Les malformations congénitales cliniquement visibles / Clinically visible congenital malformations

Contexte. Les malformations congénitales représentent l'une des causes majeures de mortalité infantile. La réduction de cette mortalité passe par la mise en place d'un système de surveillance épidémiologique. Le but de cette étude était d'établir les caractéristiques épidémiologiques et cliniques des malformations congénitales cliniquement visibles. Méthodes. Étude rétrospective descriptive et analytique réalisée entre janvier 2018 et décembre 2019.Elle a concerné les patients de moins de 15 ans présentant une ou plusieurs malformations congénitalescliniquement visibles.Les variables étudiées étaient d'ordre épidémiologique et clinique. Résultats. Cent trois patients présentant une malformation congénitale ont été colligés. Ils représentaient une fréquence de 6,8%. Les malformations ostéoarticulaires représentaient 43% (n=44), et celles de la paroi abdominale 25% (n=26). Les mères étaient paucipares (n=50,48%) et âgées en moyenne de 27,9+/- 2,7 ans. Aucun diagnostic anténatal n'a été fait.Les enfants étaient nés à terme dans 98% (n=101). Ils étaient de sexe masculin dans 60% (n=62).Le taux de décès était de 11%(n=10). Conclusion. les malformations congénitales étaient dominées par les anomalies ostéoarticulaires et celles de la paroi abdominale. Elles sont sous-estimées et nécessitent la mise en place d'un système d'enregistrement pour une maitrise de son épidémiologie.

Síndrome de Marcus-Gunn / Marcus Gunn syndrome

Introducción: El síndrome de Marcus-Gunn se manifiesta con retracción o elevación del párpado ptótico ante la estimulación del músculo pterigoideo del mismo lado y el término "guiño mandibular" es inapropiado ya que el párpado no siempre desciende. Puede producirse en la apertura bucal, masticación, avance mandibular, al sonreír, silbar, bruxar, sacar la lengua, deglutir, chupar, cantar, en la contracción esternocleidomastoidea, maniobra de Valsalva, respirar o inhalar. Es congénito y de modalidad rara y autosómica dominante. Objetivo: Contribuir al conocimiento de esta rara enfermedad y a su relación con el ámbito odontológico. Presentación del caso: Se trata de un niño de 3 años diagnosticado de síndrome de Marcus-Gunn en el periodo neonatal, por la observación de la madre de una apertura palpebral izquierda durante la succión nutritiva. No presenta otras enfermedades ni antecedentes de interés. En la exploración extraoral se advierte una ptosis palpebral derecha y apertura palpebral izquierda en los movimientos mandibulares y deglución. La intensidad del reflejo se incrementa en estados de ansiedad derivados del tratamiento dental. Conclusiones: el síndrome de Marcus-Gunn es una entidad rara en pediatría, en la cual sus hallazgos clínicos determinan el diagnóstico. Teniendo en cuenta que en ocasiones presentan alteraciones oculares, nada nos hace sospechar la presencia de una enfermedad oral específica(AU)

Introduction: Marcus Gunn syndrome manifests with retraction or elevation of the eyelid ptotico while stimulation of the pterygoid muscle on the same side and the term jaw-winking is inappropriate because the eyelid does not always goes down. It can occur during mouth opening, mastication, mandibular advancement, while smiling, whistling, bruxing, sticking out the tongue, swallowing, sucking, singing, during the sternocleidomastoid contraction, the Valsalva maneuver, breathing or inhaling. This syndrome is congenital and rare, and of autosomal dominant modality. Objective: To contribute to the knowledge of this rare disease and its relationship with the odontologic field. Presentation of the case: 3 years old boy diagnosed with Marcus Gunn syndrome in the neonatal period by the observation of the mother of a left palpebral opening during the nutritive sucking. He does not present other diseases or a background of interest. In the extraoral exploration, it is noticed a right palpebral ptosis and a left palpebral opening in the jaw movements and in swallowing. The intensity of the reflex increases in anxiety states arising from the dental treatment. Conclusions: Marcus Gunn syndrome is a rare entity in pediatrics, in which its clinical findings determine the diagnosis. Taking into account that sometimes it presents ocular alterations, nothing makes us suspect the presence of a specific oral disease(AU)

