Congenital opticmeningocele: diagnosis, treatment, and follow-up.

Congenital opticmeningoceles were recently described as intraorbital optic nerve cystic lesions, in which neural tissue is found to be part of the lining of the lesion. We describe the case of an eleven-month-old patient with a right opticmeningocele, followed through seven years. He presented with proptosis and hypotropia since birth and imaging exams disclosed an intraconal cystic lesion. A needle puncture revealed a fluid characteristic of cerebrospinal fluid. Surgical drainage performed through a lid crease approach resulted in recurrence of the proptosis. Surgical excision of the capsule finally was definitive, and pathology revealed an epithelial-lined capsule. Eight months after the capsule excision, a strabismus surgery was performed to improve the right hypotropia. Although visual prognosis of opticmeningoceles is poor, a good cosmetic result can be obtained with a multidisciplinary approach involving orbit and strabismus surgeons. The complete capsule excision is believed to be the preferable surgical option for permanently treating the proptosis once needle puncture and cyst drainage are associated with recurrence. The orbital access for approaching the lesion depends on the location of the cyst and should provide wide exposure to avoid new surgical procedures in young patients.

Optic Disc Edema and Posterior Globe Flattening Secondary to Ocular Hypotony: Case Report and Discussion Regarding Pathophysiology and Clinical Findings.

ABSTRACT: We describe a case of a young female patient presenting with ocular hypotension (4 mm Hg) secondary to cyclodialysis, and optic disc edema (ODE) after a blunt trauma in the right eye (right eye). MRI showed posterior globe flattening of the right eye, drawing our attention to the pathophysiology behind these findings. The combination of ODE and posterior globe flattening, as observed in the present case of ocular hypotony, is known from other conditions such as intracranial hypertension and space-flight neuro-ocular syndrome, pointing to a common pathophysiological mechanism, possibly resulting from axoplasmic stasis at the level of the lamina cribrosa due to a high translaminar pressure difference.

Optociliary Shunt Vessels: Role in Diagnosis and Treatment of Atypical Pseudotumor Cerebri.

A 46-year-old man presented with severe visual loss and optic atrophy associated with optociliary shunt vessels. The diagnostic work-up revealed intracranial hypertension and cerebral venous sinus stenosis, with no evidence of previous thrombosis. In view of the severe visual dysfunction, both eyes were submitted to optic nerve sheath fenestration. After surgery, a regression of collateral vessels was observed in both eyes.

Differentiating Leber Hereditary Optic Neuropathy from Normal-Tension Glaucoma.

Glaucoma is a neurodegenerative disorder characterized by thinning of neuroretinal rim, enlarged cup-to-disc ratio (CDR) and visual field damage. Although raised intraocular pressure is main risk factor for development of glaucoma, it can occur with consistently normal measurements in the intraocular pressure as normal tension glaucoma (NTG). Enlargement of CDR is a classical sign of glaucoma, but it can also result from non-glaucomatous optic neuropathies such as Leber hereditary optic neuropathy (LHON). We describe a case of LHON with increased CDR, discuss its differential diagnosis with NTG and highlight the reasons for misdiagnoses between these two entities.

Leber Hereditary Optic Neuropathy with Interval of Visual Loss Greater Than 12 Months.

A 28-year-old man presented with severe left visual loss and normal right visual acuity. The left fundus examination showed temporal pallor and complete absence of the nerve fibre layer (NFL) of papillomacular bundle. Right fundus examination showed focal loss of inferotemporal NFL. Magnetic resonance and serum aquaporin-4 antibody were negative. After 14 months of the initial visual involvement, the patient suffered subacute visual loss in contralateral eye. Genetic study revealed the 11778 point mitochondrial DNA (mtDNA) mutation associated with Leber hereditary optic neuropathy (LHON). Although very rare, interval of involvement of second eye greater than 12 months can occurs in LHON. Detailed optic nerve examination and careful interpretation of optical coherence tomography (OCT) printout support the diagnosis.

