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Tuesday, July 27, 2010

Hirschsprung's Disease

Findings
This pediatric patient presents with dilated proximal colon with a transition zone to narrowed, dilated colon.
Discussion
Hirschsprung’s disease is a functional obstruction of distal bowel (anus and usually continguous colon) due to congenital lack of intrinsic enteric ganglion cells. Hirschsprung’s usually presents in first 48hrs of life with failure to pass meconium, abdominal distention, and bilious vomiting. It is often associated with other abnormalities, most frequently Down’s syndrome (15%). Classic radiologic findings are a transition zone from abnormally small distal colon to dilated proximal colon and recto-sigmoid (R/S) ratio < href="http://www.google.com.eg/imgres?imgurl=http://eradiology.bidmc.harvard.edu/Classics/RadiologyImages/6c526324-0278-4d22-b80a-d8b402811d35/small_thumbs/Hirschsprungs%2520syndrome%2520unlab.jpg&imgrefurl=http://eradiology.bidmc.harvard.edu/Classics/list.aspx%3Fsection%3DPediatrics%2Band%2BCongenital%26pk%3D50031181-eab8-4468-8097-7daab9b893c9&usg=__T7b90YvQZVO_JS7KFJBjq7J01sw=&h=100&w=85&sz=3&hl=ar&start=3&tbnid=n6DSUIivSd6NgM:&tbnh=82&tbnw=70&prev=/images%3Fq%3DHirschsprung%2527s%2BDisease,barium%2Benema%26hl%3Dar%26sa%3DG%26gbv%3D2%26tbs%3Disch:1&itbs=1">http://www.google.com.eg/imgres?imgurl=http://eradiology.bidmc.harvard.edu/Classics/RadiologyImages/6c526324-0278-4d22-b80a-d8b402811d35/small_thumbs/Hirschsprungs%2520syndrome%2520unlab.jpg&imgrefurl=http://eradiology.bidmc.harvard.edu/Classics/list.aspx%3Fsection%3DPediatrics%2Band%2BCongenital%26pk%3D50031181-eab8-4468-8097-7daab9b893c9&usg=__T7b90YvQZVO_JS7KFJBjq7J01sw=&h=100&w=85&sz=3&hl=ar&start=3&tbnid=n6DSUIivSd6NgM:&tbnh=82&tbnw=70&prev=/images%3Fq%3DHirschsprung%2527s%2BDisease,barium%2Benema%26hl%3Dar%26sa%3DG%26gbv%3D2%26tbs%3Disch:1&itbs=1

2 comments:

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