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.

Three cases of macular hole that occurred in inferior scleral staphyloma associated with tilted disc syndrome: a case series.

BACKGROUND: The objective is to examine the clinical characteristics of three patients with macular hole that occurred in inferior posterior staphyloma associated with tilted disc syndrome. CASE PRESENTATIONS: This study involved three eyes of three Japanese female patients (mean age 76.0 years, range 73-84 years) with macular hole occurring in inferior posterior staphyloma associated with tilted disc syndrome. One of the three eyes was slightly myopic, while the other two eyes were highly myopic. In all three eyes, the macular hole was found to be located in or near the margin of the inferior posterior staphyloma. In one eye, the extent of retinoschisis was rather broad in the margin of the macular hole, and another eye had a history of treatment for choroidal neovascularization. As surgical treatment, the internal limiting membrane in areas surrounding the macular hole was detached after producing artificial posterior vitreous detachment, and a gas tamponade was performed. It was found during surgery that the extensibility of the retina in the margin of the MH was decreased in the three eyes as compared with a usual macular hole. Although the macular hole was successfully closed in all three cases post surgery, the layer structure of the central retina was poorly repaired in all three cases and choroidal neovascularization remained in one case. In all three cases, corrected visual acuity remained at 0.3-0.5 post surgery. CONCLUSIONS: Our findings showed poor improvement of visual acuity in all three cases post surgery, even if closure of the macular hole is achieved, thus suggesting that in cases of macular hole associated with tilted disc syndrome and inferior posterior staphyloma, the presence of macular dysfunction existing prior to the onset of macular hole affects visual prognosis.

A randomized clinical trial of triamcinolone acetonide injection for suppression of inflammation after blepharoptosis surgery.

This study aimed to determine the effectiveness of triamcinolone acetonide in suppressing inflammation after blepharoptosis surgery. The study was designed as prospective, randomized, two medical centers' clinical trial. Thirty-two patients with involutional blepharoptosis of the same degree in both eyelids underwent bilateral transcutaneous levator advancement. At the end of the surgery, 4 mg/0.1 ml of triamcinolone acetonide was injected into a randomly selected upper eyelid. The fellow eyelid was not injected and was used as control. Facial photographs were taken on day 1, week 1, month 1, and month 3, and the degree of inflammation, the margin reflex distance 1 (MRD-1), and levator function (LF) between the two eyelids of each patient were compared. The primary outcome was the selection of the less inflamed eyelid decided by the majority of three individuals unrelated to the study. MRD-1 and LF were analyzed for secondary outcomes. As a result, the injected eyelid was judged to be the less inflamed eyelid in all cases. The MRD-1 in the postoperative period less than 1 month was significantly larger in the injected eyelids than the control eyelids (P<0.03). The postsurgical MRD-1 at month 3, the postsurgical LF at all postsurgical examination times were not statistically different. Adverse complications by the injection, including ptosis, levator dysfunction, increase of the intraocular pressure, and visual disturbance were not observed. In conclusion, a triamcinolone acetonide injection after ptosis surgery is both safe and effective in reducing the early postsurgical inflammation and helpful in an earlier return to a daily routine for the patients.

Chronologic Analysis of Tear Dynamics on Blinking Using Quantitative Manometry in Healthy Humans.

PURPOSE: To analyze the tear dynamics during blinking by measuring the inner pressure of the upper lacrimal drainage system. METHODS: This observational study involved 11 healthy bi- or tricenarian volunteers. Direct manometry was performed using a fiber optic pressure sensor inserted into the conjunctival sac, upper/lower canaliculus (5 mm from punctum), and inferior lacrimal sac (15 mm from punctum) during both involuntary and intentional tight blinking. Pressure was measured 200 times/second during 3 separate blinks and then chronologically analyzed. RESULTS: In all subjects of all locations during both types of blinking, the inner pressures during the stationary eyelid closing/opening were positive/approximately zero, while a positive/negative pressure spike was observed during the eyelid closing/opening movement. The averages of the maximum pressure in the spike during the intentional tight blinking (tPmax: mm Hg) in the conjunctival sac, upper/lower canaliculus, and lacrimal sac were 8.00, 12.39/12.93, and 10.59, respectively, while for the minimum (tPmin: mm Hg), the pressures were -3.18, -3.91/-3.43, and -3.31, respectively. The tPmax and tPmin in the lacrimal duct were positively correlated with that of the conjunctival sac, which suggested synchronism of the drainage system. However, the tPmax in the canaliculus was significantly higher than that of the conjunctival sac, which suggested that tears do not flow from the conjunctival sac into the lacrimal duct during eyelid closure. CONCLUSIONS: The upper lacrimal drainage system functions as one united lumen in the lacrimal pump. The positive /negative pressure spike is essential for the lacrimal pump to efficiently eject/aspirate the tear from the lacrimal/conjunctival sac.

