[Pharmacological treatment for retinopathy of prematurity]. / Pharmakologische Therapie der Frühgeborenenretinopathie.

Mandatory screening performed by an experience ophthalmologist remains the most important pillar in the management of retinopathy of prematurity (ROP). The current gold standard for treatment of proliferative ROP is still panretinal laser photocoagulation, depending on severity, in combination with vitreoretinal surgery if necessary. The first case series of off-label intravitreal anti-VEGF treatment are encouraging. In addition to intravitreal anti-VEGF therapy, other treatment concepts such as supplementation with IGF-1 or omega-3 fatty acids also represent interesting pharmacological approaches to the management of ROP. However, larger controlled trials are required to validate the benefits and safety of these systemic treatment approaches.

[New therapeutic approaches in the treatment of retinopathy of prematurity]. / Neue therapeutische Ansätze in der Behandlung der Frühgeborenenretinopathie.

This review highlights recent advances in clinical research on the therapy for retinopathy of prematurity (ROP). This disease is still important, because improvements in neonatal intensive care during the last years have increased the survival of the most immature newborns, but have not diminished the ROP frequency. Laser treatment at the optimal time for advanced ROP mostly prevents blindness. This paper describes the classification, current German screening criteria and the modern concept of ROP pathophysiology. The therapeutic treatment strategies and the treatment results, as well the possible promising future therapy with anti-VEGF (bevacizumab) is discussed.

[Guidelines for ophthalmological screening of premature infants in Germany]. / Leitlinie zur augenärztlichen Screening-Untersuchung von Frühgeborenen--Arbeitsgruppe der Retinologischen Gesellschaft zur Erstellung der Leitlinie zur augenärztlichen Screening-Untersuchung von Frühgeborenen.

These revised guidelines replace the previous guidelines on the screening of preterm infants for retinopathy of prematurity (ROP) that were published in 1999. Recently published research provided the impetus for this revision. These guidelines propose the following criteria for an efficient screening programme for the early detection and treatment of ROP in Germany: Inclusion criteria for ROP screening: All preterm infants with a gestational age of less than 32 weeks (if gestational age is unknown; of <1500 g birth weight) should be examined. In addition, all preterm infants with 32-36 weeks gestational age are included if postnatal oxygen was supplemented for more than 3 days. The first examination should be performed within the 5(th) week of postnatal age (day 36-42), but not prior to 31 weeks gestational age. Indications for laser photocoagulation: in Zone I: 1. any stage with "plus disease", 2. stage 3 without "plus disease." In Zone II: stage 3 with extraretinal proliferations in 5 continuous or 8 cumulative clock hours in combination with "plus-disease". In certain cases an earlier treatment may be indicated.

[Screening for retinopathy of prematurity: results of one centre between 1991 and 2002]. / Frühgeborenenretinopathie-Screening: Ergebnisse eines Zentrums zwischen 1991 und 2002.

PURPOSE: The aim of this study was to analyse the results of retinopathy of prematurity (ROP) screening, stage of disease and time of treatment from one tertiary care centre. PATIENTS AND METHODS: Between 1991 and 2001, 666 infants with a gestational age (GA) < 33 weeks or birth weight (BW) less than < 1501 g were examined. Coagulation treatment was performed in accordance with the Cryo-ROP study, after reaching the threshold. All infants were followed-up until a stable retinal situation was reached. RESULTS: Mean GA of the 666 infants was 28.5 +/- 2.3 weeks. Mean BW was 1180 +/- 372 g. During the years of screening, the GA and BW significantly decreased, but no significant change in the yearly incidence of ROP was detected. 460 (69.1 %) of the infants did not develop any stage of ROP. Maximum stage of ROP in the worse eye was stage one in 51 (7.6 %) infants, stage two in 59 (8.9 %) infants and stage three below threshold in 62 (9.3 %) infants. 34 (5.1 %) infants were treated with coagulation treatment. Mean time of treatment was 36.5 weeks postmenstrual age, or 10.7 weeks postnatal age. Postnatal age showed a better correlation to treatment time than postmenstrual age. No infant was treated before seven weeks of life and not before 32 weeks postmenstrual age. CONCLUSION: In spite of the increasing survival of children with lower GA and BW the incidence of ROP has not increased, and the incidence of ROP in our centre appears to be comparable to other international studies. In contrast to the Cryo-ROP study, treatment time correlates better to postnatal age than to postmenstrual age. All infants who underwent treatment were detected at an appropriate time using German screening guidelines.

[Comparison of the anatomical and functional outcome after laser or cryotherapy for retinopathy of prematurity (ROP)]. / Koagulationstherapie bei Frühgeborenenretinopathie Vergleich der anatomischen und funktionellen Ergebnisse nach Laser--oder Kryokoagulation.

