A case of bilateral amyloidosis localized to extraocular muscles mimicking thyroid eye disease.

BACKGROUND: Amyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocular muscles is an exceptionally rare occurrence, with only 35 reported cases. This case report sheds light on the importance of considering amyloidosis in the differential diagnosis of patients presenting with proptosis and diplopia, which are often associated with thyroid eye disease. CASE PRESENTATION: A woman in her twenties sought medical attention due to a complaint of diplopia. Her ocular examination revealed almost normal findings except for exotropia and proptosis. Orbital magnetic resonance imaging displayed fusiform enlargement of nearly all eye muscles, a presentation typically observed in thyroid eye disease. However, despite corticosteroid therapy, her symptoms showed no improvement. Given the unusual lack of response to conventional treatment, and inhomogeneous enhancement of the muscle, an extraocular muscle biopsy was conducted. This biopsy yielded a unique finding-amyloid deposition within the muscle tissue. This discovery was particularly intriguing due to the extreme rarity of amyloidosis affecting extraocular muscles, with fewer than three dozen documented cases worldwide. CONCLUSION: This unique case underscores the critical need for a comprehensive approach to diagnosing patients with proptosis and diplopia. While these symptoms are commonly attributed to thyroid eye disease, it is essential to consider alternative diagnoses such as amyloidosis, especially when standard treatments fail to yield results. The discovery of amyloid deposition in the extraocular muscles, although exceedingly rare, emphasizes the significance of a thorough differential diagnosis. In conclusion, this case report highlights the importance of vigilance in clinical practice, encouraging ophthalmologists to explore less common diagnostic possibilities when faced with challenging cases. Further research and clinical investigation are warranted to better understand the mechanisms and potential treatments for amyloidosis affecting the extraocular muscles.

Examination of the safety and effectiveness of low-concentration nitrous oxide anesthesia in cataract surgery.

PURPOSE: To investigate the effects in cataract surgery using local anesthesia along with a 30% low-concentration nitrous oxide (N2O) anesthesia compared with local anesthesia only. SETTING: Saneikai Tsukazaki Hospital. DESIGN: Retrospective, consecutive study. METHODS: Patients who underwent bilateral cataract surgery were enrolled. 37 patients using room air inhalation (Air group) and 45 patients using 30% low-concentration N2O anesthesia (70% oxygen, total 6 L/min) at surgery start (N2O group) were retrospectively reviewed. Systolic blood pressure (BPs), diastolic blood pressure (BPd), and heart rate (HR) at surgery start and end, and mean intraoperative oxygen saturation (% SpO2) were examined. Immediately following surgery, a questionnaire using the Visual Analog Scale score was done to determine intraoperative pain, anxiety, memory, and nausea. RESULTS: No systemic symptoms in all patients and ocular complications requiring treatment were observed. For the N2O and air groups, changes in BPs were -5.38 ± 11.07(P = .01) and 1.27 ± 13.61 mm Hg, and HR were -2.24 ± 6.76 and 0.89 ± 5.18 bpm (P = .001), respectively; intraoperative SpO2 was 99.05% ± 0.74% and 97.44% ± 1.31% (P < .001), intraoperative anxiety was 21.76 ± 23.2 and 37.17 ± 32.79 (P = .002), and intraoperative memory was 55.24 ± 36.8 and 68.91 ± 33.81 (P = .01), respectively. No patients experienced intraoperative nausea. There was no statistically difference in BPd (P = .47) and intraoperative pain (P = .62). CONCLUSIONS: Low-concentration N2O anesthesia may not cause respiratory depression, abnormal vital signs, or nausea in cataract surgery. It can suppress intraoperative anxiety and memory and decrease and stabilize vital signs.

Utility and safety of low-concentration nitrous oxide anesthesia in ptosis surgery.

