Optical coherence tomography: a quantitative tool to screen for papilledema in craniosynostosis.

PURPOSE: Our aim was to evaluate if optical coherence tomography (OCT) can be used as an alternative for fundoscopy to screen for increased intracranial pressure (ICP) in children with craniosynostosis METHODS: We performed a prospective cohort study at the Dutch Craniofacial Centre. We included 38 patients with nonsyndromic scaphocephaly and Crouzon's syndrome aged 3-8 years old, in whom we scored complaints suggestive of increased ICP and performed fundoscopy and OCT. Main outcome measures total retinal thickness (TRT) which was measured on 58 OCT scans. RESULTS: Forty-three percent of fundoscopies revealed pathologic changes of the papil in at least one eye. Retinal thickness was increased in patients with an abnormal fundoscopy as compared to patients with a normal papil (TRT p < 0.001). Patients with Crouzon's syndrome had a significantly increased retinal thickness as compared to patients with scaphocephaly (TRT p < 0.001). CONCLUSIONS: The current study demonstrates that OCT in children with craniosynostosis is feasible. It confirms that retinal thickness increases in case of papilledema. Given the quantitative character, OCT has a high potential as an alternative tool to screen for papilledema in craniosynostosis and other pediatric populations.

Active removal of anterior segment-migrated dexamethasone implant (Ozurdex®).

Background: Ozurdex® (Allergan plc., Dublin, Ireland) is an intravitreal sustained-release dexamethasone (DEX) implant. The implant has been reported to migrate into the anterior chamber, potentially causing corneal decompensation. Prompt removal or relocation in the vitreous cavity is advised but troublesome due to its fragility. Several techniques exist, but elaborate setup and specialized surgical skills that are required may cause delay in treatment. We report a novel technique that avoids these shortcomings. Case presentation: A 59-year-old woman presented to the emergency department with visual loss due to an anterior chamber-migrated DEX implant and corneal edema. Using an ophthalmic viscosurgical device (OVD) and a bent 19-gauge needle, the implant has promptly been removed in a one-minute procedure under topical anesthesia. Conclusion: Aspirating an anterior chamber-migrated DEX implant using a 19-gauge bent needle is a cost-effective, time-efficient and safe technique, not requiring specialized surgical skills.

Ptosis, extraocular motility disorder, and myopia as features of pompe disease.

PURPOSE: The assets of this report are a thorough description of new clinical findings, namely the combination of ptosis, extraocular motility disorder and myopia, in consequence of prolonged survival in classic infantile-onset Pompe disease through enzyme therapy. DESIGN: Single case description. RESULTS: This manuscript describes a combination of ptosis, extraocular motility disorder and myopia in a 4.5-year-old patient with classic infantile-onset Pompe disease, who survived through enzyme therapy. This patient was treated with a bilateral frontalis suspension (modified Crawford technique) using prolene 3-0 sutures. CONCLUSIONS: The combination of ptosis, extraocular motility disorder and myopia, is a new clinical finding in children with classic infantile-onset Pompe disease.

Floppy eyelid syndrome and ptosis in a patient with pachydermoperiostosis.

A patient with the rare condition of pachydermoperiostosis with secondary ptosis and floppy eyelid was successfully treated with a combination of levator advancement and an upper eyelid tarsal strip. This is the second report in the literature of combined floppy eyelid and ptosis with this condition and the first report of this surgical approach for its management. The histopathology of the excised eyelid tissue found changes consistent with both pachydermoperiostosis and floppy eyelid syndrome.

Muscle prolapse after harvesting autogenous fascia lata used for frontalis suspension in children.

PURPOSE: To evaluate the long-term results at the donor site in children who underwent harvesting of autogenous fascia lata for frontalis suspension in ptosis correction. METHODS: The study design consists of a retrospective, nonrandomized case series. Medical records of 30 patients were reviewed. Each patient was reexamined, evaluating bulging at the donor site. Muscle prolapse was graded as not visible and not palpable, not visible but palpable, mildly visible, or obviously visible. Measurement of the width and length of the leg scar was recorded. Discomfort at the donor site was graded as no discomfort, occasional discomfort, or frequent discomfort. RESULTS: The mean age of the patients was 7 years (range, 1-15), with a mean follow-up period of 27 months (range, 3-63 months). Fifteen patients (50%) had invisible and impalpable bulging, 2 patients (6.7%) had invisible but palpable bulging, 5 patients (16.7%) had mildly visible bulging, and 8 patients (26.7%) had obviously visible bulging. The mean width of the scar was 7.5 mm, and the mean length was 3.6 cm. Twenty-seven patients (90%) had no functional discomfort of the leg, 2 patients (6.7%) had occasional discomfort, and 1 patient (3.3%) had frequent discomfort that occurred during exercise. CONCLUSIONS: After fascia lata harvesting, most patients had no cosmetic complaints or functional complications. The technique of harvesting fascia lata using a Crawford stripper seems to be safe and satisfactory.