Urrets-Zavalia Syndrome Following Trabeculectomy

PURPOSE: We report a case of Urrets-Zavalia syndrome with a fixed dilated pupil after an uneventful trabeculectomy.CASE SUMMARY: Trabeculectomy was performed on a 51-year-old male who had a history of recurrent uveitis in the left eye, with uncontrolled intraocular pressure despite maximally-tolerated medial therapy. There was no unexpected event during surgery. Topical 1% atropine was used for only 2 days after surgery. In the early postoperative period, 1% prednisolone and 0.3% ofloxacin were given four times a day, then gradually reduced. One month later, only 1% prednisolone was given once a day. Intraocular pressure in his left eye was well controlled from 8–14 mmHg after surgery. One month after surgery, the pupils remained dilated. There was no reaction to topical 2% pilocarpine and no relative afferent pupillary defect or posterior synechia.CONCLUSIONS: Our case, although rare, suggests that Urrets-Zavalia syndrome should be considered in patients with well-controlled intraocular pressure after uneventful trabeculectomy.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts / Facoemulsificação e colírio de atropina a 1% para tratamento de microcoria congênita antimetrópica associada a catarata

ABSTRACT A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts. Phacoemulsification was performed using an iris expansion device and the anterior capsule was stained using the "trypan down under" technique. Preoperative considerations, the surgical approach, and postoperative management are discussed.

RESUMO Um caso raro de microcoria congênita bilateral associada à antimetropia em um homem de 47 anos de idade é descrito aqui. O paciente queixava-se de perda visual progressiva em seu olho direito nos últimos 5 anos. Um exame com lâmpada de fenda e biomicroscopia ultrassônica confirmaram microcoria congênita e catarata. A facoemulsificação foi realizada usando dispositivo de expansão iriana, e a cápsula anterior foi corada através da técnica "trypan down under". Considerações pré-operatórias, abordagem cirúrgica e manejo pós-operatório são discutidos.

Urrets-Zavalia Syndrome following cataract surgery in a case of anterior megalophthalmos / Síndrome de Urrets-Zavalia após cirurgia de catarata em um caso de megaloftalmo anterior

ABSTRACT Intraoperative and postoperative cataract surgery in eyes with anterior megalophthalmos are challenging procedures. Herein we describe the case of a 53-year-old male with anterior megalophthalmos who developed unilateral Urrets-Zavalia Syndrome following cataract surgery.

RESUMO O intraoperatório e o pós-operatório de cirurgia de catarata em olhos com megaloftalmo anterior é desafiador. Descrevemos o caso de um homem de 53 anos com megaloftalmo anterior que desenvolveu a Síndrome de Urrets-Zavalia unilateral após cirurgia de catarata.

On the shoulders of giants: the case of the Claude Bernard Horner syndrome

The syndrome called mainly in the French world as Claude Bernard Horner was frst described by Francois Pourfour du Petit, in 1727, but more thoroughly defned by the French physiologist, Claude Bernard, in 1852, followed by several physicians who offered different interpretations, mainly Silas Weir Mitchell (1864). The clinical and pharmacological implications, with the fnal wrap-up of the syndrome, were presented by a Swiss ophthalmologist, Johann Friedrich Horner, in 1869. This is a cooperative defnition of a syndrome of the sympathetic disruption of the ocular inervation, with fnal addings mainly about pharmacological approach by Horner, but with credits to many others clinicians and physiologists. This is the case of repeated presentations of a "new sign" in neurology with few additions from one to another.

A síndrome chamada principalmente no mundo francês como Claude Bernard Horner foi descrita pela primeira vez por François Pourfour du Petit, em 1727, mas mais profundamente defnida pelo fsiologista francês, Claude Bernard, em 1852, seguido por vários médicos que ofereceram interpretações diferentes, principalmente Silas Weir Mitchell (1864). As implicações clínicas e farmacológicas, com o desfecho fnal da síndrome, foram apresentadas por um oftalmologista suíço, Johann Friedrich Horner, em 1869. Esta é uma defnição cooperativa de uma síndrome da ruptura da inervação simpática ocular, com adições fnais principalmente sobre a abordagem farmacológica por Horner, mas com créditos para muitos outros médicos e fsiologistas. É o caso de repetidas apresentações de um "novo sinal" na neurologia, com poucas adições de um para o outro.

