Original articleDiagnosis of congenital hydrocephalus and delivery of its patients in Japan
Introduction
The development of diagnostic tools has made it possible to diagnose anomalies in the central nervous system (CNS) in the early stage of life [1], [2], [3], [4]. Hydrocephalus is a representative and important treatable CNS anomaly [5], [6], [7]. Various protocols for the management of congenital hydrocephalus have been proposed, but many difficult problems remain unsolved, especially regarding the management of prenatally-diagnosed (fetal) hydrocephalus [8], [9]. In order to clarify these problems, authors performed a nationwide survey on congenital hydrocephalus in Japan in 2000, and investigated the etiology and associated conditions and treatment and outcomes on the basis of this nationwide survey [10], [11]. The aim of this study was to overview the prenatal and perinatal management of congenital hydrocephalus in Japan, including diagnostic and obstetric issues, and to investigate their differences between prenatally diagnosed (fetal) hydrocephalus and postnatally diagnosed (infantile) hydrocephalus.
Section snippets
Materials and methods
Congenital hydrocephalus was defined as increased retention of cerebrospinal fluid (CSF) in the ventricles associated with ventricular dilation diagnosed up to 12 months after birth and classified into two types: antenatally-diagnosed (fetal) hydrocephalus, postnatally-diagnosed (infantile) hydrocephalus [7], [10], [11].
A nationwide survey of congenital hydrocephalus in Japan was carried out in 2000. Details of materials and methods of the survey were described in our separate reports [10], [11]
Time of diagnosis of congenital hydrocephalus
The prenatal time of diagnosis of fetal hydrocephalus was divided into four according to gestational age: (i) before week 22, (ii) from week 22 to week 31, (iii) from week 32 to week 36, (iv) week 37 and later. Fig. 1 shows a histogram of the time of diagnosis in the 180 cases of fetal hydrocephalus (white bars) and the 175 cases of infantile hydrocephalus (black bars), whose diagnosis time had been described. Of 180 patients with fetal hydrocephalus, only 7 (3.9%) were diagnosed before 22
Discussion
Congenital hydrocephalus has an estimated population incidence of 0.2–0.8/1000 live births [12]. With improvements in diagnostic technology and methodology, congenital hydrocephalus has become recognizable earlier than before, and in some cases hydrocephalus has been diagnosed even in very preterm fetuses by antenatal ultrasound and/or the other imaging tools [12]. The sensitivity of antenatal detection by routine ultrasound screening improved during 1991–1996 compared with 1985–1990 [13]. The
Acknowledgement
This work was supported by Health Sciences Research Grants for Specific Diseases “Intractable Hydrocephalus” from the Ministry of Health, Labour and Welfare, Japan, No. 1999-SD-17, Mami Yamasaki.
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