Causes of epistaxis in children

INTRODUCTION — The epidemiology and etiology of epistaxis in children will be reviewed here. The evaluation and management of epistaxis in children are discussed separately. (See "Evaluation of epistaxis in children" and "Management of epistaxis in children".)

EPIDEMIOLOGY — Epistaxis is a common presenting pediatric complaint that occurs in an estimated 30 percent of children aged zero through five years, and over 50 percent of children over five years of age [1,2]. The incidence of epistaxis declines in adulthood, but approximately one-half of all adults with epistaxis had nosebleeds during childhood [3]. Epistaxis accounts for approximately 250,000 visits annually in the United States [4,5]. The mean age at presentation is typically seven to eight years old [1]. Epistaxis is rare in children younger than two years (approximately 1 per 10,000) and should prompt consideration of trauma (intentional or unintentional) or serious illness (eg, thrombocytopenia). Epistaxis in a young infant without a plausible explanation may be a marker for child abuse. (See "Physical child abuse: Recognition", section on 'Oral or nasal injuries'.)

Epistaxis that occurs in children younger than 10 years usually is mild, originates in the anterior nose, and typically does not require cautery or packing for control of bleeding [1]. For the rare instance where cautery or packing is required, hospitalization is seldom indicated unless the child has a serious underlying cause (eg, local tumor, bleeding disorder, or malignancy) [6]. (See "Management of epistaxis in children".)

An increased incidence of epistaxis occurs during hot or cold weather and when ambient humidity is low, all of which make the nasal septal mucosa dry and friable, and predisposed to bleeding, even with minor trauma as may occur with nose rubbing, blowing, or sneezing [7,8]. Increased atmospheric pollutant concentrations (in the form of airborne particulate matter and atmospheric ozone) also can irritate the nasal respiratory epithelium and has been associated with increased emergency department visits for spontaneous epistaxis [9].

ANATOMY AND PATHOPHYSIOLOGY — The nose is a highly vascular structure. This property enables it to filter, humidify, and warm inhaled air, but also predispose it to bleeding [6]. Kiesselbach plexus (also called Little's area) in the anterior nasal septum is one of the most vascular areas in the nose (figure 1). It is formed by the anastomosis of terminal vessels from the internal and external carotid arteries. Specifically, the septal branch of the anterior ethmoidal artery, the lateral nasal branch of the sphenopalatine artery, and the septal branch of the superior labial branch of the facial artery all end in the Kiesselbach plexus (figure 1). In addition, the branches of the sphenopalatine artery supply the posterolateral wall and posterior choana; these vessels are the most likely source of posterior nosebleeds.

The nasal mucosa provides little anatomic support or protection for the underlying blood vessels. Any factors that cause congestion of the nasal vessels, or drying or irritation of the nasal mucosa, increase the likelihood of bleeding.

ETIOLOGY

Classification — Nosebleeds in children have a variety of etiologies, ranging from self-limited mucosal irritation to life-threatening neoplasms (table 1). Common causes of nosebleeds in children include mucosal dryness, trauma, foreign body, and rhinitis (allergic, infectious, or related to mucosal irritation). Less common, but important causes of nosebleeds to remember, include bleeding disorders and other systemic diseases, tumors, and post-traumatic pseudoaneurysms of the internal carotid artery or carotid-cavernous sinus fistulae.

Several classification schemes have been used to categorize nosebleeds. Our preferred classification scheme considers whether bleeding arises from local or systemic conditions (table 1). Local causes of bleeding include trauma, mucosal irritation or drying, anatomic abnormalities, and nasopharyngeal masses.

Systemic causes of epistaxis include bleeding disorders (eg, inherited or acquired factor deficiencies, platelet disorders, or disorders of vessels), medications, neoplasms, inflammatory disorders, and hypertension.

Another commonly used approach to categorize bleeding is according to site: anterior versus posterior. Anterior nosebleeds, usually arising from the Kiesselbach plexus (figure 1), account for the vast majority of nosebleeds in children and are almost always self-limited [1,6]. Anterior nosebleeds usually result from mucosal dryness, trauma, or irritation, although many cases are idiopathic.

