Proptosis Research Articles

Heavy duty

A 54-year-old woman with myopia, hypothyroidism, and asthma presented with intermittent double vision. Serum was positive for anti-low density lipoprotein receptor-related protein 4 (LRP4) antibodies suggesting the diagnosis of myasthenia gravis. There was no improvement with pyridostigmine, prednisone, or azathioprine. Over time her double vision became constant, and she was noted to have mild limitation of abduction in both eyes with an esotropia, left hypotropia, and excyclotorsion. Magnetic resonance imaging showed nasal displacement of the superior rectus and inferior rectus muscles, inferior displacement of the lateral rectus muscles and superotemporal globe prolapse consistent with heavy eye syndrome (HES). The differential diagnosis, clinical and radiological manifestations of HES are discussed.

ANAESTHESIA MANAGEMENT IN A CASE OF HYPERTHYROID WITH PROPTOSIS FOR ENDOSCOPIC DECOMPRESSION

This article describes a case of a 65-year-old man with hyperthyroid and who underwent left orbit endoscopic decompression for left eye proptosis. The article also discusses the management of thyroid eye disease (TED) in general. Thyroid associated orbitopathy, also known as Graves orbitopathy, is the most common cause of proptosis ( bulging of the eye). It is an autoimmune disease that affects the muscles and fatty tissues around the eye. TED is most commonly associated with Graves disease, an autoimmune disease that causes an overactive thyroid gland. However, TED can also occur in people with normal thyroid function or an hyperactive thyroid gland. The article presents a case of a 65-year-old man with TED who underwent decompressive surgery to relieve pressure . The article also discusses the importance of maintaining euthyroid (normal thyroid function) in patients with TED.

Optic Nerve Pilocytic Astrocytoma in a Pediatric Patient: A Case Report

Pilocytic astrocytoma is a type of low-grade glioma that can develop in any part of the central nervous system. It primarily affects individuals in the pediatric and young adult age groups. Optic nerve pilocytic astrocytoma is an uncommon, gradually developing type of brain tumor known as a glioma. It is categorized as a grade I tumor by the World Health Organization (WHO). We aim to report rare optic nerve pilocytic astrocytomas and discuss their clinical findings and interconnection with the current literature. A 7-year-old male patient exhibited left-sided eye proptosis and complete loss of vision. A magnetic resonance imaging (MRI) performed before surgery showed the presence of a tumor in the left eye socket. The mass has a spherical shape and smooth boundaries, resulting in the compression of the left optic tract from behind. The left optic tract appears darker on T1-weighted images and brighter on T2-weighted images. On contrast administration, there is heterogenous contrast enhancement. A total resection of the mass was performed. Histopathology results show pilocytic astrocytoma (WHO Grade I). Optic nerve pilocytic astrocytoma is a tumor that can affect the optic nerve pathway. Early diagnosis and multidisciplinary team management are required. Treatment must be individualized, and the options include chemotherapy, radiotherapy, and surgical intervention. Surgery is only recommended in cases of painful or disfiguring proptosis and exposure keratopathy in eyes with severe vision impairment.

Un caso de exoftalmos progresivo en una niña de 5 años

Introduction: spindle cell sarcoma is a rare tumor, with little presentation in the literature. Soft tissue tumors constitute a heterogeneous group, requiring a multidisciplinary approach. Clinical case: 5-year-old schoolboy who goes to his pediatrician for exophthalmos. His mother refers a progressive proptosis in the left eye of 6 months of evolution. No loss of visual acuity, in the last weeks intermittent pain with eye movements. On examination proptosis of the left eye with intermittent tearing and vascular lesion in the temporal portion of the same eye, he presented restriction of left eye movements. He was referred to the hospital emergency department where a head CT scan (Figure 1,2) was performed, revealing a unilateral left retroorbital lesion measuring 28 x 14 mm, causing proptosis, mass effect on retroocular structures and loss of ocular sphericity. Given the findings in the imaging tests, it was decided surgical intervention by means of frontotemporal craniotomy, subsequently receiving complementary treatment with local radiotherapy. Due to negative molecular pathology studies, the result was finally interpreted as undifferentiated 'adult-type' spindle cell sarcoma. Conclusion: although undifferentiated spindle cell sarcoma is a rare pathology, it should be included in the differential diagnosis of retro-orbital pathologies. The objective of this case is to review the main characteristics of spindle cell sarcoma, given its rapid and aggressive form of presentation, in a rare tumor.

