoil showed similar tendency with the RMS of HOA. However, coma, trefoil, and spherical aberration were not changed. Regarding recession amount, it did not correlate with any Zernike coefficient. In summary, our data imply that the HOAs are transiently increased after lateral rectus recession surgery. These results are in collusion with previous reports that strabismus surgery induced transient corneal astigmatism.

Practical aspects and efficacy of intraoperative adjustment in concomitant horizontal strabismus surgery.

PURPOSE: To analyze the practical aspects and advantages of one-stage adjustable surgery under topical anesthesia in concomitant horizontal strabismus. METHODS: A retrospective review of 363 patients was completed to assess (1) the frequency of need to perform adjustment, (2) the amount and pattern of adjustment, and (3) the final alignment at least 6 months after surgery. Intraoperative adjustment was performed strictly toward the aim of orthophoria or slight overcorrection (heterophoria < 6 prism diopters [PD]) while avoiding diplopia. RESULTS: Of the 363 patients, 261 (72%) required intraoperative adjustment. Of these, 85% of exotropes underwent a decreased amount of surgery compared with the standard amount, whereas 58% of esotropes underwent an increased amount of surgery. Forty-two patients underwent a one-muscle surgery instead of the scheduled two-muscle surgery, and all had less than 35 PD preoperative angle of deviation. Success rates were 83% in all patients with one-stage adjustable sutures and 87% in patients who underwent adjustment. In 42 patients with one-muscle surgery instead of two-muscle surgery, 32 (76%) obtained successful results. CONCLUSION: Intraoperative adjustment was effective in concomitant horizontal strabismus surgery and can provide the opportunity to avoid a large overcorrection, especially in cases with moderate angle horizontal muscle surgery.

Changes in exodeviation following hyperopic correction in patients with intermittent exotropia.

PURPOSE: To evaluate changes in the angle of deviation after spectacle correction in patients who had hyperopia and intermittent exotropia (X(T)) and to determine whether the changes and surgical outcomes differ when compared with those of myopic and emmetropic X(T). METHODS: One hundred fourteen patients with X(T) were recruited and allocated into three groups: X(T) with hyperopia (group I; 38 patients), X(T) with emmetropia (group II; 35 patients), and X(T) with myopia (group III; 41 patients). After at least 6 months wearing spectacles, changes in exodeviation were compared. The results of surgery based on the spectacle-corrected distance angle and the ratios of accommodative convergence over accommodation (AC/A) were also assessed. RESULTS: With spectacle correction, the mean exodeviation increased significantly in group I, but did not change in groups II or III. Thirteen patients in group I (34%) showed a more than 10 prism diopters (PD) exotropic shift after wearing spectacles. The mean AC/A ratio in group I was 2.63 (PD/D), whereas in groups II and III the ratios were 4.03 and 4.06, respectively. There was no difference in surgical results among the three groups. CONCLUSION: Although hyperopic correction in patients with X(T) resulted in a limited increase in exodeviation with a subnormal AC/A ratio, one-third of the patients experienced a significant increase in exodeviation. A spectacle correction trial should be considered before surgery in patients with hyperopia and X(T).

Change in ocular alignment after topical anesthetic cataract surgery.

BACKGROUND: To report the incidence of, and change in ocular misalignment following topical anesthetic cataract surgery. METHODS: Prospectively, 160 consecutive patients who were scheduled for cataract surgery under topical anesthesia were evaluated. All patients had a complete ophthalmic examination, including ocular alignment evaluation, just before and 1 day, 1 week, 3 weeks, and 2 months after cataract surgery. RESULTS: Preoperatively, 26 patients had ocular misalignment. Mean deviation in these preexisting misalignment was 7.2 +/- 6.8 prism diopters (PD). Postoperatively, the angle of deviation improved to 5.4 +/- 7.4 PD. Acquired ocular misalignment after cataract surgery occurred in 12 of 160 patients (8%) at 1 day, and seven of 131 (5%) at 2 months. None of these seven patients sought medical attention for the diplopia. CONCLUSIONS: The overall incidence of topical anesthesia-related change in ocular alignment after uneventful cataract surgery was 5%. However, no patients had symptomatic diplopia. Topical anesthetic cataract surgery could abolish the risk of postoperative diplopia and improve the heterophoric status of preexisting misalignment.