Acute angle closure glaucoma after oculoplastic surgery.

PURPOSE: To describe two patients with acute angle closure glaucoma after oculoplastic surgery. DESIGN: Interventional case reports. METHODS: Review of clinical findings and treatment. RESULTS: A 61-year-old female developed a painful eye with decreased visual acuity a few days after bilateral blepharoplasty. A 69-year-old male developed a painful red eye with decreased visual acuity a few days after ptosis correction. In both cases the intraocular pressure increased to more than 50 mmHg. Both patients were diagnosed with acute angle closure glaucoma and treated appropriately. CONCLUSIONS: Acute angle closure glaucoma is a possible complication of oculoplastic surgery.

Silicone intubation with or without balloon dacryocystoplasty in acquired partial nasolacrimal duct obstruction.

PURPOSE: To examine if the addition of (antegrade) balloon dacryocystoplasty to bicanalicular silicone intubation affects the success rate in adults with incomplete nasolacrimal duct (NLD) obstruction. DESIGN: Prospective, randomized trial. METHODS: Seventy eyes of 70 patients with incomplete NLD obstruction and severe epiphora (Munk score grade 3 or 4) were treated randomly with dacryocystoplasty (Lacricath) and silicone intubation (Ritleng; n = 35, group 1; mean age, 54.4 years; standard deviation [SD], 11.8 years) or silicone intubation alone (n = 35, group 2; mean age, 53.5 years; SD, 13.1 years; P > .05). The silicone tubes were removed after, on average, three months. At the visit, we assessed the grade of epiphora using the Munk score. Complete success was defined as Munk score of 0 or 1, partial success was defined as Munk score of 2, and failure was defined as Munk score of 3 or 4. Long-term Munk scores were obtained through a telephone survey nine to 76 months after surgery (mean, 43.4 in group 1 and 34.9 in group 2; P > .05). RESULTS: Complete success was reported by 18 patients (52%) in group 1 and by 20 patients (57%) in group 2. Partial success was reported by one patient in group 1 and by one patient in group 2. No improvement was reported by 15 patients (44%) in group 1 and by 14 patients (40%) in group 2. Differences between the two groups proved to be not significant (P = .8, exact Chi-square trend test). CONCLUSIONS: In our patients with acquired partial NLD obstruction, treatment with a combination of antegrade dacryocystoplasty and silicone intubation was not associated with a higher success rate compared with treatment with silicone intubation alone.

Corneal topography for pancorneal toric edge rigid gas-permeable contact lens fitting in patients with keratoconus, and differences in age and gender.

PURPOSE: To determine whether pancorneal rigid gas-permeable toric edge contact lenses (pancorneal RGP-CL) fitting could be improved by using non-central corneal shape predictors, and to compare these parameters with respect to age and gender in patients with keratoconus. METHODS: The current study was a retrospective study including patients with clinically evident keratoconus (on videokeratography) from three medical centres located in the Netherlands. Medical history, visual acuity, refraction, and contact lens characteristics were documented. All participants underwent ophthalmic examination including measurement of K-values (steepest, flattest, mean, as a non-central parameter the minimum radius [K-minimum], and all combined) using corneal topography. Fitting of contact lenses was based on the fluorescence pattern as seen during slitlamp examination. RESULTS: A total of 378 eyes of 218 patients with keratoconus were included. Of these, 257 (68%) were fitted with a pancorneal RGP-CL. The central K-values, K-steep and K-flat, performed well in predicting the base curve radius, though, for pancorneal RGP-CL the non-central K-minimum added most (35.5%) to the total explained variance in base curve radius. Regarding age and gender, women tended to have larger K-values than men. No differences in K-values were found with respect to age. DISCUSSION: We found that including the K-minimum as a non-central parameter may improve prediction of the best base curve radius for pancorneal RGP-CL and may reduce the number of trial contact lenses. In line with other studies, suggestive evidence was found for a difference in pattern of progression of keratoconus with gender, but not with age.

Intrinsic and extrinsic risk factors for sagging eyelids.