Orbital solitary fibrous tumor

Introduction: Solitary fibrous tumor (SFT) is a rare borderline mesenchymal tumor typically arising in the pleura and involving the orbit as its most common extra-pleural locationCase description: We herein describe two cases of orbital SFT arising in both a 69-year-old woman presenting with progressive proptosis of the left eye and a 49-year-old woman presenting with binocular diplopia. The diagnoses relied on histopathological analysis of biopsy samples. Because of the poor local prognosis, we decided to perform an orbital exenteration in the first case. In the second case, a complete resection surgery was performed.Conclusion: SFT presents classics histological features and immunohistochemical markers that are essential to the diagnosis.SFT is classified as a benign tumor, but in some cases, it can show aggressive behavior with the potential for local tissue invasion and, more scarcely, distant metastasis. This is why complete resection remains the treatment of choice for SFT.

Recurrent Frontal Sinus Mucocle—A Novel Management Approach

A mucocele, or collection of mucoid secretion and desquamated epithelium within a paranasal sinus with distension of its walls, is considered a destructive lesion that resembles an expansile cyst. It can erode bone and therefore may extend in to the orbit or the cranium. The frontal sinus experiences the majority incidence, followed by the ethmoid sinus. This case of frontal sinus mucocele presented 8 years after an event of blunt trauma. The individual presented with a nonresolving right frontal head ache, proptosis of right eye and diplopia for the past 6 weeks. This case report emphasizes on the management of a large, chronic mucocele with prompt resolution of signs and symptoms.

Sinus surprise: The rare encounter of frontal mucocele with optic nerve compression

Mucoceles are epithelial-lined cavities filled with sterile mucus that develop due to the obstruction of a sinus ostium. Frontal mucocele is one of the most common among mucoceles. Most of them present with ophthalmic manifestations. Here, we present such a case with ocular symptoms. Despite the absence of thyroid-related symptoms or significant medical history, her examination revealed left eye proptosis, limited ocular motility, optic disc edema, and significant visual loss due to optic nerve compression. Further investigations, including orbital computed tomography, confirmed a frontal mucocele extending into the left orbit. Following endoscopic sinus surgery with mucocele excision, the patient experienced significant improvement in vision, resolution of optic disc edema, and proptosis. This case underscores the importance of considering mucoceles in the differential diagnosis of orbital pathologies. It also highlights the efficacy of endoscopic management in achieving favorable outcomes and emphasizes the significance of interdisciplinary collaboration in optimizing patient care.

Transvenous embolization of a carotid-cavernous fistula via the inferior ophthalmic vein: illustrative case.

A 49-year-old woman with a history of hypertension presented to the emergency department with right eye redness, proptosis, orbital fullness, and blurry vision. She had initially been diagnosed with an orbital pseudotumor, and the symptoms worsened over a course of steroids. Computed tomography angiography raised concern for a carotid-cavernous fistula (CCF), which was subsequently confirmed by digital subtraction angiography. She underwent fistula coil embolization via the internal maxillary artery and inferior ophthalmic vein (IOV). At the 2-month follow-up, she reported complete resolution of diplopia, orbital fullness, and proptosis. An ophthalmology examination revealed normal visual fields bilaterally. CCF embolization is rarely performed through the IOV, with only 5 reported cases in the literature. This case demonstrates that the procedure can be easily performed if the anatomy is favorable over the superior ophthalmic vein, with the illustration of good cosmetic outcomes. https://thejns.org/doi/10.3171/CASE24183.

