Neuro-ophthalmology

Chiasmal Disorders

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The chiasm sits in a crowded space so disorders usually have associated neuro/endocrine abnormalities

  • Palsies of CNs III, IV, Va, Vb, VI, sympathetic fibres

    • Horner’s syndrome, motility disorders, loss of facial sensation, see-saw nystagmus
  • Raised ICP: blockage of the foramen of Munro in posterior chiasmal lesions

  • Parinaud syndrome (dorsal midbrain syndrome)

  • Functioning pituitary tumours: acromegaly, gigantism (somatotrophic), Cushing’s (corticotrophic), hypogonadism (lactotrophic)

  • Pituitary destruction: amenorrhoea, diabetes insipidus

Clinical features

  • Field defects

    • Bitemporal hemianopia

    • Often asymmetric

    • Based on the site of the lesion

      • Superior: inferior lesion eg pituitary adenoma
      • Inferior: superior lesion eg craniopharyngioma
      • Junctional: anterior to chiasm on the side of the central scotoma
      • Bitemporal central hemianopic scotomas: posterior chiasm eg hydrocephalus
      • Nasal: lateral lesion eg ectasia of the ICA
  • Headaches

  • Hemifield slide: loss of fusion due to loss of the normal overlap of the nasotemporal fields. Manifests as diplopia in the absence of any motility disorder

  • Post-fixation blindness: during close work an object disappears when placed just beyond fixation (into temporal field)

  • See-saw nystagmus: pendular nystagmus with elevation and intorsion of one eye with depression and extortion of the fellow eye

  • Colour desaturation across the vertical midline of the uniocular visual field: early sign

  • Optic atrophy: may demonstrate a ‘bow-tie’ appearance (primarily nasal and temporal pallor)

Differential

  • Pituitary:

    • Adenomas: typical hypointense on T1 MRI and hyperintense on T2
    • Apoplexy: systemic hypertension, major surgery, anticoagulation, coagulopathies, oestrogen therapy, head trauma
    • Sheehan’s syndrome (pregnancy-related apoplexy)
  • Suprasellar:

    • Craniopharyngioma: a slow-growing tumour from the vestigial remnants of Rathke’s pouch

      • Children present with dwarfism, delayed development and obesity (hypothalamic dysfunction)
      • Adults present with visual impairment
      • Isointense tumour on T1 MRI
      • Commonly recurs after surgery
    • Meningioma: typically affect middle-aged women

      • Tumours at the tuberculum sellae often produce a junctional scotoma
  • Chiasmal:

    • Optic gliomas
    • Chiasmatic neuritis: demyelination, inflammation, vasculitis
  • Miscellaneous

    • ICA/ACA aneurysm
    • Cavernous haemangioma
    • Germinoma
    • Lymphoma
    • Sarcoidosis
    • Metastasis
    • Radionecrosis
    • Rathyke’s pouch cyst
    • Sphenoid sinus mucocoele
    • Trauma
    • Toxicity: ethambutol

Differential diagnosis of bitemporal field defects

  • Dermatochalasis
  • Tilted discs
  • Optic nerve colobomas
  • Nasal retinoschisis
  • Nasal RP
  • Functional visual loss
  • Chiasmal lesions

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