[Severe amblyopia - literature review]. / Hochgradige Amblyopie - Literaturreview.

PURPOSE: The aim of this study was to characterise the visual acuity prognosis in the therapy for severe amblyopia. METHODS: Literature review on the basis of studies in PubMed was undertaken. RESULTS: The studies investigating severe amblyopia due to anisometropia and/or strabismus are unanimous in their finding that the early onset of therapy leads to better results for visual acuity. In children under the age of seven years a visual acuity of at least 0.4 (logMAR 0.4); that is, the ability to read, is achieved. At the beginning of therapy, even a partial occlusion with a limited number of hours already shows an improvement in visual acuity. Poor visual acuity at the end of therapy worsens the long-term prognosis. Anisometropia has a poorer visual acuity prognosis than anisohyperopia. Recent studies investigating the possibility of still better visual acuity, and therefore less recurrence of amblyopia and better long-term prognosis, following long-term occlusion therapy are lacking. CONCLUSIONS: Screening and early and long-term therapy are necessary in order to reduce the prevalence of severe amblyopia.

[Papilledema and echographically detectable retro-orbital dilatation of the sub-arachnoidal space with open fontanelle - six case reports]. / Stauungspapille und echografisch nachweisbare retroorbitale Erweiterung des Subarachnoidalraums bei offener Fontanelle - Sechs Kasuistiken.

This paper analyses the case reports for three children in which a papilledema occurred before the age of one year. Furthermore, an analysis is also given of three further case reports for children aged less than one year in which, in spite of open fontanelle, no papilledema was found, however, a dilatation of the sub-arachnoidal space was demonstrated echographically. Even in children less than one year of age in which an open fontanelle still exists and in whom a neuro-paediatric clarification of internal hydrocepalus is made, in spite of opththalmoscopically inconspicuous findings for the papilla an echography is indispensable for the evaluation of the sub-arachnoidal space. Here, the early recognition of a dilatation of the retro-bulbar sub-arachnoidal space can possibly prevent the occurrence of a consecutive optic atrophy. At the present time, the data available do not allow the recommendation of an upper age limit for an echographic examination.

[Results of late onset amblyopia therapy for excentric fixation in different forms of amblyopia]. / Ergebnisse später Therapie von exzentrischer Fixation bei verschiedenen Amblyopieformen.

BACKGROUND: The objective of this study was to describe the therapeutic prognoses for excentric fixation in relation to the underlying diagnoses. PATIENTS AND METHODS: We investigated the clinical development of therapy for permanent occlusion (90%) or standard occlusion (10%) in 32 children up to a maximum age of 7 years with the following: strabismus convergens: n = 12, microstrabismus: n = 10, secondary sensory esotropia: n = 2, anisometropias: n = 6, and congenital partial clouding of the optical axis: n = 2. The mean age of all patients at the time of the first visit to the clinic (EV) was 61 ± 12 (37-86) months, with no significant difference in the sub-groups (n > 2) except for the comparison microstrabismus/anisomyopia (p = 0.05). The mean period of the follow-up examinations, obtained with the aid of a survey of the doctors performing these examinations, was 5 years and 10 months ± 4 years and 6 months (6-253 months). RESULTS: Central fixation was achieved for 20 of 32 (63%) of the patients: for 10 of 12 children with infantile esotropia, 1 of 2 children with secondary sensory esotropia, 3 of 10 children with microesotropia, and 5 of 6 children with anisometropia and 1 (already with standard occlusion) of 2 children with partial clouding of the optical axis. The mean time required to achieve central fixation was 4.5 ± 3.6 (1-11) months. The poorest success rate was found for slightly nasal excentric fixation or fixation on the nasal macular wall, and the best rate for fixation on the temporal macular wall, above the foveola or above the papilla. Achieving central fixation correlated significantly--inversely proportional--to the level of refraction of the amblyopic eye (r = -0.4, p = 0.03), as well as with the difference between the refraction of the amblyopic eye and the dominant eye (r = -0.4, p = 0.02) and with the occurrence of astigmatism in the amblyopic eye (r = -0.4, p = 0.04). CONCLUSIONS: The typical age at the time of the first visit to the clinic in this study was around 5 years for most of the excentric fixations investigated. This requires greater intervention of occlusion therapy. In this study, the patients with anisomyopia and infantile esotropia showed the best success rates, and the children with microstrabismus showed the poorest success rates. The fixations close to the papilla, temporal macular wall, and over the foveola were shown to be favourable in relation to the prognosis. With microstrabism, a quasi-burned in nasal excentric fixation with binocularity dominated the unsuccessful attempts. With the primary forms of esotropia, a highly hyperopic amblyopic eye is unfavourable in relation to the prognosis.

