[Orbital inflammatory diseases]. / Entzündliche Erkrankungen der Orbita.

CLINICAL/METHODICAL ISSUE: Inflammatory orbital processes on imaging are often misinterpreted as tumors. STANDARD RADIOLOGICAL METHODS: Imaging comprises computed tomography (CT) and magnetic resonance imaging (MRI). ACHIEVEMENTS: Clinical and laboratory data play a crucial role in diagnosing many inflammatory orbital diseases. Radiological imaging provides a supporting but relevant role. PRACTICAL RECOMMENDATIONS: Clinical examination, including specialized ophthalmological examinations, laboratory diagnostics, and MRI are important in the diagnosis of inflammatory orbital diseases.

Pediatric orbital lesions: ocular pathologies.

Orbital pathologies can be broadly classified as ocular, extra-ocular soft-tissue (non-neoplastic and neoplastic), osseous, and traumatic. In part 1 of this orbital series, the authors will discuss the differential diagnosis and key imaging features of pediatric ocular pathologies. These include congenital and developmental lesions (microphthalmos, anophthalmos, persistent fetal vasculature, coloboma, morning glory disc anomaly, retinopathy of prematurity, Coats disease), optic disc drusen, infective and inflammatory lesions (uveitis, toxocariasis, toxoplasmosis), and ocular neoplasms (retinoblastoma, retinal hamartoma, choroidal melanoma, choroidal nevus). This pictorial review provides a practical approach to the imaging work-up of these anomalies with a focus on ocular US as the first imaging modality and additional use of CT and/or MRI for the evaluation of intracranial abnormalities. The characteristic imaging features of the non-neoplastic mimics of retinoblastoma, such as persistent fetal vasculature and Coats disease, are also highlighted.

Pediatric orbital lesions: bony and traumatic lesions.

Orbital pathologies can be broadly classified as ocular lesions, extraocular soft-tissue pathologies (non-neoplastic and neoplastic), and bony and traumatic lesions. In this paper, we discuss the key imaging features and differential diagnoses of bony and traumatic lesions of the pediatric orbit and globe, emphasizing the role of CT and MRI as the primary imaging modalities. In addition, we highlight the adjunctive role of ocular sonography in the diagnosis of intraocular foreign bodies and discuss the primary role of sonography in the diagnosis of traumatic retinal detachment.

Pediatric orbital lesions: non-neoplastic extraocular soft-tissue lesions.

Orbital pathologies can be broadly classified as ocular, extraocular soft-tissue (non-neoplastic and neoplastic), osseous, and traumatic. In this paper, we discuss the key imaging features and differential diagnoses of congenital and developmental lesions (dermoid cyst, dermolipoma), infective and inflammatory pathologies (pre-septal cellulitis, orbital cellulitis, optic neuritis, chalazion, thyroid ophthalmopathy, orbital pseudotumor), and non-neoplastic vascular anomalies (venous malformation, lymphatic malformation, carotid-cavernous fistula), emphasizing the key role of CT and MRI in the imaging work-up. In addition, we highlight the adjunctive role of ocular ultrasound in the diagnosis of dermoid cyst and chalazion, and discuss the primary role of ultrasound in the diagnosis of vascular malformations.

Transcutaneous retrobulbar amphotericin B for rhino-orbital-cerebral mucormycosis: a multi-center retrospective comparative study.