Adjustable Medial Epicanthoplasty Using a Rotational Flap for Epiblepharon Repair.

ABSTRACT: This report aimed to introduce the new adjustable rotational skin flap for epicanthoplasty in combination with traditional epiblepharon repair by the modified Hotz procedure. This retrospective study involved 25 consecutive patients with superficial punctate keratitis secondary to epiblepharon complicated with epicanthal fold who underwent the combined surgery between 2019 and 2020. The mean patient age was 11.4 years in this study with a median follow-up was 8months (range, 3-12months). The rationale of the surgery was to release vertical tension of the eyelids by dissecting dense connective tissue beneath the epicanthal fold and to form a new medial canthus using a rotational skin flap supplied by the redundant the upper and/or lower eyelid skin excised during the epiblepharon repair. Postsurgical resolution ofsuperficial punctate keratitis and patient satisfaction was achieved in all patients. Additionally, there were no complications, and no revisional surgery was required in all patients for a median 8 months follow-up period. Utilizing a rotational skin flap during epiblepharon repair is a useful adjunct during epicanthoplasty surgery. This modification is well tolerated and allows for intraoperative adjustment, whereas minimizing scarring and allowing for improved tissue relaxation.

Impact of habitual swimming on the success of lacrimal surgery.

PURPOSE: To elucidate how factors associated with swimming affect the lacrimal ducts of swimmers. STUDY DESIGN: Prospective, interventional cohort study, METHODS: Five hundred seventy four consecutive epiphora patients were surveyed via a questionnaire regarding details of their swimming-pool usage; i.e., frequency, swim-goggles' wear, and type of pool activity (i.e., swimming vs. waist-depth walking). In this cohort, all the swimmers over 20 years old with anatomical lacrimal duct obstruction underwent surgical reconstruction. The surgical success rates at 12-months postoperative were compared using multivariable logistic regression analyses between swimmer/non-swimmer patients, those with a history of high/low frequency of pool usage, and those with high/low amount of conjunctival contact with the swimming-pool water. RESULTS: Of the patients with anatomical lacrimal duct obstruction, 6.4% were habitual swimmers; nasolacrimal duct obstruction was more common in the swimmers than in the non-swimmers' controls (89.1%/66.7%, P = 0.025). The success rate of lacrimal surgery for the swimmers with anatomical nasolacrimal duct obstruction was lower (60.6%) than of the non-swimmers (83.3%, P = 0.048). A receiver operating characteristic curve analysis of the frequency for the prediction of surgical failure showed that the threshold was 4 days/week. The success rate was statistically lower (26.7%) in the high-frequency swimmers compared to the low-frequency swimmers (88.9%, P = 0.037). However, no statistical difference in the high/low ocular surface contact to the swimming-pool water was observed (71.4%/57.7%, P = 0.56). CONCLUSION: Habitual swimmers have a high risk of nasolacrimal-duct damage retrogradely from the nasal cavity that lowers lacrimal surgery's success rate.

Compressive optic neuropathy (CON) in Graves' disease caused by hypertrophy of levator and superior rectus muscles: A case report.