ABSTRACT: To evaluate the utility of low-concentration nitrous oxide (N2O) anesthesia in ptosis surgeryThis study was a retrospective consecutive case series that included 54 successive patients with blepharoptosis who underwent bilateral levator aponeurosis advancement and on whom skin resection performed by the same surgeon between August 2016 and July 2017. Among these patients, 27 were operated with a local anesthesia injection (air group) and 27 with a local anesthesia injection and low-concentration N2O anesthesia (N2O group). All N2O cases used a total of 6 L of gas comprising 70% oxygen and 30% N2O. Preoperative and postoperative blood pressure (BP) and heart rate (HR) and intraoperative pain, anxiety, nausea, and memory were measured immediately after surgery using visual analog scale score (VASS). Additionally, perioperative side effects were examined.There was no significant difference in age, sex, and preoperative and postoperative margin reflex distance (MRD) between the 2 groups (all P > .05). The intraoperative mean peripheral oxygen saturation was significantly higher (97.5% ±â€Š1.6% vs 99.5% ±â€Š.6%, P < .001), intraoperative HR was significantly lower (78.2 ±â€Š12.8 vs 70.7 ±â€Š11.6 bpm, P = .02), and operation time was significantly shorter (33.1 ±â€Š8.1 vs 29.4 ±â€Š10.3 minutes, P = .03) in the N2O group than in the air group.Difference between intraoperative and preoperative systolic BP (BPs) (+15.8 ±â€Š18.0 vs + 3.1 ±â€Š21.7 mm Hg, P = .02), diastolic BP (BPd) (+7.0 ±â€Š17.4 vs -2.3 ±â€Š13.6 mm Hg, P = .04), and HR (3.2 ±â€Š8.5 vs -3.9 ±â€Š9.4 bpm, P = .01) was significantly lower in the N2O group than in the air group.VASS of intraoperative pain was significantly lower in the N2O group than in the air group (49.5 ±â€Š24.7 vs 22.6 ±â€Š14.9, P < .001), whereas intraoperative anxiety and memory did not present significant differences between the groups (P = .09 and P = .45, respectively). Intraoperative nausea score was 0 for all cases in both groups. There was no other side effect.Ptosis surgery with anesthesia using 30% N2O may effectively suppress intraoperative BP and HR along with pain and shorten the operation time without side effects such as nausea.

Idiopathic Orbital Inflammation Appearing on the Affected Side of Preceding Myasthenia Gravis.

The patient was a 70-year-old man with idiopathic orbital inflammation (IOI) that appeared on the severely affected side of preceding myasthenia gravis (MG). The patient was diagnosed with MG 5 years prior to the onset of IOI. When IOI was diagnosed, an edrophonium test was negative. IOI was considered because he complained of left orbital pain, eyelid swelling, and cerebral MRI exhibited the enhanced lesions along the left orbital periosteum. A biopsy specimen revealed pathological findings compatible with IOI. The administration of corticosteroids was effective for improving the ocular symptoms. IOI should be considered when ocular symptoms deteriorated with soft tissue swelling/pain in MG patients.

Anti-LRP4 Antibody-associated Myasthenia Gravis with a Rare Complication of Thymoma Successfully Treated by Thymectomy.

We herein report the case of a 65-year-old woman diagnosed with myasthenia gravis (MG) after complaining of double vision. The patient had anti-low-density lipoprotein receptor-related protein 4 (LRP4) antibody in her serum, although antibodies against the acetylcholine receptor and muscle-specific tyrosine kinase were not detected. Chest computed tomography showed an anterior mediastinal tumor with a high uptake on fluorodeoxyglucose-positron emission tomography. Endoscopic thymectomy successfully ameliorated her ocular symptoms and showed the lesion to be thymoma. The present case revealed that anti-LRP4 antibody-associated MG can be associated with thymoma, which has been regarded as a rare complication of this disease thus far.

Evaluation of Fundus Blood Flow in Normal Individuals and Patients with Internal Carotid Artery Obstruction Using Laser Speckle Flowgraphy.

PURPOSE: We investigated whether laser speckle flowgraphy (LSFG) results are comparable in both eyes and whether it is useful in the diagnosis of disparity in ocular ischemic syndrome (OIS) patients. METHODS: We compared the mean blur rate (MBR) value for various fundus regions in both eyes of 41 healthy subjects and 15 internal carotid artery occlusion (ICAO) cases. We calculated the standard value of the Laterality Index (LI), which was the MBR comparison of both eyes in each of the regions, in the control subjects. We then investigated the correlation between both eyes for the LIs in the entire fundus, the degree of ICAO and visual function. RESULTS: The disparity of the LIs in both eyes was least in the entire area of the fundus in control subjects and there was a significant correlation between both eyes of the 41 healthy individuals (P = 0.019). Significant correlations were found for the LI, visual acuity and degree of ICAO. The specificity and sensitivity of LI in the entire area was 93.8% and 100%, respectively. CONCLUSIONS: LSFG revealed normal individuals have symmetrical fundus blood flow. LSFG could detect OIS and might be a useful tool for detecting disparities in fundus blood flow.

