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The purpose of the present study was to investigate the ocular hypertensive and anti-inflammatory responses to two different dosage schedules of 0.1% topical dexamethasone in a population of Chinese children undergoing strabismus surgery.Children undergoing bilateral strabismus surgeries were randomly assigned to receive topical 0.1% dexamethasone eye drops four times daily (group A) or twice daily (group B) for 4 weeks. Intraocular pressure (IOP) and anti-inflammatory responses were monitored for 8 weeks.A total of 137 children with mean age 6.5 years (SD, 1.9 years; range, 3-10 years) participated in the study. The IOP increased significantly after 4 weeks in both groups compared to the preoperative values (P0.001). Peak IOP ranged from 14.0 to 50.3 mmHg in group A and 11.0-41.3 mmHg in group B. Cases in group A (mean, 13.8 mmHg; SD, 8.4 mmHg) had a greater net increase in IOP than cases in group B (mean, 10.2 mmHg; SD, 6.2 mmHg; P = 0.004). Younger-aged children had higher peak IOP (r = -0.244, P = 0.048), and attained the peak IOP earlier (r = 0.252, P = 0.041) in group A. There was no significant difference in ocular inflammatory response between the two groups.Ocular hypertensive effect to topical 0.1% dexamethasone is dose and age dependent in children. Twice-daily 0.1% topical dexamethasone eye drops control inflammation after strabismus surgery as effectively as four-times-daily dosage, but induces less increase in IOP, and may be a better treatment schedule.

To investigate the ocular hypertensive and antiinflammatory response to different dosages of fluorometholone (FML).Prospective clinical trial with randomization of fellow eyes to different postoperative treatment.Thirty-one consecutive children undergoing bilateral symmetrical strabismus operation.Among 31 children who received bilateral squint operations, one eye was randomized to receive topical FML six times daily (group 1), whereas the other eye received topical FML three times daily (group 2), both for 4 weeks. Intraocular pressure (IOP) and antiinflammatory response were measured serially in the postoperative period for 8 weeks.Intraocular pressure was measured on the day before surgery and on postoperative days 1, 3, 6, 13, 20, 27, 41, and 55. The antiinflammatory response was also assessed subjectively and objectively at days 6, 13, 20, and 27 after the operations. Peak IOP, net increase in IOP, and antiinflammatory responses in the two study groups were analyzed.Thirty-one children, age 3 to 9 years, (mean +/- standard deviation [SD], 5.52 +/- 1.81) participated in the study. Intraocular pressure increased significantly in both groups compared with the preoperative values (P0.001). The peak IOP ranged from 12.00 to 31.00 mmHg and 12.30 to 25.00 mmHg in groups 1 and 2, respectively. The mean peak IOP (19.00 +/- 5.06 mmHg vs. 17.13 +/- 3.32 mmHg) was significantly higher in group 1 (P0.001). The net increase in IOP was similar (mean +/- SD, 4.37 +/- 4.79 vs. 2.57 +/- 3.32 mmHg; P = 0.005). Ranges of the net IOP increase were -1.00 to 16.00 mmHg and -2.50 to 10.30 mmHg in groups 1 and 2, respectively. Children in group 1 reached the peak IOP earlier than those in group 2 (median, 6 vs. 13 days; P = 0.033). However, there was no significant difference in antiinflammatory response between the two groups.Ocular hypertension occurs in a dose-dependent manner in children treated with FML. Children in group 1 had a quicker onset and more severe ocular hypertensive response than those in group 2. It would be desirable to monitor the IOP regularly when FML is used with a high frequency and for a long duration in children.