Simplified medial canthoplasty: Technique and early postoperative complications in 601 dogs (1180 eyes).

To present a novel modified medial canthoplasty (MCP) technique, the application in 601 dogs (1180 eyes), and a review of the early postoperative complications related to the technique. Macroblepharon and nasal entropion is a common presentation in many brachycephalic dog breeds promoting chronic keratitis. The previously described MCP techniques limit the medial shortening of the eyelids to the location of the lacrimal puncta. A novel technique was developed allowing for a more extensive eyelid shortening nasally. The modification of the here described technique includes the longitudinal splitting of the lacrimal canaliculi allowing for an extensive shortening of the lids beyond the lacrimal puncta. Records of 601 dogs which underwent the modified MCP were reviewed regarding breed, indications for surgery, follow-up, and early postoperative surgical complications. Descriptive statistics were applied. Six hundred one dogs (1180 eyes) were included in this study. Twenty-four different breeds underwent the simplified MCP with the Pug (n = 403) being the most represented breed. Indications were macropalpebral fissure, medial entropion, pigmentary keratitis, trichiasis by haired caruncle, strabismus after globe prolapse, and facial nerve paralysis. The overall complication rate was 1.01% (12/1180) and consisted of wound dehiscence 0.59% (7/1180), corneal ulceration caused by suture material 0.25% (3/1180), and wound infection (2/1180). None of the 601 operated dogs developed serious complications related to the surgical technique. The simplified MCP is a novel technique with a very low complication rate.

Traumatic globe luxation- A case series

Traumatic globe luxation- A case series

Recurrent sinonasal chondrosarcoma with intracranial extension- A case report

Chondrosarcoma (CS) is the rare, slowly growing, malignant neoplasm. CS of the sino nasal cavity is extremely rare. It is usually present with common symptoms like nasal obstruction and epistaxis. It rarely present with ocular symptoms. Histopathological examination is useful for confirmation of diagnosis. Surgical excision is the treatment of choice. Radiotherapy is indicated if the tumor involves the vital structures. Here we are reporting a case of recurrent chondrosarcoma. Patient is presented to our department with proptosis of left eye and nasal obstruction since two months. Combined with neurosurgeon bi frontal craniotomy with endoscopic excision of mass done. Post operatively patient received adjuvant radiotherapy. Patient is on regular follow up. Keywords: chondrosarcoma; combined excision; radio therapy

Clinical characteristics and treatment outcomes of patients with IgG4-positive ocular adnexal marginal zone B-cell lymphoma.

To explore the clinicopathological characteristics of immunoglobulin G4 (IgG4)-positive ocular adnexal marginal zone B-cell lymphoma (OAML) and associated patient treatment outcomes. Medical records from patients diagnosed with IgG4-positive OAML treated at the West China Hospital between January 2016 and August 2023 were retrospectively analyzed. This study included data from 22 patients (11 males, 11 females), aged between 36 and 83 years, with disease durations from 1 month to 30 years. Sixteen cases exhibited unilateral ocular involvement (ten left eyes, six right eyes), while six exhibited bilateral involvement. Common clinical symptoms included ocular masses, eyelid swelling, and proptosis, with the orbit and lacrimal gland being the most commonly impacted sites. Among the 22 patients, 13 who were clinically suspected of having IgG4-related ophthalmic disease (IgG4-ROD) underwent serum IgG4 testing pre-operatively, revealing elevated IgG4 levels in 11 of these patients. The use of computed tomography and magnetic resonance imaging facilitated the evaluation of the location and size of lesions. All 22 patients received surgical treatment. Subsequently, 14 of these patients underwent local radiotherapy, five received post-operative chemotherapy, and three were closely observed. The follow-up period of patients in this study was 3-77 months, with an average follow-up time of 36 months. Except for one patient who died of disease progression, all others showed favorable prognoses with significant improvements. These results support the classification of IgG4-positive OAML as a distinct OAML sub-type with clinical features that partially overlap with IgG4-ROD. Therefore, accurate differentiation between OAML and IgG4-ROD is imperative, necessitating timely surgical intervention and precise pathological diagnosis to prevent diagnostic errors and inappropriate treatment. Currently, no standardized treatments for IgG4-positive OAML exist, but our results suggest that standard OAML therapies are generally efficacious.

