[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.

[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.

[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.

[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.

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.

[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.

[First results of implantation of a new, potentially accommodative posterior chamber intraocular lens]. / Erste Ergebnisse der Implantation einer neuen, potenziell akkommodierbaren Hinterkammerlinse - eine prospektive Sicherheits Studie.

PURPOSE: Conventional posterior chamber intraocular lenses (PCIOL) generally provide excellent visual acuity but do not restore accommodation. A new, potentially accommodative PCIOL has been designed after principles elaborated by K.D. Hanna using finite element models. However, before newly developed PCIOL may be implanted routinely in larger numbers of patients, careful and meticulous evaluation in clinical studies is necessary. Thus, it was the aim of this study to investigate intra- and early 3-month postoperative findings after implantation of the newly designed PCIOL. PATIENTS AND METHODS: In a prospective pilot and safety study that was approved by the ethics committee of our university, six eyes of six patients (2 males, 4 females, age range 54 to 87 years) with senile or presenile cataract underwent phacoemulsification and implantation of the new PCIOL by one surgeon between June and November 2000. The PCIOL (1 CU, HumanOptics AG, Erlangen, Germany) is a one-piece hydrophilic acrylic foldable lens with an optic diameter of 5.5 mm. Modified haptics are intended to allow anterior movement of the lens optic as a function of contraction of the ciliary muscle. Intra- and early postoperative findings obtained after one and two days, one, two and six weeks and 3 months postoperatively were documented prospectively. Postoperative examinations included recording of distance and near visual acuity both obtained with best distance correction, determination of subjective near point and measurement of distance and near refraction by streak retinoscopy. Follow-up was at least three months in all patients. RESULTS: Surgery was uncomplicated in all patients with successful in-the-bag implantation and good centration of the PCIOL. The postoperative course was uncomplicated without inflammation, hemorrhage, synechiae or decentration. Visual acuity improved in all patients according to the status of the macula with values between 20/200 (atrophic maculopathy) and 20/20. After three to six months we observed a difference between retinoscopic near and distance refraction of 0.625 to 1.875 D and subjective near points of 40 to 100 cm. Near visual acuity with distance correction ranged from 0.1 or J 16 (atrophic maculopathy) to 0.4 or J 7. CONCLUSIONS: These early and preliminary results of our small pilot study are encouraging. Our findings may indicate at least some degree of pseudophakic accommodation. However, further studies with additional methods of measurements, with longer follow-up, more patients and controlled studies with control groups are essential to further determine safety and potential accommodative power of this new PCIOL.

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.

[Eyelid and facial injuries due to dog bites]. / Lid- und Gesichtsverletzungen nach Hundebiss.

BACKGROUND: Head und neck injuries of children are mistly due to dog bite. PATIENTS AND METHODS: During the 10-year interval from 1990 to 2000 sixteen patients suffering from dog bite injuries were seen in our department. Age, gender, pattern of ocular and periocular wounds, surgical management and long-term damage were analyzed. RESULTS: Medial lower eyelid injuries with lacrimal duct involvement were most common. Ten victims were younger than four years. 87% of the dogs belonged to the family and friends of the patients. Two localized infections and no systemic infection were seen. CONCLUSION: Primate microsurgical wound repair leads to a satisfying functional, morphological, and esthetic long-term result.

[Functional results of posterior chamber lens implantation for traumatic cataracts in 22 children younger than seven years--a retrospective study]. / Funktionelle Ergebnisse der Hinterkammerlinsen-implantation wegen traumatischer Katarakte bei 22 Kindern unter 7 jahren-Eine retrospektive studie

BACKGROUND: Surgical correction of aphakia following traumatic cataract in children is discussed controversely in literature. This study shows the functional results after cataract surgery with posterior chamber intraocular lens implantation in children under 7 years of age. PATIENTS AND METHODS: This retrospective study analyzes the visual function of 22 children, who were operated after traumatic cataract between 1987 and 1997 at the department of ophthalmology, university of Erlangen-Nürnberg with PCL-implantation. Mean age at injury was 3 years 10 months +/- 1 year 8 months (range 15 to 76 months); the interval between injury and cataract surgery: 2.7 +/- 4.7 months (range 0 to 17 months). The follow-up time

[Early childhood cataract in hereditary UDP-galactose-4-epimerase deficiency--a case report]. / Frühkindliche Katarakt bei familiärem UDP-Galaktose-4-Epimerase-Mangel--eine Kasuistik.

BACKGROUND: Increased plasma galactitol levels may lead to development of bilateral pediatric cataract. PATIENT: A 3-year-old boy was found to suffer from a bilateral zonular cataract. Extracapsular lensectomy with posterior capsulotomy, transpupillar anterior vitrectomy and posterior chamber lens implantation were performed during a 4-month-interval. RESULTS: The epimerase-activity in red cells of the index patient was found to be significantly decreased (11.2 mumol/h/g Hb; normal range; 19-35). From other family members, such as the brother (16.8), the father (16.0) and the grandfather (15.6), a diminished red cell activity was observed. The mother whose epimerase activity was considerably lower than that of the above mentioned family members (13.3) showed also a zonular bilateral cataract. CONCLUSIONS: Investigation of enzymes and polyols of galactose metabolism as well as consultation of the concerned families are recommended for clarification of cataract development.

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.