RESUMEN
We aimed to investigate the changes in cupping in chiasmal lesion optic neuropathy (chON) compared to baseline optic disc and glaucoma. We used a novel study design to enroll patients who had fundus photographs incidentally taken during routine health check-ups prior to the onset of optic neuropathy. In 31 eyes (21 patients) with chON and 33 eyes (30 patients) with glaucoma, we investigated the change in cup-to-disc (C/D) area from the baseline to overt cupping using flicker analysis. Compared to the baseline, 23 eyes (74.2%) had increased cup size and 3 (9.7%) had vascular configuration changes in the chONgroup; in contrast, all glaucoma eyes exhibited changes in cup size and vascular configuration. The increase in C/D area ratio was significantly smaller in chON (0.04 ± 0.04) compared to glaucoma (0.10 ± 0.04, P < 0.001); the minimum residual neuroretinal rim width showed a more pronounced difference (29.7 ± 8.2% vs 7.1 ± 3.9%, P < 0.001). The changes distributed predominantly towards the nasal direction in chON, contrasting the changes to the arcuate fibers in glaucoma. In conclusion, our results provide the first longitudinal evidence of true pathological cupping in chONcompared to photographically disease-free baseline. The marked difference in the residual minimum rim width reaffirms the importance of rim obliteration in the differential diagnosis between the two diseases.
Asunto(s)
Glaucoma , Disco Óptico , Enfermedades del Nervio Óptico , Humanos , Disco Óptico/patología , Glaucoma/patología , Enfermedades del Nervio Óptico/patología , Quiasma Óptico/patología , Fondo de Ojo , Presión IntraocularRESUMEN
Craniopharyngiomas are histologically benign tumors that originate from squamous rests along the pituitary stalk. They make up approximately 1.2% to 4.6% of all intracranial tumors and do not show significant differences in occurrence based on sex. Adamantinomatous craniopharyngiomas have 2 peaks of incidence, commonly observed in patients from ages 5 to 15 years and again from 45 to 60 years. In contrast, papillary craniopharyngiomas mainly affect adults in their fifth and sixth decades of life.1 The "malignancy" of craniopharyngiomas is attributed to their location and the challenges associated with achieving complete removal because they can manifest in the sellar, parachiasmatic, and intraventricular regions or a combination of these.2,3 Various approaches have been used to resect these tumors.4,5 Radical resection offers the most promising option for disease control, potential cure, and the ability to transform the disease from lethal to survivable in children, allowing for a functional adult life.2,3 Meticulous evaluation is crucial to determine the appropriate approach and side, with particular emphasis on closely examining the relationship between the tumor and optic pathways (nerve, chiasm, tract), which are frequently involved. This assessment should also include the tumor's relationship with other crucial structures, such as the hypothalamus and adjacent arteries, to ensure that the strategy is adjusted accordingly to further minimize the risk of postoperative morbidity. Video 1 demonstrates a left-sided pterional transsylvian approach to remove a parachiasmatic craniopharyngioma involving the left optic chiasm and tract.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Adulto , Niño , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Hipófisis/patología , Hipotálamo/patología , Quiasma Óptico/diagnóstico por imagen , Quiasma Óptico/cirugía , Quiasma Óptico/patologíaRESUMEN
Granular cell tumors are rare vascular neoplastic lesions of the sellar and suprasellar region that usually arise from the pituitary stalk but can originate as low as the posterior pituitary or as high as the tuber cinereum.1 Complete resection, although ideal, can yield high rates of endocrine or visual morbidity.1,2 On headache workup, a 66-year-old woman was found to have a 1.2 × 1.1 × 1.3-cm contrast-enhancing lesion in the anterior-inferior third ventricle, posterior to the infundibulum. Endocrine testing was unremarkable, and a lumbar puncture was nondiagnostic. An open biopsy and possible resection were selected by the patient over short-interval imaging. A translamina terminalis approach was selected over a transsphenoidal approach to preserve the third ventricular floor (Video 1). A right frontotemporal craniotomy was performed, including flattening of the lesser sphenoid wing. The optic chiasm was exposed via subfrontal microsurgical dissection, and the lamina terminalis was opened sharply. A firm, vascular tumor was identified extending into the anterior-inferior aspect of the third ventricle. Frozen pathologic analysis was nondiagnostic. Given the proximity of the optic chiasm, a complete piecemeal microsurgical resection was performed, preserving the floor and lateral walls of the third ventricle and optic apparatus. Final pathology was a granular cell tumor. Postoperatively, the patient had transient diabetes insipidus, with preserved vision and normal endocrine function on follow-up. The trans-lamina terminalis approach can be used for safe resection of anterior third ventricular tumors. Preservation of the floor and walls of the third ventricle is critical to avoid morbidity.