Outcome of cataract surgery in pediatric uveitis (experience at King Khalid Eye Specialist Hospital).

BACKGROUND: Cataract is a major complication of uveitis in children that requires timely management to restore good vision and prevent amblyopia. In surgical management of uveitic pediatric cataracts, published literature has shown a variety of clinical factors affecting the final visual outcome. The aim of this study is to investigate and report the clinical outcomes of cataract surgery and contributing factors impacting such outcomes in children with uveitis. MATERIALS AND METHODS: A retrospective case series of all pediatric patients (<18 years) who were diagnosed with uveitis and had cataract surgery at a tertiary eye institutions, between January 2000 and October 2016. Outcomes measures include best-corrected visual acuity (BCVA) and postoperative complications. Success was defined as BCVA of ≥20/60. Factors related to successful outcome were analyzed. RESULTS: The study sample was comprised of 26 patients (39 eyes). The median postoperative follow-up was 6.4 years. At the last follow-up, only 2 eyes had active uveitis, 3 eyes were controlled without medications, and 34 eyes were controlled with systemic or topical medications. At the last follow-up, 64% of eyes had BCVA ≥20/60. Anterior uveitis and postoperative aphakia were two determinants, in statistically significant manner, found to be positively related to clinical success (P = 0.01 and 0.04, respectively). Etiology of uveitis and use of preoperative intravenous methylprednisolone were not related to the success of obtaining BCVA ≥20/60 (P = 0.5 and 0.99, respectively). CONCLUSIONS: Cataract surgery improves the visual function of children with clinically significant uveitic cataracts. In this study, we found diagnosis of anterior uveitis and postoperative aphakia to be associated with better final postoperative visual outcomes.

Case Report of a 4-Year-Old Child with Complicated Vogt-Koyanagi-Harada at a Tertiary Eye Hospital.

Vogt-Koyanagi-Harada (VKH) disease is a chronic, bilateral, granulomatous panuveitis associated with cutaneous, neurologic, and auditory manifestations. We report a 4-year-old Saudi boy who developed severe ocular complications by 5 years of age. He presented to King Khalid Eye Specialist Hospital at the age of 4 years and was previously operated on elsewhere for cataract with intraocular lens implantation in his right eye at the age of 3 years. He consecutively had iris capture and membrane formation around the intraocular lens. Examination revealed band keratopathy, posterior synechiae, and fundus depigmentation in both eyes with cataract formation in his left eye. At the age of 5.5 years, he developed subretinal neovascular membrane formation in the left eye. To the best of our knowledge, this patient is youngest VKH case that manifested most of the major complications at a young age as 5 years old.

Marcus Gunn Jaw-Winking Syndrome Associated with Morning Glory Disc Anomaly.

Marcus Gunn jaw-winking syndrome (MGJWS) is a rare form of congenital blepharoptosis and one of the congenital cranial dysinnervation disorders (CCDD). In addition, morning glory disc anomaly (MGDA) is a congenital optic disc anomaly of unknown etiology. The present report is the first to describe an association between MGJWS and MGDA in an otherwise healthy 7-year-old boy. He also had counting finger vision, anisometropia, esotropia, and monocular elevation deficiency in the same eye. In the literature, both MGJWS and MGDA have been reported to be associated with Duane retraction syndrome, a form of CCDD.

The use of one muscle recession for horizontal strabismus.