Objective To investigate the ocular-hypertensive response to different dosages of topical dexamethasone eye drops in Chinese children. Design Prospective, randomized clinical trial. Participants Thirty-one consecutive children undergoing bilateral strabismus surgery. Intervention Topical dexamethasone (0.1%) was administered to children undergoing bilateral strabismus surgery. They were all less than 10 years of age. One eye was randomized to receive a regimen of four times daily, and another received a twice daily regimen. Intraocular pressure (IOP) was serially measured in the postoperative period for 4 weeks or more. Topical steroids were discontinued if IOP was 30 mmHg or more. Main outcome measures Intraocular pressure was measured on the day before the surgery, on postoperative days 1, 3, 5, 8, 12, 15, 22, 29, and 2 weeks thereafter until the IOP reached preoperative levels. Peak IOP, IOP net increase, and time to reach an IOP more than 20 mmHg in the two study groups were analyzed. Results A total of 31 patients (20 male, 11 female) were examined. The mean age was 5.8 ± 2.0 years (range, 2–10 years). Preoperative IOP in groups treated twice daily and four times daily were similar. After topical dexamethasone treatment, both groups showed a significant rise in peak IOP compared with preoperative values (twice daily, 25.2 ± 6.8 mmHg vs. 14.3 ± 2.4 mmHg, P P P P P Conclusions In children treated with topical dexamethasone, ocular hypertension occurs in a dose-dependent manner. Children in the four times daily group had a quicker onset and more severe ocular hypertensive response than the twice daily group. Nevertheless, even the twice daily regimen produced significant IOP rise, suggesting that dexamethasone use in children should be avoided if possible, and it would be desirable to monitor the IOP twice weekly when it is administered to children.

Objective To investigate the ocular complications in pediatric bone marrow transplantation (BMT) patients. Design Cross-sectional study. Participants A total of 29 pediatric BMT patients were studied. Testing Comprehensive ophthalmic check-up, including best-corrected visual acuity, intraocular pressure (IOP), Schirmer's test, tear breakup time, and slit-lamp and fundus examinations, was performed. Main outcome measures Tear film instability and its related complications, IOP, cataract, and fundus lesions were measured. Results The mean age of patients was 9.1 years (range, 1.5–15 years). The mean post-BMT duration was 20.2 months (range, 3–54 months). Fifteen patients (51.7%) had tear abnormalities. Subconjunctival fibrosis was detected in two patients (6.9%). Dry and scaly skin of the eyelids was seen in one patient (3.4%). Lens opacities were found in 2 (33.3%) of 6 irradiated patients and 2 (8.7%) of 23 nonirradiated patients. Two patients (6.9%) had fundus changes, one with unilateral epiretinal membrane and the other with bilateral multiple discrete chorioretinal hypopigmented lesions in the middle to peripheral part of the retina. The overall complication rates for the anterior and posterior segments were 75.8% and 6.9%, respectively. Conclusion Ocular manifestations of BMT in children are not uncommon. The most common anterior segment problem is tear dysfunction. Posterior segment complications are less common but do exist. High rate of cataract formation is reported, and this probably is the most important long-term "amblyogenic" problem in these immature eyes. Awareness and management of these problems with routine eye examination and early intervention are recommended.

Primary orbital Ewing's sarcoma is a very rare condition. Since the first case was reported in 1950, only 7 other cases have been reported in the English literature. Herein we describe the ninth, the only bilateral and the youngest case of primary orbital Ewing's sarcoma occurring in a 2-year-old boy, who presented to us with bilateral painless proptosis. Tissue biopsy of the tumour was obtained through the nasal sinus. Immunohistological studies of the biopsy tissue confirmed the diagnosis of Ewing's sarcoma. No distant site of the tumour was found so this was considered a primary orbital tumour. Combined chemotherapy and radiotherapy without surgical resection achieved an encouraging result in that the patient has remained in remission for 30 months after completion of treatment. The successful use of combined chemotherapy and radiotherapy, without surgery, adds further support to evidence that surgical excision may be avoided in selected cases of primary orbital Ewing's sarcoma.