Anti-MOG (Myelin Oligodendrocyte Glycoprotein)-Positive Severe Optic Neuritis with Optic Disc Ischaemia and Macular Star.

A 44-year-old man presented with severe right visual loss. The right fundus examination showed marked optic disc oedema associated with partial macular star. Serological blood tests for infectious agents were all negative. Serum aquaporin-4 antibody was negative but anti-MOG (myelin oligodendrocyte glycoprotein) was positive. Magnetic resonance revealed extensive lesion in right optic nerve. There was no visual improvement after intravenous therapy. Patient had no further attacks after follow-up. Optic disc oedema with macular star is found in several infectious and non-inflammatory disorders, but it has not been reported in optic neuritis (ON) associated with autoantibodies to myelin oligodendrocyte glycoprotein (anti-MOG).

BARIATRIC SURGERY AS A TREATMENT FOR IDIOPATHIC INTRACRANIAL HYPERTENSION IN A MALE ADOLESCENT: CASE REPORT.

OBJECTIVE: To describe a case of a male adolescent with symptomatic idiopathic intracranial hypertension (IIH) associated with obesity treated with bariatric surgery. CASE DESCRIPTION: A 16-year-and-6-month-old severely obese boy [weight: 133.6 kg; height: 1.74 m (Z score: +0.14); BMI: 44.1 kg/m2 (Z score: +4.4)], Tanner pubertal stage 5, presented biparietal, high-intensity, and pulsatile headaches, about five times per week, associated with nocturnal awakenings, and partial improvement with common analgesics, for three months. Ophthalmologic evaluation evidenced bilateral papilledema. Cranial computed tomography revealed no mass or anatomic abnormalities. Lumbar puncture showed increased intracranial pressure of 40 cmH2O (reference value: <28 cmH2O) with a normal content. After being diagnosed with IIH, the patient was started on acetazolamide. However, after three months, he was still symptomatic. He was diagnosed with obesity due to excess energy intake and, as he had failed to lose weight after a conventional clinical treatment, bariatric surgery was indicated. The patient (at 16 years and nine months) underwent an uncomplicated laparoscopic sleeve gastrectomy. Ophthalmologic evaluation, performed five months after surgery, revealed normal visual acuity in both eyes and improvement of bilateral papilledema. Follow-up at 18 months showed a 67.5% loss of excess weight (weight: 94.5 kg and BMI: 31.2 kg/m2) and complete resolution of IIH symptoms. COMMENTS: IIH is characterized by increased intracranial pressure with no evidence of deformity or obstruction of the ventricular system on neuroimaging. It has been associated with obesity. Bariatric surgery may be a valid alternative approach for morbidly obese adolescent patients with refractory symptoms.

Recurrent idiopathic neuroretinitis as a spectrum of atypical optic neuritis: a case report and literature review.

Recurrent idiopathic neuroretinitis is an inflammatory optic neuropathy characterized by optic nerve edema and macular star, associated with painless and recurrent episodes of visual loss, poor visual prognosis, and visual field defects related to nerve fiber layer injury. The disorder is sometimes mistaken for atypical optic neuritis. However, early diagnosis is important for visual recovery. Long-term immunosuppression has been shown to reduce the rate of recurrence and protect against severe and irreversible vision loss.

Frequency doubling perimetry in patients with mild and moderate pituitary tumor-associated visual field defects detected by conventional perimetry.

