Linked Life Data

21196794

Source:http://linkedlifedata.com/resource/pubmed/id/21196794

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
2011-1-13
pubmed:abstractText
The authors report a case of iniencephaly in a 6-year-old boy with a huge occipital bone defect and encephalocele, extensive spina bifida of the cervical vertebrae and fixed retroflexion of the head due to Sprengel's deformity. He presented with some cerebellar and cranial nerve symptoms and new episodes of neck pain and drop attacks. Brain imaging confirmed progressive deformity of the brain stem, cerebellar herniation into the encephalocele sac and ventriculomegaly. Surgical repair of the encephalocele was performed with preservation of all herniated cerebellar tissue and the release of thick arachnoid adhesions to make more space to return the herniated neural tissue from the sac to the cranium. The patient was found to have progressive facial palsy and intracranial hypertension 3 days after surgery, which improved with ventriculoperitoneal shunting. Iniencephaly is generally a fatal anomaly, and only 7 such patients have been reported to have survived. Because of the fatality of this anomaly, prenatal diagnosis of iniencephly and pregnancy termination are important. The patient presented herein is only the second patient with iniencephaly and encephalocele to be operated on. The severity of associated systemic and cranial abnormalities is fundamental with regard to survival. Essential points for surgery are preparing enough space to save herniated functional neural tissues, management of associated hydrocephalus and brain stem rotation/compression due to decreased postoperative space. In the surviving child, early correction of Sprengel's deformity would provide a better aesthetic position of the neck with preservation of brachial plexus integrity.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:issn
1423-0305
pubmed:author
pubmed:copyrightInfo
Copyright © 2010 S. Karger AG, Basel.
pubmed:issnType
Electronic
pubmed:volume
46
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
290-3
pubmed:meshHeading
pubmed:year
2010
pubmed:articleTitle
Iniencephaly: clinical, radiological and surgical findings.
pubmed:affiliation
Department of Neurosurgery, Sina Hospital, Tehran, Iran.
pubmed:publicationType
Journal Article, Case Reports