Torticollis - Congenital Muscular

TORTICOLLIS

 Torticollis (“twisted neck”) is the term used for both congenital and acquired deformities (of both organic and psychogenic origin) of “wryneck”.  Also called collum distortum and caput obstipum.  Bilateral torticollis has been reported.  When the condition persists beyond a few weeks in infancy, deformational plagiocephaly develops – contralateral occipital flattening  This skeletal deformity is reversible only if the condition is corrected during the period of growth, and few/no changes will be seen in adults and older teenagers.

Classification of torticollis

Congenital

1. nonosseous a. muscular b. ocular i. most commonly due to 6th nerve palsy ii. Eye goes down and out – patient turns head away from side of lesion to obtain binocular vision 2. osseous a. vertebral maldevelopment 3. neurologic

Acquired 1. acute infection - Cervical osteomyelitis and retropharyngeal space infection 2. trauma - Occipital condyle fractures, cervical muscle spasm following motor vehicle accident, and odontoid fractures (Atlanto-axial subluxation) 3. tumors 4. ocular - hypertropia or hypotropia, inferior oblique palsy 5. drugs – L-dopa, neuroleptics - causes focal dystonia 6. spasmodic / neurogenic – idiopathic spasmodic torticollis . benzodiazepines / anticholinergics / baclofen / neuroleptics / adrenergic blocking agents - none are consistently successful 7. psychogenic 8. Benign Paroxysmal Torticollis . a dystonic phenomenon . begins in early infancy . recurrent episodes of sudden, stereotypic torticollis, usually alternating from side to side . episodes that usually continue for several hours to a few days. . disappears by 1 - 5 years of age . attacks of torticollis associated with vomiting, ataxia, drowsiness 2

. ?peripheral vestibular dysfunction

Congenital muscular torticollis

 The condition results from a shortening of the sternocleidomastoid muscle - either the sternal or clavicular heads may predominate, or they may be equally involved.

 characterized by 1. chin tilts to contralateral side 2. tilting of the head to the ipsilateral side, 3. shoulder is higher on the affected side. 4. contralateral deformational plagiocephaly (80-90%)

Aetiology

Various theories have been put forward, but the true cause is unknown: 1. Venous occlusion (most probable) – compartment syndrome of the muscle 2. Rupture of the sternocleidomastoid muscle and haematoma formation during labour 3. Intra-uterine malposition/compression causes pressure and ischaemia of the muscle, predisposing it to damage by a traumatic or even normal delivery a. preponderance of first-borns, breech and difficult deliveries 4. Congenital defect in the development of the muscle anlage

Incidence 1. 1 in 300 live births 2. equal sex and side incidence

Clinical course

 Diagnosis made on average first 2-3 months.  30-40% develop a pseudotumor/swelling over SCM - usually discovered first 2-3 weeks of life.  The swelling may increase for two to four weeks and remain stationary for two to three months, gradually regressing and disappearing in four to eight months.  In a few cases, residual shortness of the SCM may remain, producing torticollis and asymmetry.  In another small group, torticollis may not be apparent until three or four years later when the neck elongates.

Examination  Examine neck movement with tracking  Palpate muscle 3

o May feel pseudotumor o muscle feels short and inelastic.  Examine head and face shape o Head is tilted to the affected side and the chin points up and to the opposite side. The cervical vertebrae are normal. o Deformational plagiocephaly seen in 80-90% . occiput flat on contralateral side.  Examine spine

Pathology

Sternocleidomastoid tumour . immature cellular fibrous tissue containing dispersed remnants of muscle tissue

Fully developed case . swathes of adult non-cellular fibrous tissue interspersed with living, healthy, muscle fibres

DDx 1. other muscle – rarely trapezius and paraspinal muscles 2. osseous abnormalities; such as Klippel-Feil syndrome, hypoplasia of the lateral mass of C1, or atlanto-occipital abnormalities 3. Sandifer syndrome is characterized by gastroesophageal reflux and torticollis or tilting of the head, presumably an attempt on the child to be more comfortable. It may present in infancy or later childhood, usually in children with cerebral palsy. 4. Neurogenic tumors or malformations, of which posterior fossa lesions are most common.

