Differentiating Hirschsprung's Disease from other Diseases: Difference between revisions
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==Overview== | ==Overview== | ||
Hirschsprung disease must be differentiated from other diseases that cause failure to pass meconium and abdominal distension in infants, such as meconium plug syndrome, small left colon syndrome and congenital hypothyroidism. | Hirschsprung disease must be differentiated from other diseases that cause failure to pass [[meconium]] and [[abdominal distension]] in infants, such as [[meconium plug syndrome]], [[small left colon syndrome]] and [[congenital hypothyroidism]]. | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Hirschprung's disease must be differentiated from other diseases in infancy presenting with similar features such as failure to pass meconium, abdominal distension, and non-bilious vomiting. | Hirschprung's disease must be differentiated from other diseases in infancy presenting with similar features such as failure to pass meconium, abdominal distension, and non-bilious vomiting. | ||
*Meconium plug syndrome | *[[Meconium plug syndrome]] | ||
*Small left colon syndrome | *[[Small left colon syndrome]] | ||
*Distal small bowel /colonic atresia | *[[Intestinal atresia|Distal small bowel /colonic atresia]] | ||
*Meconium ileus and cystic fibrosis complex | *[[Meconium ileus]] and [[cystic fibrosis]] complex | ||
*Congenital hypothyroidism | *[[Congenital hypothyroidism]] | ||
{| class="wikitable" | {| class="wikitable" | ||
!Disease | !Disease | ||
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!Radiological findings | !Radiological findings | ||
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|Meconium plug syndrome | |[[Meconium plug syndrome]] | ||
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* Transient intestinal obstruction for 1-2 days after birth. | * Transient [[intestinal obstruction]] for 1-2 days after birth. | ||
* Obstruction is functional due to dilated colon and delayed passage of feces. | * Obstruction is functional due to dilated colon and delayed passage of feces. | ||
* Usually seen in premature infants. | * Usually seen in [[premature infants]]. | ||
|[[Image:Meconium-plug-syndrome - Case courtesy of Radswiki, Radiopaedia.org, rID 11606.jpg|center|300px|thumb|Abdominal X-ray with contrast showing inspissated meconium in the intestine proximal to the colon - Case courtesy of Radswiki, Radiopaedia.org, rID 11606]] | |[[Image:Meconium-plug-syndrome - Case courtesy of Radswiki, Radiopaedia.org, rID 11606.jpg|center|300px|thumb|Abdominal X-ray with contrast showing inspissated meconium in the intestine proximal to the colon - Case courtesy of Radswiki, Radiopaedia.org, rID 11606]] | ||
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|Small left colon syndrome | |[[Small left colon syndrome]] | ||
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* Reduced caliber of the colon starting from splenic flexure and going down causing intestinal obstruction. | * Reduced caliber of the [[colon]] starting from [[splenic flexure]] and going down causing [[intestinal obstruction]]. | ||
* Characterized by a sudden change of the diameter of the colon. | * Characterized by a sudden change of the diameter of the colon. | ||
* Usually associated with gestational diabetes. | * Usually associated with [[gestational diabetes]]. | ||
|[[Image:Small-left-colon-syndrome-1 - Case courtesy of Dr Eric F Greif, Radiopaedia.org, rID 30024.jpg|center|300px|thumb|Abdominal X-ray with contrast shows decreased caliber of the descending and sigmoid colon, loss of haustration along with filling defects corresponding to retained feces - Case courtesy of Dr Eric F Greif, Radiopaedia.org, rID 30024]] | |[[Image:Small-left-colon-syndrome-1 - Case courtesy of Dr Eric F Greif, Radiopaedia.org, rID 30024.jpg|center|300px|thumb|Abdominal X-ray with contrast shows decreased caliber of the descending and sigmoid colon, loss of haustration along with filling defects corresponding to retained feces - Case courtesy of Dr Eric F Greif, Radiopaedia.org, rID 30024]] | ||
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|Distal small bowel /colonic atresia | |[[Intestinal atresia|Distal small bowel /colonic atresia]] | ||
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* Failure to pass meconium due to failure of recanalization of the intestine. | * Failure to pass [[meconium]] due to failure of recanalization of the intestine. | ||
