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CHAPTER 38

           Sternomastoid Tumour of Infancy and


                  Congenital Muscular Torticollis


                                                 Lukman O. Abdur-Rahman
                                                     Brian H. Cameron




                           Introduction                        to poor neck control in infants before this age (Figure 38.2). Some of
        Sternomastoid tumour (SMT) of infancy is usually associated with con-  the cases are first noticed by grandmothers while doing the traditional
        genital muscular torticollis (CMT); these will be discussed together in   body massage for the babies.
        this chapter. The term “tumour” is a misnomer because it is a congenital    Physical Examination
        fibrotic process; it also is referred to as congenital fibromatosis coli.   A  sternomastoid  tumour  is  defined  by  the  presence  of  a  palpable,
        The terms “torticollis” and “wryneck” actually describe the tilting and   hard,  spindle-shaped,  painless,  1–3  cm  diameter  swelling  within  the
        rotation of the head and neck that results from the contracture of the   substance  of  the  sternocleidomastoid  muscle,  usually  located  in  the
        sternocleidomastoid muscle. The torticollis, when untreated, results in   lower and middle third of the muscle. The mass may be confused with
        plagiocephaly, hemifacial hypoplasia, and body distortion. The key to   a lymph node or neoplasm, which is far less likely at this age.
        preventing deformity is early diagnosis and passive stretching exercises   Children presenting with CMT should have the entire length of the
        (PSEs) of the affected muscle, with only 5% of cases needing surgical   sternomastoid muscle palpated to determine whether the swelling or area
                                      1
        intervention in a large prospective series.
                           Demography
                                                     2
        The  prevalence  of  SMT  and  CMT  in  Africa  is  unknown.   At  the
        University of Ilorin Teaching Hospital in Nigeria over a period of 10
        years  (1999–2008),  only  15  cases  presented  at  the  outpatient  clinic
        and only one had surgical intervention at 10 months of age because of
        severe torticollis. CMT is reported to occur in 0.3–2% of all births, with
        a slight male preponderance. The right side is affected in 60% of the
        cases, and 2–8% are bilateral.
                              3
                             Aetiology
        The  sternocleidomastoid  muscle  is  enveloped  by  the  deep  cervical
        fascia as it attaches to the clavicle, manubrium, and mastoid process.
        The  aetiology  of  SMT  is  unknown,  but  the  current  theory  is  that  it
        results from abnormal intrauterine positioning, leading to intramuscular
        compartment syndrome and ischaemic muscle injury with subsequent
        fibrosis and contracture of the sternomastoid muscle.  Muscle biopsy   Figure 38.1: Sternomastoid tumour on the right side, with the child’s head
                                                4
        shows replacement of muscle bundles with a mass of maturing fibrous   turned away from the affected side.
        tissue—evidence that the pathology may begin prenatally.
                                                  3
           Contracture of the sternomastoid muscle on one side causes the head
        and neck to tilt (flex) to the ipsilateral side and the face to turn toward
        the contralateral side (Figure 38.1).
           If untreated, the sternomastoid tumour naturally resolves completely
        in 50–70% of the cases by 6 months of age, with muscle shortening
                               1,5
        persisting in 5–7% after 1 year.  Hence, late presentations are usually
        accompanied  by  fibrotic  and  shortened  sternocleidomastoid  muscle
        from a missed or unrecognised sternomastoid tumour.
                       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.
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