Treatment

 Surgery is not advised for sternocleidomastoid tumour because most disappear  well accepted that patients older than 1 year with a definite tight band of the sternocleidomastoid muscle should be treated surgically, there is no consensus on the care of patients younger than 1 year of age.  Treatment of patients younger than 1 year of age ranges from prophylactic resection of the sternocleidomastoid tumor to observation alone until after the age of 1 year

Conservative 1. Early physical therapy a. Optimally started before 6 months of age 4

b. Manual stretching is the most common form of treatment – stretching should NOT be painful and should be carried out by parents whenever possible c. successful in over 80% 2. Positioning a. To avoid plagiocephaly 3. Adjuvant measures such as moulding helmet therapy, halo devices, and adjustable cervical collars (TOT collar) have been advocated with variable rates of success 4. Botox a. Very useful adjunct to physical therapy b. Enhance effectiveness of stretching exercises c. Shown to be safe in children and effective when used after failure of conservative management

Surgery  Surgical intervention is reserved for those patients that 1. presence of a tight band or tumor in the sternocleidomastoid muscle 2. fail to respond to physical therapy 3. progressive craniofacial growth asymmetry or severe neck angulation. 4. age >1 yr 5. recurrent disease

 There is general agreement that those patients that have muscular release before 1 year of age have the best chance of reversing their facial and skull deformities.  In long-standing cases over 1 year shortening of other structures may occur, and these structures include the trapezius, scalene, the platysma muscles, and the carotid sheath. It is important to recognize this fact because these structures may need to be released at operation to release the neck sufficiently to achieve a good result.

Principles: 1. identification and release of all restricting bands involving the sternocleidomastoid muscle and other neck structures; 2. moving the head through a full range of motion before the completion of the procedure; 3. resuming physical therapy within 2 weeks of operation to prevent recurrent scar contracture. Procedures 1. complete excision of the SCM in infancy 2. tenotomy of the superior end of the muscle (superior open tenotomy) 3. tenotomy of the inferior heads (most popular) a. with or without partial excision of the tumor or sternocleidomastoid muscle b. some divide clavicular head and lengthen the sternal head 4. tenotomy at both superior and inferior ends (ie bipolar release) a. bipolar release with excision of the proximal 2.5 - 4cm of the SCM 5

 Ferkel et al (1983) • lengthening of the sternal attachment with a Z-plasty of the sternal head and complete division of the clavicular head • concomitant release of contracted bands of fascia and muscle (platysma / scalenus anterior / trapezius) • this is followed by head halter traction for 2 - 4 weeks, and a cervical collar holding the head in an overcorrected position for 3 - 4 months

 Endoscopic

Technique of inferior tenotomy

 A transverse lower neck incision is made along skin lines about 1 cm above the clavicle, and the sternal and clavicular heads of the muscle are identified and usually both divided.  Range of motion of the neck at this time will bring into prominence any structures that may tether the neck, such as the trapezius, scalene, or platysma muscle, or the carotid sheath.  If any of these structures are shortened and tether the neck, they need to be released sufficiently under direct vision to allow full range of motion. Failure to do this may result in a suboptimal result.  Total or subtotal resection of the cosmetically important sternocleidomastoid column is not necessary and is deforming. Furthermore there is a significant risk of spinal accessory nerve or great auricular nerve injury.  Potential problems of inferior open tenotomy are hollowing at the base of the neck and a bony prominence of the sternal head of the clavicle. In practice, the occurrence of these deformities is highly variable and unpredictable.

Beware • great auricular nerve • spinal accessory nerne • facial nerve as mastoid is not well pneumatised (superior release) • external and internal jugular vein • carotid artery

Also • diplopia may be present post-operatively • the patient’s equilibrium may be disturbed for a few days

 In general, the earlier the age in childhood at which the operation is performed, the better are the results, although good results have been reported in late childhood as well. 6

 Earlier operation not only improves head tilt, neck bands, and loss of normal neck contour, but also prevents craniofacial asymmetry that appears to result from restricted growth on the affected side.

Other options:

 A release of the sternocleidomastoid muscle by division of its upper insertion at the mastoid process has the advantage of a well-hidden retroauricular scar and maintenance of lower neck fullness. In early childhood, the mastoid cells are not pneumatized, and the facial nerve is at risk for injury here.  Muscle lengthening procedures have been touted. An advantage to this procedure is the maintenance of the cosmetically important lower neck sternomastoid column fullness.  Total sternomastoid excision also has been described, but is no longer used routinely because of significant postoperative neck asymmetry and complications such as spinal accessory nerve or great auricular nerve injury.  In most patients an improvement in the aesthetic deformity is the primary objective. 7

Fig. 1. Drawing of child with various surgical incisional approaches marked with dotted lines. (A) Scalp incision for endoscopic approach. (B) Postauricular incision for superior pole release. (C) Incision for mid muscular transection. (D) Supraclavicular incision used for lower pole release or lower part of bipolar release.

Complications 1. Hematoma 2. Nerve injury 3. wound breakdown 4. superficial wound infection 5. disfiguring scar 6. tethering of the scar 7. loss of the sternocleidomastoid muscle contour 8. formation of lateral band. 9. Recurrence a. More likely to recur during growth spurts when treated conservatively