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Congenital brain tumours are a rare entity that is nowadays often already recognised during pregnancy by ultrasound (US) and magnetic resonance (MR). Even though the definitive diagnosis is usually achieved by means of histological studies, in some cases the diagnosis may remain uncertain because of the malformative origin of this type of tumour.We describe a patient with a diffuse congenital intracranial mass diagnosed by intrauterine US and foetal MR performed to further evaluate the lesion and the associated foetal hydrocephalus. After delivery by caesarean section, an endoscopic biopsy was performed. Several specimens were obtained and resulted in the diagnosis of primitive neuroectodermal tumour (PNET). Despite polychemotherapy, the tumour continued to enlarge and the patient died at 2 months of age. Post-mortem histological examination of the intracranial mass showed a diffuse intracranial teratoma.Endoscopic biopsy specimen examination resulted in a diagnosis that was not confirmed by post-mortem findings. The scarce differentiation of teratoma components and their high variability in histomorphology as well as the huge size of the tumoral mass may limit the interpretation of endoscopic biopsy specimens, even when multiple and obtained from different areas.

The management of congenital CNS anomalies differs among countries. This paper reports on a worldwide survey of individual experience and regimens for treating spina bifida (SB) in different patient populations and cultural backgrounds. Ten representative members of the International Society for Pediatric Neurosurgery (ISPN) in individual countries registered their experience, prenatal management, and care system for SB. The information collected contained the following major items: intrauterine diagnosis, prophylaxis, and legal termination in prenatal management. The prenatal diagnosis of SB is extensively performed throughout the world, but the marker alpha-fetoprotein is not widely accepted in Asia, nor is the prophylactic administration of folic acid, which is not part of the routine care in Asia. Morphological analyses by neuroimaging, especially using an advanced technique of magnetic resonance imaging (MRI), are well developed in Asian countries, e.g., Japan and Korea. The prenatal management of SB fetuses differed depending upon the region, i.e., more support for aggressive and intensive treatment in Asia than in Europe and North America. The regional and racial difference in management of and care for spina bifida fetuses will be discussed further based on the data analysis.

Hydrocephalus is one of the most common congenital anomalies affecting the central nervous system especially in developing countries for several reasons including prohibition of abortion in many countries. The technological advances in methods of intrauterine diagnosis of congenital malformation made it possible to detect early enlargement of the ventricles and diagnose hydrocephalus as early as 15 weeks of gestational age. Many trials of intrauterine shunting proved unsuccessful, mainly because of complications such as intra- or extracranial shunt migration, obstruction, infection, and malposition.The author is presenting a case of a successful ventriculo-amniotic shunt utilizing Al-Anazi ventriculo-uterine shunt, which is easy to implant. It has a one-way valve to prevent amniotic fluid backflow and special wings to prevent shunt migration, which are both relatively short and wide to reduce the possibility of malfunction and the risk of infection because there is no exposure with the external environment.Our successful trial showed that Al-Anazi ventriculo-uterine shunt might be the first step in treating congenital hydrocephalus.