One-stage bone strip reconstruction technique with balloon sinus dilatation surgery for chronic maxillary atelectasis.

PURPOSE: Chronic maxillary atelectasis is characterized by unilateral spontaneous enophthalmos and hypoglobus due to increased orbital volume secondary to maxillary sinus inward deformation. Reformation of the sinus architecture and reconstruction of the orbit are key to a successful outcome. Here, we introduce a one-staged surgery that addresses both these goals. PATIENTS AND METHODS: We retrospectively reviewed 11 patients treated with one-stage orbital and sinus surgery. A transconjunctival subperiosteal approach was used to create slats in the thinned orbital floor. A nasal endoscopic approach was utilized to access the maxillary sinus and place a modified Foley catheter balloon through the enlarged maxillary ostium. A bridge graft of nasal septal, ear cartilage, or LactSorb was placed on the reconstructed and balloon-supported orbital floor. The balloon was deflated and removed at 10-14 days. All patients underwent complete ophthalmic and orbital evaluation, including standardized photography and radiologic imaging. RESULTS: Eleven patients, mean age 39.5 years, presented with diplopia in upgaze, superior sulcus deformity, and at least 2 mm of relative enophthalmos. After initial overcorrection, enophthalmos improved in all cases. Symmetry within 1 mm was accomplished in 10 of 11 cases. Follow-up time was 259±320 days. Full motility was recovered in all patients. CONCLUSION: We describe a one-staged surgery consisting of cutting slats in the orbital floor, dilating the maxillary sinus with a balloon, and stabilizing the orbital floor with a cartilage graft placement. Our anecdotal experience suggests that this surgical approach can safely achieve normalization of the pathologic sinus outflow and restoration of the orbit anatomy. The balloon ensures orbital floor stability during the healing process, and it may act to stent open the sinus ostium during early mucosal healing.

A Case of Blunt Trauma of the Eyeball Associated With an Inferior Oblique Muscle and an Inferior Rectus Muscle Rupture.

Rupture of the extraocular muscle in the absence of significant injury to the eyeball and adnexa is uncommon. The authors report a case of blunt trauma of the eyeball associated with an inferior oblique muscle and an inferior rectus muscle rupture. A 55-year-old man slipped and fell down hitting his eye on an extended windshield wiper blade. Although he had treatment in the emergency room, he complained of diplopia in the primary position 1 day postoperatively. After noticing ruptures of the inferior oblique muscle and an inferior rectus muscle during exploratory surgery, the authors carefully repaired it. Diplopia in the primary position had disappeared within 1 month after the operation and by 6 months postoperatively. The movement of the eye had almost completely recovered.

Retinal ganglion cell analysis in multiple evanescent white dot syndrome.

BACKGROUND: Multiple evanescent white dot syndrome (MEWDS) is an acute and usually unilateral retinopathy that occurs predominantly in young adults. This report presents the outcomes of ganglion cell analysis (GCA) in MEWDS. CASE PRESENTATION: A 41-year-old woman was diagnosed as MEWDS in right eye. At her initial visit, the deviation map of the ganglion cell analysis showed there was a decrease of the ganglion cell layer (GCL) + inner plexiform layer (IPL) thickness in both eyes, even though her left eye was not affected. A 29-year-old woman was also diagnosed as MEWDS in right eye. Although the deviation map of ganglion cell analysis showed there was a decrease of the GCL + IPL thickness in both eyes at her initial visit, her right eye was not affected. CONCLUSION: GCA indicated there was a decrease (<1% of the distribution of normals) of the ganglion cell layer + inner plexiform layer thickness in both the affected and fellow eyes in 7 of 9 patients diagnosed as MEWDS in our hospital. Although the lesions responsible for MEWDS are thought to disrupt the photoreceptor outer segments, we observed changes in the inner retina in both the affected and fellow eye of MEWDS patients.

