Still an Unsolved Question: The Place of Cranial Magnetic Resonance Imaging in Acute Acquired Concomitant Esotropia.

PURPOSE: The aim of this study was to collect further data to estimate the risk of relevant intracranial pathology and thereby better assess the need for cranial imaging in children with acute acquired comitant esotropia (AACE). To date, there is still not enough literature on this topic to enable a consensus on the diagnostic algorithm. METHODS: We analyzed data from patients with convergent strabismus who received cranial imaging via magnetic resonance imaging (MRI). Twenty-one patients received a cranial MRI for the diagnostic evaluation of AACE. The age range was from 2 to 12 years, and the mean age at the time of diagnosis was 5.5 years. Of these patients, only one exhibited insignificant MRI findings, with no therapeutic consequences. CONCLUSIONS: Our data add further evidence that AACE without neurological findings or other ophthalmologic anomalies might not be an indication for cranial MRI as a diagnostic screening tool.

Evaluation of Globe Position Asymmetry in Endocrine Orbitopathy Patients and a Control Group - A Computed Tomography-Based 3D Cephalometric Analysis.

Background: Previous studies have shown that patients suffering from endocrine orbitopathy (EO) seem to present with profound asymmetry in proptosis. As asymmetry might pose a major problem in planning decompression surgery, information on the amount of variation between sides and a concise evaluation method should be available. Therefore, a study based on a concise 3D cephalometric analysis was conceived to evaluate globe position. Methods: A 3D-cephalometric analysis was performed on computed tomography (CT) data from 52 orbitopathy and 54 control data sets. Using 36 anatomic landmarks, 33 distances were evaluated to measure sagittal, vertical, and horizontal globe position. Results: EO patients presented with marked exophthalmos and statistically significant asymmetry. Depending on the 2 measured distances, 38% and 42%, respectively, presented sagittal asymmetry of >2 mm, and 12% and 13%, respectively, presented with sagittal asymmetry >4mm. No such asymmetry was seen in the control group. Furthermore, EO patients showed a larger interglobe distance due to lateral globe position. Marked asymmetry correlated with male sex. Proptosis measured to the deep bony orbit correlated with values measured to the orbital aperture or with constructed Hertel values. Conclusions: Use of 3D cephalometry and CT-based analysis confirmed findings from previous clinical studies on profound sagittal asymmetry in EO. Endocrine orbitopathy leads to a sagittal-lateral globe displacement that is even more pronounced in the current study than in earlier investigations. Concerning surgical therapy, presurgical asymmetry, especially if profound, has to be considered to achieve an esthetic symmetrical outcome. Use of 3D orbital analysis is an appropriate method to describe globe position beyond clinical measurements.

Consideration of specific key points improves outcome of decompression treatment in patients with endocrine orbitopathy: pre-/post-OP comparison and biomechanical simulation.

Endocrine orbitopathy is typically treated by resecting orbital walls. This procedure reduces intraorbital pressure by releasing intraorbital tissue, effectively alleviating the symptoms. However, selection of an appropriate surgical plan for treatment of endocrine orbitopathy requires careful consideration because predicting the effects of one-, two-, or three-wall resections on the release of orbital tissues is difficult. Here, based on our experience, we describe two specific orbital sites ('key points') that may significantly improve decompression results. Methodological framework of this work is mainly based on comparative analysis pre- and post-surgery tomographic images as well as image- and physics-based simulation of soft tissue outcome using the finite element modelling of mechanical soft tissue behaviour. Thereby, the optimal set of unknown modelling parameters was obtained iteratively from the minimum difference between model predictions and post-surgery ground truth data. This report presents a pre-/post-surgery study indicating a crucial role of these particular key points in improving the post-surgery outcome of decompression treatment of endocrine orbitopathy which was also supported by 3D biomechanical simulation of alternative two-wall resection plans. In particular, our experimental results show a nearly linear relationship between the resection area and amount of tissue released in the extraorbital space. However, a disproportionately higher volume of orbital outflow could be achieved under consideration of the two special key points. Our study demonstrates the importance of considering natural biomechanical obstacles to improved outcomes in two-wall resection treatment of endocrine orbitopathy. Further investigations of alternative surgery scenarios and post-surgery data are required to generalize the insights of this feasibility study.

