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.

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.

Convergent strabismus fixus after bilateral abducens nerve palsies due to aneurysms: A case report.

RATIONALE: Convergent strabismus fixus is an ocular motor abnormality in which the eye is fixed in adduction. This condition is mostly associated with high myopia and is caused by a displacement of the extraocular muscles. We report a nonmyopic woman with convergent strabismus fixus due to aneurysms. PATIENT CONCERNS: A 79-year-old woman complained of progressive worsening of esotropia about 50 years prior to her visit. Neuroimaging showed that the eye was not dislocated, and the extraocular muscles were not displaced. However, aneurysms were found bilaterally from the intracavernous carotid arteries and the location was on both abducens nerves. DIAGNOSES: Chronic bilateral abducens nerve palsies due to aneurysms. INTERVENTIONS: Endovascular treatment was successfully performed for the aneurysms. OUTCOMES: Convergent strabismus fixus still remained. LESSONS: Chronic abducens nerve palsies may develop to nonmyopic convergent strabismus fixus without displacement of extraocular muscles, and mass lesions in the brain including aneurysms should be ruled out when orbital MRI cannot explain the condition.

Ocular findings in Japanese children with Down syndrome: the course of visual acuity and refraction, and systemic and ocular anomalies.

PURPOSE: To investigate the age-related development of refractive errors and changes of visual acuity (VA), and the systemic and ocular anomalies in Japanese children and young adults with Down syndrome (DS). DESIGN: Retrospective cohort study. SUBJECTS AND METHODS: This study involved 222 Japanese children and young adults with DS (age range: 3 months to 19 years) seen at the Department of Ophthalmology, Shiga Medical Center for Children, Shiga, Japan. The subjects were divided into the following six age groups: 1) infant (age 0 to <4 years), 2) preschool (age 4 to <7 years), 3) lower primary-school grades (age 7 to <10 years), 4) upper primary-school grades (age 10 to <13 years), 5) junior high school (age 13 to <16 years), and 6) late teen/young adults (age 16 to <20 years). Through examination of the subjects' medical charts, we investigated the development and changes of refractive errors and VA, best-corrected VA (BCVA), and systemic and ocular anomalies. RESULTS: For vision testing, Teller Acuity Cards™ (Bernell Corporation) were used for the infants, and the Landolt ring was used for the school-age children. VA was found to develop with age. Mean BCVA was 0.19±0.17 logarithm of the minimum angle of resolution (mean age: 11.3±3.2 years). Mean of refractive errors was hyperopia in the infant (2.2±2.4 diopters [D] OD, 2.4±2.5D OS), yet became myopia to the junior high school (-0.3±4.4D OD, -0.2±4.4D OS). CONCLUSION: Our findings revealed that in children and in late-teen and young-adult subjects with DS, VA slowly develops and that refractive errors requiring correction exist and are difficult to examine.

Incidence of side effects of topical atropine sulfate and cyclopentolate hydrochloride for cycloplegia in Japanese children: a multicenter study.

PURPOSE: To investigate the incidence rate and side effects of topical atropine sulfate and cyclopentolate hydrochloride for cycloplegia in children aged 15 years or under. METHODS: This prospective study had been conducted at 9 institutions between April 1, 2016 and March 31, 2017 in patients 15 years old or younger who received either atropine or cyclopentolate for refraction assessment. The investigation included patient's age, symptoms, and whether patients with side effects had any systemic diseases. RESULTS: A total of 811 patients (mean age ± SD, 4.6 ± 2.2 years) received atropine and 71 (8.8%) patients had side effects. Except in patients under two years old, 1% eye drops showed higher incidence rate of side effects than 0.5% and 0.25% eye drops. Side effects most frequently occurred following the initiation of the instillation on the first day. The symptoms included flush (29/71, 40.8%), fever (21/71, 30.0%), and both (11/71, 15.5%). A total of 2238 patients (5.7 ± 3.0 years) used cyclopentolate and 27 (1.2%) (4.0 ± 2.2 years) patients had side effects. The symptoms included drowsiness (10/27, 37.0%), red eye (4/27, 14.8%), fever (3/27, 11.1%), and flush (3/27, 11.1%). CONCLUSIONS: Atropine has a side effect incidence rate 7 times higher than the incidence rate of cyclopentolate. Flush and fever are the most common side effects of atropine and drowsiness is the main side effect of cyclopentolate. These findings should be noted when examining cycloplegic refraction to manage amblyopia and strabismus in children.

