Superior Oblique Muscle Extramedullary Plasmacytoma in a Patient with Multiple Myeloma and a Review of Literature.

Introduction: Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are presentations of MM. EMD is usually the sign of a more aggressive form of the disease. Herein, we report a patient with refractory MM presenting with extramedullary plasmacytoma in the superior oblique (SO) muscle. Case Presentation: A 51-year-old female presented complaining of gradual protrusion of the left eye and ocular pain from 20 days prior. She received bone marrow transplantation 1 year prior and was on a chemotherapy regimen for MM for the past 1 year. Ocular examination revealed proptosis of the left eye and mild limitations of adduction and elevation. Orbital magnetic resonance imaging demonstrated remarkable enlargement of the left SO muscle with focal contrast enhancement. The patient underwent a biopsy and mass debulking. The histopathologic exam revealed fibromuscular tissue containing a neoplasm composed of sheets of plasmacytoid cells in a varying degree of differentiation with intervening scantly vascularized stromal components. The plasmacytoid cells were diffusely positive for a cluster of differentiation 138 (CD138), leading to a diagnosis of EMD involving the EOM and soft tissue of the orbit. The patient underwent palliative radiotherapy and a systemic workup. The PET-CT scan revealed involvement of the pelvic bone and left calf. Accordingly, the chemotherapy regimen was upgraded to reflect the aggressive nature of the disease. In the last follow-up, there was no sign of tumor reactivation in the orbital soft tissues. Unfortunately, the patient succumbed to her illness 7 months following her most recent presentation. Conclusion: Early recognition of disease recurrence is lifesaving in MM patients; ophthalmic manifestations should be seriously considered as a sign of MM activity.

Lacrimal Drainage System and Nasal Cavity Melanoma after Complete Treatment of Conjunctival Melanoma.

Malignant melanoma of the conjunctiva is a rare tumor of the ocular surface with potential fatal consequences and a high likelihood of recurrence. Although routes for extending the tumor, including local, hematogenous, and lymphatogenous, are pretty straightforward, the indirect extension through free-floating tumoral cells to the nasolacrimal duct is not described thoroughly. We report a case of malignant melanoma of the conjunctiva which presented with local recurrence in the intranasal cavity and lacrimal sac two years after the primary surgery (without involvement of the ocular surface and punctum on the second occasion). However, there was no evidence of distant metastasis on either occasion. This case demonstrates the possible noncontiguous spreading route of melanoma tumoral cells and highlights the need for attention to the surgical technique, and careful follow-up to detect further disease activity.

Management of Large Conjunctival Cysts in a Patient with Stevens-Johnson Syndrome: A Case Report and Review of the Literature.

Stevens-Johnson syndrome (SJS) is a life-threatening mucocutaneous disease with various etiologies including drugs, infections, and malignancies. Ocular manifestations of SJS vary from the membrane, symblepharon formation, and epithelial defect in the acute phase to trichiasis, eyelid margin keratinization, and lacrimal duct obstruction in the chronic phase. A 13-year-old boy with a history of drug-induced SJS presented to our clinic complaining of a mass in the nasal side and inferior fornix of the right eye from 1 year ago. The mass-like lesion in the medial side of the right eye was accompanied by ankyloblepharon, symblepharon, and ptosis and limited ocular movement. Orbital imaging showed cystic lesions on the medial side of the right globe and the inferior fornix. Two large cysts were entirely surgically excised. Histopathologic investigation revealed conjunctival tissue with nonkeratinized epithelium and goblet cells. There was no sign of conjunctival cyst recurrence or symblepharon formation on the 6th-month follow-up. The inferior fornix achieved acceptable depth and the ocular movements became normal.

A Survey on Orbital Space-Occupying Lesions during a Twelve-Year Period from a Referral Center in Iran.