3-D cephalometry of the the orbit regarding endocrine orbitopathy, exophthalmos, and sex.

PURPOSE: This study aimed at evaluating the orbital anatomy of patients concerning the relevance of orbital anatomy in the etiology of EO (endocrine orbitopathy) and exophthalmos utilizing a novel approach regarding three-dimensional measurements. Furthermore, sexual dimorphism in orbital anatomy was analyzed. METHODS: Orbital anatomy of 123 Caucasian patients (52 with EO, 71 without EO) was examined using computed tomographic data and FAT software for 3-D cephalometry. Using 56 anatomical landmarks, 20 angles and 155 distances were measured. MEDAS software was used for performing connected and unconnected t-tests and Spearman´s rank correlation test to evaluate interrelations and differences. RESULTS: Orbital anatomy was highly symmetrical with a mean side difference of 0.3 mm for distances and 0.6° for angles. There was a small albeit statistically significant difference in 13 out of 155 distances in women and 1 in men concerning patients with and without EO. Two out of 12 angles showed a statistically significant difference between female patients with and without EO. Regarding sex, statistically significant differences occurred in 39 distances, orbit volume, orbit surface, and 2 angles. On average, measurements were larger in men. Concerning globe position within the orbit, larger distances to the orbital apex correlated with larger orbital dimensions whereas the sagittal position of the orbital rim defined Hertel values. CONCLUSION: In this study, little difference in orbital anatomy between patients with and without EO was found. Concerning sex, orbital anatomy differed significantly with men presenting larger orbital dimensions. Regarding clinically measured exophthalmos, orbital aperture anatomy is an important factor which has to be considered in distinguishing between true exophthalmos with a larger distance between globe and orbital apex and pseudoexophthalmos were only the orbital rim is retruded. Thus, orbital anatomy may influence therapy regarding timing and surgical procedures as it affects exophthalmos.

Effect of orbital wall resection areas in the treatment of patients with endocrine orbitopathy.

In patients treated by orbital wall decompression for endocrine orbitopathy (EO) there is limited evidence on the effect of orbital wall resections. Thus, the aim of this study was to evaluate the effect of one, two, and three-wall resections on orbital parameters to determine if any such correlations exist. Preoperative and postoperative data from all patients at a tertiary care centre who underwent decompression surgery from 2010 - 2020 were digitally analysed. The effect of the number and area of resected walls on orbital area, orbital volume, and Hertel value, and the effect of lateral rim advancement (LARA) were determined. A total of 131 orbital areas showed an increase from a mean (SD) preoperative area of 42.0 (4.6) cm2 to 47.3 (6.1) cm2 postoperatively (p<0.001). In total, the mean (SD) area of osseous wall removed in all patients was 6.2 (1.7) cm2 at the lateral orbit (n = 129), 6.7 (2.3) cm2 at the orbital floor (n = 123), and 5.8 (1.8) cm2 at the medial orbital wall (n =30). The mean (SD) orbital volume increased by 6.0 (3.0) cm3 after decompression. There was also a significant reduction in exophthalmos of 7.3 (3.2) mm (from 25.2 (3.9) to 17.9 (3.5), p<0.001). LARA was performed in 50 patients. Changes in volume and area, and reduction in exophthalmos were not significantly different with or without LARA. The postoperative effects of orbital wall resection are predictable and exhibit a relation with six units of change. Two-wall resection is the most common intervention.

Eyes wide shut: necessity and effect of adjunctive procedures after decompression surgery in patients with endocrine orbitopathy.

