Pierre-Kahn Alain, Sonigo Pascale
Department of Pediatric Neurosurgery, Groupe Hospitalier Necker-Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France.
Childs Nerv Syst. 2003 Aug;19(7-8):477-83. doi: 10.1007/s00381-003-0773-6. Epub 2003 Jul 19.
Prenatal investigations make it possible to follow up malformative intracranial cysts from their detection in utero through the postnatal period. By including those that will remain silent postnatally, precious information can be provided about their real natural history.
Contrary to common belief, the vast majority of these lesions, if not associated with other fetal anomalies, are benign in nature, remain clinically silent, do not evolve or even frequently regress spontaneously. They are compatible with a strictly normal life, whether requiring postnatal treatment or not.
Surgery is rarely needed for the treatment of an evolving hydrocephalus or an expanding cyst.
Clinical outcome is not correlated with cyst volume or location. Prognosis, therefore, does not rely upon brain deformation or translation but rather more upon brain integrity. To establish a correct prognosis all efforts must therefore aim to precisely analyze the brain anatomy. In this respect, fetal MRI is mandatory. Although chromosomal anomalies are rarely associated with isolated intracranial cysts, karyotype study is necessary.
The accuracy of diagnosis and prognosis depends mainly upon our ability to correctly interpret images, which in turn depends on the resolution of prenatal imaging and on experience. Median retrocerebellar fluid collections remain the most difficult to prognosticate, retrocerebellar cysts often being difficult to differentiate from mega cisterna magna and Dandy-Walker complex. In our hands, prenatal prognostication was correct in approximately 90% of the cases.
产前检查能够对颅内畸形囊肿从子宫内发现直至出生后进行追踪。通过纳入那些出生后无异常表现的囊肿,可以提供有关其真实自然病史的宝贵信息。
与普遍看法相反,这些病变中的绝大多数,如果不伴有其他胎儿异常,本质上是良性的,临床上无异常表现,不会进展,甚至常常会自发消退。无论是否需要出生后治疗,它们都与完全正常的生活相容。
对于进展性脑积水或不断增大的囊肿,很少需要手术治疗。
临床结局与囊肿大小或位置无关。因此,预后不取决于脑变形或移位,而更多地取决于脑的完整性。所以,为了建立正确的预后,所有努力都必须旨在精确分析脑解剖结构。在这方面,胎儿磁共振成像(MRI)是必不可少的。虽然染色体异常很少与孤立性颅内囊肿相关,但核型研究是必要的。
诊断和预后的准确性主要取决于我们正确解读图像的能力,而这又取决于产前成像的分辨率和经验。小脑后正中脑脊液聚集仍然是最难预测预后的,小脑后囊肿常常难以与巨大枕大池和丹迪 -沃克综合征相鉴别。在我们的经验中,产前预后在大约90%的病例中是正确的。