* Proximal lesions has an earlier onset of symptoms than distal lesions. | * Proximal lesions has an earlier onset of symptoms than distal lesions. | ||
* Colonic atresia may affect normal children or may be associated with other abnormalities as Hirschsprung disease or gastroschisis. | * [[Intestinal atresia|Colonic atresia]] may affect normal children or may be associated with other abnormalities as Hirschsprung disease or [[gastroschisis]]. | ||
|[[Image:Small-bowel-atresia - Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID 5959.jpg|center|300px|thumb|Normal appearing colon that is small and unused. Contrast fills the whole colon and passes to the ileum - Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID 5959]] | |[[Image:Small-bowel-atresia - Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID 5959.jpg|center|300px|thumb|Normal appearing colon that is small and unused. Contrast fills the whole colon and passes to the ileum - Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID 5959]] | ||
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|Meconium ileus | |[[Meconium ileus]] | ||
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* Intestinal obstruction due to inspissation of meconium in the distal ileum. | * [[Intestinal obstruction]] due to inspissation of [[meconium]] in the [[Ileum|distal ileum]]. | ||
* Most cases of meconium ileus are secondary to cystic fibrosis. | * Most cases of [[meconium ileus]] are secondary to [[cystic fibrosis]]. | ||
* Typically presents with failure to pass meconium, abdominal distension with or without vomiting. | * Typically presents with failure to pass [[meconium]], [[abdominal distension]] with or without vomiting. | ||
* Meconium ileus may first present with complications perforation and volvulus. | * [[Meconium ileus]] may first present with complications [[perforation]] and [[volvulus]]. | ||
|[[Image:Meconium-ileus-neonate-with-cystic-fibrosis - Case courtesy of Dr Michael Sargent, Radiopaedia.org, rID 6009.jpg|center|300px|thumb|Contrast enema shows inspissated meconium starting from the mid sigmoid colon and going up till the splenic flexure. The colon is normal in diameter ruling out microcolon]] | |[[Image:Meconium-ileus-neonate-with-cystic-fibrosis - Case courtesy of Dr Michael Sargent, Radiopaedia.org, rID 6009.jpg|center|300px|thumb|Contrast enema shows inspissated meconium starting from the mid sigmoid colon and going up till the splenic flexure. The colon is normal in diameter ruling out microcolon]] | ||
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|Congenital hypothyroidism | |[[Congenital hypothyroidism]] | ||
|*Most infants are born asymptomatic. | |*Most infants are born asymptomatic. | ||
*Macroglossia | *[[Macroglossia]] | ||
*Hoarse cry | *Hoarse cry | ||
*Umbilical hernia | *[[Umbilical hernia]] | ||
*Puffy facies | *Puffy facies | ||
*Increased head circumference | *Increased head circumference | ||
*Hypothermia | *[[Hypothermia]] | ||
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Revision as of 19:30, 15 June 2017
Hirschsprung's disease Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]
Overview
Hirschsprung disease must be differentiated from other diseases that cause failure to pass meconium and abdominal distension in infants, such as meconium plug syndrome, small left colon syndrome and congenital hypothyroidism.
Differential Diagnosis
Hirschprung's disease must be differentiated from other diseases in infancy presenting with similar features such as failure to pass meconium, abdominal distension, and non-bilious vomiting.
- Meconium plug syndrome
- Small left colon syndrome
- Distal small bowel /colonic atresia
- Meconium ileus and cystic fibrosis complex
- Congenital hypothyroidism
Disease | Prominent clinical features | Radiological findings |
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Meconium plug syndrome |
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Small left colon syndrome |
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Distal small bowel /colonic atresia |
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Meconium ileus |
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Congenital hypothyroidism | *Most infants are born asymptomatic.
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