Purpose: In this study, we describe different orbital space-occupying lesions (SOLs) from a referral center in Iran. Methods: In this retrospective case series, all records of "orbital tumors" with a definite histopathologic diagnosis at a referral center in Iran were reviewed from April 2008 to May 2020. Results: A total of 375 orbital SOLs were included. The study population consisted of 212 (56.5%) female and 163 (43.5%) male subjects with overall mean age of 31.09 ± 21.80 years. The most common clinical presentation was proptosis and the superotemporal quadrant was the most frequent site of involvement. Extraconal lesions (276 cases, 73.6%) outnumbered intraconal lesions (99 cases 26.4%). The great majority of SOLs (344, 91.7%) were primary, while 24 (6.4%) were secondary and 7 (1.9%) were metastatic. Benign lesions (309, 82.4%) were much more common than malignant SOLs (66, 17.6%). Overall, dermoid cysts and malignant lymphoma were the most prevalent benign and malignant orbital SOLs, respectively. The malignant to benign lesion ratio was 0.46 in children (≤18 years), 0.81 in middle-aged subjects (19-59 years), and 5.9 in older (≥60 years) cases. The most common type of malignancy was rhabdomyosarcoma in children, lymphoma in middle-aged subjects, and invasive basal cell carcinoma in older age group. Conclusion: Over the 12-year study period, benign, primary, extraconal orbital SOLs were more frequent than malignant, secondary, and intraconal lesions. The ratio of malignant lesions increased with age in this cohort of patients.

Health-related quality of life among adult patients with visual impairments in Yazd, Iran.

BACKGROUND: Visual impairment (VI) is one of the major public health problems that cause suffering, disability, loss of productivity, and reduced quality of life (QoL). This study aimed to evaluate the health-related QoL (HRQoL) among adults with visual problems in 2021. MATERIALS AND METHODS: This analytical cross-sectional study was conducted on 300 patients with VIs referring to ophthalmology centers in Yazd, Iran. Data were collected through face-to-face interviews using EQ-5D, visual analog scale (VAS), and demographic information questionnaires. The results were analyzed using independent sample t-test, one-way analysis of variance, Pearson correlation coefficient, and adjusted limited dependent variable mixture model (ALDVMM) model by STATA, and SPSS. RESULTS: The mean and standard deviation of EQ-5D-5 L index and EQ-VAS score in the studied patients were 0.68 ± 0.25 and 72.46 ± 19.36, respectively. Most problems at unable/extreme level were related to the mobility dimension (12%) and the usual activities dimension (9%). Factors related to HRQoL scores using ALDVMM model showed that divorced or widow marital status, age over 50, having strabismus, and acuter visual problems had significant negative effects on EQ-5D-5 L index values (P < 0.05). CONCLUSIONS: The results showed that HRQoL was moderate in patients with VIs and reduced in patients with high disease severity, old age, lack of a spouse, retirement, and nonuniversity education. As a result, socioeconomic and demographic characteristics were required to be considered in visual health policies.

Invasive Orbital Squamous Cell Carcinoma in a Patient with Multiple Myeloma.

Background: Orbital squamous cell carcinoma (SCC) is a rare entity. It is often a result of local invasion of SCC originating from the skin, nasopharynx, nasal cavity, paranasal sinuses, conjunctiva, lacrimal glands, or sac or less commonly occurs through hematogenous metastasis. Herein, we report a patient with orbital SCC with a history of multiple myeloma (MM). Case presentation. A 45-year-old woman with a history of MM in the past two years presented to our clinic complaining of gradual right eye proptosis for six months. The relative afferent pupillary defect was detected in the right eye on her examination. Ocular movements of the right eye were limited in all directions. Orbital magnetic resonance imaging demonstrated an infiltrative mass in the right orbit extended from the anterior to the orbital apex and the optic canal. The patient underwent debulking, and a histopathology examination revealed SCC results. No other secondary site was found to be the origin of the tumor. Result: The patient underwent chemotherapy and subsequent radiotherapy. To our knowledge, this is the first report of concomitant MM and primary orbital SCC.