PURPOSE: To assess whether transcutaneous retrobulbar amphotericin B injections (TRAMB) reduce exenteration rate without increasing mortality in rhino-orbital-cerebral mucormycosis (ROCM). METHODS: In this retrospective case-control study, 46 patients (51 eyes) with biopsy-proven ROCM were evaluated at 9 tertiary care institutions from 1998 to 2021. Patients were stratified by radiographic evidence of local orbital versus extensive involvement at presentation. Extensive involvement was defined by MRI or CT evidence of abnormal or loss of contrast enhancement of the orbital apex with or without cavernous sinus, bilateral orbital, or intracranial extension. Cases (+TRAMB) received TRAMB as adjunctive therapy while controls (-TRAMB) did not. Patient survival, globe survival, and vision/motility loss were compared between +TRAMB and -TRAMB groups. A generalized linear mixed effects model including demographic and clinical covariates was used to evaluate the impact of TRAMB on orbital exenteration and disease-specific mortality. RESULTS: Among eyes with local orbital involvement, exenteration was significantly lower in the +TRAMB group (1/8) versus -TRAMB (8/14) (p = 0.04). No significant difference in mortality was observed between the ±TRAMB groups. Among eyes with extensive involvement, there was no significant difference in exenteration or mortality rates between the ±TRAMB groups. Across all eyes, the number of TRAMB injections correlated with a statistically significant decreased rate of exenteration (p = 0.048); there was no correlation with mortality. CONCLUSIONS: Patients with ROCM with local orbital involvement treated with adjunctive TRAMB demonstrated a lower exenteration rate and no increased risk of mortality. For extensive involvement, adjunctive TRAMB does not improve or worsen these outcomes.

Case Report: Three-Day Albendazole Regimen for Orbital Cysticercosis and the Stardust Sign.

Ultrasonography findings have been used to diagnose and treat 10 cases of orbital cysticercosis. Although oral prednisolone has a key role in symptomatic alleviation, 3-day albendazole has been demonstrated to be curative without any recurrence.

Transnasal endoscopic drainage of neonatal orbital abscess.

Neonatal orbital complications are rare and potentially fatal, demanding prompt diagnosis and adequate treatment. A 25-day-old neonate presented with rapidly progressive orbital complications as evidenced by proptosis, chemosis, lid oedema and restricted eye movements, developing within 3 days. There was no significant medical history or risk factors for developing infection. An initial conservative approach with antimicrobial therapy failed to show any resolve. An MRI brain, orbits and paranasal sinuses demonstrated that there were features suggestive of right orbital cellulitis with possibility of abscess formation with right ethmoidal mucoinflammatory disease and mass effect on the optic nerve causing stretching and compression by the surrounding inflammation.The patient was treated successfully with transnasal endoscopic drainage and decompression. Endoscopic access was challenging owing to the restrictive anatomy. Postoperatively, the patient showed improvement, with gradual decrease in proptosis and resolve in eye movements.

Infectious and Inflammatory Processes of the Orbits in Children.

Most primary orbital pathology in children is due to bacterial infection. Radiologists typically encounter these cases to evaluate for clinically suspected postseptal orbital involvement. Contrast-enhanced cross-sectional imaging is important for the detection and early management of orbital infection and associated subperiosteal/orbital abscess, venous thrombosis, and intracranial spread of infection. Benign mass-like inflammatory processes involving the pediatric orbit are rare, have overlapping imaging features, and must be distinguished from orbital malignancies.

Orbital abscess a rare complication after bichectomy surgery.

Bichectomy is the partial removal of the Bichat's fat pad for the aim of smoothing the facial contour. The complications of bichectomy include soft tissue infections, hematoma, facial paralysis (especially buccal branch paralysis), stenon canal injuries and related complications such as sialocele and sialoadenitis, however these are not common complications in clinical practice. Here we report a case of a 29-year-old white female with a right sided orbital abscess following bichectomy. Right sided orbital abscess and orbital cellulitis was managed with endoscopic decompression and drainage. The patient healed completely with no sequela. The frequency of major complications leading to moridity after bichectomy is rare in the literature. This is the first reported intraorbital complication of bichectomy.

Acute-Onset Orbital Inflammation in a Patient With Multiple Myeloma.

A 72-year-old man had right-eye conjunctival injection, chemosis, eyelid edema, and pain with eye movements. Examination showed visual acuity of 20/50, reduced ocular motility in all gazes, proptosis, and chemosis. Treatment with vancomycin and ceftriaxone and ampicillin-sulbactam was followed by symptom worsening. What would you do next?

High risk and low prevalence diseases: Orbital cellulitis.

