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CHAPTER 60
Torticollis
Spencer W. Beasley
There are many causes of torticollis in childhood (Table 60-1),
but most are rare. The most common cause of torticollis is
tightness and shortening of one sternomastoid muscle, a con-
dition that occurs in about 0.4% of all births. Typically, at
about 3 weeks of age, a visible or palpable swelling develops
in part or all of the muscle; this swelling is called a sternomas-
toid tumor. It affects the right side in about 60%,
1
is bilateral in
2%to 8%,
2,3
and often persists for up to 1 year. Older children
may present with a fibrotic, shortened sternomastoid muscle,
which is presumed in many to be the legacy of a previously
unrecognized sternomastoid tumor.
History
Alexander the Great may have had torticollis, according to
Plutarch.
4
Antyllus is said to have performed tenotomies in
350 AD, but the first authenticated division of the sternoclei-
domastoid was by Minnus in Amsterdam in 1641.
5
A sterno-
cleidomastoid tumor was described by Heusinger in 1826.
6
Torticollis was also a subject of interest to Dupuytren.
7
Etiology
Although often referred to as congenital torticollis, the ster-
nomastoid mass and the torticollis are rarely noticeable at
birth. Little is known about the etiology of sternomastoid
fibrosis, although several theories have been put forward to
explain the condition. It may be due to an idiopathic intrauter-
ine embryopathy
8
or could be the manifestation of an intra-
uterine positional disorder producing sternocleidomastoid
compartment syndrome.
9
The high incidence of obstetric diffi-
culties, suchas breechpresentationand the needfor assistedde-
livery,
10,11
may be the result rather than the cause of the
shortened sternomastoid muscle, as was initially thought. There
is no report of a sternomastoid tumor detected by antenatal
ultrasonography.
12
Concomitant hip dysplasia is common.
10
Pathology
The basic abnormality on histology is fibrous replacement of
muscle bundles.
13
The lesion, called fibromatosis colli, is of-
ten classified with other types of fibromatoses, such as the
Dupuytren contracture and plantar fibromatosis. Jones
8
has
described endomysial fibrosis involving the deposition of col-
lagen and fibroblasts around individual muscle fibers that un-
dergo atrophy. The sarcoplasmic nuclei are compacted to form
giant cells that appear to be multinucleated. The maturity of
the fibrous tissue in neonates suggests that the disease may be-
gin before birth
8,14,15
and may therefore contribute to the fre-
quency of obstetric difficulties during delivery. The reported
incidence of breech deliveries is about 20% to 30%
16
much
higher than the normal incidence. About 60% of affected in-
fants are involved in a complicated birth,
16
which suggests
that the fibrosis may affect the position of the fetus in utero
and perhaps even prevent normal engagement of the head
in the maternal pelvis.
The natural history of untreated sternomastoid fibrosis is
complete resolution in 50% to 70% of patients at 6 months
of age. In about 10%, the tumor and sternomastoid shortening
persist beyond 12 months of age.
2,17
The severity and distri-
bution of the fibrosis within the sternomastoid muscle is var-
iable and has led to a variety of classifications based either on a
palpable localized sternomastoid tumor or thickening and
shortening of the whole muscle or on the basis of ultrasono-
graphic findings.
18,19
The systems of classification have some
prognostic significance in that localized lesions within the
sternomastoid (clinically or ultrasonographically) are more
likely to resolve spontaneously than those involving the whole
muscle. In older children with torticollis, the appearance of
degenerating fibers is more consistent with disuse atrophy
produced by limitation of movement caused by the fibrosis.
Clinical Features
STERNOMASTOID TORTICOLLIS
The tumor is a hard, spindle-shaped, painless, discrete swell-
ing usually about 1 to 3 cmin diameter within the substance of
one sternomastoid muscle. Almost always, it first becomes ev-
ident at about 3 weeks after birth. Obvious head tilt or torti-
collis tends to develop later.
12
In infants, the head is rotated to
the side opposite the tumor, with only slight flexion of the
head to the affected side (Fig. 60-1).
In other patients, the sternomastoid tumor is less discrete,
and the sternomastoid appears to be thickened and tight-
ened along its whole length. The shortening of the muscle
restricts rotation and lateral flexion of the head (Fig. 60-2).