Huge heterotopic brain cyst of the orbit: report of a case and its management.

Heterotopic brain tissues are a group of extracerebral neuroglial tissues. Heterotopic brain tissue in the orbit remains a rare clinical entity. This article presents a 7-year-old male child who presented with severe globe displacement, proptosis, and vision loss in the right eye. The orbital imaging showed a huge orbital cystic mass displacing the globe. The cyst was excised entirely from the orbit. The histopathological investigations revealed the presence of a cystic lesion containing brain tissue that was immune reactive for S-100 and glial fibrillary acidic proteins. The diagnosis was confirmed to be heterotopic brain tissue due to the lack of visible bony defect. The relevant literature was also reviewed.

Facial and Abducens Nerve Palsies Following COVID-19 Vaccination: Report of Two Cases.

A broad spectrum of neurological side effects has been reported after immunisation for COVID-19, including functional neurological disorders, cerebral vascular events, cerebral venous thrombosis, intracerebral haemorrhage, neuroleptic malignant syndrome, cranial nerve palsies, and otologic manifestations. Multiple cranial neuropathies have also been reported following vaccination in which involvement of VII nerve is the most prevalent, followed by the VI, III, and IV nerves. We describe two male patients, one with with facial nerve palsy and the other with abducens nerve palsy following COVID-19 vaccination. The patient with facial nerve palsy received the AstraZeneca vaccine 2 days before the symptoms began. In contrast, the patient with the abducens palsy had received his first dose of the Sinopharm vaccine 7 days previously. Both patients demonstrated a gradual recovery within the next 2 months. Further studies are required to investigate the proper relationship between cranial nerve palsies and vaccinations.

Non-absorbable versus Absorbable Sutures for Medial Rectus Advancement in Consecutive Exotropia, a Pilot Randomized Clinical Trial.

PURPOSE: To compare non-absorbable and absorbable sutures for medial rectus advancement in consecutive exotropia. METHODS: In a pilot randomized clinical trial, 40 subjects with consecutive exotropia >15 PD that underwent unilateral medial rectus advancement with or without resection were randomly assigned to two groups: using non-absorbable (polyester) suture in the non-absorbable group and absorbable (vicryl) suture in the absorbable group. The success rate was defined as the final postoperative angle of deviation <10 PD. RESULTS: Thirty-three patients (18 in the non-absorbable and 15 in the absorbable group) had completed the study. At last follow-up, the distance deviation improved from 29.2 ± 15.5 to 7.6 ± 7.9 in the non-absorbable group and from 25.9 ± 8.4 to 8.1 ± 10.7 in the absorbable group. The near deviation improved from 31.4 ± 15.9 to 7.2 ± 8.0 in the non-absorbable and from 29.0 ± 7.6 to 6.8 ± 11.9 in the absorbable group. The amount of the final correction of the distance and near deviation was not statistically different between the groups (P = .80 and P = .99, respectively). At the final examination, the exoshifts for distance and near were not statistically different between 2 groups (p = .61 and 0.54, respectively). At the final examination, the success was obtained in 12 patients (66.7%) and 8 patients (53.3%) in the non-absorbable and absorbable group, respectively (p = .73). CONCLUSION: In our study, there was no statistical difference in success rate or exoshift between non-absorbable and absorbable sutures. So, considering local inflammation induced by non-absorbable sutures in some cases, the absorbable sutures can be an appropriate option for the treatment of consecutive exotropia.

Hummelsheim procedure combined with medial rectus recession in complete sixth nerve palsy and esotropic Duane Retraction Syndrome.

