Kennedy H B, Smith R J
Br J Ophthalmol. 1975 Dec;59(12):689-95. doi: 10.1136/bjo.59.12.689.
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.
我们回顾了45例颅咽管瘤患者,特别关注其临床表现和神经眼科检查结果。其中50%的患者最初被转诊至眼科诊所。超过半数的儿童表现为颅内压升高的临床症状,尽管其中三分之一患者存在视神经萎缩而非视乳头水肿。三分之一的儿童有斜视病史,仅有1例患者为麻痹性斜视。大多数成人表现为视力减退和视神经萎缩。双颞侧偏盲较为常见(在我们的患者中,诊断时27%的患者存在),但其发展不对称且难以预测。同向偏盲相对常见,在本研究中,有11%的患者以此为首发症状。最初有9例患者(20%)视野正常,与垂体腺瘤或鞍上脑膜瘤相比,这一比例较高。我们认为多形性,即随着疾病进展,视野缺损类型发生明显变化,是该肿瘤的一个特征性表现(实际上临床状态和视力波动也是如此);在我们22%的患者中发现了这种情况。内分泌紊乱很常见,尤其是在成人中,30岁以上患者常以精神衰退为首发症状。儿童颅骨平片对颅咽管瘤几乎具有诊断价值,但成人颅骨平片正常并不能排除诊断。