Having an eye for myotonic dystrophy: A qualitative study on experiences and support needs in myotonic dystrophy type 1 patients with a diagnostic delay after early-onset cataract.

Myotonic dystrophy type 1 is a neuromuscular disorder affecting multiple organ systems and is characterized by a variety of clinical presentations. Anticipation leads to an earlier and more severe phenotype in subsequent generations. Early-onset cataract is a common initial manifestation of the late or adult-onset type of myotonic dystrophy 1. Due to its multicausal nature, early-onset cataract is often not recognized as a feature of this disease, leading to diagnostic delay resulting in consequences for successive generations, treatment and counseling. A qualitative study with semi-structured interviews was performed with purposive sampling of eight participants with myotonic dystrophy type 1 and early-onset cataract to investigate the physical and psychosocial consequences experienced due to diagnostic delay. Prior to the early-onset cataract, all participants experienced other multisystem symptoms that could have been explained by myotonic dystrophy. The diagnostic delay had severe hereditary consequences: a subsequent generation with more severely affected (grand)children was born resulting in large emotional burden for the patients. To conclude, early-onset cataract is a warning sign and ophthalmologists play a crucial role in the early detection of myotonic dystrophy type 1 by recognizing this symptom and preventing the birth of severely affected children leading to emotional and psychosocial consequences.

Holmium laser thermal keratoplasty for hyperopia and astigmatism after photorefractive keratectomy.

PURPOSE: To report results of holmium laser thermal keratoplasty used to treat induced hyperopia and induced, as well as pre-existing astigmatism after photorefractive keratectomy. METHODS: Sixteen eyes of 16 patients were included in this study. Contact holmium laser (Technomed Holmium 25) was used in 7 patients to correct hyperopia (8 spots at 8 or 9 mm) and in 9 patients to correct astigmatism (4 spots at 7, 8, or 9 mm). Follow-up evaluation was done after at least 6 months. The effectiveness, stability, and safety of the procedure were investigated. RESULTS: Spherical correction was ineffective (1.00 D or less) when applied at the 9-mm diameter treatment zone. Spherical correction applied at the 8-mm diameter treatment zone was ineffective in 1 eye. Three eyes achieved 1.00 to 2.00 D change, but 2 of these eyes showed an induced astigmatic change as well. Correction of astigmatism at the 7-mm diameter treatment zone resulted in a 0 to 4.00 D cylinder component change. Treatment at the 8-mm diameter treatment zone showed a 0 to 1.50 D effect and at the 9-mm treatment zone, 0.25 to 1.50 D. All eyes that achieved significant improvement (1.00 D or more change in cylinder component) showed significant overcorrection in the first postoperative phase. There were no sight threatening complications. CONCLUSION: Holmium laser thermal keratoplasty can be useful for the treatment of overcorrection and induced as well as pre-existing astigmatism after photorefractive keratectomy. However, predictability is low and astigmatism can be induced with the attempted spherical correction.

Treatment of hyperopia with contact Ho:YAG laser thermal keratoplasty.

PURPOSE: To evaluate the effectiveness, safety, and stability of contact Ho:YAG laser thermal keratoplasty for low to moderate hyperopia. METHODS: Fifty-five hyperopic eyes of 39 patients were treated with a Technomed contact Ho:YAG laser; 23 eyes were treated a second time. Treatment parameters were 1 octagonal ring of 8 spots with a treatment diameter of 6 mm, 7 mm, or 8 mm. Efficacy of the Ho:YAG laser treatment was evaluated after 6 months, comparing 3 treatment zone diameters. Stability and efficacy after 12 months was evaluated comparing 7-mm and 8-mm treatment zone diameters. RESULTS: Mean reduction of spherical equivalent refraction after 6 months was not statistically significantly different between the 6-mm or 7-mm diameter zones: 1.42 (+/- 1.30) D versus 2.22 (+/- 0.44) D. An 8-mm diameter treatment zone was significantly less effective, 1.12 (+/- 0.47) D. Longer follow-up did not show stability: mean reduction of spherical equivalent manifest refraction was 1.58 (+/- 0.45) D for the 7-mm diameter treatment zone and 0.82 (+/- 0.61) D for the 8-mm diameter treatment zone after approximately 12 months. Retreatment had a limited additive effect. No clinically significant loss of spectacle-corrected visual acuity was reported. No eyes lost more than 1 line of visual acuity. CONCLUSION: Contact Ho:YAG laser thermal keratoplasty corrected hyperopia up to 2.50 D, but predictability was poor and a regression of initial effect occurred. Instability of refraction persisted to 1 year after surgery.

Photorefractive keratectomy with an ablatable mask for myopic astigmatism.

