Impact of sphenoid trigone size and extraocular muscle thickness on the outcome of lateral wall orbital decompression for thyroid eye disease.

BACKGROUND: To retrospectively review lateral wall orbital decompression for thyroid eye disease (TED) and to evaluate pre-operative CT scans to analyse the variation in proptosis reduction. METHODS: Consecutive lateral wall orbital decompressions performed by a single surgeon were retrospectively reviewed. Pre-operative CT scan features and post-operative proptosis reduction were analysed. The sphenoid trigone cross-sectional areas were summed and multiplied by the slice thickness to yield bone volume. Cumulative extraocular muscle thickness was calculated by combining the maximum thickness of the four recti. "Trigone volume" and "cumulative muscle thickness" were correlated with proptosis reduction at 3 months post-surgery. RESULTS: Out of 73 consecutive lateral wall orbital decompressions, 17 orbits had prior endonasal medial wall orbital decompression. In the remaining 56 orbits, the mean pre-operative and post-operative proptosis were 24.3 ± 1.6 mm and 20.9 ± 2.3 mm respectively. The proptosis reduction ranged from 1 to 7 mm (mean of 3.5 mm ± 1.3 (p < 0.001)). Mean sphenoid trigone volume was 895 ± 434.4 mm3. The mean cumulative muscle thickness was 20.4 ± 5 mm. The correlation coefficient between muscle thickness and proptosis reduction was - 0.3 and was statistically significant (p = 0.043). The correlation coefficient between sphenoidal trigone volume and proptosis reduction was 0.2 (p = 0.068). With a multivariate analysis, the coefficient of efficient of regression for muscle thickness was - 0.007 (p = 0.42) and the coefficient of regression for trigone volume was 0.0 (p = 0.046). CONCLUSION: Proptosis reduction following lateral wall orbital decompression can be variable. Extraocular muscle thickness had a significant correlation with the outcome, with greater proptosis reduction in orbits with thin muscles. The sphenoidal trigone size had a weak correlation with decompression outcome.

Hertel-exophthalmometry-like multi-detector-row-CT-exophthalmometry: inter-disciplinary inter-observer reproducibility of measurements.

OBJECTIVES: To investigate the interdisciplinary interobserver reproducibility of Hertel-exophthalmometry-like protrusion measurements on multidetector-row-computed-tomography- (MDCT-) images of the orbit to facilitate structured evaluation of the orbit and mid-face. METHODS: Respective reproducibility of base-length along the interfronto-zygomatic line, right and left ocular protrusion, and deriving interocular difference was measured in this retrospective (04/2009-03/2020) single-centre observational study. MDCT-series and slice-positions were selected independently, using picture-archiving-and-communication-system- (PACS-) tools on tilt-corrected axial MDCT-images (slice-thickness 0.6-3.0 mm, window/centre 350/50 HU) in 37 selected adult patients (24 female, age 57 ± 13 years, average±standard-deviation) with clinical indication for Hertel-exophthalmometry, by one radiology-attending, two ophthalmology-attendings, one critical-care-attending, and one ear-nose-throat-surgery resident, respectively. Bland-Altman plots and Wilcoxon-matched-pairs-signed-rank-tests compared interobserver results. RESULTS: Mean and median interobserver and intraobserver (radiology-attending) deviations were within 1 mm of respective averages of base-length (98 ± 4 mm), right and left ocular protrusion (21 ± 4 mm) and interocular difference (2 ± 1 mm). Relative interobserver deviations were within 2.0% of average (all patients) for base-length, and 5.0% (>80% of patients) for ocular protrusion. Pairwise interobserver comparison showed no significant differences between interocular differences of protrusion. CONCLUSIONS: Respective measurements of base-length, ocular protrusion, and deriving interocular difference show high interdisciplinary interobserver reproducibility in tilt-corrected axial MDCT-images of the orbit or mid-face. ADVANCES IN KNOWLEDGE: Hertel-exophthalmometry-like protrusion measurements did not depend on the years of experience or the medical subspecialty of the observer. Measurements are objective, well reproducible and important for multiple medical disciplines and should thus be included in pertinent radiology reports.

[3D modeling for planning of minimally invasive approach to the orbit]. / Ispol'zovanie 3D-modelirovaniya dlya planirovaniya minimal'no invazivnogo dostupa k oblasti orbity. Sluchai iz praktiki i obzor literatury.

