The hypertelorism is divided into congenital muscular hypertelorism and congenital bony hypertelorism. The former is a congenital neck deformity caused by the contracture of the sternocleidomastoid muscle on one side, which is quite common; the latter is due to the developmental deformity of the cervical vertebrae, which is less common.
Etiology
The etiology of congenital myotonic squint is still unknown. However, most scholars believe that abnormal intrauterine pressure or malposition of the embryo is the main cause of congenital myotonic squamous neck. The fetal malposition in the uterus or abnormal pressure on the uterine wall may cause pressure on one side of the neck and local blood circulation in the sternocleidomastoid muscle, resulting in ischemic fibrous degeneration of the muscle and causing oblique neck.
Difficult delivery and the use of forceps is one of the causes of myotonic rhomboid. This view has not been conclusively confirmed because it occurs more often in breech births, but examination of the local mass of the sternocleidomastoid muscle did not reveal signs of old hemorrhage.
It is thought to be genetically related because of a clear family history in 1/5 of children with this condition, and it is often combined with congenital acetabular dysplasia and other malformations.
The main clinical manifestations of the oblique neck are as follows.
1. Oblique neck deformity
After birth, the mother may find that the child’s head is tilted to the affected side, the face is rotated to the healthy side, and the lower jaw points to the healthy shoulder. Turning the head to the healthy side is obviously limited, and the symptoms should be observed carefully in milder cases. This symptom becomes more and more serious as the child grows and develops.
2. Neck mass
A cervical mass is usually palpable after birth or within 2 weeks after birth and is located in the middle and lower part of the sternocleidomastoid muscle, most often on the right side. The mass is shuttle-shaped, without pressure pain, and usually reaches its maximum size after 1 to 2 months, after which it gradually shrinks until it disappears completely, and some of these children may have a mass that does not disappear and produces muscle fibrosis and contracture, resulting in oblique neck deformity.
3.Facial deformity
If congenital myotonic squint is not treated effectively in early stage, facial deformity will appear after 2 years old. The main manifestation is facial asymmetry, the distance from the outer corner of the eye to the corner of the mouth is asymmetric, the distance on the affected side is shortened, and the healthy side grows. The affected side of the eye position plane is reduced, because both eyes are not on the same level, easy to produce visual fatigue and vision loss. The healthy side of the face is round and full, while the affected side is narrow and flat. The cervical spine may develop compensatory scoliosis. In addition, the whole face, including the nose and ears, may be asymmetrically altered.
In addition to these major manifestations, the syndrome may be combined with congenital dislocation of the acetabulum and other deformities of the cervical spine.
Examination.
X-ray of the cervical spine can determine whether it is a bony oblique neck.
Local examination: measuring the length of the sternocleidomastoid muscle on both sides can determine the degree of deformity and surgical lengthening.
Treatment.
Non-surgical treatment: For children under half an age, satisfactory results can be obtained with non-surgical treatment. Therefore, once the diagnosis is made, treatment should be given as early as possible. Non-surgical treatment includes local heat, massage, bed immobilization and manual traction.
The specific method of manual traction is: the mother will lay the child on her knees, so that the child’s neck is stretched back, the mother gently presses the child’s thorax with her left hand, holds the head and neck with her right hand, rotates the child’s face to the affected side as far as possible, and the occiput is rotated to the shoulder peak of the healthy side, the operation process should be gentle, so that the contracted sternocleidomastoid muscle gets a greater stretch. When the child is lying in bed, he/she is placed in the supine position, and the head is fixed with a small sandbag with the face to the affected side and the occiput to the healthy side.
Surgical treatment.
1, surgical indications and contraindications.
(1)Applicable to those who are more than half a year old who are ineffective in conservative treatment;
(2) Oblique neck deformity under 12 years old;
(3)If the facial deformity is not serious above 12 years old, surgery can also be considered;
(4) For adults, because the deformity has existed for many years, not only will the facial deformity be more obvious after surgery, but also the vision will be changed because it is not adapted to the new position after surgery, so surgery is not suitable.
2.The following surgical methods are mainly available;
(1) Sternocleidomastoid excision is one of the more commonly used surgical methods. A transverse incision is made on the clavicle to reveal the sternocleidomastoid head and the clavicular head, and the attachment point is cut above, and the surrounding fascial tissue is released.
(2)Sternocleidomastoid muscle partial excision For obvious neck mass, the mass of sternocleidomastoid muscle can be excised.
(3) Total sternocleidomastoidectomy For adolescent patients, if the entire sternocleidomastoid muscle is scarred, the entire segment can be removed.