PURPOSE: To evaluate efficacy, safety, and stability of photoastigmatic keratectomy (PARK) carried out with a Summit Apex Plus laser using an ablatable mask. METHODS: Forty-one eyes of 41 patients with myopic astigmatism with follow-up of 12 months were evaluated. Treatment efficacy was compared in groups with high (>6.00 D) versus low (< or =6.00 D) preoperative spherical equivalent subjective manifest refraction, in groups with high (>2.00 D) versus low (< or =2.00 D) preoperative cylindrical component and in groups divided according to preoperative axis of cylinder. RESULTS: At 12 months after surgery, mean spherical equivalent manifest refraction in all 41 eyes was -0.30 +/- 0.90 D. Mean cylinder component was 0.60 +/- 0.70 D. Mean reduction in astigmatic component was 67 +/- 47%. Uncorrected visual acuity of 0.5 or more was achieved in 79% of eyes; 71% of eyes achieved 0.8 or more. At 1 month after surgery, 49% of eyes had a loss of 2 or more lines of spectacle-corrected visual acuity. This loss was restored at 12 months. No statistically significant differences were found between the different subgroups. CONCLUSION: Photoastigmatic keratectomy with ablatable mask gives satisfactory results. No relation in efficacy was found when taking into account the amount of preoperative spherical component, the cylindrical component, or the cylinder axis direction.

Bilateral, anterior stromal ring opacity of the cornea.

AIMS/BACKGROUND: To describe a bilateral, mid peripheral, ring-shaped corneal opacity, not resembling any known corneal degeneration, dystrophy, or other disorder, and occurring without ocular or systemic disease. METHODS: Ophthalmic examination, haematological screening, and ultrasound biomicroscopy. RESULTS: A 25 year old man showed grey-white, granular opacities in both corneas, with an 8 mm diameter ring configuration, and a V-shaped distribution in the anterior stroma. The surrounding corneal stroma was clear, and the tear film, the epithelium and its basement membrane, Descemet's membrane, and the endothelium were normal. Evidence of systemic disease was not found. Family members did not show corneal abnormalities. CONCLUSION: A bilateral corneal ring opacity may occur in healthy, asymptomatic, young people. These corneal rings may result from depositions of unknown origin, or possibly a rare corneal dystrophy.

[Acanthamoeba keratitis]. / Acanthamoeba-keratitis.

Three patients, women aged 69, 25 and 30 years, with refractory keratitis were found to have a rare infection with Acanthamoeba. All wore soft contact lenses. The frequency of Acanthamoeba keratitis increased in recent years. At present, an efficacious antiprotozoal therapy is available, for instance in the form of polyhexamethylene biguanide and propamidine. In cases of refractory keratitis, the possibility of an Acanthamoeba infection should be taken into account.

[Results of photorefractive keratectomy using the excimer laser in the treatment of myopia; 1-year follow-up]. / Resultaten van fotorefractieve keratectomie met de excimer-laser ter behandeling van myopie; 1 jaar follow-up.

OBJECTIVE: Evaluation of the first excimer laser treatments of myopia. DESIGN: Descriptive. SETTING: Excimer Laser Centrum, Department of Ophthalmology, University of Nijmegen, Nijmegen, the Netherlands. METHOD: 312 patients underwent spherical excimer laser treatment to correct myopia of 1.2 up to 10 diopters between February 1992 and October 1993. 245 patients completed a follow-up of one year or more; 36 retreatments were carried out. Group I (treatment 1.2 to 6 D) numbered 174 patients, group II (6.1-10 D) 71 patients. RESULTS: After a follow-up period of one year or just before retreatment 79% of group I and 48% of group II achieved a refractive correction within 1 D of the attempted correction. Visual acuity without correction was 0.5 or more in 94% of group I and in 76% of group II. Less than one percent (n = 1) of group I and 6% (n = 4) of group II lost more than one line of best corrected visual acuity. Retreatment could correct 50% of those eyes that did not achieve a refraction within 1 D of attempted correction. Loss of visual acuity was corrected by retreatment in 5 of 6 cases of group I and in 7 of 11 cases of group II. CONCLUSION: Based on a one-year follow-up, refractive surgery with the excimer laser appears to correct myopia between 1 and 10 D effectively. Predictability is diminishing on correcting higher amounts of refractive error. Thorough information of the patients regarding the results to be expected will prevent disappointment.

[Ocular infection by Pseudomonas aeruginosa in a mechanically ventilated patient]. / Ooginfectie door Pseudomonas aeruginosa bij een mechanisch beademde patiënt.

