Descemet Membrane Endothelial Keratoplasty versus Ultrathin Descemet Stripping Automated Endothelial Keratoplasty: A Multicenter Randomized Controlled Clinical Trial.

PURPOSE: To compare best spectacle-corrected visual acuity (BSCVA), endothelial cell density (ECD), refractive astigmatism, and complications after Descemet membrane endothelial keratoplasty (DMEK) and ultrathin Descemet stripping automated endothelial keratoplasty (UT-DSAEK). DESIGN: Prospective, multicenter randomized controlled trial. PARTICIPANTS: Fifty-four pseudophakic eyes of 54 patients with corneal endothelial dysfunction resulting from Fuchs endothelial corneal dystrophy were enrolled in 6 corneal centers in The Netherlands. METHODS: Participants were allocated to DMEK (n = 29) or UT-DSAEK (n = 25) using minimization randomization based on preoperative BSCVA, recipient central corneal thickness, gender, age, and institution. Donor corneas were prestripped and precut for DMEK and UT-DSAEK, respectively. Six corneal surgeons participated in this study. MAIN OUTCOME MEASURES: The primary outcome measure was BSCVA at 12 months after surgery. RESULTS: Central graft thickness of UT-DSAEK lamellae measured 101 µm (95% confidence interval [CI], 90-112 µm). Best spectacle-corrected visual acuity did not differ significantly between DMEK and UT-DSAEK groups at 3 months (0.15 logarithm of the minimum angle of resolution [logMAR] [95% CI 0.08-0.22 logMAR] vs. 0.22 logMAR [95% CI 0.16-0.27 logMAR]; P = 0.15), 6 months (0.11 logMAR [95% CI 0.05-0.17 logMAR] vs. 0.16 logMAR [95% CI 0.12-0.21 logMAR]; P = 0.20), and 12 months (0.08 logMAR [95% CI 0.03-0.14 logMAR] vs. 0.15 logMAR [95% CI 0.10-0.19 logMAR]; P = 0.06). Twelve months after surgery, the percentage of eyes reaching 20/25 Snellen BSCVA was higher in DMEK compared with UT-DSAEK (66% vs. 33%; P = 0.02). Endothelial cell density did not differ significantly 12 months after DMEK and UT-DSAEK (1870 cells/mm2 [95% CI 1670-2069 cells/mm2] vs. 1612 cells/mm2 [95% CI 1326-1898 cells/mm2]; P = 0.12). Both techniques induced a mild hyperopic shift (12 months: +0.22 diopter [D; 95% CI -0.23 to 0.68 D] for DMEK vs. +0.58 D [95% CI 0.13-1.03 D] for UT-DSAEK; P = 0.34). CONCLUSIONS: Descemet membrane endothelial keratoplasty and UT-DSAEK did not differ significantly in mean BSCVA, but the percentage of eyes achieving 20/25 Snellen vision was significantly higher with DMEK. Endothelial cell loss did not differ significantly between the treatment groups, and both techniques induced a minimal hyperopic shift.

[Acute red eye]. / Een acuut rood oog, en dan?

The general physician as well as the medical specialist may be confronted with a patient with one or two red eyes. To be adequately equipped we answer a series of questions on diagnosis, treatment and referral of patients with red eyes after trauma, or with conjunctivitis, keratitis, scleritis, uveïtis, endophthalmitis or acute glaucoma. Refer to an ophthalmologist or not?

[Infectious keratitis and corneal transplantation in a 15-year-old patient wearing contact lenses]. / Bedreiging van het oog bij contactlensgebruik.

BACKGROUND: Soft contact lens wearing increases the risk of an infectious keratitis. A patient present with a red painfull eye, in which case a corneal erosion (coloring with fluorescein) was seen by the general practitioner. It is possible that the clinical course worsens in a few days with a corneal perforation as a result. In these cases a timely referral is necessary. CASE: A fifteen year old soft contact lens wearing girl developed an infectious keratitis. Within a few days she developed a corneal perforation. As a result of an emergency transplantation within 24 hours the patient preserved her globe and her visual acuity recovered. CONCLUSION: Emergency transplantations usually have a complicated course with a high risk of re-transplantation and long-term pharmacological therapy. Such an emergency transplantation is primarily performed to preserve the globe. In the Netherlands a separate pool of emergency donor transplants is available, so that a transplantation can be performed within 24 hours.

Quality of vision and vision-related quality of life after Descemet membrane endothelial keratoplasty: a randomized clinical trial.

