Hirschsprungs Disease - SNguyen

Hirschsprungs Disease - SNguyen - Gastrointestinal Surgery...

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Gastrointestinal Surgery Conference Scott Nguyen Englewood Hospital May 21, 2003
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Patient S.C. Newborn male Full-term, uncomplicated vaginal delivery Normal birth weight: 3115 g Apgars 9 1 , 9 5 Mother: 36 yo, G 1 P 0 , healthy
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Patient S.C. Started breast feeding DOL 1 DOL 2-3 noted to have increasing abdominal distention No meconium passed in first 24 hrs of life 1 episode Non-bilious emesis
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Patient S.C.
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Patient S.C. Pediatric Surgical Consult Rectal Exam Empty rectal ampulla Tight anal sphincter Large amount of stool and air upon withdrawal of finger
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Patient S.C.
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Patient S.C. Rectal mucosal biopsy No ganglia identified
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Patient S.C.
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Patient S.C. Pt taken to OR for end colostomy and Hartmann’s pouch Dilated descending and sigmoid colon Prominent colonic blood vessels Site of colostomy, frozen section of colonic muscularis propria revealed ganglion cells
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Patient S.C.
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Patient S.C. Postoperative course uneventful Stool from colostomy POD 1 Tolerated breast feeding Discharged POD 6 2 nd stage pull through procedure planned in several weeks
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Hirschsprung’s Disease Scott Nguyen Englewood Hospital May 21, 2003
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Neurogenic form of intestinal obstruction Absence of ganglion cells in the myenteric and submucosal plexus Failure in relaxation of the internal anal
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Hirschsprungs Disease - SNguyen - Gastrointestinal Surgery...

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