Pediatric emergency medicine trisk 562

Type of hemorrhage

Factor replacement
guidance

Additional management

• Life-threatening
hemorrhage
• Severe head injury
• CNS bleed or nerve
compression
• Major trauma
• Throat/neck swelling
• Intra-abdominal/GI
bleed

100% factor correction; may Appropriate intensive care
consider continuous
Head imaging
factor infusion depending Additional imaging studies
on injury (FVIII) or q12hr
dosing (FIX)

Major bleed/procedure
50–100% initial factor
correction and will likely
• Hemarthrosis
need repeated doses
• Iliopsoas or thigh bleed
• Compartment syndrome

(forearm/calf)
• Periodontal surgery
• Major surgery

Rest, ice, compression,
elevation
No weight bearing
Non-NSAID pain control
Measurements may be
helpful to check for
enlargement

Minor bleed/procedure
• Expanding SQ bleed
• Muscle
• Fracture
• Tooth extraction/filling
• Mild/moderate trauma
• Sutures

25–50% factor correction;
may need repeated doses
Fractures require factor
replacement at least until
swelling subsides (at least
2 days and generally 5–7
days)

Rest, ice, compression,
elevation

No weight bearing
Non-NSAID pain control
Measurements may be
helpful to check for
enlargement

Minimal bleeding
• Epistaxis
• Subcutaneous bleed
• Mouth/tongue
laceration
• Abrasions/lacerations
(no sutures)

Factor replacement typically Hold pressure/cold
not needed
compress. Consider
antifibrinolytic agent
Measurement of bruise may
be helpful to check for
enlargement

Hematuria
Factor replacement only if
(painless/nontraumatic)
fails all conservative
measures

Increase fluid intake PO/IV
Exclude infectious etiology

Bed rest
Steroids (0.5–1 mg/kg/day)
× 3 days if persists after


24 hrs of increased
hydration
Antifibrinolytic medications
contraindicated
Emicizumab is a recombinant, humanized, bispecific monoclonal antibody that
mimics part of the cofactor function of activated factor VIII promoting improved
hemostasis in individuals with FVIII deficiency with or without inhibitors. It is indicated
for prophylaxis in both adults and children and is administered subcutaneously typically
weekly. Emicizumab is not indicated for bleed treatment. It may provide adequate
hemostasis for minor procedures; however, major surgeries or trauma will require
additional hemostatic agents. For patients without inhibitors, FVIII replacement using
conventional dosing strategies is appropriate. For patients with inhibitors, rFVIIa is the
bypassing agent of choice as several severe adverse events have occurred with
concomitant use of activated prothrombin complex concentrate (aPCC) FEIBA (Factor
Eight Inhibitor Bypassing Agent). Emicizumab interferes with routine coagulation
assays such as aPTT and FVIII activity assays. These tests should not be sent or used to
inform clinical decision making about a patient’s level of hemostasis.
CNS Bleed/Head Injury. All major head injuries require evaluation by a physician and
immediate treatment with a goal of correction to 100% factor activity level. A pathway
for the management of head trauma in patients with hemophilia is avaliable at
.
For patients with minor trauma without any symptoms or external evidence of injury, or
the child with mild hemophilia, replacement therapy may not be required. Any child
with moderate or severe hemophilia needs careful attention to the type of trauma and
bleeding history for the physician to decide whether to use replacement therapy.

A CT scan of the head is indicated for patients with hemophilia if there is any sign of
trauma (e.g., hematoma), history of falling downstairs or from crib/changing table,
hitting the head on a hard surface or corner, altered mental status, vomiting, or focal
neurologic changes. There should be a low threshold to perform a CT scan on patients
with prior history of head bleed even if the mechanism of injury seems minor. Children
with seemingly insignificant trauma may develop the first obvious signs of intracranial
bleeding several days later when concern has diminished. For patients with mild to
moderate hemophilia, imaging is indicated for any clinical features concerning for
neurologic injury such as lethargy, vomiting, or focal neurologic changes. For children
with indeterminate presentations (e.g., mild mechanism of injury and normal neurologic
examination), consideration of imaging versus observation is based on the severity of
the hemophilia combined with usual management strategies for pediatric head trauma. If
the trauma is mild (e.g., a light bump on the forehead), patients may be observed at
home for the usual signs of intracranial hemorrhage or increased intracranial pressure.