C-reactive protein may be useful to differentiate idiopathic orbital inflammation and orbital cellulitis in cases with acute eyelid erythema and edema.

PURPOSE: Idiopathic orbital inflammation (IOI) and orbital cellulitis can present similar clinical features, and the diagnoses of these two disorders are sometimes confused. The purpose of the present study was to determine whether or not inflammatory markers in the blood can be useful to differentiate between IOI and orbital cellulitis in cases with acute eyelid erythema and edema. SUBJECTS AND METHODS: In this retrospective single-institute study, we reviewed the medical records spanning the past 10 years at the Department of Ophthalmology, Osaka Medical College Hospital, Takatsuki, Osaka, Japan, and found 45 cases, with patients >15 years of age, with presumed IOI. Their blood samples were obtained within 5 days after the onset of IOI. Of those cases, 15 patients (10 males, 5 females, mean age of 56.9 years; range 38-76 years) presented acute eyelid erythema and edema, and were initially misdiagnosed as orbital cellulitis. Thus, inflammatory markers in the blood (ie, white blood cells [WBCs] and C-reactive protein [CRP]) of those 15 patients were analyzed with 17 patients (10 males, 7 females) having orbital cellulitis. The receiver operating characteristic curve analysis was performed to determine the optimal cut-off values. RESULTS: The mean ± standard error (SE) levels of the WBC were 6.80±0.70×103/µL in the IOI patients, and 8.54±0.91×103/µL in the orbital cellulitis patients, and no significant differences were observed (P=0.15, Student's t-test). However, the mean ± SE levels of CRP were 1.04±0.43 mg/dL in the IOI patients, yet were significantly increased to 4.65±1.21 mg/dL in the orbital cellulitis patients (P=0.01, Student's t-test). The area under the curve value was 0.80 and the optimal cut-off value was 0.43 for orbital cellulitis, with sensitivity and specificity being 82% and 73%, respectively. CONCLUSION: The findings of this study indicate that CRP may be useful in distinguishing patients with idiopathic orbital inflammation from those with orbital cellulitis.

Bilateral rhegmatogenous retinal detachment due to unusual retinal degeneration in Down syndrome: A case report.

RATIONALE: The aim of this study was to report a case of Down syndrome (DS) complicated with bilateral retinal detachment (RD) due to unusual retinal degeneration. PATIENT CONCERNS: A 9-year-old girl complained of bilateral visual disturbance during a follow-up examination for myopia and strabismus. DIAGNOSES: Slit-lamp examination revealed moderate posterior subcapsular cataract in both eyes. B-mode echography showed bilateral bullous RD; however, it was difficult to detect the causal retinal breaks due to poor mydriasis. INTERVENTIONS: For treatment, the patient underwent bilateral lensectomy, vitrectomy, and silicone oil tamponade. OUTCOMES: Intraoperative findings revealed symmetrical retinal breaks and unusual caterpillar-like retinal degeneration on the upper temporal side of both eyes. Three months later, the patient underwent bilateral silicone oil removal and intraocular lens implantation. LESSONS: In this case, the retinal degeneration was morphologically different from retinal lattice degeneration, thus suggesting that it might be involved in the onset of DS-related bilateral RD.

A Case of Retinal Detachment with Unique Optical Coherence Tomography Findings after Gamma Knife® Radiosurgery Treatment for Choroidal Melanoma.

