Left Orbit Research Articles

Frontal nerve schwannoma in a 16-year-old girl presenting with a rapid growth for 2 years

Background: Orbital schwannomas arising from the frontal nerve are a rare but distinct entity. Case Description: A 16-year-old girl presented to our hospital with gradually progressive proptosis. Computed tomography (CT) performed 2 years ago incidentally detected a retrobulbar mass in the left orbit measuring 13 mm × 14 mm × 10 mm, which was not identified on CT performed at the age of 4 years. CT taken at presentation revealed marked tumor growth over 2 years, measuring 24 mm × 20 mm × 17 mm. On magnetic resonance imaging (MRI), the tumor appeared homogeneously isointense both on the T1- and T2-weighted sequences. The patient underwent transcranial tumor resection. Following unilateral frontal craniotomy, an osteotomy was made to the left anterior fossa floor. On reflecting, the periorbita revealed the frontal nerve with the proximal segment intermingled with the tumor. The levator and superior rectus muscles were flattened and located immediately beneath the tumor. A gross total resection was performed. Microscopic findings of the resected tumor were consistent with schwannoma. Conclusion: Frontal nerve schwannomas may grow rapidly under certain conditions. Periodic follow-up using MRI and timely resection is recommended when an asymptomatic orbital tumor, possibly a frontal nerve schwannoma, is detected in young patients.

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.

Choroidal Infiltration in Erdheim-Chester Disease: A Case Series

Purpose: We describe two cases of choroidal involvement in patients with Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis. Methods: Short case series. Results: The first patient was a 25-year-old man with asymptomatic ECD at the level of bones, lung, and central nervous system. Fundus examination revealed bilateral yellowish choroidal infiltration in the superior macula and nasal mid-periphery. After initial mild enlargement of the choroidal lesions, treatment with weekly subcutaneous pegylated interferon α2a (PEG-IFN) was started inducing partial regression of the lesions. The second patient was a 45-year-old man with ECD involving the left orbit, maxillary sinus, heart, blood vessels, kidney, retroperitoneum, bones and testes. Fundus examination of the left eye showed choroidal folds. Macular spectral-domain optical coherence tomography confirmed the folds and revealed underlying choroidal infiltration. Final ocular diagnosis was left choroidal, scleral, and orbital involvement. The patient was initially treated with PEG-IFN, and later with cobimetinib, a MEK inhibitor, with favorable results. Neither patient developed choroidal neovascularization (CNV) during the follow-up. Conclusions: ECD is a rare multisystem disorder that can present with ocular involvement, including orbital masses, xanthelasma-like lesions, and occasionally, choroidal infiltrates. Early recognition and prompt treatment can lead to improvement of ophthalmic and systemic symptoms and prevent further complications. We recommend treating patients with choroidal ECD, regardless of the presence of symptoms, considering these patients are at increased risk of developing CNV.

A case report of orbital Castleman’s disease

Castleman's disease is a group of atypical lymphoproliferative disorders. Extranodal involvement is rare. We report a case of a 39-year male who had left painless proptosis for three months. Examination revealed a firm mass in the left inferior orbit, displacing the globe superiorly. MRI showed an irregular mass causing enlargement of the left recti muscles. Systemic workup was unremarkable. The mass was excised via inferior orbitotomy. Histopathology showed hyaline vascular variant of Castleman's disease. Postoperatively, proptosis resolved and no recurrence was noted. This highlights the importance of including CD while evaluating irregular orbital lesions for early detection and management.

