Prenatal or Perinatal Injury? Diagnosing the Cortically Blind Infant.

PURPOSE: To document the association of prenatal brain disruption with secondary perinatal distress in children diagnosed as having cortical visual impairment (CVI). DESIGN: Retrospective case series. METHODS: Eight children with severe CVI and clinical history of perinatal events were included. Case histories and neuroimaging studies were reviewed. The main outcome measures were perinatal history, visual and neurologic findings, and magnetic resonance (MR) imaging. RESULTS: In our patient cohort, MR imaging showed signs of cortical dysgenesis leading to congenital brain malformations such as polymicrogyria consistent with a prenatal timing of CNS injury. Although subcortical white matter changes were common, signs of watershed injury to the visual cortex were absent, suggesting that the visual loss was attributable to a prenatal etiology with secondary birth complications. CONCLUSION: Some children with CVI and a history of perinatal distress have prenatal dysgenesis of the developing brain. Therefore, a clinical history of perinatal hypoxia-ischemia is nonspecific and merits neuroimaging to identify antecedent brain malformations and timing of injury, which can influence patient diagnosis and management.

Bilateral visual loss and cerebral infarction after spleen embolization in a trauma patient with idiopathic thrombocytopenic purpura: A case report.

RATIONALE: Splenic artery embolization (SAE) is a common procedure in trauma patients with blunt splenic injuries. We report a case of acute ischemic stroke following orthopedic surgery in a patient with post-SAE reactive thrombocytosis. PATIENT CONCERNS: A 37-year-old woman with idiopathic thrombocytopenic purpura (ITP) suffered from multiple trauma scheduled for open reduction and internal fixation for right tibial and left radius fracture five days after SAE. The patient did not have any thromboembolic complications, although the platelet counts increased from 43 × 10/L to 568 × 10/L within two days after SAE. Surgery was completed under general anesthesia with tracheal intubation without complications. The patient complained of visual loss followed by limb weakness on the fourth and eighth hour postoperatively. DIAGNOSES: Magnetic resonance imaging (MRI) of head demonstrated ischemic change over bilateral basal ganglia, and occipital areas, suggesting the diagnosis of cortical blindness. INTERVENTIONS: To suppress platelet count and avoid platelet hyper-aggregation, anti-platelet drug (i.e., oral aspirin 100 mg daily), hydration, and hydroxyurea (i.e., 20 mg/kg daily) were used for the treatment of reactive thrombocytosis. OUTCOMES: Although right-sided hemiparesis persisted, the patient reported mild visual recovery. She was discharged four months after SAE with active rehabilitation. LESSONS: Our report highlights an increased risk of acute arterial thromboembolic events in patients with reactive thrombocytosis, especially those undergoing surgery.

A pilot study regarding basic knowledge of "cortical visual impairment in children" among ophthalmologists.

PURPOSE: A pilot study was done to evaluate knowledge regarding "cortical visual impairment (CVI) in children" among ophthalmologists. METHODS: This study was conducted during the annual conference of a zonal ophthalmological society. All ophthalmologists who attended the conference were requested to participate in this study. Those who agreed were given a validated questionnaire to assess knowledge regarding CVI. Cronbach's alpha of the questionnaire was 0.6. Participants were asked to respond to multiple choice questions by choosing the single best option. The responses obtained were then evaluated. RESULTS: The total number of registered delegates in the conference was 448. A total of 103 ophthalmologists showed interest to participate in the study with a response rate of 22.9%. Only 89/103 interested delegates were included in the study as remaining were unaware of CVI. No participant gave correct answers to all questions. Although more than 80% of them knew the most common association (87%) and site of pathology (84%), only 52% were sure about clinical features and even lesser respondents (39%) knew that magnetic resonance imaging is the correct investigation of choice. The majority responded correctly that these children need eye examination (89%) and can be managed by rehabilitation through multidisciplinary approach (82%), but only 58% could recognize differential diagnoses and had a correct idea regarding the prognosis of CVI. There was no correlation between the number of patients diagnosed per month by the respondent with knowledge of the disease. CONCLUSION: In this pilot study, ophthalmologists were found to have limited knowledge regarding clinical features, investigation, differential diagnosis, and visual prognosis of CVI in children. There is a need to improve awareness regarding CVI among ophthalmologists.

Severe neurodegeneration, progressive cerebral volume loss and diffuse hypomyelination associated with a homozygous frameshift mutation in CSTB.

