Neck Masses in Children Who Are Acutely Unwell
25.2.1 Clinical Assessment
In a child who presents acutely with pain, fever, malaise, and a neck mass, the most likely cause is infection, but the clinician should be aware of a few noninfective inflammatory conditions that require specific treatment.
We should also never forget that some malignancies (particularly lymphoma and neuroblastoma) and histiocytoses can present acutely with a neck mass and with symptoms such as weight loss and malaise, mimicking an infection.
For the child presenting as an emergency with a neck mass and fever, it is worth making an initial assessment of the child’s general state. Deep neck-space infections may cause airway compression, while fever, septicemia, and poor oral intake may all lead to hypovolemia. These may need immediate attention regardless of the exact underlying diagnosis. The same is true for pain, and analgesia may allow for a more complete physical examination.
Once the situation is stabilized, focus on the duration of the illness, any preceding upper respiratory symptoms, symptoms associated with the mass itself, and systemic symptoms such as night sweats and weight loss. Any history of foreign travel or known conditions such as tuberculosis (TB) or human immunodeficiency virus (HIV) in the family is clearly important. The clinician should ask specifically if there was any preexisting lump or sinus opening to suggest an underlying branchial cleft or thyroglossal duct anomaly. Recurring neck abscesses around the left thyroid lobe may suggest a third/fourth pharyngeal pouch anomaly (see ▶ 24).
Evaluate the child’s general health including temperature, state of hydration, and demeanor.
Note specific features of the mass such as size, site, consistency, fluctuance, tenderness, and discoloration of the overlying skin.
Examine the ears, nose, and oral cavity and consider awake transnasal fiberoptic laryngoscopy depending on the circumstances. Trismus, torticollis, palatal bulging, retropharyngeal swelling, and stertor are all worrying signs of deep neck-space infection. General examination may reveal diagnostic clues such as heart murmurs and swelling of the hands and feet in Kawasaki’s disease; exudative tonsillitis and splenomegaly in Epstein–Barr virus (EBV) infection; and abdominal masses or lymphadenopathy in the axillae and groins in children with malignancy.
For the acutely unwell child with a neck mass, the most important initial investigation is ultrasound.
A full blood count and routine blood chemistry can give useful information, but ultrasound is the single test most likely to guide initial treatment. It will distinguish solid lesions from cysts and abscesses, as well as providing anatomical information. Cross-sectional imaging (such as computed tomography [CT]) is useful when deep neck-space infection is suspected.
25.2.2 Acute Lymphadenitis
Acute Viral Lymphadenitis
Acute viral lymphadenitis is extremely common in children and, it can be considered normal for a child with a viral upper respiratory tract infection to have palpable cervical lymph nodes for 3 or 4 weeks. A normal preschool child may get as many as eight upper respiratory tract infections a year, so fluctuating enlargement and regression of cervical lymph nodes is a normal physiological phenomenon as the lymphocytes in the lymph nodes do their job of processing and responding to foreign antigens. No specific investigation or management is required other than reassurance. More persistent lymphadenopathy can be associated with EBV or cytomegalovirus (CMV) infection and with the illness that accompanies the acquisition of HIV infection.
Acute Bacterial Lymphadenitis
Acute bacterial lymphadenitis most often occurs after an upper respiratory tract infection. Streptococcus pneumoniae and Staphylococcus aureus are common pathogens.
In the initial stages, the child is feverish and unwell with a mass of swollen lymph nodes.
These continue to enlarge and undergo central necrosis until a superficial lymph node abscess forms.
At this stage, the lymph node mass will be large, fluctuant, red, hot, and tender.
Treatment is with systemic antibiotics, but if a fluctuant abscess has developed, incision and drainage under general anesthesia may be needed. Needle aspiration may be useful in selected cases. The child’s general condition then improves quickly with fluid rehydration, intravenous if needed, analgesia, antipyretics, and continued antibiotics. This is a very common clinical situation with which every otolaryngologist is familiar.