[Bilateral posterior persistent hyperplastic primary vitreous]. / Bilateraler posteriorer persistierender hyperplastischer primärer Glaskörper.

Bilateral persistent hyperplastic primary vitreous (PHPV) represents a rare entity of a congential malformation. This casuistic presents for the first time in the German literature the case of a 4-month-old child with bilateral posterior PHPV.

[Partial homonymous hemianopia of traumatic origin after riding a high-speed amusement ride]. / Partielle homonyme Hemianopsie traumatischer Genese nach Besuch eines Hochgeschwindigkeitsfahrgeschäftes.

Amusement park injuries have become more common in recent years. Especially neurological symptoms dominate clinical findings. In this article, the case of a 15-year-old child with homonymous hemianopia due to an atypical intracranial bleeding with subdural hematoma after a giant swing ride is described for the first time.

[Cavernous sinus thrombosis as a rare cause of exophthalmos in childhood : A case report]. / Sinus-cavernosus-Thrombose als seltene Ursache eines Exophthalmus im Kindesalter : Ein Fallbericht.

Complications of acute bacterial sinusitis mostly occur in children and adolescents. In particular, intracranial spread of the infection can lead to severe even fatal courses of the disease. This article is a case report about a 13-year-old boy suffering from left-sided headache, meningismus and exophthalmos as presenting symptoms. Cranial magnetic resonance imaging (MRI) showed merely right-sided sphenoid sinusitis; however, the diffusion-weighted MRI sequence indicated a left-sided cavernous sinus thrombosis, which could be confirmed by computed tomography (CT) angiography. Cerebrospinal fluid diagnostics showed significant leukocytosis confirming secondary meningitis. Finally, exophthalmos was explained by parainfectious cavernous sinus thrombosis and periorbital edema. This case report highlights the importance of extended and specific diagnostic imaging in cases of clinically suspected complications in children and adolescents with sinusitis and the diagnostic significance of diffusion-weighted MRI.

Primary congenital glaucoma: three case reports on novel mutations and combinations of mutations in the GLC3A (CYP1B1) gene.

PURPOSE: To describe three patients with congenital glaucoma homozygous and compound heterozygous for different mutations and benign sequence variants in the cytochrome P 450 1B1 (CYP1B1) gene. METHODS: All patients were examined by slit-lamp biomicroscopy, gonioscopy, measurement of the cornea and optic disc, ultrasound biometry, and automated static threshold perimetry when possible. Direct sequence analysis was performed on DNA extracted from peripheral blood from the patients and their parents. RESULTS: For patient 1, a newborn boy with buphthalmos and an opaque cornea, a novel homozygous C/T transition in codon 355 (CGA>TGA) led to a predicted nonsense codon Arg355X truncating the protein by 188 amino acids. For patient 2, a 24-year-old man, a compound heterozygous mutation 1410-1422del/1546-1555dup was found. For patient 3, a 34-year-old man, two novel heterozygous missense mutations resulting in an Ala443Gly and a Glu229Lys amino acid exchange and five benign sequence variants were found. CONCLUSION: Our results confirm the crucial role of CYP1B1 mutations for congenital glaucoma.