Rebamipide ophthalmic suspension for the treatment of dry eye syndrome: a critical appraisal.

Rebamipide was initially developed and approved for use in treating gastric ulcers and lesions associated with gastritis. Discovery of its ability to increase gastric mucin led to investigations of its effect on ocular surface mucin and the subsequent development for use in dry eye patients. Investigations have confirmed that rebamipide increases corneal and conjunctival mucin-like substances along with improving corneal and conjunctival injury. Clinically, rebamipide ophthalmic suspensions can effectively treat tear deficiency and mucin-caused corneal epithelial damage, and can restore the microstructure responsible for tear stability. Topical rebamipide has also been shown to be effective in treating other ocular surface disorders such as lagophthalmos, lid wiper epitheliopathy, and persistent corneal erosion. Rebamipide's ability to modify epithelial cell function, improve tear stability, and suppress inflammation in the absence of any known major side effects suggest that it may be a beneficial first drug of choice for severe dry eye treatment and other ocular surface disorders. This review summarizes the history and development of this innovative dry eye treatment from its initial use as an effective stomach medication to its current use in the treatment of dry eye in Japan.

Animal-type malignant melanoma associated with nevus of Ota in the orbit of a Japanese woman: a case report.

We present a patient with an animal-type malignant melanoma associated with the nevus of Ota in the orbit who showed a good prognosis after a combination of orbital extirpation, chemotherapy, stereotactic radiotherapy, and gamma knife. A 42-year-old Japanese woman presented with two tumors, one pathologically diagnosed as right-sided intraconal animal-type malignant melanoma and the other intracranially, presumed to be of the same pathogenesis and both were considered to have arisen from the nevus of Ota. She underwent an extirpation of the orbit, chemotherapy (DAV therapy, which is a combination of dacarbazine, nimustine, and vincristine), stereotactic radiotherapy (54 Gy in 27 fractions), and gamma knife (marginal dose was 17 Gy, target volume was 0.2 ml). She has been alive for 33 months since the extirpation, with no sign of local recurrence, new metastasis, nor enlargement of the intracranial tumor. Not just combination therapy but also the low malignancy of animal-type melanoma may have contributed toward the good prognosis.

Intravitreal gas injection without vitrectomy for macular detachment associated with an optic disk pit.

PURPOSE: To evaluate the clinical outcomes after gas tamponade without vitrectomy for retinal detachment associated with an optic disk pit using optical coherence tomography. METHODS: Intravitreal gas injection was performed on 8 consecutive patients (mean age, 35.0 years; range, 15-74 years) with unilateral macular detachment associated with an optic disk pit. A 0.3-mL injection of 100% sulfur hexafluoride 6 gas was carried out without an anterior chamber tap. Patients treated with gas injection were instructed to remain facedown for 5 days. RESULTS: Complete retinal reattachment after only gas tamponade was achieved in four out of eight eyes. The mean number of gas injections was 1.8. The mean best-corrected visual acuity before and after the treatment with gas tamponade was approximately 30/100 and 20/20, respectively. The period required for reattachment after final gas treatment was 12 months. There were no incidences of recurrence after complete reattachment by gas tamponade in any of the cases during the 94-month average follow-up period (range, 64-132 months). CONCLUSION: Gas tamponade appears to be an effective alternative method for macular detachment associated with an optic disk pit, even though the mechanisms of optic disk pit maculopathy are still unknown.

Alteration of choroidal thickness in a case of carotid cavernous fistula: a case report and a review of the literature.

BACKGROUND: To measure the alterations of the choroidal thickness in Carotid cavernous fistula (CCF) using enhanced depth imaging optical coherence tomography (EDI-OCT). CASE PRESENTATION: A 64-year-old woman was referred to us for redness, exophthalmos and visual disturbance in her right eye. She was diagnosed with CCF by magnetic resonance imaging (MRI) and magnetic resonance angiography.Observations; Embolization resulted in improvement of ocular symptoms, and there was a reduction of the subfoveal choroidal thickness in the right eye from 351 µm preoperatively to 142 µm postoperatively in EDI-OCT. CONCLUSION: EDI-OCT demonstrated that the choroidal thickness increases occurred due to congestion in a CCF case.