Intra-orbital Foreign Body

Abstract Background: This case report describes a young man with an intra-orbital foreign body (FB) in both eyes. Case Report: Intra-orbital FBs are rare, with few cases reported in the literature. This is because the orbit protects the globe from external forces, and the orbit is a closed cavity with one opening. This report details the case of a 28-year-old man who presented to the emergency unit with an 11-hour history of an intra-orbital FB. The patient was lying on a bench when a stationary motorcycle fell on him. He noticed that he was unable to open both eyes and was rushed to a peripheral hospital, then subsequently referred to this tertiary center. On examination, he was ill-looking and anxious but not febrile or pale. Systemic examination did not reveal any abnormality. Unaided visual acuity in the right eye (OD) was nil perception of light, while left eye visual acuity (OS) was counting fingers. The right eyelid was ptotic due to edema. He underwent basic laboratory investigations that were all normal. A plain X-ray revealed a radio-opaque FB penetrating the left orbit, fracturing the medial walls into the right orbit, with the round end positioned behind the right globe. The FB was identified as a broken part of a brake/clutch lever from the motorcycle. A computerized tomography scan showed a metallic object in both orbits, accompanied by pneumocephalus, and suggested transection of the right optic nerve. The patient was prepared for FB removal and repair of the lid penetration under general anesthesia, following review by the ENT surgeon and neurosurgeon. The brake lever was successfully removed, and the patient was administered antibiotics. Post-operatively, visual acuity in the right eye remained at nil perception of light, while that in the left eye improved to 6/9 upon discharge. Conclusion: There is no established protocol for managing intra-orbital FBs, as removal depends on factors such as the size, composition, and potential compression damage to structures around the globe, which could further compromise vision or function. Small inert objects may be left in place if removal would cause damage. However, in this case, removal was deemed necessary for several reasons.

Laser Scanning Morphometric Measurements of the Main Orbital Communications in Dry Human Skulls

Background and Objectives: This research investigated the morphometric dimensions of the optic foramen (OF), superior orbital fissure (SOF) and inferior orbital fissure (IOF), using indirect measurement techniques such as laser scanning, making it likely the first study of its kind. This study aimed to identify the morphometric variability of the main orbit communications and to highlight the differences between genders. Materials and Methods: The anthropometric study was conducted on sixty dry skulls (120 orbits) of adults aged between 20 and 70 years. Measurements of orbital communications were made using the RS6 laser scanner. The orbital parameters that were investigated are as follows: length and width of the SOF and IOF, and height and width of the OF. Results: In males, the average height of the OF was 8.27 mm and 8.13 mm in females, while the average width of the OF was 6.34 mm in males and 5.83 mm in females. The SOF average length was 21.09 mm in males and 17.58 mm in females. The widths of the SOF in the three thirds (anterior, middle and posterior) in males were 5.14/4.77/7.11 mm and 2.28/3.48/5.80 mm in females. The average length of the IOF was 33.05 mm in males and 32.30 mm in females. The widths of the IOF in the three thirds (anterior, middle and posterior) were 5.61/3.92/4.70 mm in males and 7.24/4.68/4.08 mm in females. Conclusions: The OF height and width were higher in males compared to females. The SOF length and width were higher in males compared to females. The IOF length was higher in males for the right orbit and higher in females for the left orbit. The IOF width for both orbits was higher in females in the anterior and middle third, and higher for males in the posterior third. Evaluation of dry skulls using laser scanning is reliable and recommended for data accuracy. Laser scanning can become a usable method for all indented and hard-to-reach regions of the cranial skeleton.

A novel technique for skull base reconstruction in craniofacial fibrous dysplasia surgery using three-dimensional printing and a dental alginate mold: illustrative case.

Fibrous dysplasia is a rare and benign skeletal lesion characterized by fibrous tissue proliferation due to an abnormal osteogenesis replacing normal bone. An 18-year-old male with fibrous dysplasia of the left sphenoid, ethmoid, orbit, and frontal bones was managed with excision and skull base reconstruction. After complete removal of the tumor, skull base reconstruction was commenced by making a reverse temporalis flap and placing it over the opened paranasal sinuses for a robust vascularized graft, followed by an abdominal fat graft, and then a pedicled pericranal flap was added to complete the multilayer onlay graft. To recreate the skull base, a mirror image of the contralateral skull base was constructed using three-dimensional (3D) printing, and the 3D-printed model was sterilized prior to the surgery. Intraoperatively, the model was then pressed onto dental alginate gel to make a negative mold. This was used to make the definitive flap using polymethylmethacrylate. Temporoplasty was also performed using polymethylmethacrylate to fill the defect left by the temporalis graft. The patient recovered well following the procedure. Appropriate, personalized skull base reconstruction techniques can be successfully done with 3D printing using alternative low-cost materials and implements, especially following resection of cases like craniofacial fibrous dysplasia. https://thejns.org/doi/10.3171/CASE24262.

