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Eye signs in craniopharyngioma
Insights
Craniopharyngioma often presents with visual disturbances and signs of increased intracranial pressure, even in the absence of papilledema. Early referral to eye clinics is common, highlighting the importance of neuro-ophthalmological assessment for these tumors.
Area of Science:
- Neuro-ophthalmology
- Pediatric Neurology
- Endocrinology
Background:
- Craniopharyngioma is a rare tumor affecting the pituitary gland and optic pathways.
- Understanding its clinical presentation is crucial for early diagnosis and management.
Observation:
- 50% of patients were initially seen in an eye clinic.
- Children often presented with raised intracranial pressure (50%), optic atrophy (33%), or strabismus (33%).
- Adults predominantly showed visual failure and optic atrophy.
Findings:
- Bitemporal hemianopia occurred in 27% of patients, often asymmetrical.
- Homonymous hemianopia was seen in 11% of patients.
- Pleomorphism, or changing visual field defects, was noted in 22% of cases, alongside fluctuating clinical status and visual acuity.
Implications:
- Craniopharyngioma diagnosis requires careful neuro-ophthalmological evaluation, especially given varied presentations.
- Skull X-rays are diagnostic in children but less reliable in adults.
- Endocrine disorders and mental deterioration are significant comorbidities, particularly in adults.
Abstract:
A total of 45 patients with craniopharyngioma are reviewed, with particular reference to the presenting clinical features and neuro-ophthalmological findings. Of these 50 per cent had been referred in the first instance to an eye clinic. More than half the children presented with the clinical picture of raised intracranial pressure, although one-third of these had optic atrophy rather than papilloedema. One-third of the children had a history of concomitant strabismus, and only one patient had a paretic squint. The majority of the adults presented with visual failure and optic atrophy. Bitemporal hemianopia was fairly frequently found (27 per cent of our patients at the time of diagnosis) but was asymmetrical and unpredictable in its evolution. Homonymous hemianopia was relatively common, a presenting feature in II per cent of patients in this study. Full fields were found initially in nine patients (20 per cent), a high incidence compared with pituitary adenomas or suprasellar meningiomas. We consider pleomorphism, that is a distinct change from one type of field defect to another with progress of the disease, to be a characteristic feature of the tumour (as indeed are fluctuations in the clinical state and visual acuity); it was detected in 22 per cent of our patients. Endocrine disorders were common, especially in adults, and mental deterioration was a frequent presenting feature in patients over 30 years of age. Straight x rays of the skull are practically diagnostic of craniopharyngioma in children, but in adults a normal x ray does not exclude the diagnosis.
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