PURPOSE: To analyze and compare the anatomical and functional outcome following cryotherapy or laser therapy for ROP. METHODS: Between April 1991 and February 2002, 127 eyes with ROP (65 patients) were treated after threshold ROP was reached. Ten additional eyes with zone I disease were treated with prethreshold. All patients were followed up until a stable retinal situation occurred. RESULTS: An "unfavorable outcome," as described in the Cryo ROP study, occurred in 1 of 91 (1%) eyes with laser treatment and in 3 of 46 (6.5%) eyes with cryotherapy. Temporal dragging of vessels was noticed in 6 of 91 eyes (6.6%) with laser treatment vs 7 of 46 eyes (15.2%) with cryotherapy, respectively. Visual acuity > or =20/25 was achieved in 39.2% in eyes with laser therapy and in 17.6% with cryotherapy (p<0.05). CONCLUSION: A strict screening protocol and treatment at onset of threshold disease results in an excellent anatomical and functional outcome. The results of laser therapy were superior over cryotherapy, indicating that laser treatment is the therapy of choice.

[Retinopathy of prematurity]. / Die Frühgeborenenretinopathie.

This review summarizes the knowledge and highlights recent advances in clinical research of retinopathy of prematurity (ROP). This disease is still important, because improvements in neonatal intensive care during the last years have increased the survival of the most immature newborns, but did not diminish the ROP frequency. Ophthalmologic screening and, if applicable, laser treatment at the optimal time for advanced ROP prevents blindness in most cases. The history, classification and the modern concepts of ROP pathophysiology are described. The results of various multicenter treatment trials are summarized. The current German screening guidelines are discussed with the guidelines of other nations. The therapeutic treatment strategies and the treatment results are discussed. The ophthalmologic disorders of former preterm infants, as higher rates of amblyopia, strabismus, and refractive error, are mentioned. Possible future therapies, e. g. anti-angiogenic factors are discussed.

[Vitrectomy in advanced Coats disease]. / Vitrektomie bei fortgeschrittenem Morbus Coats.

BACKGROUND: Coats disease is a retinal vasculopathy of unknown cause. Untreated cases usually lead to an exudative retinal detachment and rubeosis iridis with secondary glaucoma. Photocoagulation and/or cryotherapy are generally the first interventions in treating the disease. Pars plana vitrectomy may be indicated in cases of vitreous hemorrhage or retinal detachment. METHODS: We performed pars plana vitrectomy in 9 eyes with Coats disease between 1992 and 1999. A retinal detachment was present in 3 cases, and three showed a vitreous hemorrhage. In two cases surgery was indicated because of paramacular localization of the pathological vessels with associated exudations. RESULTS: The two cases with paramacular involvement showed improvement in visual acuity of eight lines. In the remaining cases visual acuity remained within two lines compared to the initial visual acuity. All eyes except one could be saved. CONCLUSION: Pars plana vitrectomy is a useful option in treating advanced Coats disease, especially in cases associated with vitreous hemorrhage or retinal traction.

Open globe injuries in children.

BACKGROUND: Eye injuries are the leading cause of monocular blindness in children. At present, however, only limited follow-up studies exist. METHODS: The files of 38 consecutive patients aged 16 years or younger who had been treated for open globe injuries were examined. The type of injury was classified and the severity of injury was categorized in four grades. RESULTS: Our series included 5 eyes (13%) with a rupture, 30 (79%) with a penetrating and 3 (8%) with a perforating injury. Follow-up varied from 3 days to 7 years (mean 15.5 months). The macula was attached at the last follow-up in all eyes. The final visual acuity ranged between 1.0 and light projection. In 47% of cases (18 eyes), the visual acuity was > or =0.5; 74% (28 eyes) achieved at least 0. 1. Eighteen of 38 eyes (47%) had severe injury with posterior segment involvement. Visual acuity of at least 0.5 was achieved in 44% (8/18) of these. Eyes with grade 2-4 injury treated with early vitrectomy had a final visual acuity of > or =0.5 in 58% of cases (7/13). Twenty-one (55%) of 38 children were < or =8 years of age and eight of them (38%) had a final visual acuity of > or =0.5. CONCLUSION: Salvage of the eye with an attached macula was possible in all eyes. Even in severely injured eyes good visual acuity can be established in about 50% of cases.

Extraocular muscle myositis and restrictive strabismus in 10 dogs.

Ten cases of uni- or bilateral restrictive ventromedial strabismus in young dogs of different breeds are reported. Clinically, abnormalities were restricted to the extraocular muscles with sparing of the masticatory muscles and limb muscles. This was supported in some cases by imaging studies, electrophysiology, and immunocytochemical assay for antibodies against type 2M fibers. Histologically, there was variable lymphocytic plasmacytic mononuclear cell infiltration and fibrosis. This disorder is similar in many aspects to chronic masticatory myositis with focal myositis and subsequent fibrosis. Surgical correction may restore eye position and vision.

