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Chapter16: Abnormalities of the Neck and Face 107

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Table 16.1 Swellings in the neck

Developmental anomalies

Branchial cleft: sinus, fistula or cyst Branchial arch: cartilage Thyroglossal cyst Ectopic thyroid Lymphatic malformation Epidermal cyst

Acquired lesions

Inflammation of cervical lymphatics:

Acute lymphadenitis Atypical mycobacterial infection Acute lymph node abscess

Lymph node tumours:

Primary neoplasia

Secondary Submandibular gland: calculus Parotid gland: sialectasis Thyroid gland: goitre

contains cholesterol crystals. The cyst may become infected and should be excised.

Branchial arch remnants usually arise from the second branchial arch and present as a skin tag (containing cartilage) at the anterior border of the lower third of the sternomastoid. They are excised for cosmetic reasons.

Table 16.2 Midline neck swellings

Submental lymphadenitis Thyroglossal cyst Ectopic thyroid Epidermal cyst Goitre

Figure 16.1 Thyroglossal cyst that has become infected.

middle third of the hyoid bone to minimise the risk of recurrence (Sistrunk operation).

Thyroglossal cyst

The embryological descent of the thyroid anlage from the floor of the mouth leaves a track from the foramen caecum of the tongue to the thyroid isthmus. A cyst (lined by respiratory epithelium) may arise anywhere along the track, but is usually close to and adherent to the hyoid bone (75%), and is one of the common causes of midline neck lump [Table 16.2]. Recognising the attachment of this midline swelling to the underlying hyoid bone is the key to both clinical diagnosis and surgical excision. Typically, there is a tense rounded cyst in the midline or just to one side, which moves on swallowing as well as with protrusion of the tongue. The cyst may also be submental (15%), suprasternal (8%) or lingual (2%) in position. Infection may supervene [Fig.16.1] and an infected thyroglossal cyst may be mistaken for acute bacterial lymphadenitis in the submental lymph nodes. The thyroglossal cyst and the entire thyroglossal track should be excised, preferably before infection occurs. The resection must include the

Ectopic thyroid

Ectopic thyroid is now a rare cause of midline neck swelling, as it presents as low thyroid function on neonatal screening. The swelling tends to be softer than that of a thyroglossal cyst but the diagnosis may not be apparent until at operation, when the lesion is found to be solid and vascular. If this lesion is suspected preoperatively, a thyroid isotope scan should be performed to determine the distribution of all functioning thyroid, because the ectopic thyroid may be the only functioning thyroid tissue present. In this situation, it is not excised: the mass is divided in the midline and rotated on its vascular pedicle laterally to lie behind the strap muscles. Other thyroid swellings in children are rare. Neonatal goitre may result from excessive maternal iodine ingestion. Thyrotoxicosis is rare in young children. Adenoma, papillary carcinoma and medullary thyroid carcinoma areseen occasionally in older children, the latter most often in association with a multiple endocrine neoplasia (MEN) syndrome.

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Lymphatic malformations

Lymphatic malformations in the region of the head and neck are relatively common. In the past, these malformations were termed cystic hygromas, but this nomenclature is now discouraged as the suffix oma wrongly implies these lesions to be tumours. Lymphatic malformations are present at birth but may not become noticeable until infancy. They are more common in boys than girls [Fig.16.2]. Lymphatic malformations in the head and neck region are of broad two types: (1) a simple or multicystic lesion compressing adjacent structures or (2) a complex lesion infiltrating other structures including the mouth, pharynx, larynx or mediastinum. This second type resembles lymphatic malformations found elsewhere in the body and may contain cavernous haemangiomatous elements.

Simple lymphatic malformations are more common and are usually found as unilateral fluctuant, transilluminable swellings in the anterior triangle. The cysts are of varying sizes and contain clear fluid (lymph). They may enlarge suddenly and rapidly, due to viral or bacterial infection or haemorrhage. The effect of this will depend on the site and size of the cysts. A clinical emergency may arise if the increased swelling compromises the airway. In the absence of these complications, injection of sclerosant is undertaken (and uncommonly an operation) for cosmetic reasons and the prognosis isgood.

Complex lymphatic malformations are less common, and complications arise because of extensive soft tissue

involvement. These malformations may involve the oropharynx (leading to difficulty with speech and swallowing) or the larynx and trachea (leading to a life-threatening respiratory obstruction). Involvement of the mediastinum and pleural cavity likewise may lead to respiratory embarrassment. They may present on the first day of life and emergency care may necessitate insertion of an endotracheal tube and sometimes a tracheostomy. The baby should be referred for assessment by a multidisciplinary team (e.g. at a Vascular Anomalies Clinic). Imaging, including MRI and ultrasound, is used to delineate the anatomical extent and injection of sclerosants or operative excision may be undertaken relatively early.