Ruling out intracranial hemorrhage does not exclude the possibility of a concussion.
Persistent symptoms after an intracranial bleed have been excluded warrant follow-up
with the patient’s primary provider and specialists as indicated.
Emergent or Major Surgery. The indications for surgery are similar to those for children
without coagulation disorders, provided an appropriate correction of clotting
abnormalities is planned. When the need for surgery has been definitively established,
correction up to 100% should be given and the aPTT should be measured to ensure its
normalization.
Throat or Neck Swelling. For bleeds of the neck muscles, careful evaluation of airway
patency is essential. Pressure on the airway from a fascial bleed of the neck may become
life threatening, requiring steps to ensure airway patency such as endotracheal
intubation, in addition to correction of the factor level to 100%. Complete airway
obstruction may also result from extensive bleeding in the tongue. Early anticipation of
the need to secure the airway is essential in these presentations.

Compartment Syndrome. If there is concern for nerve compression or vascular
insufficiency in a child with a muscle or soft tissue bleed, immediate intervention is
important. Compartment syndrome is a limb-threatening event. Imaging of the affected
area and consultation with orthopedics is necessary.
Iliopsoas or Thigh Hemorrhage. Retroperitoneal bleeds can be life threatening and may
present with lower abdominal pain. A mass is sometimes palpable deep in the pelvis,
and sensation in the distribution of the femoral nerve may be diminished. Loss of the
psoas shadow may be seen on an abdominal radiograph, and a hematoma may be
demonstrated by ultrasonography. The hemoglobin level should be measured initially
and, if bleeding persists, at regular intervals thereafter.
Hematuria. When the child with hemophilia develops hematuria or flank tenderness
after trauma, a more aggressive approach to diagnosis and treatment is required.
Perform ultrasound, CT, or MRI as soon as possible to look for subcapsular or intrarenal
bleeding or an obstructive clot at the pelvic–ureteral junction. To prevent parenchymal
damage and deterioration of renal function, administer replacement therapy to achieve a
level of 70% to 100% immediately. Atraumatic, painless hematuria generally does not
require replacement therapy, but rest and hydration are key.
Management of Patients with Inhibitors. The treatment of bleeding episodes in the child
with hemophilia and alloantibodies (i.e., inhibitors) against the missing or diminished
factor is challenging and should be managed in conjunction with a hematologist
especially for those hemophilia A patients on emicizumab prophylaxis. Options for
treatment depend on the clinical scenario and inhibitor titer. Possible therapies include


massive doses (100 to 200 units/kg) or continuous infusion of factor product, aPCCs
such as FEIBA, or rFVIIa [NovoSeven]). Hemophilia B patients with inhibitors are at
risk of severe allergic reaction/anaphylaxis when receiving factor; FEIBA is
contraindicated in this setting because it contains factor IX. Factor IX inhibitor
complexes can precipitate and eventually cause nephrotic syndrome and renal failure.
Clinical Indications for Discharge or Admission

Severe, life-threatening bleeding events require monitoring for CNS or airway
complications. Hemarthrosis may require hospitalization for immobilization and
elevation of limb, pain control, or serial examinations. Minor or moderate bleeds
including some hemarthroses and superficial muscle bleeds can usually be managed on
an outpatient basis.
VON

Willebrand Disease

CLINICAL PEARLS AND PITFALLS
DDAVP is a good therapeutic option for patients with type 1 disease who
have previously demonstrated response to a DDAVP challenge.
DDAVP is generally ineffective for those with subtypes of type 2 and type 3
disease and can exacerbate symptoms in patients with type 2B disease by
accelerating platelet clearance.
Patients with type 3 VWD typically manifest a clinical phenotype similar to
patients with severe hemophilia.

Current Evidence
VWD is the most common inherited bleeding disorder affecting about 1% of the
population; however, only a small fraction of patients manifest bleeding symptoms.
VWF plays a key role in coagulation by facilitating platelet aggregation and adhesion
and serving as a carrier molecule for factor VIII. There are three main types of VWD:
Type 1 is a deficiency of VWF, type 2 subtypes are functional defects associated with
VWF, and type 3 is an absence of VWF. The vast majority of patients with VWD have
type 1, inherited in an autosomal dominant pattern with variable expressivity and
penetrance.

Clinical Considerations
Clinical Recognition

The sites of bleeding in VWD (types 1 and 2) resemble the mucocutaneous bleeding
found in patients with platelet disorders such as bruising, epistaxis, oral bleeding, GI
bleeding, and menorrhagia. Joint and deep muscle bleeding is unusual except in cases of
children affected by type 3 VWD. Since the most common type of VWD is a dominant