Perioperative anaphylaxis to fibrin sealants in children with Noonan Syndrome: A retrospective study

In the diagnosis of perioperative anaphylaxis, it is essential to define its cause to prevent future reexposures, especially in children with Noonan Syndrome who are characterized by a large number of systemic features and wide-ranging dysmorphia. From an oral surgeon's point of view, apart from an increased risk of tumor incidence, diverse hematologic anomalies are of high concern.

Perioperative management of such patients often requires the use of fibrin sealants, which contain aprotinin. A certain number of anaphylaxis cases have been observed in our daily practice during such treatment, which was the reason for this evaluation.

The study was conducted to retrospectively review perioperative anaphylaxis grade II and above in children with Noonan Syndrome who underwent surgeries in the Department of Oral Surgery, Medical University of Lodz, between 2006 and 2021.

Out of the 16 cases of suspected anaphylaxis to aprotinin, 14 were observed in children with Noonan Syndrome. The postoperative serologic screening revealed positive results for qualitative aprotinin-specific immunoglobulin (Ig) G, highly elevated quantitative aprotinin-specific IgG, and slightly elevated aprotinin-specific IgE antibodies. Interestingly, previous aprotinin administration or contact in the past 12 months was excluded.

Given that fibrin sealants are typically used in various surgical practices and although the anaphylaxis reactions in such cases are rare, it is essential to be cautious in patients with RASopathies who are at a high risk of developing anaphylaxis.