Epidermoid cysts

Inclusion dermoids arise from entrapment of ectodermal cells within the mesodermal layer during fetal growth. They are often in the midline or along lines of fusion, for example, at the external angle of the eye, orin the midline of the neck at which location an epidermoid cyst may be mistaken for a thyroglossal cyst. They contain sebaceous cheesy material surrounded by squamous epithelium. They enlarge slowly and should be removed. The most common inclusion dermoid is the external angular dermoid at the orbital margin (see in the succeeding text).

Less common varieties include the sublingual dermoid in the floor of the mouth between the mylohyoid and genioglossus muscles. It may interfere with speech and swallowing and is usually excised through a submental incision. It may be confused with a ranula or mucocele of the floor of the mouth, a lesion that contains mucus.

A rare developmental anomaly found in this region is the midline cervical cleft, a vertical open groove that results from failure of fusion of the branchial arches. Operative repair should be undertaken.

Figure 16.2 Lymphatic malformation in a baby with Down

syndrome.

Periorbital cellulitis

Infection in the soft tissues and sinuses around the eye may cause periorbital cellulitis with rapid extension across the face [Fig.16.3]. The danger with this infection is that it may spread to the cavernous sinus, which

Figure 16.3 Periorbital cellulitis: sinusitis is a common source

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of infection.

Chapter16: Abnormalities of the Neck and Face 109

Reactive hyperplasia

Persistently enlarged lymph nodes are seen in many children with frequent upper respiratory tract infections. These nodes are not painful and are a normal response to infection. Occasionally, a markedly enlarged hyperplastic node (>3 cm) may require an excision biopsy to exclude tumour or other diagnoses.

Acute lymphadenitis

Acutely tender enlarged lymph glands are commonly seen during upper respiratory tract infections. Lymphadenitis usually settles with rest, analgesia and – if bacterial infection is suspected – intravenous antibiotics.

Acute lymph node abscess

Lymphadenitis may progress to an abscess, particularly in children aged 6 months to 3 years. The swelling enlarges over 3 or 4 days and may become fluctuant. An abscess in deeper nodes may not exhibit fluctuance, mainly because it is beneath the investing deep cervical fascia. The overlying skin eventually becomes red and, if untreated, the abscess will finally point and discharge. The management of an abscess is incision and drainage under general anaesthesia, taking care not to damage the mandibular branch of the facial nerve when submandibular abscesses are incised.

is potentially lethal. Children with periorbital cellulitis should be admitted to the hospital for treatment with intravenous antibiotics and ophthalmological assessment (see Chapter13) and warrant an opinion from an ear, nose and throat surgeon regarding drainage of pus from the sinuses abutting the orbital cavity (see Chapter14).

Diseases of the lymph nodes

Infection is the most common cause of lymph node enlargement in childhood. It may be caused by bacteria, viruses or non-tuberculous mycobacteria. In many cases, the lymph nodes are reacting to an upper respiratory tract or ear infection leading to non-specific reactive hyperplasia. Lymph nodes also may become enlarged in primary or secondary malignancy. A surgical biopsy is indicated when the diagnosis is in doubt or if persistently enlarged lymph nodes (>3 cm) are present for longer than 4–6 weeks.

Non-tuberculous (atypical) mycobacterialadenitis

Mycobacterium avium-intracellulare, Mycobacterium scrofulaceum, Mycobacterium fortuitum and Mycobacterium

cause chronic cervical lymphadenitis and collar-stud abscesses in children. Although human TB and bovine TB strains have been nearly eradicated in most Western countries, non-tuberculous mycobacterial lymphadenitis is still a problem in preschool children. Often termed atypical, such mycobacteria are found in the soil, and infection is from the child’s dirty hand to the mouth and then to a tonsillar or parotid lymph node. Initially, the node is enlarged and firm but non-tender. Over 4–6 weeks, the node erupts to produce a collar-stud abscess in the subcutaneous tissue, thus causing the overlying skin to become a characteristic blue–purple colour [Fig.16.4]. Untreated, the collar-stud cold abscess will ulcerate through the skin with multiple chronic discharging sinuses. Nontuberculous mycobacteria respond poorly to antibiotics and require operative excision to remove the infected lymph nodes.

chelonae

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Figure 16.4 Atypical mycobacterial cold abscess.

The mandibular branch of the facial nerve may be at risk during excision of an affected jugulodigastric lymph node. The diagnosis of atypical lymphadenitis is confirmed by histological examination of the lymph node and culture of the pus and lymph node tissue.

Lymph node tumours

Primary neoplasia

Hodgkin’s and non-Hodgkin’s lymphomas may occur in cervical lymph nodes in older children. They tend to grow rapidly, and several contiguous nodes may feel spherical and rubbery. There may be associated malaise, night sweats and weight loss.

