Surgical Management of Pre-Chiasmatic Intraorbital Optic Nerve Gliomas in Children after Loss of Visual Function-Resection from Bulbus to Chiasm.

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the subgroup of pre-chiasmatic gliomas, intraorbital and intradural resection is a curative option after blindness. We present a two-center cohort using different surgical approaches. A retrospective analysis was performed, including 10 children. Mean age at surgery was 6.8 years. Interval between diagnosis and surgery was 1-74 (mean 24 ± 5.5, median 10) months. Indications for surgery were exophthalmos, pain, tumor progression, or a combination. Eight patients underwent an extradural trans-orbital-roof approach to resect the intra-orbital tumor, including the optic canal part plus intradural pre-chiasmatic resection. Gross total resection was achieved in 7/8, and none had a recurrence. One residual behind the bulbus showed progression, treated by chemotherapy. In two patients, a combined supra-orbital mini-craniotomy plus orbital frame osteotomy was used for intraorbital tumor resection + intradural pre-chiasmatic dissection. In these two patients, remnants of the optic nerve within the optic canal remained stable. No patient had a chiasmatic functional affection nor permanent oculomotor deficits. In selected patients, a surgical resection from bulb to chiasm ± removal of optic canal tumor was safe without long-term sequela and with an excellent cosmetic result. Surgery normalizes exophthalmos and provides an effective tumor control.

Restriction of eye motility in patients with RETINA IMPLANT Alpha AMS.

PURPOSE: To evaluate the motility of the eye in patients with the RETINA IMPLANT Alpha AMS. METHODS: Eye motility was determined in eight gaze directions in ten blind retinitis pigmentosa patients, who had received the RETINA IMPLANT Alpha AMS, before implantation of the subretinal implant and at six time-points up to one year after. RESULTS: The analysis of eye motility showed a restriction in the upgaze and gaze to the temporal side directly after surgery in eight of the nine patients included. The degree of motility restriction decreased continuously with recovery during the observation time. One year after surgery, eye motility was still restricted in the majority of patients, especially in the upgaze to the temporal side at 20° (five of seven patients). CONCLUSION: Retinal implants with intraorbital parts (e.g. connecting cables) caused restriction in the temporal and superior viewing directions in the majority of patients. Although this restriction might be cosmetically visible, this limitation in eye motility has no effects on the monocular vision and the implant's efficacy for daily use.

Interim Results of a Multicenter Trial with the New Electronic Subretinal Implant Alpha AMS in 15 Patients Blind from Inherited Retinal Degenerations.

Purpose: We assessed the safety and efficacy of a technically advanced subretinal electronic implant, RETINA IMPLANT Alpha AMS, in end stage retinal degeneration in an interim analysis of two ongoing prospective clinical trials. The purpose of this article is to describe the interim functional results (efficacy). Methods: The subretinal visual prosthesis RETINA IMPLANT Alpha AMS (Retina Implant AG, Reutlingen, Germany) was implanted in 15 blind patients with hereditary retinal degenerations at four study sites with a follow-up period of 12 months (www.clinicaltrials.gov NCT01024803 and NCT02720640). Functional outcome measures included (1) screen-based standardized 2- or 4-alternative forced-choice (AFC) tests of light perception, light localization, grating detection (basic grating acuity (BaGA) test), and Landolt C-rings; (2) gray level discrimination; (3) performance during activities of daily living (ADL-table tasks). Results: Implant-mediated light perception was observed in 13/15 patients. During the observation period implant mediated localization of visual targets was possible in 13/15 patients. Correct grating detection was achieved for spatial frequencies of 0.1 cpd (cycles per degree) in 4/15; 0.33 cpd in 3/15; 0.66 cpd in 2/15; 1.0 cpd in 2/15 and 3.3 cpd in 1/15 patients. In two patients visual acuity (VA) assessed with Landolt C- rings was 20/546 and 20/1111. Of 6 possible gray levels on average 4.6 ± 0.8 (mean ± SD, n = 10) were discerned. Improvements (power ON vs. OFF) of ADL table tasks were measured in 13/15 patients. Overall, results were stable during the observation period. Serious adverse events (SAEs) were reported in 4 patients: 2 movements of the implant, readjusted in a second surgery; 4 conjunctival erosion/dehiscence, successfully treated; 1 pain event around the coil, successfully treated; 1 partial reduction of silicone oil tamponade leading to distorted vision (silicon oil successfully refilled). The majority of adverse events (AEs) were transient and mostly of mild to moderate intensity. Conclusions: Psychophysical and subjective data show that RETINA IMPLANT Alpha AMS is reliable, well tolerated and can restore limited visual functions in blind patients with degenerations of the outer retina. Compared with the previous implant Alpha IMS, longevity of the new implant Alpha AMS has been considerably improved. Alpha AMS has meanwhile been certified as a commercially available medical device, reimbursed in Germany by the public health system.

