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Anesthesia for a patient of acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia.
MedLine Citation:
PMID:  24249999     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM), also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.
Authors:
Rudrashish Haldar; Prakhar Gyanesh; Sukhen Samanta
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of anaesthesiology, clinical pharmacology     Volume:  29     ISSN:  0970-9185     ISO Abbreviation:  J Anaesthesiol Clin Pharmacol     Publication Date:  2013 Oct 
Date Detail:
Created Date:  2013-11-19     Completed Date:  2013-11-19     Revised Date:  2013-11-21    
Medline Journal Info:
Nlm Unique ID:  9516972     Medline TA:  J Anaesthesiol Clin Pharmacol     Country:  India    
Other Details:
Languages:  eng     Pagination:  555-7     Citation Subset:  -    
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