RATIONALE: Enlargemento of the medial rectus is the most predominant factor of compressive optic neuropathy (CON) in Graves' disease. This case report indicates that CON could develop only from the hypertrophic superior levator and superior rectus (SL/SR) muscle in a patient with poorly controlled Graves' disease, and described the possible risk of FT3-thyrotoxicosis with a prominent goiter to develop the current rare case with a review of the literature. PATIENT CONCERNS: A 66-year-old woman undergoing endocrine management of hyperthyroidism with prominent goiter visited the Department of Ophthalmology due to right-eye upper-eyelid retraction. DIAGNOSES: At initial presentation, the right and left margin reflex distance-1 (MRD-1) was 3.2 mm and 2.1 mm, respectively, and no proptosis or visual dysfunction was observed. Despite insufficient hormonal regulation, she refused to undergo goiter removal. The upper eyelid retraction gradually worsened to 7.7 mm of MRD-1, followed by the onset of 20 prism diopters (PD) of the right hypertropia, resulting in right-eye CON after 6 months. Her free thyroxin level was 3.88 ng/dl and free triiodothyronine was 24.90 pg/ml. Computed tomography and magnetic resonance imaging showed only SL/SR enlargement in the right orbit. INTERVENTIONS: Intravenous steroid and radiation therapy resulted in visual improvement; however, a prominent upper eyelid retraction and 35PD of hypertropia remained in her right eye. Orbital decompression, upper retraction repair, and superior rectus recession were performed to prevent the recurrence of CON and correct any disfigurement. OUTCOMES: The combination of conventional intravenous steroid pulse therapy, radiotherapy, and orbital decompression was effective, and no recurrence was observed for more than 1.5-years postoperatively. LESSONS: Enlargement of the SL/SR muscle complex may independently induce the CON. We believe that strict attention should be paid to patients with triiodothyronine thyrotoxicosis with progressive eyelid retraction and hypertropia.

Dacryoendoscopy-guided re-canalization of canaliculops: Two case reports.

RATIONALE: Canaliculops is a rare condition, and only 11 cases have been reported previously. We report 2 cases of canaliculops, which were successfully treated using the new recanalization technique under dacryoendoscopy followed by bicanalicular lacrimal intubation. PATIENT CONCERNS: A 78-year-old man and a 76-year-old woman had 3- and 1-year histories of medial-upper eyelid swelling (left and right, respectively) without any inflammatory signs, history of periocular trauma, herpes infection, use of specific drugs, or ophthalmic diseases of note. DIAGNOSES: The cystic lesions were evaluated using ultrasound biometry or computed tomography to find the lumen of the horizontal canaliculus was exceedingly expanded, and to confirm the clinical diagnosis of canaliculops. INTERVENTIONS: As the 2 cases of canaliculops were caused by upper puncta and common canaliculus obstructions, canaliculops of the upper eyelid were recanalized under dacryoendoscopic guidance, followed by bicanalicular intubation. The tubes were kept in situ involving bi-weekly irrigation and instillation of antibiotic and anti-inflammatory eye drops, and were removed after 2 to 3 months of follow-up. OUTCOMES: Epiphora, and eyelid swelling were completely resolved immediately after the procedure, and the lesions did not recur on follow-up after more than 6 months. LESSONS: Eleven case series of canaliculops have been described previously, but this is the first report of this recanalization procedure offering a new, less invasive treatment option compared to complete or partial resection of the cystic lesion.

Rituximab Monotherapy for Compressive Optic Neuropathy With Giant Ocular Adnexal Mucosa-Associated Lymphoid Tissue lymphoma.

A rare case of compressive optic neuropathy due to giant mucosa-associated lymphoid tissue lymphoma in the orbit was presented. A 87-year-old woman was aware of a slow progressive left ocular proptosis for 10 years and presented after becoming aware of a sudden progression of the proptosis accompanying visual disturbance over the previous 2 months. Orbital imaging and a biopsy of the tumor revealed a mucosa-associated lymphoid tissue lymphoma occupying her left orbit compressing and stretching the left optic nerve. Considering her age and the additional adverse effects of external beam radiation therapy to her damaged optic nerve, rituximab monotherapy was utilized. The intervention resulted in almost complete regression without any serious adverse effect, with left eye best-corrected visual acuity improving from 12/200 to 16/20. Rituximab monotherapy can be one of the first-choice treatment options for mucosa-associated lymphoid tissue lymphoma, especially in cases with the critical damage in the optic nerve.