Secondary neoplasia

Nasopharyngeal and thyroid tumours and neuroblastoma may present with cervical node enlargement. In most cases, the marked enlargement and rocky hardness of the lymph nodes make the diagnosis of neoplasia

obvious, but sometimes, the differential diagnosis between a large hyperplastic lymph node and a neoplastic node is difficult and necessitates an excisional biopsy.

The submandibular gland

The most common cause of enlargement is a small calculus in the submandibular duct, which produces rapid and painful swelling during eating. The gland becomes hard and tender and fluctuates in size. The submucous part of the duct in the floor of the mouth should be inspected for a tiny calculus impacted near the orifice under the tongue. An x-ray of the floor of the mouth may show an opaque calculus, which may be removed by simple incision of the duct.

The parotid gland

Recurrent enlargement of the parotid gland is due to recurrent parotitis associated with sialectasis, a condition analogous to bronchiectasis, which affects the lesser ducts and their tributaries. Parotid calculi are extremely rare.

Symptoms of sialectasis usually commence at 2–4 years of age, and the first attack may be misdiagnosed as mumps, although both sides are seldom swollen at the same time. The gland becomes enlarged and mildly tender, and the attacks may alternate from side to side. Fever and malaise are mild or absent.

Purulent saliva may issue from the orifice when the duct is compressed, and Streptococcus viridans or other weakly pathogenic organisms may be found on culture.

The diagnosis is clinical. However, if a sialogram is performed, it will show a snowstorm of sacculations

mm in diameter along the radicles of the gland

2–4 [Fig. 16.5] but no duct obstruction. The changes are often present in both glands, even when the symptoms are confined to one side.

The condition is self-limiting and treated by massage of the parotid, tart drinks to promote the flow of saliva and chewing gum. The attacks typically last for 3–4 days, but the symptoms may persist intermittently for several years. Most children improve by about 10 years of age, and sialograms during adolescence often show that the sialectasis has disappeared. Parotidectomy is not necessary.

Figure 16.5 Sialogram showing sialectasis. A contrast x-ray of

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the parotid duct showing a snowstorm of saccular dilatations of the lesser ducts in the enlarged parotid.

Torticollis

The common causes of torticollis in infants and children are, in order of frequency:

1 Fibrosis in the sternomastoid muscle (often following

an injury at birth)

2 Postural torticollis (a legacy of the neck position in

utero)

3 Cervical hemivertebrae 4 Imbalance of the ocular muscles (producing a

squint) A posterior fossa tumour presenting with torticollis is a rare cause, although usually there would be obvious CNS signs and symptoms.

Postural torticollis is present from birth and disappears in a few months. Likewise, the associated plagiocephaly (Chapters 12 and 15) and scoliosis do not require treatment, for they are caused by intrauterine moulding.

Cervical hemivertebrae produce a mild angulation of the head and neck. The cause is readily seen in x-rays,

Chapter16: Abnormalities of the Neck and Face 111

which should be taken in all cases of torticollis where the sternomastoid muscle is not tight. No treatment is necessary, for the degree of torticollis is mild and the course is not progressive.

Ocular torticollis is not detectable until the age of 6 months and is usually not noticed until the child is at least 1 or 2 years old. Strabismus is the cause, but it is not always obvious and may be latent or intermittent. An ocular imbalance is the most likely cause of torticollis in a child without hemivertebrae, with normal sternomastoid muscles and a full normal range of passive rotation (i.e. the chin may be made to touch each acromion). Treatment is the correction of the imbalance by adjusting the attachment of the eye muscles to the globe.

Sternomastoid fibrosis

Sternomastoid muscle pathology may present in two groups of patients:

1 Neonates 2–3 weeks old present with a localised

swelling in one sternomastoid muscle, that is, a sternomastoid tumour [Fig. 16.6]. It is often associated with plagiocephaly because the relatively malleable skull bones become distorted as a consequence of the head being consistently turned to the one side.

2 Older children present with torticollis and a tight,

short fibrous sternomastoid muscle. Rotation of the head towards the affected side is limited, growth of the face on the side of the affected muscle is reduced (hemihypoplasia of the face [Fig.16.7]) and the ipsi-

lateral trapezius muscle may be wasted. The aetiology is unknown, but birth injury is suspected.On histology, there is endomysial fibrosis around individual muscle fibres, which undergo atrophy.

Clinical features

In the infant, the tumour is so characteristic that it is diagnostic, a hard, painless spindle-shaped swelling 2–3 cm long within the sternomastoid muscle.

Due to shortening of the affected muscle, the infant’s head adopts a characteristic position: rotated to the opposite side and often also angulated to the same side of the tumour. Plagiocephaly (Chapters 12 and 15) becomes evident during the first 3 months as a result of this preferred position and may be limited by putting the infant down to sleep on each side in turn.

