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Child's Nervous System
Published in: Child's Nervous System 6/2020

01-06-2020 | Computed Tomography | Original Article

Management and problems of prolonged survival with hydranencephaly in the modern treatment era

Authors: Nobuyuki Akutsu, Mai Azumi, Junji Koyama, Atsufumi Kawamura, Masaaki Taniguchi, Eiji Kohmura

Published in: Child's Nervous System | Issue 6/2020

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Abstract

Purpose

Hydranencephaly is a rare condition that occurs during embryogenesis after neurogenesis and is characterized by the near complete absence of the cerebral hemispheres. In general, patients with hydranencephaly have been considered to have a markedly reduced life expectancy. We present 4 patients with hydranencephaly who have survived for over 5 years. The management and problems encountered in these cases are discussed.

Methods

A retrospective review was conducted at our institution. Medical charts and radiographic studies were reviewed. Data including age at follow-up, sex, clinical complications, and surgical procedures were recorded.

Results

Six patients were radiologically diagnosed with hydranencephaly during the period from January 2000 to December 2012. Two patients were excluded from our study: one because of death from pneumonia at 1 year of age and another because of transfer to another hospital. Four patients (3 males and 1 female) were included in the analysis. All 4 patients underwent ventriculoperitoneal shunt (VPS) placement and shunt revision. VPS infection occurred in 3 of 4 cases, and bloody cerebrospinal fluid (CSF) was observed in 2 of 4 cases. One patient underwent successful choroid plexus cauterization (CPC) and shunt removal after shunt infection.

Conclusions

Prolonged survival with hydranencephaly is not unusual in the modern treatment era. CSF shunt problems, such as recurring shunt malfunction and shunt infection, represent one of the major problems, and avoiding CSF shunt with CPC is particularly desirable in patients with hydranencephaly.
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Metadata
Title
Management and problems of prolonged survival with hydranencephaly in the modern treatment era
Authors
Nobuyuki Akutsu
Mai Azumi
Junji Koyama
Atsufumi Kawamura
Masaaki Taniguchi
Eiji Kohmura
Publication date
01-06-2020
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 6/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04479-4

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