PURPOSE: To evaluate the effect of half tendon vertical rectus muscle transposition (HVRT) combined with medial rectus muscle recession (MRrec) in complete sixth nerve palsy (CSNP) and esotropic Duane Retraction Syndrome (eDRS). METHODS: A retrospective study of patients with unilateral CSNP or eDRS who underwent HVRT combined with medial rectus recession over the course of 18 years. The patients with previous strabismus surgery and follow up less than 3 months were excluded. Overcorrection was defined as any amount of exotropia, and undercorrection was defined as residual esotropia of ⩾10 prism diopter (PD). RESULTS: A total of 39 patients were enrolled including 22 CSNP and 17 eDRS patients. Esotropia improved from 45.8 ± 22 and 22.5 ± 6.4 PD to 0.8 ± 2.5 and 0.3 ± 1 PD in CSNP group and eDRS group respectively. The angle of face turn improved from 34.3 ± 8.4° and 26.5 ± 9° to 0.6 ± 2.4° and 0.8 ± 1.6° in the CSNP group and eDRS group respectively. Abduction limitation improved from -4.5 ± 0.5 and -4 ± 0 units to -2.9 ± 0.5 and -2.7 ± 0.5 units in CSNP group and eDRS group respectively. No patient developed a new vertical deviation. An overcorrection occurred in one patient of the eDRS group who improved after botulinum toxin injection in the ipsilateral lateral rectus muscle. Undercorrection was seen in two patients. CONCLUSION: HVRT combined with MRrec is an effective procedure to improve esotropia, face turn, and abduction limitation in CSNP and eDRS.

Rhino-orbital mucormycosis during steroid therapy in COVID-19 patients: A case report.

PURPOSE: To report two cases of COVID-19 under treatment with a corticosteroid; in one case rhino-orbitocerebral mucormycosis and in another one rhino-orbital mucormycosis developed. CASE PRESENTATION: A 40-year old woman and a 54-year old man with severe COVID-19 underwent corticosteroid therapy for immune-related lung injuries. The first case presented with a bilateral visual loss and complete ophthalmoplegia of the right eye. The second case presented with vision loss, proptosis, orbital inflammation, and complete ophthalmoplegia on the left side. Histopathologic, nasal endoscopic examinations, and radiologic findings confirmed mucormycosis in both patients. The patients denied orbital exenteration and were managed with systemic amphotericin B and daily endoscopic sinus debridement and irrigation with diluted amphotericin B. Because of the intracranial space involvement, the first case died. The second case was successfully managed surgically and medically. CONCLUSION: Rhino-orbital/cerebral mucormycosis may be developed in COVID-19 patients under treatment with corticosteroid, and requires prompt diagnosis and management.

Medial rectus disinsertion for management of chronic complete sixth nerve palsy.

PURPOSE: To report the outcomes of the medial rectus (MR) disinsertion procedure for the management of refractory esotropia (ET) with or without Abnormal head posture (AHP) in chronic complete sixth nerve palsy. METHODS: This is a retrospective case series of patients with sixth nerve palsy who suffered from residual ET and diplopia following the conventional strabismus surgeries and underwent MR disinsertion procedure between April 2017 and February 2020. This procedure was offered to the patients who declined to use prism and did not wish to perform surgery on the fellow eye. The demographic and clinical data, including sex, age, visual acuity, pre and postoperative angle of strabismus, duction limitations, results of forced duction and force generation tests, details of prior strabismus surgeries, orbital CT scan findings, and follow up duration were collected from the medical records. RESULTS: Six patients were enrolled in this study. Mean age was 35.0 ± 14.0 years, and mean follow-up was 15.3 ± 5.9 months. The ET at the Primary position (PP) was 35.0 ± 18.4 prism dioptre (PD) before MR disinsertion, which decreased to 14.2 ± 17.4 PD after MR disinsertion procedure. Four cases needed additional complementary surgeries to improve residual ET in PP. No case developed overcorrection. Abduction deficiency was -5.0 ± 1.3 before MR disinsertion, which improved to - 2.8 ± 0.5 units at last follow-up. The mean of induced adduction deficiency was - 2.9 ± 0.4 at last follow-up. CONCLUSIONS: MR disinsertion can be considered in patients with chronic complete sixth nerve palsy and refractory diplopia when the conventional methods have failed.