Posterior nose bleeds are unusual in children. When they occur, they are typically caused by significant nasal trauma. Posterior bleeds usually arise from the posterolateral branches of the sphenopalatine artery.

Local causes

Trauma

●Nose picking – In children with epistaxis, trauma to the septum due to nose picking is common but frequently unwitnessed. Parents/primary caregivers and patients should be routinely queried about the frequency of nose picking. (See "Evaluation of epistaxis in children".)

●Facial and head trauma – Blunt facial trauma leading to nasal fractures are due most commonly to auto accidents, sports injuries, intended injuries, and home injuries and may result in significant bleeding [10]. The anterior ethmoidal artery, which enters the nasal cavity through the fragile lamina papyracea of the ethmoid bone, can be lacerated by bone fragments in mid-face trauma.

Massive epistaxis after head injury is suggestive of internal carotid pseudoaneurysm [11], but this entity may present initially with mild epistaxis or with recurrent epistaxis that does not respond to conservative management and may occur weeks after the injury [11-17]. Pseudoaneurysm of the cervical internal carotid artery has also been reported in a child after a deep neck space infection [18]. Carotid-cavernous sinus fistulae can also result from blunt head trauma and can lead to intracranial hemorrhage, blindness, cranial nerve palsy, and stroke [19].

Child abuse may also be a cause of epistaxis, particularly in the child younger than two years of age. (See "Physical child abuse: Recognition", section on 'Oral or nasal injuries'.)

●Foreign body – When epistaxis is unilateral and accompanied by foul-smelling nasal drainage, a foreign body must be presumed present until proven otherwise. The foreign body can cause mucosal irritation, laceration, and/or ulceration. If the foreign body has been in the nose for only a short time (minutes to hours), its attempted removal by the patient or parent may be the cause of the nosebleed. If the foreign body has been in place for several days, the fetid nasal drainage will commonly be blood-stained. Nasal foreign bodies most frequently are found in the floor of the nose, just below the inferior turbinate. Common nasal foreign bodies include beads, rubber erasers, paper wads, pebbles, marbles, beans, peas, nuts, sponges, and chalk. Button batteries are a less common but more serious nasal foreign body which may present with epistaxis. These require immediate removal to prevent tissue necrosis, permanent septal perforation, or a saddle nose deformity. (See "Diagnosis and management of intranasal foreign bodies", section on 'Button batteries'.)

●Postoperative – Postoperative epistaxis may occur after adenoidectomy, sinus surgery, rhinoplasty, and/or surgery on the nasal septum or turbinates [20]. Epistaxis can occur up to two weeks post-operatively and in most cases will resolve spontaneously. Otolaryngology intervention is occasionally needed to control the bleeding. (See "Management of epistaxis in children".)

●Nasotracheal intubation and nasogastric tube placement – Nasotracheal intubation and nasogastric tube placement may injure the nasal mucosa and cause epistaxis.

●High-flow nasal cannula – High-flow nasal cannula used for children with respiratory distress can lead to epistaxis [21]. This may result from direct trauma from the cannula or mucosal desiccation.

●Barotrauma – Barotrauma, as occurs with scuba diving, may contribute to epistaxis in patients with upper respiratory infection or allergy. (See "Complications of SCUBA diving", section on 'Sinus barotrauma'.)

Mucosal irritation — Irritation of the nasal mucosa may be caused by a number of factors, including changes in humidity, viral or bacterial upper respiratory infections, chronic rhinitis with frequent nose blowing and increased vascularity and friability of the nasal mucosa, chronic usage of nasal sprays or drying agents, intentional or unintentional exposure to irritant inhalants or substances of abuse (eg, tobacco smoke, cocaine, heroin, volatile inhalants).