Initial Frontal Sinus and Orbit Tumor Signaling Late Distant Metastasis in Kidney Cancer.

Although metastases to the paranasal sinuses are exceedingly rare, when they do occur, renal clear cell carcinoma is the most frequent primary tumor involved. The aim of this work was to present a case of metastatic clear cell renal carcinoma in the frontal sinus and orbit region, 7 years after the initial diagnosis and treatment. The patient was referred to our clinic due to drooping of the left eyelid and displacement of the left eyeball. Computed tomography revealed an expansive lesion in the left frontal sinus and orbit, causing destruction of its anterior, lateral, and roof walls. Profuse bleeding from the tumor and left orbit hindered a comprehensive assessment of tumor extension into the orbit and residual tumor size. Therefore, complete tumor removal was not possible; instead, reduction was achieved. Subsequent histopathologic and immunohistochemical analysis confirmed the lesion as a metastasis of renal cell carcinoma in the sinonasal tract. Unfortunately, 10 weeks later, the patient passed away. Treatment of metastatic clear cell renal carcinoma depends on the localization of the disease as well as the overall condition of the patient. If there is a solitary metastatic lesion, as in the case of the sinonasal region, surgical treatment is recommended. Metastasis of clear cell renal carcinoma to the sinonasal region is a rare occurrence; however, it is essential to consider it in cases of rapidly growing tumor masses, typically presenting with symptoms such as epistaxis and nasal obstruction.

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.

IgG4-связанное заболевание с вовлечением тканей глазницы у пятилетнего ребенка

Case study IgG4-related disease involving the tissues of the orbit in a five-year-old child Yu.V. Kotova, E.E. Grishina, E.I. Kondratieva, G.I. Fokanova, I.M. Tarabanko Moscow Regional Research Institute of Childhood, Mytishchi, Moscow Region, Russian Federation M.F. Vladimirsky Moscow Regional Research Institute, Moscow, Russian Federation Purpose. To demonstrate a rare clinical case of IgG4-related disease in the practice of a rheumatologist, a pediatrician and an ophthalmologist. Material and methods. A 5-year-old child had a lesion in the left orbit, manifested by left-sided exophthalmos, medial displacement of the left eye, and limited outward eye mobility. He underwent an orbitotomy with histological and immunohistochemical examination of the biopsy specimen in the ophthalmology department of M.F. Vladimirsky Moscow Regional Research Institute. A diagnosis of IgG4-related disease involving the left orbital tissue was made. Results. In the rheumatology department of the Moscow Regional Research Clinical Institute of Childhood, the boy received pulse therapy with glucocorticoids. When trying to reduce the dose of glucocorticoids, exophthalmos and swelling of the eyelids on the left increased again. Cyclophosphamide therapy was ineffective. In addition to oral Prednisolone, Rituximab, a drug from the group of synthetic chimeric monoclonal antibodies, was prescribed. A stabilization of the process in the left orbit and an improvement in the general condition of the child ocurred. The duration of remission is 2 years. Discussion. IgG4-related disease is characterized by lymphoplasmacytic infiltration of tissues with a predominance of IgG4-positive cells and subsequent formation of fibrosis. Ophthalmological manifestations of IgG4-related disease are most often characterized by damage to the tissues of the orbit and lacrimal gland. Usually develops in middle-aged people; it is rare in pediatric practice. Conclusion. Patients with IgG4-related disease can be encountered in the practice of a rheumatologist, pediatrician and ophthalmologist. If diagnosis and treatment are not urgent, the disease is prone to progression, which leads to disability of the patient. The main treatment method is long-term use of glucocorticoids. In case of steroid resistance or steroid dependence, early use of immunosuppressive drugs or monoclonal antibodies could be considered. Key words: immunoglobulin G4-related disease, autoimmune orbital myositis, pediatric rheumatology, immunosuppressive therapy

Three-Dimensional Computed Tomography Scanning Study of the Superior Labial Artery in Chinese Individuals for Assessing Filler Injection Safety.