From the "small doll" until "prostitute's pupil" through Argyll Robertson: the Argyll Robertson's sign, one hundred fifty years from his findings (1869) / Da "pequena boneca" até "pupila da prostituta" através de Argyll Robertson: o sinal de Argyll Robertson, cento e cinquenta anos de seus achados (1869)

A tiny structure, the pupil, attracts too much attention, since the antiquity. The pupil as part of the "'window of/to the soul", the eyes, it can demonstrate a clinical disorder sign, or simply a psychological expression. In this paper, it is studied the situation in which the pupillary reflex to light is compromised, but the accommodation reflex is preserved, what is named after Argyll Robertson, the frst Scottish ophthalmologist, who besides described the signal (1869), he also tried to defned its clinical signifcance. Afterwards, it was clearly demonstrated its relationship with tertiary neurosyphilis.

Uma estrutura minúscula, a pupila, atrai muita atenção, desde a antiguidade. A pupila como parte da "'janela da alma'", os olhos, poderia demonstrar um sinal de desordem clínica, ou, simplesmente, uma expressão psicológica. Neste trabalho, estuda-se a situação em que o reflexo pupilar à luz é comprometido, mas o reflexo de acomodação é preservado, o que leva o nome de Argyll Robertson, o primeiro oftalmologista escocês que além de descrever o sinal (1869), também tentou defniu seu signifcado clínico. Posteriormente, foi claramente demonstrada sua relação com a neurossíflis terciária.

A Report of Two Case of Ocular Toxicity Resulting from Direct or Indirect Bee Venom

PURPOSE: To report a patient stung by a bee, who was diagnosed with sterile endopthalmitis and another patient diagnosed with optic neuritis, with decreasing visual acuity, after refined bee venom injection around the orbital tissue. CASE SUMMARY: A 82-year-old female visited our hospital for decreased visual acuity in the right eye and ocular pain due to a bee sting. The bee sting penetrated the sclera into the vitreous. In the anterior segment, severe cornea edema and anterior chamber cells were seen. Using ultrasonography, inflammation was seen around the intravitreal area. After 3 months, intravitreal inflammation regressed but the patient's visual acuity was light perception negative, and corneal opacity, neovascularization, and phthisis bulbi were detected. A 55-year-old male visited our hospital for ocular pain in the right eye and decreasing visual acuity after refined bee venom injection around the orbital tissue. The best-corrected visual acuity in the right eye was 15/100, there was moderate injection on the conjunctiva. A relative afferent pupillary defect, abnormal color vision test results, and a defect in the visual field test were observed. There was no pain during external ocular movement, and other general blood tests, and a brain MRI were normal. Based on these symptoms, methylprednisolone megatherapy was started for treatment of optic neuritis. After treatment, visual acuity of the right eye was 9/10 and all other clinical optic neuritis symptoms regressed. CONCLUSIONS: Based on these two cases, ocular toxicity from bee venom could result from both direct and indirect courses. Treatment using refined bee venom might be harmful, and caution is recommended in its use.

Frosted Branch Angiitis Associated with Epstein-Barr Virus Infection

PURPOSE: To report a case of frosted branch angiitis (FBA) associated with Epstein-Barr virus (EBV) infection in a child. CASE SUMMARY: A 7-year-old boy presented with bilateral blurred vision. On ophthalmic examination, his best-corrected visual acuity was 20/25 in the right eye and 20/32 in the left eye. The pupils were equal, round, and reactive to light without a relative afferent pupillary defect. He had normal color vision in both eyes. Slit-lamp examination revealed no abnormalities in the anterior parts of the eyes. Fundoscopic examination revealed prominent white sheathing retinal vasculitis predominantly on the veins in all quadrants, as well as macular edema and irregular foveal reflex in both eyes. Fluorescein angiography showed normal blood flow, but late diffuse staining and leakage of the affected vessels. Spectral domain optical coherence tomography (SD-OCT) showed thickening of the vessel walls, swelling due to hyperreflective material, and hyperreflective retinal depositions. Serological tests and the serum polymerase chain reaction for EBV were positive. A diagnosis of FBA associated with EBV was made. He was treated with systemic acyclovir and steroids. The response was rapid, with improvement in visual acuity to 20/20 in both eyes by day 3. After 7 weeks, all clinical signs resolved and SD-OCT examination showed normal vessel wall thickness and the absence of hyperreflective depositions. CONCLUSIONS: EBV may present with FBA even in the absence of a systemic sign of primary EBV infection. Thus, EBV should be considered as the etiology of FBA.