Stimulus deprivation myopia in human congenital ptosis: a study of 95 patients.

PURPOSE: To establish differences between the frequency of suspected deprivation myopia in unilateral and bilateral congenital ptosis with and without covered optical axis. METHODS: Ametropia was evaluated in both eyes of 95 patients with congenital ptosis. The amount of refraction was documented as spherical equivalent (100% cycloplegia). Statistical analysis was performed using the chi-square and sign tests. RESULTS: In unilateral ptosis, the frequency of myopia was lower (10/68: 15%) than that of hyperopia (58/68: 85%) in the ptotic eye (P <0.001). However, myopia occurred more often in the ptotic eye (10/68: 15%) than in the fellow eye (3/68: 4.4%). Myopic anisometropia was found only in the ptotic eye (5/68 vs 0/68), but was less frequent than hyperopic anisometropia (6/68 vs 8/68). In bilateral ptosis 7/54 myopia as compared with 47/54 hyperopia were observed and 1/27 myopic anisometropia vs 6/27 hyperopic anisometropia. Covered center of the pupil, in children < or = 8 years of age, was associated with myopia more frequently in bilateral than in unilateral ptosis (6/30 vs 1/27). We found a significantly higher rate of myopia <-1 diopter and hyperopia >2 diopter in comparison of children 5 to 7 years old with first-grade school children. CONCLUSIONS: Two expected results were (1) compared with the normal population, an overall higher frequency of myopia in human congenital ptosis; (2) in unilateral ptosis, a higher frequency of myopia in the ptotic, than in the fellow eye.

[Two years experience with the new accommodative 1 CU intraocular lens]. / Zwei Jahre Erfahrung mit der akkommodativen Hinterkammerlinse 1 CU.

After marked improvement of optical rehabilitation of cataract patients during the last decades due to small incision surgery and foldable intraocular lenses (IOL), presbyopia is now one of the great unsolved questions in ophthalmology. During recent years a new accommodative IOL, the 1CU lens, has been developed based on the concepts of K.D. Hanna and on finite element computer simulation models. The 1CU IOL is designed to transform contracting forces of the ciliary muscle into anterior movement of the IOL optic (optic-shift concept). After the first implantation of a 1CU IOL in Erlangen in June 2000, we have now successfully implanted the 1CU IOL in over 90 patients. Our experiences and the results of several clinical studies indicate good and safe implantability, good centration, no IOL-specific complications, and good distance visual acuity. In comparison to control groups with conventional IOL, patients with the 1CU enjoyed significantly better distance-corrected near visual acuity, a larger accommodative range, and increased anterior and posterior axial movement of the lens optic after medical stimulation or inhibition of the ciliary muscle. We interpret our results as confirmation of the optic-shift concept of the 1CU IOL. Overall, the concept of accommodative IOL appears attractive and may have a great potential in the future. Additional studies including randomized blind multicenter evaluation of the 1CU IOL are necessary to further evaluate long-term and accommodative results.

Stimulus deprivation amblyopia in human congenital ptosis: a study of 100 patients.