Temporalis Fascia for the Management of Medial Rectus Damage Secondary to Endoscopic Sinus Surgery: A Case Report.

PURPOSE: To report a challenging case of medial rectus (MR) damage secondary to endoscopic sinus surgery (ESS). METHODS: A 37-year-old woman presented with diplopia, exotropia, and adduction deficit of the right eye due to the right MR injury secondary to the ESS. RESULTS: As a stepwise approach, she respectively underwent repairing of the damaged MR plus ipsilateral lateral rectus recession, half-tendon vertical rectus transposition to the MR insertion, and eventually nasal globe fixation with temporalis fascia that ultimately provided a single vision in the primary position with orthophoria. CONCLUSIONS: Re-establishment of an acceptable field of a single binocular vision in the context of iatrogenic MR injury is challenging and often requires multiple step-wise operations. Temporalis fascia is a safe and strong autogenous material, which can be used for globe fixation in this setting.

Lacrimal Drainage System Problems After Rhinoplasty.

BACKGROUND: To report case series of permanent nasolacrimal system problems following rhinoplasty METHODS: The documents of patients with epiphora and history of rhinoplastic surgery were reviewed. The data of patients with permanent epiphora (continued over 3 months or started after 3 months of post-rhinoplasty surgery) and lacrimal drainage system (LDS) problems were analyzed for demographics, the result of diagnostic probing and irrigation, findings of orbital and paranasal sinuses CT scan, abnormalities in nasal endoscopy, treatment, and follow-up data. RESULTS: Forty-three patients with epiphora and history of rhinoplasty were referred to our clinic. Ten of them had permanent epiphora and LDS problems. In these patients, the mean time between rhinoplasty and initial symptoms of LDS problems was 10.3 ± 15.1 (range, 0-45 months) and the mean time between rhinoplasty and the LDS surgery was 22.2 ± 19.5 months (range 4-60 months). Diagnostic probing and irrigation test revealed canalicular stenosis in four (40%) patients, pus reflux in four (40%), clear reflux without passage in four (40%), and partial nasolacrimal duct obstruction (NLDO) in two (20%) of patients. DCR was performed in eight (80%) patients. Therapeutic probing and lacrimal intubation were performed in two (20%) patients. CONCLUSION: A permanent injury of the LDS is one of the important complications of the rhinoplastic surgery that should be managed based on the onset and duration of the symptoms and the location of the injury. LEVEL OF EVIDENCE IV: 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 .

Intraconal Orbital Hidrocystoma in an Infant.

ABSTRACT: Intraconal orbital hidrocystoma is a very rare entity. Herein, a 6-month old boy with an intraconal apocrine hidrocystoma will be reported. The patient presented with a left-sided mild proptosis and significant anisometropic hypermetropia. Clinical examinations revealed choroidal folds and optic disc blurring in the left eye. Orbital MRI disclosed an intraconal well-defined cystic lesion that was hypointense in T1 and hyperintense in T2 weighted images. The lesion was excised completely through a lateral orbitotomy and diagnosed histologically as an apocrine hidrocystoma. Excepting partial improvement of anisometropia, other clinical signs were improved after surgery.

Management of Thyroid Eye Disease-Related Strabismus.