Visual Loss with Ophthalmoplegia after Prone Position Spinal Surgery

PURPOSE: We report a case of acute visual loss with ophthalmoplegia after prone position spinal surgery who had blood supply dependence on collateral circulation due to occlusion of the Internal carotid artery. CASE SUMMARY: A 74-year-old man was referred to the department of ophthalmology for acute visual loss and ophthalmoplegia after lumbar spine surgery performed in prone position. On the initial visit, his right visual acuity was 0.8 and the left visual acuity was negative light perception. Intraocular pressure was normal. There was a relative afferent pupillary defect and ophthalmoplegia of all directions in the left eye. Because of the ptosis of the upper eyelid in the left eye, it was impossible to tune the eye voluntarily. The cherry red spot and pale retina were observed on the fundus examination. On brain magnetic resonance imaging angiography, we found complete obstruction of the left internal carotid artery. He had intravenous injection of 1 g methylprednisolone for 3 days, and discharged with per oral medicine. After 1 month of treatment, the ophthalmoplegia was slightly improved, but visual acuity was not recovered. CONCLUSIONS: In this case, unlike previous reports, acute visual loss and ophthalmoplegia occurred after spinal surgery the patient who had collateral circulation for ocular blood supply because of complete obstruction of the left internal carotid artery. This report highlights the importance of being aware of the anatomical variant in possible complications of external ocular compression after non-ocular surgery.

Optic Neuritis Accompanied by Optic Perineuritis after Peribulbar Retinalamin Injection

PURPOSE: To report a case of optic neuritis accompanied by optic perineuritis after retinalamin peribulbar injection. CASE SUMMARY: A 46-year-old Kazakhstan woman presented with sudden vision loss, headache, and pain in both eyes which began 2 hours after the peribulbar injection of retinalamin approximately 10 days prior to her initial visit. At the initial visit, the best-corrected visual acuity was light perception in both eyes. A fundus examination showed bilateral optic disc swelling and fluorescein angiography showed late-phase leakage around the optic nerve and optic nerve sheath enhancement. On brain magnetic resonance imaging, the tram track sign and donut sign were seen in both eyes. Steroid pulse therapy was started after the diagnosis of optic neuritis accompanied by optic perineuritis. After 6 months, the patient's visual acuity improved up to 0.8 in the right eye and 1.0 in the left eye. The light reflex was recovered, the relative afferent pupillary defect decreased, and there was no evidence of optic disc swelling. CONCLUSIONS: When an unverified drug injection is performed (e.g., retinalamin), it could result in complications such as optic neuritis and optic perineuritis from an allergic reaction or peribulbar injection trauma. In such cases, high-dose steroid pulse therapy may be considered.

Abducens Nerve Palsy and Optic Perineuritis Caused by Fungal Sphenoidal Sinusitis

PURPOSE: To report a case of abducens nerve palsy and optic perineuritis caused by fungal sphenoidal sinusitis. CASE SUMMARY: A 48-year-old male visited emergency department for retrobulbar pain, decreased vision, and horizontal diplopia for 3 days. He reported that previous medical history was non-specific, however, blood glucose level was 328 mg/dL (70–110). He had experienced severe headache for 7 days. The best corrected visual acuity was 20/20 at right eye and 20/25 at left eye. The pupil of left eye did not have relative afferent pupillary defect. Left mild proptosis was noted. The extraocular examination showed 30 prism diopters left esotropia at primary gaze and −4 abduction limitation of left eye. The left eye showed mild optic disc swelling and inferior field defect by field test. Brain magnetic resonance imaging showed enhancement of sphenoidal sinus, ethmoidal sinus, and around optic nerve at left eye. Three days after antibiotics treatment, the vision of left eye deteriorated to 20/40 and periorbital pain developed. The drainage and biopsy of sphenoidal sinus were performed. The histopathologic examination showed hyphae consistent with aspergillosis. The ocular symptoms were improved with anti-fungal treatment. Follow-up magnetic resonance imaging performed 1 month after treatment showed improvement of lesion at left orbit. Five months after surgery, the visual acuity of left eye was improved to 20/25. The patient showed orthotropia at primary gaze without limitation. CONCLUSIONS: The abducens nerve palsy and optic perineuritis can be caused by fungal sphenoidal sinusitis. The early diagnosis and appropriate treatment can lead to favorable outcome.

