Open Access Case

Report DOI: 10.7759/cureus.3324

A Newborn with Simmering Bleeding after

Circumcision
Lars Mense 1 , Emanuela Ferretti 2 , Raveena Ramphal 3 , Thierry Daboval 4

1. Neonatology, University Hospital Carl Gustav Carus, Dresden, DEU 2. Pediatrics, Children's Hospital of
Eastern Ontario, Ottawa, CAN 3. Haematology / Oncology, Children's Hospital of Eastern Ontario,
Ottawa, CAN 4. Paediatrics, Children's Hospital of Eastern Ontario, Ottawa, CAN

 Corresponding author: Thierry Daboval, tdaboval@cheo.on.ca

Disclosures can be found in Additional Information at the end of the article

Abstract
We present a case of a healthy male neonate born at term, circumcised on Day 1 of life. Facing
ongoing bleeding at the incision site, the baby was transferred to a level III neonatal intensive
care unit for further investigation and management. His family history was unremarkable for
bleeding disorders. On arrival, the baby was hemodynamically stable with abnormal
coagulation values. Further investigations revealed a diagnosis compatible with severe
hemophilia A. He deteriorated on Day 2, developing acute severe anemia which required two
red blood cell transfusions. This rare but potentially fatal event reminds clinicians to remain
extremely vigilant with minor surgical procedures such as circumcision even in the absence of
family history.

Categories: Pediatrics
Keywords: hemophilia, circumcision, bleeding disorder, newborn, coagulation factors

Introduction
Diagnosing inherited bleeding disorders in newborns can be difficult including interpretation of
preliminary blood results and identifying the cause of the bleeding [1]. Whether bleeding in a
healthy newborn, who has had a minor procedure, is considered abnormal is often a judgement
call and can cause anxiety to parents and health care providers. Clinicians need to remain
extremely vigilant for bleeding disorders after minor surgical procedures. We present a case of a
healthy male neonate born at term who was circumcised on his first day of life and developed
persistent bleeding at the surgical site. The patient had a negative family history for
coagulopathy but was later diagnosed with hemophilia A. The differential diagnosis of
abnormal bleeding in a newborn is reviewed and treatment is discussed.

Received 07/05/2018
Review began 07/09/2018 Case Presentation
Review ended 09/14/2018
Published 09/18/2018 After an unremarkable pregnancy and negative family history for bleeding, a male term neonate
(39+5 weeks of gestation, birth weight 3295 grams) was born via spontaneous vaginal delivery
© Copyright 2018
at a level II hospital. He did not require resuscitation. He received one dose of vitamin K
Mense et al. This is an open access
article distributed under the terms of intramuscularly (IM) as per recommendation [2] without bleeding complications. The mother
the Creative Commons Attribution mentioned later that she had noted persisting bleeding after heel pricks.
License CC-BY 3.0., which permits
unrestricted use, distribution, and
reproduction in any medium, provided
Parents opted to circumcise their son for social reasons. Persistent bleeding complicated the
the original author and source are procedure done on Day 1 of life. Repeated sutures to stop the bleeding were ineffective,
credited. however the baby stayed hemodynamically stable. The following day, the baby was transferred
to a level III neonatal intensive care unit for further management. On admission, the physical

How to cite this article

Mense L, Ferretti E, Ramphal R, et al. (September 18, 2018) A Newborn with Simmering Bleeding after
Circumcision. Cureus 10(9): e3324. DOI 10.7759/cureus.3324
 examination was unremarkable except for some fresh blood noted at the surgical site (Figure 1)
from a diffusely simmering bleed and hematomas in the typical locations for blood sampling.
The amount of blood loss was difficult to assess with certainty given the high absorption
capacity of the diapers. We therefore remained vigilant for significant bleeding by regularly
checking the hemoglobin level. In keeping with earlier observations, the baby developed
significant hematomas after blood work and intravenous (IV) access; however, there was no
evidence of ecchymosis or muscle hematoma at the site of the vitamin K intra-muscular
injection. Blood work showed: hemoglobin 142 g/L (reference range: 135–200 g/L), platelets
139 x 109/L (reference range: 150–600 x 109/L), prothrombin time (PTT) > 150s (reference
range: 25.0–60.0s), prothrombin time (PT) international normalized ratio 1.07 (reference range:
0.80–1.16).

FIGURE 1: Surgical site on patient admission.

Simmering bleeding is noted. Yellow arrow shows the surgical site.

Initially, the estimated blood loss was not concerning and further surgical interventions did not
seem helpful given the poorly circumscribed origin of the bleeding. The bleeding seemed to
slow down remarkably with sterile dressings and topical tranexamic acid.

Surprisingly, acute severe anemia (hemoglobin 58 g/L) was noted the following morning, but
fortunately the baby was clinically and hemodynamically stable. Results of further
investigations to explore the prolonged PTT were now available and revealed: Factor VIII
(FVIII) < 0.01 IU/ml (reference range: 0.50–1.57 IU/ml), normal values for factor IX, XIII, von
Willebrand-antigen and von Willebrand-activity. This was compatible with the diagnosis of
severe hemophilia A (FVIII < 0.01 UI/ml).