Mutations of the cystatin B gene (CSTB; OMIM 601145) are known to cause Unverricht-Lundborg disease or progressive myoclonic epilepsy-1A (EPM1A, MIM #254800). Most patients are homozygous for an expanded (>30) dodecamer repeat in the promoter region of CSTB, or are compound heterozygotes for the dodecamer repeat and a point mutation. We report two adolescent sisters born to consanguineous parents of Sri Lankan descent who presented with profound global developmental delay, microcephaly, cortical blindness and axial hypotonia with appendicular hypertonia. Neither sibling ever developed head control, independent sitting or ambulation, and never developed speech. The elder sister had a seizure disorder. Both sisters had profound microcephaly and distinct facial features. On serial brain imaging, they had progressive atrophy of the corpus callosum and supratentorial brain, and diffuse hypomyelination with progressive loss of myelin signal. Exome sequencing revealed both siblings to be homozygous for a c.218dupT (p.His75Serfs*2) mutation in exon 3 of CSTB. The neuroimaging features of our patients are consistent with those observed in Cstb-knockout mice, which supports the hypothesis that disease severity is inversely correlated with the amount of residual functional cystatin B protein.

Transient cortical visual impairment after video-assisted thoracic surgery: a case report.

BACKGROUND: Visual loss associated with thoracic surgery has been reported mostly after coronary angiography or bypass surgery. The position of video-assisted thoracic surgery (VATS) is usually lateral, thus not compressive to the globe. Visual loss after VATS has not been reported. Herein we report a patient without any cardiovascular risk factors who experienced transient cortical blindness after an uneventful VATS. CASE PRESENTATION: A 40-year-old man noticed a visual loss at the recovery room after VATS. He showed normal pupillary reflex, normal optic disc appearance, and homonymous hemianopia respecting the vertical meridian, thus was typical for cortical visual impairment. CONCLUSIONS: Transient cortical visual impairment could be encountered after an uneventful VATS in a patient without any cardiovascular risk factors.

Transient Loss of Vision after Coronary Angiogram - A Case Report.

A 50-year old, diabetic, hypertensive patient with post-CABG status developed complete loss of vision about one hour after coronary angiogram (CAG). Thorough ophthalmological and neurological examination as well as magnetic resonance imaging of brain especially of the occipital region revealed no abnormality. The patient had complete recovery of vision about 48 hours later. We could not document any specific cause or mechanism for the visual loss, although the selective vulnerability of occipital lobes to contrast agent toxicity (Cortical blindness) was the most likely underlying mechanism.

Anton syndrome during oxygen-ozone therapy.

Ozone (O3) gas is a molecule that consists of 3 oxygen atoms, found out in the mid-19th century [1]. Ozone gas preserves humans from detrimental influences of ultraviolet radiation [1]. In spite of harmful effects of O3 gas, investigators think that it has excessive curative effects [1]. Nowadays, O3 therapy is used for many fields of medicine in precise therapeutic doses [1] and [2]. It is known that O3 therapy is helpful in dental procedures, cerebrovascular diseases, tinnitus, acquired immunodeficiency syndrome, hypercholesterolemia, sensorial hypoacusis, senile dementia, multiple sclerosis, irradiation sensitive tumors, herpes simplex and herpes zoster virus infections, muscular hypertonia, and chronic otitis media, etc.[2]. The complications and disadvantages of O3 therapy could be observed in the future. Herein, we presented a case of ischemic stroke after an oxygen-O3 therapy, which is called also Anton syndrome.

Acute visual loss with ophthalmoplegia after spinal surgery: report of a case and review of the literature.

We report a case of a 15-year-old boy who presented with profound visual loss and complete ophthalmoplegia after an uneventful spinal surgery for removal of cervical osteoblastoma. Postoperative visual loss following nonocular surgery is, fortunately rare, yet a devastating complication. The most common cause is ischemic optic neuropathy, but it can also be due to central retinal occlusion or cortical blindness. Visual loss in conjunction with ophthalmoplegia is rarely seen, and there are very few reports in the literature. We also review the related literature and highlight the mechanism and preventive measures.

Transient cortical blindness after coronary angiography: a case report and literature review.

Transient cortical blindness after coronary angiography is a rarely encountered, rapidly progressive complication with complete return of vision within hours to up to five days.. Patients at risk include those undergoing coronary artery bypass graft study and those with renal failure. Although, the exact mechanism is not clear, the most likely explanation is a breakdown of blood brain barrier with direct neurotoxicity of the contrast media as most reported patients underwent coronary bypass graft study. We report a case of transient cortical blindness following diagnostic coronary angiography in a 39-year old patient with spontaneous recovery of vision within 1 hour.