●Intranasal corticosteroids (ICS) – Allergic rhinitis, turbinate hypertrophy and/or adenoid hypertrophy are often treated with intranasal corticosteroids (ICS), which may cause epistaxis due to the steroid's direct drying effect on the nasal septum or local trauma from administration of the medication [22-24]. However, some experts do not regard ICS as a risk factor for epistaxis in children. In a meta-analysis of 72 trials evaluating the risk of epistaxis in adults and children receiving ICS compared with placebo, the risk of epistaxis was increased (RR [relative risk] 1.5, 95% CI 1.3-1.7) [24]. In a separate meta-analysis of 33 studies that evaluated adverse effects of ICS in children compared with placebo, the risk of epistaxis for ICS was not significantly different (RR 0.8, 95% CI 0.6-1.2), although interpretation of this study is limited by low numbers of events, small sample size, and potential for reporting bias [23]. In a subsequent small, randomized trial not included in these meta-analyses, of 60 children with untreated allergic rhinitis and first presentation of epistaxis, those receiving ICS with or without antihistamines had significantly lower severity and frequency of subsequent epistaxis and higher rates of resolution [25]. These findings cast further doubt on ICS as a cause of epistaxis in children.

●Infection – Viral and bacterial upper respiratory infections, including sinusitis and systemic illnesses that are accompanied by nasal congestion, result in inflammation of the nasal mucosa with increased likelihood of bleeding. Nasal colonization with Staphylococcus aureus appears to play a prominent role in leading to the mucosal irritation associated with childhood epistaxis [26]. In one study, 24 of 42 (57 percent) children with epistaxis had S. aureus cultured from their anterior nasal cavities, compared with 6 of 19 (24 percent) children with no history of nasal bleeding [27].

Local skin infections (eg, staphylococcal furuncles) also may cause epistaxis. Nasal tuberculosis is a rare, chronic, granulomatous infection that can cause nasal obstruction and epistaxis [28]. Epistaxis is one of the most common spontaneous bleeding features of dengue hemorrhagic fever in children, occurring in 25 percent of affected patients [29-31].

Tumors — Neoplasms of the nasal cavity usually cause unilateral symptoms such as intermittent epistaxis, foul discharge, nasal obstruction, or change in smell sensation. Severe bleeding is uncommon, except in patients with vascular tumors such as juvenile nasopharyngeal angiofibroma.

Benign localized neoplasms that can cause epistaxis in children include juvenile nasopharyngeal angiofibromas, hemangiomas, and pyogenic granulomas, and inverting papillomas. Malignant neoplasms of the nose, sinuses, or nasopharynx are rare but important causes of epistaxis in children. They include rhabdomyosarcoma, mesenchymal chondrosarcoma, non-Hodgkin lymphoma, and nasopharyngeal carcinoma [32-35].

●Juvenile nasopharyngeal angiofibroma – Juvenile nasopharyngeal angiofibroma is a histologically benign but locally aggressive tumor that is markedly vascular and can cause severe epistaxis (image 1) [36]. It occurs primarily in adolescent males with lesions that arise in the lateral nasopharynx and are hormonally sensitive [37]. The tumor’s blood supply is derived principally from the maxillary artery. Although juvenile nasopharyngeal angiofibroma is a benign tumor, it can cause severe problems through local invasion of adjacent structures.

The clinical features of juvenile nasopharyngeal angiofibroma were described in a review of 120 cases [38]. The mean patient age was 15 years (range 7 to 29 years). The triad of nasal obstruction, epistaxis, and nasal drainage was the most common combination of signs and symptoms; serous otitis media and diminished hearing also may be present [38,39]. The tumors may bulge into the nasal cavity but often require specialty examination and advanced imaging of the nasopharynx to be identified [38].

The diagnosis is usually confirmed by computed tomography (CT) or magnetic resonance imaging (MRI) with contrast that shows a vascular enhancing nasopharyngeal mass with involvement and widening of the pterygomaxillary fissure [38,40]. Intranasal biopsy of these lesions should be avoided because of the risk of life-threatening bleeding [41].

●Lobular capillary hemangioma (Pyogenic granuloma) – Lobular capillary hemangioma, also known as Pyogenic granuloma or granuloma telangiectaticum, is a benign tumor associated with capillary proliferation. Pyogenic granuloma often, but not always, occurs after a history of trauma [42,43]. (See "Pyogenic granuloma (lobular capillary hemangioma)".)