OBJECTIVE: To demonstrate own experience in 3D modeling for planning of minimally invasive approach to the orbit and anterior skull base. MATERIAL AND METHODS: A 17-year-old patient admitted to the Department of Pediatric Neurosurgery with complaints of decreased visual acuity of the left eye, lacrimation and exophthalmos. MRI revealed a tumor of the left orbit. We have preoperatively modeled frontoorbital region, anterior skull, as well as eyeball and tumor within the same model. Considering young age and potentially favorable prognosis of disease, we preferred a minimally invasive intervention (microsurgical resection of tumor through minimally invasive frontoorbital access). RESULTS: Total resection of tumor was followed by examination of anterior skull base. There was postoperative regression of visual disturbances, lacrimation and exophthalmos. Sutures were removed after 5 days, and the patient was discharged. CONCLUSION: Minimally invasive frontoorbital access is adequate for approach to the orbit, anterior and middle cranial fossa, adequate resection of orbital tumor and examination of anterior skull base. 3D modeling is an additional preoperative tool to improve the quality of preoperative planning and facilitate intraoperative navigation.

Post-traumatic orbital sub periosteal hematoma in children: Clinical spectrum and management outcomes.

Orbital subperiosteal hematoma (OSPH) is a rare entity following blunt trauma that can significantly affect the vision of a growing child. The purpose of this study was to describe the spectrum of the clinical presentations along with the imaging findings and treatment outcome of traumatic OSPH. This is a retrospective case series of six patients below 16 years of age, diagnosed with OSPH following trauma. Electronic medical records were reviewed for details of clinical features, imaging findings, management details and outcomes. The median duration of the presentation was 7 days (range 6-50 days). Proptosis and dystopia remained the most common presenting features. Significant vision loss was noted in four patients at presentation. A computed tomography (CT) scan revealed the superior quadrant of the orbit to be involved most frequently. Five patients were treated surgically and one conservatively. Significant vision loss was noted in one patient despite of initial surgical management. Although rare, OSPH should be considered a differential diagnosis in children presenting with proptosis and dystopia following blunt trauma. Younger children are particularly at higher risk for developing permanent visual loss and should be treated promptly by draining the OSPH.

Spontaneous Retrobulbar Hemorrhage in a Previously Healthy Infant.

Spontaneous retrobulbar hemorrhage is a rare yet vision-threatening condition. We reported a 5-month-old male infant with sudden onset of left eye proptosis with no prior history of getting vitamin K injection after birth. Head computed tomography scan revealed retrobulbar and intracranial hemorrhages. Laboratory results showed anemia, prolonged prothrombin and activated partial thromboplastin time, supporting the diagnosis of vitamin K deficiency bleeding. After the bleeding and clotting profile were stabilized, lateral canthotomy and cantholysis; and drainage following orbital decompression were successfully performed, yielded in a completely resolved proptosis. The right timing for surgery with the goal of releasing intraorbital pressure represent the merit of this paper. Our case also led to a crucial emphasis on vitamin K prophylaxis at birth.

Correlation of brachycephaly grade with level of exophthalmos, reduced airway passages and degree of dental malalignment' in Persian cats.

For many years, there has been a trend to breed cats with an increasing degree of brachycephalic head features, which are known to have a severe impact on the animals' health and welfare. The direct relation between different grades of brachycephaly and their negative implications have not been researched in this species. The aim of this study was therefore to establish correlations between the different grades of brachycephaly and reduced upper respiratory airways, exophthalmos of the eye globes and malalignment of the teeth in Persian cats. Sixty-nine Persian cats of various skull dimensions and ten Domestic shorthair cats were recruited for the study. The cats' skulls were examined using three-dimensional reconstructions created from Computed Tomography datasets. Brachycephaly was graded using established craniometric measurements (facial index, cranial index, skull index, craniofacial angle). The flow area of the nasal passageways at different locations, the amount of the eye globe not supported by the bony orbit and the axial deviation of the teeth were quantified and evaluated for a correlation with the grade of brachycephaly. The results of this study clearly show that increased grades of brachycephaly in Persian cats resulted in larger extra-orbital parts of the ocular bulbs. The brachycephalic skull dimension also resulted in a lower height of the naso-osseal aperture, while other areas of the nasal airways were not correlated with the severity of brachycephaly. Persian cats showed a significantly increased occurrence of premolar tooth displacement in the upper jaw with increasing brachycephaly grades. It was interesting to note that the measured values had a broad range and values of some individual Persian cats showed an overlap with those of Domestic shorthair cats.