Familiarity breeds complacency and there are several pitfalls in the management of acute cervical lymphadenopathy:
First among these is the preschool age child presenting with a fluctuant subcutaneous abscess of a few weeks duration with reddish-purple skin discoloration and no fever or malaise. At first glance this looks like a typical bacterial abscess but it is in fact nontuberculous mycobacterial (NTM), sometimes called “atypical mycobacterial” (ATM), infection for which incision and drainage would be a poor choice of treatment (see section on Nontuberculous Mycobacterial Infection).
The second pitfall is the child with a serious deep neck-space infection who is getting steadily sicker on the ward. This is often misdiagnosed as a superficial lymph node abscess. Thorough examination, including the oropharynx, with ultrasound scanning as needed should avoid this.
The third is the occasional failure of radiologists and otolaryngologists to communicate effectively: the radiologist may report a small amount of early central necrosis in a lymph node as “liquefaction,” which the otolaryngologist might interpret as meaning a collection large enough to justify drainage, only to find no pus at surgery. If surgery is being considered, it should go without saying that there is no substitute for a direct discussion with the radiologist about exactly what they have found and where.
25.2.3 Deep Neck-Space Infection
Peritonsillar Abscess
In older children and adolescents, management can usually proceed as for adults, with transoral needle drainage of the collection under local anesthetic, administration of antibiotics, and the expectation of rapid resolution. Diagnosis and treatment in younger children can be much more difficult as it may be almost impossible to see the oropharynx in an uncooperative, unhappy toddler with trismus.
Peritonsillar abscess (quinsy) occurs less often in children than in adults.
Look carefully for airway compromise.
Investigation
Ultrasound scanning can sometimes give surprisingly good views of the tonsil capsule to make the diagnosis in a cooperative child, but in many cases, the diagnosis is presumptive. If there is any doubt, a CT scan will exclude any more serious deep neck-space infection, but the child will usually need a general anesthetic with endotracheal intubation to achieve good quality images and to ensure that the airway is secure throughout the procedure. This is no small undertaking as endotracheal intubation may be very difficult in the presence of trismus and oropharyngeal swelling.
Treatment
Assuming the child is stable, the best initial course of action is often simply to treat the child with intravenous rehydration, analgesia, antipyretics, and broad-spectrum antibiotics and then reassess after 12 to 24 hours. 1 Consider metronidazole, especially if there is not a rapid response as anaerobes are commonly implicated. The clinical condition usually improves quickly and physical examination becomes easier allowing the definitive diagnosis to be made. Many peritonsillar abscesses will settle in a few days with conservative treatment.
If the child is not stable or not improving rapidly with conservative management, then general anesthesia and a scan become inevitable. At this point, it is worth making a plan in advance to intervene under the same anesthetic once the diagnosis of peritonsillar abscess is confirmed. Needle aspiration under general anesthesia is often inadequate because of the high reaccumulation rate; it is much better to do something definitive at this stage to avoid the risk of having to repeat the general anesthetic. There is much variation in management strategies in different countries and health care systems, with immediate surgery the routine in Denmark. 2, 3
“Hot” tonsillectomy drains the abscess and removes the focus of infection in one simple procedure.
Retropharyngeal Abscess
A retropharyngeal abscess most commonly occurs in children under the age of 5 years due to suppuration in lymph nodes secondary to upper respiratory tract infection.
Retropharyngeal abscess in an infant will often present in a similar manner to acute epiglottitis with a sick, feverish baby drooling saliva and breathing with a soft stertor. Such cases should be managed in the same way as epiglottitis until the diagnosis is established:
Disturb the child as little as possible.
Quickly assemble a team of experienced clinicians including an anesthetist and an otolaryngologist.
Gently induce anesthesia with an inhalational agent to allow examination of the pharynx and larynx.
Intubation can proceed at this point, along with transoral drainage of the abscess through a vertical incision in the posterior pharyngeal wall if the diagnosis is apparent.
