Neck Lumps in Children: An Imaging & Referral Guide

• Background
• Clinical Evaluation
• Differential Diagnosis: Common Neck ‘Masses’ in Children & Adolescents
  • Cervical Lymphadenopathy
  • Acute Bilateral Cervical Lymphadenopathy
  • Acute Unilateral Cervical Lymphadenitis
  • Subacute/Chronic Lymphadenitis
  • Parotitis
  • Sternocleidomastoid Pseudotumour
  • Thyroglossal Duct Cyst
  • Dermoid Cyst
  • Branchial Cleft Anomalies
  • Lymphoma and Neoplastic Neck Masses
  • Haemangioma
  • Cystic Hygroma (Lymphatic Malformation)
  • Cervical Ribs
• Summary: Indications for Ultrasound
• Referral Pathway Flowchart

Background

Neck masses are common in the paediatric population. In infants and young children, congenital lesions (i.e. lymphatic and vascular malformations) account for the majority of cases. In older children, infection (systemic or loco-regional) is by far the commonest cause, with neoplasms (usually lymphoma) being rare but increasing throughout adolescence. Developmental lesions (such as branchial arch cysts) may present at any age throughout childhood although most frequently encountered during the first decade of life.

Evaluation of the likely cause of a neck mass in children initially requires a careful history and clinical examination. The patient’s age is critical, particularly in the context of congenital lesions which usually present in infancy or childhood.1 Correlating age and clinical history with the examination findings will allow the differential diagnosis to be significantly narrowed, and in the majority of cases, the diagnosis can be reached with this alone. Imaging, serology and/or biopsy is therefore reserved for selected cases only.

There are no national radiological guidelines in the imaging of neck lumps and masses in children. The easy availability of ultrasound (US), the introduction of national targets and increasing time pressure on primary care and emergency departments (ED) has resulted in an exponential rise in the number of children being referred for neck US in Leeds. In 2016, an audit of our referral practice and outcomes for neck US in children demonstrated that over 90% of our referrals from General Practice (GP) and ED added no additional ‘value’ to clinical examination.

This evidence-based clinico-radiological guideline has been developed in conjunction with Paediatric ENT with the aim of providing clinical and imaging guidance and referral criteria for GPs and hospital clinicians treating children with neck lumps.

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Clinical Evaluation

History

Ask about the site of the lump, including its onset, growth, change in nature (including the timescale) and pain.

Specific features in the history that may suggest cause

• A combination of symptoms indicative of an upper respiratory tract infection — fever, cough, and sore throat.
• Recent travel, insect bites or exposure to pets/other animals — may suggest inflammatory / infectious cause.
• Skin problems such as eczema and acne are a common cause of reactive lymphadenopathy

Features which suggest:

• Airway compromise - Retropharyngeal or parapharyngeal abscesses may present with airway compromise +/- usually systemically unwell, drooling, trismus, neck stiffness, stertor.
• Malignancy - Head & neck tumours in infants and young children are rare and when seen, are usually large at diagnosis. Clinical symptoms depends upon site of the tumour. In older children/adolescence haematological malignancies, (Hodgkin lymphoma), account for the majority. They usually present with a palpable rubbery focal neck mass, increasing in size over weeks or months. These masses are typically painless and may be associated systemic symptoms such as; fatigue, night sweats, fever, weight loss, generalized itching or breathlessness.

Clinical Examination

• Site of lump
• Size and mobility i.e. whether fixed to underlying structures.
• Assess whether lump moves- ask patient to swallow
• Characteristics of lump, ie. Compressible, firm or pulsatile
• Assess skin of head, neck and scalp, ears, teeth, tonsils and pharynx. Infection or inflammation in these areas, particularly the throat & skin may cause reactive lymphadenopathy.

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Differential Diagnosis: Common Neck ‘Masses’ in Children & Adolescents

The site/position of the lump should help narrow down the likely cause. In infancy, a congenital cause is the most likely cause, whereas in children, reactive lymphadenopathy is the commonest cause. Malignancy is rare at all ages.

