What does congenital myotonic neck look like?

  The concept of congenital sternocleidomastoid is a deformity in which the neck and head and face are skewed to the affected side after a fibrous contracture of the sternocleidomastoid muscle on one side. It is generally believed that the cause of this deformity is the hemorrhage and mechanization of the sternocleidomastoid muscle during obstructed labor, resulting in fibrous degeneration and contracture of the muscle. In addition, there are other causes such as intra-uterine and extra-uterine infections, genetics and arteriovenous embolism leading to myonecrosis.  Clinical manifestations After birth, a hard, fixed mass can be felt in the sternocleidomastoid muscle on one side of the baby. After 3 to 4 months, the mass gradually disappears and contracture occurs, resulting in the gradual appearance of a sloping neck. However, there are some children who do not have significant contracture and no deformity due to their mild condition. At about 1 week of age, the oblique neck deformity becomes more obvious, with the head tilted to one side and the chin turned to the healthy side. If the head is forced to be squared, the sternocleidomastoid muscle can be seen to be tense and protrude under the skin, shaped like a stiff cord. During the development process, the face is gradually asymmetrical, the healthy side is full and the affected side is short; the cervical vertebrae are laterally convex and the head movement is restricted.  Diagnosis and differential diagnosis Congenital myelomeningocele is not difficult to diagnose, but it should be differentiated from other causes of myelomeningocele: 1. Myelomeningocele caused by acute lymphadenitis of the neck Most often seen in infants, with fever, swollen lymph nodes and pressure pain in the neck, but there is no syrinx mass or contracture in the sternocleidomastoid muscle.  2.Cervical spine congenital malformation or cervical spine tuberculosis can cause slanting neck, and X-ray can be used to differentiate.  3.Ocular muscle abnormalities The muscle strength of the extraocular muscles is unbalanced, and patients with strabismus coordinate their vision with neck deviation.  Treatment If not treated in time, the deformity can be aggravated with the growth of age. Therefore the earlier the treatment, the better the result. The older the patient is, the more difficult it is to completely correct the strabismus and facial deformity.  1.Manipulation correction therapy: It is suitable for infants within one year old, including local hot compress, massage, manipulation and head fixation. The purpose is to make the mass dissipate early and prevent the muscle from contracture. After 2 weeks of life, the mother can massage the affected sternocleidomastoid muscle and manipulate the head to bend slightly to the healthy side, rotate the chin to the affected side as much as possible, and rotate the neck to the healthy side during each nursing session. Infants sleep with sandbags to keep in the above corrected position, generally within 2 to 3 months most can be cured.  2.Surgical treatment: Applicable to sick children above 1 year old. In 12 years old and above, although the face and neck deformity is difficult to correct, but the surgical treatment can still make the deformity improved. The surgical method is mostly sternocleidomastoidotomy. A transverse incision is made one finger above the proximal clavicle and the clavicular and sternal heads of the sternocleidomastoid muscle are severed. 4 years of age and older, the clavicular and sternal heads can be removed for severe cases of oblique necks about 1 to 50 px. care is taken not to damage the phrenic nerve/general carotid artery and internal jugular vein. The head is then placed in an overcorrected position and immobilized in a cephalocervical-thoracic cast for 3 to 4 weeks.