763
The rotational component of the action of the sternomas-
toid is easy to measure. It is assessed by standing behind
the childs head and passively rotating the head while it
is held between both hands. The sternomastoid muscle is
stretched to its maximum length by rotation to the side
of the affected muscle. Where the muscle is fibrotic, it can-
not be stretched to its full length, and rotation to the ipsi-
lateral side is restricted.
Older childrenwithtorticollis compensate for the more pro-
nounced tilt by elevating one shoulder to enable the eyes to
keep as level as possible (Fig. 60-3). Such compensation is
not seen in infants, because there is no need for them to main-
taintheir eyes ina horizontal plane until they standup.
20
More-
over, older children do not turn their heads to the contralateral
side as much, because they tend to compensate by twisting the
neck and back to keep their eyes pointing forward.
DIFFERENTIAL DIAGNOSIS
Initial clinical assessment must establish whether the wry neck
is caused by shortness of one sternomastoid muscle or by
some other condition. In sternomastoid fibrosis, the anterior
border of the muscle stands out as a tight band, although in
some small infants in whom the neck is relatively short, the
muscle may be difficult to see readily. For this reason, the full
length of the muscle must be palpated to determine whether
there is an area of thickening or fibrosis along part or all of its
length. In about two thirds, there is a definite localized swell-
ing (tumor) in the muscle; in the remainder, the whole muscle
appears to be affected. There is no role for plain radiography
where the sternomastoid is tight or shortened.
21
Although not
required for diagnosis, appearance on ultrasonography may
help predict (to a degree) the likelihood of spontaneous reso-
lution.
1,19,22
Inexperienced ultrasonographers, worried by the
infiltrative and ill-defined appearance, may recommend
TABLE 60-1
Causes of Torticollis in Infants and Children
Cause Comment
Sternomastoid tumor Common; appears at 3 weeks of age
Abnormal position in utero Tends to improve with age
Cervical hemivertebrae Structural; confirmed on plain radiograph
Cervical lymphadenitis/abscess Acute; usually occurs in first 2 years of life
Retropharyngeal abscess
31,58
and pyogenic cervical spondylitis
59
Acute; signs of toxicity, cervical pain
Posterior fossa tumors
61
A rare cause; headaches, vomiting, and other neurologic signs present
61
Acute atlantoaxial subluxation May occur after tonsillectomy
30
Atlantoaxial rotatory subluxation Significance disputed
58,59
; diagnosed on dynamic CT
Spasmodic with Sandifer syndrome
59
Due to gastroesophageal reflux
Congenital absence of sternomastoid Unilateral, extremely rare
60
Postural Familial
CT, computed tomography.
Neutral position Limited range Normal range
FIGURE 60-2 Restriction of rotation of the head secondary to shortening of the sternomastoid muscle as viewed from above the head. The black bars
represent the right sternomastoid muscle and show that its inability to lengthen limits rotation to that side.
FIGURE 60-1 Appearance of a right sternomastoid tumor in infancy; the
head is turned to the contralateral side.
764 PART V HEAD AND NECK
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further imaging or biopsy of the tumor; this is not indicated
in the presence of the typical palpable mass within the sterno-
mastoid and torticollis. It is possible to diagnose a sternomas-
toid torticollis on magnetic resonance imaging (MRI)
22,23
and
computed tomography (CT),
24
but neither alters management
and should not be performed routinely.
23,25
An obvious mass or fibrosis of the muscle may not always
be noticeable in idiopathic torticollis, but in such instances,
alternative diagnoses must be sought (see Table 60-1 and
Fig. 60-4).
20,26
A squint may cause head tilt from imbalance in rotation of
the eyes. The squint may not be obvious at first because the tilt
compensates for the abnormal position of the eyes. When the
head is straightened passively, the squint becomes apparent.
Occasionally, sternomastoid fibrosis may occur coincidentally
with ocular torticollis.
Posterior fossa tumors may compress the brainstem at the
foramen magnum and produce acute stiffness of the neck that
causes it to be held to one side. The neck is frozen in this po-
sition and is difficult to move actively or passively. The pres-
ence of a central nervous systemtumor may be known already,
but occasionally, acute torticollis is the first manifestation.
Careful neurologic examination may show abnormalities of
the lower cranial nerves and cerebellar function, and the caus-
ative lesion is demonstrated on CT or MRI.