Topical rebamipide improves lid wiper epitheliopathy.

Administration of topical rebamipide increases the mucin level of tear film and improves the ocular surface in short break-up time type of dry eye. Lid wiper epitheliopathy (LWE) is a disorder of the marginal conjunctiva of the upper eyelid with dry eye symptoms. LWE may be related to mechanical forces during blinking resulting in inflammation of the ocular surface. Rebamipide also has various anti-inflammatory effects. In this report, we tried treatment with topical rebamipide for two cases of LWE. One case had been treated with sodium hyaluronate ophthalmic solution and diquafosol sodium eye drops by other doctors for several weeks. The other case was not previously treated. In both cases, fluorescein staining of the cornea and lid margin was remarkably improved, ocular symptoms decreased, and tear film break-up times increased with rebamipide eye drops four times daily for 2-3 weeks. Topical rebamipide was effective for corneal and conjunctival disorders in LWE. This drug may provide a novel approach to the treatment of LWE.

Topical rebamipide improves the ocular surface in mild lagophthalmos.

Administration of topical rebamipide improves the ocular surface in dry eye. We consecutively studied seven eyes in seven cases (three males and four females) with mild lagophthalmos (three cases after eyelid surgery, two cases of incomplete facial nerve palsy, and two cases of senile ectropion) during the treatment of corneal disorders with rebamipide eye drops four times daily for 2 weeks. The fluorescein corneal staining (FCS) score, tear film break-up time (TBUT), Schirmer's test, and decimal visual acuity were examined. Ocular symptoms were examined and scored by questioning each patient before and after administration of the drug. In all seven eyes, inferior corneal erosion decreased or disappeared within 2 weeks after administration of topical rebamipide. The FCS score was significantly improved (P < 0.05). The TBUT was significantly extended from 2.9 ± 0.5 seconds to 5.2 ± 0.4 seconds (P < 0.05). The scores of ocular symptoms, such as eye pain, dryness, blurred vision, and foreign body sensations, were significantly improved (P < 0.05).Topical rebamipide was effective for corneal disorders in mild lagophthalmos. This drug may provide a novel approach to treat lagophthalmos.

Resolution of persistent corneal erosion after administration of topical rebamipide.

Rebamipide is an antiulcer agent used to treat gastric ulcer and gastritis. Biological effects of rebamipide include cytoprotection, wound healing, and anti-inflammatory properties that are known to be universal for a variety of tissues in addition to gastrointestinal mucosa. The therapeutic effects of rebamipide eye drops are due to its ability to increase corneal and conjunctival mucin-like substances and improve corneal and conjunctival injury in vivo. In this paper, we report a case of Sjögren's syndrome with complete disappearance of corneal erosion after administration of rebamipide eye drops. This was observed even though corneal erosion had not improved for 6 months after punctal occlusion surgery. The patient was a 33-year-old female, diagnosed with Sjögren's syndrome by a salivary gland biopsy. The corneal and conjunctival surfaces were filled with dense erosions, which did not improve with topical drugs. Punctal plugs were applied several times; however, the plugs were repeatedly shed. All four puncta of both eyelids were surgically occluded, and both corneal and conjunctival erosion was clearly improved. However, the erosion in the inferior cornea of both eyes had not improved for 6 months after surgery. We used the newly approved topical rebamipide for treatment of this patient. The corneal erosion gradually improved and completely disappeared 4 weeks after administration of the drug. Dry eye sensation disappeared at the same time. Both membrane-associated mucin and secreted mucin in the ocular surface are thought to be essential for maintenance of the tear film. Induction of mucin from ocular surface epithelium could be an effective treatment in cases of dry eye caused by mucin deficiency. Through its various mechanisms, rebamipide improves ocular surface conditions. To our knowledge, this is the first clinical case report using rebamipide ophthalmic solution. This drug may provide a novel approach to treat drying diseases of the eye.

Longitudinal tear of the inferior rectus muscle in orbital floor fracture.