PURPOSE: To compare quality of vision and vision-related quality of life (QOL) in patients undergoing Descemet membrane endothelial keratoplasty (DMEK) or ultrathin Descemet stripping automated endothelial keratoplasty (DSAEK). METHODS: Fifty-four eyes of 54 patients with Fuchs' dystrophy from six corneal clinics in the Netherlands were randomized to DMEK or ultrathin DSAEK and examined preoperatively, and 3, 6 and 12 months postoperatively. Main outcome measures were corneal higher-order aberrations (HOAs), contrast sensitivity, straylight and vision-related QOL. RESULTS: Posterior corneal HOAs decreased after DMEK and increased after ultrathin DSAEK (p ≤ 0.001) 3 months after surgery and correlated positively with best spectacle-corrected visual acuity (12 months: r = 0.29, p = 0.04). Anterior and total corneal HOAs did not differ significantly between both techniques at any time point. Contrast sensitivity was better (p = 0.01), and straylight was lower (p = 0.01) 3 months after DMEK compared with ultrathin DSAEK; 95% confidence interval [CI] of log(cs) 1.10-1.35 versus 95% CI: 0.84 to 1.12, and 95% CI: log(s) 1.18 to 1.43 versus 95% CI: 1.41 to 1.66, respectively. Both were comparable at later time points. Vision-related QOL (scale 0-100) did not differ significantly between both groups at any time point and improved significantly at 3 months (ß = 12 [95% CI: 7 to 16]; p < 0.001), and subsequently between 3 and 12 months (ß = 5 [95% CI: 0 to 9]; p = 0.06). CONCLUSIONS: Descemet membrane endothelial keratoplasty (DMEK) results in lower posterior corneal HOAs compared with ultrathin DSAEK. Contrast sensitivity and straylight recover faster after DMEK but reach similar levels with both techniques at 1 year. Vision-related QOL improved significantly after surgery, but did not differ between both techniques.

Papilledema in isolated single-suture craniosynostosis: prevalence and predictive factors.

The purpose of this retrospective study was to assess the prevalence of papilledema in patients with isolated craniosynostosis. Second, we wanted to assess if the presence of ventricular dilatation on computed tomography (CT) scan is a predictive factor for the occurrence of papilledema. We included 205 consecutive children with an isolated single-suture craniosynostosis, who had at least 1 fundus examination. Preoperative CT scans of the brain were examined for the presence of ventricular dilatation.Papilledema developed in 14 of 205 patients: 10 developed papilledema before surgery and 4 during the follow-up period. Ten of the patients with papilledema had a synostosis of the sagittal suture, and 4 of the metopic suture. Prevalence of papilledema in scaphocephaly was 9.7%, and in trigonocephaly, 5.6%. Based on evaluation of all CT scans, ventricular dilatation seemed not to be a predictive factor for papilledema in children with isolated craniosynostosis.The incidence of papilledema in almost 10% of scaphocephaly patients is remarkably higher than expected. Therefore, we recommend routine preoperative screening, especially for patients with scaphocephaly, but also for patients with trigonocephaly. Postoperative screening is recommended in all patients when there is any uncertainty.

The influence of incision-induced astigmatism and axial lens position on the correction of myopic astigmatism with the Artisan toric phakic intraocular lens.