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Treatment

Infants with a sternomastoid tumour initially should be managed non-operatively, because in 90% it will heal completely and without complications in 3–6 months.

In the remaining cases, the resultant fibrosis causes permanent muscle shortening and persistent torticollis. Surgical division of the sternomastoid muscle is indicated if torticollis persists beyond 12 months of age or if there is progressive hemihypoplasia of the face. Following surgery, symmetry of the face improves over several years, but may never recover completely.

Figure 16.6 Sternomastoid tumour in an infant.

Developmental anomalies of the face

External angular dermoid

As introduced earlier, this is a common anomaly of fusion between the frontonasal and maxillary processes during formation of the head and face (Chapter12). The cyst is noticed in infancy as it enlarges gradually [Fig.16.8]. Often, it is beneath the pericranium, givingit a firmer consistency than may be expected. Occasionally, it is misdiagnosed as a bony lump. Excision through a lateral supra-eyebrow incision is curative.

Many varieties of facial clefts have been described and classified, but most of them are rare. Cleft lip and

Figure 16.7 Sternomastoid torticollis. A tight (scarred) right

sternomastoid muscle is apparent along with secondary hypoplasia of the right side of the face.

Hemihypoplasia of the face [Fig.16.7] describes the decreased growth of one side that may occur as a nonspecific result of any type of long-standing immobilisation and is not directly attributable to fibrosis in the sternomastoid.

Figure 16.8 External angular dermoids, just above and lateral

to the right eyebrow, and the left under the lateral edge of the left eyebrow..

palate, by contrast, are very common. Clefts are

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described in Chapter15.

Microstomia

Malformations of the structures derived from the first pharyngeal arch may cause microstomia, a misshapen ear, absence of the external auditory canal, a rudimentary middle ear, hypoplasia of the mandible and its teeth, hypoplasia of the malar–maxillary complex and, sometimes, facial paralysis. Complex defects of the facialskeleton, such as this, are managed by craniofacial surgery (see Chapter15).

Deformities of the ear

Accessory auricles

Small tags of skin and cartilage may be present, usually close to the tragus, but sometimes along a line extending to the angle of the mouth. They are excised for cosmetic reasons.

Chapter16: Abnormalities of the Neck and Face 113

Figure 16.9 Microtia, associated with a maldevelopment of the

dorsal ends of the first and second branchial arches. The external auditory canal is a shallow pit.

Pre-auricular sinus

This is a common condition (1/50 of Asian and 1/200 of Caucasian children) and is often bilateral and asymptomatic. There is a tiny hole just in front of the upper crus of the helix, from which an epithelial track extends deeply forwards and downwards. The track is often short, but sometimes extends deeply towards the pharynx.

Where there are no symptoms, or only an occasional bead of watery discharge, it is best left alone. If it becomes infected with purulent discharge and the opening becomes sealed, an abscess may develop. In such cases, the abscess should be incised and drained and the sinus then excised once the infection has settled.

Microtia

A rudimentary ear of irregular skin and cartilage is associated with absence of the external auditory canal, a rudimentary middle ear and a small mandible on the same side [Fig.16.9]. When the site of the ear is acceptable, it may be used as the basis for reconstruction, which is preferable to a prosthetic ear. Only when the condition is bilateral is it necessary to create an external auditory canal and provide a hearing aid within the first few months of life to enable the infant to hear and develop speech. Further operations are required in later years.

Figure 16.10 Bat ears.

Bat ears

Bat ears, both unilateral and bilateral, are common and often familial [Fig.16.10]. The concavity of the concha extends to the rim that stands out farther than normal. The ear is often bigger than normal, as well as more protuberant.

Corrective surgery is advisable when there is gross protrusion, particularly when there are adverse comments from other children. Strapping in the neonatal period achieves nothing, and removal of skin from the postauricular groove is inadequate. The fold of the antihelix must be fashioned, shaping and fixing the cartilages in a new relationship and holding them in position for the approximately 3 weeks required for union of the cartilages. Operative correction may be done any time after infancy.

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Shell ears

Shell ears are similar to bat ears in protruding from the scalp, but they are small, and the rim of the helix is so short that the ear cannot be readily folded back into the normal position. Operative correction is much more difficult than in bat ears and typically performed as a staged procedure.

KEY POINTS

• Thyroglossal cyst typically presents as a midline neck lump uctuating in size and that may become inamed.

• Lymphatic malformations (previously known as cystic hygromas) are a congenital anomaly of the jugular lymph sacs that frequently become infected. Early referral to the regional, multidisciplinary Vascular Anomalies Clinic is recommended.

• Enlarged lymph nodes need excisional biopsy if greater

cm in diameter and present for more than 4–6

than 3 weeks.

• Sternomastoid brosis with torticollis may need operative division to prevent facial asymmetry.

• External angular dermoids require excision.