IMPORTANCE: Sagging eyelids, or dermatochalasis, are a frequent concern in older adults. It is considered a feature of skin aging, but risk factors other than aging are largely unknown. OBJECTIVE: To study nongenetic and genetic risk factors for sagging eyelids. DESIGN: Upper eyelid sagging was graded in 4 categories of severity using digital photographs. Dermatochalasis was defined as the eyelid hanging over the eyelashes. Age, sex, skin color, tanning ability, hormonal status in women, current smoking, body mass index, and sun protection behavior were analyzed in a multivariable multinomial logistic regression model. Genetic predisposition was assessed using heritability analysis and a genome-wide association study. SETTING AND PARTICIPANTS: The study was performed in 2 independent population-based cohorts. The Rotterdam Study included older adults from one district in Rotterdam, the Netherlands, and the UK Adult Twin Registry (TwinsUK) included twins from all over the United Kingdom. Participants were 5578 unrelated Dutch Europeans (mean age, 67.1 years; 44.0% male) from the Rotterdam Study and 2186 twins (mean age, 53.1 years; 10.4% male) from the TwinsUK. MAIN OUTCOMES AND MEASURES: Sagging eyelid severity levels, ranging from 1 (normal control) to 4 (severe sagging). RESULTS: Among 5578 individuals from the Rotterdam Study, 17.8% showed dermatochalasis (moderate and severe sagging eyelids). Significant and independent risk factors for sagging eyelids included age, male sex, lighter skin color, and higher body mass index. In addition, current smoking was borderline significantly associated. Heritability of sagging eyelids was estimated to be 61% among 1052 twin pairs from the TwinsUK (15.6% showed dermatochalasis). A meta-analysis of genome-wide association study results from 5578 Rotterdam Study and 1053 TwinsUK participants showed a genome-wide significant recessive protective effect of the C allele of rs11876749 (P = 1.7 × 10(-8)). This variant is located close to TGIF1 (an inducer of transforming growth factor ß), which is a known gene associated with skin aging. CONCLUSIONS AND RELEVANCE: This is the first observational study to date demonstrating that other risk factors (male sex, genetic variants, lighter skin color, high body mass index, and possibly current smoking) in addition to aging are involved in the origin of sagging eyelids.

Refractive, topographic, and visual outcomes of same-day corneal cross-linking with Ferrara intracorneal ring segments in patients with progressive keratoconus.

PURPOSE: To report refractive, topographic, and visual outcomes 12 months after same-day treatment with corneal cross-linking (CXL) and Ferrara intracorneal ring segments (ICRS) in eyes with progressive keratoconus (KC) and contact lens intolerance. METHODS: This was a case series of 7 eyes (7 patients) with progressive mild to moderate keratoconus and contact lens intolerance undergoing implantation of Ferrara ICRS immediately followed by corneal cross-linking. Uncorrected visual acuity (UCVA), best spectacle-corrected visual acuity (BSCVA), refractive error, pachymetry, and keratometry were evaluated at 3, 6, and 12 months of follow-up. RESULTS: Mean follow-up was 11.7 ± 3.6 months (range, 5-17 months). Mean preoperative UCVA and BSCVA were 0.10 ± 0.07 (range, 0.05-0.2) and 0.56 ± 0.08 (range, 0.5-0.7), respectively. One year after the treatment, mean UCVA and BSCVA measured 0.60 ± 0.24 (range, 0.32-0.9) and 0.82 ± 0.25 (range, 0.5-1.2), respectively. The mean spherical equivalent decreased significantly (P < 0.05) with 3.5 diopters (D). Mean K values (average keratometry of the cornea) decreased from 46.81 ± 2.13 D (range, 44-51 D) to 43.97 ± 2.22 D (range, 42-47.5 D) 1 year after the treatment. The average preoperative thinnest pachymetry measured 462 ± 46 µm (range, 410 ± 546 µm) and did not change significantly after the treatment. In patient 1, the inferior ICRS was removed 5 months postoperatively because of implant migration. CONCLUSIONS: The combined treatment of corneal cross-linking and Ferrara ICRS is a safe procedure that may be considered in patients with progressive mild to moderate KC and contact lens intolerance.

A posterior khodadoust line in a graft rejection episode after descemet stripping automated endothelial keratoplasty.