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Tercer Ventrículo , Anciano , Craneofaringioma/cirugía , Femenino , Humanos , Hipotálamo/diagnóstico por imagen , Hipotálamo/patología , Hipotálamo/cirugía , Quiasma Óptico/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/cirugía , Tercer Ventrículo/diagnóstico por imagen , Tercer Ventrículo/patología , Tercer Ventrículo/cirugíaRESUMEN
OBJECT: Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification. METHODS: We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification. RESULTS: Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests). CONCLUSION: Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system.
Asunto(s)
Procedimientos Neuroquirúrgicos/clasificación , Procedimientos Neuroquirúrgicos/métodos , Neoplasias del Nervio Óptico/clasificación , Neoplasias del Nervio Óptico/cirugía , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Hipotálamo/diagnóstico por imagen , Hipotálamo/cirugía , Lactante , Imagen por Resonancia Magnética/métodos , Masculino , Recurrencia Local de Neoplasia/clasificación , Recurrencia Local de Neoplasia/diagnóstico por imagen , Recurrencia Local de Neoplasia/cirugía , Quiasma Óptico/diagnóstico por imagen , Quiasma Óptico/cirugía , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/cirugía , Glioma del Nervio Óptico/clasificación , Glioma del Nervio Óptico/diagnóstico por imagen , Glioma del Nervio Óptico/cirugía , Neoplasias del Nervio Óptico/diagnóstico por imagen , Estudios RetrospectivosRESUMEN
This two-dimensional video shows the technical nuances of complete microsurgical resection of a hypothalamic craniopharyngioma located in the retrochiasmatic region by the transpetrosal approach. This 49-yr-old man presented with progressive fatigue, excessive sleepiness, and difficulty in vision in both eyes. He was found to have right CN 3 paralysis and bitemporal hemianopsia on neurological examination. Further workup revealed panhypopituitarism. Brain magnetic resonance imaging (MRI) demonstrated a large solid retrochiasmatic hypothalamic lesion with homogeneous contrast enhancement, measuring 2.1 × 2.6 × 2.4 cm. Optic chiasm was prefixed, and the tumor was just posterior to the pituitary stalk area. The preoperative differential diagnosis included hypothalamic astrocytoma, craniopharyngioma, germinoma, and histiocytosis. Because of the prefixed chiasm, a presigmoid, transpetrosal approach was performed. Our initial plan was a large biopsy, but based on frozen section histology, we decided to excise the tumor completely. The tumor had a pseudocapsule, which was firm and yellowish. It was debulked, dissected from the surrounding hypothalamus, and removed completely. The pituitary stalk was found at the anterior and inferior ends of the tumor and was preserved. Postoperatively, the patient developed diabetes insipidus and requires desmopressin replacement, which was gradually tapered. For panhypopituitarism, he is receiving thyroxine, hydrocortisone, and testosterone. Postoperatively, patient had an improvement in vision in his left eye and ptosis was improving in the right eye with mRs 1- at 10-wk follow-up. An informed consent was obtained from the patient prior to the surgery, which included videotaping of the procedure and its distribution for educational purposes. All relevant patient identifiers have also been removed from the video and accompanying radiology slides.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Humanos , Hipotálamo/diagnóstico por imagen , Hipotálamo/cirugía , Masculino , Persona de Mediana Edad , Quiasma Óptico/diagnóstico por imagen , Quiasma Óptico/cirugía , Hipófisis , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugíaAsunto(s)
Glaucoma/complicaciones , Quiasma Óptico/patología , Disco Óptico/patología , Enfermedades del Nervio Óptico/diagnóstico , Enfermedad por Deficiencia del Complejo Piruvato Deshidrogenasa/diagnóstico , Escotoma/etiología , Campos Visuales , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Agudeza Visual/fisiología , Pruebas del Campo VisualRESUMEN
TITLE: Tuberculomas optoquiasmaticos como reaccion paradojica al tratamiento de tuberculosis meningea.