PURPOSE: To evaluate postoperative astigmatism with regard to incision-induced astigmatism and deviation in axial alignment with the use of preoperative limbal marking with the Javal keratometer (Haag Streit, Bern, Switzerland) in eyes implanted with the Artisan toric phakic intraocular lens (IOL) (Ophtec, Groningen, The Netherlands). DESIGN: Prospective nonrandomized trial. PARTICIPANTS: Fifty-four eyes of 33 patients with myopia (mean, -9.67 diopters [D]) and astigmatism (mean, -3.44 D). INTERVENTION: The enclavation site was marked on the limbus using the Javal keratometer. The Artisan toric phakic IOL was implanted according to the axis marked on the limbus. Follow-up was a minimum of 6 months. MAIN OUTCOME MEASURES: Safety index, efficacy index, predictability, safety, and vector analysis of total refractive correction were determined. The effects of axis misalignment and incision-induced astigmatism on the final refractive error were evaluated. RESULTS: At 6 months after surgery, the safety index was 1.29+/-0.29 and the efficacy index was 1.04+/-0.35. Mean spherical equivalent subjective refraction reduced from -11.39+/-4.86 D before surgery to -0.38+/-0.57 D at 6 months. Sixty-seven percent of eyes were within 0.50 D of attempted refraction and 89% were within 1.00 D. Mean preoperative cylinder was 2.92+/-1.60 D at 91.4 degrees . At 6 months, the mean cylinder was 0.28+/-0.54 D at 174.3 degrees . No eyes lost 2 or more lines of best-corrected visual acuity at 6 months. Eighty-three percent of eyes achieved uncorrected visual acuity of 20/40 and 28% achieved 20/20. Vector analysis of total surgically induced astigmatism revealed a mean cylindrical change of 3.21+/-1.71 D. Average axis misalignment was 0.37+/-5.34 degrees . The mean incision-induced astigmatism was 0.74+/-0.61 D at 0.2 degrees . CONCLUSIONS: Implantation of the myopic toric IOL leads to safe, efficacious, and predictable results. The level of unpredictability caused by minor axis IOL misalignment has minimal effects on the residual refractive error. The procedure of axis alignment with the Javal keratometer seems to be an accurate method of marking the eye for toric IOL implantation. Incision-induced astigmatism can result in an overcorrection of the cylinder. A systematic undercorrection of -0.50 D for attempted cylindrical outcome could result in an achieved correction closer to emmetropia.

Toric phakic intraocular lens for the correction of hyperopia and astigmatism.

PURPOSE: To evaluate the Artisan toric phakic intraocular lens (pIOL) for the correction of hyperopia and astigmatism. SETTING: Department of Ophthalmology, Erasmus MC, Rotterdam, The Netherlands, and Department of Ophthalmology, Sint Truiden, Belgium. METHODS: In this prospective study of 47 eyes of 28 patients with hyperopia and astigmatism, Artisan toric pIOLs were implanted between April 1999 and June 2004. Uncorrected visual acuity (UCVA), best corrected visual acuity, refraction, astigmatism, safety, and predictability were analyzed. Change in astigmatism was analyzed with vector analysis. Refractive cylinders are expressed in minus form. RESULTS: Mean preoperative spherical equivalent was +4.33 diopters (D) +/- 2.26 (SD). Mean follow-up was 11.1 months (range 6 to 36 months). A gain of 1 or more lines in best spectacle-corrected visual acuity (BSCVA) was seen in 36.2%. Safety index and efficacy index after 6 months were 1.06 and 0.87, respectively. The mean postoperative astigmatism at 6 months was 0.19 D at an axis of 144 degrees. At 6 months, about three quarters (76.6%) of the eyes had a UCVA of 20/40 or better. One eye lost 2 lines of BSCVA. In 1 eye, the lens position had to be changed because of a large axis misalignment. No serious complications developed in any of the treated eyes during follow-up. CONCLUSIONS: Artisan toric pIOLs can correct moderate to high hyperopia combined with astigmatism with good refractive results. In this study, there were no serious complications. However, the predictability of the refractive results appeared to be lower than those in the correction of myopia and astigmatism with toric Artisan lenses.

Comparison of complication rates and postoperative astigmatism between nylon and mersilene sutures for corneal transplants in patients with Fuchs endothelial dystrophy.

PURPOSE: The purpose of this study is to evaluate corneal astigmatism and suture-related complications for transplants sutured with nylon and transplants sutured with Mersilene in primary corneal transplants for Fuchs endothelial dystrophy. METHODS: A retrospective, observational, and comparative study between transplants sutured with either nylon 10-0 or 11-0 (n = 108) or Mersilene 11-0 (n = 58) was done. One hundred sixty-six eyes of 140 patients who received a primary penetrating keratoplasty for Fuchs endothelial dystrophy between 1995 and 2001 at the Rotterdam Eye Hospital in Netherlands were included. RESULTS: Overall, transplant survival did not differ between groups (log-rank test; P = 0.24). During the first 2 years after transplantation, significantly lower astigmatism was seen in transplants sutured with nylon (P = 0.03). Transplants sutured with Mersilene had a significantly higher risk of surgical intervention to correct astigmatism or wound dehiscence after transplantation (hazard ratio, 2.83; 95% confidence interval, 1.34-6.01). Time to first infiltrate, metaplasia (marked scarring along the sutures), or cheesewiring was significantly less in the Mersilene group (P < 0.01). There was a tendency toward a higher risk of complications associated with loose or broken sutures in the nylon group (hazard ratio, 2.00; 95% confidence interval, 0.57-6.98), which was more pronounced after 2 years. CONCLUSIONS: During the first years after corneal transplant surgery, Mersilene sutures are associated with a higher risk of complications and more interventions to correct suture-related problems. However, after 2 years, the grafts seem to be able to retain Mersilene sutures with less risk of suture-related complications as compared with grafts with retained nylon sutures. These favorable long-term effects of Mersilene sutures may outweigh its short-term disadvantage in patients with Fuchs endothelial dystrophy.

