Unilateral Proptosis Research Articles

Isolated hydatid cyst in the medial rectus muscle: unveiling a rare orbital occurrence.

Orbital hydatid disease, while rare, should be included in the differential diagnosis of unilateral proptosis, particularly in endemic areas. Accurate diagnosis and comprehensive management are essential for effective treatment and favorable long-term outcomes. A 12-year-old boy presented with a one-month history of diplopia and left-sided proptosis. A CT scan revealed a cystic mass within the left medial rectus muscle. Surgical intervention confirmed the presence of a hydatid cyst following cyst rupture and irrigation with hypertonic saline. The patient underwent a two-month course of albendazole therapy. Initially, the patient experienced persistent exotropia and diplopia, but at the three-year follow-up, he exhibited no diplopia or proptosis and only mild residual exotropia. This case underscores the importance of considering orbital hydatid cysts in the differential diagnosis of unilateral proptosis in endemic regions. Heightened awareness, accurate diagnosis, and a tailored therapeutic approach, including surgical removal and antiparasitic treatment, are crucial for successful management and improved long-term outcomes.

Sinus mucocele leads to unilateral proptosis in an infant patient with cystic fibrosis; a literature review and a case report study

Introduction and Importance: Cystic fibrosis (CF) is a widespread life-shortening recessive genetic disease and can present with sinus mucocele. Sinus mucocele is a rare condition, with limited prevalence data on unilateral proptosis. Case presentation: We present a case of a 19-month-old boy with CF who experienced worsening proptosis and exotropia in his right eye. A brain and orbit MRI revealed diffuse polypoid mucosal thickening, possible dense fungal deposit, deformity of the mid face, especially on the right side, with more prominent bulging of medical and inferior walls of the right lobe, a right ethmoidal mucocele causing ocular globe displacement, medial rectus compression, and optic nerve. An examination of the eye fundus showed disc edema and vascular congestion. Endoscopic sinus surgery successfully drained the mucocele, and treatment with antibiotics and corticosteroids led to symptom improvement and resolution of proptosis within three weeks. Clinical Discussion: Mucoceles represent an uncommon complication associated with CF in pediatric patients. Consequently, any child presenting with this issue should undergo evaluation for CF. Investigating this infrequent condition’s underlying mechanisms and consequences may improve treatment approaches and outcomes for those impacted. Conclusion: Sinus mucocele with unilateral proptosis in CF patients is uncommon, and endoscopic sinus surgery appears to be an effective cure for this complication, even in the pediatric population at high risk, like CF patients.

Unilateral Proptosis as the Initial Manifestation of Acute Myeloid Leukemia in a Young Adult

Unilateral Proptosis as the Initial Manifestation of Acute Myeloid Leukemia in a Young Adult

Failure to Thrive With Unilateral Proptosis and Xanthocoria in a Young Boy.

Failure to Thrive With Unilateral Proptosis and Xanthocoria in a Young Boy.

Progressive Unilateral Proptosis and [18F]FDG-Avid Lesions Connoting Aggressive Disease in Radioiodine-Refractory Differentiated Thyroid Carcinoma

AbstractRadioiodine-refractory differentiated thyroid carcinoma (RAIR-DTC) have poor prognosis as compared with radioiodine concentrating thyroid carcinoma. We present a case of follicular thyroid carcinoma presenting as a disseminated disease initially, underwent thyroid surgery and radioiodine (RAI) therapy. Following RAI, the patient developed a sudden-onset unilateral proptosis, finally diagnosed as orbital metastasis. [18F]-Fluorodeoxyglucose (FDG)-positron emission tomography-computed tomography identified FDG-avid metastatic lesions more numerous than the RAI-avid lesions. The patient was treated with local and systemic targeted therapy, but despite these treatments, he developed progressive disease indicating aggressive nature of the disease consistent with [18F]FDG-avid RAIR-DTC.

Two cases of unilateral pediatric proptosis: A diagnostic challenge

Abstract Pediatric proptosis often presents with confusing clinical features requiring a systematic approach to avoid diagnostic dilemmas. We present two children of retinoblastoma and congenital uveal malignant melanoma, with late presentation. Through this article, we highlight the importance of systematic clinical examination, appropriate investigations for executing timely treatment, clinical correlation, and the value of histopathologic examination.