PURPOSE: To test the ability of frequency doubling technology (FDT) perimetry to identify pituitary tumor-associated visual field defects. METHODS: Twenty-three eyes from patients with pituitary tumor (PT) and 28 normal eyes were studied. Subjects were eligible when presenting with normal visual acuity and mild or moderate temporal field loss in at least one eye on Goldmann and standard automated perimetry (SAP). FDT testing was performed using the C-20-5 screening and the C-20 full-threshold test. The sensitivity and specificity for identification of the field defect were determined and the groups were compared with regard to several parameters. Finally, we compared the ability of FDT and SAP to detect the characteristic pattern of temporal hemianopia/quadrantanopia. RESULTS: In the screening test, sensitivity ranged from 21.74% to 43.48% while specificity was 100%. In the threshold test, sensitivity ranged from 52.17% to 82.61% (specificities of 85.71% and 60.71%, respectively), according to total deviation analysis, and from 30.43% to 73.91% (specificities of 96.42% and 64.28%, respectively), according to the pattern deviation analysis. Patients with PT presented a significantly higher number of abnormal points in the temporal hemifield. In 12 eyes FDT and SAP were both able to identify the characteristic pattern of visual field defect; in 4 eyes FDT performed better than SAP; in 4 eyes, SAP performed better than FDT, while in 3 neither test was able to determine the pattern of visual field defect correctly. CONCLUSIONS: Threshold FDT perimetry seems to be a sensitive instrument for identifying abnormality in eyes with chiasmal compression-induced field defects detected on conventional perimetry.

Extraocular muscle cysticercosis mimicking idiopathic orbital inflammation: case report.

Presentation of one case of extraocular muscle enlargement caused by cysticercosis, its clinical, diagnostic and treatment aspects, and review of the literature on this theme. A female 38-year-old patient with extraocular muscle enlargement and a small cystic lesion at the superior rectus muscle insertion was treated with oral prednisone for almost one year, with a non-specific inflammation of right orbit diagnosis. There were important ocular motility restriction and pain. Computerized tomography disclosed a superior rectus muscle thickening with a small cystic and apparently empty lesion at the muscle's insertion. Excisional biopsy and histopathological study confirmed the clinical suspicion of cysticercosis. There was partial resolution of the restricted motility. Extraocular muscle cysticercosis is the most common site of this disease when involving the orbit. Oral albendazole and prednisone are efficient, but a long history of disease can lead to important residual ocular motility restriction.

Seesaw nystagmus caused by giant pituitary adenoma: case report.

Giant pituitary adenomas are uncommonly large tumors, greater than 4 cm in size that can produces endocrine symptoms, visual loss and cranial nerve palsies. We report the rare occurrence of seesaw nystagmus as the presenting sign of giant pituitary adenoma. A 50-year-old man presented with headache associated with visual loss and seesaw nystagmus. Perimetry revealed bitemporal hemianopia and magnetic resonance imaging showed a giant pituitary adenoma. After surgery, nystagmus disappeared. Our case is relevant in understanding its pathogenesis since it documents seesaw nystagmus in a patient bitemporal hemianopia due to a large tumor but without mesencephalic compression.

[Comparison of scanning laser polarimetry, optical coherence tomography 1 and Stratus optical coherence tomography for the detection of axonal loss in band atrophy of the optic nerve]. / Comparação entre o polarímetro de varredura a laser, a tomografia de coerência óptica 1 e o Stratus-oct na detecção da perda axonal da atrofia em banda do nervo óptico.

PURPOSE: To compare the abilitiy of scanning laser polarimetry (GDx), optical coherence tomography (OCT) 1 and Stratus- optical coherence tomography to discriminate between healthy eyes and eyes with band atrophy of the optic nerve and temporal hemianopsia. METHODS: Twenty-three eyes with band atrophy of the optic nerve and 23 eyes from healthy subjects were included in this observational prospective case-control study. All eyes underwent retinal nerve fiber layer (RNFL) thickness analysis using GDx, optical coherence tomography-1 and Stratus-optical coherence tomography. Mean values obtained with each equipment were compared between band atrophy and normal eyes. Receiver operating characteristic (ROC) curves and sensitivities at fixed specificities (80% e 95%) were calculated for each parameter calculated with each equipment and compared. RESULTS: When compared with healthy subjects, the parameters corresponding to the global average as well as each of the four quadrant retinal nerve fiber layer thickness in eyes with band atrophy were significantly smaller (p<0.05), than in normal eyes, with the exception of GDx's temporal thickness parameter. Comparison of the areas under ROC curves (AUCs) of the parameters from the three equipments revealed significantly greater values for the Stratus-OCT when compared to the OCT-1 in the global average and in the temporal quadrant thickness measurement. Stratus-OCT was significantly more sensitive than GDx in the global average as well as in the temporal, nasal and inferior quadrant. OCT-1 was superior to GDx only in the temporal quadrant. All three equipments revealed a similar ability to identify retinal nerve fiber layer reduction in the superior quadrant. CONCLUSIONS: The Stratus OCT showed the best ability to discriminate between eyes with band atrophy of the optic nerve and healthy eyes although all three equipments were able do identify most of the abnormal eyes. OCT-1 was inferior to Stratus-OCT and superior do GDx in the temporal quadrant. Although GDx was able identify some abnormality in most studied eyes, the equipment showed poor sensitivity in the detection of axonal loss in the nasal and temporal aspect of the optic disc and should be used with caution in neuro-ophthalmic diseases that cause retinal nerve fiber layer loss in those areas of the optic disc.

