Bailey W, Freidenberg G R, James H E, Hesselink J R, Jones K L
Department of Pediatrics, University of California, San Diego 92093-0609.
Prenat Diagn. 1990 Oct;10(10):623-9. doi: 10.1002/pd.1970101002.
We report an infant with a craniopharyngioma which was detected in utero. Maternal uterine ultrasonography, done at 27 weeks because of polyhydramnios, revealed a 4 cm midline mass near the base of the fetal skull. At 31 weeks, magnetic resonance imaging of the maternal abdomen confirmed the presence of a mass in the region of the third ventricle and revealed hydrocephalus. Two days post-partum a computed tomography (CT)-guided needle biopsy of the mass was performed and recovered tissue which was histologically consistent with a craniopharyngioma. The infant's postnatal period was complicated by seizures, which were treated with phenobarbital, and by progressive hydrocephalus, necessitating placement of a ventriculo-peritoneal shunt. He also received therapy for central hypothyroidism and diabetes insipidus. The infant's parents refused permission for attempted resection of the tumour and he died at 8 weeks of age. This represents the second reported case of an antenatally detected craniopharyngioma. Four other cases of different intracranial tumours have been detected in utero using ultrasound, with no reported survivors past 3 days of age. There is a uniformly poor prognosis of such infants, but earlier diagnosis and intervention may change this result.
我们报告一例在子宫内被检测出患有颅咽管瘤的婴儿。因羊水过多,在孕27周时进行了母体子宫超声检查,发现胎儿颅骨底部附近有一个4厘米的中线肿块。孕31周时,母体腹部磁共振成像证实第三脑室区域存在肿块,并显示有脑积水。产后两天,在计算机断层扫描(CT)引导下对肿块进行了针吸活检,获取的组织在组织学上与颅咽管瘤一致。婴儿出生后的时期出现了癫痫发作,用苯巴比妥进行了治疗,还出现了进行性脑积水,需要放置脑室-腹腔分流管。他还接受了中枢性甲状腺功能减退和尿崩症的治疗。婴儿的父母拒绝同意尝试切除肿瘤,他在8周龄时死亡。这是第二例报告产前检测出颅咽管瘤的病例。另外还有4例不同类型的颅内肿瘤在子宫内通过超声检测出,但没有报告存活超过3天的病例。这类婴儿的预后普遍很差,但早期诊断和干预可能会改变这一结果。