Purpose: To study the safety and efficacy of scleral intraocular lens (IOL) fixation in children. Setting: A university practice. Methods: This retrospective review evaluated the results and complications in 6 consecutive eyes of 3 children who had anterior vitrectomy, with or without lensectomy, and scleral IOL fixation to correct ectopia lentis or aphakia. Results: At a mean follow-up of 17.3 months (range 13 to 21 months), all eyes had a stable and well-positioned posterior chamber IOL and good visual improvement. The only complication was asymptomatic pupillary capture of the IOL in 3 eyes. Reversal of the pupillary capture was achieved by pupil dilation with the patient in a supine position. Conclusions: The preliminary results of scleral IOL fixation in children are encouraging. The procedure's application in well-selected cases can be considered. However, its long-term safety and efficacy must be further assessed through studies with larger sample sizes and a longer follow-up.

Purpose: To determine the safety and efficacy of autogenous palmaris longus tendon as a sling material in frontalis suspension surgery for ptosis correction in children. Methods: In a prospective study, the authors evaluated 15 frontalis sling suspension surgeries using palmaris longus tendon in 14 consecutive children with congenital ptosis. The method of harvesting the palmaris longus tendon is described in detail. Results: The mean age of the patients was 4.7 years (range, 2 to 7 years). At a mean follow-up of 30 months (range, 20 to 40 months), all ptotic eyelids were successfully corrected with good final lid position. No recurrence of ptosis was encountered. In one eye, knot slippage of the sling was related to upper eyelid trauma, which was readily repaired. Otherwise, no exposure keratitis, wound infection, eyelid contour abnormality, or ptosis overcorrection occurred. There were no complications associated with the palmaris longus tendon donor site. Conclusion: Frontalis suspension with autogenous palmaris longus tendon appears to be an effective treatment for congenital ptosis with poor levator function in children. Its use as an alternative material to fascia lata for sling surgery in children can be considered.

Ten consecutive cases of severe unilateral congenital ptosis were surgically corrected before 1 year of age (range 3-11 months) in an attempt to achieve early functional and cosmetic improvement. In all cases, a frontalis suspension using Mersilene mesh was performed. With a mean follow-up of 40.3 months (range 33-54 months), all patients achieved normal or near normal eyelid position and all had their chin-up head posture resolved. One patient developed a mild exposure keratitis that was treated successfully with topical antibiotics and lubricants. Our findings suggest that the Mersilene mesh sling has good potential for ptosis management in infants who are too young for fascial harvesting. However, a larger series with a longer follow-up period is required before the eventual safety and efficacy of the Mersilene mesh sling can be properly ascertained.

Cystic schwannoma of the orbit is unreported in the world literature. A 54-year-old Caucasian man presented to our clinic with a 5 year history of progressive right-sided proptosis and diplopia. A large paramedian cystic mass displacing the right globe downwards and laterally was demonstrated. From the clinical and radiological features, the presumptive diagnosis of right frontal sinus mucocoele was made. However, the excisional biopsy of the lesion revealed the rare diagnosis of cystic schwannoma, arising from a branch of the first division of the trigeminal nerve. Post-operatively, the patient had a smooth recovery with visual acuity of 20/20 in each eye; full binocular single vision was also re-established. The differential diagnoses of cystic orbital mass, and the radiological and histological findings of the lesion, are described and discussed.

Purpose To report the ocular hypertensive response to high-dose systemic corticosteroid in a pediatric patient. Design Observational case report. Methods A 9-year-old patient with leukemia received oral prednisolone at a dosage of 2.3 mg/kg/d for 5 weeks, followed by a 4-month break and then a 4-week course of oral dexamethasone at 10 mg/d. Detailed ocular examination was performed for both eyes before and regularly throughout the two courses of treatment. Results The intraocular pressure in both eyes rose to almost 40 mm Hg after only 8 days of oral corticosteroid. On stopping systemic corticosteroid, the intraocular pressure rapidly returned to baseline level within 2 days. A similar intraocular pressure profile was recorded for both eyes during the course of oral dexamethasone. The patient remained largely asymptomatic throughout. Conclusions Systemic corticosteroid may give rise to significant but asymptomatic ocular hypertension in pediatric patients.