Type III uveal effusion syndrome suspected to be related to pachychoroid spectrum disease: A case report.

INTRODUCTION: We report a case of type III uveal effusion syndrome (UES) suspected to be related to pachychoroid spectrum disease. PATIENT CONCERNS: A 42-year-old man became aware of visual field constriction and deterioration of visual acuity in his right eye. DIAGNOSIS: Upon examination, a bullous non-rhegmatogenous retinal detachment was observed in the inferior 2 quadrants of the right eye fundus, and the subretinal fluid moved with postural changes. The axial length in that eye was 22.36 mm, thus indicating no nanophthalmia. Preoperative indocyanine green angiography revealed dilated choroidal vessels in the posterior pole of the right eye and mild leakage in the late phase. Optical coherence tomography examination revealed choroidal thickening in both eyes. INTERVENTIONS: For treatment, we first performed sclerotomy, and the intraoperative findings showed no thickening of the sclera. Following surgery, reattachment of the retina was not achieved. OUTCOMES: Thus, we next performed vitrectomy, which led to successful reattachment of the retina. LESSONS: In this case, we theorize that pachychoroid spectrum disease might have been involved in the pathogenesis of type III UES.

Concurrent vitrectomy for persistent pupillary membrane complicated by severe myopia and vitreomacular traction syndrome: A case report.

INTRODUCTION: In cases of persistent pupillary membrane (PPM), the eye is usually slightly microphthalmic and emmetropia or hyperopia is often present, yet severe myopia is reportedly rare. Here we presented a case of PPM complicated by vitreomacular traction syndrome and posterior staphyloma due to severe myopia. PATIENT CONCERNS: This study involved a 63-year-old female patient who had been diagnosed with bilateral PPM at a local eye clinic and who was subsequently referred to our department for a more detailed examination due to a recent decrease of visual acuity. DIAGNOSES: Slit-lamp microscopy examination revealed bilateral PPM. The ocular fundus revealed peripapillary conus and myopic change in both eyes. Optical coherence tomography examination revealed no particular abnormalities in the right eye, yet did show findings indicative of vitreomacular traction syndrome in the left eye. INTERVENTIONS: In both eyes, we performed surgical removal of the PPM, phacoemulsification aspiration, and intraocular lens implantation, yet in the patient's left eye, vitrectomy was also performed. OUTCOMES: After surgery, the patient's visual acuity improved in both eyes. CONCLUSION: The findings in this case show that when required, vitrectomy should be considered based upon the preoperative Optical coherence tomography findings for PPM.

Two cases of diabetic macular edema complicated by an atypical macular hole.

BACKGROUND: Here we report two patients who developed an atypical macular hole (MH) during the treatment course for diabetic macular edema (DME). CASE PRESENTATIONS: Patient 1 was a 73-year-old male. Optical coherence tomography (OCT) revealed perifoveal retinoschisis (RS) in addition to cystoid macular edema and serous retinal detachment (SRD) in his left eye, and that an MH had developed during the clinical course. A convex surface was formed at the MH margin toward the vitreous cavity, and granular shadows were observed in the fluid cuff. Intraoperative findings revealed a thin epiretinal macular membrane (ERM) around the MH. Patient 2 was a 79-year-old male. Although the patient underwent pars plana vitrectomy (PPV) for proliferative diabetic retinopathy (PDR) in both eyes, RS and a thin ERM in addition to SRD was observed in his left eye after surgery, and an MH developed during the clinical course. As in Patient 1, a convex surface was formed at the fluid cuff margin toward the vitreous cavity. CONCLUSIONS: Both patients had persistent DME, SRD, RS, and a thin ERM before the development of the MH. OCT revealed the formation of a convex surface at the MH margin toward the vitreous cavity, suggesting that the fragility of the layered structure of the retina combined with tangential retinal traction may have been involved in the atypical MH form.

Newly Designed Universal Trans-punctal Rigid Lacrimal Microendoscope.