INTRODUCTION: Orbital cellulitis is an uncommon but serious condition that carries with it a potential for significant morbidity. OBJECTIVE: This review highlights the pearls and pitfalls of orbital cellulitis, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: Orbital cellulitis refers to infection of the globe and surrounding soft tissues posterior to the orbital septum. Orbital cellulitis is typically caused by local spread from sinusitis but can also be caused by local trauma or dental infection. It is more common in pediatric patients compared to adults. Emergency clinicians should first assess for and manage other critical, sight-threatening complications such as orbital compartment syndrome (OCS). Following this assessment, a focused eye examination is necessary. Though orbital cellulitis is primarily a clinical diagnosis, computed tomography (CT) of the brain and orbits with and without contrast is critical for evaluation of complications such as abscess or intracranial extension. Magnetic resonance imaging (MRI) of the brain and orbits with and without contrast should be performed in cases of suspected orbital cellulitis in which CT is non-diagnostic. While point-of-care ultrasound (POCUS) may be useful in differentiating preseptal from orbital cellulitis, it cannot exclude intracranial extension of infection. Management includes early administration of broad-spectrum antibiotics and ophthalmology consultation. The use of steroids is controversial. In cases of intracranial extension of infection (e.g., cavernous sinus thrombosis, abscess, or meningitis), neurosurgery should be consulted. CONCLUSION: An understanding of orbital cellulitis can assist emergency clinicians in diagnosing and managing this sight-threatening infectious process.

Imaging Spectrum of Coronavirus Disease- 2019 Associated Rhino-Orbital-Cerebral Mucormycosis; From Sinonasal Inflammation to Intracranial Involvement.

RATIONALE AND OBJECTIVES: Rhino­Orbital­Cerebral Mucormycosis (ROCM) is a life-threatening opportunistic fungal infection, which mostly affects immunocompromised patients. There has been a notable rise in the incidence of ROCM during the COVID-19 outbreak. In this study we described imaging characteristics of ROCM in detail, from early sinonasal inflammation to late intracranial involvement. MATERIALS AND METHODS: In this retrospective study, Computed Tomography (CT) scan and Magnetic Resonance Imaging (MRI) of 48 patients with proven ROCM in biopsy or culture were evaluated. All the patients had a history of COVID-19 infection within the previous three months. The imaging findings were described and the frequency of different parameters was reported. RESULTS: Paranasal inflammation was detected in all the patients on imaging. The most common involved paranasal sinuses were ethmoid sinuses (97.9%). On diffusionweighted images, restricted diffusion was seen in the paranasal sinuses of 81.1% of the patients. In addition, sinus wall bone involvement was observed in 87.5% of the cases. The most common anatomical sites for extrasinus involvement were the retroantral soft tissue (89.6%) and orbital cavity (87.5%). Dacryocystitis in 50%, optic nerve inflammation in 43.2%, globe involvement in 18.9%, and trigeminal nerve involvement in 16% of the patients were detected. There was extension of inflammation through the cavernous sinuses and alongside the internal carotid arteries in 24% of the patients. CONCLUSION: Characteristic imaging findings of ROCM not only play a vital role in the early diagnosis of this infection, but they also contribute to the assessment of the extension of inflammation, which is vitally important in surgical planning.

Fetal Orbital Epidermal Cyst Pre-postnatal Imaging Findings: A Case Report and Literature Review.

BACKGROUND: Fetal ultrasonographic evaluation is a routine part of pregnancy follow-up, and examination of orbital structures is also part of the routine evaluation. Although orbital developmental anomalies are common in the neonatal period, diagnosis in the intrauterine period is not common. To our knowledge, three cases with a diagnosis of congenital orbital epidermal cysts have been reported in the literature, and two of them had fetal imaging findings. In this article, we present the prepostnatal imaging findings of a case diagnosed with orbital cyst in the fetal period and histopathologically diagnosed as epidermal cyst in the neonatal period. CASE REPORT: A 25-year-old woman applied for ultrasonography (USG) examination at 22 weeks of gestation. A 35x45 mm cystic lesion causing proptosis, without solid component and vascularity, surrounding the optic nerve and causing its thinning was observed in the left orbit. In fetal magnetic resonance imaging (MRI), the intraorbital cystic lesion, which was hyperintense on T2W images and hypointense on T1W images, had no relationship with intracranial structures and no solid component. The lesion of the patient, followed up with a multidisciplinary approach, was shown similarly with computed tomography (CT) and magnetic resonance imaging (MRI) in the postnatal period. Subsequently, the patient underwent globe-sparing surgery, and the pathological diagnosis was made as the epidermal cyst. CONCLUSION: Orbital epidermal cysts are rarely seen, and detection in the fetal period is even rarer. It should be considered in the differential diagnosis of orbital cystic lesions that cause proptosis in fetal and neonatal periods.

