Uveal melanoma in the Iranian population: two decades of patient management in a tertiary eye center.

BACKGROUND: To assess the characteristics and outcomes of uveal melanoma management at a tertiary center in the Middle East. METHODS: A study on 164 patients with uveal melanoma was conducted by reviewing the available medical records, ultrasound, and pathology report results. Age at diagnosis, tumor location and size, treatment mode, visual outcome, metastasis, mortality, and survival were studied. RESULTS: The mean age of patients was 52.0 ± 15.0 years, and 52.5% were male. Choroidal melanoma was the most common uveal melanoma, followed by the ciliary body and iris melanoma. The mean thickness of tumors was 8.29 ± 3.29. The majority of patients (n = 111, 67.9%) were managed by brachytherapy with ruthenium-106 plaques. Enucleation was performed primarily in 46 (28%) patients and secondarily in nine (5.5%) patients. The sexual disparity was detected as the proximity of uveal melanoma to the fovea in males. For a 61-month mean follow-up period, mortality occurred in eight of our cases, six of which were due to metastasis. The most common site for distance metastasis was the liver (5/6), followed by the lung (1/6). The five-year and eight-year overall survival (OS) rate was 0.947%± 0.019. The 5-year survival rate reached zero in metastatic patients. OS was not statistically different depending on the age, tumor diameters, the primary treatment received, or the histopathologic findings (p > 0.50 for all). CONCLUSION: In this study, individuals diagnosed with UM exhibited an OS rate of around 94% at the five-year mark, which remained consistent up to eight years. Notably, the presence of distance metastasis emerged as the sole statistically significant factor influencing overall survival.

Orbital primary intraosseous hemangioma in a three-month old infant: A case report.

Purpose: To report a 3-month-old boy with rapid progressive orbital intraosseous hemangioma which was treated with excisional biopsy and orbital rim reconstruction. Observation: A 3-month-old boy was referred with the aggressive growth of a mass on the right orbital region. The mass was noted to develop over 4 weeks. On presentation this firm nontender orbital mass measuring 5 × 5cm mimicked a more ominous malignancy. The spiral computed tomography scan showed a destructive mass with protrusion superiorly and inferiorly toward the orbital cavity and anterior cranial fossa. The patient underwent gross tumor resection and reconstruction of the orbital rim. Histology findings revealed an intraosseous hemangioma. There was no evidence of recurrence after 1-year follow-up. Conclusion and Importance: Due to rapid progression, the patient's age, and lesion size, this case is unique. There were additional challenges regarding complete resection, intra-cranial extension, and significant blood loss in an infant. Therefore, in the face of rapidly progressing orbital tumors in infants, despite the very low prevalence of intraosseous hemangioma, this diagnosis should be considered.

Surgical Correction of Cicatricial Lower Eyelid Retraction: A Systematic Review.

BACKGROUND: Cicatricial lower eyelid retraction is a challenging condition. It involves scarring of the lower eyelid, which causes it to retract and expose the sclera. This can lead to complications such as dry eye syndrome and corneal melting. It can be caused by trauma, burns, or previous eyelid surgery. Detailed assessment and understanding of eyelid anatomy and retraction are critical for successful surgical planning. Dynamic and static examinations of the eyelid including measurements of the lower eyelid margin reflex distance (MRD2) and scleral show are also essential to determine the appropriate treatment approach. METHODS: A systematic review was conducted using Medline, Scopus, and Cochrane databases with keywords related to cicatricial lower eyelid retraction. The publication language was limited to English after 2000. A total of 29 articles were included for data extraction and analysis. RESULTS: The main surgical techniques include tarsoconjunctival grafts, spacers, midface lift, and lateral canthal tendon suspension, although no single procedure has been universally recognized as the gold standard. New innovations such as synthetic grafts and xenografts are being explored for their potential in eyelid reconstruction. Severe cases, defined as those with inferior scleral show greater than 2 mm, may require a combination of reconstruction methods. CONCLUSIONS: Correcting cicatricial lower eyelid retraction is a major challenge in oculoplastic reconstruction. The surgical approach should be individualized, considering the pathologies and etiologies of lid retraction. In-depth knowledge and careful surgical planning are essential for best outcomes. There is no gold standard technique, and postoperative outcomes, complications, and management vary depending on the surgical approach used.

The role of Whitnall's ligament position in the success of levator resection surgery in congenital ptosis.