Metastatic Orbital Tumor From Breast Ductal Carcinoma With Neuroendocrine Differentiation Initially Presenting as Ocular Symptoms: A Case Report and Literature Review.

Background: Orbital metastases from cancers of various organs can arise via the hematogenous route, and many originate from breast, prostate, and lung cancers. Such metastatic orbital tumors may be diagnosed before the primary tumor. We have encountered a case of breast ductal carcinoma with neuroendocrine differentiation that metastasized to the orbit and responded to chemotherapy, with improvement in visual function. Case Presentation: A woman in her fifties visited our ophthalmology department with a chief complaint of foreign body sensation and exophthalmos in her right eye. An elastic soft mass was palpated from the lateral orbit to the temporal region. A systemic examination revealed breast cancer and a metastatic orbital tumor. Excisional biopsy of the breast revealed a diagnosis of invasive ductal carcinoma with neuroendocrine differentiation, and immunohistochemical examination was negative for cytokeratin 7, making the case unusual. Chemotherapy was remarkably effective, and the tumor size decreased, resulting in improvement of visual function. Her general condition and quality of life are still good at present. We searched the PubMed English language literature focusing on metastatic orbital tumors from breast cancer in which ocular symptoms had been the initial presenting sign. No previous reports have documented neuroendocrine differentiation or cytokeratin 7 expression in isolated orbital metastases from breast cancer. Although it is not possible to be certain from this case alone, we speculated that some such cases might involve cytokeratin 7-negative invasive breast cancer with neuroendocrine differentiation. Conclusion: We have described our experience of a very rare case of cytokeratin 7 negative breast ductal carcinoma with neuroendocrine differentiation that metastasized to the orbit and formed a solitary giant tumor initially manifesting as ocular symptoms.

Unilateral periorbital swelling in children: avoid delays in diagnosis.

A 10-year-old child had painful periorbital swelling in the left eye. It was diagnosed as preseptal cellulitis and treated with oral antibiotics. Three days later, the ocular condition worsened so the child was referred for further management. On examination, the child had a temperature of 102 °F. Ocular examination revealed proptosis, restricted ocular movements and a relative afferent pupillary defect in the left eye. Ocular examination of the right eye was normal. There was a history of recurrent episodes of cold in the past. CT scan orbit and sinuses revealed signs of orbital cellulitis with sinusitis on the left side. The child was treated with parenteral antibiotics and endoscopic sinus surgery. A child presenting with unilateral periorbital swelling needs to be thoroughly evaluated. It is important to differentiate orbital cellulitis from preseptal cellulitis. Orbital cellulitis is an emergency and delay in diagnosis can lead to vision and life-threatening intracranial complications.

Quantitative analysis of extraocular muscle volume and exophthalmos reduction after radiation therapy to treat Graves' ophthalmopathy: A pilot study.

PURPOSE: The aim of this study was to provide radiological information on the inherent response of Graves' ophthalmopathy after radiation therapy. METHODS: Quantitative analysis of extraocular muscle volume was performed on 96 involved extraocular muscles in a total of 16 patients. A total of 48 computed tomography images were analyzed. Exophthalmos was also measured. The percentage reductions in extraocular muscle volume and exophthalmos length were determined and compared to the pre-radiation therapy values at 6-, 12-, and 24-month follow-up. RESULTS: The median follow-up time was 21.5 months (range: 7.2-29.4 months). The mean reduction in relative tumor volume compared to the pre-radiation therapy extraocular muscle volume was 46.1% (range: 33.3%-58.8%). The mean relative extraocular muscle volumes were 71.5% at 6 months, 59.2% at 12 months, and 54.3% at 24 months after radiation therapy. The volume of the involved extraocular muscles decreased rapidly within the first 12 months of follow-up. The mean pre-radiation therapy length of exophthalmos was 21.7 mm (range: 17.6-26.1 mm). The mean percentages of exophthalmos length by comparison with the pre-radiation therapy length were 96.7% at 6 months, 92.3% at 12 months, and 88.5% at 24 months after radiation therapy. Exophthalmos decreased slowly and steadily during the follow-up period. CONCLUSION: Quantitative volumetric analysis of the pattern of extraocular muscle volume reduction and exophthalmos length reduction in response to radiation therapy will allow clinicians to better understand the effect of radiotherapy on Graves' ophthalmopathy.