We report a case of longitudinal avulsion of the inferior rectus muscle following orbital floor fracture and describe its clinical presentation, computed tomography (CT) features and management. A 53-year-old man felt vertical diplopia in all gaze immediately after the trauma. Orthoptic assessment showed left over right hypertropia of 20 prism diopters and left exotropia of 10 prism diopters in primary position. The left orbital floor fracture and the prolapse of orbital contents into the maxillary sinus were presented by CT. Exploration of the orbit was performed under general anesthesia. The displaced bone fragment was elevated and repositioned below the slastic implant. Diplopia continued in all directions of gaze, although the impairment of depression was reduced postoperatively. A residual left hypertropia of 10 prism diopters and exotropia of 10 prism diopters was present in primary position 1 month after surgery, though there were no enopthalmos or worsening of hypesthesia. Repeated CT revealed the muscle avulsion of inferior rectus at the lateral portion of the belly. The avulsion of a small segment of the inferior rectus and its herniation into maxillary sinus in more posterior views was detected by review of the preoperative images. Muscle avulsion should be considered in the management of orbital fracture if orbital tissue entrapment and nerve paresis are excluded as causes of reduction in ocular motility. A thorough review of the imaging studies for possible muscle injury is required before surgery in all cases of orbital fracture.

Asymmetric severity of diabetic retinopathy in Waardenburg syndrome.

A 30-year-old female patient was referred to our institution due to vitreous hemorrhage. Best corrected visual acuity of her right and left eyes at her initial visit was 10/20 and 20/20, respectively. Although hypochromic iris was observed in the superior iris between the 10 and 2 o'clock positions in her right eye, her entire left eye exhibited hypochromic iris. Hypopigmentation of the fundus was seen in the superior part of her right eye. This eye also had a huge neovascularization on the optic disc that was 7 discs in diameter. Conversely, her left fundi showed hypopigmentation of the fundus in the entire region of the left eye, and dot hemorrhages were observed all over the left fundi, although no neovascularization could be seen microscopically. Fluorescein angiography showed a huge neovascularization in the right eye and a tiny neovascularization in the left eye. Gene analysis revealed the presence of the PAX3 gene homeobox domain mutation, which led to her being diagnosed as Waardenburg syndrome type 1. Magnetic resonance angiography showed there was no obstructive region at either of the internal carotid arteries and ophthalmic arteries. The severity of the diabetic retinopathy appeared to be correlated with the degree of hypopigmentation in the posterior fundus. We speculate that hypopigmentation of the fundus in Waardenburg syndrome may be responsible for the reduction in retinal metabolism, which led to a reduction in oxygen consumption and prevented further aggravation of the diabetic retinopathy. Only laser treatments using short wavelengths was effective in this case. While the extinction coefficient for hemoglobin when using green light is higher than when using yellow light, the differences between these wavelengths tend to disappear when oxygenated hemoglobin is present. To the best of the authors' knowledge, this is the first case report of a patient with Waardenburg syndrome and diabetic retinopathy.

Improved subjective symptoms of conjunctivochalasis using bipolar diathermy method for conjunctival shrinkage.

PURPOSE: To evaluate the improvement in subjective symptoms of conjunctivochalasis after bipolar coagulation. METHODS: Forty-three eyes of 26 patients (average age, 75.7 ± 8.4 years) were included in this study. The inferior conjunctivas were cauterized between April 2009 and June 2010. Surgery involved pinching the excess conjunctiva and performing bipolar cauterization after subconjunctival injection of a local anesthetic agent. Patients were asked to describe the postoperative foreign-body sensation and change in subjective symptoms 1 month postoperatively, with the preoperative symptom score defined as 10. RESULTS: Twenty-two patients (84.6%) reported symptom relief immediately after the procedure, though all patients had a mild gritty sensation for 1-2 weeks postoperatively. The mean postoperative symptom score obtained from the questionnaire was 3.27 ± 3.31, which was significantly lower than the preoperative score (P < 0.0001). No patients had experienced recurrent symptoms at the end of the follow-up period. CONCLUSION: Thermal cauterization can achieve conjunctival shrinkage and adherence to the subconjunctival tissues. Operation time is only a few minutes, postoperative pain is not severe, and the procedure can be performed in an outpatient clinic, all of which represent benefits to the patients.