Bowel Surgery Necessary for HAE Patient After Therapy Fails

Older woman's gastrointestinal attack responds poorly to C1-INH concentrate

Patricia Valerio, PhD avatar

by Patricia Valerio, PhD |

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Despite being treated for gastrointestinal symptoms of a hereditary angioedema (HAE) attack, an 80-year-old woman with the disease had to undergo surgery to pinpoint their exact cause.

Her abdominal pain, vomiting, and nausea eased only mildly with a C1-esterase inhibitor (C1-INH) concentrate, a type of medication usually recommended to manage HAE attacks, and diagnostic surgery was required.

The surgical procedure revealed she had a small bowel or intestine obstruction secondary to an internal hernia — a condition in which a portion of an organ pokes through a weakened tissue wall. After surgery, no bowel resection or removal was necessary.

“Although C1-INH concentrate remains the principal treatment for HAE, gastrointestinal attacks may potentially cause surgical emergencies,” the researchers wrote.

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Her case was described in the report, “Bowel obstruction secondary to internal hernia in a hereditary angioedema patient: a case report,” published in the International Journal of Emergency Medicine.

HAE is characterized by sudden and recurrent swelling episodes in the deeper layers of the skin, the upper airways, and the gastrointestinal tract. The most common gastrointestinal symptoms include nausea, vomiting, abdominal pain, and diarrhea, which can result from intestinal edema or swelling.

C1-INH is a protein that helps control blood clotting and inflammation, but with HAE is either lacking or does not function normally. Administering a concentrate of this protein was found to be an effective way of managing disease attacks and quickly became a first-line choice of HAE treatment.

Invasive procedures, such as abdominal surgeries, are now limited, with “treatment typically medical in nature,” the researchers wrote.

Scientists in Japan reported the case of an 80-year-old woman with HAE who was admitted to the hospital with severe pain in the lower left abdomen, nausea, and vomiting. She had experienced several gastrointestinal attacks before, but they all tended to go away on their own without disease treatment.

A CT scan revealed edema, or swelling, of the small bowel and stomach, and possible signs of a small bowel obstruction. However, the underlying cause was not evident.

Other tests suggested that her HAE attack episode possibly triggered the obstruction.

She was given a C1-INH concentrate to ease the symptoms. However, contrary to other cases where the medication provided relief in 15 to 20 minutes, she experienced only mild relief an hour later.

A follow-up CT scan also showed little change in her bowel obstruction. As a result, a laparoscopy — a minimally invasive surgery used to access the inside of the abdomen — was needed to determine the cause of her persisting symptoms.

The procedure showed internal herniation and strangulation of the small bowel caused by adhesions — a band of scar tissue that can form between bowel loops. These adhesions can twist or pull the intestine out of place, causing an obstruction.

“This suggests that a patient with adhesions can become symptomatic during a swelling attack, and the adhesions may need to be addressed surgically,” the researchers wrote.

In this case, surgical treatment rapidly improved blood flow in the patient’s small bowel, without the need to remove parts of the organ. She was discharged with no apparent complications after seven days at the hospital.

Overall, the patient did not have a prior clinical history that could explain the encountered diagnosis, suggesting an idiopathic or unknown, spontaneous origin.

The researchers noted that “physicians need to be aware that although C1-INH concentrate remains a major treatment option, HAE may potentially cause bowel obstruction where surgical intervention is necessary.”