The feasibility analysis of calculating proptosis by simple Pythagorean theorem.

PURPOSE: We propose a new method to analyze the feasibility of calculating proptosis on the basis of simple Pythagorean theorem. METHOD: This is a non-inferiority trial, and the registration number is ChiCTR1900026490. The absolute value of proptosis of two eyes of patients without eye injury or diseases visiting our clinic from December 2017 to June 2019 was measured by computed tomography, Hertel exophthalmometer, and by simple Pythagorean theorem. With the application of MedCalc software version 19.0.4, the differences between these methods in two eyes were showed by Bland and Altman plot. RESULTS: A 95% limit of agreement between computed tomography and Hertel exophthalmometer is -0.7 to 0.62 mm in right eye proptosis. A total of 4.44% (4/90) points were outside 95% limit of agreement. Similarly, the same method was used to compare the proptosis between computed tomography and other ways. We also compared the proptosis measured by homolateral and heterolateral simple Pythagorean theorem method in order to find out the consistence between them. The points in all Bland and Altman plots were lower than 5%, which means that the results of comparison between any two methods had a good consistency in the measurement of proptosis of both eyes. CONCLUSION: Pythagorean theorem can be applied to evaluate proptosis and has a good consistency comparing with computed tomography and the Hertel exophthalmometer. The method can be used for measuring proptosis of unilateral orbital, maxillofacial trauma, and dysplasia accurately. It is practical in clinical use of proptosis assessment because of its accuracy, reliability, and simplicity.

Acute Orbital Compromise after Intra-Arterial Chemotherapy in a Complex Retinoblastoma Associated with 13q Deletion Syndrome.

INTRODUCTION: The intra-arterial chemotherapy (IAC) is increasingly used as a first-line therapy for retinoblastoma. The IAC has proved to be relatively safe. However, many local side effects of IAC have been described. CASE PRESENTATION: This case report describes a local side effect presenting as proptosis and myositis with vascular access difficulty of the middle meningeal artery, in a 2-year-old male with left eye diffuse multifocal stage Vb retinoblastoma complicated with retinal detachment. DISCUSSION/CONCLUSION: IAC is assured to provide as efficient results in eliminating the tumor as the systemic chemotherapy, without causing the systemic side effects. It has become an alternative to systemic chemotherapy. A better understanding of the local side effects is required.

Juvenile Psammomatoid Ossifying Fibroma of the Orbital Roof: A Rare Cause of Proptosis among Children.

INTRODUCTION: Juvenile psammomatoid ossifying fibroma (JPOF) is a rare bone tumor characterized by a predilection for the sinonasal region and a tendency to affect younger patients, with a potential for aggressive growth and high recurrence (30-56%). JPOF warrants complete surgical resection to avoid recurrence. CASE PRESENTATION: In this article, we report a young boy who presented with unilateral prop-tosis with an expansile bony tumor with ground glass appearance involving the left frontal bone and orbital roof on his images. Complete surgical resection was done, and histopathological examination revealed JPOF with abundant psammomatoid bodies. DISCUSSION: This patient is a rare case of neurocranial JOPF and adds new features to the typical features already described for JPOF.

Orbital Decompression for Exorbitism and Exophthalmos in a Patient With Thyroid Eye Disease.

A 34-year-old man with Basedow disease consulted us to treat disfiguring proptosis. On examination, the patient showed right lower eyelid retraction and right inferior rectus muscle enlargement, which were consistent with thyroid eye disease. Hertel's exophthalmometric examination demonstrated proptosis of 25.5 and 24.0 mm on the right and left sides, respectively. Computed tomography (CT) images revealed absence of the sphenoid door jamb. To correct both exorbitism and exophthalmos, the patient underwent bilateral "shallow" decompression of the lateral and medial orbital walls with orbital fat removal under general anesthesia. The authors removed 2.5 and 1.0 mL of orbital fat from the inferolateral quadrant of the right and left orbits, respectively. At 3 months postoperatively, based on the measurements of the axial globe position on sagittal CT images, postoperative proptosis reductions of 7.5 and 6.5 mm were observed on the right and left sides, respectively.

Blebophthalmos: proptosis due to a massive bleb following implant surgery in refractory paediatric glaucoma.