Safety evaluation of "retina implant alpha IMS"--a prospective clinical trial.

BACKGROUND: To restore vision in patients with retinitis pigmentosa, several types of electronic devices have been developed to stimulate neurons at different levels along the visual pathway. Subretinal stimulation of the retina with the Retina Implant Alpha IMS (Retina Implant AG, Reutlingen, Germany) has been demonstrated to provide useful vision in daily life. Here we evaluated the safety of this device. METHODS: An interventional, prospective, multi-center, single-arm study was conducted in patients with retinitis pigmentosa with the Retina Implant Alpha IMS. The results from the first nine patients of a single center regarding safety of the device are reported. Any untoward medical occurrence related or unrelated to the tested device was documented and evaluated. RESULTS: Nine adult subjects were included in the study at the Tübingen site. Seventy-five adverse events occurred in total, and 53 affected the eye and its adnexa. Thirty-one ocular adverse events had a relationship to the implant that was classified as "certain" while 19 had a probable or possible relationship; three had no relationship to the implant. Thirty-nine ocular adverse events resolved without sequelae, two resolved with sequelae, 11 remained unresolved, and in one the status was unknown. The intensity of ocular adverse events was mild in the majority of cases (n = 45), while six were of moderate and two of severe intensity. There was no non-ocular adverse event with certain relationship to the device. One subject lost light perception (without light localization) in her study eye. CONCLUSIONS: In conclusion, this prospective study, "Safety and Efficacy of Subretinal Implants for Partial Restoration of Vision in Blind Patients," shows that the Retina Implant Alpha IMS is an option for restoring vision using a subretinal stimulation device with a clinically acceptable safety profile.

Visual loss without headache in children with pseudotumor cerebri and growth hormone treatment.

We report on two prepubescent girls with visual loss due to idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, both treated with recombinant human growth hormone for growth failure. The interval from starting hormone therapy to diagnosis of IIH was 3 and 18 months, respectively. Both girls did not complain of headache and nausea. They were neither obese nor did they suffer from renal insufficiency. In both patients, we observed bilateral optic disc edema with visual loss and elevated cerebrospinal fluid (CSF) pressures. Other causes of IIH were excluded with neuroimaging and CSF examination. Cessation of drug administration is often sufficient for symptom resolution in cases of hormone therapy-associated IIH. However, visual field defects in one girl remained unchanged during follow-up of 8 months. In children with IIH, the spectrum of neurologic and visual manifestations might be variable and unspecific. Diagnosis and management of IIH can be difficult in the absence of headache. Blurred or double vision due to cranial nerve palsy might be the only symptom rather than complaints about reduced visual acuity. Therefore, regular clinical monitoring of visual function and fundus appearance is essential for early diagnosis, efficient management, and improvement of visual outcome in children receiving recombinant human growth hormone.

Amniotic bands as a cause of congenital anterior staphyloma.

BACKGROUND: Congenital anterior staphyloma is a rare, complex malformation syndrome of the anterior segment. Only a few reports on associated systemic malformations have been published. We herein present a rare manifestation of congenital anterior staphyloma (CAS) combined with amniotic band disruption syndrome (ABS). PATIENT AND METHODS: Shortly after birth, a massive enlargement of the left eye was observed in a female child. Furthermore, an extensive bilateral congenital cleft lip and cleft alveolar ridge with oblique facial cleft extending into the left medial canthal region, coloboma(s) of the left eyelids, extensive adhesions between lids and eye bulb, as well as circumferential grooves, clubfeet, and terminal transverse defects in both hands and feet were present. Due to severe progression of eye bulb protrusion with thinning of the sclera, enucleation of the left eye was performed at the age of 3 years in order to prevent complications including perforation of the globe and with the aim of improving cosmetic aspects. RESULTS: Histopathological examination of the enucleated eye disclosed findings typical of congenital anterior staphyloma, including massive corneal staphylomatic deformation with superficial vascularization and elapsed corneoscleral margin, destruction of Bowman's layer, absence of Descemet's layer, corneal endothelium, and angle structures. The lens was only partially formed, and had mainly dissolved. The neural retina appeared normal. The optic nerve disc revealed a pronounced excavation. Facial clefts, lid colobomas, congenital constriction bands, and amputation of distal limbs match ABS. This malformation complex develops in early pregnancy, probably prior to 35 days post conception. CONCLUSION: This is the first report on an association of these two rare complex congenital malformations, congenital anterior staphyloma and amniotic band syndrome. The anterior staphyloma was unilateral, and related to facial clefts and lid coloboma in the area adjacent to the anterior staphyloma. Furthermore, the systemic deformities are clearly due to the amniotic bands, and the timing of the development of both complex malformations seems to be similar. All findings suggest that the presence of amniotic bands is a causative factor for all observed abnormalities including anterior staphyloma.