Lip filler injection is one of the most common minimally invasive cosmetic procedures involving the face; however, vascular complications are not uncommon. The aim of this study was to investigate the anatomy of the superior labial artery (SLA) and provide precise topographic information for dermal filler injection into the lips. Computed tomography (CT) scans of 52 cadaveric heads injected with lead oxide were obtained. We then used Mimics software to construct 3D images of the SLA described by a coordinate system based on the bilateral external auditory canal and the left orbit. This study aimed to classify the SLA in the Han Chinese population, measure its diameter at specific points, and determine the thickness of the lip at those points. Ultimately, we utilized a thermal imaging technique to illustrate the course and depth of the SLA within the lip. The objective of this study was to provide safe guidance for clinical injections. In this study, the SLA was successfully identified in all cadavers. The mean overall diameter of the superior labial arteries was 1.36±0.28 mm. The superior labial artery showed a general course from deep to shallow with an average depth of 5.68±1.68 mm from the oral commissure to the midline. There are anatomical differences in the superior labial arteries among Chinese people. Furthermore, 3D CT images can digitally elucidate the exact positions of the superior labial artery via a coordinate system, improving the safety of upper lip filler injections in clinical settings. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Multicompartmental Paranasal Sinus Osteoma Complicated by Frontal Bone Hyperostosis, Intracranial Mucocele, and Inflammatory Pseudotumor

Parasinusal osteoma complicated by intracranial and orbit extension, cranial vault hyperostosis, intracranial mucocele and inflammatory pseudotumor is exceptional. A 68-year-old man complaining a long history of progressive proptosis and recurrent episodes of keratoconjunctivitis of the left eye, with restriction in upward gaze, was observed. Contrast-enhanced magnetic resonance imaging revealed a frontal sinus lesion extending to the left anterior fossa and orbit, featuring intracranial cystic component and heterogeneous contrast-enhancement. Head computed tomography confirmed the double calcific-cystic nature of the lesion. A left supraorbital-pterional approach allowed the complete resection of mucocele and drilling of intracranial and orbital osteoma, including the intra-sinusal component. The frontal sinus was cranialized and a flap of pericranium, reinforced by Gelfoam sponge, was reflected on the anterior cranial base/orbital roof. The postoperative course was uneventful; MRI depicted resolution of the proptosis. The histologic examination was in favor of parasinusal osteoma associated to intracranial mucocele, frontal bone hyperostosis and inflammatory pseudotumor.

Radiologist’s approach in diagnosing fronto-ethmoidal meningoencephalocele in an adult: a case report

BackgroundFronto-ethmoidal meningoencephalocele is an uncommon pathology. Most of the cases present a history of trauma or paranasal surgical intervention. Spontaneous development of such pathology is extremely rare. The aim of this case report was to demonstrate a rare appearance of spontaneous fronto-ethmoidal meningoencephalocele in an adult male with high-quality images in multiple modalities used in radiology.Case presentationThis case report demonstrates an extremely rare pathology of spontaneous development of fronto-ethmoidal meningoencephalocele in a 50-year-old male. The patient complained of swelling involving the nasal bridge, the left side of the nose, and the left cheek for more than 20 years, which was spontaneous without any trauma or surgical intervention. The patient underwent radiological investigations of ultrasonography, computed tomography of the paranasal sinus, and contrast-enhanced magnetic resonance imaging of the brain. The diagnosis of spontaneous development of left fronto-ethmoidal meningoencephalocele abutting the left orbit was made. No other complications of the brain tissue were noted.ConclusionsKnowledge about etiopathogenesis and various types of meningoencephalocele helps manage this pathology.

Peripheral giant cell granuloma of orbit: A case report

Giant cell reparative granuloma (GCRG) is a rare locally aggressive benign lesion of orbit with a total of less than 15 reported cases worldwide. A 35-year-old male presented with a left upper eyelid mass. Contrast-enhanced computed tomography orbit revealed a soft-tissue density lesion of the extraconal compartment of the left orbit. Wide local excision was done. Histopathology showed peripheral giant cell granuloma of the orbit with fibroosseous component. No recurrence has been seen after eight months. Definite diagnosis of GCRG is only possible by excision biopsy. Early intervention is important to differentiate from malignant lesion.

