and Spencer W. Beasley2
(1)
Department of Urology, Royal Children’s Hospital, Melbourne, Australia
(2)
Paediatric Surgery Department Otago, University Christchurch Hospital, Christchurch, New Zealand
Abstract
The chapter opens with a description of torticollis and sternomastoid tumour. There is then a description of anomalies in the side of the neck. The final section is on midline neck lumps, including thyroglossal cyst.
The surgical lesions affecting the neck are numerous and common, with enlarged lymph nodes being responsible for most examinations. To enable discussion of the various presentations of neck abnormalities, the chapter has been divided into postural deformities (i.e. wry-neck) and lesions laterally or in the midline of the neck.
Torticollis (Wry-Neck)
The common cause of torticollis or wry-neck is tightness of one sternomastoid muscle following an idiopathic inflammatory process in the perinatal period which may represent a muscle injury during delivery. At 2–3 weeks of age, a visible or palpable swelling develops in the sternomastoid muscle (the so-called sternomastoid tumour), which may persist for some months. Older children may present with a fibrotic, short sternomastoid which is presumed to be the legacy of previous muscle damage.
The appearance of head tilt varies with age. Small infants have their heads turned to one side and a little tilted, but make no correction for this. Since they do not need to stand up, there is no need for them to maintain the plane of their eyes horizontally. Children able to walk compensate for the more pronounced tilt by elevating one shoulder to keep the head and eyes as level as possible. Furthermore, they do not turn their heads to the contralateral side but instead compensate by twisting the neck to keep the eyes pointing forwards. The first question in torticollis is to establish whether the wry-neck is caused by shortness of one sternomastoid muscle or not (Table 9.1). It is important to realize that alternative diagnoses must be sought if the sternomastoid muscle is not short and tight (Fig. 9.1).
Table 9.1
Causes of wry-neck
1. Sternomastoid ‘tumour’ or subsequent fibrosis |
2. Ocular imbalance |
3. Cervical hemivertebrae |
4. Posterior fossa tumours |
5. Deep cervical infection |
6. Atlanto-occipital subluxation |
Fig. 9.1
The clinical questions in the differential diagnosis of torticollis
Differential Diagnosis
Is the Sternomastoid Tight?
The anterior border of the muscle stands out as a tight band, except in small infants where the neck may be so short that the muscle cannot be seen readily. It is important, therefore, to palpate the full length of the muscle to define whether an inflammatory ‘tumour’ or area of fibrosis is present. Where the sternomastoid is abnormal, further abnormalities of growth tend to occur (see below). Where the torticollis is not caused by an abnormality of the sternomastoid, the muscle is neither prominent nor shortened and tight.
Is a Squint Present?
A squint is a common cause of head tilt which results from an imbalance in the rotation of the eyes. The tilt compensates for the abnormal position of the eyes, such that the squint may not be obvious at first. By straightening the head passively, the squint will become apparent and can be confirmed by checking eye movements.
Is There a Brain Tumour?
Posterior fossa tumours, which are not uncommon in children, may compress the brain stem at the foramen magnum to produce acute stiffness of the neck and cause the head to be held to one side. The neck is ‘frozen’ in this position and is difficult to move actively or passively. The presence of a central nervous system tumour may be known already, but occasionally, acute torticollis is the first presentation. A careful neurological examination should be performed with emphasis on the lower cranial nerves and cerebellar function.
Is There a Vertebral Anomaly?
Structural lesions in the cervical vertebrae will produce a tilt of the head which may be confused with that of other causes. Congenital anomalies such as hemivertebrae will produce torticollis from birth without progression. Vertebral lesions can be identified clinically by inspection and palpation of the dorsal cervical spines and confirmed on x-ray.
Sternomastoid Torticollis
A sound clinical understanding of this abnormality depends on knowledge of how the sternomastoid muscle works (Fig. 9.2). It has a complicated action which combines lateral flexion with rotation towards the opposite shoulder. The mastoid is not only above the sternum but also significantly posterior to it. The rotation towards the contralateral side on contraction of the sternomastoid is important to appreciate because it is the primary effect of shortening in infancy (Fig. 9.3). Ipsilateral head flexion occurs as well, but is less noticeable in a tiny infant bundled up in a rug!
Fig. 9.2
Sternomastoid muscle action causes tilting and rotation of the head
Fig. 9.3
Infantile sternomastoid ‘tumour’. (a) The head is turned away from the fusiform cervical swelling. (b) The inflammation may affect (i) the clavicular (or sternal) head, (ii) the entire muscle or (iii) one part of the main muscle
The sternomastoid tumour may affect all or part of the muscle, including its two inferior heads (Fig. 9.3). The resulting fibrosis likewise can affect a localized part of the muscle. Remember that not all ‘tumours’ lead to significant fibrosis and shortening later in life: The majority resolve with healing after a few months with no sequelae. Furthermore, there are some older children who present with fibrosis without a history of a ‘tumour’ in infancy.