A sick baby with potential airway obstruction should never be sent to the radiology department for a lateral soft-tissue neck X-ray; to do so is potentially dangerous and a plain X-ray is too prone to artifact to be of much use.
If a diagnosis of retropharyngeal abscess is being seriously considered, then the airway should be secured by endotracheal intubation and definitive imaging (a CT scan of the neck) arranged ( ▶ Fig. 25.1).
Fig. 25.1 Computed tomography of retropharyngeal abscess.
Parapharyngeal Abscess
Children may develop a parapharyngeal abscess secondary to lymph node suppuration, tonsillitis, or dental infection. The clinical presentation is very variable. 4
Some present with a rapidly progressive, fulminating illness and may even require admission to the intensive care unit.
Others run a more insidious course with subtle signs such as neck stiffness, torticollis, low-grade pyrexia, and malaise.
The external swelling in the neck may be difficult to discern in the early stages, and oropharyngeal swelling may also be difficult to see in the early stage. The tonsil may be deviated if the parapharyngeal collection is large.
Ultrasound is an excellent first-line investigation, as it involves no ionizing radiation and the child does not need general anesthesia. Some radiologists may even attempt needle drainage of the abscess under the guidance of the ultrasound probe. Cross-sectional imaging such as CT gives much more useful anatomical information on the position of the abscess and its relationship to the carotid sheath and the pharynx ( ▶ Fig. 25.2)—information which is very useful for surgical planning. It allows estimation of the volume of pus present, which may support conservative management if the volume is small.
In most cases of parapharyngeal abscess, the treatment is formal incision and drainage under general anesthesia.
An external incision allows wide drainage and the placement of a drain to reduce the chance of reaccumulation, but in some cases, a transoral approach is quicker and easier. The tonsil is often the original site of suppuration, and once it has been removed, the abscess can be identified through the tonsil bed using a needle, followed by blunt dissection with forceps. This approach is best when the abscess has displaced the carotid sheath laterally. Preoperative imaging will identify the relationship of the abscess to the carotid sheath and aids surgical planning. An external scar is avoided with a transoral approach, but the chance of reaccumulation of pus with the need for a second procedure is higher.
It is not uncommon to find that there is no pus at surgery despite the CT appearance of a collection. Early necrosis can be difficult to differentiate from liquid pus. magnetic resonance imaging (MRI) scanning tends to have even more false-positives for pus than CT. Ultimately, if there is doubt, then proceed with surgery.
Fig. 25.2 Axial computed tomography scan with contrast of a child showing a large right parapharyngeal abscess displacing the tonsil medially and the carotid sheath laterally.
25.2.4 Noninfective Inflammatory Conditions
There are a few nonsuppurative inflammatory causes of acute cervical lymph node swelling with fever of which the clinician should be aware. Some are serious in their own right, but some are important in that early diagnosis prevents the need for unnecessary further investigation and treatment. These diagnoses should be considered particularly for children whose fever does not respond rapidly to antipyretics and antibiotics.
Kawasaki’s Disease
Kawasaki’s disease (mucocutaneous lymph node syndrome) is an autoimmune vasculitis affecting children under the age of 5 years. It presents with fever, nontender cervical lymph node enlargement, edema of the hands and feet, and erythema of the conjunctiva, oral mucosa, and skin. Arthritis, aseptic meningitis, and myocarditis may also occur in the early stages. Skin desquamation on the hands and feet occurs later.
The most important feature is the potential for coronary artery aneurysms and the small but significant risk of death within the first few weeks after onset without treatment.
The fever is usually high and unresponsive to paracetamol, ibuprofen, or antibiotics. It lasts for a week or two in most cases, and the longer the duration of fever, the higher the risk of cardiac sequelae. Early diagnosis is essential but this can be difficult in practice as there is no single definitive test.
