Torticollis .
Congenital Muscular Torticollis .
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Incidence. 

The different types of congenital muscular torticollis are frequently observed.

 
Clinical significance.Illustrations

The later the disease is diagnosed and the treatment started, the worse are the primary findings and the secondary sequels (plagiocephaly, asymmetry of the face, scoliosis of the neck, and ophthalmological sequels), and the more difficult a successful treatment.


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Etiology. 

The congenital muscular torticollis is probably due to an intrauterine compartment syndrome of the sternocleidomastoid muscle.

 
Pathology, anatomical types. 
In neonates and infants the muscular changes lead to a more or less distinct torticollis and/or to a tumor in the sternocleidomastoid muscle following a generalized or localized tumor-like fibrosis. In toddlers and schoolchildren there is only a generalized or localized fibrosis, and the tumor-like fibrosis (which is not a hematoma) is not visible any more, but the torticollis is now pronounced in every case.
Pathophysiology .  
The congenital muscular torticollis leads to a decreased torsion and increased inclination of the head on the same side and secondarily to an abnormal posture.  
Clinical presentation (history, findings). Illustrations

The parents tell about a firm mass in the neck and/or about a preferred position of the head and a preferred look to one side; or they are worried about an abnormal shape of the skull (see craniosynostosis).
Local findings: Often, a firm mass in the distal part of the sternocleidomastoid muscle is visible and palpable which tapers off in the proximal and distal part of the muscle = sternomastoid (pseudo) tumor, or a muscle which is rough when compared to the normal side is palpable = sternocleidomastoid fibrosis.
The completely developed clinical picture of a congenital muscular torticollis is mainly visible in toddlers and schoolchildren: Inclination of the head to the involved and torsion to the contralateral side with prominence of the involved muscle like a string, and secondary sequels to the head, face and cervical spine.


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Clinical presentation (clinical Skills). Illustrations

An optimal clinical examination should be performed in supine position of the infant/toddler which allows a simultaneous palpation of both sternocleidomastoid muscles, and a determination of the rotation of the head to both sides as an objective and reproducible measure of a possible limitation in the movement of the head (normal values 80 to 90 degree to both sides. Cheng JCY et al. J Pediatr Surg 35:1091,2000).


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Natural history.Illustration

The congenital muscular torticollis may be reversible clinically in infants in 80 %. 20 % need surgery either as infants or as toddlers, or later in life in missed cases (see clinical picture).


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Differential diagnosis.Illustrations

Amongst the congenital causes of torticollis, the following should be considered: congenital postural torticollis, Klippel-Feil syndrome and Sprengel's deformity, and C1 to C2 articular malformations.
Acquired causes of torticollis include the acute torticollis or wryneck, trauma to the cervical spine, and some rare causes (Sandifer's syndrome = torticollis and gastrooesophageal reflux, ocular and neurological torticollis such as strabism or tumor of the posterior fossa).
Finally, all lesions that may lead to the same primary signs and to the same secondary sequels of congenital muscular torticollis (shoulder elevation, plagiocephaly, and so on).


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Work-up examinations.Illustration

Additional examinations depend on the age of the patient and the suspected differential diagnoses according to the history and the local findings.
Beyond infancy: X-rays in two planes of the whole cervical spine should be performed in every case.


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Therapy.Illustrations

The sternocleidomastoid tumor and fibrosis are resistant to all therapies; however, the former disappears spontaneously.
Depending on the patient's age, the degree and sequels of the congenital muscular torticollis, the following measures may be taken:
a) stimulation of movements of the head and positioning of the head.
b) passive neck range-of-motion exercices by or under the guidance of an experienced physiotherapist.
c) low division of the sternocleidomastoid muscle followed by a postoperative physiotherapy.

Surgery is indicated:
a) in infants if physiotherapy fails, or with progressive sequels;
b) in all toddlers and older children.

No long-term case-controlled studies are available about alternative methods of treatment.


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Prognosis.Illustrations

Prognosis depends on the time of diagnosis, the degree of sequels, and the appropriateness of the measures.


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