To determine the role of transcutaneous retrobulbar amphotericin B in COVID-19-related rhino-orbital-cerebral mucormycosis.

The purpose of this study was to determine the role of transcutaneous retrobulbar amphotericin B (TRAMB) injection in COVID-19-related rhino-orbital-cerebral mucormycosis (ROCM). This prospective interventional case series included 7 eyes of 7 patients diagnosed with COVID-19 ROCM presenting from May to September 2021 at a tertiary care center in North India. Active or recovered cases of COVID-19 with clinical features suggestive of ROCM and early orbital involvement were included. Following functional endoscopic sinus surgery, patients were given TRAMB 3.5 mg/ml in single ocular quadrant depending upon the muscle involved on contrast-enhanced magnetic resonance imaging (MRI). Patients were followed up for a period of 12 weeks. All patients were diabetic and COVID-19 reverse transcription-polymerase chain reaction positive. The mean age of patients was 48.4 years ± 11 years, out of which 5 patients were male and 2 were female. The left eye was involved in 57.14% of cases whereas 42.85% of cases had right eye involvement. Presenting complaints were drooping of the upper lid (57.14%; n = 4), proptosis (28.57%; n = 2), facial pain and swelling (85.71%; n = 6), and nasal block (57.14%; n = 4). Stagewise distribution was 3a in four patients (57.14%), 3b in two patients (28.57%), and 4a in one patient. TRAMB injections (median - 3) were given, and improvement was seen in all patients in terms of ocular movements, ptosis, proptosis, and muscle enhancement on MRI scan. Three patients had an increase in intraocular pressure and inflammation which settled within a week with favorable anatomical and functional outcomes. Transcutaneous retrobulbar amphotericin B should be considered an adjunctive treatment modality for ROCM patients with limited orbital disease to preserve ocular function.

Langerhans Cell Histiocytosis in the Sphenoid Wing

Langerhans Cell Histiocytosis (LCH) is a rare and broad-spectrum of disease caused by uncontrolled proliferation of pathologic Langerhans cell resulting in localize and systemic organ infiltration. The clinical presentation can be unspecific and varies between individuals depending on the anatomical location and organ involvement. In view of bony infiltration is the most common in LCH, clinicians have to deal with the challenge of diagnosis dilemmas such as Giant Cell Tumour, Ewing’s sarcoma, osteogenic sarcoma, plasmacytoma, osteomyelitis or bony metastasis, particularly from neuroblastoma. Hence, it requires an excellent multidisciplinary team to establish the diagnosis and start targeted treatment early to prevent fatal complications. The treatment modalities include surgery, immunosuppressive therapy, chemotherapy or in combination. Recently, a seven months old boy presented to our centre with one-week duration of left cheek swelling and left eye proptosis. After detailed clinical evaluation together with imaging and biopsy, the lesion over the bilateral sphenoid and left zygoma were concluded to be Langerhans Cell Histiocytosis (LCH).