Management of strabismus with hemianopic visual field defects.

BACKGROUND: Hemianopia and strabismus leads to severe disturbance of visual orientation and diplopia under binocular conditions if the deviated eye has a normal retinal localization. SUBJECTS AND METHODS: Four cases with homonymous (two) and bitemporal (two) hemianopia and strabismus will be described with respect to binocular visual field and diplopia/confusion. All of them were recommended for strabismus surgery. Preoperatively, prism adaptation test was carried out to analyze functional results and fusional competence. In three of the four cases, strabismus surgery was successfully performed to avoid diplopia/confusion. All three patients revealed normal retinal correspondence. Furthermore, in two cases surgery led to an extension of binocular visual field; in one case with a bitemporal hemianopia and hemifield-slide phenomenon, blurred central vision and reading problems reduced significantly postoperatively. In one patient with anomalous retinal correspondence due to early childhood trauma exotropia led to an extension of the binocular visual field. In this case, strabismus surgery would have been unfavourable. RESULTS: Case 1 showed a homonymous hemianopia to the left and acquired exotropia of the right eye, leading to binocular diplopia. Case 2 with homonymous hemianopia to the right and exotropia of right eye revealed anomalous retinal correspondence after history of perinatal brain injury, resulting in absence of diplopia and enlargement of visual field to the right. Cases 3 and 4 with bitemporal hemianopia suffered from sensory disturbances caused by additional acquired strabismus. The exodeviation of the right eye in case 3 led to a restriction of binocular visual field with overlap of the nasal parts causing diplopia, whereas the esodeviation of case 4 resulted in a "gap" between the nasal parts (blind area). CONCLUSION: Depending on the extent of visual field defects and on retinal correspondence, functional consequences for binocular vision and binocular visual field should be considered prior to surgery. In normal retinal correspondence, strabismus surgery will be indicated in most cases because of diplopia. However, surgery might result in a reduction of binocular visual field. Preoperatively, it is important to map monocular and binocular visual fields, to examine retinal correspondence, and to undertake prism adaptation test to imitate the postoperative functional result and risk of double vision.

Subretinal electronic chips allow blind patients to read letters and combine them to words.

A light-sensitive, externally powered microchip was surgically implanted subretinally near the macular region of volunteers blind from hereditary retinal dystrophy. The implant contains an array of 1500 active microphotodiodes ('chip'), each with its own amplifier and local stimulation electrode. At the implant's tip, another array of 16 wire-connected electrodes allows light-independent direct stimulation and testing of the neuron-electrode interface. Visual scenes are projected naturally through the eye's lens onto the chip under the transparent retina. The chip generates a corresponding pattern of 38 × 40 pixels, each releasing light-intensity-dependent electric stimulation pulses. Subsequently, three previously blind persons could locate bright objects on a dark table, two of whom could discern grating patterns. One of these patients was able to correctly describe and name objects like a fork or knife on a table, geometric patterns, different kinds of fruit and discern shades of grey with only 15 per cent contrast. Without a training period, the regained visual functions enabled him to localize and approach persons in a room freely and to read large letters as complete words after several years of blindness. These results demonstrate for the first time that subretinal micro-electrode arrays with 1500 photodiodes can create detailed meaningful visual perception in previously blind individuals.

Reading performance with low-vision aids and vision-related quality of life after macular translocation surgery in patients with age-related macular degeneration.