Bilateral Delayed Nonarteritic Anterior Ischemic Neuropathy Following Acute Primary Angle-closure Crisis

PURPOSE: We report a case of bilateral nonarteritic anterior ischemic optic neuropathy (NAION) following acute angle-closure crisis (AACC). CASE SUMMARY: A 76-year-old female visited our clinic because of a 1-day history of ocular pain and vision loss in both eyes. The visual acuity was 0.02 in both eyes and her intraocular pressure (IOP) was 52 mmHg in the right eye (RE) and 50 mmHg in the left eye (LE). She had corneal edema and a shallow anterior chamber in both eyes, with 4 mm fixed dilated pupils. After decreasing the IOP with intravenous mannitol, laser iridotomy was performed. However, 2 days later, visual acuity was further reduced to finger counting at 10 cm RE and at 50 cm LE, and her optic disc was swollen. Bilateral NAION following AACC was diagnosed. One month later, visual acuity slightly improved to 0.02 RE and 0.04 LE, and the optic disc edema resolved. A small cup-disc ratio, optic disc pallor, and atrophy were observed. Humphrey visual fields demonstrated superior and inferior altitudial visual field defects in the LE, and almost total scotoma in the RE. CONCLUSIONS: AACC can be a predisposing factor for NAION, so the relative afferent pupillary defect, papilledema, and presentation of other risk factors are important clues to a diagnosis of NAION.

Toxic Optic Neuropathy Caused by Chlorfenapyr Poisoning

PURPOSE: To report a case of toxic optic neuropathy caused by chlorfenapyr ingestion accompanied by central nervous system involvement. CASE SUMMARY: A 44-year-old female visited our clinic complaining of reduced visual acuity in both eyes for 7 days. She had ingested a mouthful of chlorfenapyr for a suicide attempt 2 weeks prior to the visit. Gastric lavage was performed immediately after ingestion at the other hospital. Her best-corrected visual acuity was finger count 30 cm in the right eye and hand motion in the left eye. Both pupils were dilated by 5.0 mm and the response to light was sluggish in both eyes. A relative afferent pupillary defect was detected in her left eye. Funduscopy revealed optic disc swelling in both eyes. Magnetic resonance imaging of the brain showed a symmetric hyper-intense signal in the white matter tract including the internal capsule, corpus callosum, middle cerebellar peduncle, and brainstem. The patient was diagnosed with toxic optic neuropathy induced by chlorfenapyr ingestion, and underwent high-dose intravenous corticosteroid pulse therapy. Three days later, the best-corrected visual acuity was no light perception in both eyes. Three months later, optic atrophy was observed in both eyes. Optical coherence tomography revealed a reduction in the thicknesses of the retinal nerve fiber layer and ganglion cell and inner plexiform layer in the macular area. CONCLUSIONS: Ingestion of even a small amount of chlorfenapyr can cause severe optic nerve damage through the latent period, despite prompt lavage and high-dose steroid treatment.

Optic Neuritis of Korean Adult

PURPOSE: The aim of the study was to describe the clinical features and characteristics of optic neuritis patients compared with those of patients enrolled in the Optic Neuritis Treatment Trial (ONTT). METHODS: We retrospectively included consecutive patients diagnosed with optic neuritis between 2006 and 2015. The inclusion criteria were the same as those of the ONTT; an acute or subacute unilateral visual symptom ≤8 days in duration, a relative afferent pupillary defect, and a visual field defect in the affected eye. RESULTS: The 41 patients (14 females, 27 males) were of mean age 32.7 years. The incidence of papilledema was 58.5%, thus higher than that of ONTT patients (p = 0.004), and the incidence of ocular pain was 75.6%, lower than that of ONTT patients (p = 0.002). Six months after intravenous methylprednisolone therapy, 29 of 32 eyes (90.6%) recovered visual acuity of ≥1.0 or better, and only one eye exhibited poor visual acuity (≤0.5). Orbital magnetic resonance imaging (MRI) was performed on 34 patients, and most (91.2%) exhibited optic nerve enhancement. The three patients who did not exhibit enhancement reported no pain. CONCLUSIONS: The clinical features of Korean optic neuritis patients differed from those of patients of the ONTT. Most patients exhibited optic nerve MRI enhancement associated with ocular pain. If enhancement of the optic nerve is lacking, diseases other than optic neuritis should be suspected because most optic nerves with neuritis exhibit enhancement on MRI.

Approach to pupillary abnormalities via anatomical pathways / 영남의대학술지

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.

Approach to pupillary abnormalities via anatomical pathways / 영남의대학술지

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.