BACKGROUND: Orbital decompression surgery is frequently the last therapeutic measure in the surgical treatment of endocrine orbitopathy (EO). Additional rehabilitative and corrective surgical treatments are often used to improve the resulting eyelid stigmata, such as an increased lid aperture and scleral show. The aim of the study was to evaluate the effect of adjunctive surgical procedures after orbital decompression surgery in patients with EO. METHODS: A total of 120 orbitae from 65 patients with EO from 2010 to 2020 at a tertiary care center in Germany were retrospectively evaluated. Ocular surface area (OSA) and vertical palpebral fissures were three-dimensionally analyzed at the following stages: presurgical decompression, postsurgical decompression, and post-adjunctive surgical procedures. For the analysis of vertical palpebral fissures, predefined vertical line distances were measured on the upper and lower lids in the central, medial, and lateral pupillary regions. RESULTS: The initial OSA was 2,98 ± 0.85 cm2, and it decreased significantly after decompression surgery to 2.52 ± 0.62 cm2. After adjunct surgical procedures, OSA further decreased to 2,31 ± 0,55 cm2. Furthermore, a statistically significant reduction in all pupillary parameters was noted after each treatment step. More lid-lengthening procedures were performed on the lower lid than on the upper lid. Canthoplasty (n = 13) was the most frequently performed procedure during rehabilitation. CONCLUSION: Surgical decompression surgery improves OSA and leads to a significant reduction in lid aperture. Adjunctive surgical procedures, addressing the upper and lower lid, have a significant influence on the ongoing clinical course and contribute to a reduction in OSA.

Yokoyama procedure for esotropia associated with high myopia: real-world data from a large-scale multicentre analysis.

PURPOSE: High myopic patients may develop strabismus due to globe dislocation out of the normal extraocular muscle cone. Surgical correction of this strabismus type is possible by joining the superior and lateral rectus muscles without the need for a scleral suture called the Yokoyama procedure. Data from large patient samples and the evaluation of a potential effect of an additional medial rectus recession (MRR) have been lacking so far. METHODS: We pooled retrospective patient data of 14 departments of ophthalmology in Germany and Switzerland and analysed determinants of postoperative results using multivariable regression models. RESULTS: We included 133 patients (mean age: 59.7 ± 13.4 years, surgery between 2008 and 2017) with a mean preoperative esotropia (both Yokoyama with and without MRR) of 23.8°±4.6°. The angle of preoperative esotropia increased with age. The postoperative esotropia was 8.7° ± 9.9°, and six patients were overcorrected. While preoperative esotropia was highly associated with postoperative results, we found no association of additional MRR with any of our postoperative outcome measures. The Yokoyama procedure had a higher absolute effect in patients with higher preoperative esotropia. CONCLUSION: Our study confirms the positive effect of the Yokoyama procedure on strabismus due to high myopia in large-scale real-world data. In some cases, MRR may be needed because of muscle contracture, although additional MRR statistically did not affect the postoperative outcome. In patients with bilateral high myopic strabismus, correction of both eyes seems beneficial. The effect size of the Yokoyama procedure appears to be mainly driven by preoperative esotropia.

Retrospective cohort study of three-wall orbital resection for treatment of endocrine orbitopathy using 3D tomographic data and biomechanical modeling.

BACKGROUND: Surgical treatment of endocrine orbitopathy can be performed by way of resecting orbital walls, which effectively releases superfluous tissue from the surgically enlarged orbital space allowing the eyeballs to move back. Existing approaches aim to select an optimal surgical strategy based on statistical correlations between the extent of the surgical procedure and the resulting bulbus displacement but do not provide an individual surgery plan or predict surgery outcome. METHODS: In this retrospective study, we performed a quantitative analysis of pre- and post-surgery 3D tomographic data of six patients and applied explorative biomechanical modeling of orbital mechanics to dissect factors influencing patient-specific outcome. FINDINGS: Our experimental results showed a large variability of the backward eyeball displacement in dependency on the amount of orbital volume flow, which could partially be described by computational simulation. Our detailed analysis revealed that patients with regular fat tissue show a good correlation between bulbus displacement and relative volume of decompressed tissue, which, in turn, correlates with decrease in hydrostatic pressure. In contrast, patients with fibrotic tissue exhibit significantly reduced and computationally less predictable eyeball translation in response to surgical tissue decompression. INTERPRETATION: Based on the results of this study we see a great potential for quantitative planning of surgical exophthalmos treatment using 3D biomechanical modeling. Conventional approaches to planning of soft tissue interventions consider, however, only the patient's 3D anatomy and widely disregard individual tissue properties. Further investigations are required to establish reliable procedures for assessment of individual tissue properties and incorporating them into patient-specific models of orbital mechanics.