PURPOSE: To report the development and clinical findings of a universal trans-punctal lacrimal microendoscope design. METHODS: In this study, we examined a unique and original "2-bend" (i.e., double-angle) rigid lacrimal microendoscope designed for universal applications for anatomical variations. The shape of endoscope was initially evaluated in 6 lacrimal systems of cadavers (4 Caucasian-cadaver lacrimal systems and 2 Asian-cadaver lacrimal systems). Second, a prospective clinical study involving 45 consecutive cases of nasolacrimal duct obstruction in Japanese patients was conducted to compare the facility and ease of use between the conventional single-bend type and our original 2-bend type trans-punctal lacrimal microendoscope for examination of the lacrimal drainage system. RESULTS: The findings in the cadaver study revealed that original 2-bend lacrimal microendoscope, which was designed double-angled 20°-30° at 10 and 30 mm from the tip of the scope, could more easily used to insert and image all lacrimal systems, while the conventional single-bent lacrimal microendoscope was difficult to insert in subset of patients with a prominent nasal process of the frontal bone. In the clinical trial, our findings showed that 26.7% of lacrimal systems could not be passed using the conventional single-bend design, while using the 2-bend design, all cases could successfully be investigated. CONCLUSIONS: The original 2-bend-design microendoscope was found to be effective and valuable for universal use in examination and evaluation of the lacrimal passage.

A Case of Giant Pituitary Adenoma Associated with a Postoperative Mental Disorder That Ultimately Resulted in Bilateral Blindness.

PURPOSE: To report the case of a patient with adrenocorticotropic hormone (ACTH)-producing pituitary adenoma who developed a mental disorder after initial surgery that kept him from undergoing scheduled follow-up visits and who ultimately had a giant recurrent tumor that resulted in blindness. CASE REPORT: A 37-year-old male presented with the primary complaint of decreased visual acuity (VA) in both eyes and visual field defects. Visual field examination revealed bitemporal hemianopia. Magnetic resonance imaging (MRI) showed a pituitary tumor of approximately 4 cm in diameter extending from the intrasellar region to the sphenoid sinus and the suprasellar region. Transnasal transsphenoidal surgery was performed. Immunostaining of tumor tissue collected intraoperatively showed ACTH-positive cells, thus leading to the diagnosis of ACTH-producing pituitary adenoma. Postoperatively, the patient reportedly developed mental disorder that possibly interfered with scheduled appointments or continuous follow-up visits for many years, so we had no postoperative data about the vision/visual filed. Seven years later, he presented with markedly decreased VA (i.e., no light perception) in both eyes. Fundus examination showed bilateral marked optic disc atrophy. MRI showed a larger than 8-cm diameter giant recurrent pituitary adenoma in the suprasellar region, for which craniotomy was performed for partial tumor resection. Preoperatively, his blood cortisol level was low, and the lesion was deemed a nonfunctioning pituitary adenoma. Postoperatively, no significant complications occurred, yet his VA was no light perception OD and light perception OS. CONCLUSIONS: Clinicians should be aware that patients with ACTH-producing pituitary adenomas may develop a mental disorder following surgery and possibly be unable to undergo scheduled follow-up, thus illustrating the importance of establishing an adequate patient follow-up system.

Analysis of Periorbital Soft Tissue in Thyroid Eye Disease.

PURPOSE: To investigate periorbital tissue enlargement in thyroid eye disease (TED) by 3-dimensional CT volumetric analysis. METHODS: Twenty-four adult subjects, 16 with TED and 8 controls, were studied. Three-dimensional volumetric calculations were performed on CT imaging of the orbit and face, focusing on the retroorbicularis oculi fat, suborbicularis oculi fat, facial muscles in periorbital region, orbital fat, extraocular muscles, and orbital volume. Analysis was performed using JMP version 12 software. Each measure was compared between the TED and control groups using Wilcoxon rank sum test. Correlations were investigated between periorbital and orbital tissue using the Spearman's correlation coefficient method. RESULTS: A statistically significant increase in volume was measured in TED patients in the superior and inferior periorbital fat tissue (p = 0.0044, p = 0.047), including the retroorbicularis oculi fat (p = 0.0011), suborbicularis oculi fat (p = 0.0093), and a decrease in facial muscle of superior periorbital region (p = 0.035). Strong positive correlation was noted between the muscles of superior and inferior periorbital region (rs = 0.65; p = 0.0006), and between the suborbicularis oculi fat and retroorbicularis oculi fat (rs = 0.50; p = 0.013). No correlation was observed between orbital and periorbital tissue, except between the orbital fat and the inferior periorbital fat tissue (p = 0.047). CONCLUSIONS: Facial fat in the periorbital region is enlarged in TED, with the superior component correlating with orbital fat tissue expansion. These findings may assist in the clinical evaluation and management of disfigurement in TED patients.Superior and inferior periorbital fat is enlarged in thyroid eye disease with the superior periorbital fat expansion correlating with orbital fat expansion.