Ectopic orbital brain tissue: a case report with radiographic and clinical review.

Orbital heterotopic brain tissue is a rare entity with heterogenous clinical features requiring a multi-faceted diagnostic approach. The authors present a case of ectopic orbital brain tissue in an infant with a comprehensive literature review to highlight the radiographic findings of these lesions. Imaging findings are variable but describe well-circumscribed homogenous lesions with variable enhancement, without communication intracranially. The combination of computed tomography and magnetic resonance imaging can identify associated bony abnormalities, lesion-specific features, and effects on surrounding structures, which in combination with the clinical exam can be a valuable diagnostic and surveillance tool. Although ectopic orbital brain tumors are benign with excellent outcomes following complete resection, conservative management with observation and serial imaging may be an alternative method of management in patients with mild, non-vision threatening, non-distorting tumors.

Osteolytic sarcoidosis of the orbit without pulmonary involvement.

A 69-year-old woman with a history of a left orbital mass presented to the emergency room with progressive breakthrough pain in her left orbit despite medical therapy. On examination, there was extraocular motility restriction with diplopia upon left supraduction. Computed tomography (CT) scan of the orbits revealed soft tissue thickening of the left medial and superior periorbita and left lacrimal fossa; bony erosion of the left frontal bone, left orbital roof, and left lamina papyracea; and bilateral mass-like enlargement of the extraocular muscles. An orbitotomy with incisional biopsy was performed, and histopathological examination revealed non-caseating granulomatous inflammation consistent with sarcoidosis. Chest imaging demonstrated no sequela of pulmonary sarcoidosis, and her serum angiotensin converting enzyme (ACE) level was within normal range. She was treated with high-dose oral steroids with resolution of her symptoms. Her pain returned at the conclusion of the steroid taper, and it was controlled with chronic subcutaneous methotrexate and adalimumab injections.

Erdheim-Chester disease and vemurafenib: a review of ophthalmic presentations and clinical outcomes.

PURPOSE: To provide a comprehensive review of ocular and orbital manifestations of Erdheim-Chester Disease (ECD) and compare clinical outcomes with vemurafenib (INN) to historical treatments (HT). Primary outcomes are ophthalmic findings on presentation, changes in visual acuity, and mortality rate. Secondary outcomes include the progression of ocular findings, systemic involvements, and treatment modalities. METHODS: All published literature from January 1983 to March 2021 was searched for ophthalmic manifestations of ECD. Clinical outcomes following HT were collected and compared with INN. RESULTS: Forty-seven patients with ECD and ophthalmic presentations were identified. The mean age was 49.6 years (SD = 15.0). Proptosis (65.6%) and extraocular muscle restrictions (42.5%) were the most common presenting signs. Of 41 (87.2%) patients with orbital masses on radiologic examination, 90.2% were bilateral, and 53.7% were located in the intraconal space. Ophthalmic examination was significant for xanthelasma (27.2%), optic disc edema (34.0%), and subretinal changes (21.3%). Common treatments were systemic steroids (76.6%), interferon-α (17.0%), and cyclophosphamide (14.9%). INN was less commonly used (12.8%). The mean change in logMAR visual acuity declined with HT (29.9%) but improved with INN (79.1%) (p > 0.05). The proportion of eyes with complete vision loss increased after HT (p < 0.05). The overall mortality rate was 27.7% and notably higher in the HT group (29.3%) when compared to the INN group (16.7%) (p > 0.05). CONCLUSION: ECD presents with many ophthalmic manifestations. Although the intraocular treatments remain controversial, INN should be highly considered in treating orbital ECD patients with BRAF-V600E mutations to prevent and reverse vision loss.

Radiological Determinants of Complicated Immunoglobulin G4-Related Ophthalmic Disease: A Territory-Wide Cohort Study.

PURPOSE: To evaluate the presenting radiological features of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). METHODS: A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. RESULTS: A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. CONCLUSIONS: In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.