Neglected Spheno-orbital Meningioma Presenting with Unilateral Blindness

Sphenoorbital meningiomas (SOM) constitutes 9% of all intracranial meningiomas and are originated from sphenoid wing dura. They are also called en plaque meningioma. They cause hyperostosis of involved bone and their soft tissue growth may spread the orbit, the infratemporal fossa, and the temporal fossa. Most patients with SOM are middle- aged women and most commonly presents with unilateral, nonpulsating, progressive proptosis. Recently a woman with unilateral progressive proptosis from a remote area of Bangladesh presented to us with blindness of her left eye due to diagnostic delay. After thorough evaluation and imaging studies, she was diagnosed with a large spheno-orbital meningioma with extensive bony involvement of skull base and orbit. Objective: This report highlights the necessity of creating awareness among optometrists, undergraduate medical students as well as and ophthalmologists who are practicing in remote areas of Bangladesh about clinical presentation of sphenoorbital meningiomas which is a benign lesion but may be a cause of unilateral blindness if remain undiagnosed for long Bang. J Neurosurgery 2023; 13(1): 51-54

Vertebro-Vertebral Arteriovenous Fistulae: A Case Series of Endovascular Management at a Single Center.

Vertebro-vertebral arteriovenous fistulae (VVFs) are a rare disorder characterized by a direct shunt between the extracranial vertebral artery and the veins of the vertebral venous plexus. This study aims to comprehensively review the characteristics and outcomes of endovascular treatments for VVFs at our center. A retrospective review was conducted on 14 patients diagnosed with a VVF who underwent endovascular treatment at Siriraj Hospital from January 2000 to January 2023. The study assessed patient demographics, presentation, fistula location, treatment strategies, endovascular techniques employed, and treatment outcomes. Among the 14 patients, 11 (78.6%) were female, with an age range from 25 to 79 years (median: 50 years). Spontaneous VVFs were observed in 64.3% of the cases, including three associated with neurofibromatosis type 1 (NF-1). Iatrogenic injury accounted for two cases, and three patients had VVFs resulting from traffic accidents. A pulsatile neck mass and tinnitus, with or without neurological deficits, were common presenting symptoms. Active bleeding was observed in three cases with vascular injury, while unilateral proptosis, congestive heart failure, and incidental findings each presented in one patient. All the VVFs were successfully obliterated without major treatment complications. Parent vessel sacrifice was performed in 85.7% of the cases, while vertebral artery preservation was achieved in the remaining two patients. Embolic materials included detachable balloons, detachable coils, and n-butyl cyanoacrylate (NBCA) glue. All the presenting symptoms showed improvement, and no morbidity or mortality was observed. Endovascular embolization is a feasible and effective approach for achieving complete VVF obliteration with safety. Parent artery sacrifice should not be reluctantly performed, particularly when adequate collateral circulation is demonstrated.

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.

Clinical Feature of Langerhans Cell Histiocytosis of the Orbit : A First Case Series in Indonesia

Introduction : Langer hans histiocytosis (LCH) is an inexplicable disease characterized by proliferation of Langerhans and intermixed with inflammatory cell, in particular eosinophil, which represents only 1% pediatric orbital tumours. Orbital involvement of LCH is a rare clinical entity as the disease simulate variety of malignancies and present as a diagnostic and management dilemma for ophthalmologist.
 Case Illustration : Two cases of toddler presented with unilateral progressive upper eyelid swelling and proptosis for more than a month. Both cases has history of orbital trauma 1 month before swelling was observed. Initial CT scan revealed extraconal mass, with destruction of lateral orbital wall and frontal bone. Both patients had histopathological biopsy confirmed eosinophlic cytoplasm. One patient confirmed immunohistochemistry markers S100 and CD1a which is specific for Langerhans cells. Both patients continue with chemotherapy as definitive treatment.
 Discussion : LCH is a wide clinical spectrum which manifestation simulate other pediatric orbital malignancies. Case illustration present unilateral eyelid swelling with history of trauma may resemble other diagnosis unless further work up is performed. This case series exhibits clinical manifestations that may be suggestive of LCH. Considering rapid progression of disease, definitive diagnosis is a mandatory based on histopatological analysis through biopsy surgery.
 Conclusion : LCH is rare disease, it should be considered as one of deferential diagnosis in pediatric orbital malignancy with initial presentation acute unilateral eyelid swelling, proptosis, history of trauma, extraconal mass and destruction of bones as radiological features. This might be helpful for early recognition and prompt treatment of disease suggestive of LCH.

