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Introduction
Sternomastoid tumour (SMT) of infancy is usually associated with congenital muscular torticollis (CMT); these will be discussed together in
this chapter. The term tumour is a misnomer because it is a congenital
fibrotic process; it also is referred to as congenital fibromatosis coli.
The terms torticollis and wryneck actually describe the tilting and
rotation of the head and neck that results from the contracture of the
sternocleidomastoid muscle. The torticollis, when untreated, results in
plagiocephaly, hemifacial hypoplasia, and body distortion. The key to
preventing deformity is early diagnosis and passive stretching exercises
(PSEs) of the affected muscle, with only 5% of cases needing surgical
intervention in a large prospective series.1
to poor neck control in infants before this age (Figure 38.2). Some of
the cases are first noticed by grandmothers while doing the traditional
body massage for the babies.
Physical Examination
Demography
Aetiology
Figure 38.1: Sternomastoid tumour on the right side, with the childs head
turned away from the affected side.
Clinical Presentation
History
Infants present with either a lump in the neck, or with a head tilt that
is not correctable by repositioning. SMT is usually absent at birth and
presents between 3 weeks to 3 months of age. There is a high incidence
of breech presentation or assisted delivery. It is important to reassure
the parents that the obstetric difficulty is thought to be the result rather
than cause of the shortened sternomastoid muscle.4
Many parents present their children after 3 months of age in the
African setting because they presume the abnormal position to be due
Figure 38.2: Note the posture of the older baby with left sternomastoid tumour.
Figure 38.3: Normal rotation of the neck to the left past the shoulder.
Figure 38.4: Limitation of passive neck rotation towards the right (affected) side.
Differential Diagnoses
The clinical features of SMT when associated with CMT are pathognomonic and should not be confused with other lateral neck masses, such as
cystic hygroma, branchial cyst, or hemangioma. Enlarged cervical nodes
are rare in infancy, as are neoplasms.
Congenital torticollis may present without an SMT, but most will still
have some palpable thickening and shortening of the muscle. If there is
a head tilt without any limitation of rotation of the neck, then causes of
postural torticollis should be considered. These would include congenital
hemivertebra, Klippel-Feil syndrome (atlanto-axial fusion), strabismus,
and Sandifer syndrome caused by chronic gastro-oesophageal reflux (see
Table 38.1). Familial and hereditary sternomastoid muscle aplasia have
also been reported.8,9
Figure 38.5: Measurement of passive range of neck motion from the midline.
Investigations
Management
Most cases of SMT and CMT are managed nonoperatively. Babies are
encouraged to actively look towards the affected side. In the University
of Ilorin Teaching Hospital, mothers are encouraged to be turning the
face of the child in the ipsilateral direction while backing their babies
in the traditional way (Figure 38.10). This position also helps in keeping the hip flexed and abducted, preventing or reducing the chance
of a hip dislocation; this could serve as treatment for associated hip
dysplasia. This management replaces the Pavlik harness in the African
setting. For kangaroo bag (frontal child carrier bags) users, this affords
the mother the opportunity to do the massage of the neck and manual
stretching even when in transit.
Minor cases will resolve spontaneously with or without treatment,
but there is evidence that early PSEs are effective in almost all cases
when initiated prior to 3 months of age.5,6,12,13
The PSE technique is as follows:6 With the baby supine and head
suspended over the side of the examining table, one adult holds the
babys shoulders while the other rotates the neck to the same side as the
affected muscle. Gentle but firm pressure and some flexion are applied
at the limit of rotation to maximally stretch the muscle for 1015 seconds (Figure 38.11). This procedure is repeated 10 times, twice daily.
The keys to success are to explain the diagnosis and prognosis to the
parents, demonstrate the PSE, watch them do the PSE in the clinic, and
then follow-up in the clinic at 2 and 6 weeks to ensure progress. Parents
are motivated by telling them that PSE prevents the need for surgery in
most cases. Reassure the parents that babies get used to the exercises
and do not remember the discomfort when they are older. With proper
instruction, PSE will not harm the child. The sternomastoid scar will
occasionally snap, which may lead to some temporary swelling, but
this often results in an improved range of motion.14
The PSE should be consistent and continuous until the muscle
tumour and contracture resolve, generally within 68 months.12 PSE
treatment is successful in more than 90% of cases when commenced
within the first 3 months of life.1,6,13 Emery15 followed 101 children
who started treatment at a mean age of 4 months and found that the
average PSE treatment duration was 4.7 months, with all but one child
achieving a full passive range of motion.