Lymphadenopathy and Lymphadenitis

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Cervical Lymphadenopathy

Lymphadenopathy is the commonest cause of a neck lump in children, with up to 90% of children between 4–8 years of age having cervical lymphadenopathy at some stage.2 Approximately 55% of children will have palpable lymph nodes even in the absence of an underlying systemic infection. In adults, an abnormal lymph node is commonly quoted to be > 1 cm in size. However, in the paediatric population, where lymphadenopathy is seen much more frequently, a lymph node should be considered potentially abnormal if > 1.5 cm in short axis. Most cases of reactive lympadenopathy are self-limiting and due to a viral or systemic cause. Reactive lymphadenopathy can take a long time to resolve, and therefore persistence of nodes is not necessarily worrying.

Further investigation with imaging and/or laboratory tests is NOT routinely indicated.2-5 ENT referral should be considered after a period of observation, particularly if lymph nodes are enlarging.

A simple message: ‘pea sized nodes are common and need no action, unless they ‘grow’ to malteser size or more!’ Mr Lindsey Knight. Consultant Paediatric ENT Surgeon

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Acute Bilateral Cervical Lymphadenopathy

Acute bilateral cervical lymphadenopathy (< 2 weeks duration) usually results from a viral upper respiratory tract infection or a bacterial streptococcal pharyngitis. These ‘reactive’ nodes are usually small, firm and non-tender. They may persist for weeks to months.

Imaging is not routinely recommended.

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Acute Unilateral Cervical Lymphadenitis

Acute unilateral cervical lymphadenitis is usually characterised by larger nodes (> 1cm in short axis) which are tender and often fluctuant. These lymph nodes most commonly occur within the anterior triangle of the neck and are frequently associated with fever, warmth and erythema of the overlying skin. In 40-80% of cases, acute cervical lymphadenitis is caused by Streptococcal or Staphylococcal infection with the site of entry being the mouth or scalp/skin.

Imaging is not routinely indicated. USS may be helpful if there are clinical signs to indicate abscess formation.

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Subacute/Chronic Lymphadenitis

Subacute (2-6 weeks in duration) or chronic lymphadenitis (> 6 weeks in duration) is most commonly caused by generalised or localised viral infections with EBV, CMV and influenza being most common. In infants and young children, generalised lymphadenopathy may occasionally be associated with hepatosplenomegaly. Rarely, widespread

lymphadenopathy may indicate an underlying haematological disorder such as leukaemia, a systemic disease process or autoimmune disease.

USS is not routinely indicated and in the vast majority of cases, adds little to clinical examination

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Parotitis

Mumps may cause a parotitis and is an acute, self-limiting systemic viral illness characterised by swelling of one or more of the salivary glands, most commonly the parotid. In addition to symptoms of malaise, fever and myalgia, patients typically present with tender swelling at the angle of the mandible. Recurrent parotid swelling can also occur in children as “juvenile recurrent parotitis”.

USS may suggest the diagnosis and aid clinical decision-making. These children should be referred to Paediatric ENT for clinical evaluation.

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Sternocleidomastoid Pseudotumour

Sternocleidomastoid pseudotumour usually presents within the first 7 - 28 days of life as a firm mass within the neck. The infant may present with torticollis, typically with the head tilted to the ipsilateral side and chin pointing away from the lesion. Torticollis may be associated with DDH.

Where there is clinical doubt, USS is highly sensitive and specific for the diagnosis.

Infants with a sternocleidomastoid pseudotumour, should be referred to Paediatric Physiotherapy and an USS of the hips should be performed to exclude DDH.

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Thyroglossal Duct Cyst

Thyroglossal duct cysts are the commonest cause of a midline neck lump in children. Half of all cases present within the first decade of life, with the remainder presenting in adulthood. Typically, thyroglossal duct cysts are mobile midline masses sited at or below the level of the hyoid bone. They may be lateral to the midline, particularly if inferior to the hyoid. Neck lumps above the hyoid bone are more likely to be a lymph node or dermoid cyst. Typically thyroglossal duct cysts increase in size over time and usually elevate with tongue protrusion. They often become symptomatic if infected and rarely, a sinus tract with the skin may subsequently develop.⁶

USS is highly sensitive and specific and is the imaging modality of choice. These children should be referred to paediatric ENT for clinical evaluation.