PURPOSE: To evaluate the outcomes of one muscle recession for horizontal comitant strabismus at a major referral hospital in the Middle East. METHOD: Retrospective charts review of postoperative outcomes of 90 patients who had undergone one muscle recession for small to moderate angle esotropia or exotropia. Data were collected for age, vision, amblyopia, previous surgery or botulinum toxin injection, preoperative deviation, amount and type of one muscle surgery, and postoperative deviation at the initial and last (six months or more) postoperative visit. Successful alignment was defined as ±10 prism diopters (PD) of orthophoria. RESULTS: Sixty patients underwent medial rectus recession and 30 patients underwent lateral rectus recession. The average preoperative and last follow up deviation -respectively- was 24 ±â€¯6.1 PD (15-35) PD and 14.62 ±â€¯8.91 PD in the medial rectus recession group and 21.3 ±â€¯5.1 PD (12-30) and 12.60 ±â€¯8.74 in the lateral rectus recession group. The final success rates were 63.3% in both groups. CONCLUSION: Single muscle strabismus surgery to correct horizontal strabismus had a variable outcome. Larger recession may help in achieving better outcomes. Properly designed prospective studies may help in identifying the factors affecting the outcomes of single muscle strabismus surgeries.

Effect of Chloral Hydrate Sedation on Intraocular Pressure in a Pediatric Population.

PURPOSE: To determine the effect of oral chloral hydrate (CH) sedation on intraocular pressure (IOP) in an outpatient pediatric population. DESIGN: Prospective, noncomparative case series. METHODS: Children aged 1 month to 5 years undergoing CH sedation for ocular imaging/evaluation at a tertiary eye hospital were included. IOP was measured using an Icare tonometer prior to sedation (in some, not all), at 25 minutes after sedation, and then every 10 minutes until sedation completion. Change in IOP over time was assessed using mixed model linear regression to account for correlation of IOP readings. RESULTS: A total of 112 children were enrolled, 50.9% were female, and mean age was 2.1 (standard deviation [SD]: 1.3) years. Of the total, 83 (74.1%) participants had IOP measurement attempted prior to sedation, with 64 having presedation IOP completed. Among those completing presedation IOP, 46.9% were asleep/calm, and the rest (53.1%) were slightly/more distressed (IOP did not differ by level of agitation). Those with and without presedation IOP available had similar demographics and health status (P > .05). Heart rate, respiratory rate, and oxygen saturation all declined after sedation (P < .001). The mean dose of CH administered was 80.9 (SD: 13.2) mg/kg, and sedation was deemed "adequate" in 97.3% after a single dose. Mean IOP among those with presedation IOP was 19.5 mm Hg and, although not significant, declined to 18.7 mm Hg at 25 minutes (P = .12). There was no trend toward further decline in IOP over time (P > .05). CONCLUSIONS: CH sedation for outpatient pediatric ophthalmic procedures as administered in this prospective assessment had no impact on IOP.

Chloral Hydrate Administered by a Dedicated Sedation Service Can Be Used Safely and Effectively for Pediatric Ophthalmic Examination.

PURPOSE: To determine safety and efficacy of oral chloral hydrate sedation (CHS) for outpatient pediatric ophthalmic procedures. DESIGN: Prospective, interventional case series. METHODS: Setting: King Khaled Eye Specialist Hospital. SUBJECTS: Children aged 1 month to 5 years undergoing CHS for ocular imaging/evaluation. PROCEDURES: Details on chloral hydrate dose administered, sedation achieved, vital signs, and adverse events were recorded. OUTCOME MEASURES: Primary outcome was percentage of patients with a sedation level ≥ 4 at 45 minutes post chloral hydrate administration. Secondary outcomes were time from sedation to discharge and adverse events, including changes in vital signs following chloral hydrate administration. RESULTS: A total of 324 children were recruited with a mean age of 2.2 (SD: 1.3) years and mean weight of 10.9 (SD: 3.3) kg. Adequate sedation was obtained with a mean chloral hydrate first dose of 77.4 (SD: 14.7) mg/kg in 306 (94.4%) patients, with an additional 6 patients (1.9%) achieving adequate sedation with a second dose (overall adequate sedation: 96.3%). Mean reductions in heart rate, respiratory rate, and oxygen (O2) saturation from pre-sedation to 25 minutes post-sedation were 11.7 (SD: 14.3) beats per minute, 1.2 (SD: 2.4) breaths per minute, and 0.81% (SD: 1.2%), respectively (P < .001 for all). In multivariable regression, odds of remaining sedated 45 minutes after chloral hydrate administration were 2.53 times higher for American Society of Anesthesiologists (ASA) class II or III patients than for ASA class I (95% confidence interval [CI]: 1.11-5.78, P = .03), 1.03 times higher per mg increase in initial dose of chloral hydrate (95% CI: 1.01-1.06, P = .006), and 2.70 times higher per unit increase in number of planned procedures (95% CI: 1.63-4.47, P < .001). Three patients developed minor adverse events: 2 cases of O2 desaturation and 1 paradoxical reaction, none requiring significant intervention. Patients were discharged a median of 90 minutes after chloral hydrate administration. CONCLUSION: Chloral hydrate administered by a dedicated sedation service, as in this prospective assessment, can be used safely and effectively for outpatient pediatric ophthalmic procedures.

