Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology
This was a full term male who initially presented at two days of age with intermittent bilious vomiting and a right lower quadrant mass.
Clinical Physical Exam:
There was a question of a palpable mass in the right lower quadrant.
Clinical Differential Diagnosis:
Distal bowel obstruction in a neonate - meconium ileus, meconium plug, ileal atresia, Hirschsprung disease.
An abdominal film from the second day of life showed a distal bowel obstruction. An enema performed on the third day of life showed the colon to be of a normal caliber, with no evidence of a transition zone.
Images 2 and 3
Imaging Differential Diagnosis:
Hirschsprung disease cannot be excluded.
At the beginning of the second week of life the patient underwent surgical exploration and colonic biopsy. The frozen biopsies showed no ganglion cells all the way up to the terminal ileum. A transition zone was noted in the terminal ileum. A loop ileostomy was performed in the normal segment of ileum.
Examination of the surgical specimen revealed complete colonic aganglionosis.
Hirschsprung Disease (Total Colonic)
Follow-up and Prognosis:
Once the pathologic diagnosis of total colonic Hirschsprung disease was made, the patient was taken back to the operating room and the patient had a total colectomy with an ileoanal Soave pull-through. After the Soave procedure, the patient developed post operatively recurrent diarrhea. He also had significant difficulty emptying his ileoanal pull through and was rectal tube dependent. He therefore had a temporary diverting ileostomy performed 6 months after the colectomy.
Case 46, Case 48
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