●Rhabdomyosarcoma – Rhabdomyosarcoma may present with episodic epistaxis. Parameningeal rhabdomyosarcoma can cause nasal, aural, or sinus obstruction with or without mucopurulent or bloody nasal discharge. Other signs and symptoms include middle ear effusion, deep facial pain, and cranial neuropathies, such as sixth nerve palsy [35]. (See "Rhabdomyosarcoma in childhood and adolescence: Clinical presentation, diagnostic evaluation, and staging".)

●Nasopharyngeal carcinoma – Nasopharyngeal carcinoma in children may present with epistaxis, rhinitis, headache, torticollis, trismus, unilateral cervical lymphadenopathy, retrobulbar or ear pain, hearing loss and/or neck pain [34]. (See "Epidemiology, etiology, and diagnosis of nasopharyngeal carcinoma".)

●Inverting papillomas – Inverting (or inverted) papillomas, which grow with an inversion of the epithelium into the connective tissue stroma, are rare in children. They manifest as unilateral red, polypoid masses attached to the lateral nasal wall, middle turbinate, the septum, or the nasal vestibule. Approximately two percent may undergo malignant transformation, which typically occurs in adulthood [44].

Other — Increased nasal venous pressure secondary to paroxysmal coughing as occurs in pertussis or cystic fibrosis occasionally may cause nosebleeds.

Systemic causes — Systemic causes of nosebleeds in children include bleeding diatheses, disorders of blood vessels, medications, neoplasms, inflammatory disorders, and hypertension. Epistaxis is rarely the only manifestation of systemic disease. Systemic disease should be considered in children with constitutional signs and symptoms, severe or recurrent nosebleeds, and a family history of bleeding disorder or other heritable systemic disease.

Bleeding disorders — Although an uncommon cause of epistaxis in children, bleeding disorders must be considered in children with recurrent, frequent, spontaneous epistaxis, and those with a prolonged, difficult-to-control nosebleed, especially in children with a family history of a bleeding disorder [45-47]. Platelet disorders and von Willebrand disease are the most frequently identified hematologic causes. Bleeding disorders encompass inherited and acquired disorders of coagulation, platelet disorders, and disorders of blood vessels. Initial laboratory evaluation for a bleeding disorder in a child with prolonged or frequent spontaneous epistaxis and interpretation of findings are discussed in detail separately. (See "Approach to the child with bleeding symptoms".)

Important hematologic etiologies of epistaxis include:

●von Willebrand disease (VWD), hemophilia, and other clotting factor disorders – Patients with VWD, hemophilia, and other clotting factor disorders are prone to epistaxis. For example, in one case series, >50 percent of over 100 children with von Willebrand disease had epistaxis [48]. (See "Clinical presentation and diagnosis of von Willebrand disease" and "Clinical manifestations and diagnosis of hemophilia".)

●Immune thrombocytopenia (ITP) and other platelet disorders – Epistaxis is one of the most common forms of mucosal bleeding in patients with ITP. Among patients who present to a pediatric hematologist for evaluation of recurrent epistaxis, platelet aggregation disorders have also been described [45]. (See "Immune thrombocytopenia (ITP) in children: Clinical features and diagnosis".)

●Malignancy – Pediatric oncologic processes such as leukemia can also present with epistaxis and are usually secondary to thrombocytopenia. Coagulopathy, although less common, may accompany acute promyelocytic leukemia, T cell acute lymphoblastic leukemia, lymphoma, and neuroblastoma. (See "Overview of common presenting signs and symptoms of childhood cancer".)

●Systemic lupus erythematosus – Children with systemic lupus erythematosus can develop lupus anticoagulant hypoprothrombinemia syndrome, which can present with bleeding manifestations such as epistaxis [49]. The coagulation profiles of these children show prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT) with low prothrombin levels and positive Lupus anticoagulant. Mean age of presentation is 10 years and the female to male ratio is 4:1.