Hemivertebrae involving the cervical spine may produce a
tilt of the head that is evident from birth and does not pro-
gress. Vertebral lesions can be identified clinically by inspec-
tion and palpation of the dorsal cervical spines and confirmed
on plain radiographs of the neck.
Acute torticollis has been attributed to atlantoaxial rotatory
subluxation as determined on dynamic CT,
27,28
but others
doubt the existence or significance of these findings and sug-
gest that CTscans are not necessary at the initial examination.
29
Atlantoaxial subluxation has been reported after tonsillec-
tomy.
30
Acute torticollis can also result from inflammatory
conditions of the neck, including retropharyngeal abscess,
31
and can be a symptom of acute lymphoblastic leukemia.
32
Secondary Effects of Torticollis
Table 60-2 lists the secondary effects of torticollis.
FIGURE 60-3 Appearance of torticollis as a result of sternomastoid fibro-
sis in an older child. The eyes are kept horizontal, but the shortened ster-
nomastoid muscle causes compensatory elevation of the shoulder. (From
Beasley SW, et al: Pediatric Diagnosis. London, Chapman & Hall, 1994, with
permission.)
FIGURE 60-4 Torticollis caused by atlanto-occipital subluxation after
tonsillectomy. Notice that there is no tightness of the sternomastoid mus-
cle on either side. (From Beasley SW, et al: Pediatric Diagnosis. Chapman &
Hall, London, 1994, with permission.)
TABLE 60-2
Secondary Effects of Torticollis
Infants Plagiocephaly
Hemifacial hypoplasia
Older children Compensatory scoliosis
765 CHAPTER 60 TORTICOLLIS
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PLAGIOCEPHALY
In small infants with torticollis and fixed rotation of the head,
gravity deforms the relatively soft head as it lies in the same po-
sition for a prolonged period. Flattening of one occiput leads to
secondary flattening of the contralateral forehead (Fig. 60-5).
This asymmetric skull deformity is called plagiocephaly and de-
velops in the first few months of life.
33
It is best observed from
above the head. Once the child begins to sit up or the torticollis
resolves, the plagiocephaly tends to resolve as well.
34
It may take
several years to disappear, and a few children have a slight
permanent deformity. It is possible that many children with pla-
giocephaly have had unrecognized torticollis during infancy.
35
HEMIFACIAL HYPOPLASIA
Progressive facial deformity is seen when one sternomastoid
muscle immobilizes the face for a long time. The malar eminence
on the side of the face limited by the fibrotic muscle grows more
slowly than the normal side does
9
and causes progressive asym-
metry (Fig. 60-6). This inhibitionof growth of the mandible and
maxilla embodies an important principle of pediatrics: Normal
growth of bones depends on normal muscular movement.
The degree of hypoplasia of one side of the face can be deter-
mined by the angle between the plane of the eyes and the plane
of the mouth. Normally these lines are parallel, but they form
an angle to each other when the face is asymmetric. The devel-
opment of hemifacial hypoplasia is one indication for surgery;
division of the tight sternomastoid muscle allows resolution
of the skeletal abnormality and subsequent normal growth.
9
Significant hemifacial hypoplasia takes about 8 months to
develop
8
but is more often recognized at about 3 to 4 years of
age.
17
It becomes less obvious with ongoing growth once the
torticollis has resolved.
POSTURAL COMPENSATION
When children are old enough to walk, the eyes are kept hor-
izontal to facilitate balance and horizontal eye movement. The
child compensates for the short fibrous sternomastoid by el-
evating the ipsilateral shoulder (see Fig. 60-3). In addition,
there may be compensatory cervical and thoracic scoliosis.
Adjacent muscles, such as the trapezius, may be wasted
because of relative inactivity.
8
Conservative Management
Sternomastoid fibrosis resolves spontaneously in the vast ma-
jority of infants. Therefore surgery is required only rarely, in
those in whom the torticollis has not resolved. The value of
manipulation of the head and neck has not been proven,
12
al-
though it is widely used and may have some benefit in the first
year of life.
36
Physiotherapy and regular neck exercises appear
to be safe
36
and may make the parents feel that something is
being done for their infant. Unintentional snapping during
manipulation has been reported with no apparent deleterious
effect on outcome.
37
Some clinicians advocate early institu-
tion of intensive passive neck range-of-motion stretching
exercises and have reported high rates of resolution,
9,3840
while others believe that there is no convincing evidence that
these measures alter the natural history of the condition.