Acute unilateral childhood proptosis as a presenting symptom of sickle cell disease

Although rare, orbital bone marrow infarction is a serious complication in patients with sickle cell disease (SCD). Ophthalmologists should have a high index of suspicion for this disease in cases of acute childhood proptosis. Appropriate early management will prevent optic nerve dysfunction and permanent vision loss. Here, we present a case where the patient presented to the ophthalmologist with complaints of pain and proptosis, which led to the diagnosis of SCD.

Not Every Anosmia is COVID, a Case of Nasal Mass

Smell and test problems especially anosmia is a frequent symptom for COVID infection. Anosmia was experienced by almost 53% of those affected, in some cases persisting for longer periods after resolution of respiratory symptoms. Nasal blockage is another common symptom for emergency department patients and frequently associated with allergic rhinitis. A 25-year-old male patient presented with persistent bilateral nasal blockage, blood-stained nasal discharge, anosmia, and headache since last 1 year. During the peak of the pandemic, he was diagnosed with COVID. He was complaining of anosmia and nasal blockage and was treated with antihistaminic medicine for allergic rhinitis, but his symptoms persisted. He had breathing, bleeding and smelling problems in the last 2 years and started complaining of left sided proptosis for 3-4 weeks before being admitted to our department and had computerized tomography (CT) imaging at another facility. He was referred by an ophthalmologist to our hospital for advanced imaging with magnetic resonance (MRI) with contrast. He was investigated for exophthalmos in his left eye. He denied any other prior disease or medication. On examination he has nasal voice, left side exophthalmos, no diplopia or vision problem. The right tympanic membrane (TM) was intact, left sided dull TM with effusion. Other system evaluations were unremarkable. The patient was referred to Ear Nose and Throat consultant for further examination. Nasal endoscopy revealed fungating mass with covered blood clots and engorged vessels filling both the nasal cavities. MRI report described soft tissue mass, infiltrating the nasal cavity, paranasal sinuses and clivus with intracranial extension, to involve the left sub frontal region. Also noted associated destruction of the left lamina papyracea, showing orbital extension, compressing the orbital muscle cone, and compressing the medial rectus, with consequent left sided unilateral proptosis. In addition, there was nasopharyngeal extension. The epicenter of the mass was likely to be the ethmoid sinuses. His MRI revealed complete obstruction of the osteomeatal complexes, complete obliteration of the maxillary, ethmoids and frontal sinuses, due to retention of secretions and mass infiltration. Biopsy was taken and patient was followed up for surgical intervention. The differential diagnosis included vascular tumor, meningothelial tumor, undifferentiated carcinoma, and neuroendocrine carcinoma. Smell and taste disorders emerge during COVID infection, seem to be more common than other upper respiratory tract infections. Allergic rhinitis is also common amongst emergency department patients and frequently associated with nasal blockage. Rhinitis can be differentiated from sinus problems by the increased response of nasal obstruction to treatment, clear nasal discharge, and absence of pain or fever. Rhinitis does not lead to ostial obstruction, and patients do not complain of facial pain or headache. For suspected rhinitis or acute sinusitis, routine radiographic examination is not recommended and should be limited to the diagnosis of chronic or recurrent admissions, cases of questionable diagnoses, patients with unresponsive disease, or investigation of complications. Axial and coronal CT is the imaging modality of choice. In this case the patient was investigated for sinusitis complications and additional further imaging was necessary like MRI with contrast for proper diagnosis and management of the patient. Nasal blockage and upper respiratory system findings may be overseen by the patients or even the healthcare workers. Especially repeated admissions and persistence of these symptoms should be suspicious for the emergency physicians. Additional imaging and thorough examination are crucial for differential diagnosis and proper management of these patients. Emergency physicians should be careful on their diagnoses for common symptoms like nasal blockage especially for the patient

Presentation pattern and survival rate of retinoblastoma following chemotherapy: a prospective study