(4) Sternocleidomastoid lengthening is to sever the clavicular head of the sternocleidomastoid muscle and lengthen the sternal head in a “Z” shape. The advantages of this surgery are
① Correct the skewed head and neck and restore the normal function of neck movement;
(2) It does not destroy the normal body shape of the cervical triangle and avoids the sunken deformity or abnormal flat deformity of the neck left by other surgical methods, which makes the neck beautiful and symmetrical.
(5) Combined release of superior and inferior sternocleidomastoid muscle plus plication
Conservative treatment method for pediatric oblique neck
The earlier the better. Young mothers and fathers must pay attention to their baby after birth, and if his head is always tilted to one side, they must look at his neck. Pediatric oblique neck is a relatively common congenital disease of the head and neck of children, which can be completely cured in most children with correct and effective non-surgical treatment at an early stage.
1. The presence or absence of a mass in the transverse part. Once it is a squint, don’t panic and go to the doctor as soon as possible. One day earlier treatment will increase the hope of successful treatment.
2.Local drug closure. This is very obvious to promote the absorption of the local mass, often choose prednisolone or hyaluronidase, plus an appropriate amount of lidocaine for local closure. Because it is in the neck, the injection should be very careful.
The drug should be injected in the center of the lump, not too deep injection, so as not to cause accidents by injecting into the blood vessels. If the injection is too shallow under the skin, in addition to being useless, it is also likely to cause infection at the injection site. At the beginning, because of the hard texture of the mass, the resistance to injection is high and should be pushed slowly. Generally, once a week, most children can gradually disappear the lump with 6 times. However, the effect may not be obvious for some people who are not sensitive to drugs or those who are treated late.
3.Local massage. Apply talcum powder and massage the lump repeatedly with the thumb or index finger in the lump.
4.Continue to turn the head repeatedly to keep the head and neck in a corrected position, which is very important for treatment. The specific method is: about 30 to 50 times a day, can be done in parts. When doing so, put the child flat on the bed, the parents hold his head with both hands, turn his jaw to the affected shoulder (i.e., at the neck mass), pause for about 1 minute after turning, so that the muscles are in the elongated and extended state, and then turn again and again. But in the process of turning, the technique should be gentle and prevent violence to prevent damage to the neck muscles and even cervical vertebrae. You can make 2 small sandbags (with green or yellow sand, cleaned with water, placed in the sun or heated with an iron pot to sterilize. Choose a double layer of cloth sewn, the diameter of about 20 × 10 cm size can be), the sandbags placed on both sides of the head when the child is sleeping, can be fixed in a corrected position. This treatment should last for more than 6 months.
5, the mother should choose her position in relation to breastfeeding and sleeping according to the position of the different lesions. For example, if the child has a right-sided sloping neck, you should put the child on your left side during feeding and sleeping, and vice versa. This will facilitate the correction of the affected child.
It should be told that any method can be successful or unsuccessful, and if conservative treatment fails, you should choose to undergo surgery, mostly at the age of about 1 year, preferably no older than 1.5 years. Surgery can also achieve the desired therapeutic effect.
Note the identification of.
1, congenital bony oblique neck This condition is mostly caused by congenital occipital and cervical malformations, including short neck deformity, skull base depression, hemivertebral deformity, atlanto-occipital fusion and dentate developmental malformation. These diseases may cause oblique neck and facial asymmetry, but usually do not produce the typical striated contracture bands and masses of the sternocleidomastoid muscle.
2.Pediatric cervical lymphadenitis Cervical lymphadenitis in infancy can lead to rapid onset of squint and neck mass, but this mass is often painful and not located within the sternocleidomastoid muscle.
3.Spontaneous atlantoaxial rotational subluxation Rotational subluxation of the atlantoaxial spine can also cause a squint, but this disease mostly has a history of minor trauma or upper respiratory tract infection, mainly manifesting as limited rotational movement of the neck and painful symptoms in the neck, and there are no tension bands in the sternocleidomastoid muscle, which can be distinguished by X-ray examination.
4.Cervical spine tuberculosis can cause the sternocleidomastoid muscle spasm and produce slanting neck, but such patients have obvious neck pain, obvious restriction of neck movement and jaw deviation to the affected side, and X-ray examination can make a clear diagnosis.
In addition, it should be distinguished from hysterical squint, habitual squint, injury squint, and squint caused by post-polio.
Complications.
If congenital myotonic squint is not treated effectively at an early stage, facial deformity will appear after 2 years of age. The main manifestations are facial asymmetry, asymmetry of the distance from the outer corner of the eyes to the corner of the mouth, shortening of the distance on the affected side and growth on the healthy side. The affected side of the eye position plane is reduced, because both eyes are not on the same level, easy to produce visual fatigue and vision loss. The healthy side of the face is round and full, while the affected side is narrow and flat. The cervical spine may develop compensatory scoliosis. In addition, the entire face, including the nose and ears, may be asymmetrically altered.