Agreement and Repeatability of Noncycloplegic and Cycloplegic Wavefront-based Autorefraction in Children.

SIGNIFICANCE: Increasing prevalence of refractive error requires assessment of ametropia as a screening tool in children. If cycloplegia is not an option, knowledge about the increase in uncertainty for wavefront-based autorefraction is needed. The cycloplegic agent as the principal variant presents cross-reference and allows for extraction of the influence of accommodation. PURPOSE: The purpose of this study was to determine the repeatability, agreement, and propensity to accommodate of cycloplegic (ARc) and noncycloplegic (ARnc) wavefront-based autorefraction (ZEISS i.Profiler plus; Carl Zeiss Vision, Aalen, Germany) in children aged 2 to 15 years. METHODS: In a clinical setting, three consecutive measurements were feasible for 145 eyes (OD) under both conditions. Data are described by spherical equivalent (M), horizontal or vertical astigmatic component (J0), and oblique astigmatic component (J45). In the case of M, the most positive value of the three measurements was chosen, whereas the mean was applied for astigmatic components. RESULTS: Regarding agreement, differences for ARc minus ARnc were statistically significant: for M, 0.55 (0.55 D; mean [SD]; P < .001), that is, more hyperopic in cycloplegia; for J0, -0.03 (0.11 D; P = .002); and for J45, -0.03 D (SD, 0.09 D; P < .001). Regarding repeatability, astigmatic components showed excellent repeatability: SD < 0.11 D (ARnc) and SD < 0.09 D (ARc). The repeatability of M was SD = 0.57 D with a 95% interval of 1.49 D (ARnc). Under cycloplegia, this decreased to SD = 0.17 D (ARc) with a 95% interval of 0.50 D. The mean propensity to accommodate was 0.44 D from repeated measurements; in cycloplegia, this was reduced to 0.19 D. CONCLUSIONS: Wavefront-based refraction measurement results are highly repeatable and precise for astigmatic components. Noncycloplegic measurements of M show a systematic bias of 0.55 D. Cycloplegia reduces the propensity to accommodate by a factor of 2.4; for noncycloplegic repeated measurements, accommodation is controlled to a total interval of 1.49 D (95%). Without cycloplegia, results improve drastically when measurements are repeated.

Three-dimensional changes of scleral show after surgical treatment of endocrine orbitopathy.

PURPOSE: Surgery in endocrine orbitopathy should address exophthalmos and adjunct stigmata such as increased lid aperture and scleral show. Secondary to decompression, rehabilitative surgical treatment such as blepharoplasty is routinely used to achieve this goal. Until now, however, there has been no investigation to measure the effect of decompression surgery on scleral show and lid aperture 3-dimensionally. MATERIALS AND METHODS: Ocular surface area (OSA) and lid aperture of 34 patients (67 orbits) were measured pre and post decompression surgery in a retrospective investigation using 3-dimensional (3D) stereophotogrammetry. The mean follow-up after decompression was 6 ± 4 months. RESULTS: Mean OSA ranged between 3.1 ± 1.5 cm2 (pre orbital decompression) and 2.5 ± 0.6 cm2 (post orbital decompression). Orbital decompression caused a statistically significant reduction of the surface area of about 19.4% (p < 0.001). Lid apertures showed average values between 12.7 ± 3.3 mm (pre orbital decompression) and 11.3 ± 2.2 mm (post orbital decompression). Thus orbital decompression led to a statistically significant reduction of the palpebral fissure of about 11% (p < 0.001). OSA correlated with lid aperture pre and post surgery (p < 0.001). The extent of OSA reduction showed no correlation with the amount of exophthalmos reduction. CONCLUSION: Our results show that surgical decompression, besides correcting exophthalmos, leads to a significant reduction of scleral show and lid aperture. However, it is not possible to estimate its effect on an individual basis.

Calculation of resected orbital wall areas in the treatment of endocrine orbitopathy.

