Congenital Muscular Torticollis

CONGENITAL MUSCULAR TORTICOLLIS

• Congenital muscular torticollis or congenital wry neck, is the most common cause of torticollis in the infant and young child, presenting at a median age of 2 months
• Common on the right side and associated with DDH, metatarsus adductus and CTEV
• The deformity is caused by contracture of the sternocleidomastoid muscle, with the head tilted toward the involved side and the chin rotated toward the opposite shoulder
• Localised near the clavicular attachment of the muscle.
• Usually disappears within a year. If it fails to disappear, the muscle becomes permanently fibrotic and contracted and causes torticollis.
• Asymmetry of face and skull (Plagiocephaly) may be seen rarely in untreated cases.
• This is caused by the sleeping position of the child especially when lying in the prone position with the affected side down. 
• Grisel’s syndrome: Atlantoaxial subluxation secondary to upper respiratory infection in children may present as torticollis.

Theories of Origin:

1) Malposition of the fetus in utero(in utero crowding)

2) Primary neurogenic cause(spinal accessory to clavicular head getting entrapped and causing progressive deformity)

3) Mesenchymal cells remaining in the sternocleidomastoid from fetal embryogenesis- leading to their proliferation.

4) Vascular injury (intrauterine or perinatal compartment syndrome).

Cheng’s Clinical Groups

 i. Sternomastoid tumour group: Those with a clinically palpable sternomastoid tumour.

ii. Muscular group: Those with clinical thickening and tightness of the sternocleidomastoid muscle.

iii. Postural torticollis: Those with postural head tilt and clinical features of torticollis but without tightness or tumour of the sternocleidomastoid muscle. Surgery was commonly done in sternomastoid tumour group.

Differential Diagnosis:

1. Klippel Feil syndrome

2. Ocular dysfunction

3. Lymphadenitis of Neck

4. # Subluxation of C1 – C2

5. Pterygium coli

6. Patients with Down’s syndrome, Spondyloepiphyseal dysplasia and Morquio’s disease may have C1- C2 anomalies and may present with torticollis.

Treatment

Manipulation and stretching exercises in infancy:

• The ear opposite the contracted muscle should be positioned to the shoulder, and the chin should be positioned to touch the shoulder on the same side as the contracted muscle. 
• The child’s toys and crib should be modified so that the neck is stretched when the infant is reaching for or looking at objects of interest
• These exercises are performed at 6 to 7 sessions per day, each session should consist of 20 to 30 manipulations Surgery is undertaken if deformity not corrected by 1 year: Surgery may be unipolar release /bipolar release:
• In unipolar release the clavicular attachment is released and a Z plasty is done for the sternal attachment to maintain the V shaped contour of the neck.
• A unipolar release with postoperative stretching for 3 months with orthotic support is usually sufficient
• A bipolar release is indicated for severe and neglected deformities. For a bipolar release the mastoid attachment is also released

Ref:

1. Cheng JCY, Wong MWN, Tang SP, et al: Clinical determinants of the outcome of manual stretching in the treatment of congenital muscular torticollis in infants: a prospective study of eight hundred and twenty-one cases. J Bone Joint Surg 2001; 83A:679.