PURPOSE: This study aimed to investigate the role of Whitnall's ligament position in the success of levator resection surgery in congenital ptosis. METHODS: It was an interventional case series on patients with congenital ptosis who underwent levator muscle resection in Farabi Eye Hospital (2020-2022). Patients with incomplete follow-up, a history of trauma, poor Bell's phenomenon, previous ocular and lid surgeries, poor levator function (≤ 4mm), and syndromic ptosis or systemic diseases were excluded. During the surgery, several factors, including the distance between Whitnall's ligament and the upper edge of the tarsus (W-distance), the vertical length of the tarsus (T-length), and the amount of levator muscle resection (LMR), were measured. A successful outcome was defined as the inter-eye difference of margin reflex distance-1 (MRD1) ≤ 1 and post-op MRD1 ≥ 3 OR the inter-eye difference of MRD1 ≤ 0.5 with any value of post-op MRD1 in unilateral cases and Postop-MRD1 > 3 in bilateral cases during the 3-months period. RESULTS: Thirty four eyes of 34 patients were included, and 79.4% of patients achieved successful outcomes. In univariate analysis, Preop-MRD1 and Preop-LF had meaningful negative correlations with the amount of LMR to reach the successful outcome (p < 0.05), which was only meaningful for Preop-LF in multivariable analysis (p < 0.05). Noticeably, W-distance had a significant positive correlation in univariate and multivariable linear regression (p < 0.05). CONCLUSIONS: W-distance can be considered a significant new parameter other than Preop-LF influencing the amount of levator resection needed to achieve success in levator resection surgery.

Orbital histiocytosis and fibrohistiocytosis: the clinicopathological characteristics of 117 patients, over a decade of experience.

PURPOSE: To describe the clinicopathological features of a large cohort of patients with orbital histiocytoses and fibrohistiocytosis, such as Langerhans cell histiocytosis (LCH) and non-LCH disorders, and correlate patients' clinical characteristics with their pathological diagnosis. METHODS: In this retrospective study, medical records of patients presenting to Farabi Eye Hospital, a tertiary eye care center in Tehran, Iran, from 2010 until 2022, were reviewed. Patients' demographics, chief complaint, location and laterality of the tumor, best-corrected visual acuity, presence of bone erosion on imaging, and their pathological diagnosis were retrieved. Excisional biopsy was performed and evaluated through light microscopy and immunohistochemistry study for their respective markers, including CD1a, CD68, CD207, and S100. RESULTS: A total of 117 patients with 11 pathological subtypes of histiocytoses and fibrohistiocyosis were identified, with 56.4% male and 43.6% female patients. The mean age at presentation was 23.4 years (range 1.5 months-73 years). Swelling and palpable mass were the most common chief complaints. LCH was the most common pathology (32.5%), followed by juvenile xanthogranuloma (26.5%) and adult xanthogranuloma (21.4%). Age, lesion location, and bone erosion had a statistically significant difference among the various diagnosed subtypes. CONCLUSIONS: Histiocytoses and fibrohistiocytosis are diverse and rare disorders potentially involving multiple organ systems. Ophthalmic manifestations of these diseases are even more uncommon. We reviewed their orbital presentation along with their respective histopathological findings. Our results also suggested that an orbital CT scan can be of diagnostic value to discriminate LCH from other histiocytic pathologies.

Assessment of retinal and choroidal vessel density and nerve fibre layer thickness changes after orbitotomy in patients with severe non-active thyroid orbitopathy: a prospective study.

PURPOSE: To evaluate the optical coherence tomography angiogram changes in non-active severe thyroid-related ophthalmopathy patients after cosmetic bone decompression. METHODS: Eighteen patients (25 eyes) with severe not active not compressive (NANC) TED who were candidates for decompression surgery for cosmetic reasons were included in this study, and a 3 × 3 mm macular scan was used to measure vessel density and RNFL thickness. Whole macular vessel density in its superficial, deep and choriocapillaris layers was evaluated. The following data were extracted for each of layers: superior and inferior hemispheres, fovea, parafoveal vessel density, its superior and inferior hemispheres, and temporal, superior, nasal and inferior quadrant. RESULTS: The mean RPC increased postoperatively, which was statistically significant in small vessels of peripapillary area (p-value = 0.045). The mean RNFL thickness decreased after surgery and it was statistically significant in the peripapillary (p-value = 0.032) and Inferior-Hemifield area (p-value = 0.036). The choriocapillaris changes were significant in Superior-Hemifield (p-value = 0.031) and Fovea (p-value = 0.03). CONCLUSION: Thyroid-associated orbitopathy patients have a tendency to decrease vascular density and correlated with disease activity more than stage of orbitopathy. There was not a strong and even discrepant result in linkage of RNFL thickness and other optic nerve function tests and TED patient status and it is needed to do studies with more epidemiologic power and same methodology of study to be more comparable.