Asunto(s)
Antituberculosos/efectos adversos , Interacciones Huésped-Patógeno , Quiasma Óptico/patología , Tuberculoma/etiología , Tuberculosis Meníngea/patología , Adulto , Antituberculosos/uso terapéutico , Infarto Encefálico/etiología , Errores Diagnósticos , Progresión de la Enfermedad , Farmacorresistencia Microbiana , Sustitución de Medicamentos , Etambutol/efectos adversos , Etambutol/uso terapéutico , Femenino , Humanos , Isoniazida/efectos adversos , Isoniazida/uso terapéutico , Imagen por Resonancia Magnética , Meningitis Viral/diagnóstico , Moxifloxacino/uso terapéutico , Mycobacterium tuberculosis/efectos de los fármacos , Mycobacterium tuberculosis/aislamiento & purificación , Neuroimagen , Quiasma Óptico/diagnóstico por imagen , Paresia/etiología , Prednisona/uso terapéutico , Pirazinamida/efectos adversos , Pirazinamida/uso terapéutico , Rifampin/efectos adversos , Rifampin/uso terapéutico , Tálamo/irrigación sanguínea , Tuberculoma/patología , Tuberculosis Meníngea/líquido cefalorraquídeo , Tuberculosis Meníngea/complicaciones , Tuberculosis Meníngea/tratamiento farmacológico , Trastornos de la Visión/etiologíaRESUMEN
No disponible
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Humanos , Femenino , Niño , Adulto , Fotofobia/complicaciones , Fotofobia/diagnóstico , Retina/lesiones , Tálamo/diagnóstico por imagen , Traumatismos del Nervio Óptico/diagnóstico por imagen , Quiasma Óptico/diagnóstico por imagen , Quiasma Óptico/lesiones , Tálamo/lesiones , Imagen por Resonancia Magnética/métodos , Resonancia Magnética Nuclear Biomolecular/métodos , Corteza Prefrontal/lesionesRESUMEN
Although the beneficial effects of quercetin on oligodendrocyte precursor cell (OPCs) population has been evaluated in-vitro, there are few studies about the effects of quercetin on myelin repair in the context of demyelination. The aim of this study was to investigate the effects of querectin on functional recovery and myelin repair of optic chiasm in lysolecithin (LPC)-induced demyelination model. Demyelination was induced by local injection of LPC 1% (2⯵l) into rat optic chiasm. Querectin at doses 25 or 50â¯mg/kg was administrated daily by oral gavage for 7 or 14 days post LPC. Visual evoked potential (VEPs) recordings were used to assess the functional property of the optic pathway. Immunostaining and myelin staining were performed on brain sections 7 or 14 days post lesion. Electrophysiological data indicated that LPC injection increased the latency of VEPs waves and quercetin effectively reduced the delay of visual signals. The level of glial activation was alleviated in animals under treatment of quercetin compared to vehicle group. Furthermore, quercetin treatment decreased the extent of demyelination areas and increased the remyelination process following LPC injection. Overall, our findings indicate that quercetin could remarkably improve the functional recovery of the optic pathway by its protective effects on myelin sheath and attenuation of glial activation.