BackgroundThis study presents the clinical pattern of presentation and survival rate of retinoblastoma, which is the most prevalent form of pediatric intraocular cancer. The aim of this study is to provide baseline information about the clinical presentation and management of retinoblastoma at ECWA Eye Hospital. Additionally, the study identifies priority areas for enhancing medical care for children diagnosed with this cancer. ECWA Eye Hospital, situated in Kano State, Nigeria, is a specialized eye center located in the North-Western region of the country.MethodsA prospective study spanning five years was conducted at ECWA Eye Hospital to investigate clinically diagnosed cases of retinoblastoma. The study took place from January 2018 to December 2022. The patients received standardized pre-medication and chemotherapy protocols for retinoblastoma. Subsequently, a five-year follow-up was conducted to monitor the patients’ progress. The collected data was analyzed, descriptive statistics were generated, and the survival rate was calculated.ResultsDuring the five-year study period, a total of 35 cases of retinoblastoma were diagnosed. The patients had an average age of 3.21 ± 1.32 years. The most common presentation patterns observed were fungating ocular mass and proptosis. Among the cases, there were 10 instances of bilateral proptosis and 25 instances of unilateral proptosis. While no patients exhibited bilateral leukocoria, eight cases of unilateral leukocoria with anterior segment seedlings were identified. The additional patterns of presentation are proptosis, leukocoria, fungating orbital mass, redness and loss of vision. The mortality rate was 80% (28 cases), while the survival rate was 20% (7 cases). Notably, all the survivors had unilateral retinoblastoma.ConclusionThe majority of cases observed at ECWA Eye Hospital involve advanced retinoblastoma. In low-resource settings where alternative treatment options are limited, chemotherapy is considered a viable treatment option. Early presentation of retinoblastoma in patients may lead to a higher survival rate when chemotherapy is administered.

Primary plasma cell leukaemia presenting as unilateral proptosis.

Primary plasma cell leukaemia presenting as unilateral proptosis.

Orbital Liposarcoma Progression From a Well-Differentiated to a Dedifferentiated Type in a Young Patient.

A young male patient presented with unilateral proptosis of the left eye. Laboratory tests and an initial computed tomography scan of the orbit were compatible with euthyroid orbitopathy. He subsequently underwent decompression surgery for unfavorable cosmesis and corneal exposure symptoms with good results, but returned 1 year later for recurrence of proptosis. There was a superomedial fatty density extraconal mass on computed tomography scan. A biopsy done a year later confirmed the diagnosis of well-differentiated liposarcoma from the previously noted mass and a dedifferentiated component in the newly appeared mass in the intraconal space. Orbital exenteration with radial forearm free flap was performed followed by orbital radiotherapy. No disease recurrence or metastasis was seen on follow-ups for 7 years since undergoing treatment.

Granular cell tumor: A rare presentation as intraconal orbital space occupying lesion

Granular cell tumor (GCT) is a rare benign soft tissue tumor. They have been reported in several regions of the body but mostly in the skin and subcutaneous tissue of head and neck. It may involve the orbit, periocular skin, lacrimal sac, optic nerve, ciliary body, conjunctiva and caruncle. Here we present a rare case of intraconal GCT in a 45 year old woman who presented with unilateral proptosis. She presented with forward protrusion of right eye for the past 3 months,MRI orbit revealed a well defined T1/T2/FLAIR hypointense lesion in the inferolateral aspect of intraconal compartment of right orbit. Transconjunctival excision of the lesion was done. Histopathological Examination (HPE) and immunohistochemistry confirmed the diagnosis of Granular cell Tumor.

Oral and Maxillofacial Radiology

Clinically (Figure 1) a unilateral swelling, proptosis and obliteration of the nasolabial fold was noted. Intraoral examination revealed normal-appearing overlying mucosa. A pantomograph (Figure 2) demonstrates a mixed diffuse expansile lesion and thinning of the cortices affecting both jaws. 3D reconstruction (Figure 3) overview the lesions' extent. CBCT interpretation (Figures 4 and 5) indicated engrossment of the frontal, parietal, temporal, sphenoid, ethmoid, maxillary, palatine, zygomatic, and mastoid bones. T1-weighted gadolinium-enhanced MRI image (Figure 6) of a patient with a similar lesion in the right maxilla demonstrates a heterogeneous appearance.

