Overview
Definition:
Recurrent epistaxis in children refers to frequent episodes of bleeding from the nasal mucosa, typically occurring more than once a week or significantly impacting quality of life
When associated with underlying coagulopathy, it signifies a systemic bleeding tendency that requires prompt and thorough investigation.
Epidemiology:
Epistaxis is common in children, with a peak incidence in preschool and school-age years
While most cases are benign, approximately 10-20% are considered recurrent
The prevalence of underlying coagulopathies presenting as recurrent epistaxis varies depending on the specific disorder, but inherited bleeding disorders are a significant cause.
Clinical Significance:
Recurrent epistaxis in the context of coagulopathy is a critical red flag for potentially serious underlying hematological conditions
Early identification and management are vital to prevent complications such as anemia, shock, airway compromise, and significant morbidity, impacting long-term health and well-being.
Clinical Presentation
Symptoms:
Frequent or prolonged nosebleeds
Bleeding from other sites such as gums, gastrointestinal tract, or genitourinary system
Easy bruising (ecchymoses)
Petechiae
Prolonged bleeding after minor trauma or surgery
Family history of bleeding disorders.
Signs:
Pallor
Tachycardia (if significant blood loss)
Evidence of active nasal bleeding
Nasal mucosal pallor or dryness
Petechiae or ecchymoses on skin or mucous membranes
Signs of anemia (e.g., conjunctival pallor)
Enlarged lymph nodes
Hepatosplenomegaly (less common, but seen in some hematological conditions).
Diagnostic Criteria:
No universal diagnostic criteria exist for recurrent epistaxis with coagulopathy, as it is a presentation rather than a specific diagnosis
The diagnosis relies on identifying recurrent epistaxis and subsequently confirming an underlying bleeding disorder through a systematic workup.
Diagnostic Approach
History Taking:
Detailed history of epistaxis frequency, duration, severity, and triggers
Nature of bleeding (anterior vs
posterior)
History of bleeding after dental procedures, tonsillectomy, or trauma
Presence of other bleeding sites (gums, GI, GU, skin)
Family history of bleeding disorders, easy bruising, or prolonged bleeding
Medications (e.g., NSAIDs, anticoagulants)
Birth history (e.g., prematurity, birth trauma).
Physical Examination:
Thorough examination of the nasal cavity for source of bleeding, including anterior rhinoscopy
Assess for pallor, petechiae, ecchymoses, and bleeding from other mucosal surfaces
Palpate abdomen for hepatosplenomegaly
Assess lymph nodes
Examine skin for signs of trauma or purpura
Assess neurological status if trauma is suspected.
Investigations:
Complete Blood Count (CBC) with differential and platelet count: to assess anemia and thrombocytopenia
Peripheral smear: to assess platelet morphology and red blood cell indices
Coagulation profile: Prothrombin Time (PT), Activated Partial Thromboplastin Time (aPTT), International Normalized Ratio (INR)
Clotting Factor Assays: if initial coagulation tests are abnormal, assay specific factors (e.g., Factor VIII, Factor IX, vWF antigen, ristocetin cofactor activity, Factor VII, Factor X, Factor V, Factor II, Factor XIII)
Platelet Function Tests: Platelet aggregation studies, bleeding time (less commonly used now)
Other tests: Fibrinogen level, D-dimer (if DIC is suspected).
Differential Diagnosis:
Common epistaxis (idiopathic, trauma, infection, foreign body)
Hereditary bleeding disorders (Hemophilia A/B, Von Willebrand Disease types 1, 2, 3, other factor deficiencies)
Platelet disorders (thrombocytopenia - ITP, TTP, congenital thrombocytopenias
platelet function defects - Glanzmann thrombasthenia, Bernard-Soulier syndrome)
Acquired coagulopathies (liver disease, vitamin K deficiency, DIC, anticoagulation therapy)
Hereditary hemorrhagic telangiectasia (HHT)
Nasal tumors or polyps (less common in children as cause of recurrent epistaxis)
Vasculitis.
