Elsevier

Brain and Development

Volume 30, Issue 6, June 2008, Pages 381-386
Brain and Development

Original article
Diagnosis of congenital hydrocephalus and delivery of its patients in Japan

https://doi.org/10.1016/j.braindev.2007.11.002Get rights and content

Abstract

The study population included 193 patients with prenatally diagnosed (fetal) hydrocephalus and 181 with postnatally (12 or less than 12 months after birth) diagnosed (infantile) hydrocephalus identified by a nationwide questionnaire survey of congenital hydrocephalus performed in 2000. Of 180 patients with fetal, 101 (56.1%) were diagnosed before week 32 of gestation and 18 (10%) were diagnosed week 37 and later of gestation. In patients with fetal hydrocephalus, US was used in more than 80% of the cases, whereas in patients with infantile hydrocephalus, CT was used in more than half of the cases. For diagnosis of fetal hydrocephalus, either US or MRI had become dominantly utilized and CT had gone out of use in 1996–2000. The adoption ratio of cesarean delivery to transvaginal delivery was around 7 to 3 in patients with fetal hydrocephalus, and 2 to 7 in patients with infantile hydrocephalus, respectively, with significant difference between fetal hydrocephalus and infantile hydrocephalus groups (p < 0.001). Clinical outcomes in patients with fetal hydrocephalus was better in those delivered transvaginally than in those by cesarean delivery, although without no statistical significance (p = 0.124) and those in patients with infantile hydrocephalus showed almost no difference between transvaginal and cesarean delivery groups. There was a tendency for the Apgar score at 5 min to be lower in smaller birth weight infants with a body weight of less than 2000 g. This score could be useful as an index for predicting immediate postnatal death in patients with fetal hydrocephalus.

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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