Retinal nerve fiber layer loss documented by Stratus OCT in patients with pituitary adenoma: case report.

PURPOSE: To report abnormalities of retinal nerve fiber layer (RNFL) thickness using optical coherence tomography (Stratus OCT) in patients with pituitary adenoma. METHODS: Two patients with long-standing bitemporal visual field defects and optic nerve band atrophy were submitted to optical coherence tomography examination (Stratus OCT). RESULTS: Both patients with band atrophy revealed diffuse loss of the retinal nerve fiber layer on Stratus OCT, with severe reduction in the nasal and temporal areas of the optic disc. Retinal nerve fiber layer loss correlated well with visual field loss and with previous histological studies of band atrophy of the optic nerve. CONCLUSIONS: Stratus optical coherence tomography can provide useful information in the diagnosis of band atrophy from chiasmal lesions such as pituitary adenomas.

[Cranial idiopathic hypertrophic pachymeningitis associated with orbital pseudotumor: case report]. / Paquimeningite hipertrófica idiopática craniana associada a pseudotumor orbitário: relato de caso.

Hypertrophic pachymeningitis is a rare disorder characterized by meningeal thickness, that can be caused by infection, tumoral infiltration, inflammatory disorders or idiopathic. We report the case of a 40 year-old man that presented with longstanding headache and progressive bilateral visual loss and proptosis. Cranial and orbital magnetic resonance imaging revealed diffuse dural thickness and bilateral extraconal orbital lesion. Extensive investigation did not reveal any systemic condition. Histopathological study after meningeal and orbital biopsy disclosed a chronic inflammatory process compatible respectively with idiopathic hypertrophic pachymeningitis (IHP) and orbital pseudotumor. This case emphasizes that orbital involvement can occur in IHP and that its early identification is of great importance in order to improve the visual prognosis of this condition.

[Geniculate hemianopia. Diagnostic importance of retinal nerve fiber layer analysis using optical coherence tomography: case report]. / Hemianopsia por lesão no corpo geniculado lateral. Importância diagnóstica da análise da camada de fibras nervosas pela tomografia por coerência óptica: relato de caso.

Lesions of the lateral geniculate body (LGB) are the most unusual lesions of the visual pathways. Imaging studies are very important in establishing the correct diagnosis. However, due to its small size and particular location, the lateral geniculate body and its lesions are sometimes difficult to detect in imaging studies possibly causing diagnostic confusion. The purpose of this paper is to document an unusual case of a lesion of the lateral geniculate body for which an optical coherence tomography study was very important in confirming the anatomic diagnosis of a lateral geniculate body lesion. A 39-year-old woman with a previous diagnosis of uveitis and central nervous system vasculitis was referred for investigation of a right temporal quadrantanopia. She had already been submitted to a magnetic resonance imaging (MRI) that did not show any lesion along the visual pathway. Ophthalmoscopy revealed retinal nerve fiber layer (RNFL) loss that was confirmed by optical coherence tomography. Such finding associated with the observations on the neurological examination strongly suggested a lateral geniculate body lesion. The patient was submitted to another new magnetic resonance imaging obtained with especially oriented thin sections and an ischemic lesion of the lateral geniculate body was observed establishing the correct diagnosis. This case serves to confirm the importance of optical coherence tomography in determining the pattern of retinal nerve fiber layer loss in neuro-ophthalmic diseases and therefore to help in locating a lesion along the visual pathway.