A 5-year-old boy with failed combined trabeculotomy and trabeculectomy underwent Ahmed glaucoma valve (AGV) implantation and presented 5 weeks postoperatively, with sudden onset proptosis downward displacement of the globe, limited extraocular movements and displacement of tube from anterior chamber. CT scan of the orbits showed a large cystic bleb with posterior dislocation of the implant and was explanted. In vitro testing of implant for fluid flow characteristics revealed a dysfunctional valve with anterior leak, which could have possibly lead to anterior and inferior fluid accumulation resulting in displacement of implant, a probable cause for this rare complication. The case was successfully managed later with the Aurolab aqueous drainage device implantation. This case report cautions the surgeon for the possibility of a large cystic bleb following abnormal leak in AGV resulting in orbital complications like proptosis, motility limitation and impending optic nerve compression.

Zigomicosis y síndrome hemofagocítico en un paciente inmunocompetente: informe de caso / Zygomycosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient: a case report

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)

Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)

A Novel Method of CT Exophthalmometry in Patients With Thyroid Eye Disease.

PURPOSE: Conventional computed tomography (CT) exophthalmometry requires an intact lateral orbital wall and is therefore not feasible in patients who have undergone any form of lateral orbital wall surgery where the normal bony landmark may be lost or displaced. The purpose of our study is to validate an alternative method of CT exophthalmometry utilizing the posterior clinoid (PC) process as a new reference point that will allow for reproducible comparison of the anterior-posterior globe position in the preoperative and postoperative settings. DESIGN: Cohort study. METHODS: This is a retrospective study of 48 patients with clinically diagnosed thyroid eye disease who had undergone cross-sectional CT imaging in the pre- or postoperative settings. CT exophthalmometry was performed using both the conventional interzygomatic method and our proposed PC process method on all pre- and postoperative CT imaging by two independent observers. Interobserver variability analysis was performed with intraclass correlation coefficient. Correlation and agreement between the two methods were analyzed with Pearson correlation coefficient and linear regression method. All analyses were conducted at 5% level of significance with Stata MP V14. RESULTS: Interobserver variability analysis showed an intraclass correlation coefficient of >0.9 for both interzygomatic and PC methods. There is good correlation between the two different measurements observed in both the pre- and postoperative groups (r = 0.68 and r = 0.72, respectively, P < 0.001). Linear regression showed good agreement between the two different measurements with most of the points lying within the 95% limits. CONCLUSIONS: Our new method agrees well with the conventional method and has the added benefit of being able to reliably assess the anterior-posterior globe position in patients who do not have intact lateral orbital walls after decompressive surgery.

MR imaging features of orbital Langerhans cell Histiocytosis.

BACKGROUND: To investigate the magnetic resonance imaging (MRI) features of orbital Langerhans cell histiocytosis (LCH) to improve diagnostic accuracy. METHODS: We retrospectively reviewed clinical manifestations and MRI findings of 23 patients with histopathology-confirmed LCH of the orbit. The findings were evaluated for the following: (a) symptoms, (b) disease duration, (c) location, (d) configuration, (e) margin, (f) MR imaging signal intensity and enhanced performance. RESULTS: Eighteen patients (78%) in our series were male, only five (22%) patients were female, and the mean age at presentation was 6.3 years. The common symptoms include swollen eyelids, exophthalmos, and a palpable mass. Fourteen patients presented with swollen eyelids and/or exophthalmos. Twenty-two cases involved unilateral orbits, and one case involved bilateral orbits. In our study, there was one patient with cough and expectoration, and one patient with polydipsia and polyuria. Lesions were located in the superior or superlateral orbital roof of seventeen patients (74%). Lesions formed masses or irregular shapes. The 12 out of 23 (52.2%) cases appeared heterogeneous isointense and 10 out of 23 (43.5%) cases showed iso-hypointense on T1-weighted imaging, there were 15 out of 23 (65.2%) cases showed hyper-hypointense mixed signals on T2-weighted imaging. 7 cases found patchy hyperintense signal on T1WI, and 11 cases showed markedly hyperintense signal near the edge of lesions on T2WI. After enhancement, 21 out of 23 (91.3%) cases lesions presented marked enhancement at the edges and the surrounding tissues, and with heterogeneous obvious enhancement of the lesion center. Besides, four cases lesions were surrounded by a low circular signal. CONCLUSION: There were several characteristics MRI features that can provide crucial information for clinicians and improve our understanding and the diagnostic accuracy of the orbital LCH.