Male babies born to mothers with HAE: circumcision guidelines
Question:
7/19/2017
Recent international consensus guidelines recommend considering neonates born to mothers with HAE as having HAE themselves for the first year of life. However, this raises the question of whether to prophylax empirically for minor procedures in the first year of life. Male circumcision is common practice in many countries around the world, and often is performed in the first weeks of life. Have high volume HAE centers in areas of the world with high neonatal circumcision rates found it necessary or advisable to empirically administer C1 inhibitor to neonates born to mothers with HAE prior to performing circumcision on those neonates? Thank you for your help with this question.
Answer:
Thank you for this question. We sought the expert opinion of Dr. Marc Riedl. See his response below.
"No published study data exists to assist us with this question, but in a large number of families I’ve managed over the years, I’m not aware that circumcision has triggered angioedema symptoms in infant boys who ultimately are proven to have HAE established by C1INH testing at or after 12 months of age. I also checked with Dr. Andrew MacGinnitie at Boston Children’s given his experience with pediatric HAE - he also has not observed HAE attacks associated with infant circumcision. So anecdotally it appears that HAE symptoms are a very uncommon complication with this procedure done shortly after birth. However, I always recommend that if a parent is affected by HAE, the infant/toddler should be monitored closely for symptoms of HAE in general and certainly following medical procedures. An acute treatment plan should be devised (typically with C1INH concentrate used for acute treatment) in the uncommon scenario of angioedema occurring prior to 1 year of age. By age 1, C1INH laboratory tests are reliably accurate and children from a parent with HAE should be tested. With the cost of gene sequencing dropping rapidly, familial genetic testing may be more routinely done in the future, enabling accurate HAE testing even earlier in life."
We hope this helps.
Patricia McNally, MD, FAAAAI