PURPOSE: To report a case of retinal detachment with unique optical coherence tomography (OCT) findings after Gamma Knife® (GK; Elekta Instrument AB, Stockholm, Sweden) treatment for choroidal melanoma (CM). CASE REPORT: A 48-year-old woman underwent GK therapy for CM in her right eye from the macula to the temporal side. While the tumor subsequently shrank, the patient developed radiation retinopathy, which was treated with laser photocoagulation. The tumor lesions later subsided; however, her visual acuity (VA) decreased 8 years after the initial treatment. Although the tumor lesions in the right eye had become scarred, a bullous retinal detachment with fixed folds occurred in the superior-nasal quadrants. OCT examination revealed a preretinal membrane, vitreoretinal traction, and an inner retinal break; however, no outer retinal break was clearly detectable. MRI scans showed no increase in tumorous lesions, and 123I-IMP SPECT imaging showed no photon accumulation. Thus, it was determined that there was no tumor activity. The corrected VA in her right eye was light perception, and it was determined that there was no indication for vitreous surgery. CONCLUSION: In this case, an inner retinal break was formed by the vitreoretinal traction around the scarred tumor and radiation retinopathy, thus suggesting the possibility of the development of a rhegmatogenous retinal detachment presumably complicated with an outer retinal break.

A Case of Fundus Oculi Albinoticus Diagnosed as Angelman Syndrome by Genetic Testing.

PURPOSE: To report a case of fundus oculi albinoticus diagnosed as Angelman syndrome (AS) via genetic testing. CASE REPORT: This study reports on a 4-year-old boy. Since he had been having respiratory disturbance since birth, he underwent a complete physical examination to investigate the cause. The results indicated that he had various brain congenital abnormalities, such as a thin corpus callosum, as well as hydronephrosis, an atrial septal defect, and skin similar to patients with fundus oculi albinoticus. Examination revealed bilateral fundus oculi albinoticus, mild iridic hypopigmentation, optic atrophy, and poor visual tracking. Genetic testing revealed a deletion in the Prader-Willi syndrome/AS region on chromosome 15, and together with the results of methylation analysis, his condition was diagnosed as AS. Follow-up examinations revealed no change in the fundus oculi albinoticus and optic atrophy, nor did they indicate poor visual tracking. CONCLUSIONS: When fundus oculi albinoticus and optic atrophy are observed in patients with multiple malformations, AS should be considered as a differential diagnosis.

A comparison of sex steroid concentration levels in the vitreous and serum of patients with vitreoretinal diseases.

The purpose of this study was to compare steroid hormone concentration levels in the vitreous and serum of vitreoretinal disease patients to elucidate the possibility of neurosteroid production in the retina. Serum and vitreous samples were collected from vitrectomy patients, and estradiol (E2) and testosterone (T) concentrations were measured using electro-chemiluminescence immunoassay. We measured E2 in epiretinal membrane (ERM, n = 14), macular hole (MH, n = 18), proliferative diabetic retinopathy (PDR, n = 20), and retinal detachment (RD, n = 19) cases, and T in ERM (n = 14), MH (n = 17), PDR (n = 13), and RD (n = 17) cases. No statistically significant age differences existed among the groups. Mean respective E2 concentrations (pg/ml) in the male/female vitreous were ERM: 6.67±4.04/18.82±7.10, MH: 10.3±7.02/17.00±4.8, PDR: 4.2±3.05/15.83±3.46, and RD: 10.00±4.58/16.06±4.57, while those in serum were ERM: 31.67±5.51/5.82±1.08, MH: 21.00±8.89/7.53±3.2, PDR: 29.20±7.07/12.75±10.62, and RD: 24.33±6.51/7.5±4.42. E2 concentrations were significantly higher (P<0.001) in the male serum than vitreous, yet significantly higher in the female vitreous than serum. Mean respective T concentrations (ng/ml) in the male/female vitreous were ERM: 0.15±0.03/0.15±0.01, MH: 0.15±0.01/0.15±0.01, PDR: 0.15±0.03/0.16±0.12, and RD: 0.14±0.01/0.17±0.08, while those in serum were ERM: 4.54±1.46/0.16±0.01, MH: 8.04±2.29/0.16±0.10, PDR: 5.14±1.54/0.22±0.11, and RD: 3.24±0.75/0.17±0.10. T concentrations were high in the male serum, yet extremely low in the male and female vitreous and female serum. High concentrations of E2 were found in the vitreous, and women, in particular, exhibited significantly higher concentrations in the vitreous than in the serum. This finding suggests the possibility that in vitreoretinal disease cases, the synthesis of E2 is increased locally only in female eyes.