Asunto(s)
Enfermedades Desmielinizantes/inducido químicamente , Enfermedades Desmielinizantes/tratamiento farmacológico , Lecitinas/farmacología , Vaina de Mielina/efectos de los fármacos , Quiasma Óptico/efectos de los fármacos , Quercetina/farmacología , Animales , Modelos Animales de Enfermedad , Potenciales Evocados Visuales/efectos de los fármacos , Masculino , Ratas , Ratas WistarRESUMEN
Optic pathway/hypothalamic gliomas (OP/HGs) are rare astrocytic tumors that appear more commonly among young children and often are unresectable. They comprise approximately 2% of all central nervous system tumors and account for 3-5% of pediatric intracranial tumors. Initial manifestations are often visual disturbances, endocrinopathies and hypothalamic dysfunction such as the diencephalic syndrome, and sometimes hydrocephalus due to cerebrospinal fluid (CSF) outflow obstruction. In many cases, the tumors are diagnosed late in the clinical course because they silently enlarge. These tumors consist mostly of histologically benign, World Health Organization (WHO) grade I tumors represented by pilocytic astrocytomas (PA), the rest being pilomyxoid astrocytomas (PXA) - WHO grade II tumors. In young pediatric patients, however, can be seen PXA that show aggressive clinical course such as CSF dissemination. Our small series of 14 non-Neurofibromatosis type 1 (NF-1) OP/HGs PA patients underwent extended resection without any adjuvant treatments. The median age at initial treatment was 11.5 ± 6.90 years (range, 1-25 years) and median follow up 85.5 ± 25.0 months. Surgical resection for OP/HGs results in acceptable middle-term survival, tumor control and functional outcome equivalent to chemotherapy. There is, however, no longer doubt that chemotherapy with or without biopsy and as-needed debulking surgery remains the golden standard in management of OP/H. Clinical conditions and treatment plans for OP/HGs vary depending on their structure of origin.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Glioma/diagnóstico , Glioma/terapia , Hipotálamo , Tracto Óptico , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Quiasma Óptico , Adulto JovenRESUMEN
Craniopharyngiomas are benign, partly cystic epithelial tumors that can rarely occur in a retrochiasmatic location with involvement of the third ventricle. The lamina terminalis is an important neurosurgical corridor to these craniopharyngiomas in the anterior portion of the third ventricle. We present a video case of a large midline suprasellar and third ventricular craniopharyngioma in a 32-year-old male with visual disturbances. The tumor was approached with a subfrontal translamina terminalis exposure, and a gross-total resection of the tumor was achieved. This surgery involved working through a lamina terminalis fenestration around the optic nerve, optic chiasm, optic tracts, and the anterior communicating artery complex. This video illustrates the techniques employed in performing a transbasal anterior skull base approach to the third ventricle and demonstrates vivid surgical anatomy of neurovascular structures around the lamina terminalis. The video can be found here: https://youtu.be/fCYMgx8SnKs .
Asunto(s)
Craneofaringioma/cirugía , Procedimientos Neuroquirúrgicos , Quiasma Óptico/cirugía , Neoplasias Hipofisarias/cirugía , Tercer Ventrículo/cirugía , Adulto , Craneofaringioma/diagnóstico , Humanos , Hipotálamo/cirugía , Masculino , Procedimientos Neuroquirúrgicos/métodos , Neoplasias Hipofisarias/diagnóstico , Base del Cráneo/cirugíaRESUMEN
Retrochiasmatic craniopharyngiomas involving the anterior third ventricle are challenging to access. Although the pterional approach is a common route for suprasellar lesions, when the craniopharyngioma extends behind the chiasma into the third ventricle, access is even more difficult, and the lamina terminalis may offer a good working window. The translamina terminalis approach provides direct access to the retrochiasmatic portion of the tumor with minimal brain retraction and no manipulation of the visual nerves. In this video, we emphasize the utility of using the lamina terminalis corridor to resect the retrochiasmatic intraventricular portion of a craniopharyngioma. The video can be found here: https://youtu.be/hrLNC0hDKe4 .