Protean Neuroophthalmic Presentations of Common Childhood Malignancies—A Report of Two Cases

AbstractCommon pediatric malignancies often surprise clinicians with unusual presentations. In this narrative, we report two patients with common childhood cancer having unique neuroophthalmic characteristics. In the first case, we have a child with a common childhood solid tumor presenting with blindness without proptosis, while the second case is of a child with a common hematological malignancy presenting with unilateral proptosis without visual impairment. The report highlights that common childhood cancers may present with neuroophthalmic symptoms on rare occasions, creating a diagnostic conundrum.

Abstract #1400968: A Case of Co-Existing Alemtuzumab-Induced Thyroid Disorders

Alemtuzumab (ALZ) is an anti-CD52 monoclonal antibody effective in treating relapsing-remitting Multiple Sclerosis (MS). ALZ is hypothesized to augment immune function due to mature lymphocyte depletion; it may also predispose patients to secondary autoimmune disorders during the immune system recovery phase, with thyroid dysfunction occurring in up to 33%. We a case of a woman with MS who developed Hashimoto’s thyroiditis followed by Graves’ disease after ALZ use. A 43-year-old woman with MS was referred for management of hyperthyroidism after experiencing 1 year of palpitations, insomnia, and tremors. She completed two doses of ALZ nineteen months apart. Nine months after her 2nd ALZ dose, she developed hypothyroidism (TSH 38 mIU/L, TPO 856 IU/L, and low FT4 0.2ng/dL); she was treated to euthyroidism with levothyroxine. Sixteen months after her 2nd dose, she developed hyperthyroidism, persisting after levothyroxine cessation: TSH 0.02 mIU/L, FT4 1.9ng/dL, FT3 7.4pg/mL, TRAb 9.6, TSI 385, and TPO 83 IU/L. Her Graves’ disease course was complicated by the development of mild unilateral proptosis and diplopia. Past medical history included neurogenic bladder and anxiety, treated with mirabegron and alprazolam. Family history was notable for thyroidectomy in her mother for unknown indication. Physical exam showed stable vitals, mild active thyroid eye disease, goiter, and fine bilateral hand tremor. She was initially treated with methimazole, but unfortunately developed significant hepatotoxicity. She was recommended for total thyroidectomy, which she underwent without complication following preparation with potassium iodide. This case highlights an interesting presentation of co-existing autoimmune thyroid disorders in an ALZ-treated patient. ALZ has a rapid and long-lasting effect on the immune system, leading to qualitative and quantitative lymphocyte changes. ALZ induced Graves’ disease (AIGD) is the most common thyroid disorder, followed by Hashimoto’s thyroiditis & silent thyroiditis at 63%, 15%, & 9%, respectively. Most of those with AIGD had overt symptoms, and 10% reported ophthalmopathy. They peak around 28 months after the last dose, concurring with the B cell slow recovery phase. Over half of patients with AIGD required anti-thyroid medications (56%), 22% underwent radioactive iodine, and 11% underwent thyroidectomy. Interestingly, MS patients who developed AIGD had better overall MS disease prognosis. The frequency of ALZ-associated thyroid disease underscores the importance of routine thyroid function monitoring every 3 months following ALZ, and a provision of thyroid education materials for earlier identification of thyroid disorders.

Unilateral Orbitopathy Caused by Skull Base Chordoid Meningioma

Chordoid meningioma (CM) makes up only 1% of all meningiomas. Most cases of this variant are locally aggressive, have high growth potential, and are likely to recur. Although CMs are known to be invasive, they rarely extend into the retro-orbital space. Herein, we report a case of a central skull base CM in a 78-year-old woman whose only manifestation was unilateral proptosis with impaired vision resulting from the tumor extending into the retro-orbital space through the superior orbital fissure. The diagnosis was confirmed by analysis of specimens collected during endoscopic orbital surgery, which simultaneously relieved the protruding eye and restored the patient's visual acuity by decompressing the oppressed orbit. This rare presentation of CM reminds physicians there may be lesions outside the orbit that can cause unilateral orbitopathy and that endoscopic orbital surgery can be used to confirm its diagnosis as well as treat it.