PURPOSE: Orbital wall decompression is routinely used to treat proptosis in endocrine orbitopathy. Until now, however, there has been no investigation to measure the area/extent of the removed walls. MATERIALS AND METHODS: The inner areas of 154 orbital walls (lateral, inferior, medial) which had been resected in 38 patients were measured using pre- and postsurgical computed tomographic data in Brainlab iPlan software. Furthermore the effect of concomitant centrolateral orbital rim advancement was calculated in 48 cases. Surgery was performed after preoperative planning using intraoperative navigation. RESULTS: The mean area of resected inferior and medial orbital walls lay at 6.7 cm2 and 6.2 cm2, while the area of the lateral orbital wall was 6.9 cm2. Rotation-advancement of the lateral rim added an area of 1.8 cm2 (∼25% of the lateral orbital wall). Comparison of the pre- and postsurgical computed tomographic data showed excellent conformity of the presurgical planning and postsurgical results. CONCLUSIONS: This investigation is a first step in analyzing the potential surgical effect of bony decompression surgery by stating the metric amount of orbital wall removal. Using these data, further studies will be performed in the future.

Magnetic resonance imaging of a phakomatous choristoma.

Phakomatous choristoma is a rare congenital benign tumour in the inferomedial eyelid or orbit that is thought to be of lenticular anlage origin. We describe the MRI findings in an infant boy with histopathologically confirmed phakomatous choristoma.

Blunt forehead trauma and optic canal involvement: finite element analysis of anterior skull base and orbit on causes of vision impairment.

BACKGROUND: Clinical studies report on vision impairment after blunt frontal head trauma. A possible cause is damage to the optic nerve bundle within the optic canal due to microfractures of the anterior skull base leading to indirect traumatic optic neuropathy. METHODS: A finite element study simulating impact forces on the paramedian forehead in different grades was initiated. The set-up consisted of a high-resolution skull model with about 740 000 elements, a blunt impactor and was solved in a transient time-dependent simulation. Individual bone material parameters were calculated for each volume element to increase realism. RESULTS: Results showed stress propagation from the frontal impact towards the optic foramen and the chiasm even at low-force fist-like impacts. Higher impacts produced stress patterns corresponding to typical fracture patterns of the anterior skull base including the optic canal. Transient simulation discerned two stress peaks equalling oscillation. CONCLUSIONS: It can be concluded that even comparatively low stresses and oscillation in the optic foramen may cause micro damage undiscerned by CT or MRI explaining consecutive vision loss. Higher impacts lead to typical comminuted fractures, which may affect the integrity of the optic canal. Finite element simulation can be effectively used in studying head trauma and its clinical consequences.

Therapeutic options for treatment of Merkel cell carcinoma.

Merkel cell carcinoma (MCC) is a rare malignant neuroendocrine carcinoma of the skin. Owing to the aggressiveness of this tumor and the bad overall survival, we reviewed the therapeutic strategies, including surgery, radiation, and chemotherapy to find out the potentially best treatment option for one patient treated at our hospital. In addition, we investigated MCC biopsies using the FLAVINO assay to find out if individual chemoresponse testing might be a useful supplement in decision-making for the optimal therapeutic option for our MCC patient. The different results achieved using cisplatin, docetaxel, and cetuximab led to the conclusion that an individual chemoresponse testing in a predictive short-time assay might potentially be a useful diagnostic tool in identifying potentially effective chemotherapy treatments.

Visual field constriction in children with shunt-treated hydrocephalus.