A Case of Childhood-Onset Giant Cell Tumor that Caused Optic Nerve Atrophy in Both Eyes.

PURPOSE: The purpose of this study was to report the case of a female patient who had a giant cell tumor in the paranasal sinus during childhood, and while undergoing multiple resection surgeries experienced optic atrophy in both eyes. CASE PRESENTATION: This study involved a 35-year-old woman who was previously diagnosed with a giant cell tumor of the paranasal sinus bone at age 13. A CT scan revealed a large tumor extending from the sphenoid sinus to the ethmoid sinus. At age 14, a tumor resection was performed in conjunction with radiation therapy. However, after resection and radiation therapy there were repeated recurrences, and additional resections were performed. Ophthalmically, there was marked optical atrophy in both eyes, and Goldmann visual field perimetry revealed that only the arcuate peripheral area remained on the nasal side of the right eye, and that there were dark spots in the paracentral area of the left eye. CONCLUSIONS: In this case, a large giant cell tumor occurred in the sphenoid sinus and ethmoid sinus during childhood, and it is thought that optic atrophy was caused by compressive optic neuropathy. The sphenoid sinus and ethmoid sinuses are anatomically close to the optic nerve, and when a tumor grows larger at this site it can easily put pressure on the optic nerve. Therefore, early detection and treatment are important.

Complete Recovery from Blindness in Case of Compressive Optic Neuropathy due to Unruptured Anterior Cerebral Artery Aneurysm.

It is not common for an isolated visual symptom to be the first indication of an aneurysm compressing the optic nerve. The compression can lead to blindness, and a recovery from the blindness is rare. We report a female with a left painless optic neuropathy caused by an unruptured anterior cerebral artery aneurysm. The patient had a temporal hemianopic visual field defect, which progressed to blindness in the left eye, while the right visual function was not affected. A coil embolization of the aneurysm completely restored her visual acuity to 20/20. These findings suggest that aneurysmal lesions should be ruled out in case of unilateral optic neuropathy with hemianopic visual field defects and progressive visual loss.

A Case of Hydranencephaly in Which Ophthalmic Examinations Were Performed.

PURPOSE: We performed ophthalmic examinations, including optical coherence tomography (OCT), on a case diagnosed with hydranencephaly. CASE REPORT: This case involved a female infant born at the gestational age of 35 weeks and 4 days, with the birth weight of 2,152 g, who was one of monochorionic diamniotic twins, and the identical twin died in utero at the gestational age of 24 weeks. After that, examination by fetal echo indicated that she had microcephaly and ventriculomegaly. Postnatal magnetic resonance imaging (MRI) of her head indicated microcephaly and significant enlargement of the lateral ventricle on both sides, with no obvious signs of elevated intracranial pressure. The brain parenchyma of both sides of the frontal lobe, parietal lobe, and occipital lobe had marked thinning, yet that of the temporal lobe, basal ganglia, thalamus, brain stem, and cerebellum had been maintained. Moreover, no obvious hematoma or neoplastic lesions were observed. Ophthalmic examinations indicated that both of her eyes had slight light reflex, attributed to optic nerve atrophy. Examination by use of a hand-held OCT system indicated a layered structure of the retina and thinning of the ganglion cell layer. Flicker electroretinogram (ERG) examination by use of a hand-held ERG system indicated an almost normal wave. However, no clear visual reaction was observed when she was 10 months old. CONCLUSION: Our findings in this case of hydranencephaly revealed that even though the outer layer functions of the patient's retina were maintained, extensive damage to her cerebral cortex resulted in poor visual function.

A case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft.

