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Circumcision and Factor XI Deficiency: My Personal Experience and Approach

My experience managing circumcision in babies at risk of Haemophilia C

By Dr Martin Harris BPharm Hons, MB BS, LRCP MRCS FRCGP RCPathMe
Circumcision London

Factor XI deficiency, sometimes called Haemophilia C, is an uncommon inherited bleeding disorder which requires particular care when a baby is undergoing surgery.

Circumcision is one of the first surgical procedures that may raise this issue in a baby known to come from a family affected by Factor XI deficiency.

Over the years I have developed considerable experience in this area, including working with specialist haematology teams in London and applying a protocol developed specifically for the management of circumcision in infants at risk of Factor XI deficiency.

My approach has always been that circumcision in a baby with a possible bleeding disorder should never be treated as an ordinary circumcision.

The haematological risk needs to be understood before the procedure takes place.

My involvement in developing a protocol for Factor XI deficiency

During my work at Circumcision London and as a Circumcision Mohel, I worked with Dr Niamh O’Connell when she was a research fellow at the Haemophilia Centre and Haemostasis Unit at the Royal Free Hospital in London.

She was working with Professor Christine Lee and Dr Ri Liesner, Consultant Haematologist and Director of the Haemophilia Centre at Great Ormond Street Hospital for Children.

Dr O’Connell subsequently became a Consultant Haematologist in Ireland.

Together, we investigated aspects of Factor XI deficiency and developed a protocol for the management of circumcision in male infants born to families affected by Factor XI deficiency.

The purpose of the protocol was straightforward:

to identify babies at increased risk of bleeding and to plan their circumcision with appropriate haemostatic support when required.

This work has been particularly important to me because it brought together two areas of my professional practice: circumcision and the careful management of infants with a known or suspected bleeding disorder.

What is Factor XI deficiency?

Factor XI is one of the proteins involved in the blood-clotting system.

When Factor XI is significantly reduced or absent, the normal clotting process can be impaired and some patients may experience prolonged or excessive bleeding, particularly after surgery or other haemostatic challenges.

Factor XI deficiency is uncommon in the general population but is considerably more frequent in Ashkenazi Jewish populations. It affects males and females because the responsible gene is not located on a sex chromosome.

The severity of bleeding does not always correspond precisely to the laboratory Factor XI level. Some people with relatively low levels have little bleeding, while others can experience significant bleeding after surgery.

This is an important point.

A Factor XI result is extremely useful, but it should not be interpreted in isolation.

The baby’s clinical circumstances and the family’s bleeding history also matter.

Why circumcision requires particular planning

Circumcision involves a surgical wound.

In a baby with significant Factor XI deficiency, there may therefore be a greater risk of postoperative bleeding.

Circumcision has historically been reported as one of the situations in which previously unrecognised Factor XI deficiency may become apparent.

This is why I do not regard a family history of Factor XI deficiency as something that can simply be noted in the medical history and then forgotten.

It needs to be actively considered before the circumcision.

Testing the baby

When there is a known family history of Factor XI deficiency, the baby’s Factor XI level can be measured.

An important complication is that Factor XI levels in newborn babies are naturally lower than adult levels.

An apparently low neonatal result may therefore require specialist interpretation using appropriate neonatal reference ranges and, in some circumstances, repeat testing later in infancy.

This is why the laboratory result obtained at birth should not simply be labelled “deficient” using an adult reference range.

The result needs to be interpreted by clinicians familiar with neonatal haemostasis.

The protocol I worked with

The protocol that I worked with used the baby’s Factor XI level to help determine the appropriate management.

In broad terms, the historical approach was:

If the cord blood Factor XI level was above the relevant normal range, circumcision could generally proceed without specific haemostatic treatment.
If the Factor XI level was reduced but above the level considered to represent severe deficiency, circumcision could be considered with appropriate antifibrinolytic cover and haematology involvement.
If the Factor XI level was very low, circumcision was deferred and the baby was reassessed later.
If significant deficiency persisted, circumcision was planned in a hospital environment with appropriate haemostatic support.

The exact thresholds and treatment plan should not be copied from an old protocol and applied independently to a baby today.

Current specialist management may vary according to the individual patient, the bleeding history, the Factor XI result, the planned procedure and the recommendations of the treating haemophilia centre.

The important principle remains the same:

The circumcision should be planned together with the appropriate haematology team before the procedure takes place.

Why the six-month assessment can be important

One of the difficulties with Factor XI deficiency in infancy is distinguishing a genuinely deficient baby from a newborn whose Factor XI level is temporarily low as part of normal neonatal physiology.

For this reason, a very low result at birth is particularly important, but an intermediate result may require specialist interpretation and subsequent reassessment.

This is one reason why, in babies with a particularly low Factor XI level, delaying circumcision can be the safest course.

It gives the haematology team the opportunity to obtain a more informative assessment as the baby becomes older.

Tranexamic acid

One of the treatments that has been used extensively in Factor XI deficiency is tranexamic acid.

Tranexamic acid is an antifibrinolytic medicine. Rather than replacing Factor XI, it helps stabilise a clot once it has formed.

It has an established role in the management of bleeding associated with Factor XI deficiency, particularly around procedures where local fibrinolysis can contribute to bleeding.

Historically, the protocol with which I worked used tranexamic acid for infants whose Factor XI levels were within the range where circumcision could proceed with haemostatic cover.