Management
Initial Management:
Direct pressure to the anterior nasal vault for 10-15 minutes
Patient should sit upright and lean forward to prevent aspiration of blood
Nasal packing (anterior or posterior) if bleeding is severe and persistent
Intravenous access for fluid resuscitation if hypovolemia is present
Identify and treat any immediate life-threatening complications like airway obstruction or hemorrhagic shock.
Medical Management:
Management is primarily directed at the underlying coagulopathy
This may involve: Factor replacement therapy (e.g., recombinant factor concentrates or plasma-derived concentrates for hemophilia)
Desmopressin (DDAVP) for mild Hemophilia A and Von Willebrand Disease type 1
Antifibrinolytic agents (e.g., tranexamic acid, aminocaproic acid) for mucosal bleeding
Management of anemia with iron supplementation or transfusion if severe
Treatment of underlying causes like liver disease or vitamin K deficiency.
Surgical Management:
Surgical intervention is reserved for cases refractory to medical management
Options include: Cautery (chemical or electrical) of bleeding points
Nasal packing (balloon catheters, gauze)
Surgical ligation of specific arteries (e.g., anterior ethmoidal artery, internal maxillary artery).
Supportive Care:
Close monitoring of vital signs, hemoglobin levels, and coagulation parameters
Adequate hydration and pain management
Humidification of inspired air to prevent mucosal dryness
Education of parents and child regarding bleeding precautions and management strategies.
Complications
Early Complications:
Anemia from chronic blood loss
Airway compromise due to massive bleeding or airway obstruction from packed nares
Hemorrhagic shock
Hematoma formation
Acute infection secondary to packing or trauma.
Late Complications:
Chronic anemia
Sinusitis secondary to nasal packing
Nasal septal deviation or perforation
Psychological impact on the child and family due to recurrent bleeding episodes
Development of inhibitors to factor replacement therapy in hemophilia patients.
Prevention Strategies:
Prompt and accurate diagnosis of the underlying coagulopathy
Strict adherence to prescribed medical management and factor replacement
Avoidance of triggers like nasal trauma, dry air, and certain medications (NSAIDs)
Patient and family education on home management and bleeding precautions
Regular follow-up with hematology.
Prognosis
Factors Affecting Prognosis:
The specific type and severity of the underlying coagulopathy
Timeliness and adequacy of diagnosis and management
Development of inhibitors
Presence of comorbidities
Adherence to treatment protocols.
Outcomes:
With appropriate management of the underlying coagulopathy, the prognosis for recurrent epistaxis is generally good, with significant reduction in bleeding frequency and severity
However, patients with severe bleeding disorders or those who develop inhibitors may face a more challenging long-term course
Lifelong monitoring and management are often required.
Follow Up:
Regular follow-up appointments with a pediatric hematologist are essential for monitoring disease activity, treatment efficacy, and managing potential complications
This includes periodic laboratory assessments and assessment for inhibitor development
Patients and families should be educated on when to seek immediate medical attention.
Key Points
Exam Focus:
Systematic approach to recurrent epistaxis workup in children
Differentiating common causes from serious bleeding disorders
Key laboratory tests for coagulopathy (PT, aPTT, platelet count, specific factor assays)
Management principles for Hemophilia A/B and Von Willebrand Disease
Role of DDAVP and antifibrinolytics.
Clinical Pearls:
Always suspect an underlying coagulopathy in recurrent or severe pediatric epistaxis, especially with a positive family history or bleeding from other sites
A normal PT and aPTT do not rule out all bleeding disorders
specific factor assays are crucial if suspicion remains high
Early identification of inhibitors in hemophilia patients is critical for optimal management
Empower parents with knowledge about home management and emergency signs.
Common Mistakes:
Attributing all recurrent epistaxis to simple nasal dryness or trauma without adequate investigation
Delaying coagulation screening or specific factor assays
Inadequate management of severe bleeding episodes leading to anemia or shock
Failing to consider less common but serious causes like HHT or acquired coagulopathies
Not educating families on long-term management and precautions.