Effectiveness of cultured human keratinocyte onlays on epithelial healing and clinical outcome after photorefractive keratectomy.

PURPOSE: To evaluate epithelial healing time, postoperative pain, corneal haze, and visual and refractive outcomes following the application of cultured sheets of human allogeneic epidermal keratinocyte (CEAK) onlays on the photorefractive keratectomy (PRK) -ablated corneal surface as dressing material. METHODS: In total, 204 eyes from 103 patients with myopia or myopic astigmatism were prospectively evaluated for 6 months after PRK. The ablated cornea was dressed in three different ways. Specifically, CEAK onlays were placed in 80 eyes (CEAK group), CEAK with amniotic membrane (AM) strips in 63 eyes (CEAK with AM group), and therapeutic contact lenses only in 61 eyes (control group). All eyes were covered with bandage contact lens after the operation. Contact lens removal time, intensity of postoperative pain score on postoperative day 2, corneal haze, Snellen visual acuity, and remaining refractive errors were measured. RESULTS: The mean contact lens removal time was shorter in the CEAK with AM group (1.84 +/- 0.72 days) compared to the control (2.77 +/- 1.59 days) and CEAK only (2.24 +/- 0.79 days) groups (P < .001). However, no significant differences were evident among the groups in terms of immediate postoperative pain, Snellen visual acuity, remaining refractive errors, and corneal haze at 6 months postoperative. CONCLUSIONS: The CEAK onlay with AM facilitates epithelial healing, and is thus a good candidate dressing material to decrease the epithelial healing time after PRK. However, this onlay method did not affect the intensity of postoperative pain or final visual outcomes after surgery.

Facioscapulohumeral muscular dystrophy (FSHD) myoblasts demonstrate increased susceptibility to oxidative stress.

Facioscapulohumeral muscular dystrophy is an autosomal dominant disorder resulting from an unusual genetic mechanism. The mutation, a deletion of 3.3 kb subtelomeric repeats, appears to disrupt the regional regulation of 4q35 g ene expression. The specific gene(s)responsible for facioscapulohumeral muscular dystrophy have not been identified. However, the 'vacuolar/necrotic' phenotype exhibited by facioscapulohumeral muscular dystrophy myoblasts suggests that aberrant gene expression occurs early in facioscapulohumeral muscular dystrophy muscle development. In order to test this hypothesis, global gene expression profiling and in vitro characterization of facioscapulohumeral muscular dystrophy and control myoblasts were carried out. Genes involved in several cellular processes such as oxidative stress were found to be dysregulated. In vitro studies confirmed this susceptibility to oxidative stress, as proliferative stage facioscapulohumeral muscular dystrophy myoblasts exhibit greatly reduced viability when exposed to the oxidative stressor paraquat. This effect was not seen in either normal or disease control myoblasts, or in any of the cell lines upon differentiation to multinucleated myotubes. Immunocytochemical studies of the cyclin dependent kinase inhibitor p21 demonstrated increased expression in facioscapulohumeral muscular dystrophy myoblasts, suggesting an early cell cycle arrest. Another process distinguishing facioscapulohumeral muscular dystrophy from controls involves the transcription of extracellular matrix components. Expression of elastin, decorin, lumican and the extracellular matrix remodeling factor TIMP3 were reduced in facioscapulohumeral muscular dystrophy myoblasts. These studies suggest that facioscapulohumeral muscular dystrophy muscular dystrophy results from a defect in early myogenesis, manifested as increased susceptibility to oxidative stress, morphological aberrations and early cell cycle arrest.