Vision loss as a complication of nonophthalmologic surgery: implications for care for the perianesthesia nurse.

Postoperative vision loss not associated with ophthalmic surgery is a topic that has emerged in recent literature. Although presentation of this complication is rare, perianesthesia nurses should update their knowledge base to include knowledge of these devastating events. A review of common ocular events associated with postoperative vision loss, and the symptoms, assessment, management, and treatment of these events are indicated. The aim of this article is to explore the process of postoperative vision loss and present useful strategies to nurses for optimization of perianesthesia care.

Reversible cortical blindness: posterior reversible encephalopathy syndrome.

Cortical blindness is defined as visual failure with preserved pupillary reflexes in structurally intact eyes due to bilateral lesions affecting occipital cortex. Bilateral oedema and infarction of the posterior and middle cerebral arterial territory, trauma, glioma and meningioma of the occipital cortex are the main causes of cortical blindness. Posterior reversible encephalopathy syndrome (PRES) refers to the reversible subtype of cortical blindness and is usually associated with hypertension, diabetes, immunosuppression, puerperium with or without eclampsia. Here, 3 cases of PRES with complete or partial visual recovery following treatment in 6-month follow-up are reported.

Cysticercal encephalitis with cortical blindness.

The authors report a 6-year-old boy, who had presented with low-grade fever, altered sensorium, headache and seizure for 5 days. On examination, he had features of raised intracranial pressure with left VI cranial-nerve palsy and bilateral extensor plantar response. CT scan showed multiple calcifications in cerebral cortex. MRI cranium showed multiple cysts involving whole of the brain. He was diagnosed as having cysticercal encephalitis, based on immunological and imaging study. He was managed with 20% mannitol, phenytoin and albendazole, and regained consciousness 7 days later, but had residual neurological deficit as left-lower-limb monoparesis and visual acuity of just projection of rays (PR+) and perception of light (PL+).

Transient cortical blindness following vertebral angiography in a young adult with cerebellar haemangioblastoma.

Transient cortical blindness is reported to occur in 0.3% to 1% of cerebral angiography procedures. It develops within minutes of contrast medium injection and lasts for up to several days. We report a long episode of transient cortical blindness in a 17-year-old boy with cerebellar haemangioblastoma, which started during the preoperative vertebral angiography and lasted for 5 days. CT performed 2 days after the sudden onset of bilateral visual loss showed multiple asymmetrical lesions within the brain parenchyma in the distribution of the posterior cerebral circulation. Even though the patient's vision was completely restored 5 days after angiography, repeat MRI performed 2 months after angiography showed improvement but with residual lesions in the thalami, cerebellum and occipital lobe.

Surgical considerations in the management of falcotentorial meningiomas: advantages of the bilateral occipital transtentorial/transfalcine craniotomy for large tumors.

OBJECTIVE: Meningiomas arising from the falcotentorial junction are relatively rare, and the description of the surgical nuances in approaching these tumors is limited. We describe our surgical management of these lesions in detail. METHODS: From 2001 to 2005, 9 patients underwent operation for meningiomas arising from the falcotentorial junction, with some extending to and/or invading the torcula. All patients were assessed preoperatively with magnetic resonance neuroimaging and cerebral angiography. Furthermore, preoperative embolization was attempted in all cases. A supratentorial/infratentorial torcular craniotomy technique was used in all but 1 of these cases. RESULTS: The average dimensions of the falcotentorial meningiomas were 5.1 x 4.4 x 4.2 cm. The angiograms revealed that these tumors were fed by branches of the internal carotid artery, choroidal arteries, branches of the meningohypophyseal trunk, and branches of the posterior cerebral artery. Preoperative embolization was achieved in only 2 patients. Five patients had gross total resection (Simpson grade 1), and 4 had subtotal resection (Simpson grade 4). Two of the tumors (22%) recurred during a mean follow-up period of 49 months (range, 17-88 months). The most common complication after surgery was cortical blindness, but all postoperative visual deficits had fully recovered at the last follow-up evaluation within several months. CONCLUSION: An excellent outcome can be expected with detailed preoperative neuroimaging and knowledge of the nuances of the surgical technique that we describe in detail in this article.