Tailored prophylaxis prevented HAE attacks during tooth extraction

Previous dental work resulted in the man asphyxiating, needing tracheotomy

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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A boy opens his mouth as dental floss, toothpaste, a toothbrush, and other items for oral health hover next to him.

Adding a short-term regimen of C1-INH concentrate to the preventive treatment of a 63-year-old man with hereditary angioedema (HAE) enabled a successful tooth extraction without a swelling attack in his larynx, a case study reports.

The tailored Orladeyo (berotralstat) prophylaxis regimen was designed to prevent an attack after the man asphyxiated when his larynx swelled following a previous extraction more than 20 years earlier, before he received a HAE diagnosis.

“Administering short-term prophylaxis with ongoing long-term prophylaxis for HAE and perioperative multidisciplinary management for tooth extraction helped prevent recurrent fatal angioedema due to dental procedures and this can be useful when managing patients with HAE,” a team led by researchers at the Chiba University, Japan wrote in “Multidisciplinary Prophylactic Strategies for Recurrence of Laryngeal Edema After Tooth Extraction in a Patient With Hereditary Angioedema: A Case Report,” which was published in Cureus. Perioperative refers to the period immediately before, during, and after a surgery.

Swelling attacks in HAE are driven by excessive levels of bradykinin, a pro-inflammatory molecule, whose production is controlled by the kallikrein enzyme. In HAE types 1 and 2, mutations in the SERPING1 gene lead to deficient levels of working C1 inhibitor (C1-INH), a protein that blocks kallikrein’s activity.

HAE attacks can affect several parts of the body and routine dental procedures, such as tooth extraction, can trigger the larynx to swell, posing a risk for asphyxiation. Worldwide guidelines recommend short-term prophylaxis in HAE patients before any invasive medical or dental procedure, along with long-term prophylaxis to prevent swelling attacks.

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A history of swelling attacks

The man had swelling in his hands and feet since junior high school and it recurred in other parts of his body throughout his life.

When he was in his 40s, a tooth extraction led to his larynx swelling up and asphyxiation. An emergency tracheotomy, a surgical opening through the neck into the windpipe, saved his life.

After that, the man avoided invasive dental procedures due to a fear of asphyxiation, but he still had two to three attacks every month, mostly on his hands.

“He knew that his father and older brother had been diagnosed with HAE, but he had not been definitively diagnosed,” the researchers wrote.

In September 2022, he was referred to undergo another tooth extraction due to advanced caries in a molar. Before the procedure, and given his clinical history, he had blood work that confirmed C1-INH’s deficient function, with levels 25% below normal, and low levels of the immune complement protein C4 — another common feature of HAE.

The man was diagnosed with HAE, based on these findings and personal and family history, and the dental procedure was delayed because of the high risk of asphyxiation.

His primary physician was asked to find an alternative for one of his diabetes medications, linagliptin (sold as Tradjenta, among others), since it can trigger and worsen swelling attacks. In January, the man was prescribed Orladeyo, a once-a-day oral therapy for preventing HAE attacks, and began long-term treatment, which effectively prevented further ones.

He also was prescribed and trained to self-administer icatibant injection (sold as Firazyr, with generics available), which is used on demand for swelling attacks.

A successful tooth extraction

In April, the man was hospitalized as a high-risk case a day before his scheduled tooth extraction. Along with Orladeyo, he was given a C1-INH concentrate two hours before the procedure as a short-term prophylaxis. He was also given into-the-vein, or intravenous, tranquilizers and local anesthesia in the operating room. The tooth was removed without any problems.

“Dentists performed [the] tooth extraction with minimum trauma to surrounding structures, anesthesiologists managed the intravenous anesthesia, and emergency physicians were prepared to perform lifesaving tracheal intubation or tracheotomy procedures when laryngeal edema appeared,” the researchers wrote.

The healthcare team was also ready to administer C1-INH concentrate and icatibant on demand during his hospitalization if any signs of attack were detected. The patient was discharged the next day without recurrence of a HAE attack.

“We can consider that short-term prophylaxis with C1-INH concentrate under long-term prophylaxis with [Orladeyo] in this case could partially contribute to preventing the recurrence of laryngeal edema after tooth extraction,” the researchers wrote. “We believe that it is important for physicians and dentists to always recognize HAE and ask patients with a history of angioedema to rule out HAE before invasive medical and dental procedures.”