Intraorbital epidermoid cyst mimicking cysticercosis: A case report

A 37-year-old female presented with right eye proptosis, eyelid swelling, and pain lasting for 7 days upon admission. There was no pertinent personal or family medical history or prior incidence of cysticercus infestation. The computed tomography (CT) images revealed an oval soft tissue density mass in the right orbit, containing a low-density shadow within. Magnetic resonance imaging (MRI) revealed an oval-shaped mass, exhibiting hypointense on T1-weighted images (T1WI) and hyperintense on T2-weighted images (T2WI). In addition, a circular signal that was hyperintense on T1WI and hypointense on T2WI was observed within the mass. B-mode ultrasonography showed an oval mass within the hypoechoic lesion, which changed with position. All imaging features mimicked those of cysticercosis. The lesion was excised, and the pathologic examination showed an epidermoid cyst. When encountering a round mass within a fluid lesion that exhibits positional changes, it is essential to consider not only cysticercosis, but also an epidermoid cyst.

Emergency Decompression of Orbital Emphysema, a Sight-Saving Tool in a Patient With Precious Eye.

A woman, 19 years old, with a history of falling from a height with resulting zygomatico-maxillar complex fracture on the right side, a mandibular fracture on the left side for which she underwent repair with plate insertion, and traumatic optic atrophy in her right eye, presented 9 months later with eye facial swelling, proptosis, and acute rapid loss of vision in the left eye. The diagnosis was done immediately aided by radiology assistance and a decision was taken to admit the patient to undergo urgent decompression to save the vision and the patient did recover well. This case presented here and the associated literature review focus on severe orbital emphysema with compressive optic neuropathy and orbital compartment syndrome as a morbidity that can exist with delayed presentation after trauma and not elicited by sneezing or forced blowing, as well as the drastic importance of brisk intervention, to save vision and prevent visual complications if left untreated.

Clinical assessment of corneal alterations in endocrine disorders

Corneal alterations are frequent in many endocrine disorders like Grave’s ophthalmopathy and diabetes mellitus. Since early diagnosis of such a change can prevent visual impairment, it presents a challenge to an ophthalmologist.Clinical assessment & significance of corneal alterations in endocrine disorders.1. To conduct comprehensive assessment of corneal alterations in endocrine disorders. 2. To Identify specific corneal abnormalities in patients with endocrine disorders. 3. To delineate the clinical significance of corneal alterations in endocrine disorders by determining their impact on visual function and guiding therapeutic strategies.This study was a prospective, observational, hospital-based study conducted over a period of two years including 150 patients of different endocrine disorders. Ocular examination included physical examination to check for any pain on ocular movements, ophthalmoplegia, diplopia, or proptosis. Visual acuity was examined by Snellen’s chart, anterior segment by slit-lamp, intra-ocular pressure by non-contact tonometer, qualitative measurement of corneal sensation by a cotton-wisp and central corneal thickness was measured by pachymetry. Corneal endothelial cell parameters like endothelial cell density (ECD), coefficient of variation of cell area (CV), percentage of hexagonal cell (HEX) were measured by clinical specular microscope.The age range was 14 to 82 years with mean age being 48.43±14.2 years. The male to female ratio was 1.34:1. Type-2 diabetes mellitus was the most frequently encountered endocrine disorder (64.7%), followed by hypothyroidism (13.3%). Addison’s disease was the least encountered (0.7%). The most severe changes to the cornea, such as chronic epithelial abnormalities, superficial punctate keratopathy, altered corneal sensations, and dry eyes, were observed in patients with type 2 diabetes, followed by Graves’s ophthalmopathy and hypothyroidism. Patients with T2DM and T1DM had elevated CCT values, reduced endothelial cell density, abnormally high coefficient of variance and low hexagonal cells.The present study showed that different corneal alterations can be possible in endocrine disorders. So all endocrine disorders should undergo comprehensive ophthalmological examination to prevent visual impairment.