The diagnosis is based on clinical features, blood tests, and echocardiography. A full blood count will show a degree of anemia and thrombocytosis, and the erythrocyte sedimentation rate and C-reactive protein (CRP) will be elevated. Coronary artery aneurysms on echocardiogram are highly diagnostic in the right clinical setting. Temporal artery biopsy is rarely required. Diagnostic criteria 5 are set out in Box 25.1. 5
Box 25.1 Diagnostic Criteria for Kawasaki’s disease 5
Fever of at least 5 days’ duration with any four of the following five criteria:
Erythema or cracking of the lips or oral mucosa.
Rash on the trunk.
Swelling or erythema of the hands or feet.
Conjunctival injection.
Enlarged cervical lymph node (15 mm or more).
Or, fever of at least 5 days’ duration, coronary artery disease on echocardiography, and any three of the aforementioned criteria, when the features cannot be explained by any other known disease process.
The child needs to be admitted under the care of a pediatrician. Treatment should be prompt. This is one of the very few situations when aspirin is recommended for children. The fever responds rapidly to aspirin and intravenous immunoglobulin. The role of systemic steroids is uncertain.
Kikuchi–Fujimoto’s Disease
Kikuchi–Fujimoto’s disease (KFD or necrotizing lymphadenitis) is rare outside Japan but isolated cases have been reported in other parts of Asia, in Europe, and in North America. It usually occurs in people in their twenties, but cases have been reported in very young children.
The cause of the disease is unknown but it may be an excessive T-cell reaction to a nonspecific infectious trigger in a genetically susceptible individual.
Presentation is with tender cervical lymphadenopathy in association with headache, skin rash, weight loss, and fever. Ultrasound scanning of the neck shows enlarged coalescent nodes, with distortion of the normal hilar architecture. This is nonspecific but an important sign as a normal hilar architecture pattern virtually excludes significant lymph node disease. Blood tests are unhelpful.
KFD is a benign, self-limiting condition but may be misdiagnosed as more serious pathology such as TB ( ▶ Fig. 25.3, ▶ Fig. 25.4) or malignancy. 6 Diagnosis is by means of lymph node excision biopsy and histology. The histological pattern is characteristic and shows coagulative necrosis in the paracortical areas of the involved lymph nodes.
The disease usually resolves spontaneously over a period of a few weeks or months. Treatment is supportive.
Fig. 25.3 Tuberculous lymph node in the neck of an adolescent girl.
Fig. 25.4 Ultrasound showing “collar-stud” abscess typical of cervical tuberculosis.
Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome
Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is of unknown etiology. It typically occurs in young children and is characterized by episodes of high fever with mouth ulcers, pharyngitis, and tender enlargement of cervical lymph nodes occurring every 3 to 5 weeks for at least 6 months. Throat cultures show no growth, but tonsillar fauna are now thought to play a role in some cases. 7 Blood tests may be useful to exclude cyclical neutropenia. In between episodes, the child is perfectly well.
Episodes often resolve quickly with steroids, but if the condition is disabling, then surgery may be considered. A review of 159 children in 13 observational studies and 2 randomized controlled trials suggested that a significant majority of children have complete resolution or improvement after adenotonsillectomy. 8 However, the condition usually regresses anyway as the child gets older, with one study showing 50 out of 59 children having resolution after a mean of 6.3 years, 9 suggesting that milder cases can be treated conservatively.
25.3 Neck Masses in Children Who Are Systemically Well
Cervical lymphadenopathy is one of the commoner presentations in pediatric otolaryngology practice. Reactive lymphoid hyperplasia is, of course, a physiological response to an infective or inflammatory stimulus. Children suffer from frequent upper respiratory infections, mostly viral in origin, so it should not be surprising that palpable lymph nodes are present in 62% of healthy children aged 3 weeks to 6 months, 52% of those aged 7 to 23 months, and up to 41% of those aged 2 to 5 years. 10
Persistent enlargement of cervical lymph nodes raises the concern that there may be serious underlying pathology (such as Hodgkin’s disease), but the majority of children with persistently enlarged nodes have reactive hyperplasia and nothing else.
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