AIM: To investigate the frequency of stimulus deprivation amblyopia (SDA) in comparison with other reasons for amblyopia in human congenital ptosis. METHODS: The frequency and causes of amblyopia were evaluated in the 200 eyes of 100 patients. Congenital ptosis was present in 128 eyes (72 unilateral, 28 bilateral). The age at investigation was one year and older, with an average of 11 years and 10 months. Amblyopia was defined as best corrected visual acuity less than 1.0 or a difference between the two eyes of at least 0.2. The following causes of amblyopia were identified: amblyopiogenic refractive errors: astigmatism > or = 1 dpt, anisometropia > or = 1 dpt (79% cycloplegia) and strabismus. In cases with no other reasons for amblyopia, SDA was assumed. Statistical analysis was performed using the chi-square and the sign tests. RESULTS: The overall incidence of amblyopia in ptotic eyes was 89/128 (70%). In 3.9% of the cases (5/128; 2 eyes with unilateral and 3 eyes with bilateral ptosis) we assumed SDA. A comparison of ptotic eyes with (unilateral: n = 35, bilateral: n = 34) and without covered optical axis revealed the following: in the case of unilateral ptosis, amblyopia was found more often in ptotic eyes with covered optical axis: 30 out of 35 vs. 24 out of 37 (p = 0.06); in the case of bilateral ptosis this difference was significant: 27 out of 34 vs. 8 out of 22 (p < 0.05). In the case of SDA, the optical axis was covered in only a single eye, in a patient with bilateral ptosis. There was no difference in the incidence of anisometropia: 19 out of 53 vs. 14 out of 47 (p = 0.52). Astigmatism was found more frequently in ptotic eyes with covered optical axis in unilateral ptosis: 23 out of 35 vs. 16 out of 37 (p = 0.06) but not in bilateral ptosis: 21 out of 34 vs. 13 out of 22 (p > 0.9). Strabismus was found significantly more frequently in ptotic eyes with covered optical axis: 13 out of 35 vs. 4 out of 37 (p < 0.05) in unilateral ptosis and 7 out of 34 vs. 1 out of 22 (p = 0.13) in bilateral ptosis. CONCLUSION: In contrast to the classical animal models of stimulus deprivation amblyopia, this entity is rare in human congenital ptosis, perhaps because of the counter effect of compensating head posture. Disruption of fusion resulting in strabismus might be an additional indirect cause of amblyopia in congenital ptosis. Prophylactic amblyopia treatment in ptosis cases is important as long as no testing of visual acuity is possible in a child.

Stimulus deprivation myopia in human congenital ptosis: a preliminary report of 50 unilateral cases.

OBJECTIVE: To investigate the frequency of hyperopia and suspected stimulus deprivation myopia in human congenital ptosis. METHODS: Ametropia was evaluated in both eyes of 50 patients with congenital ptosis. The age at investigation was one year and older, average 6 years and 10 months. The amount of refraction was documented as the spherical equivalent (100% cycloplegia). Differences between the paired eyes of 1.0 dpt or more were defined as anisometropia. Statistical analysis was performed using the chi-square and sign tests. RESULTS: The frequency of myopia was lower (7/50: 14%) than that of hyperopia (43/50: 86%) in the ptotic eye (p<0.001). However, myopia occurred more often in the ptotic eye (7/50: 14%) than in the fellow eye (3/50: 6%, p>0.3). Myopic anisometropia was found only in the ptotic eye (5/50 vs. 0/50, p = 0.06), but was not more frequent than hyperopic anisometropia: (5/50 vs. 9/50, p>0.4). A covered center of the pupil in 27 out of 50 eyes was not associated significantly with myopia (2 of 27 versus 5 of 23, p>0.2). Altogether, we found a significantly higher rate of myopia <-1 dpt and hyperopia > 2 dpt: 10% vs. 1.4% and 40% vs. 10.2% (p<0.001) in comparison with normal school children. CONCLUSION: In summary, the following three findings were noted, of which the first two were expected and the third was not. 1. Compared with the normal population, there was an overall higher frequency of myopia in unilateral congenital ptosis. 2. There was a higher frequency of myopia in the ptotic than in the fellow eye. 3. Compared with the normal population, there was also a higher frequency of hyperopia. The clinical presentation of human congenital ptosis may be influenced by compensating head posture, strabismus, accommodation and biochemical effects, and the condition may therefore differ from the classical well-defined animal model of stimulus deprivation myopia.

A randomised comparison of bilateral recession versus unilateral recession-resection as surgery for infantile esotropia.

