| Rett syndrome and gastric perforation. | |
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MedLine Citation:
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PMID: 18453295 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Rett Syndrome is associated with decreased peristaltic esophageal waves and gastric dysmotility, resulting in swallowing difficulties and gastric dilation. Rarely, gastric necrosis and perforation occur. Our case represents the third reported case of gastric necrosis and perforation associated with Rett Syndrome. A 31-year-old female after 11 hours of intermittent emesis and constant, sharp abdominal pain presented with evidence of multiorgan system failure including hypovolemic shock, metabolic acidosis, coagulopathy, and hepatorenal failure. A chest radiograph revealed intra-abdominal free air necessitating emergent laparotomy. During exploration, a severely dilated, thin-walled stomach with an area of necrosis and gross perforation was noted. Wedge resection of the necrotic tissue and primary closure were performed. Despite aggressive perioperative resuscitation and ventilation support, the patient died 3 hours postoperatively secondary to refractory shock and hypoxemia. Severe gastric dilation can occur with Rett Syndrome and may cause gastric necrosis and perforation. Prolonged elevated gastric pressures can decrease perfusion and may contribute to perforation. Timely decompression via percutaneous endoscopic or surgical gastrostomy could decrease the risk of perforation particularly when significant gastric distention is present. Consideration of gastric necrosis and perforation in patients with Rett Syndrome may lead to earlier intervention and decreased mortality. |
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Authors:
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Malay B Shah; James G Bittner; Michael A Edwards |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: The American surgeon Volume: 74 ISSN: 0003-1348 ISO Abbreviation: Am Surg Publication Date: 2008 Apr |
Date Detail:
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Created Date: 2008-05-05 Completed Date: 2008-06-03 Revised Date: 2010-03-30 |
Medline Journal Info:
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Nlm Unique ID: 0370522 Medline TA: Am Surg Country: United States |
Other Details:
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Languages: eng Pagination: 315-7 Citation Subset: IM |
Affiliation:
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Virtual Education and Surgical Simulation Laboratory, Department of Surgery, Medical College of Georgia School of Medicine, Augusta, Georgia 30912, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Female Humans Necrosis / etiology, pathology, surgery Rett Syndrome / complications*, pathology*, surgery Stomach Rupture / etiology*, pathology, surgery |
| Comments/Corrections | |
Comment In:
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Am Surg. 2010 Mar;76(3):345-6
[PMID:
20349675
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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