Implantation of Artisan toric phakic intraocular lenses for the correction of astigmatism and spherical errors in patients with keratoconus.

PURPOSE: To evaluate the correction of astigmatism and spherical ametropia in patients with keratoconus through implantation of an Artisan toric phakic intraocular lens (PIOL) (Ophtec, Groningen, The Netherlands). METHODS: Artisan toric PIOLs were implanted uneventfully in both eyes of three patients with keratoconus with clear central corneas and contact lens intolerance. RESULTS: Best spectacle-corrected subjective visual acuity after lens implantation was unchanged in one eye and improved in five eyes. Spherical equivalent refraction was significantly reduced in all eyes (P=.03). The safety index was 1.49. CONCLUSIONS: The implantation of an Artisan toric PIOL may be an alternative for treating astigmatism and myopia in contact lens intolerant patients with keratoconus with clear central corneas. Especially in patients with associated myopia, this procedure is worth considering before planning a penetrating keratoplasty.

Implantation of a toric phakic intraocular lens to correct high corneal astigmatism in a patient with bilateral marginal corneal degeneration.

We present a patient with marginal corneal degeneration and corneal astigmatism of more than 10.0 diopters (D). A toric phakic intraocular lens (IOL) of 7.0 D cylindrical power was implanted in both eyes to correct the high astigmatism. An uncorrected visual acuity of 20/40 was achieved in both eyes, and the best corrected visual acuity improved by 4 Snellen lines to 20/20 in both eyes. Refraction and visual acuity remained stable at 1.5 years postoperatively. Implantation of a toric phakic IOL can be an option to correct high corneal astigmatism even when the full corneal astigmatism cannot be treated.

Long-term outcome in high-risk corneal transplantation and the influence of HLA-A and HLA-B matching.

PURPOSE: To evaluate long-term follow-up of high-risk corneal transplants allocated after matching for broad HLA-A and HLA-B antigens and to establish whether matching for HLA-A and -B antigen "splits" would result in a reduced risk of immunologic graft failure. METHODS: A total of 303 high risk corneal transplants was included. Class I antigen-matched donor corneas were obtained using broad HLA-A and -B antigen data and accepting 0 or 1 mismatch at each locus. Analysis of HLA antigens was performed also on the split typing level. The influence on immunologic graft failure for an increasing number of matched class I antigens based on split typing was analyzed with Kaplan-Meier statistics and Cox regression. Graft survival and indication for transplantation were investigated. RESULTS: Rejection was the cause of 34% of all graft failures. A significantly higher immune failure free graft survival was found in a group with 0 or 1 HLA-A and -B mismatch based on split typing (log-rank test, P = 0.002). A beneficial effect of matching for split antigens was shown with multivariate analysis (odds ratio, 0.41). CONCLUSIONS: One third of graft failures in our high-risk population was caused by irreversible graft rejection. Allocation of donor corneas based on a 0 or 1 split antigen mismatch at both HLA-A and -B loci could contribute to a higher immune failure free graft survival and could result in a higher overall graft survival.

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.

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.

Manual trephination of mushroom keratoplasty in advanced keratoconus.

PURPOSE: To evaluate the technique of mushroom keratoplasty for patients with advanced keratoconus, with respect to postoperative astigmatism, visual improvement and stability, and endothelial cell count. METHODS: Sixteen eyes of 15 patients with advanced keratoconus received a mushroom keratoplasty at the Erasmus Medical Centre at Rotterdam between April 2003 and May 2006. The donor cornea had a larger anterior stromal lamella (9.0 mm) and a smaller posterior part (7.0 mm). Visual acuity and keratometry were measured at 6, 12, 24, and 36 months. Specular microscopy was performed annually. RESULTS: Best-corrected visual acuity improved significantly (P = 0.003) from 0.25 +/- 0.20 preoperatively to 0.62 +/- 0.22 at 6 months postoperatively. Visual acuity remained stable with a best-corrected visual acuity of 0.70 +/- 0.22 from 1 year onward. At all follow-up periods, mean postoperative astigmatism remained <3.5 D. Mean postoperative astigmatism at 1, 2, and 3 years measured 2.67 +/- 1.95 (n = 15), 2.54 +/- 1.86 (n = 6), and 3.30 +/- 2.12 D (n = 5). The average endothelial cell count at 1 and 2 years was 1755 +/- 678 (n = 12) and 1573 +/- 546 cells/mm2 (n = 5), respectively. At the end of follow-up, all transplants remained functional and clear. CONCLUSIONS: The mushroom-shaped graft can combine the benefits of a good visual acuity, a low astigmatism, and rapid postoperative healing. The short-term results of our limited series of patients are encouraging. Further research is necessary to draw stronger conclusions regarding the long-term effects.