[A comparative study between the results of antro-ethmoidal orbital decompression isolated and associated with orbital fat removal]. / Comparação entre os resultados da descompressão orbitária antro-etmoidal isolada e associada à remoção de tecido adiposo.

PURPOSE: To compare the results of isolated antral-ethmoidal orbital decompression and that associated with orbital fat removal in patients with dysthyroid orbitopathy. METHODS: Nineteen isolated antral-ethmoidal orbital decompressions were performed in a group of 12 patients (19 orbits) with the diagnosis of dysthyroid ophthalmopathy in the quiescent stage. The same procedure, but in association with orbital fat removal, were performed in another group of 8 patients (10 orbits) also with the diagnosis of dysthyroid ophthalmopathy in the quiescent stage. The results of both groups were compared. RESULTS: In the first group the amount of retroplacement of the globe achieved 1-5 mm (mean 3.68+/-1.10 mm) and in the second group it achieved 1-5 mm (mean 3.25+/-1.36 mm). CONCLUSIONS: There was no significant difference between the results of the isolated antral-ethmoidal orbital decompression and that associated with orbital fat removal. The removal of 1 ml of seems not to improve the retroplacement of the globe as an association with a 2-wall orbital decompression. However, many conditions must be considered to conclude any comparison between the two orbital decompression techniques, such as orbital soft tissue compliance, amount of fat removal and size of bone opening.

[Severe bilateral visual loss as the presenting sign of cerebral venous sinus thrombosis: case report]. / Perda visual bilateral grave como sinal de apresentação de trombose de seio venoso cerebral: relato de caso.

Our purpose is to report a case of cranial venous thrombosis causing pseudotumor syndrome, where the principal clinical manifestation was severe bilateral vision loss. A 24-year-old man presented with severe bilateral vision loss, transient obscuration of vision, headache, bilateral papilledema and constricted visual fields. Computed tomography as well as neurologic examination was unrevealing. A lumbar puncture disclosed an increased cerebrospinal fluid opening pressure and complete blood count (CBC) showed increased numbers of platelets. A brain magnetic resonance imaging disclosed characteristic sings of chronic papilledema and suggestive signs of superior sagittal sinus thrombosis. A cranial magnetic resonance venography disclosed thrombosis of superior sagittal, transverse and sigmoid sinuses. The patient was treated with an optic nerve sheath decompression in the left eye, acetazolamide and anticoagulation. His ocular evaluation showed reduction of the papilledema, but severe vision loss remained. Pseudotumor cerebri with severe vision loss can be the presenting sign of cranial venous thrombosis secondary to a potentially life-threatening hematologic disorder.

Bariatric surgery as a treatment for idiopathic intracranial hypertension in a male adolescent: case report / Cirurgia bariátrica como tratamento de pseudotumor cerebral primário em adolescente do sexo masculino: relato de caso

ABSTRACT Objective: To describe a case of a male adolescent with symptomatic idiopathic intracranial hypertension (IIH) associated with obesity treated with bariatric surgery. Case description: A 16-year-and-6-month-old severely obese boy [weight: 133.6 kg; height: 1.74 m (Z score: +0.14); BMI: 44.1 kg/m2 (Z score: +4.4)], Tanner pubertal stage 5, presented biparietal, high-intensity, and pulsatile headaches, about five times per week, associated with nocturnal awakenings, and partial improvement with common analgesics, for three months. Ophthalmologic evaluation evidenced bilateral papilledema. Cranial computed tomography revealed no mass or anatomic abnormalities. Lumbar puncture showed increased intracranial pressure of 40 cmH2O (reference value: <28 cmH2O) with a normal content. After being diagnosed with IIH, the patient was started on acetazolamide. However, after three months, he was still symptomatic. He was diagnosed with obesity due to excess energy intake and, as he had failed to lose weight after a conventional clinical treatment, bariatric surgery was indicated. The patient (at 16 years and nine months) underwent an uncomplicated laparoscopic sleeve gastrectomy. Ophthalmologic evaluation, performed five months after surgery, revealed normal visual acuity in both eyes and improvement of bilateral papilledema. Follow-up at 18 months showed a 67.5% loss of excess weight (weight: 94.5 kg and BMI: 31.2 kg/m2) and complete resolution of IIH symptoms. Comments: IIH is characterized by increased intracranial pressure with no evidence of deformity or obstruction of the ventricular system on neuroimaging. It has been associated with obesity. Bariatric surgery may be a valid alternative approach for morbidly obese adolescent patients with refractory symptoms.

