Case history

Case history #1

A 2-year-old girl presents with a 2- to 3-week history of nausea, vomiting, and an enlarging head. Physical exam reveals megacephaly and poor visual regard (acuity 20/200).

Case history #2

A 16-year-old girl presents with primary amenorrhea, galactorrhea, and mild headaches. Ophthalmologic examination reveals loss of vision in the right eye (20/40).

Other presentations

Approximately 50% of patients present acutely with signs and symptoms of raised intracranial pressure (headache, nausea, vomiting, depressed sensorium, diplopia) or acute visual loss requiring emergent surgery.[2][3][4] Visual impairment is found preoperatively in at least 75% of patients, although this is uncommon as the only presenting symptom.[2][4][5][6]

At presentation, endocrinopathies may be found in 30% to 80% of patients, manifesting as diabetes insipidus, growth failure, and, in adults, sexual dysfunction (impotence, amenorrhea, and galactorrhea).[2][4][7][8][9] A purely endocrinologic presentation is more common in adolescents and adults.[4][8][10]

The most common presentation of craniopharyngioma is a mixture of the above symptoms, including visual loss, intracranial hypertension, and endocrinologic disturbance.[2][4][11][12]

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