An Unusual Case of Orbital Inflammation Preceding Herpes Zoster Ophthalmicus

PURPOSE: To present a case of orbital inflammation and optic perineuritis preceding vesicular eruption in herpes zoster ophthalmicus(HZO). CASE SUMMARY: An 84-year-old woman with a history of gall bladder cancer and hypertension complained of left periorbital erythematous edema and discomfort. On examination, visual acuity was 20/25 bilaterally; no tenderness, proptosis or ophthalmoplegia was observed. Pupils were equal, round, and reactive to light without relative afferent pupillary defects. Slit-lamp examination revealed severe conjunctival injection and chemosis without keratitis or uveitis. The remainder of the ocular examination was unremarkable. Magnetic resonance imaging confirmed left-sided preseptal swelling with an enlarged left lacrimal gland, high signal intensity of the retrobulbar fat and optic nerve sheath. Systemic antibiotic therapy with steroids was started under a presumed diagnosis of idiopathic orbital inflammatory disease, but the clinical presentation was unresolved. After 2 days, vesicular lesions confined to the first division of the trigeminal nerve and pseudodendritic keratitis developed on the left side leading to a diagnosis of HZO. Treatment with acyclovir immediately resolved anterior segment inflammation and periorbital edema. While on therapy, visual acuity deteriorated to 20/125 and the pupil became dilated and unresponsive to light over a few days. All signs and symptoms of acute orbitopathy and postherpetic neuralgia had resolved 3 months later with the exception of pupil abnormality and visual acuity. CONCLUSIONS: HZO may present with symptoms and signs of orbital inflammation and optic perineuritis even in the absence of a vesicular rash. Thus, HZO should be considered in the differential diagnosis of unexplained acute orbital syndromes.

A Case Report of Central Retinal Artery Occlusion Caused by Cardiac Myxoma

PURPOSE: We report the case of a patient diagnosed with central retinal artery occlusion caused by cardiac myxoma who underwent surgery to remove the myxoma. CASE SUMMARY: A 47-year-old woman came to our clinic presenting with a sudden decrease of visual acuity in the left eye. At the first visit, left eye visual acuity was hand motion, and intraocular pressure was 15.4 mmHg. A relative afferent pupillary defect was observed in the left eye. On fundus examination, a pale retina and cherry-red spot were observed at the posterior pole. On optical coherence tomography, macular edema was found. On fluorescein angiography and indocyanine green angiography, delayed blood circulation of the retina and choroid was found at early and late stages. Cerebral angiography was performed in the neurosurgery department and showed no occlusion of the ophthalmic artery. Cardiac ultrasonography and brain magnetic resonance imaging were performed. On cardiac ultrasonography, 4.46 × 2.09 cm cardiac myxoma was found. Resection of the cardiac myxoma was conducted in the thoracic and cardiovascular surgery department. Multiple cerebral infarcts were detected by brain imaging, and antithrombotic treatment was administered. After one month, blood circulation in the retina and choroid was observed in fluorescence angiography, but there was no improvement of visual acuity. At the 3-month follow-up visit, macular edema was decreased, but retinal atrophy and epiretinal membrane were observed on optical coherence tomography. CONCLUSIONS: Central retinal artery occlusion is a disease related to one's general condition. We experienced this case of central retinal artery occlusion caused by cardiac myxoma.

A Case of Acute Macular Neuroretinopathy in a Young Male

PURPOSE: We report a rare case of unilateral acute macular neuroretinoapthy in a young male. CASE SUMMARY: A 35-year-old male presented with a 2-day history of paracentral scotoma. He had suffered for 2 days from a flu-like illness, and his best corrected visual acuity was 20/20 OD and 20/20 OS. Pupillary reflex was normal and no relative afferent pupillary defects were not found. Ocular movement test was normal and pain on ocular movement was not noticed. Ophthalmoscopic examination of the left eye revealed multiple exudates lining the nasal macula toward the fovea. A Humphrey visual field study identified small paracentral scotoma. Spectral domain optical coherence tomography (SD-OCT, Heidelberg Engineering, Heidelberg, Germany) of the lesions showed a hyper-reflective lesion located in the outer plexiform layer and inflammatory cell infiltration. Fluorescent angiography was normal in the macula but showed late leak at the disc. The multifocal electroretinogram (mfERG) showed decreased foveal P1 amplitude in the left eye. The patient was diagnosed with acute macular neuroretinopathy and was treated with 60 mg of prednisolone. His subjective symptoms were improved, the paracentral scotoma disappeared, and the lesions appeared different upon SD-OCT; specifically, the hyper-reflective lesion disappeared and the outer plexiform layer showed thinning. CONCLUSIONS: Acute macular neuroretinopathy is a rare disease, and we report a case using SD-OCT and mfERG.