The Mechanisms of Secondary Deformity
The Effect of Gravity
In small infants with torticollis and fixed rotation of the head, gravity deforms the head as it lies on the bed because it remains in the same position for a prolonged period. The baby’s head is turned towards the contralateral side with the contralateral occiput pressing on the bed. Flattening of the occiput leads to secondary flattening of the ipsilateral forehead (Fig. 9.4). This asymmetrical skull deformity is called plagiocephaly and is best observed from above the head.
Fig. 9.4
The cause of plagiocephaly
Plagiocephaly can become marked if the child is not nursed prone with the head turned towards the affected side. The skull bones are very soft and pliable in the small baby and readily deform, but once the torticollis resolves, the plagiocephaly tends to resolve as well.
The Effect of Growth
Progressive deformity with growth is seen in older children when one tight sternomastoid muscle immobilizes the face for a long time. The side of the face that is limited by the fibrotic muscle grows more slowly than the normal side and causes progressive asymmetry (Fig. 9.5). This inhibition of the growth of the mandible and maxilla embodies an important principle of paediatrics: normal growth of the bones depends on normal muscular movement.
Fig. 9.5
Estimating the degree of hemihypoplasia of the face by comparing the plane of the eyes with the plane of the mouth, which should be parallel. When the shoulders are level, the head is tilted
The degree of hemihypoplasia of the face can be determined by the angle between the plane of the eyes and the plane of the mouth. Normally, these lines are parallel but form an angle to each other when the face is asymmetrical. The larger the angle between these lines, the more severe is the asymmetry. Hemihypoplasia is a crucial sign to observe because it indicates that surgery is necessary to release the tight muscle.
The Effect of Postural Compensation
When children are old enough to walk, the eyes are kept horizontal to facilitate balance and coping with gravity. If a short, fibrous sternomastoid muscle causes tilting and rotation of the head, the child will compensate for this by elevating the ipsilateral shoulder to keep the plane of vision horizontal (Fig. 9.6). Examination of the child will show the eyes to be level and (possibly) hemihypoplasia of the face. The ipsilateral shoulder is lifted up to relieve tension on the sternomastoid. There is cervical and thoracic scoliosis to compensate further for this. Other adjacent muscles, such as the trapezius, may be wasted because of inactivity.
Fig. 9.6
Torticollis compensated for by cervical scoliosis and a high shoulder to keep the eyes level with the horizon
How to Assess Limitation of Movement
The rotational component of the action of the sternomastoid is easy to measure. Rotation is assessed by standing behind the child’s head and passively rotating the head while it is held between both hands. The aim is to rotate the head until the nose reaches each shoulder (180° total range) (Fig. 9.7). Note that the sternomastoid is stretched to its maximum length by lateral rotation to the side of the affected muscle. Where the muscle is fibrotic, it cannot be stretched to its full length and rotation to the ipsilateral side is restricted. The angle reached at the limit of movement is judged easily from above and can be recorded for future reference.
Fig. 9.7
Determination of the degree of limitation of head rotation. (a) Position of the hands to rotate the head passively. (b) Normal versus limited rotation
The Side of the Neck
Lumps in the side of the neck are caused most commonly by inflammation in lymph nodes. The mouth permits entry of pyogenic bacteria, viruses or rarer organisms (such as atypical mycobacteria), which colonize the surrounding lymphatic tissue. In addition, the sheer volume of lymphatic tissue in the neck means that often malignancies affecting lymph nodes will be found here first. Leukaemia, lymphoma and secondary neuroblastoma may present in this way (Table 9.2). The embryological development of the neck is complicated by the large number of structures within it, including the branchial clefts and arches – the distant homologues of the gill slits. Various branchial cysts and sinuses may occur, although most are rare. Also, hamartomatous overgrowth of some tissues, for example, jugular lymphatic spaces or blood vessels, may produce various vascular anomalies, such as a ‘cystic hygroma’ or haemangioma.
Table 9.2
Lateral neck swellings
1. Acute lymphadenitis |
Short history (days) |
Signs of inflammation (unless exposed to antibiotics) |
2. Atypical tuberculous (MAIS) lymphadenitis |
1–2-month history in a 1–2-year-old |
Very-low-grade inflammatory signs (= cold abscess) |
Purple discoloration of the skin (when near the surface) |
Collar-stud abscess |
3. Malignant nodes |
Child <5 years – leukaemia, neuroblastoma |
Child >5 years – lymphoma, leukaemia |
Non-tender, rubbery/stony |
+ History/signs of disease elsewhere |
4. Branchial cyst (2nd or 3rd clefts) |
Single cyst beneath the middle third of the sternomastoid |
Transilluminable (clear mucus) |
Branchial sinus opening at the anterior border of the lower part of the sternomastoid |
5. Vascular malformation |
Sign of emptying (cavernous spaces) |
+ Overlying skin vessels/colour |
+ Bruit |
(+ Excess flow – cardiac failure – enlarged liver) |
(+ Platelet trapping – Haemorrhage) |
6. Lymphatic malformation (= ‘cystic hygroma’) |
Brilliantly transilluminable |
Enlarges rapidly with infection or haemorrhage |
Single cyst/diffuse multilocular |
Soft, flabby, non-tender (except if secondary infection)
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