Risk of angioedema following invasive or surgical procedures in HAE type I and II – the natural history

Abstract

BackgroundHereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of subcutaneous tissues with potentially fatal complications. As surgery can be a trigger for edema episodes, current guidelines recommend preoperative prophylaxis with C1-INH or attenuated androgens in patients with HAE undergoing surgery. However, the risk of an HAE attack in patients without prophylaxis has not been quantified.ObjectivesThis analysis examined rates of perioperative edema in patients with HAE not receiving prophylaxis.MethodsThis was a retrospective analysis of records of randomly selected patients with HAE type I or II treated at the Frankfurt Comprehensive Care Centre. These were examined for information about surgical procedures and the presence of perioperative angioedema.ResultsA total of 331 patients were included; 247 underwent 700 invasive procedures. Of these procedures, 335 were conducted in 144 patients who had not received prophylaxis at the time of surgery. Categories representing significant numbers of procedures were abdominal (n = 113), ENT (n = 71), and gynecological (n = 58) procedures. The rate of documented angioedema without prophylaxis across all procedures was 5.7%; in 24.8% of procedures, the presence of perioperative angioedema could not be excluded, leading to a maximum potential risk of 30.5%. Predictors of perioperative angioedema could not be identified.ConclusionThe risk of perioperative angioedema in patients with HAE type I or II without prophylaxis undergoing surgical procedures ranged from 5.7% to 30.5% (CI 3.5–35.7%). The unpredictability of HAE episodes supports current international treatment recommendations to consider short-term prophylaxis for all HAE patients undergoing surgery.

Short‐Term Prophylaxis in Odontostomatological, Maxillofacial and ENT Procedures in Patients with Hereditary Angioedema Due to C1‐Inhibitor Deficiency

“…Moreover, a single patient could present or not present AE after the same procedure [70][71][72]. There are no controlled studies that assess STP efficacy; current available data come from observational studies: The risk of perioperative AE without STP (not taking into account dental procedures) is 6-31% [73].…”

“…There is no increased risk related to the procedure location or surgical area size [73]. The prevalence of AE attacks after dental procedures performed without STP varies from 5 to 37% of patients [71][72][73]. Farkas et al observed that 40% of the patients who had not performed STP developed AE post-procedures, when assessing all the interventions as a whole [74].…”

“…Farkas et al observed that 40% of the patients who had not performed STP developed AE post-procedures, when assessing all the interventions as a whole [74]. Most times, AE is local, but distant AE has also been described [72,73]. AE can be triggered by minor procedures, such as local injection of a local anesthetic [43], suture of a hand cut or an aesthetic injection of hyaluronic acid in the lips [72].…”

“…AE can be triggered by minor procedures, such as local injection of a local anesthetic [43], suture of a hand cut or an aesthetic injection of hyaluronic acid in the lips [72]. In some patients, perioperative angioedema was the first manifestation of C1-INH-HAE [72].…”

Hereditary Angioedema in Children — A Rare Clinical Case in the Practice of the Abdominal Surgeon

“…

ВВЕДЕНИЕБоль в животе -одна из наиболее частых причин обращения за помощью к врачу во всем мире [1,2]. Причины боли в животе многочисленны, но в первую очередь необходимо исключить хирургическую патоло-гию, что особенно актуально у детей.

…”