To compare the ocular-hypertensive and anti-inflammatory response to rimexolone (116-hydroxy-16alphafluoro-6alphamethylpresdnisolone) and fluorometholone (21-deoxy-9alphafluoro-6alphamethylprednisolone) therapy in children's eyes.With parental consent, children who underwent surgical procedures for bilateral symmetric strabismus from January 18, 2000, through November 16, 2001, were recruited. One eye was randomized to receive topical 1% rimexolone while the contralateral eye received topical 0.1% fluorometholone, 4 times daily for 4 weeks.Intraocular pressures and anti-inflammatory responses were the main outcome measures and were serially measured postoperatively for 8 weeks.Fifty-four children, aged from 4 to 8 years (mean [SD] age, 5.33 [1.26] years), participated in the study. Intraocular pressure increased significantly in both treatment groups compared with the preoperative values (P.001). The mean (SD) peak intraocular pressure was significantly higher in the rimexolone-treated group, 19.7 (6.1) vs 17.6 (4.6) mm Hg (P.001). Similarly, the mean (SD) net increase in intraocular pressure (P.001), was also higher in the rimexolone-treated eyes, 5.9 (4.4) vs 3.9 (4.1) mm Hg (P.001). In addition, a greater percentage of the rimexolone-treated patients had no conjunctival erythema on days 13 (11.1% vs 0.0%) and 20 (88.9% vs 55.6%) (P =.03).Rimexolone seems to be a more effective anti-inflammatory agent than fluorometholone. However, unlike adults, the ocular-hypertensive effect in children treated with rimexolone was higher. It would be desirable to monitor the intraocular pressure regularly when rimexolone therapy is used in children.

To report the unusual association of bilateral corneal keloids and anterior segment mesenchymal dysgenesis in a child with Rubinstein-Taybi syndrome.Case report of a 2-year-old boy.Excision of the epicorneal mass in the right eye was followed by recurrence of the lesion. Multiple penetrating keratoplasties were unsuccessful in reconstructing the anterior segment because of recurrent corneal epithelial breakdown, suggesting limbal stem cell insufficiency. Histopathology and electron microscopy of the excised mass lesion showed features typical of a corneal keloid: thickened keratinized epithelium, absent Bowman's layer, and fibrovascular hyperplasia, with haphazard orientation of the collagen lamellae. Ultrasound biomicroscopy and intraoperative findings suggested a diagnosis of Peter anomaly, but genetic analysis did not show a PAX6 mutation.The findings in our patient add to the spectrum of ocular changes described in Rubinstein-Taybi syndrome and confirm earlier reports of poor ocular prognosis in corneal keloids and Rubinstein-Taybi syndrome.

A 4-year-old boy with bilateral idiopathic and progressive ectopia lentis had phacoaspiration of the lens and capsular tension ring (CTR) insertion in his left eye. Postoperatively, lens capsule centration remained poor. Single-point scleral fixation of the CTR was performed and centration of the lens capsule achieved. In-the-capsule intraocular lens (IOL) implantation was tried, but excessive IOL manipulation sliced open the capsule at the equator. Anterior vitrectomy and scleral IOL fixation were required. A similar approach was adopted in the right eye with meticulous attention given to gentle maneuvers. The operation was uneventful, with good IOL centration. Three and 11 months after surgery in the right and left eye, respectively, best corrected visual acuity was 20/50 in both eyes. Scleral fixation of the CTR, accompanied by gentle IOL manipulation, is an option to improve IOL centration in patients with severe zonular deficiency.

PURPOSE: To investigate a case of isolated pedunculated congenital corneal dermoid. METHODS: Case report. RESULTS: In a 14-day-old infant, the pedunculated portion of the dermoid was removed and confirmed by histopathologic examination. No surgical complication or recurrence was encountered, and the patient is waiting for a rotational autokeratoplasty. CONCLUSIONS: Prompt treatment of such an unusual tumor is important to allow for visual rehabilitation and development.