Asunto(s)
Neoplasias del Ventrículo Cerebral/cirugía , Craneofaringioma/cirugía , Hipotálamo/cirugía , Procedimientos Neuroquirúrgicos , Neoplasias Hipofisarias/cirugía , Tercer Ventrículo/cirugía , Neoplasias del Ventrículo Cerebral/diagnóstico , Craneofaringioma/diagnóstico , Humanos , Procedimientos Neuroquirúrgicos/métodos , Quiasma Óptico/cirugíaRESUMEN
Chordoid glioma of the third ventricle is a rare and challenging tumor to surgery because of its unique anatomical location and its close juxtaposition to the neurovascular structures and hypothalamus. The authors report a case of chordoid glioma of the third ventricle in a 43-year-old woman, who presented with headache and somnolence. The tumor was approached by endoscopic transnasal technique with a favorable result. Histopathologic examination disclosed a neoplastic tissue composed of eosinophilic epithelioid cells, mucinous, periodic acid Schiff-diastase positive, extracellular matrix, and scattered lymphoplasmacytic infiltrates. The best treatment option remains controversial. Customarily, the surgical route to remove chordoid glioma is transcranial; however, the undersurface of the optic chiasm and optic nerves preclude an adequate surgical visualization. In contrast, an expanded endoscopic transnasal approach provides a direct midline corridor to this region without any brain retraction.
Asunto(s)
Adulto , Femenino , Humanos , Encéfalo , Eosinófilos , Células Epitelioides , Matriz Extracelular , Glioma , Cefalea , Hipotálamo , Mucinas , Quiasma Óptico , Nervio Óptico , Ácido Peryódico , Tercer VentrículoRESUMEN
Chiasmatic-hypothalamic masses are commonly seen in children with neurofibromatosis type 1 and often demonstrate a benign clinical course. These masses are, however, rare in adults and require tissue sampling for diagnosis. Here, we present four cases of chiasmatic-hypothalamic masses in adults with pathologically proven diagnoses. We also provide a review of the literature and propose a limited differential in this age group.
Asunto(s)
Neoplasias Encefálicas/patología , Glioma/patología , Hipotálamo/patología , Imagen por Resonancia Magnética/métodos , Quiasma Óptico/patología , Neoplasias del Nervio Óptico/patología , Adulto , Anciano , Diagnóstico Diferencial , Humanos , Masculino , Persona de Mediana EdadAsunto(s)
Glioma/complicaciones , Isquemia/etiología , Quiasma Óptico , Neoplasias del Nervio Óptico/complicaciones , Nervio Óptico/irrigación sanguínea , Diagnóstico Diferencial , Glioma/diagnóstico , Humanos , Isquemia/diagnóstico , Imagen por Resonancia Magnética , Neoplasias del Nervio Óptico/diagnósticoAsunto(s)
Encefalitis/microbiología , Enfermedades Hipotalámicas/microbiología , Infecciones por Mycobacterium , Mycobacterium haemophilum , Quiasma Óptico , Enfermedades del Nervio Óptico/microbiología , Síndrome de Inmunodeficiencia Adquirida/complicaciones , Síndrome de Inmunodeficiencia Adquirida/patología , Adulto , Encefalitis/patología , Humanos , Enfermedades Hipotalámicas/patología , Hipotálamo/patología , Imagen por Resonancia Magnética , Masculino , Infecciones por Mycobacterium/microbiología , Infecciones por Mycobacterium/patología , Quiasma Óptico/patología , Enfermedades del Nervio Óptico/patología , Linfocitos T/patología , Vías Visuales/patologíaRESUMEN