OBJECT: Many ophthalmological abnormalities are described in conjunction with hydrocephalus. The results of visual field diagnosis remain a matter of further discussion. The aim of this study was to investigate visual field deficits in children with shunt-treated hydrocephalus. METHODS: All children over 6 years of age treated for hydrocephalus at the authors' institute between December 2007 and December 2008 were included in the study. The children underwent an ophthalmological investigation for strabismus and binocular function, ophthalmoscopy, visual acuity, and refraction. The special focus was the visual field diagnosis, which the authors established in all children with cognitive conditions. The investigation was made by using the Goldmann visual field examination (kinetic perimetry). Children with and without visual field defects were compared concerning age at the time of ophthalmological examination, genesis of hydrocephalus, and fronto-occipital horn ratio measured on current CT or MR images. RESULTS: Complete investigations were undertaken in 56 children (24 girls and 32 boys, mean age 15.1 years). The following orthoptic pathological entities were diagnosed: 29 children have a strabismus in 29 cases, 17 of these have an exotropia, 12 an esotropia, 4 children a hypotropia, 2 a hypertropia and 3 children a heterophoria. A nystagmus was found in 10 children. The ocular fundus investigation showed 13 children with an optic nerve atrophy. A visual field diagnosis was possible in 44 of the 56 patients and was incomplete in 12 patients with cognitive deficits or inadequate compliance. In 24 of 42 children there was a concentric visual field constriction between 10° and 50° out of the center. Children with visual field deficits were older than those with a normal visual field (p = 0.051). Nine of 10 children with postmenigitic hydrocephalus had a visual field defect (p = 0.025). In children with visual field defects the fronto-occipital horn ratio was significantly higher (p = 0.013). CONCLUSIONS: The results suggest that children with shunt-treated hydrocephalus have a higher risk of having ophthalmological abnormalities. Visual field deficits are often a problem in these patients. A diagnostic visual field examination can complete the ophthalmological monitoring in patients with hydrocephalus, especially in patients with large ventricles. Children with postmeningitic hydrocephalus should be ophthalmologically monitored more frequently and intensively.

Spheno-orbital meningiomas: outcome after microsurgical treatment: a clinical review of 30 cases.

OBJECTIVE: Spheno-orbital meningiomas represent a delicate subtype of intracranial meningiomas involving the sphenoid wing, orbit and important neurovascular structures such as cavernous sinus, carotid artery or optic nerve. Insidious and aggressive dural, bone and orbital involvement contains several defiances to adequate resection, which provides high rates of recurrence. METHODS: This retrospective case analysis consisted of 30 patients, who were surgically treated for spheno-orbital meningiomas performing a fronto-pterional approach by or under the supervision of the senior author (J. Meixensberger) between May 2001 and February 2006. There were 22 woman and eight men with a mean age of 54.4 years. The follow-up period ranged from 3 to 75 months (mean: 33.7 months). RESULTS: The majority of patients presented with a clinical triad of visual impairment (74%), progressive proptosis (55%) and visual field defects (40%). Total microscopic tumor resection was achieved in ten patients (33%). Visual acuity improved in 65% of the patients, and 40% of these returned to normal vision. Pre-existing cranial nerve deficits remained unchanged in the majority of patients (88%) and improved in 12%. Temporary new cranial nerve deficits occurred in three patients. The rate of permanent non-visual morbidity was 10% (three of 30 patients). Eight patients (27%) received post-operative radiotherapy with an overall tumor growth control rate of 63%. The overall recurrence rate was 27% (eight of 30 patients). CONCLUSION: Sufficient tumor control can be achieved with minimal morbidity and satisfying functional results.

Reconstructive and ophthalmologic outcomes following resection of spheno-orbital meningiomas.

PURPOSE: To assess long-term stability and aesthetic and functional outcomes of orbital reconstruction after resection of spheno-orbital meningiomas using calvarial bone grafts. METHODS: A series of 21 patients were retrospectively examined after an average follow-up period of 65.6 months with regard to their personal satisfaction and also proptosis, ocular function, and reconstructive outcome. The sagittal position of the globe was analyzed by CT and MRI. Patients were treated surgically in an interdisciplinary approach between 1997 and 2006. RESULTS: A permanent postoperative decrease in globe protrusion of 2.7 mm on average was achieved. Most patients (76%) reported identical or improved vision, 85% were satisfied with the reconstructive result, and 55% were free of discomfort in daily life. Seven patients suffered from recurrences that were invariably accompanied by globe protrusion. CONCLUSIONS: Calvarial bone grafts provide good, stable long-term results in the reconstruction of orbital defects. Globe protrusion is a reliable indicator for the detection of recurrences in spheno-orbital meningiomas. Because of high recurrence rates, it is advisable to continue follow-up for 20 or more years.