Ciliary body location in eyes with and without primary congenital glaucoma.

OBJECTIVE: To compare the location of ciliary body (CB) in children with and without primary congenital glaucoma (PCG). METHODS: In this cross-sectional study, we enrolled Saudi children who were less than 5 years old. CB distance (CBD) was measured and compared in eyes with PCG (PCG group) and without PCG (control group). CBD was measured with a caliper and defined as the distance between the edge of the corneoscleral limbus and the anterior edge of CB as delineated by transillumination. The difference in the CBD between groups was correlated with the axial length, corneal thickness, and corneal diameter. RESULTS: CBD was measured in 15 eyes in the PCG and control groups. The mean CBD (1.6 ± 0.4 mm) in the PCG group was significantly greater than that in the control group (1.3 ± 0.3 mm) (p < 0.001). The mean difference in the CBD of 2 groups was 0.33 mm (95% CI 0.15-0.54). In PCG eyes, the CBD was farthest in the superior quadrant (1.7 mm) followed by inferior (1.6 mm), temporal (1.6 mm), and nasal (1.5 mm) quadrants. The variability in CBD between quadrants in PCG eyes was greater than that in the control group. CBD in the PCG group showed a significant correlation with increasing axial length (p = 0.05), corneal thickness (p < 0.001), and corneal diameter (p = 0.0002). CONCLUSIONS: The CBD from the limbus was greater in PCG eyes compared to the controls and varied significantly in different quadrants. The knowledge regarding the greater CBD and its variability in PCG eyes could enable better planning of surgical treatment in congenital glaucoma.

Patterns of Uveitis in a Tertiary Care Referral Institute in Saudi Arabia.

PURPOSE: To present the pattern, causes and complications of uveitis in one of the tertiary eye centers in Saudi Arabia. METHODS: In this retrospective study, clinical records of randomly selected cases of uveitis attending the King Khaled Eye Specialist Hospital, Saudi Arabia, from 2001 to 2010, were reviewed. RESULTS: Our series included 888 out of 1286 cases of uveitis. Among them, there were 390 men (43.9%) with a mean age of 39.6 ± 11 (range: 6-94 years). The prevalence of uveitis among all eye patients was 1.5% and the annual incidence was 129 cases. Uveitis was bilateral in 567 patients (63.8%), mainly anterior in 27.3%, intermediate in 12.7%, posterior in 7.1%, while 52.9% were panuveitis. Infective etiology was seen in 180 patients (20.3%), of which presumed tuberculous uveitis (PTU) was the commonest cause in 94 patients (52%), followed by herpetic in 46 (26%) and toxoplasmosis in 29 (16%). Vogt-Koyanagi-Harada (VKH) 22%, Behçet uveitis (BU) 14.6%, idiopathic anterior uveitis 12.5%, presumed tuberculous uveitis (PTU) 10.6%, and idiopathic intermediate uveitis (10.1%) accounted for the majority of cases, whereas other diagnostic entities accounted for 30%. The most common cause of anterior uveitis was idiopathic uveitis (101; 41.7%), followed by herpetic (46; 19%). Toxoplasmosis was the most frequent cause of posterior uveitis (29; 46.3%). Intermediate uveitis was most commonly idiopathic (86; 76.1%). VKH disease was the most common cause of panuveitis (195; 41.5%), followed by Behçet disease (118; 25.1%). The common complications of uveitis were glaucoma (34.1%), posterior synechiae (22.3%) and cataract (15.1%). CONCLUSIONS: The most common anatomic diagnosis was panuveitis. VKH disease and Behçet disease were the most frequent causes and glaucoma was the most common complication of uveitis.