Synchronous Ewing sarcoma of the fibula with involvement of the orbit and abducens nerve palsy

Simultaneous or synchronous Ewing sarcoma (ES) is the occurrence of multifocal lesions without pulmonary involvement. This is a very rare condition. We present a case of multifocal synchronous ES of the fibula with orbital involvement and adducens nerve palsy. A 17-year-old boy presented with pain and swelling in both legs for 6 weeks. He was unable to walk due to pain. He was pale and cachexic. There was proptosis and ptosis in the right eye. His investigations were consistent with a multifocal malignant tumor. There were lesions in the left fibula, tibia, right tibia, and right orbit. The histopathological diagnosis was Ewing sarcoma (ES). This is the first report of synchronous ES involving the orbit and abducens nerve.

INFERIOR RECTUS FIBROSIS AND RESIDUAL DIPLOPIA FOLLOWING SCLEROTHERAPY FOR ORBITAL VENOUS MALFORMATION

Orbital vascular lesions are challenging to treat and observation is often preferred if symptoms are mild. Historically, surgical excision of the lesions used to be the preferred treatment, but intraoperative bleeding and incomplete removal were common. Therefore, sclerotherapy has become the treatment of choice by virtue of being minimally invasive and extremely effective. Sclerotherapy involves multiple intralesional injections of sclerosing agents like bleomycin and sodium tetradecyl sulfate. In this communication, we describe an unusual complication associated with sclerotherapy to treat an orbital venous malformation in a young woman diagnosed with a type-2 low flow venous malformation (orbital varices) in her left orbit. There was complete resolution of the lesion. However, following the procedure, she complained of diplopia accompanied by left hypotropia. The condition persisted six months after the procedure. Forced duction test confirmed a tight inferior rectus and she refused any further intervention. This case report discusses the possible mechanisms of this rare and unusual complication associated with sclerotherapy while treating orbital vascular lesions.

Tolosa Hunt Syndrome: An Atypical Presentation of a Rare Condition

Abstract Purpose: Tolosa-Hunt Syndrome (THS) is a rare disorder with signs and symptoms that mimic various other diseases. Diagnosis of THS, especially in atypical cases, can be challenging due to the need to exclude other diseases. Observations: A 59-year-old-male presented with acute vision loss of his left eye preceded by acute worsening of chronic headaches. Additionally, he had near complete ophthalmoplegia of this eye. He underwent extensive workup including numerous hematologic studies, cerebrospinal fluid studies, and neuro-radiological imaging studies. All tests had normal results except for an elevated ESR of 92 mm/hour and an abnormal MRI of the brain and orbits. The MRI revealed the left orbit as abnormal with proptosis and intraconal inflammatory change. It also showed abnormal enhancement of the left orbital apex and adjacent lateral aspect of the left sphenoid sinus. He was diagnosed with THS and rapidly responded to corticosteroid treatment. Conclusions and Importance: THS can be challenging to diagnose because it is a diagnosis of exclusion. An added difficulty with THS is the need to quickly recognize and diagnose it since early treatment with corticosteroids can produce a rapid treatment response and decreases the chance of severe residual symptoms. THS does not usually affect the optic nerve, but optic nerve damage can occur and involvement of the optic nerve further increases the urgency of diagnosing it and promptly initiating treatment.

Primary intraorbital inflammatory lumpy lesion: A rare case report.

Eosinophilic angiocentric fibrosis (EAF) is considered to be a kind of benign IgG4-related disease, and it is more often found in the nasal cavity. We present a pretty rare case of orbital EAF that is unlike any other reported case for this case is an IgG4 negative orbital EAF and successfully treated by the fronto orbitozygomatic approach surgery. This is a 68-year-old man from a rural area of Inner Mongolia Autonomous Region, went to our hospital for a 2-month history of vision loss with a local hospital orbital computer tomography which showed that there was a lesion in his left orbit. The inspection of the patient revealed that the patient left eye was protruding outward and the left eyelid unable to complete open or close. And his left eyeball movement had difficulty in all directions. Postoperative pathology diagnosed that this was a case of IgG4-negative EAF case. Orbital EAF. Surgical radical resection and postoperative glucocorticoid therapy. After surgery, the left eye vision of this patient increased to 0.6 tested in the standard logarithmic visual acuity chart. And his left eyeball movement dysfunction and eyeball outward protruding get a partially relief. EAF occurring in the orbit is a very rare disease and immunohistochemical results of EAF can be IgG4 negative.