The precise dose, preparation, timing and duration must, however, be prescribed by the treating clinical team.

Parents should never take the dose from an old protocol or from an internet article and administer it independently.

When Factor XI replacement or plasma may be required

For babies with very low Factor XI levels, a different approach may be necessary.

Depending on the individual circumstances, specialist haematologists may consider Factor XI replacement or fresh frozen plasma.

These treatments are not something I would ask parents to arrange themselves.

They require specialist haematological assessment and, where appropriate, treatment within a hospital setting.

This is precisely why severe Factor XI deficiency changes the setting in which I would consider circumcision.

The importance of planning before the day of circumcision

In my experience, good management begins well before the baby arrives for circumcision.

I want to know:

whether there is a confirmed family history of Factor XI deficiency;
which parent is affected or known to carry the condition;
whether other family members have experienced abnormal bleeding;
whether the baby has had Factor XI testing;
what the result was;
whether the result requires repeat assessment;
what the haematology team recommends;
and what haemostatic treatment, if any, is required.

The circumcision should then be arranged around that plan.

This is very different from discovering on the morning of circumcision that there may be a significant family history of a bleeding disorder.

My relationship with the haematology team

For me, this is one of the most important aspects of caring for these babies.

I do not regard the circumcision practitioner as working independently when a baby has a potentially significant bleeding disorder.

The haematologist understands the coagulation disorder.

I understand the circumcision procedure and the practical aspects of performing and monitoring it.

The parents understand their baby and their family history.

These three parts need to work together.

The aim is not to make the situation unnecessarily complicated.

The aim is to make sure that the right information is available before the procedure takes place.

Why I do not treat Factor XI deficiency as simply a number

There is an understandable temptation to say:

“His Factor XI is X, therefore we do Y.”

Real clinical medicine is rarely quite that simple.

The Factor XI level is important, but bleeding tendency does not always correlate perfectly with the laboratory result.

A previous bleeding history can therefore be highly relevant.

So can the nature of the planned surgery.

This is why specialist haematology input is so important when a baby with possible Factor XI deficiency is being considered for circumcision.

My personal approach to the procedure

When I perform circumcision in this setting, I approach the baby with the same care that I would give to any infant undergoing the procedure, but with additional attention to the haemostatic plan.

I want the Factor XI result to be known and understood.

I want the haematology plan to be clear.

I want the parents to know what treatment has been arranged and what they should do if there is unexpected bleeding.

And I want appropriate medical support to be available if it is required.

For me, this is not simply about performing the circumcision.

It is about managing the entire episode of care responsibly.

Why I believe experience matters

Factor XI deficiency is uncommon.

Consequently, many parents may understandably find it difficult to know who has genuine experience of managing circumcision in this particular situation.

My involvement in developing and applying a protocol for circumcision in infants from families affected by Factor XI deficiency has given me experience of the practical issues involved.

That experience has taught me one particularly important lesson:

Preparation is far better than reacting to bleeding after the event.

A baby with a known or suspected bleeding disorder should be assessed and planned for before the circumcision.

Circumcision and Haemophilia C: the key points for parents

If your baby has a family history of Factor XI deficiency, I would encourage you to discuss the situation with the relevant haemophilia or haematology team before arranging circumcision.

The important points are:

Factor XI deficiency can increase the risk of bleeding following surgery.
Factor XI deficiency is particularly common in Ashkenazi Jewish populations.
Newborn Factor XI levels are naturally lower than adult levels.
A low neonatal result therefore needs specialist interpretation.
Some babies require repeat testing as they become older.
A very low Factor XI level may mean that circumcision should be delayed.
Tranexamic acid may be used as haemostatic cover in appropriate circumstances.
Babies with severe deficiency may require hospital-based management and replacement therapy.
The baby’s bleeding history and clinical circumstances are important as well as the laboratory result.
Parents should not use an old protocol to calculate or administer medication independently.

My final message to parents

I understand that parents may initially find the words “Factor XI deficiency” and “circumcision” a worrying combination.

My experience is that careful planning makes a significant difference.

There is no reason to approach the situation with unnecessary alarm, but there is also no reason to be casual about it.

The important thing is to know about the possible bleeding disorder before the procedure, obtain the appropriate laboratory information, involve the specialist haematology team and agree a haemostatic plan before circumcision takes place.

That is the approach I have followed in my work with families affected by Factor XI deficiency.

For me, the central principle is simple:

Know the risk. Plan the procedure. Work with the haematology team. And never leave the management of a potentially significant bleeding disorder to chance.

Medical Information Disclaimer

This article describes my professional experience and explains the principles behind a historical protocol with which I was involved.

It is provided for general educational information and is not an individual treatment plan.

Factor XI deficiency is a specialist bleeding disorder. A baby’s Factor XI result should be interpreted by an appropriately qualified haematologist or paediatric coagulation specialist, particularly because normal Factor XI levels in newborns differ from adult levels.

Parents should not use the information in this article to decide whether their baby should undergo circumcision, to calculate a dose of tranexamic acid or another medicine, or to arrange haemostatic treatment independently.

Current specialist recommendations may differ from historical protocols, and treatment decisions should be made by the baby’s treating clinicians and haemophilia centre.

If your baby has known or suspected Factor XI deficiency and circumcision is being considered, the procedure should be discussed with the appropriate specialist team before it is undertaken.

Last reviewed: October 2026