A 59-year-old man developed bilateral keratitis several weeks after the initiation of mechanical ventilation because of respiratory failure and sepsis following abdominal surgery. Colonisation of the upper airways by P. aeruginosa had been established before. Invasion through corneal epithelial defects based on dehydration keratitis was the presumed route of infection. Despite aggressive treatment, including antibiotics, the infection was rapidly progressive in both eyes. The patient died of deterioration of his general condition. In order to prevent such eye infections in a patient on mechanical ventilation, there is a need of good eye care, prevention of corneal lesions and alertness, especially when the patient is colonised by virulent micro-organisms like P. aeruginosa.

Peters' anomaly: an unusual case.

The authors report on a case of congenital bilateral protruding corneal opacities. Clinical and histological examination was consistent with a diagnosis of Peters' anomaly with a defect in the endothelium and Descemet's membrane but without abnormalities of iris and lens. Corneal grafting was performed on both eyes. During a follow-up period of one year the corneas have remained clear. However, surgical intervention for glaucoma has been necessary.

Severe keratitis due to Nocardia farcinica.

Keratitis due to Nocardia farcinica occurred in a 49-year-old female after inappropriate cleaning of her semipermeable rigid contact lenses with basin-stored water during a holiday in France. N. farcinica was differentiated from Nocardia asteroides by its growth at 45 degrees C, acid production from rhamnose, its opacification of Middlebrook 7H10 agar, and its marked degree of resistance to all cephalosporins, aminoglycosides, tetracyclins, macrolides, and trimethoprim-sulfamethoxazole. To the best of our knowledge, this is the first reported case of human N. farcinica keratitis, confirming that this microorganism can be responsible for serious human disease.

Ocular hypotony after laser suture lysis following trabeculectomy with mitomycin C.

BACKGROUND: To report our experience with laser suture lysis (LSL) following trabeculectomy with mitomycin C, its timing, effectiveness and related complications. METHODS: We retrospectively examined 38 consecutive eyes of 36 patients that underwent LSL following trabeculectomy with mitomycin C. RESULTS: The mean preLSL intraocular pressure (IOP) was 27.0 mm Hg (SD 6.3, range 16-39 mm Hg) and the postLSL IOP (IOP 1 h after the last session of LSL) was 16.0 mm Hg (SD 7.2, range 3-31 mm Hg). Following the LSL in 7 eyes (7 of 38) hypotony (IOP<6 mm Hg, lasting more than 24 h) developed. Two groups were defined. In group I no hypotony was found after LSL and group II went through a period of hypotony. The time interval between surgery and LSL was significantly shorter in group II (mean 5.7, SD 7.5, range 1-19 days), compared to group I ( mean 14.7, SD 13.0, range 1-44 days) (p=0.041). The mean final IOP (IOP measured at the last visit) was 13.3 mm Hg (SD 3.4, range 6-20 mm Hg) and mean follow-up was 6.1 months (SD 3.9, range 2.0-15.2 months). No hypotony was found at final examination. PostLSL IOP and final IOP were significantly lower in group II (p=0.002 and p=0.024 respectively). IOP reduction by LSL was significantly greater in group II (p=0.046). CONCLUSION: LSL is an effective and safe procedure to lower the IOP following trabeculectomy with mitomycin C. Early application of LSL results in lower final IOPs, but has a higher risk of hypotony.

Dominant inherited tilted disc syndrome and lacquer cracks.

Three patients with the tilted disc syndrome from one family were examined. The presence of the trait in three consecutive generations suggests an autosomal dominant mode of inheritance, although in these patients with variable expression. The propositus showed bilateral inferonasal retinal ectasia, with atrophic subretinal scars. Linear-like lacquer cracks, radiating from the central scars, were also present running parallel to the margin of the optic nerve head. The linear streaks were very similar to those usually seen in traumatic tears of Bruch's membrane. The mechanical stretching of the ectatic area, and its abnormal location inferonasal to the optic disc might have been responsible for the unusual pattern of the lacquer cracks in our patient.

Toxic keratopathy due to the accidental use of chlorhexidine, cetrimide and cialit.

Due to economical reasons some ophthalmologists are using an irrigating solution made by the hospital pharmacy instead of the commercially available solutions. These irrigating solutions come in bottles which are identical to the ones used for other solutions. During the last three years bottles were accidentally mixed up five times. Consequently, bottles containing solutions such as chlorhexidine, cetrimide, chlorhexidine/centrimide and cialit solutions were used during cataract surgery. This resulted in immediate corneal edema which, in its turn resulted in a bullous keratopathy. Four patients underwent a penetrating keratoplasty. In one patient the cornea was covered with a conjunctival flap. Light microscopy of the corneas included epithelial edema, loss of keratocytes, and a disrupted and sometimes absent endothelial cell layer.