Congenital Muscular Torticollis (CMT)

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Congenital muscular torticollis is a common cause of wry neck. This is also a common clinical case in orthopedic examinations for students, interns & residents. In this post, I try to summarize the most salient features of this disease.

 

What is Congenital muscular torticollis?

It is wry neck caused by fibromatosis within the sternocleidomastoid muscle.

 

What is the natural history ?

• presents as mass in neck most commonly  near clavicular attachment of sternoclaviculomastoid muscle
• at birth or in first 2 weeks.
• right >left.
• attains maximal size within 2 months and then remains so or regresses
• usually it diminishes and disappears within a year.
• If it fails to disappear, the muscle becomes permanently fibrotic and contracted and causes torticollis that is also permanent unless treated.

 

What are common Associations?

· difficult delivery.

· metatarsus adductus,

· developmental dysplasia of the hip 7% to 20%, and

· talipes equinovarus.

 

Why does it happen?

· malposition of the fetus in utero,

· birth trauma,

· infection, and

· vascular injury

. sequela of an intrauterine or perinatal compartment syndrome.

 

 

clinical groups:

· sternomastoid tumor group (those with a clinically palpable sternomastoid tumor)-needs surgery more often

· muscular groups (those with clinical thickening and tightness of the sternocleidomastoid muscle), and

· postural torticollis (those with postural head tilt and clinical features of torticollis but without tightness or tumor of the sternocleidomastoid muscle)-corrects with stretching

 

Does ultrasonography help?

· helps predicting which infants would require surgical treatment.

· if fibrotic change is limited to lower third sternocleidomastoid muscle ,most patients recover without surgery

· whole muscle fibrotic involvement requires surgical release

 

Does non-operative treatment help?

· Yes, during infancy.

· parents should stretch the sternocleidomastoid muscle by manipulating the child's head manually.

· effective in about 95% before the age of 1 year.

· more likely to be successful if the restriction of motion was less than 30 degrees and no facial asymmetry or the facial asymmetry was noted only by the examiner.

 

What is the Surgical treatment?

· Unipolar release

.Bipolar release

 

When to operate? Why?

. operation be delayed until the child is between the ages of 1 and 4 years.

. CMT did not resolve spontaneously if it persisted beyond age of 1 year.

· Nonoperative therapy after the age of 1 year was rarely successful.

· Children who were treated during the first year of life had better results

· surgery should be delayed until evolution of the fibromatosis is complete,

· Surgery performed before the age of 6 to 8 years may allow remodeling of any facial asymmetry and plagiocephaly. Helpful upto 12 years of age.

· tethering of the scar to the deep structures is common before the age of 1 year

 

UNIPOLAR RELEASE

. It is release of sternal and clavicular attachments of the sternocleidomastoid muscle

. It is appropriate for mild deformity

· Skin incision: 5 cm long just superior to and parallel to the medial end of the clavicle

Incise the tendon sheath of  sternocleidomastoid muscle longitudinally and resect 2.5 cm of tendon ends.

· Next, with the child's head turned toward the affected side and the chin depressed, & divide remaining bands of contracted muscle or fascia until the deformity can be overcorrected.

· If overcorrection is not possible, do a bipolar release by a small transverse incision inferior to the mastoid process. Avoid damaging the spinal accessory nerve.

 

 

BIPOLAR RELEASE

. It is release of sternocleiodmastoid muscle at both its attachments

. It is suitable for moderate /severe deformity, after failed operation or in patients older than 6 years of age

· For this, make a short transverse proximal incision behind the ear

. divide the sternocleidomastoid muscle insertion transversely just distal to the tip of the mastoid process.

·  Next, make a distal incision 4 to 5 cm long in line with the cervical skin creases, a fingerbreadth proximal to the medial end of the clavicle and the sternal notch.

· Cut the clavicular portion of the muscle transversely and perform a Z-plasty on the sternal attachment so as to preserve the normal V-contour of the sternocleidomastoid muscle in the neckline.

 

 

Postoperative protocol?

· Syres’s traction for 1 week

· At 1 week, manual stretching to maintain the overcorrected position is begun.

· Manual stretching should be continued three times daily for 3 to 6 months

· Molded cervical orthosis for 6-12 weeks.

 

 

What are possible complications of surgery?

• tethering of the scar to the deep structures
• reattachment of clavicular or sternal head
• failure to correct the tilt of the head
• failure of facial asymmetry to correct.
• damage to anterior and external jugular veins and the carotid vessels
• damage to spinal accessory nerve
• recurrence
• loss of v-contour of neck

 

Which cases have Bad prognosis

· established facial asymmetry

· limitation of motion of more than 30 degrees at the beginning of treatment

· rotation deformity of more than 15 degrees

· clinical group (sternomastoid tumor)

· older age at presentation

 

What are the possible Sequelae of no treatment/

· torticollis slowly becomes worse during growth.

· head becomes inclined toward the affected side and the face toward the opposite side.

· If the deformity is severe, the ipsilateral shoulder becomes elevated

· frontooccipital diameter of the skull may become less than normal.