PURPOSE: To review various treatment methods in thyroid eye disease (TED) related strabismus. METHODS: We searched in PubMed and Google Scholar and Ovid MEDLINE for keywords including TED-related strabismus, strabismus in thyroid-associated ophthalmopathy, Graves' ophthalmopathy related strabismus or squint, and restrictive strabismus. Two expert strabismus specialists selected and evaluated the English articles that were related to our paper and had been published since 2000. Some articles were added based upon the references of the initial articles. RESULTS: One hundred fifteen articles were found, 98 of which were mostly related to the topic of this review. Management of TED-related strabismus was reviewed and categorized in non-surgical and surgical. Botulinum toxin A (BTA) is a useful non-surgical management of strabismus in an active TED and residual deviation after strabismus surgery. Postoperative under-correction is relatively more common in TED-related esotropia. Lateral rectus resection and BTA are the options to manage the problem. Muscle rectus muscle resection should be performed after maximum recession of restricted muscles. It should be avoided on a restricted or enlarged muscle. Management of TED-related vertical deviation is challenging. In these cases, the surgical treatment selected depends on forced duction test (FDT) (pre and intraoperative), orbital imaging (which muscle is enlarged), and the amount of vertical deviation (in both down-gaze and primary position). CONCLUSIONS: TED-related strabismus needs careful evaluation and management to achieve optimal outcome. Different surgical and non-surgical options are available for intervention in TED-related strabismus.

Arteritic Anterior Ischaemic Optic Neuropathy with Unusual Systemic Manifestations.

An 84-year-old man presented with anterior ischaemic optic neuropathy with unusual histopathological findings for giant cell arteritis on temporal artery biopsy. Further systemic manifestations occurred three months later with lung nodules, renal dysfunction and sinusitis. A probable diagnosis of polyangiitis overlap syndrome was made and he was treated with steroids and an immunomodulator.

Orbital, Periorbital, and Intracranial Emphysema Caused by Compressed Air Injury in a 5-Year-Old Child.

A 5-year-old boy was accidentally injured by a compressed air blast to his right eye and developed periorbital and subconjunctival emphysema and proptosis subsequently. CT scan showed periorbital subcutaneous and intraorbital emphysema around the right eye, as well as intracranial and cervical emphysema without frank fractures in orbital bones. He was examined under sedation, and the subconjunctival air was aspirated. He was also prophylactically treated with topical and systemic antibiotics to prevent infection. Emphysema was resorbed entirely within 3 weeks. In conclusion, compressed air injury can occur in a child and manifest with orbital and periorbital emphysema without any detectable fracture and any clear entrance site and may need emergent management to save the vision or upper respiratory airways.

Goldmann Applanation Tonometer Versus Ocular Response Analyzer for Measuring Intraocular Pressure After Descemet Stripping Automated Endothelial Keratoplasty.

PURPOSE: To evaluate agreement between IOP measured using the ocular response analyzer (ORA) versus using the Goldmann applanation tonometer (GAT) in patients treated by Descemet stripping automated endothelial keratoplasty (DSAEK). METHODS: This prospective, case-control study comprised 38 normal eyes and 37 post-DSAEK eyes. IOP was measured using the GAT (IOPGAT). The ORA was used to measure corneal biomechanics and cornea-compensated IOP (IOPCC). Corneal hysteresis (CH), corneal resistance factor, and IOP measurements of the 2 groups were compared. Intertonometer agreement was evaluated in each study group using Bland-Altman plots and 95% limits of agreement. Univariate analysis was used to evaluate the effect of independent factors on the difference between the 2 IOP readings (ΔIOP). RESULTS: The IOPCC and IOPGAT readings showed no significant intergroup differences. IOPCC was significantly higher than IOPGAT, with a mean difference of 3.0 ± 3.3 mm Hg in the normal group (P < 0.001) and 4.5 ± 3.1 mm Hg in the DSAEK group (P < 0.001). The 95% limits of agreement for the IOPCC and IOPGAT was between -3.4 and 9.5 mm Hg and between -1.6 and 10.6 mm Hg in the normal and DSAEK groups, respectively. CH (r = -0.57, P < 0.001) and donor thickness (r = 0.81, P = 0.04) were identified as the main predictors of ΔIOP after DSAEK. ΔIOP did not vary significantly with the central corneal thickness or corneal resistance factor. CONCLUSIONS: The IOPGAT and IOPCC were significantly different, which indicates that the GAT and ORA cannot be used interchangeably for measuring IOP after DSAEK. The difference between these 2 measurements was primarily affected by the variations in CH and donor thickness.