INTRODUCTION: The incidence of facial cleft is rare and ranges between 1.43 and 4.85 per 100,000 births. To date, there have been few reports of detailed ophthalmologic examinations performed in cases of facial cleft. Here, we report a case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft. CASE REPORT: A 9-day-old female infant was delivered by cesarian section at 34 weeks of gestational age (the second baby of twins) and weighed 2,276 g upon presentation. She had a facial cleft and ectrodactyly at birth. Right eye-dominant blepharophimosis was obvious. Examination of the right eye revealed inferior corneal opacity with vascularization, downward corectopia, and optic-nerve hypoplasia. The corneal diameter was 8 mm in both eyes, and tonometry by use of a Tono-Pen(®) XL (Reichert Technologies, Depew, NY, USA) handheld applanation tonometer revealed that her intraocular pressure was 11-22 mmHg (Oculus Dexter) and 8 mmHg (Oculus Sinister). B-mode echo revealed no differences in axial length between her right and left eyes. When she was 15-16 months old, we attempted to examine her eyes before she underwent plastic surgery under general anesthesia. She had a small optic disc in both eyes and the right-eye disc was tilted. After undergoing canthotomy, gonioscopy and ultrasound biomicroscopy revealed that almost all directions were open except for the peripheral anterior synechia. Since magnetic resonance imaging revealed ventriculomegaly associated with an interhemispheric cyst at birth, a ventriculoperitoneal shunt was inserted at 12 days of age. At 25 months of age, her condition suddenly deteriorated due to occlusion of the ventricular shunt catheter, and she died 5 days later. In this patient, amniotic band syndrome was presumed to be the primary cause due to the clinical findings. CONCLUSION: We experienced a case of optic-nerve hypoplasia and anterior segment abnormality that occurred with facial cleft. The cause of these abnormalities is unclear, yet amniotic band syndrome is a possible candidate.

Long-Term Follow-Up Case of Multiple Retinal Arterial Macroaneurysms Developing Branch Retinal Vein Occlusion following Ruptured Macroaneurysm.

PURPOSE: Retinal arterial macroaneurysm (RAM) has been reported in association with branch retinal vein occlusion (BRVO), and usually BRVO precedes RAM. We present a long-term follow-up case report of unilateral multiple RAMs that developed BRVO following ruptured RAM in the same retinal quadrant. CASE PRESENTATION: An 80-year-old woman presented with floaters in her right eye in June 2012. Visual acuity (VA) was 20/25 in her right eye with posterior capsular opacity. Her fundus showed the first ruptured RAM at the superotemporal vascular arcade with subinternal limiting membrane and subretinal hemorrhages not involving the macula. These were absorbed gradually with a VA of 20/20. After 2 years, the second RAM at the proximal superotemporal vascular arcade developed and impending BRVO occurred with macular edema at the distal site of the RAM. With the RAM located close to the arteriovenous crossing, her VA was dropped to 20/60. Intravitreal injection of ranibizumab was performed and macular edema was resolved with improved vision of 20/30. Three months later, she realized a sudden vision loss of 2/200. Her posterior pole showed massive pre- and subretinal hemorrhages, and vitrectomy was performed. The source of bleeding was the third RAM's rupture in a different artery. Her vision improved to 20/30. The unaffected eye showed no RAMs. CONCLUSION: We experienced a long-term follow-up case of multiple RAMs showing different courses. We should cautiously note that BRVO can occur following RAM at the arteriovenous crossing.

A Case of a Newborn with Agenesis of the Corpus Callosum Complicated with Ocular Albinism.

PURPOSE: To report a case of ocular albinism found in a newborn infant in whom agenesis of the corpus callosum (ACC) was indicated in utero. CASE REPORT: This study involved a female newborn who was delivered after a gestational period of 41 weeks. The patient was referred to the Obstetrics Department at Takatsuki Hospital, Takatsuki City, Japan, after the indication of ACC by magnetic resonance imaging (MRI) at a nearby clinic during the fetal period. At birth, the baby's weight was 2,590 g, and ACC and ventricular enlargement were found by cranial sonography and cranial MRI. While initial ophthalmic findings noted partial loss of pigmentation of the iris and hypopigmentation of broad areas of the fundus in both eyes, nystagmus was not observed. The patient's hair pigment was slightly diluted, and the color of her skin was slightly off-white. At 2 years after birth, obvious mental retardation was observed. With regard to other systemic findings, no apparent heart, kidney, or immune system abnormalities were found. CONCLUSION: Although the patient in question is presently growing without any major systemic problems, it will be necessary in the future to pay attention to any changes in systemic and ophthalmic findings.