The newborn received one packed red blood cell (PRBC) transfusion (15 ml/kg) and 250 units
(70 IU/kg) of recombinant (genetically produced) FVIII concentrate. The dose was based on the

2018 Mense et al. Cureus 10(9): e3324. DOI 10.7759/cureus.3324 2 of 5

 entire vial being administered to prevent wastage of an expensive product. The following day,
severe anemia (hemoglobin 72 g/L) was noted again and a second PRBC transfusion was given.
Head and abdominal ultrasound ruled out internal hemorrhage. After educating the parents, the
baby was discharged home and since then is regularly followed by the hemophilia clinic at the
local institution.

Gene investigations revealed that the baby has a nonsense mutation which is associated with a
30% to 40% of developing inhibitors. This is the typical rate of inhibitor development in severe
haemophiliac patients [3]. His mother was also found to carry the same mutation. The child is
currently two years and seven months old and receives weekly prophylaxis with plasma derived
FVIII concentrate. He has no evidence of circulating inhibitors after 87 exposure days to FVIII
concentrate.

Discussion
Hemophilia is a mostly inherited genetic disorder typically inherited through an X
chromosome. Hemophilia A (FVIII deficiency) affects <1 in 10,000 people (14.2 per 10,000
males), approximately 2500 Canadians. Hemophilia B (Factor IX (FIX) deficiency) affects 1 in
50,000 people (1 per 20,000 males), approximately 600 Canadians. About 30% of cases are a
spontaneous mutation with no family history [3]. The severity of hemophilia A is defined by the
amount of FVIII measured (mild 0.05–0.40 IU/ml; moderate 0.01–0.05 IU/ml and severe < 0.01
IU/ml). Muscle and joint bleeding is typical. Administration of the decreased clotting factor
allows effective treatment nowadays but can be complicated by the development of antibodies
against the infused factor (“inhibitor”) in 25–50% of people with severe FVIII deficiency, 1–2%
of people with mild or moderate FVIII deficiency and 1.5–5% of people with severe FIX
deficiency [4,5]. Earlier publications suggested an inverse correlation between the age (<6
months) of first exposure and inhibitor formation. However, more recent studies that
controlled for known confounders were unable to demonstrate an association with age [6].
Patients at higher risk of developing inhibitors are people with haemophilia with relatives who
have inhibitors, those who have large genetic mutations and, people with haemophilia of
African and Latino descent [5,7,8]. Historically, infections with HIV and hepatitis C posed a risk
of factor treatment but today recombinant concentrates have overcome this hazard.

This case reminds clinicians to be extremely vigilant even with minor surgical procedures to
avoid unexpected, potentially fatal, outcomes. Two important observations are worth
mentioning:

First, major bleeding after circumcision is a rare event [9] and should always call for a
diagnostic workup. The differential diagnoses of abnormal bleeding in a newborn include
diseases of cellular and plasmatic coagulation (Table 1). Considering that approximately 30% of
hemophilia cases are new mutations, absence of family history for coagulopathy may mislead
clinicians and potentially delay the diagnosis. Blood work is not generally necessary before
circumcision, but given the short history in a newborn baby, physicians should consider
investigations like complete blood cell count, PT, PTT and consulting a pediatric hematologist
in the presence of subtle signs of coagulopathy prior to performing even minor surgery. A
critical risk-benefit appraisal is essential for decision making in these patients and special
precautions to minimize bleeding can be taken if a coagulopathy is diagnosed before surgery
and the decision [3] to pursue is made.

2018 Mense et al. Cureus 10(9): e3324. DOI 10.7759/cureus.3324 3 of 5

 Cause Diagnoses

Surgical Local bleeding

Neonatal alloimmune thrombocytopenia

Disseminated intravascular coagulation

Cellular
Other forms of thrombocytopenia

Platelet dysfunction

Hemophilia A

Hemophilia B
Plasmatic
Von Willebrand syndrome

Vitamin K deficiency

TABLE 1: Selected differential diagnoses of abnormal bleeding after neonatal

circumcision in a well newborn.

Second, the extraordinary absorption capacity of the modern diapers could be really challenging
when trying to estimate blood loss volume in such a circumstance. This phenomenon needs to
be recognized and could explain the team’s underestimation of blood loss.

Here below some important points for clinicians to remember:

Clinical pearls
· Minimum workup for coagulopathies should be considered before minor surgical
procedures such as neonatal circumcision if subtle clinical signs of bleeding disorders are
present even with a negative family history. Mainly male newborns are at higher risk of severe
hemophilia.

· A pediatric hematologist should be consulted to help guide treatment decisions and

ensure adequate followup when hemophilia is suspected.

· Simmering bleeding in superabsorbent diaper might lead to underestimation of blood

loss.

Conclusions
We described a very rare case of neonatal haemophilia A with negative family history for
bleeding disorder that presented after what seemed to be a limited but persistent bleed after
circumcision. Clinicians need to be extremely attentive in such circumstances to be able to
diagnose a bleeding disorder early to prevent more severe complications associated with
unrecognized but significant bleeding. Benefits and risks of administration of FVIII should be
always discussed with a pediatric hematologist.

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 Additional Information
Disclosures
Human subjects: Consent was obtained by all participants in this study. Conflicts of interest:
In compliance with the ICMJE uniform disclosure form, all authors declare the following:
Payment/services info: All authors have declared that no financial support was received from
any organization for the submitted work. Financial relationships: All authors have declared
that they have no financial relationships at present or within the previous three years with any
organizations that might have an interest in the submitted work. Other relationships: All
authors have declared that there are no other relationships or activities that could appear to
have influenced the submitted work.

Acknowledgements
We would like to thank Roxane Daboval for her editorial assistance.

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