Cerebral blood flow changes in maintenance hemodialysis patients with restless legs syndrome and their clinical significance:a cross-sectional case-control study

ObjectiveRestless legs syndrome (RLS) stands as a prevalent neurological complication within maintenance hemodialysis (MHD) patients. However, the alterations in cerebral blood flow (CBF) among MHD-RLS patients remain uncharted. Through the utilization of the arterial spin labeling (ASL) technique, we evaluated the fluctuations in CBF within distinct brain regions and analyzed the risk factors for the development of RLS in MHD patients in the context of the clinic.MethodsThirty-one MHD patients with concomitant RLS (MHD-RLS group) and thirty-one non-RLS patients matched based on age, gender, as well as cognitive function (MHD-nRLS group) were included. Through image preprocessing and data analysis, the changes in CBF values in distinct brain regions were obtained, and the CBF values of brain regions with substantial differences between the two groups were correlated with the RLS scores. Furthermore, the differences in baseline data were compared, and through the utilization of multifactorial logistic regression, the independent risk factors for the development of RLS were examined.ResultsCompared with the MHD-nRLS group, the MHD-RLS group had increased CBF in the right superior temporal gyrus, reduced CBF in the right hippocampus, left middle frontal gyrus, inferior frontal gyrus of right triangle, middle frontal gyrus of left orbit, left precentral gyrus, and left precuneus. Only left precentral gyrus CBF were negatively correlated with RLS scores after correction for dialysis duration(r = -0.436, P = 0.016). Accordingly, multifactorial regression analysis by stepwise method yielded that the left precentral gyrus CBF values(OR: 0.968, 95%CI: 0.944–0.993, P = 0.012) remained an independent risk factor for RLS in MHD patients. In addition, the results showed that hemodialysis duration (OR: 1.055, 95%CI: 1.014–1.098, P = 0.008) and serum iron levels (OR: 0.685, 95%CI: 0.551–0.852, P = 0.001) were also risk factors for the development of RLS.ConclusionPatients afflicted with MHD-RLS exhibit alterations in CBF across several brain regions. Notably, the left precentral gyrus might serve as a pivotal region influencing the onset of RLS among MHD patients. Furthermore, extended hemodialysis duration and a relative insufficiency in serum iron levels independently contribute as risk factors for RLS development within the MHD patient population.

Kimura's Disease Finding on Ocular Adnexal Mass

Introduction: Kimura's disease is an unknown chronic lymphoproliferative inflammatory disease affecting the skin, soft tissues, and lymph nodes. Until September 2020, only 200 cases of Kimura's disease were reported worldwide, however, their exact incidence is unknown. Here, we are interested in reporting a patient with Kimura's disease of the ocular adnexa due to its rarity and to enhance the knowledge of ophthalmologists about confirming the diagnosis of Kimura's disease. Case Presentation: A 40-year-old female presented with a chief complaint of a mass on her left nasal orbit for the last year. The mass gradually increased for six months, and it was painless. The systemic laboratory workup revealed eosinophilia and increased serum IgE. Contrast CT-Scan revealed a benign tumor, suspect dermoid cyst. She underwent surgery, and the histopathologic showed Kimura's disease. The patient was followed up on a scheduled basis, and there was no recurrence during four months of monitoring. Conclusions: A clinical, systemic laboratory, and histopathological examination is required to confirm the diagnosis of Kimura's ocular adnexa disease and determine the best therapy for the patient due to the high recurrence rate. Combining surgical excision with postoperative radiation is recommended as the most effective treatment in terms of controlling the residual lesion and minimizing the recurrence rate while causing the fewest side effects. Eosinophil screening regularly is advised to evaluate the recurrence rate.