OBJECTIVE: Infantile esotropia, a common form of strabismus, is treated either by bilateral recession (BR) or by unilateral recession-resection (RR). Differences in degree of alignment achieved by these two procedures have not previously been examined in a randomised controlled trial. DESIGN: Controlled, randomised multicentre trial. SETTING: 12 university clinics. PARTICIPANTS AND INTERVENTION: 124 patients were randomly assigned to either BR or RR. Standardised protocol prescribed that the total relocation of the muscles, in millimetres, was calculated by dividing the preoperative latent angle of strabismus at distance, in degrees, by 1.6. MAIN OUTCOME MEASURE: Alignment assessed as the variation of the postoperative angle of strabismus during alternating cover. RESULTS: The mean preoperative latent angle of strabismus at distance fixation was +17.2 degrees (SD 4.4) for BR and +17.5 degrees (4.0) for RR. The mean postoperative angle of strabismus at distance was +2.3 degrees (5.1) for BR and +2.9 degrees (3.5) for RR (p = 0.46 for reduction in the angle and p = 0.22 for the within-group variation). The mean reduction in the angle of strabismus was 1.41 degrees (0.45) per millimetre of muscle relocation for RR and 1.47 (0.50) for BR (p = 0.50 for reduction in the angle). Alignment was associated with postoperative binocular vision (p = 0.001) in both groups. CONCLUSIONS: No statistically significant difference was found between BR and RR as surgery for infantile esotropia.

[Corneal endothelial cell density following strabismus surgery]. / Hornhautendothelzellzahl nach Augenmuskeloperationen an zwei und mehr geraden Augenmuskeln.

BACKGROUND: The short-term impact of eye muscle surgery on the blood-aqueous barrier has been described by iris fluoresceinangiography and laser tyndallometry. The aim of this study was to investigate the long-term effects of eye muscle surgery on the oxygen supply of the anterior segment of the eye by determination of the corneal endothelial cell density. PATIENTS AND METHODS: We investigated the corneal endothelial cell density of 32 patients with unilateral rectus muscle surgery pre- and postoperatively (24 cases of primary surgery on 2 horizontal muscles, 4 revision surgeries on 2 horizontal muscles, and 4 primary eye muscle surgeries on the 2 vertical recti including: 3 transpositions of the lateral halves of the vertical recti additional to recession of the medial rectus and one complete transposition of the two vertical recti). Exclusion criteria were previous intraocular surgery and retinal detachment surgery. Mean age at surgery was 37.5 +/- 16.3 (14.5 to 79.9) years. Mean follow-up time was 5.0 +/- 2.2 (1.9 to 10.1) months. The corneal endothelial cell density (CECD) was quantified by means of a specular microscope (EM-1000, Tomey). RESULTS: The intraocular difference in CECD was not statistically significant either pre- or postoperatively. Mean values were 84 +/- 297 (731 to 700) vs. 52 +/- 357 (600 to 800), p = 0.64. In the subgroups of patients with revision surgery and those with primary eye muscle surgery involving the vertical recti, there was also no significant intraocular difference of CECD. CONCLUSIONS: Based on the corneal endothelial cell density, there was no evidence of a chronically decreased oxygen supply of the anterior segment even after complex eye muscle surgery.

[How useful is the prescription of glasses in intermittent exotropia and decompensating exophoria?]. / Was bringt der Refraktionsausgleich bei Strabismus divergens intermittens und dekompensierender Exophorie?

