Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
12
pubmed:dateCreated
2005-11-29
pubmed:abstractText
We report a case of esophageal achalasia (EA) in Downa9s syndrome. A six-year-old girl with DS was referred from another hospital for further management of dysphagia and growth retardation (weight 16.3 kg, height 105.4 cm: both below -1.5 SD). Dysphagia commenced when she was one year old and gastroesophageal reflux (GER) was diagnosed when she was four. Routine investigations suggested EA or esophageal stenosis secondary to GER. While dissecting the esophago-gastric junction in preparation for a Heller-Dor esophagocardiomyotomy (HD-ECM), the crus of the diaphragm was noted to be narrowed, severely fibrosed and attached to the lower esophagus which was covered by dense scar tissue suggestive of an old esophageal perforation secondary to GER esophagitis. A Nissen fundoplication was performed, but dysphagia persisted postoperatively, and a narrowing 2 cm above the fundoplication wrap with proximal dilatation was found on repeated barium studies. At re-laparotomy, the fundoplication was unwrapped and HD-ECM performed. Eight months postoperatively, she is well with no recurrence. EA must be considered in any DS patient presenting with dysphagia.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Dec
pubmed:issn
0179-0358
pubmed:author
pubmed:issnType
Print
pubmed:volume
21
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
997-1000
pubmed:meshHeading
pubmed:year
2005
pubmed:articleTitle
Down's syndrome and esophageal achalasia: a rare but important clinical entity.
pubmed:affiliation
Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan.
pubmed:publicationType
Journal Article, Case Reports