1999
DOI: 10.1590/s0004-282x1999000400022
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Abstract: -The purpose of this study is to describe two infants that were diagnosed with Walker-Warburg syndrome (WWS), a rare form of congenital muscular dystrophy (CMD).They were studied in their clinical, laboratory, and neuroradiologic features. The index case had a brain magnetic resonance imaging (MRI) and the second patient had a head computerized tomography (CT). In addition, a literature review was performed to describe the main forms of CMD. The index case fulfilled all criteria for WWS. A brain MRI performed … Show more

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Cited by 4 publications
(8 citation statements)
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“…Regarding preoperative fasting requirements, a previous publication suggested that WWS might be associated with reduced gastrointestinal motility and an increased risk for pulmonary aspiration of gastric contents 10. We were unable to verify this information in the cited original literature 11,12, but our patient indeed showed signs of gastric retention and gastroesophageal reflux shortly after birth. Since gastric retention had resolved by the time of the operation, and since prolonged fasting times in neonates are associated with hypoglycemia and hypovolemia, we determined that a standard fasting period was sufficient.…”
Section: Discussionmentioning
confidence: 60%
“…Regarding preoperative fasting requirements, a previous publication suggested that WWS might be associated with reduced gastrointestinal motility and an increased risk for pulmonary aspiration of gastric contents 10. We were unable to verify this information in the cited original literature 11,12, but our patient indeed showed signs of gastric retention and gastroesophageal reflux shortly after birth. Since gastric retention had resolved by the time of the operation, and since prolonged fasting times in neonates are associated with hypoglycemia and hypovolemia, we determined that a standard fasting period was sufficient.…”
Section: Discussionmentioning
confidence: 60%
“…Children with these defects exhibit profound psychomotor retardation and tend to develop seizures (17). WWS may also be associated with congenital muscular dystrophy which might involve cardiac, skeletal and smooth muscles (18–20). Many characters of this disease overlap with those of cerebro‐ocular‐cerebral syndrome, muscle‐eye‐brain disorder and Fukuyoma congenital muscular dystrophy.…”
Section: Discussionmentioning
confidence: 99%
“…These children tend to develop recurrent episodes of central and obstructive apnoea, seizures, profound psychomotor retardation, swallowing difficulty and poor airway control. These complications may further lead to postoperative cardiorespiratory failure, aspiration pneumonia and sepsis (18,19). In our patient signs and symptoms of increased ICP such as vomiting, decreased level of conciousness, hypertension, cardiac dysrhythmias, seizures were absent.…”
Section: Discussionmentioning
confidence: 99%
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