Multiple suture synostosis, macrocephaly, and intracranial hypertension in a child with alpha-D-mannosidase deficiency. Case report.

Abstract:

:The authors present an unusual case in which increased intracranial pressure developed because of multiple-suture craniosynostosis and megaloencephaly in a child with a previously undiagnosed lysosomal storage disease, alpha-D-mannosidase deficiency. This 3-year-old boy presented with a history of frequent naps, headaches, florid papilledema, enlarged head (> 95th percentile), elevated opening pressure by lumbar puncture, a "beaten copper" appearance on skull radiographs, and no hydrocephalus. Multiple synostectomies were performed. Postoperatively, the child's headaches and papilledema resolved and his level of physical activity increased dramatically. The authors discuss the paradoxical presentation of prematurely fused sutures and macrocrania in light of this lysosomal storage disease and its subsequent management.

journal_name

J Neurosurg

journal_title

Journal of neurosurgery

authors

Grabb PA,Albright AL,Zitelli BJ

doi

10.3171/jns.1995.82.4.0647

subject

Has Abstract

pub_date

1995-04-01 00:00:00

pages

647-9

issue

4

eissn

0022-3085

issn

1933-0693

journal_volume

82

pub_type

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    authors: Simpson RK Jr,Harper RL,Bryan RN

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  • Spinal cord injury.

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    journal_title:Journal of neurosurgery

    pub_type: 评论,信件

    doi:

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    更新日期:2001-04-01 00:00:00

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    pub_type: 杂志文章

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    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.3171/jns.1999.90.6.1042

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  • Migration of subdural atrial shunt catheter into the pulmonary arteries. Case report.

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