Anaplastic Large Cell Lymphoma With Intraocular or Ocular Adnexal Involvement: A Case Report and Systematic Review.

PURPOSE: To review the existing literature on patients with anaplastic large cell lymphoma (ALCL) affecting the globe and/or ocular adnexa, and to present a report documenting the clinical course of a patient with ALCL that involved their globe and ocular adnexa. METHODS: PubMed, Scopus, and Google scholar were systematically searched for all cases of ALCL involving intraocular or adnexal ocular structures from inception to May 2023. Moreover, a new reported case added to the cases found in searches. RESULTS: The review identified 1680 studies, with 8 meeting inclusion criteria. A total of 9 patients were included with a mean age of 29.7 years (median: 30.0, range: 1.3-48). Primary ALCL was present in 5/9 (55.6%) patients. The most common ophthalmic manifestations included periorbital swelling (5/8), chemosis (5.8), and decreased vision (5/7). Misdiagnoses were initially made in 3 patients, and the lag time to correct diagnosis from 3 weeks to 3 months. CD30 expression was positive in all cases, and 6/9 patients were positive for anaplastic lymphoma kinase, resulting in 6/9 patients being diagnosed with anaplastic lymphoma kinase-positive ALCL. In terms of management modalities, chemotherapy was administered in 8/9 patients, while radiation therapy was utilized in 4/9 patients, and 2 underwent autologous stem cell transplantation. Five (55.6%) patients succumbed to ALCL while 4 (44.4%) were alive and disease-free at the last follow-up. The median times from the initial presentation of ALCL to death, ophthalmic presentation to death, and diagnosis to death were 4.12 months (range: 1.1-168.0), 2.62 months (range: 1.1-144), and 4.00 months (range: 0.10-168), respectively. The median follow-up duration was 21.0 months (range: 1.1-168.0). CONCLUSIONS: ALCL involving the globe and ocular adnexa is a rare and highly malignant tumor that can mimic benign clinical conditions. Early biopsy and aggressive treatment with chemotherapy regimens such as CHOP and radiation therapy may be useful.

Corneal biophysical changes after upper eyelid blepharoplasty and ptosis surgery: a review.

Upper eyelid surgeries, such as blepharoplasty and ptosis correction, are commonly performed procedures worldwide. This review examines the effects of these surgeries on ocular properties and visual function. A search of the PubMed and Google Scholar databases was conducted to identify relevant articles published after 2000. The results demonstrate that the ocular and adnexal organs function as a unified visual system, with changes in one component affecting the functions of others. Eyelid surgery can alter ocular properties and functions by modifying retinal lighting and ocular optics. These alterations can affect intraocular pressure estimation, corneal curvature, corneal epithelial thickness, refractive power of the cornea, and intraocular lens calculation. Additionally, eyelid surgery can exacerbate dry eye symptoms and impact contrast sensitivity, which is a significant factor in visual quality. Therefore, understanding these interactions is crucial before performing eyelid surgery and during follow-up. This review summarizes recent literature on the effects of upper eyelid surgery on corneal properties and visual function, emphasizing the importance of considering these factors when planning or undergoing such procedures.

A novel technique for small-incision levator resection with a double mattress suture for ptosis correction.

