Congenital muscular torticollis is an asymmetrical deformity of the head and neck in which the head is tilted toward the side with the shortened muscle and the chin rotated towards the opposite side. This is caused by unilateral contracture of the sternocleidomastoid muscle.
The term torticolis is derived form two Latin words, tortus, meaning “twisted,” and collum, meaning “neck,.”
Wryneck is a lay term is used to describe torticollis arising from any cause.
The condition is more common in girls than in boys.
The immediate cause of the deformity is fibrosis within the sternocleidomastoid muscle, which subsequently contracts and shortens. The exact pathogenesis of the fibrosis is unknown. Edema, degeneration of muscle fibers and acute inflammation lead to fibrosis.
Brooks also found that permanent occlusion of only the arterial supply of the muscle resulted in extreme atrophy ad necrosis of the muscle but did not cause fibrosis and replacement with fibrous tissue. Intramuscular hemorrhage with or without interference with the nerve or arterial supply did not produce fibrosis. Jepson, in 1926, and Middleton, in 1930, did similar experimental work and arrived at essentially the same conclusions as Brooks.
Right side is involved in about 75 percent of cases.
The exact cause of fibrosis of the sternocleidomastoid muscle in congenital muscular torticollis is not known. Intrauterine malposition is commonly associated with the deformity and possibly it is due to a local ischemic process resulting from intrauterine malposition.
On section, the “tumor” appears white and glistening, in gross appearance resembling a soft fibroma. Microscopic study shows that it consist of dense fibrous tissue. In an older child, after the disappearance of the tumor, tissue excised from the sternocleidomastoid muscle shows that the muscle has been replaced fibrous tissue.
Other findings in the muscle consist of muscle giant cells, loss of transverse striations, vacuolozation, and disruption of endomysial sheaths.
The deformity may be present at birth or it may become evident about the second or third weeks.
The head is tilted towards side of the affected muscle, and the chin is rotated to the opposite side. Rotation of the neck to the side of the deformity and lateral motion to the opposite side are limited.
Palpation reveals a hard, nontender, fusiform swelling, or tumor, in the sternocleidomastoid muscle. Usually both the sternal and clavicular heads are involved. Occasionally only the sternal head is affected. The superior portion of the muscle close to its mastoid attachment is rarely, if ever, involved.
It gradually enlarges during the ensuing two to four weeks, reaching the size of the distal phalanx of the adult thumb. Then it begins to regress and gradually disappears in two to six months.
If the contracture is not treated secondary deformities of the face and head develop. The face on the side of the contracted muscle becomes flattened because of external pressure. The infant usually sleeps in prone posture.
Spontaneously, as it is more comfortable, the neck is rotated so that the affected side is down. Ipsilaterally, the face is flattened by remodeling to conform to the bed. A word of caution is appropriate plagiocephaly can be congenital owing to synostosis of the coronal fissures; this is ruled out by radiograms of the skull.
With skeletal growth, asymmetry of the face increase. The levels of the eyes and ears change, defects that are less noticeable when the head is tilted to one side and more obvious when the head and neck are straight in the midline. Eyuestrain may result from ocular imbalance. A lower cervical-upper dorsal scoliosis with concavity towards the affected side may develop.
If the deformity is not corrected the soft tissues of the affected side undergo adaptive shortening as growth proceeds. The deep cervical fascia becomes thickened and contracted. The scalenus anterior and medius muscles become shortened. Later, the carotid sheath and the enclosed vessels contract.
In rare cases of double torticollis, in which both sternocleidomastoid muscles are affected, the neck is in the midline but appears short, the chin is elevated, and the face is tilted upward.
Recognition of congenital muscular torticollis is not usually difficult because of the characteristic cordlike contracture of the sternocleidomastoid muscle. The early fusiform tumor may escape notice. Postural torticollis should be distinguished from congenital muscular torticollis.
The postural deformation is caused by intrauterine malposture, and the deformity is less severe. Although the sternocleidomastoid muscle is shortened, there is no true fibrotic replacement of the muscle. Often there are other findings associated with intrauterine malposture such as pelvic obliquity with abduction-adduction contracture of the hips, or postural metatarsus varus or valgus.
Postural torticollis responds quickly to passive manipulative stretching.
Torticollis may be caused by contracture muscles other than the sternocleidomastoid, such as the scalenus anterior and the omohyoid.
When surgical release corrected torticollis, the larynx returned to midline position and the asymmetrical face returned to normal. Every patient should have radiograms of the cervical spine made to exclude congenital anomalies of the vertebrae, such as hemivertebrae, unilateral atlanto-occipital fusion, and the Klippel-Feil syndrome.
Differential Diagnosis of Congenital Muscular Torticollis
- Postural torticollis
- Hemivertebra, cervical-superior dorsal spine
- Unilateral atlanto-occipital fusion
- Klippel-Feil syndrome
- Unilateral congenital absence ofsternocleidomastoid muscle
- Pterygium colli
Trauma-particularly C1, C2
- Rotatory subluxation
Inflammatory conditions unilateral
- Cervical lymphadenitis
- Spontaneous hyperemic subluxation of the atlas
- Rheumatoid arthritis
- Visual disturbances
- Cervical spinal cord tumor
- Brain tumor, posterior fossa
Treatment should be begun as soon as the diagnosis is made. Manipulations consisting of passive stretching of the contracted sternocleidomastoid muscle should be performed by the parents after adequate instruction.
First the head is bent laterally so that the ear on the side opposite the shortened muscle approaches the shoulder, then the head is rotated so that the chin approaches the shoulder of the affected side.
During these manipulations, in order to obtain maximum stretching of the sternocleidomastoid muscle, the neck should be in comfortable hyperextended position and counter traction should be applied by holding the ipsilateral shoulder and chest.
An additional method of stretching the sternocleidomastoid muscle is to make use of gravity by placing the infant supine on the mother’s lap with the head hanging into hyperextension. One hand stabilizes the shoulders and chest, and the other hand tilts the head laterally away from the contracted muscle so that the contralateral ear touches the opposite shoulder. The chin is then rotated toward the contracted muscle.
It is important to hold the muscle stretched to the count of 10. The exercises should be performed 15 to 20 times in each direction, 4 to 6 times a day. In addition, the crib should be turned so that the infant’s unaffected side is against the wall and he will rotate his head to look toward the involved side when his attention is attracted, actively stretching the muscle when reaching and grasping for toys.
Prone posture during sleep should be avoided, as it will aggravate the facial deformities and the contracture.
Ordinarily, if the stretching exercises are begun at a very early age and performed faithfully and correctly every day, the contracture of the sternocleidomastoid muscle will corrected and surgery will not be necessary.
Surgery is indicated when the torticollis does not respond to conservative measures up to one year of age,
in cases in which the condition is neglected until the child is a year old or when the parents have not complied in performing an effective exercise regimen.
It is unlikely that the fibrous cord that replaces the sternocleidomastoid muscle can be stretched by manipulation after the age of one year. This is especially true is restriction of rotation of the neck is greater than 30 degrees and there is an established facial asymmetry.
Satisfactory results are usually obtained by division or partial excision of the muscle, provided the head is kept in the corrected position for a sufficient length of time after the operation, and active and passive exercises are carried out to prevent any recurrence of the deformity.
The muscle may be divided at either end or at both ends.
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