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Dermoid Cyst

Dermoid cysts are an unusual cause of neck lumps in children. They are conventionally located in the midline of the neck, typically in the suprasternal notch although they can occur anywhere in the midline and may even be located within the floor of the mouth.6 Dermoid cysts may also present lateral to the midline and may be confused with thyroglossal duct cysts, especially when close to the hyoid. However in contrast to thyroglossal ducts cysts, dermoid cysts do not elevate with tongue protrusion.

USS is the imaging modality of choice. These children should be referred to Paediatric ENT or Paediatric Surgery for clinical evaluation.

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Branchial Cleft Anomalies

Branchial cleft anomalies are the second most frequently encountered congenital neck lesion in children. The face, neck and pharynx are formed from branchial arches. Abnormalities in the development of one of the branchial arches can result in cyst, sinus or fistula formation with the location varying according to the branchial arch involved. Sinuses and fistulae usually present from birth but cysts may not present until adulthood. Sinuses present as a skin pit, usually weeping a small amount of matter or mucus. If a sinus track becomes infected it can develop a lump within the track. 50% of sinuses connect internally and are thus technically fistulae.

• First branchial cleft anomaly: These track parallel to the external auditory canal and middle ear, often begin as benign fistulous tracts in the post-auricular/pre-tragal area and are always superior to the hyoid bone.
• Second branchial cleft anomaly: These are usually located within the anterior triangle of the neck and are the commonest branchial anomaly (90%). If the anomaly is a branchial sinus or fistula, the external opening is most commonly found at the anterior border of the sternocleidomastoid muscle at the middle and inferior two-thirds junction.
• Third branchial cleft anomaly: These are rare (<2%) and may present similar to a second branchial cleft cyst although the internal opening located within the pyriform sinus.

USS is the imaging modality of choice to confirm the presence of a cyst and to exclude a solid lesion. USS is however of limited value if there is no palpable lump. If the USS is inconclusive or if a fistula is suspected, MRI or a CT fistulogram (CT following the injection of contrast medium into the sinus track) may be helpful.

These patients should be referred to Paediatric ENT.

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Lymphoma and Neoplastic Neck Masses

Head and neck tumours in children and adolescents are rare. In infants and young children, rhabdomyosarcoma and neuroblastoma are the commonest tumour types and are typically large at diagnosis. Symptoms are often few, although will depend upon the site of origin. In older children and adolescents, haematological malignancies are the commonest cause of a neoplastic neck mass with Hodgkin’s lymphoma being the most common cause in children over 12 years.

Primary malignant tumours of the thyroid and salivary glands and soft tissue sarcomas are rare, but when they occur they usually occur in adolescence.

USS may aid clinical diagnosis and direct further management.

USS is the imaging modality of first choice to assess thyroid nodules. Thyroid nodules and masses should be referred to Paediatric ENT.

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Haemangioma

Capillary haemangiomas are the most common developmental ‘tumour’ of infancy and typically present within the first few months of life. They typically demonstrate rapid growth followed by a period of quiescence, prior to involuting. 50% of children will have complete resolution of the lesion by 5 years of age, increasing to 70% by 7 years of age. Cavernous haemangiomas are usually larger, associated with deeper soft tissue involvement and less likely to resolve spontaneously.

USS is highly sensitive and specific and is the imaging modality of choice to confirm the diagnosis, characterise the lesion and aid in medical and surgical planning.

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Cystic Hygroma (Lymphatic Malformation)

Cystic hygromas are the commonest congenital lymphatic malformation, usually presenting by 3 years of age as a large, soft and painless mass. These lesions are most commonly sited within the posterior triangle of the neck where there is little mass effect. Occasionally they may be found in the anterior triangle of the neck and result in airway obstruction.

USS is highly sensitive and specific and is imaging modality of first choice. MRI (or CT where this is unavailable) may be required characterise and evaluate extent.

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Cervical Ribs

Cervical ribs are accessory ribs arising from the seventh cervical vertebrae and may be unilateral or bilateral. Although usually asymptomatic, they may present with thoracic outlet syndrome due to pressure on the brachial plexus nerves or vessels. Rarely, it may present as a spurious ‘mass’ within the supraclavicular fossa which is clinically hard and immobile.

A plain radiograph of the cervical spine and thoracic inlet is the imaging modality of first choice.

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Summary: Indications for Ultrasound

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Referral Pathway Flowchart