PURPOSE: The aim of the present study was to evaluate the power of magnification required, reading performance with low-vision aids and vision-related quality of life with reference to reading ability and ability to carry out day-to-day activities in patients after macular translocation. METHODS: This study included 15 patients who had undergone macular translocation with 360-degree peripheral retinectomy. The mean length of follow-up was 19.2 +/- 10.8 months (median 11 months). At the final examination, the impact of visual impairment on reading ability and quality of life was assessed according to a modified 9-item questionnaire in conjunction with a comprehensive clinical examination, which included assessment of best corrected visual acuity (BCVA), the magnification power required for reading, use of low-vision aids and reading speed. Patients rated the extent to which low vision restricted their ability to read and participate in other activities that affect quality of life. Responses were scored on a scale of 1.0 (optimum self-evaluation) to 5.0 (very poor). RESULTS: In the operated eye, overall mean postoperative BCVA (distance) was not significantly better than mean preoperative BCVA (0.11 +/- 0.06 and 0.15 +/- 0.08, respectively; p = 0.53). However, 53% of patients reported a subjective increase in visual function after treatment. At the final visit, the mean magnification required was x 7.7 +/- 6.7. A total of 60% of patients needed optical magnifiers for reading and in 40% of patients closed-circuit TV systems were necessary. All patients were able to read newspaper print using adapted low-vision aids at a mean reading speed of 71 +/- 40 words per minute. Mean self-reported scores were 3.2 +/- 1.1 for reading, 2.5 +/- 0.7 for day-to-day activities and 2.7 +/- 3.0 for outdoor walking and using steps or stairs. Patients' levels of dependency were significantly correlated with scores for reading (p = 0.01), day-to-day activities (p < 0.001) and outdoor walking and using steps (p = 0.001). CONCLUSIONS: The evaluation of self-reported visual function and vision-related quality of life in patients after macular translocation is necessary to obtain detailed information on treatment effects. Our results indicated improvement in patients' subjective evaluations of visual function, without significant improvement in visual acuity. The postoperative clinical benefits of treatment coincide with subjective benefits in terms of reading ability, quality of life and patient satisfaction. Our study confirms the importance and efficiency of visual rehabilitation with aids for low vision after surgery.

Dose, timing and frequency of subconjunctival 5-fluorouracil injections after glaucoma filtering surgery.

BACKGROUND: Although adjunctive postoperative 5-fluorouracil (5-FU) injections are known to improve the success rate of glaucoma surgery, it is still unknown what dose, timing and frequency of application will give the best results with respect to the inhibition of postoperative scarring and intraocular pressure regulation. We therefore designed the following retrospective investigation. METHODS: We studied 172 eyes from 172 patients who had undergone trabeculectomy with adjuvant 5-FU-therapy. Variations of dosage, timing and frequency were analysed retrospectively. Surgery was defined as a complete success when the patient reached an intraocular pressure under 21 mmHg and a reduction of 20% 12 months after the operation. A relative success was achieved with these criteria under additional local medication. Not reaching these postoperative criteria for a complete success was classified as failure. RESULTS: On average, adjunctive 5-FU-treatment was started 4.6+/-5.85 days postoperatively. The injections contained between 2 mg and 5 mg FU, and the mean total dose was 26.6+/-13.2 mg (range 5-65 mg). Surgery on 94 patients (54.65%) was classified as "complete success", that on 25 patients (14.53%) was classified as "relative success" and that on 53 eyes (30.81%) was classified as "failure" 12 months (+/-3 months) postoperatively. The best results were obtained when the treatment started on or before the first postoperative day (68.0-71.4% complete success; P<0.05). In contrast, an increase in 5-FU dosage did not result in an increased success rate of trabeculectomy. None of the 172 patients suffered from vision-threatening complications such as endophthalmitis or hypotony maculopathy. CONCLUSION: Early treatment with 5-FU significantly increases the success rates of filtering surgery.

Delayed otogenic hydrocephalus after acute otitis media in pediatric patients: the changing presentation of a serious otologic complication.

CONCLUSIONS: The clinical presentation of otogenic dural sinus thrombosis (DST) as a complication of acute otitis media (AOM) can be masked by antibiotic treatment. Morning episodes of vomiting and/or headache, visual impairment and a history of AOM seem to be indicative of otogenic hydrocephalus. We therefore advocate that the MRI scans of patients with similar symptoms should be carefully studied to facilitate the early diagnosis of a potentially life-threatening complication. OBJECTIVE: To describe the frequency, pathognomonic signs, clinical course and outcome of otogenic hydrocephalus and DST as complications of AOM in pediatric patients. MATERIAL AND METHODS: We undertook a retrospective chart review of all pediatric patients (age 1-14 years) treated for otitis media and its complications at an academic medical center between 1999 and 2003. The main outcome measures were otologic and ophthalmologic findings and CT and MRI scans at the beginning of treatment and 3 months later. RESULTS: We report on five cases with otogenic DST following AOM. All but one of them presented initially with diplopia caused by otogenic hydrocephalus. In four cases the otologic complaints had already disappeared by the time of MRI confirmation of the diagnosis. Only one child was referred with severe otologic symptoms. Management included systemic antibiotics, short-term heparin anticoagulation and surgical decompression. In our cases, even after intensive i.v. antibiotic treatment, only surgery led to a significant improvement in the clinical condition.