PURPOSE: To introduce a novel technique for small-incision levator resection in ptosis surgery and evaluate its efficacy in a pilot study among patients affected by congenital or aponeurotic ptosis. METHODS: We prospectively enrolled congenital and aponeurotic ptosis patients if their levator function was not poor (≥5 mm) from June 2021 through October 2022. Surgical technique involved a 1-cm lid crease incision, minimal dissection, and creating a loop passing through the tarsus and levator aponeurosis. Success was defined as postoperative MRD-1 ≥3 mm and inter-eyelid MRD-1 difference ≤1 mm. Eyelid contour quality was scored excellent, good, fair, and poor according to its curvature and symmetry. RESULTS: Sixty-seven eyes (35 congenital and 32 aponeurotic) were included in the study. Mean age was 34±19 years (range, 5-79 years). Mean preoperative levator function and levator resection amount were 9.53 mm and 8.39 mm in the congenital group and 12.34 mm and 4.15 mm in the aponeurotic group, respectively. Mean pre- and postoperative MRD-1 was 1.61 mm and 3.27 mm respectively (P<0.001). The overall success rate was 82.1% (95% C.I: 71.7-89.8%); the result was failure in 12 cases, of which 11 had under-correction. Preoperative MRD-1 was correlated with a success rate (P=0.017). CONCLUSION: The described technique shows non-inferior results to the previously described surgical methods and also it shows very good lid contour outcome and minimal lag. The findings suggest that the double mattress single suture technique can be used in both congenital and aponeurotic ptosis.

Unexpected recurrence and rapid progression of lacrimal gland adenoid cystic carcinoma during pregnancy: a case report.

Adenoid cystic carcinoma (ACC) is the most common malignant neoplasm involving the lacrimal glands, with high rates of recurrence and metastasis. During the pregnancy, reports of recurrence of ACC of the salivary glands and trachea have previously been published, but no lacrimal gland ACC recurrence has been reported. We present a 35-year-old woman with lacrimal gland ACC who was initially treated by surgical resection and adjunctive radiotherapy, but her cancer recurred during pregnancy, with rapid progression to cavernous sinuses and brain. Estrogen and progesterone receptors have been detected on lacrimal glands and ACCs of salivary glands. Thus, hormonal changes during pregnancy might contribute to the recurrence of ACC. However, the inherent invasive and recurrent nature of ACC could also account for the regrowth in this patient and further molecular studies can provide more accurate explanations.

Isolated lateral rectus muscle large B cell lymphoma: A rare case report and review of the literature.

Purpose: To report a 65-year-old male patient with primary lateral rectus large B cell lymphoma. Observations: The patient had been referred because of progressive proptosis and limitation of ductions, especially abduction (ortho position at primary gaze) and conjunctival injection. Computerized tomography of the orbit and paranasal sinuses depicted a massive lateral rectus muscle enlargement without any other orbital involvement. Lateral orbitotomy and lateral rectus belly incisional biopsy was done, and histopathologic and immunohistochemical staining and systemic evaluations revealed the diagnosis of primary orbital large B-cell lymphoma. Conclusions and importance: This case indicated that, though rare, extraocular muscle enlargement could be the main finding of primary orbital lymphoma. Large B-cell lymphoma could involve only the orbital tissues, although it is more prevalent with systemic involvement.

Alveolar soft part sarcoma of the superior rectus muscle: Case report and review of literature.

Purpose: Alveolar soft part sarcoma (ASPS) is a very rare type of soft tissue sarcomas which usually occurs in the limbs and trunk. Observations: A 25-year-old woman presented with proptosis and redness of the right eye for 8-month. She suffered from severe right upper lid edema, conjunctival chemosis, downward displacement of the globe, and proptosis. Radiological imaging was nonconclusive. Histopathological evaluations confirmed ASPS. The patient underwent exenteration as a lifesaving procedure. At 16-month follow-up, the patient is stable without any signs of recurrence or metastasis. Conclusions and importance: We report an extremely rare case of ASPS occurring in the superior rectus muscle. Few orbital ASPS cases have been reported in the literature. A literature review of orbital ASPS was done to shed lights on the diagnosis and management of this rare tumor.

Huge Lateral Rectus Solitary Plasmacytoma Causing Shunt Extrusion.

A 54-year-old man with a history of radiotherapy for right maxillary sinus plasmacytoma 3 years previously was referred to an orbital clinic with progressive proptosis in his right eye. His vision had deteriorated after an initial improvement after phacoemulsification 2 years before. He had undergone shunt implantation and later shunt removal following plate extrusion with the diagnosis of neovascular glaucoma following CRVO. His vision remained at no light perception afterwards, despite a controlled IOP with topical medications. In his CT scan, a large orbital mass was seen with lateral rectus involvement. He underwent deep orbitotomy for tumor resection following worsening of symptoms, and his symptoms were improved afterwards. Pathology report was consistent with plasmacytoma with anaplastic features. After tumor resection, he underwent another course of radiotherapy with complete remission of symptoms afterwards.

