Colon With Question Mark Appearance

Image Source: The New England Journal of Medicine

A 28-year-old female gave birth to a baby boy born at 39 weeks of gestation. She presented as the baby had green-colored vomiting (bilious vomiting), failure to pass meconium, and abdominal distention within the first 24 hours of life.

An obstruction was suspected, so abdominal Xray was performed, which revealed dilated bowel, a finding consistent with distal obstruction. An enema with water-soluble contrast was performed, which revealed uniform distension and a short colon that had acquired ‘a question mark’ shape.

The findings were suggestive of total colonic aganglionosis.

A rectal biopsy was performed at the bedside using transanal suction to confirm the diagnosis. The biopsy sample showed no ganglion cells on hematoxylin and eosin staining, a negative result on calretinin staining, and abnormally thickened nerve fibers.

A diagnosis of Hirschsprung’s disease was made.

Laparoscopic serial frozen-section specimens were obtained from the entire colon in the operating room. Biopsy showed no ganglion cells, whereas the samples from the terminal ileum showed abundant ganglion cells. An end ileostomy was performed.

After 6 months, Duhamel procedure was performed (a colectomy with stapled side-to-side anastomosis of the terminal ileum to the rectal remnant. The surgery was successful, and the postoperative course was uneventful too. At subsequent follow-up visits, the infant was healthy, had achieved developmental milestones, and was having normal stool with 5 to 8 bowel movements daily.

Hirschsprung’s disease is a congenital disease characterized by the absence of enteric ganglion cells (nerve cells) in the bowel, which consequently result in a loss of bowel motility in affected areas. With no nerve cells in the bowel, the muscles of the bowel are unable to move. This results in obstruction of the bowel with stool.

Hirschsprung’s disease is responsible for 15-20% of the intestinal obstructions in the neonates. The most common presentation is the inability to pass meconium/stool in the first 48 hours of life accompanied by abdominal distension, and gradual onset of vomiting. The vomiting is bilious (contains bile).

The cause is still unknown; however, there may be a genetic predisposition. It is seen more commonly in males. An affected sibling increases the chances, and so do other congenital diseases, like Down’s syndrome.

A life-threatening complication of Hirschsprung’s disease is enterocolitis. Correct diagnosis and timely treatment are necessary to avoid complications.

References

Peter T. Masiakos, M. a. (2019, May 23). Question-Mark Appearance of the Colon. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1811998

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Dr. Arsia Parekh
Dr. Arsia Hanif has been a meritorious Healthcare professional with a proven track record throughout her academic life securing first position in her MCAT examination and then, in 2017, she successfully completed her Bachelors of Medicine and Surgery from Dow University of Health Sciences. She has had the opportunity to apply her theoretical knowledge to the real-life scenarios, as a House Officer (HO) serving at Civil Hospital. Whilst working at the Civil Hospital, she discovered that nothing satisfies her more than helping other humans in need and since then has made a commitment to implement her expertise in the field of medicine to cure the sick and regain the state of health and well-being. Being a Doctor is exactly what you’d think it’s like. She is the colleague at work that everyone wants to know but nobody wants to be. If you want to get something done, you approach her – everyone knows that! She is currently studying with Medical Council of Canada and aspires to be a leading Neurologist someday. Alongside, she has taken up medical writing to exercise her skills of delivering comprehensible version of the otherwise difficult medical literature. Her breaks comprise either of swimming, volunteering services at a Medical Camp or spending time with family.

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