Search

Background: Because of the retinal alterations visual acuity of patients with retinitis pigmentosa is more affected by loss of contrast than in normal persons. Therefore cataract in retinitis pigmentosa (mostly posterior subcapsular cataract) leads earlier to decreasing visual acuity than is expected from the lens opacity. In a retrospective study we have looked up all patients with retinitis pigmentosa and cataract which were operated between 1989 and 1997 at our department., Patient and Methods: Between 1989 and 1997 50 eyes of 36 patients (21 female, 15 male; mean age 51 +/- 11.4 years) with the diagnosis of retinitis pigmentosa and cataract underwent extracapsular cataract extraction and IOL-implantation. In a retrospective study we studied all case histories of these patients. Mean follow up time was 1.2 +/- 1.2 years., Results: Preoperative visual acuity was 0.1 +/- 0.1, best postoperative visual acuity was 0.3 +/- 0.2. Visual field was not affected by cataract-surgery. Postoperative complications were not found. 2/3 of all patients were satisfied with the postoperative visual acuity, 1/3 of all patients did not give comments., Conclusion: In patients with retinitis pigmentosa cataract surgery leads to significant increase of visual acuity. Cataract extraction should not be with held from patients with retinal dystrophy.

Background: Retinitis pigmentosa (RP) is associated with the development of a posterior subcapsular cataract (PSC). The development of posterior capsule opacification (PCO) after cataract surgery and a decrease of central visual acuity is sometimes misinterpreted by the patients as natural course of RP. Therefore, therapeutic intervention is often delayed., Patients and Methods: In a retrospective study (part 1) the incidence of PCO was evaluated in a group of 26 RP patients who underwent cataract surgery and IOL implantation. In a prospective analyse (part 2) PCO was quantified in 13 RP patients using the standardized photographic technique and image analysis system introduced by Tetz et al. Matched pairs were formed with a control group of 13 patients without retinal disease who matched the RP group in terms of age distribution and postoperative follow-up time. In part 3 the parameters of Nd:YAG laser capsulotomy in 12 RP patients and 14 controls were evaluated., Results: Part 1: The cumulative PCO rate in RP at the end of the first postoperative year was 14.6%, 26.8% in the second, 53.7% in the third and 70.7% after the third year. Nd:YAG laser capsulotomy was performed in 70% of eyes (after 18.4 +/- 14.7 months). In eyes with significant PCO development 70% had PSC preoperatively, while in eyes without PCO formation only 41.7% showed PSC. Part 2: The matched pairs analysis showed a significantly higher PCO value for RP patients (2.11 +/- 1.42) than for the control group (0.89 +/- 0.72) (P = 0.038). Part 3: Average Nd:YAG laser energy levels were 12.8 +/- 11.2 MJ (RP) and 7.6 +/- 6.7 MJ (control). Some 25% of RP patients required further laser treatment of regrown secondary cataract., Conclusions: Patients with RP showed a significantly higher incidence and density of PCO. Whether RP-specific pathomechanisms are responsible for this needs further investigation.

Background: Retinitis pigmentosa (RP) is associated with the development of posterior subcapsular cataract (PSC). Due to their retinal pathology, RP patients need optimal contrast conditions to attain good visual acuity. Lens opacities like PSC, therefore, decrease central visual acuity in RP patients earlier and more markedly than in patients without retinal problems., Patients and Methods: We examined 39 patients aged 52.2 +/- 15.4 years who had underwent 61 cataract operations with intraocular lens implantation 1-4 years previously. Patients with autosomal dominant (n = 7), autosomal recessive (n = 1), X chromosomal recessive (n = 2), and simplex RP (n = 21), as well as eight patients with Usher's syndrome (RP and sensoneurinal deafness), were evaluated., Results: Visual acuity (VA) of all patients increased from 0.17 +/- 0.13 preoperatively to 0.33 +/- 0.22 postoperatively. The mean age of the patients at the onset of RP was 28.8 +/- 19.6 years. The average time between subjective onset of RP and cataract operation was 20 years. Patients with early manifestation of RP, before the age of 20 years, had significantly lower postoperative VA (P = 0.0005) than patients with late manifestation. A short duration of RP, less than 20 years, was associated with significantly better postoperative VA (P = 0.016). The surgical trauma of the cataract operation did not influence the course of RP. Visual field testing did not show statistically significant differences between preoperative and postoperative (1-4 years) results. The development of clinically significant posterior capsule opacification was observed in 70.7% of all RP patients., Conclusions: Patients with RP and cataract should be operated early to provide, for as long as possible, an optimal optical image to compensate and support the inferior retinal function. Early onset of RP symptoms and longer duration of the disease have an negative impact on postoperative visual outcome in cataract surgery.

Coats disease itself is a rather rare condition, whereas Coats syndrome has been observed in about 1.2-4.9% of all retinitis pigmentosa (RP) patients. The spectrum of clinical presentations and our therapeutic experience are illustrated with reference to case reports of three patients with clinically and electrophysiologically verified RP and Coats syndrome. As in classic Coats disease, Coats-like changes with RP are defined by aneurysmatic and teleangiectatic vessels and intra- and subretinal lipid deposits leading to exudative retinal detachment. Both entities differ with regard to age of onset, gender, bilaterality and location of the retinal changes. The reports published up to now show that there is no consensus concerning the success of the different treatments of Coats syndrome associated with RP. Laser therapy, cryocoagulation, diathermy and buckling procedures have been suggested for treatment of this complex. As in the therapy of Coats disease, our results confirm that the outcome of laser therapy depends on early detection of Coats-type vascular abnormalities and early initiation of the treatment. Therefore, thorough fundus examination should be regularly performed in all RP patients.

According to the literature, a fully developed cataract should be removed generally when the macula is intact. However, patients with a slowly progressing retinal disease find themselves in a particularly difficult situation. In this clinical, retrospective study, 24 retinitis-pigmentosa (RP) patients after cataract surgery were asked whether they recommend cataract surgery early or late in the course of the development of the retinal disease. A correlation was found between the answers to this question and the time elapsed between each patient's own operation and his interview. Three preference groups resulted through statistical evaluation: 1) those who recommend an early operation (n = 8), 2) those who recommend a late operation (n = 8), and 3) those who were undecided (n = 8). All patients had been operated at the same time following the RP onset and cataract diagnosis. Those patients who preferred a late operation had been questioned in this regard considerably later after their own operation than those who preferred an early operation. In other words, the preference for an early or late operation depended on how much prior to the questioning the patient's own operation had been conducted. The RP patient, quite disease conscious, has an exact conception of the ideal time of cataract operation in RP patients in general, but this does not always coincide with the opinion of his physician. It is considerably more necessary to consider the individual situation of the RP-patient than that of normal cataract patients.

A twenty-two-year-old woman with a history of long-Standing retinitis pigmentosa and recently discovered bilateral exudative retinal detachments of the Coats' type is described. This widely unrecognized association has been reported to occur in up to 2% of patients with retinitis pigmentosa. Unlike the usual Coats' syndrome, it is more often bilateral, shows no sex preference and occurs in older individuals. Heightened awareness of this potentially treatable complication of retinitis pigmentosa may facilitate earlier recognition of the problem, which may lead to more timely treatment and a better prognosis.