Objective: The purpose of the study is to investigate the rate and degree of ocularhypertensive response to topical steroids in Chinese children. Design: The study design was an institutional, randomized, clinical trial. Participants: A total of 19 consecutive patients were studied. Intervention: Topical steroids were administered to Chinese children younger than 10 years of age who underwent bilateral strabismus surgery. One eye was randomized to receive topical 0.1% dexamethasone (DMS), whereas the fellow eye received 0.1% fluorometholone (FML) six times per day for up to 4 weeks. Intraocular pressure (lOP) was measured on the day before operation and at postoperative days 1, 3, 6, 10, 13, and 27, then every 2 weeks thereafter until the lOP fell to preoperative levels. Topical steroids would be stopped if lOP was 30.00 mmHg or greater. Main Outcome Measures: Peak lOP and maximal change of lOP from baseline were measured and categorized into low, intermediate, and high levels. Time to peak lOP also was studied. Results: A total of 16 patients were included. The peak lOP for DMS-treated eyes was 30.66 ± 8.35 mmHg (range, 13.00–48.00 mmHg), whereas that in FML-treated eyes was significantly lower at 20.66 ± 6.03 mmHg (range, 11.30–36.30 mmHg) ( P = 0.001). The maximal change in lOP ranged from −2.60 to +31.00 mmHg in OMS-treated eyes (mean, 15.48 ± 8.71 mmHg), almost double that of FML-treated eyes (range, +1.00 to +17.00 mmHg; mean, 5.83 ± 4.96 mmHg) ( P = 0.001). When the ocular-hypertensive responses of both DMS and FML groups were categorized into three levels of severity, significant differences were found between the two treatment groups ( P = 0.001). In the OMS group, nine patients (56.25%) were high responders and six patients (37.5%) were intermediate responders. In the FML group, only one patient (6.25%) was a high responder. Conclusions: The ocular-hypertensive response to topical OMS in children occurs more frequently, more severely, and more rapidly than that reported in adults. A total of 56% of the studied children, all younger than 10 years of age, were high responders to topical OMS. Of these, 89% attained their peak lOP within 8 days. Its use in children should best be avoided if possible. It would be desirable to monitor the lOP when it is being used. Conversely, FML produced a much less ocular-hypertensive effect and therefore poses an acceptable risk of clinically significant pressure elevation.

We investigated sequence alternation, promoter methylation, and loss of heterozygosity (LOH) of the RB1 gene as possible mechanisms of its inactivation in retinoblastoma. In 42 Chinese patients with sporadic retinoblastoma, the promoter and entire coding region of RB1 were examined for sequence changes. Status of methylation of the CpG-rich island at the 5'end was determined by methylation specific PCR assay. We detected 15 RB1 mutations in 38% (16/42) of the retinoblastoma patients, among them 19% (8/42) were germ-line mutations. A total of nine novel mutations were identified: E54X, S114X, I126S, g73779insG, D718N, IVS2+1G>C, IVS14+1G>C, IVS21+1G>C, and a complex alteration g78177G>T/g78176insTT leading to 543X. Most of them are likely to affect the RB1large pocket domain through the production of truncated gene products. None of the DNA samples showed methylation at the RB1promoter. In 15 cases where both normal and cancerous retinoblastoma tissue specimens were available, allelic loss according to microsatellite markers within or distal to the RB1 locus was analyzed and immunohistological staining for RB1 expression performed. Among them, frequency of LOH at 13q14 was found to be high at 60% (9/15) with no segregation with unilateral tumors. All these nine tumors did not express RB1 protein, showing an association of LOH at the RB1 locus with its loss of expression in retinoblastoma. Our results indicate that the RB1 gene in sporadic retinoblastoma is commonly inactivated because of loss-of-function mutations and loss of heterozygosity but not by the epigenetic phenomenon of promoter hypermethylation.