Giant Cell Tumor: Changing Behavior from Intraorbital to Intraosseous Mass.

PURPOSE: To present a patient with giant cell tumor (GCT) of the orbit by changing behavior from an intraorbital mass to an intraosseous tumor. METHODS: A 16-year-old boy presented with pain, swelling, erythematous of the left upper and lower eyelids, proptosis, and diplopia. Ophthalmic examination revealed chemosis, conjunctival injection, limited elevation, depression as well as abduction in the left eye. RESULTS: Multislice computed tomography scan (CT scan) of the orbit and paranasal sinuses showed a hyperdense, oval, extraconal mass with bone erosion. Magnetic resonance imaging of the orbit showed an inferior lateral isointense, oval, extraconal mass that had indented the globe. The patient underwent superior lateral orbitotomy, and the orbital mass was excised. Two months later, the patient developed proptosis, severe chemosis, and eyelid erythema in the same eye. CT scan showed an intraosseous mass in the lateral wall of the orbit that had pushed the globe anteromedially. Intraosseous tumor was resected, and the lateral orbital wall was drilled during the second surgery. GCT was diagnosed based on pathological survey. CONCLUSION: Following the resection of the orbital GCT, the tumor behavior may change to an intraosseous lesion.

Phakomatous Choristoma of the Eyelid and Orbit: A Case Report.

Phakomatous choristoma was first described as a distinct pathologic entity by Zimmerman in 1971. Report of only 26 cases of this tumor so far is an indicator of the rarity of phakomatous choristoma. We present a 4-month-old infant with an orbital mass beneath the right lower eyelid. Surgical excision was undertaken and the histopathologic findings of a dense fibrocollagenous stroma containing small to medium size islands and glandular-like structures surrounded by thick basement membrane and filled by amorphous eosinophilic material confirmed the diagnosis. Immunohistochemical study showed positive staining for S-100 and vimentin and negative staining for cytokeratins, glial fibrillary acidic protein, smooth muscle actin, synaptophysin, CD34, melan-A, and epithelial membrane antigen markers. This is the first patient with phakomatous choristoma presented from our country and the 27th reported case worldwide. Phakomatous choristoma is a rare, benign congenital tumor of lenticular anlage, almost always presenting in the medial lower eyelid and anterior orbit. Surgical excision is curative and allows precise diagnosis due to the unique histopathologic and immunostaining characteristics of this choristoma.

A Rare Case of Congenital Orbital Cystic Neuroblastoma in an Infant.

Neuroblastoma, a relatively common tumor in infants, is rarely present at birth. A solid mass is the typical appearance of this neoplasm, while cystic formation is uncommon. Congenital cystic neuroblastoma is extremely rare and no previous report exists of its occurrence in the orbit. A newborn girl was referred with an extremely large mass on the right side of her face on the third day of her life. Prenatal ultrasonography had revealed a large mass (80 × 80 mm) in the frontal area. MRI revealed a heterogeneous mass composed of cystic and solid, contrast-enhancing components. Excisional biopsy was successfully performed and histopathologic diagnosis of neuroblastoma was confirmed. Systemic workup was negative for involvement of other organs and levels of catecholamine metabolites of serum and urine (homovanillic acid and vanillylmandelic acid) were normal. Postoperative course was uneventful with complete healing of the anophthalmic socket and normal physical and mental growth and development of the infant. To our knowledge, this is the first report of congenital cystic neuroblastoma primarily arising in the orbit.

Successful Total Resection of an Orbital Epithelioid Hemangioendothelioma with the Aid of Endovascular Embolization.

Hemangioendothelioma is rarely encountered in the orbit. We present a patient with a growing orbital mass for whom surgical excision was planned. Two previous attempts at removing the mass failed due to profuse bleeding. Endovascular embolization was performed before surgery to prevent massive hemorrhage. After embolization, retinal vascular accident (combined central retinal artery and vein occlusion) occurred. However, surgical excision (lateral orbitotomy and transcoronal craniotomy) was successful, and the vision improved postoperatively. Histopathologic examination and immunohistochemistry study confirmed the diagnosis of epithelioid hemangioendothelioma. The combined approach by a team of specialists, including an interventional radiologist performing preoperative embolization of the feeding vessel and joint surgery by a neurosurgeon and oculoplastic surgeon, was the key to the effective treatment of this vascular orbital neoplasm.