BACKGROUND: Neuroinflammation has been proven to play a crucial role in early brain injury pathogenesis and represents a target for treatment of subarachnoid hemorrhage (SAH). Astaxanthin (ATX), a dietary carotenoid, has been shown to have powerful anti-inflammation property in various models of tissue injury. However, the potential effects of ATX on neuroinflammation in SAH remain uninvestigated. The goal of this study was to investigate the protective effects of ATX on neuroinflammation in a rat prechiasmatic cistern SAH model. METHODS: Rats were randomly distributed into multiple groups undergoing the sham surgery or SAH procedures, and ATX (25 mg/kg or 75 mg/kg) or equal volume of vehicle was given by oral gavage at 30 min after SAH. All rats were sacrificed at 24 h after SAH. Neurologic scores, brain water content, blood-brain barrier permeability, and neuronal cell death were examined. Brain inflammation was evaluated by means of expression changes in myeloperoxidase, cytokines (interleukin-1ß, tumor necrosis factor-α), adhesion molecules (intercellular adhesion molecule-1), and nuclear factor kappa B DNA-binding activity. RESULTS: Our data indicated that post-SAH treatment with high dose of ATX could significantly downregulate the increased nuclear factor kappa B activity and the expression of inflammatory cytokines and intercellular adhesion molecule-1 in both messenger RNA transcription and protein synthesis. Moreover, these beneficial effects lead to the amelioration of the secondary brain injury cascades including cerebral edema, blood-brain barrier disruption, neurological dysfunction, and neuronal degeneration. CONCLUSIONS: These results indicate that ATX treatment is neuroprotective against SAH, possibly through suppression of cerebral inflammation.
Asunto(s)
Neuritis/tratamiento farmacológico , Fármacos Neuroprotectores/farmacología , Hemorragia Subaracnoidea/tratamiento farmacológico , Animales , Barrera Hematoencefálica/efectos de los fármacos , Edema Encefálico/tratamiento farmacológico , Edema Encefálico/inmunología , Edema Encefálico/metabolismo , Muerte Celular/efectos de los fármacos , Modelos Animales de Enfermedad , Interleucina-1beta/metabolismo , Masculino , FN-kappa B/metabolismo , Neuritis/inmunología , Neuritis/metabolismo , Quiasma Óptico/efectos de los fármacos , Quiasma Óptico/inmunología , Quiasma Óptico/metabolismo , Ratas Sprague-Dawley , Hemorragia Subaracnoidea/inmunología , Hemorragia Subaracnoidea/metabolismo , Factor de Necrosis Tumoral alfa/metabolismo , Xantófilas/farmacologíaRESUMEN
Our objective was to measure how the misrouting of retinal ganglion cell (RGC) fibers affects the organization of the optic chiasm and lateral geniculate nuclei (LGN) in human albinism. We compared the chiasmal structures and the LGN in both pigmented controls and patients with albinism by using high-resolution structural magnetic resonance imaging (MRI). We studied 12 patients with oculocutaneous albinism and 12 age-matched pigmented controls. Using a 3T MRI scanner, we acquired a T1 -weighted three-dimensional magnetization-prepared rapid gradient-echo (MPRAGE) image of the whole brain, oriented so that the optic nerves, chiasm, and tracts were in the same plane. We acquired multiple proton density-weighted images centered on the thalamus and midbrain, and averaged them to increase the signal, enabling precise manual tracing of the anatomical boundaries of the LGN. Albinism patients exhibited significantly smaller diameters of the optic nerves, chiasm and tracts, and optic chiasm and LGN volume compared with controls (P < 0.001 for all). The reductions in chiasmal diameters in the albinism compared with the control group can be attributed to the abnormal crossing of optic fibers and the reduction of RGCs in the central retina. The volume of the LGN devoted to the center of the visual field may be reduced in albinism due to fewer RGCs representing the area where the fovea would normally lie. Our data may be clinically useful in addressing how genetic deficits compromise proper structural and functional development in the brain.