Endoscopic Evaluation of Lacrimal Mucosa With Indigo Carmine Stain.

PURPOSE: Histological investigation in the use of indigo carmine for detection of mucosal pathology in lacrimal microendoscopy. To investigate the histopathological correlation of staining found on lacrimal microendoscopy. METHODS: A prospective case series was conducted in patients with a history of nasolacrimal duct obstruction and dacryocystitis requiring treatment with dacryocystorhinostomy or dacryocystectomy. Prior to lacrimal surgery, all patients underwent endolacrimal evaluation using transpunctal lacrimal microendoscopy. Indigo carmine dye (0.2 mg/0.5 ml) was injected into the lacrimal system via the irrigation channel of the endoscope, and after copious irrigation, the differential mucosal staining was recorded. Histopathologic analysis of tissue samples of positively and negatively stained lacrimal sac mucosa collected after surgery was performed to investigate the correlation with the results of each endoscopic evaluation. RESULTS: Four patients underwent dacryocystorhinostomy and 2 patients underwent dacryocystectomy. Histopathology of positive-stained lacrimal sac mucosa corresponded to areas of the advanced fibrous stage of mucosal changes resulting from dacryocystitis, which showed atrophy of epithelium associated with the loss of goblet cells and subepithelium fibrous scarring. In contrast, areas of lacrimal mucosa that did not stain with indigo carmine were still in an active inflammatory stage of dacryocystitis preserving columnar epithelium with goblet cells. CONCLUSIONS: This study suggests that indigo carmine staining is a useful adjunct in lacrimal microendoscopy for accurately detecting areas of highly damaged fibrous lacrimal mucosa, as it allows for direct visualization of lacrimal mucosal pathology and can provide prognostic insight and guidance in regard to treatment options for patients with lacrimal drainage obstruction.Use of indigo carmine dye as an in vivo adjunct in lacrimal microendoscopy is useful for accurately detecting areas of highly damaged lacrimal mucosa.

Investigation of scleral thermal injuries caused by ultrasonic pars plana phacoemulsification and aspiration using pig eyes.

PURPOSE: The purpose of this study was to investigate the thermal injuries caused by ultrasonic pars plana phacoemulsification and aspiration (PPPEA) using pig eyes. METHOD: Using a 20-gauge (G) vitrectomy system (Accurus®, Fragmatome; Alcon Laboratories) in both the 'open-tip' and 'closed-tip' techniques, PPPEA was performed in pig eyes and the subsequent thermal injuries generated around the scleral wound were measured by infrared thermal imaging (thermography). Post surgery, the state of the scleral wound was observed under a microscope, and a tissue slice containing the scleral wound was then prepared and observed under an optical microscope. RESULTS: Thermography measurements revealed a slight temperature rise around the scleral wound in the open-tip case, yet a marked temperature rise in the closed-tip case. The scleral wound incision produced by the open tip was linear, while that produced by the closed tip was expanded. Histological examination revealed mild degeneration of the sclera around the wound in the open-tip case, yet marked tissue degeneration by thermal injuries in the closed-tip case. CONCLUSION: Our findings showed that in PPPEA, the temperature of the tip of a 20G vitrectomy system rapidly increases due to the closed-tip technique, thus producing obvious thermal damage to the scleral wound. In order to prevent thermal injuries to the scleral wound during PPPEA, it is important to shorten the time of ultrasonic oscillation during surgery as much as possible while the tip is occluded with nuclear fragments.

Case Report of a Family Affected by Stickler Syndrome in Which Rhegmatogenous Retinal Detachment Occurred in Five Eyes of Three Siblings.

