Abdominal Distention in an 18 day old baby boy

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Abdominal Distention in an 18 day old baby boy

• Sarah Banner, MD/MPH candidate 08

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Clinical Presentation

• Mother brings her 18 day old baby boy Henry to
  the ER with increased fussiness and abdominal
  bloating.
• Henry was born at term without complications to
  his G2P2 mother.
• Henrys mother breast feeds him as she did her
  other child.
• He has no diarrhea, but has not had regular bowel
  movements like his sibling did in her first few
  weeks of life, and he has been vomitting after
  his feeds for the past 24 hrs.

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Differential?

• Muconeum ileus?
• Constipation?
• Hirshprungs?

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What next?

• When suspecting Hirchsprungs disease, start with
  plain abdominal radiography
• Follow up with a contrast enema examination of
  the colon to confirm the diagnosis
• Occationally U/S may also suggest the diagnosis.

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Radiologic Studies

• Abdominal X-ray

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• See dilated small and large bowel with associated
  air-fluid levels- essentially a distal bowel
  obstruction
• Often an empty rectum is seen
• Lets look at some more before we get the barium
  enema

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• Now the barium enema

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• Shows reduced caliber rectum and a transition
  zone to an enlarged-caliber sigmoid
• Would see irregular contractions, mucosal
  irregularity and delayed evacuation of the
  contrast material
• A few more..

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Irregular mucosal contour
Small caliber rectum juxtaposed to large caliber
sigmoid
24 hrs after barium enema- retained contrast
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Hirschprungs Disease (aka congenital aganglionic
megacolon)

• 1 case per 5,000 live births
• Males Females
• Generally manifests in first few weeks of life,
  may be in first 5 yrs, and rarely all the way
  into adulthood
• Abnormal innervation of the bowel is present with
  absence of Meissner's and Auerbach's plexuses and
  hypertrophied parasympathetic ganglia
• Failure of normal neural crest cell migration at
  weeks 5-12 of gestation

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Diagnosis

• Diagnosis is histological with either a
  submucosal or a full thickness rectal biopsy in
  association with either anorectal manometry or
  barium enema.

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Treatment

• Surgery
• Remove site DISTAL to the dilated bowel.
• Early detection and surgery is essential for
  these children to ensure adequate growth and to
  prevent enterocolitis.

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References

• 1. Kleinhaus S and Boley SJ. Hirschsprung's
  Disease.?In Robert Wiley editor Pediatric
  Gastroenterology., pp. 698-705.
• 2. Rosenfield NS, Ablow RC, Markowitz RI, et al.
  Hirschsprung Disease?Accuracy of the Barium
  Enema Examination. Radiology. 1984 150
  (2)393-400.
• 3.http//www.emedicine.com/radio/topic343.htm
• 4.http//www.uhrad.com/pedsarc/peds030.htm

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THANKS!