A pedigree of Leber's hereditary optic neuropathy with visual loss in childhood, primarily in girls.

BACKGROUND: Leber's hereditary optic neuropathy (LHON) mostly affects young males. In patients carrying one of the primary mutations the risk to develop LHON is 50% for males and 10% for females. We report a family with predominantly young girls affected. METHODS: In a family with 14 known maternal relatives (11 females, 3 males) 9 patients in 4 generations developed LHON. Eight of the 9 patients were females. Three affected females could be examined and followed. RESULTS: The only affected male showed the typical course of LHON with acute visual loss in both eyes (20/400-20/800) within 6 weeks at 20 years of age. Eight of 9 females developed signs of LHON. In these females acute visual loss occurred at about 10 years of age. Final visual acuity was about 20/200. Central or paracentral scotomata, color vision defects and delayed P100 latencies in the VEP were seen. Ophthalmoscopy showed hyperemic discs in the acute stage and optic atrophy in later stages. Molecular genetic analysis revealed the presence of the mtDNA ND4/np11778 mutation in this family. Specific clinical or additional molecular genetic risk factors could not be detected. CONCLUSION: Families with LHON may show considerable variations of the clinical course and the gender- or age-specific risk. We present a family with a high disease penetrance of 64% and a 2 times higher risk for young females than for males. Furthermore, early visual loss in this family is permanent.

[Hereditary macular dystrophies]. / Hereditäre Makuladystrophien.

PURPOSE: Analysis of a consecutive series of patients suffering from hereditary macular dystrophies. PATIENTS AND METHODS: Between 1991 and 1997, 136 patients underwent clinical and electrophysiologic examinations (Ganzfeld ERG, EOG or multifocal ERG). RESULTS: The time of first examination was similar to the onset of clinical signs in most patients. In half of the patients onset was not before 40 years of age. In younger patients, Stargardt disease, X-linked retinoschisis and Best disease were the most frequent disorders. In patients aged 40 year and older, pattern dystrophies and central areolar choroidal dystrophy were more frequent. In 41 cases (30.2%) a specific diagnosis could not be made. Color vision and multifocal ERG were abnormal in all patients examined. Ganzfeld ERG was important for the diagnosis of X-linked retinoschisis, as was the EOG for Best disease. CONCLUSIONS: Hereditary macular dystrophies are a heterogeneous group of disorders. In several cases a definite diagnosis could not be made. Pattern dystrophies are the most frequent hereditary macular dystrophies in patients older than 40 years of age.

[Septo-optic dysplasia (de Morsier syndrome)]. / Septooptische Dysplasie (de-Morsier-Syndrom).

Septo-optic dysplasia (or de Morsier syndrome) is a congenital disorder characterised by anomalies in cerebral midline structures, optic nerve hypoplasia, and hormonal deficiencies. Diagnosis should be made early, due to the possibility of treating the hormonal disturbances. We describe here a case with decreased visual acuity, one-sided hemianopia, nystagmus und agenesis of the septum pellucidum and discuss the heterogeneous appearance of this syndrome. There are two theories regarding its pathogenesis. The first postulates simultaneous damage to both cerebral structures and optic nerve development around the 6th week of gestation, while the other favours secondary degeneration of optic nerve fibres due to a cerebral lesion.

Autosomal dominant vitreoretinochoroidopathy with normal electrooculogram in a German family.

BACKGROUND: Autosomal dominant vitreoretinochoroidopathy (ADVIRC) is a rare disorder previously described in four families residing in the USA and one family residing in Germany. We report the clinical and unexpected electrophysiological findings in a sixth family, residing in Germany. METHODS: An affected 23-year-old man, his 52-year-old affected mother and his 55-year-old unaffected father were examined by testing visual acuity, fluorescein angiography, visual fields, dark adaptation, electrooculography (EOG) and electroretinography (ERG). RESULTS: The 23-year-old man showed a circumferential retinochoroidal dystrophy extending from the mid-periphery to the ora serrata. There was a sharp demarcation between affected and nonaffected retina. Peripheral to the damarcation, bone spicules and yellow-white deposits were present, and the retinal vessels were severely attenuated. In addition, vitreous opacities were present. The EOG light rise was normal. The ERG amplitudes were reduced to 35% of the normal in all recording conditions. The 52-year-old mother showed marked peripheral pigmentation, but no bone spicules, deposits or vitreous opacities. Her EOG and ERG recordings were normal. CONCLUSIONS: Expression of ADVIRC can be very variable within the same family. A reduced EOG light rise, previously suggested as a characteristic sign for ADVIRC, is not a typical sign for all affected patients.