A child older than 68 months of age is less cooperative with the
PSE, and surgery is more likely to be necessary because the contracture
is tighter and more fibrotic.
A physiotherapist can be engaged to do the initial demonstration
and follow-up of progress, especially when mothers are afraid to do
the stretching or there is no response to this treatment as expected.
Physiotherapy would need to be done at least three times a week to
be effective;5 a professional physiotherapist service is an additional
cost that many families would want to avoid and may lead to hospital
default. To prevent this, the confidence of the parents is gained by
providing adequate information and instruction on the pathology and
the treatment options.
A neck brace has been used in older children as an adjunct to PSE
or after surgical treatment.16 If used, the neck brace should be carefully
fitted and not worn at night.
Plagiocephaly can be prevented or treated by positioning the head to
avoid persistent lying on the same side of the occiput. Measures tried
Fibrotic mass
Spontaneous resolution
Spontaneous resolution
Type III, 5%
Surgical intervention
Type IV, 3%
Source: Adapted from Hsu TC et al. Correlation of clinical and ultrasonographic features of
congenital muscular torticolis. Arch Phys Med Rehabil 1999; 80:637641.
Figure 38.10: A woman backing an infant with the head turned to the ipsilateral
side of the sternomastoid tumour.
Postoperative Complications
have included placing toys and desirable objects on the ipsilateral side
of the lesion so that the child turns towards it. The child could also be
put to sleep with the head facing the ipsilateral side. Helmet treatment
has been described in some children with severe plagiocephaly,17 but it
is usually impractical.
Operative Treatment
Sternomastoid tumour
Head tilt
Physical exam:
Is there a limitation of neck rotation
indicating muscular torticollis (CMT)?
Plagiocephaly? Hemifacial hypoplasia?
Check for congenital hip dysplasia.
If tumour gets
larger, is atypical, or
doesnt resolve by
6 months, consider
further investigations
and biopsy.
Operative intervention
1. Age > 12months.
2. Failed after 6 months of PSE.
3. Neck rotation <75 degrees.
4. Palpable muscle fibrosis.
5. Progressive facial hemihypoplasia.
Refer to
ophthalmologist
neurologist
orthopaedist
neurosurgeon
Prevention
Ethical Issues
Authors
Cheng JCY, Wong MWN, Tang SP, Chen TMK, Shum SLF, Wong
EMC
Institution
Reference
Problem
Intervention
Comparison/
control
(quality of
evidence)
Outcome/
effect
Historical
significance/
comments
Summary
The flow chart presented in Figure 38.12 summarises the recommendations of this chapter.
Evidence-Based Research
References
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2.
3.
4.
Cheng JC, Wong MW, Tang SP, Chen TMK, Shum SLF, Wong
EMC. Clinical determinants of the outcome of manual stretching
in the treatment of congenital muscular torticollis in infants. A
prospective study of 821 cases. J Bone Joint Surg Am 2001;
83:679687.
Nwako FA. Torticollis. In: Nwako FA, ed. A Textbook of Pediatric
Surgery in the Tropics. MacMillan Press Limited, 1980, Pp 277
278.
Beasley S. Torticollis. In: Grosfeld JL, ONeill JA Jr, Fonkalsrud
EW, Coran AG. Pediatric Surgery, 6th ed. Mosby, 2006, Pp
875881.
Davids JR, Wenger DR, Mubarak SJ. Congenital muscular
torticollis: Sequela of intrauterine or perinatal compartment
syndrome. J Pediatr Orthop 1993; 13:141.
5.
Cheng JC, Tang SP, Chen TM, Wong MWN, Wong EMC. The
clinical presentation and outcome of treatment of congenital
muscular torticollis in infantsa study of 1086 cases. J Pediatr
Surg 2000; 35:10911096.
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torticollis. Pediatr 2006; 118:e1779e1784.
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Correlation of clinical and ultrasonographic features of congenital
muscular torticollis. Arch Phys Med Rehabil 1999; 80:637641.
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treatment is critical. A prospective study. Pediatr Int 2000; 42:504
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C. Snapping during manual stretching in congenital muscular
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muscular torticollis. Phys Ther 1994; 74(10):921929.
16. Symmetric Designs: The TOT Collar for Torticollis Treatment.
Available at: www.symmetric-designs.com/tot-collar-.html
(accessed 30 July 2009).
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