BACKGROUND: The aim of this study was to investigate the effect of full correction of the retinoscopic measurements done in cycloplegia in two groups of patients with intermittent exotropia (IE) and decompensating exophoria (DE), respectively. PATIENTS AND METHODS: 58 patients (n = 29 each of IE and DE) fulfilled the inclusion criteria: retinoscopy in cycloplegia, follow-up of at least 6 weeks, age more than 2.5 years and reliable visual acuity. Exclusion criteria were all forms of secondary and constant exotropia, A-V incomitance of high amount and eyes with amblyopia (visual acuity in far distance < 0.5). The age median of refractometry was not higher in patients with IE compared to those with DE: 7.4 (3.10; 47.8) vs. 7.3 (3.8; 40.11) years, p = 0.33. Glasses were prescribed in any case of myopia and astigmatism as well as in hyperopia of > + 0.5 dpt. RESULTS: Mean refraction of both eyes (mean value of spherical equivalent of both eyes) was higher for IE compared to DE: 0.7 +/- 1.8 (- 5.13; + 4.75) vs. 0.1 +/- 1.7 (- 5.25; + 2.5) dpt, p = 0.9. Visual acuity improved in both groups significantly: median of visual acuity in IE: 0.9 (0.3; 1.25) vs. 1.1 (0.5; 1.25), p = 0.02; in DE: 1.0 (0.4: 1.25) vs. 1.0 (0.6; 1.25), p = 0.03. Considering only patients aged over 7 years the difference stayed significant only in the group of DE: IE: median 1.0 (0.5; 1.25) to 1.0 (0.5; 1.25), p = 0.2; DE: median 1.0 (0.8; 1.25 to 1.0 (0.8; 1.25): visual acuity was nearly always 1.0 and better, p = 0.009. In hyperopic patients a significant improvement of visual acuity could be seen: in IE from median 0.9 (0.3; 1.25) to 1.1 (0.5; 1.25), p = 0.02 , in DE visual acuity: median 1.0 (0.4; 1.25) to 1.1 (0.6: 1.25), p = 0.02, as well as stereoacuity improved significantly: median 60 (30; 240)'' vs. 60 (15; 240)'', p = 0.03. In patients aged over 7 years the improvement was no longer significant in IE: p = 0.7, but stayed significant in DE: p = 0.03. There was no significant change of the angle deviation in both groups. CONCLUSIONS: According to the results of this study, full correction of refractive errors in IE and DE leads to an improvement of the visual acuity mainly due to correction of myopia and astigmatism, but not to a better compensation of the angle deviation.

[Protective ptosis by botulinum A toxin injection in corneal affectations]. / Protektive Ptosis durch Botulinumtoxin-A-Injektion bei Hornhautaffektionen.

BACKGROUND: Botulinum toxin A has been introduced as a local injection therapy of different conditions with focal muscular hypercontractions. In the ophthalmologic field the toxin has proven its efficacy in the therapy of blepharospasm and hemifacial spasm. There are only few reports on the use of a botulinum toxin A to induce a protective ptosis in patients with persistent corneal ulcers. PATIENTS AND METHODS: 21 patients who failed to respond to conservative therapy of corneal erosions or ulcers of different origin received a botulinum toxin A injection into the levator palpebrae superioris muscle. RESULTS: The ptosis began after a mean of 1.5 days (1-3 days) and was complete after a mean of 5.1 days (3-12 days) after injection. Complete recovery of the levator function could be observed after a mean of 12.4 weeks (4-24 weeks). In 13 patients (61.8%) the botulinum toxin A induced protective ptosis lead to a complete healing of indolent ulcers or erosions, in 4 patients (19%) an additional tarsorrhaphy was necessary. In 3 patients no healing could be observed during follow up, in one patient (with neuroparalytic ulcer) the injection was given prophylactically. The period of healing on average was 3.8 weeks. There was no relationship between the healing rate and the duration of the corneal disease prior to the botulinum toxin injection. The mean healing rate of younger patients was higher (75%) than that of older patients (53.8%) and higher in erosions (70%) than in ulcers (30%). No side effects were observed besides in one patient the undesirable duration of the ptosis of a half year. CONCLUSION: The induction of a protective ptosis with botulinum toxin A injection is an efficacious treatment alternative in persistent corneal erosions and ulcers before performing a tarsorrhaphy. This method is preferrable especially in patients with lagophthalmos due to facial nerve paresis with potential recovery.