Microphthalmia with a Large Orbital Cyst: A Case Report

Purpose: We present the case of a 4-month-old boy with a large orbital cyst that caused lateral displacement of the optic nerve resulting in progressive exophthalmos of the left eye since birth.Case summary: A 4-month-old boy presented with progressive exophthalmos of the left eye since birth. Physical examination revealed marked left eye proptosis. Magnetic resonance imaging revealed microphthalmia and a 2.2 × 1.9 cm orbital cyst in the retrobulbar region. Subsequently, an anterior orbitotomy of the left eye was performed to dissect and excise the cyst. Histopathological examination revealed an orbital cyst containing neuroectodermal tissue. No complications or recurrences were observed during the 3-month post-surgery period.Conclusions: Congenital microphthalmia with orbital cysts is a rare condition that does not always require surgical intervention. However, timely surgical intervention in cases of severe exophthalmos can result in favorable functional and cosmetic outcomes. We present a case that exhibited significant improvement following surgical removal of the orbital cyst.

Exploring the Intricacies of Carotid Cavernous Fistula: A Case Series

Introduction : Carotid cavernous fistula (CCF) is caused by an abnormal connection between the carotid artery and the cavernous sinus, which can lead to eye-related problems. It is crucial to recognize its signs, as they can resemble other conditions.
 Case Illustration : First patient is a male 3-month-old baby, with unilateral right eye proptosis that was observed soon after birth and history of hypothyroid. Proptosis is more prominent while the baby is crying. Orbital ultrasonography revealed ophthalmica vein dilatation with S configuration, complex flow, and velocity of 10 cm/s. Second case is a 50-year-old male also with unilateral proptosis and orbital bruit with history of head trauma 2 months prior to admission. Patient came with complain of sudden blurry vision for 1 week. The results of the magnetic resonance venogram revealed tortuos dilated superior ophthalmica (SOV), cavernous sinus bulging, and dilated superior petrosal, sigmoid, transverse sinus, and internal jugular vein.
 Discussion : CCFs can occur at any age, but are more common between 40-60. Risk factors like head trauma, hypertension, connective tissue disorders increase the likelihood of developing CCF. They are often misdiagnosed as other conditions such as thyroid ophthalmopathy or conjunctivitis. Ocular doppler ultrasound can detect SOV dilation and arterial flow, while magnetic resonance imaging can show superior ophthalmic vein engorgement, muscle hypertrophy, and cavernous sinus dilatation. Treatment for symptomatic CCFs involves endovascular obliteration using balloons, embolic agents, or metallic coils, while asymptomatic and mildly symptomatic patients can be treated conservatively.
 Conclusion : Awareness of CCF symptoms, varied workup, prompt treatment is important to prevent severe complications.

Proptosis in Adult Onset of Unilateral High Myopia Patient : A Case Report

Introduction : Unilateral high myopia is an uncommon refractive condition, because two eyes typically grow bilaterally by the same amount and resulting in symmetric myopia. Proptosis can appeared due to high axial length in myopia.
 Case Illustration : A 41-year-old female patient complain protruding eyeball in her left eye, gradually decrease vision, eye discomfort, and headache in her left eye since last year. There was no family history of eye disease, trauma or mass. On examination, her left eye was proptotic 3mm, best corrected visual acquity (BCVA) was 6/21 with -10.00 Sphere, and high intraocular pressure 23 mmhg. Her right eye BCVA 6/6 with -2.75 Sphere, other within normal limits. Her fundus examination showed features of high myopia and B-scan ultrasonographic showed different in axial lengths in both eyes. Orbita computerized tomography non contrast confirmed enlarge globe to posterior and proptotic in left eye.
 Discussion : Unilateral high myopia developing in adult onset is a rare case. It appears to be a progressive condition that have a variable age of onset. Amblyopic eye, high intraocular pressure and proptosis demonstrated as the symptoms of unilateral high myopia in this patient. Left eye proptosis confirmed by CT orbita appears secondary due to elongation anteroposterior of the globe / changesin axial components of the eye.
 Conclusion : High myopia can be a consideration of proptotic eye beside of mass/ tumor. Several studies to investigate patients with unilateral high myopia should be done to know the etiology and progression of the disease, thus can achieve good treatment and visual outcomes for patient.