RESUMO Objetivo: Descrever um caso de cirurgia bariátrica como tratamento de pseudotumor cerebral primário (PTCP) em adolescente do sexo masculino com obesidade. Descrição do caso: Adolescente, sexo masculino, 16 anos e 6 meses, com obesidade exógena [peso:133,6 kg; estatura:1,74 m (escore z: +0,14); IMC: 44,1 kg/m2 (escore z: +4,4)], estadiamento puberal de Tanner 5, apresentando cefaleia bi-parietal, pulsátil e de alta-intensidade, cerca de cinco vezes por semana, associada a despertares noturnos, e com melhora parcial com analgésicos comuns, há três meses. A avaliação oftalmológica evidenciou papiledema bilateral e a tomografia computadorizada de crânio não revelou massas ou alterações anatômicas. A punção lombar mostrou pressão intracraniana elevada de 40 cmH2O (Referência: <28 cmH2O) com conteúdo normal. Feito o diagnóstico, o paciente foi iniciou uso de acetazolamida. No entanto, após 3 meses, o paciente mantinha-se sintomático. Ele foi diagnosticado com obesidade devido ao consumo calórico excessivo e, como não havia obtido sucesso na perda de peso com tratamento clínico convencional, a cirurgia bariátrica foi indicada. Aos 16 anos e 9 meses, o paciente foi submetido a gastrectomia vertical laparoscópica sem complicações. A avaliação oftalmológica, cinco meses após a cirurgia, revelou melhora do papiledema bilateral com acuidade visual normal em ambos os olhos. Apresentou perda de excesso de peso de 67,5% (peso: 94,5 kg e IMC:31,2 kg/m2) e resolução completa dos sintomas de PPTC 18 meses após a cirurgia. Comentários: O PTCP é caracterizado pelo aumento da pressão intracraniana, sem evidência de deformidade ou obstrução do sistema ventricular na neuroimagem. Está associado à obesidade. A cirurgia bariátrica pode ser uma alternativa terapêutica válida para pacientes adolescentes obesos graves com sintomas refratários.

Recurrent idiopathic neuroretinitis as a spectrum of atypical optic neuritis: a case report and literature review / Neurorretinite recorrente idiopática como espectro da neurite óptica atípica: relato de caso e revisão da literatura

ABSTRACT Recurrent idiopathic neuroretinitis is an inflammatory optic neuropathy characterized by optic nerve edema and macular star, associated with painless and recurrent episodes of visual loss, poor visual prognosis, and visual field defects related to nerve fiber layer injury. The disorder is sometimes mistaken for atypical optic neuritis. However, early diagnosis is important for visual recovery. Long-term immunosuppression has been shown to reduce the rate of recurrence and protect against severe and irreversible vision loss.

RESUMO Neurorretinite recorrente idiopática é uma neuropatia óptica inflamatória caracterizada por edema do nervo óptico e estrela macular associada a episódios recorrentes de perda visual indolor, baixo prognóstico visual e desfeitos de campo visual relacionados a injúria da camada de fibras nervosas. Essa condição pode ser confundida com neurite óptica atípica e seu correto diagnóstico é importante para o prognóstico visual, uma vez que a imunossupressão continua previne episódios recorrentes que podem levar a perda visual severa e irreversível.