Pathological and Immunohistochemical Alterations of the Cornea in Congenital Corneal Opacification Secondary to Primary Congenital Glaucoma and Peters Anomaly.

PURPOSE: To examine the immunohistochemical alterations in the corneal stroma in Peters anomaly (PA) and congenital glaucoma (CG) compared with age-matched normals and acquired adult corneal scarring (AACS). METHODS: The clinical features of PA and CG patients who underwent penetrating keratoplasty were recorded. Immunohistochemistry of cornea and control tissue (normal and acquired corneal scars) was performed with antibodies against collagen types I, III, keratan sulfate, lumican, decorin, and smooth muscle actin followed by semiquantitative analysis of immunolabeling. RESULTS: Clinical features in 2 groups were consistent with PA and CG. Microscopy showed thickened stromal collagen bundles in PA (n = 15), CG (n = 11), and AACS (n = 20) compared with normals (n = 18). PA and CG had distinct immunophenotypes compared with controls. Type I collagen labeling was more intense in CG compared with PA (intensity grading (IG) 2.73 vs. 2.07; P < 0.001). Decorin, lumican, and keratan sulfate labeling was significantly less intense in PA versus AACS (IG; 1.91, 0.38, 1.75 in PA and 2.7, 1.11, 2.61 in AACS. respectively; P = 0.002, P = 0.001 and P = 0.004) and normals (IG 1.92, 1.06, 2.59 respectively; P < 0.001, P < 0.001 and P = 0.005). Collagen I labeling was less intense in CG versus AACS (IG 2.73 vs. 3.09) (P = 0.007). Collagen III labeling was more intense in PA/CG than in normals (IG 0.9, 0.64, 0.62 retrospectively) (P < 0.001 in both). CONCLUSIONS: The immunophenotype of the corneal scar in PA and CG differs from normal corneas and so does PA from AACS. The similarities between CG and AACS suggest that CG scarring has an acquired component.

Congenital cranial dysinnervation disorder in a boy with congenital mirror movements.

"Mirror movements" are an axonal guidance disorder that consists of involuntary contralateral movements that mimic unilateral intentional ones, typically involving the fingers of the hand. They can be isolated or associated with conditions such as Klippel-Feil syndrome, Kallmann syndrome, or congenital hemiplegia. Isolated congenital mirror movements are sometimes caused by autosomal dominant mutation in the genes DCC or RAD51. At least 4 previously reported cases had strabismus, 3 with Moebius syndrome and 1 with Duane retraction syndrome. We report the case of a boy with an unusual incomitant strabismus consistent with orbital dysinnervation and suggest that for some patients with congenital mirror movements the neurological miswiring extends to the orbit, causing congenital cranial dysinnervation disorder.

Update of intraocular lens implantation in children.

Cataract is a common problem that affects the vision in children and a major cause of amblyopia in children. However, the management of childhood cataract is tenuous and requires special considerations especially with regard to intraocular lens (IOL) implantation. Age at which an IOL can be implanted is a controversial issue. Implanting an IOL in very young children carries the risk of severe postoperative inflammation and posterior capsule opacification that may need other surgeries and may affect the vision permanently. Accuracy of the calculated IOL power is affected by the short eyes and the steep keratometric values at this age. Furthermore, choosing an appropriate IOL power is not a straight forward decision as future growth of the eye affects the axial length and keratometry readings which may result in an unexpected refractive error as children age. The aim of this review is to cover these issues regarding IOL implantation in children; indications, timing of implantation, types of IOLs, site of implantation and the power calculations.