The n e w e ng l a n d j o u r na l of m e dic i n e

Images in Clinical Medicine

Chana A. Sacks, M.D., Editor

Question-Mark Appearance of the Colon

Peter T. Masiakos, M.D.

Massachusetts General Hospital
Boston, MA
pmasiakos@partners.org

Paul W. Wales, M.D.

Hospital for Sick Children
Toronto, ON, Canada

A
baby boy born at 39 weeks of gestation to a 28-year-old woman had bilious emesis, failure to
pass meconium, and abdominal distention within 24 hours after birth. Abdominal radiography revealed
dilated bowel, a finding consistent with a distal obstruction. A water-soluble contrast enema showed a uni-
formly distended and shortened colon that appeared in the shape of a question mark, a finding consistent with
total colonic aganglionosis. A specimen obtained at the bedside by transanal suction rectal biopsy had no ganglion
cells found on hematoxylin and eosin staining, a negative result on calretinin staining, and abnormally thickened
nerve fibers. These findings confirmed the diagnosis of Hirschsprung’s disease, a congenital absence of enteric
ganglion cells resulting in a loss of bowel motility in affected areas. The patient was taken to the operating room,
where laparoscopic serial frozen-section biopsy specimens of the entire colon were obtained and no ganglion cells
were identified. Abundant ganglion cells were found at the level of the terminal ileum, and an end ileostomy was
performed. Six months later, the patient underwent a colectomy with stapled side-to-side anastomosis of the termi-
nal ileum to the rectal remnant (Duhamel procedure). At the postoperative visit, the infant was having five to eight
bowel movements daily and was growing well.
DOI: 10.1056/NEJMicm1811998
Copyright © 2019 Massachusetts Medical Society.

n engl j med 380;21 nejm.org May 23, 2019 2051

The New England Journal of Medicine
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