Others consider it important to encourage parents to place
toys and other desirable objects onthe ipsilateral side to encour-
age the infant to turn toward the affected side.
8
Again, this
Gravity
Tight
muscle
Head
held
rotated
Half
occiput
and face
flattened
Plagiocephaly
FIGURE 60-5 Plagiocephaly.
FIGURE 60-6 Hemifacial hypoplasia on the right side.
766 PART V HEAD AND NECK
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strategy probably helps the parent more than the infant, but is
unlikely to do any harm. Attempts to put the infant to sleep with
the headfacingtowardtheaffectedsidetendtofail, particularlyif
the muscle is tight. Botulinum toxin injection appears to be in-
effective inpatients presenting inlate childhoodor adulthood,
41
but results are more encouraging in younger children.
42
In most cases, reassurance is all that is required. The passage
of time is probably as effective as the various manipulations
when torticollis is due to sternomastoid fibrosis.
Operative Treatment
INDICATIONS FOR SURGERY
Indications for surgery include
1. Persistent sternomastoid tightness limiting head rotation in
children more than 12 to 15 months of age
43
2. Persistent sternomastoid tightness with progressive hemi-
facial hypoplasia
3. Diagnosis in children older than 1 year
44
OPERATIVE TECHNIQUE
The procedure is performed under general anesthesia with la-
ryngeal or endotracheal intubation, according to the expertise
and preference of the pediatric anesthetist. The child is placed
supine with the shoulders elevated and the neck rotated to
the contralateral side. The muscle is best divided at its lower
end,
4,9
although division at its upper end,
45
at both ends,
4648
or in its midportion
8,49
have all been described. Endoscopic
tenotomy of the muscle is also feasible
5053
including through
a transaxillary approach.
54,55
A3- to 4-cmtransverse incision is made ina skin crease about
1 cm above the sternal and clavicular heads of the affected ster-
nomastoid (Fig. 60-7). The platysma is divided with diathermy
sothat no bleeding occurs inthe line of the incision. The external
jugular vein can be retracted if it is within the field of view.
The tight fibrosis of the two heads of sternocleidomastoid are di-
vided with diathermy near their lower end. Tightness of the
cervical fascia betweenthe sternomastoidandtrapezius is usually
palpable once the sternomastoid has been divided, and this
fasciaalsoshouldbedivided(Fig. 60-8). This is doneunder direct
vision to avoid damage to other structures, particularly the
spinal accessory nerve and brachial plexus.
The wound is infiltrated with bupivacaine or other local
anesthetic agent. The platysma is closed with continuous
4-0 absorbable suture and the skin with subcuticular 5-0
Monocryl absorbable suture. No drains are required.
The procedure can be performed as a day case, and no post-
operative restriction of movement is necessary. Full range of
the neck is normally achieved within 1 week of surgery. Phys-
iotherapy is usually unnecessary, although some advocate an
extended period of physiotherapy postoperatively.
46
In older
children, restoration of a full range of movement may take
longer, and the final cosmetic appearance is less certain.
56
COMPLICATIONS
A hematoma may develop if hemostasis was inadequate at the
time of surgery. Diathermy dissection keeps blood loss to a
minimum. Larger superficial veins may require ligation and
division if they cannot be retracted.
Incomplete division of both heads of the sternocleidomas-
toid muscle or failure to divide the cervical fascia over the pos-
terior triangle of the neck may produce persistent torticollis.
Careful inspection and palpation of the neck for residual tight-
ness and bands at the time of surgery should prevent this com-
plication from occurring. Recurrent torticollis is rare after
surgical treatment and is seen in less than 3% of patients.
57
FOLLOW-UP
Patients should be monitored until (1) the torticollis has re-
solved completely, (2) there is full range of movement of the
head and neck, and (3) the sternomastoid muscle feels nor-
mal. In an older child with secondary scoliosis, follow-up, in-
cluding radiologic studies, if required, should continue until
the scoliosis has resolved.
The complete reference list is available online at www.
expertconsult.com.
Sternomastoid
Accessory
nerve
Trapezius
Brachial plexus
(immediately deep to fascia)
FIGURE 60-8 Division of the sternomastoid and investing cervical fascia
to the anterior border of the trapezius.
Clavicle
Sternomastoid
FIGURE 60-7 Skin incision for low division of the sternomastoid muscle.
767 CHAPTER 60 TORTICOLLIS