Posterior mushroom keratoplasty in patients with Fuchs endothelial dystrophy and pseudophakic bullous keratopathy: transplant outcome.

PURPOSE: To report the outcome of posterior mushroom or top-hat-shaped keratoplasty for patients with Fuchs endothelial dystrophy (FED) or pseudophakic bullous keratopathy (PBK), concerning postoperative astigmatism, visual improvement, and endothelial cell density. METHODS: Twenty-two eyes of 21 patients who received a posterior mushroom keratoplasty for FED (n = 19) or PBK (n = 3) between March 2003 and January 2006 at the Erasmus MC, Rotterdam, The Netherlands, were included. Visual acuity, refraction, and keratometry were measured at 6, 12, 24, and 36 months. Specular microscopy was performed annually. RESULTS: Best-corrected visual acuity (BCVA) significantly (P < 0.05) improved from 0.25 preoperatively to 0.47 at 6 months and 0.64 at 1 year postoperatively. Visual acuity remained stable with a BCVA of 0.62 2 years and 0.69 3 years after keratoplasty. At the last follow-up, the BCVA measured 0.5 or more in all but 2 (91%) eyes. Mean postoperative astigmatism at 1, 2, and 3 years measured 2.72 +/- 1.48, 2.69 +/- 1.85, and 1.75 +/- 1.76 D. Endothelial cell density averaged 2143 +/- 428 cells/mm2 at 1 year, 1539 +/- 573 cells/mm2 at 2 years, and 1920 +/- 474 cells/mm2 at 3 years. All studied transplants remained functional and clear throughout the follow-up period. CONCLUSIONS: The posterior mushroom keratoplasty in FED and PBK results in a relatively good visual acuity, low astigmatism, and good endothelial cell density. The short-term results of our limited series of patients are encouraging.

Papilledema in patients with Apert, Crouzon, and Pfeiffer syndrome: prevalence, efficacy of treatment, and risk factors.

Patients with syndromic craniosynostosis are at risk for elevated intracranial pressure because of various physiologic and anatomic abnormalities. The aims of this study were to determine the prevalence of papilledema in syndromic craniosynostosis, to evaluate the results of the treatment, and to examine the risk factors. This is a retrospective study on 84 patients with Apert, Crouzon, or Pfeiffer syndrome. Papilledema was defined as blurring of the margins of the optic disk. The association between clinical symptoms, beaten-copper pattern on skull radiograph, ventricular dilatation on computed tomography scan, and papilledema was assessed. Papilledema was present in 51% of the patients. No relation between specific clinical symptoms and papilledema was found. The significant associations were complex craniosynostosis, exorbitism, and ventricular dilatation. The prevalence of papilledema in patients with Apert, Crouzon, or Pfeiffer syndrome is high, not only before cranial decompression but also after vault expansion. Annual fundoscopy is recommended to screen for papilledema. We consider that early decompressive surgery (within the first year of age) prevents the development of papilledema and, most likely, elevated intracranial pressure.

Visual loss in syndromic craniosynostosis with papilledema but without other symptoms of intracranial hypertension.

Four children with syndromic craniosynostosis (Crouzon's syndrome and Apert syndrome) developed irreversible visual loss. Apart from papilledema, there were no other clinical symptoms of intracranial hypertension. Ventriculomegaly or hydrocephalus was present in all cases. Two children were known to have obstructive sleep apnea syndrome. These cases are reported to inform specialists involved in follow-up of children with syndromic craniosynostosis that visual loss can develop suddenly without other symptoms of intracranial hypertension. To prevent visual loss, papilledema should be detected at an early stage and intervention should be instituted promptly. Therefore, periodic funduscopy should be performed in children at risk, such as children with syndromic craniosynostosis and additional hydrocephalus or obstructive sleep apnea syndrome.