PURPOSE: To describe the presence of an endothelial rejection line in immunological graft rejection after Descemet stripping automated endothelial keratoplasty (DSAEK). METHODS: Case report. RESULTS: A 77-year-old woman underwent DSAEK procedure of the left eye because of visual limiting bullous keratopathy. One week postoperatively, the donor lamella was partially detached and an additional air bubble was injected into the anterior chamber. Thereafter, complete adherence of the lamella was seen. Four months after surgery, vision deteriorated, and a transient immunological graft rejection episode with endothelial rejection line was seen. CONCLUSIONS: In contrast to other reports, an endothelial rejection line (Khodadoust line) can be seen during the endothelial rejection episode after DSAEK.

Not only hard contact lens wear but also soft contact lens wear may be associated with blepharoptosis.

OBJECTIVE: The authors attempt to establish an association between prolonged hard and soft contact lens wear and ptosis. DESIGN: Single-center retrospective consecutive series. PARTICIPANTS: All patients between 18 and 50 years of age who were diagnosed with unilateral or bilateral ptosis between January 2002 and December 2005 (35 patients). METHODS: In a retrospective consecutive series, we included all patients between 18 and 50 years of age, with unilateral or bilateral ptosis between January 2002 and December 2005. Patients with congenital ptosis, ophthalmic surgery or disease, trauma, giant papillary conjunctivitis, unknown duration of contact lens wear, or muscular or neurologic disorders were excluded. We compared this study group to a Dutch reference population (the total underlying population from which the ptosis cases derive). RESULTS: The group included 35 patients: 20 (57%) (ages 18 to 50 years, average 37 years) had been wearing hard contact lenses for, on average, 17.6 years (range 6 to 27 years); 9 (26%) (ages 18 to 45 years, average 30 years) had been wearing soft contact lenses for, on average, 9 years (range 1.5 to 20 years); and 6 (17%) (ages 23 to 39 years, average 33 years) had no history of contact lens wear. The odds ratio for soft contact lenses was 14.7 (4.2 to 50.7; CI = 95) and for hard contact lenses 97.8 (22.5 to 424). CONCLUSIONS: This study suggests that not only hard contact lens wear but also soft contact lens wear may be associated with ptosis.

Endothelial cell decay after descemet's stripping automated endothelial keratoplasty and top hat penetrating keratoplasty.

PURPOSE: To analyze endothelial cell density (ECD) decay after Descemet's stripping automated endothelial keratoplasty (DSAEK) and top hat keratoplasty (THPK) in patients with Fuchs' endothelial dystrophy (FED) and/or pseudophakic bullous keratopathy (PPBK). METHODS: Patients underwent either THPK (n = 33) or DSAEK (n = 39) at the Erasmus Medical Center, Rotterdam. For each nonrandomized cohort, a biexponential regression model for ECD decay was fitted. Factors associated with higher ECD decay were evaluated. RESULTS: Median follow-up was 31.2 months (range, 11-91) in the THPK cohort, and 23.4 months (range, 6-61) in the DSAEK group. The early ECD decay was much higher after DSAEK (half time, 2.2 months) than after THPK (half time, 12.8 months). The late ECD decay after DSAEK was less steep (half time, 75.5 months) than after THPK (half time, 62 months). The 1-, 3- and 5-year endothelial cell losses derived from the models after DSAEK were 56%, 66%, and 73%, respectively, and after THPK were 24%, 50%, and 64%, respectively. For the DSAEK cohort, PPBK as an indication for surgery was associated with significantly higher late-phase decay rates. For the top-hat cohort, a significantly lower late-phase decay rate was found in PPBK. FED and same-session cataract surgery were confounding variables in the DSAEK cohort. Regarding DSAEK, postoperative re-bubbling was not found to have significant effects on early or late ECD decay rates. However, the small sample size and other limitations related to the method of evaluation may have influenced these findings. CONCLUSIONS: After DSAEK, early ECD decay was stronger than after THPK, as opposed to late decay. Late decay was faster for PPBK than for FED after DSAEK.

Long-term follow-up of hydrogel intracorneal lenses in 2 aphakic eyes.

We report the outcome of hydrogel intracorneal lens implantation in 2 patients. The lenses were implanted at approximately 50% depth in the cornea to correct high hyperopic refractive errors of 10.5 diopters (D) and 14.0 D, respectively. Both patients were contact lens intolerant and not suitable for intraocular lens implantation. Surgery was performed in 1988, and the patients were followed until early 2010. The patients showed good tolerance for the intracorneal lenses, but both developed opacities around the implant, leading to reduced visual acuity in 1 patient. Long-term patient monitoring is essential since corneal opacities can develop after many years. Removing the implant is not necessary as the lens can easily be rinsed by lifting the corneal cap.