PURPOSE: Stickler syndrome is an autosomal dominant inherited disorder that is well known to be highly associated with the development of rhegmatogenous retinal detachment. In this study, we report the case of a family affected by Stickler syndrome in which rhegmatogenous retinal detachment developed in 5 eyes of 3 siblings. CASE SERIES: For treatment, we performed vitreous surgery on 1 eye of the eldest son, and bilateral scleral buckling surgery on the 2 younger children. A good postoperative outcome was obtained on the 4 eyes that underwent scleral buckling surgery, yet the prognosis was poor on the 1 eye that underwent vitrectomy due to redetachment of the retina and corneal complication. Since vitreous surgery was quite difficult due to strong vitreoretinal adhesion, we created an artificial posterior vitreous detachment via the bimanual technique combined with encircling. For the scleral buckling surgery, broad scleral extrusion was needed to seal multiple retinal breaks. CONCLUSIONS: The findings of this study showed a high prevalence of rhegmatogenous retinal detachment in a single family with Stickler syndrome. In these cases, scleral buckling surgery was effective for treating the detached retina, and some prophylactic treatment, such as laser photocoagulation to prevent the occurrence of rhegmatogenous retinal detachment, should be considered for such cases in the future.

Orbital fat regeneration following hormonal treatment of metastatic breast carcinoma.

Enophthalmos in the setting of breast cancer metastatic to the orbit results primarily from the disease pathogenesis, or secondary to treatment effects. Orbital volume restoration and fat regeneration following endocrine treatment monotherapy has not been previously reported. A 76- year-old previously healthy female presented with progressive right enophthalmos secondary to metastatic lobular breast carcinoma. Treatment with an aromatase inhibitor (letrozole) resulted in tumor regression and orbital fat restoration with a corresponding improvement in orbital volume and enophthalmos on clinical exam. The patient is alive on continued letrozole with no progressive disease ten years after diagnosis. This case illustrates the resilience of orbital soft tissues and ability of orbital fat to regenerate in face of breast cancer metastasis. We hypothesize that endocrine monotherapy, and avoidance of radiation therapy, allowed for differentiation of remaining orbital stem cells, and facilitated the fat regenerative process.

P7C3 Suppresses Neuroinflammation and Protects Retinal Ganglion Cells of Rats from Optic Nerve Crush.

Purpose: To determine whether P7C3-A20 can inhibit the phosphorylation of the mammalian target of rapamycin (mTOR), depress neuroinflammation, and protect retinal ganglion cells (RGCs) of rats from optic nerve crush (ONC). Methods: The left optic nerve was crushed, and 5.0 mg/kg/d of P7C3-A20, 1.0 mg/kg/d of rapamycin, or their vehicle was injected intraperitoneally for 3 consecutive days beginning 1 day before the ONC. The protective effects on the RGCs were determined by immunohistochemical staining for Tuj-1. The level of phosphorylated mTOR was determined by immunoblotting. The neuroinflammation in the optic nerve was determined by changes in the expression of CD68, TNF-α, MCP-1, and iNOS. Results: The density of Tuj-1-stained cells in the control was 2010 ± 81.5/mm2 and 1842 ± 80.4/mm2 on days 7 and 14 after the sham operation. These levels were lower at 995 ± 122/mm2 and 450 ± 52.4/mm2 on days 7 and 14 after the ONC, respectively. Rapamycin and P7C3-A20 preserved the density at significantly higher levels on both days (P < 0.05, Scheffe test). The level of phosphorylated mTOR increased by 1.56-fold above the control level on day 7. Rapamycin and P7C3 significantly lowered the level of phosphorylated mTOR to 0.89-fold and 0.67-fold of the control, respectively. There was an accumulation of CD68+ cells that were immunoreactive to TNF-α at the crush site. The expression of MCP-1 and iNOS was increased chiefly in the astrocytes around the lesion. These inflammatory events were suppressed by both rapamycin and P7C3. Conclusions: P7C3-A20 can inhibit mTOR phosphorylation in the crushed optic nerve, which may suppress neuroinflammation and preserve the RGCs.