●Hereditary hemorrhagic telangiectasia (HHT) – Epistaxis is the most common presenting symptom among patients with HHT, also known as Osler-Weber-Rendu disease. HHT is a rare autosomal dominant condition, characterized by widespread mucocutaneous telangiectasias (picture 1). Ninety percent of patients with HHT present with epistaxis by age 12 years; bleeding may be mild. Epistaxis in patients with HHT progressively worsens with age and can be difficult to control; the friable lesions appear to bleed more with treatment than without. A high index of suspicion is necessary to make the diagnosis, which should be considered on the basis of family and clinical history in patients with multiple mucocutaneous telangiectasias [6]. The approach to the diagnosis of HHT, including recommendations for genetic testing, is provided separately. (See "Clinical manifestations and diagnosis of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)", section on 'Clinical features'.)

Medications — Nosebleeds may be more frequent, or more difficult to control in children taking certain medications, particularly antiinflammatory agents (aspirin) and anticoagulants (eg, those with complex congenital heart disease or thromboembolic disease). In addition, anticoagulating agents (warfarins and "superwarfarins") are contained in a number of rodenticides, which may be unintentionally ingested by children [50].

Inflammatory disorders — Bloody nasal discharge may occur in various systemic inflammatory disorders, such as granulomatosis with polyangiitis (GPA) [51,52]. The most common presenting symptoms of GPA include persistent rhinorrhea, purulent/bloody nasal discharge, oral and/or nasal ulcers, polyarthralgias, myalgias, or pain. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis".)

Hypertension — Epistaxis is known to be associated with hypertension in adults. However, it is a less well-established cause of epistaxis in children. (See "Approach to the adult with epistaxis", section on 'Etiology'.)

Recurrent epistaxis — In children and adolescents, recurrent nosebleeds usually are related to chronic irritation of the nasal mucosa as may occur from dry air, nose picking, recurrent upper respiratory infection, or inhalation of medications or substances of abuse.

However, recurrent epistaxis also may be the presenting symptom of a bleeding disorder, hereditary hemorrhagic telangiectasia, nasopharyngeal tumor, or post-traumatic pseudoaneurysm of the internal carotid artery. These disorders must be considered in selected patients based on clinical findings (eg, positive family history, mucocutaneous telangiectasias, head trauma, or nasopharyngeal mass) and may warrant specific laboratory studies, imaging, and/or specialty consultation. (See "Evaluation of epistaxis in children".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Nosebleeds (The Basics)")

●Beyond the Basics topic (see "Patient education: Nosebleeds (epistaxis) (Beyond the Basics)")

PATIENT PERSPECTIVE TOPIC — Patient perspectives are provided for selected disorders to help clinicians better understand the patient experience and patient concerns. These narratives may offer insights into patient values and preferences not included in other UpToDate topics. (See "Patient perspective: von Willebrand disease".)

SUMMARY AND RECOMMENDATIONS

●Epidemiology – Nosebleeds are common in children. Approximately 30 percent of children younger than 5 years and over half of those aged 6 to 10 years have had at least one nosebleed. (See 'Epidemiology' above.)

Epistaxis is rare in children younger than 2 years and should prompt consideration of trauma (intentional or unintentional) or serious illness (eg, thrombocytopenia). Epistaxis in a young infant without a plausible explanation may be a marker for child abuse. (See "Physical child abuse: Recognition", section on 'Oral or nasal injuries'.)

●Pathophysiology – Any factors that cause congestion of the nasal vessels or drying or irritation of the nasal mucosa increase the likelihood of nosebleeds. Nosebleeds are more common during cold weather and when ambient humidity is low. (See 'Anatomy and pathophysiology' above.)

●Anatomy – Most nosebleeds in children originate from the anterior nasal septum, in an area known as Kiesselbach plexus, which is formed by the anastomosis of terminal vessels from the internal and external carotid arteries (figure 1). (See 'Anatomy and pathophysiology' above.)

●Etiology – Local, systemic, and recurrent causes of pediatric epistaxis are listed in the table (table 1). (See 'Local causes' above and 'Systemic causes' above and 'Recurrent epistaxis' above.)

Common causes of nosebleeds in children include trauma (nose picking), dry air, nasal foreign body, and rhinitis. Nasal colonization with Staphylococcus aureus is commonly found in children with recurrent epistaxis. Less common, but important, causes of nosebleeds include a button battery foreign body, bleeding disorders and other systemic diseases, tumors, and post-traumatic anomalies of the internal carotid artery. (See 'Classification' above.)