Vitrectomy for Tractional Retinal Detachment with Twin Retinal Capillary Hemangiomas in a Patient with Von Hippel-Lindau Disease: A Case Report.

PURPOSE: The purpose of this study was to report a case of Von Hippel-Lindau disease (VHL) with twin retinal capillary hemangiomas that was successfully treated by vitreous surgery for tractional retinal detachment following laser photocoagulation. CASE: A 44-year-old male presented at our university hospital after noticing decreased visual acuity in his right eye. The patient had previously undergone multiple operations for cerebellar, thoracic, and lumbar spine hemangioblastomas when he was approximately 19 years old. Upon initial examination, ocular findings revealed twin connected retinal capillary hemangiomas around the temporal upper area of the patient's right eye. The patient was subsequently diagnosed with VHL based on his medical history and current observations of the ocular fundus. Tractional retinal detachment had occurred as the result of the formation of proliferative membranes following laser photocoagulation. The patient underwent vitreous surgery to treat the tractional retinal detachment, resulting in a successful postoperative outcome. CONCLUSION: The findings of this study show the possibility that proliferative changes and tractional retinal detachment can arise following photocoagulation for retinal capillary hemangiomas in patients with VHL.

A Case of Proliferative Retinopathy Complicated with Tuberous Sclerosis Treated by Vitreous Surgery.

We report a case of proliferative retinopathy complicated with retinal hamartoma in a tuberous sclerosis patient. This study involved a 16-year-old female patient who was diagnosed as having tuberous sclerosis at birth. Ophthalmic examination revealed retinal hamartoma surrounding the optic disc in both eyes. Vitreous surgery involving a vitrectomy and resection of the proliferative membranes was performed for proliferative retinopathy in her right eye. Postoperative fundus findings showed improvement and decreased exudative changes. The proliferative and exudative changes appeared to be due to the retinal hamartoma, and vitreous surgery proved effective in this case.

A Case of Coats' Disease with Spontaneous Retinal Reattachment after Total Detachment.

PURPOSE: To report a case of Coats' disease in which spontaneous reattachment occurred after total retinal detachment. PATIENT AND METHODS: A young boy (patient age: 4 years and 11 months) presented with leukocoria in the left eye. Slit-lamp examination revealed total retinal detachment with abnormal retinal blood vessels and subretinal exudation just behind the lens. Computed tomography imaging showed no solid mass lesion in the intraocular space. Secondary total retinal detachment as a complication of Coats' disease was diagnosed. No light perception was detected, so we determined that surgical treatment was not indicated. RESULTS: Four months after the initial diagnosis, the retina showed complete reattachment with a large amount of subretinal hard exudate. Visual acuity remained unchanged, with no light perception. CONCLUSIONS: We speculate that the spontaneous retinal reattachment in the present case was caused by the decreased permeability of the abnormal retinal vessels and the good functional effect of the retinal pigment epithelium.

Vitrectomy for Proliferative Diabetic Retinopathy Associated with Klinefelter Syndrome.

INTRODUCTION: We encountered a patient with Klinefelter syndrome (KS) who experienced poor outcomes after vitrectomy for proliferative diabetic retinopathy (PDR). CASE: A 44-year-old male with poorly controlled diabetes was diagnosed with KS by chromosome analysis. Ocular findings revealed severe PDR complicated with extensive preretinal hemorrhages and traction retinal detachment in his left eye, and pars plana vitrectomy was subsequently performed for treatment. RESULTS: A clotting hemorrhage developed during surgery and proved difficult to control. Due to postoperative bleeding and redetachment, the vitrectomy was repeated. At the second operation, we performed a silicone oil tamponade; however, the retina was redetached under the silicone oil, and the light perception vision ultimately disappeared. CONCLUSION: The patient, despite showing increased blood coagulability due to diabetes, presented severe coagulopathy, likely related to KS. In patients with KS and severe PDR, the potential difficulty of vitrectomy should always be kept in mind.