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Child's Nervous System

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01-07-2018 | Case Report

Shunt surgery for early-onset severe hydrocephalus in methylmalonic acidemia: report on two cases and review of the literature

Authors: Minglei Ma, Mingxing Wu, Yanbin Li, Di Wu, Bingke Zhang

Published in: Child's Nervous System | Issue 7/2018

Abstract

Object

Methylmalonic acidemia (MMA) with early-onset severe hydrocephalus is rare. In this paper, we described two cases of MMA with hydrocephalus and review the literature to elucidate the clinical features of the disease, treatment options, and follow-up results.

Methods

The PubMed and Embase databases were searched for clinical reports on MMA with severe hydrocephalus, and two unreported cases were presented to illustrate the clinical spectrum.

Results

Six cases of MMA with severe hydrocephalus were observed in the previous literature. Our two patients with severe hydrocephalus but not bulging fontanelle received a ventriculo-peritoneal shunt, and intracranial hypertension was confirmed in both cases during the operation. These patients’ clinical symptoms significantly improved after the operation.

Conclusions

Intracranial hypertension can exist in early-onset severe hydrocephalus in MMA, even if the bulging anterior fontanelle is not apparent. These patients could benefit from a ventriculo-peritoneal shunt.

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Title: Shunt surgery for early-onset severe hydrocephalus in methylmalonic acidemia: report on two cases and review of the literature
Authors: Minglei Ma
Mingxing Wu
Yanbin Li
Di Wu
Bingke Zhang
Publication date: 01-07-2018
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 7/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-018-3753-6

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Shunt surgery for early-onset severe hydrocephalus in methylmalonic acidemia: report on two cases and review of the literature

Abstract

Object

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Object

Methods

Results

Conclusions

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