Short neck in a newborn: a disease or physiological problem that can be corrected

Causes of the disease

Short neck syndrome, in fact, is not a disease, but a special anomaly in the development of the spine, leading to the occurrence of many other diseases of the spine.
The development of this disease in the prenatal period can occur as a result of:

• segmentation;
• vascularization disorders; aplasia;
• delayed fusion in the fetal and embryonic periods of paired formation of vertebrae;
• hypoplasia.

Formed synostoses of the upper thoracic and cervical vertebrae, a decrease in the number of cervical vertebrae to 4 - 5, non-fusion of the vertebral bodies and arches allow us to ultimately determine not only the general clinical picture, but also to understand how severe the deformation is in this syndrome.

All experts identify hereditary factors that contribute to the occurrence of Klippel-Feil syndrome:

1. Genetic hereditary defect in chromosome 12 or 5, 8. In a sick child, there is a complete disruption of the formation of the so-called growth differentiation, which is necessary for the normal further development of the skeleton (including the formation of boundaries between joints and bones). This inevitably leads over time to disruption of the formation and formation of the upper thoracic and cervical vertebrae in the 3rd - 8th week of development inside the womb.
2. Autosomal dominant type of inheritance of the disease. With this form of inheritance in a family in which one of the parents is sick, the probability of having a sick child is from 50 to 100 percent. This type of inheritance occurs most often in Klippel-Feil syndrome.
3. Autosomal recessive type of inheritance of the disease. In this case, in a family in which one of the parents is sick, the probability of having a sick child is from zero to 50 percent.

Symptoms and course of the disease

The main signs of short neck syndrome are:

• low back hairline;
• shortened neck (brevicollis);
• “proud head position” (the head tilts slightly backward);
• restriction of neck mobility - may not be noticeable if there is fusion of fewer than three vertebrae or if fusion is limited to only the lower cervical vertebrae. Mobility limitations are more noticeable with rotation than with flexion/extension or lateral flexion of the neck.

The following symptoms may also occur, the presence of which is not mandatory, but with Klippel-Feil syndrome they occur quite often:

• scoliosis - sideways curvature of the spine;
• facial asymmetry;
• torticollis (curvature of the neck);
• wrinkling of the skin of the neck;
• high shoulder blade;
• weakness of facial muscles (paralysis);
• cleft palate;
• deafness.

It is important to note that Klippel-Feil syndrome is quite often combined with many other anomalies. So, in approximately 25 - 30 percent of patients the following are determined upon examination:

• scoliosis;
• bone rigid form of torticollis;
• wing-shaped folds on the neck;
• high position of the shoulder blades - Sprengel's disease.

In addition, Klippel-Feil syndrome may be accompanied by the development of abnormalities of the lower and upper extremities:

• hypoplasia of the first finger of the hand;
• syndactyly;
• extra fingers;
• absence of the ulna;
• hypoplasia of the pectoral muscle;
• hypoplasia of the upper limb;

deformation of the feet. Hidden anomalies in the development of internal organs in patients with this disease pose a greater danger to their lives than Klippel-Feil syndrome itself. The fact is that approximately 35 percent of patients have very severe and dangerous anomalies of kidney development in the form of hypoplasia of the pelvis, aplasia, hypoplasia, ectopia of the ureters and hydronephrosis. In addition, anomalies in the development of the cardiac and vascular systems are quite often observed (patent fusion of the ductus arteriosus, defects in the development of the interventricular septum, absence of a lung, dextroposition of the aorta, and others).

Disruption of the development of the entire spinal column is often accompanied by serious disruptions in the development of the child’s nervous system: at an early age, negative changes in the central nervous system can manifest themselves in the form of synkinesis, that is, friendly, involuntary movements of the arms and hands; at an older age, neurological symptoms in most cases are supplemented by complex secondary changes in the roots and spinal cord, which developed as a result of various degenerative changes in the spine, as well as due to protrusion of the cranial base and narrowing of the entire spinal canal.

Forms

Based on X-ray data, the following forms of Klippel-Feil syndrome are distinguished:

• fusion of several cervical vertebrae;
• reduction in the size of the cervical vertebrae;
• absence of several cervical vertebrae.

A combination of these signs is possible.

Treatment of the disease

Many people believe that this syndrome is incurable. However, in order to understand whether this disease can be cured and whether surgery will help, you should undergo examination by a highly qualified specialist.

If you have Klippel-Feil syndrome, you should contact a traumatologist and surgeon. You may also need to consult a medical geneticist and neurosurgeon.

1. Non-surgical methods (ineffective):

• a set of exercises, physical therapy and massage that increase the mobility of the cervical spine;
• fixation of the cervical spine with a special collar;
• Drug therapy is resorted to in those severe cases when the patient experiences compression of the roots (nerves) and severe pain. In this situation, doctors rarely prescribe anti-inflammatory non-steroidal drugs, since they are ineffective. And since inflammation is not observed in patients with Klippel-Feil syndrome, doctors prescribe them traditional analgesics and prescribe various physiotherapeutic procedures that are designed to reduce pain.

1. Surgical methods:

• to increase the mobility of the neck, 3-4 upper ribs are removed (cervicalization according to Bonola) - the surgeon makes a paravertebral incision along the edge of the scapula (inner) and the spinous processes. From the edge of the scapula, the doctor cuts off the rhomboid and trapezius muscles - they are retracted inward, and the scapula outward. Then, over a distance of 10-18 cm, the doctor resects the 1st-4th ribs and removes the periosteum. Afterwards, immobilization is carried out with a plaster bed, and later with a polyethylene head holder. The operation is performed on one side, and when the recovery period is complete, the operation is performed on the other side. After the operation, the prognosis is favorable, the neck not only becomes longer, but also acquires significantly greater mobility;
• fixation of underdeveloped vertebrae with special metal pins.

How to treat diaper rash on a baby's neck?

If diaper rash occurs in a child, it is important to begin treatment as soon as possible. Following the rules of hygiene and caring for your baby’s skin will help you quickly cope with diaper rash and prevent the development of complications.

To relieve the first symptoms of skin diaper rash, instead of the usual baby cream, it is necessary to use products with such active substances as: panthenol, benzalkonium, cetrimide.

If, in addition to redness, the baby already has pimples, pustules and microcracks, then in addition to the above measures, it is recommended to treat the skin with drying agents based on zinc and talc, and you can also use methyluracil, tannin and other ointments.

Diaper rash with cracks and erosions is the most difficult to treat. For such manifestations of skin diaper rash, lotions with various solutions are used (for example: tannin, silver nitrate).

After eliminating weeping, it is recommended to apply zinc-containing preparations, as well as antibacterial ointments.

If a secondary infection is clearly attached, topical antibiotic-based drugs are prescribed (in the case of a bacterial infection), and external antifungal drugs (in the case of a fungal infection).

In any case, either a pediatrician or a dermatologist makes a diagnosis and prescribes treatment, so if any rashes appear on your baby’s skin, you should consult a specialist!

Self-medication can lead to unpleasant consequences, but a correct diagnosis in time will save the mother’s nerve cells and the baby’s health!

Diagnosis of the disease

Diagnosis of Klippel-Feil-Sprengel syndrome is based on a combination of symptoms: shortening of the neck observed from birth, low hair growth on the neck and limited head mobility.

To clarify the type of deformation, an X-ray examination of the cervical and thoracic spine is performed. X-ray of the cervical and upper thoracic spine (spondylography) allows you to accurately assess the condition of the vertebrae, identify their reduction and fusion. X-ray examination is also carried out in the position of maximum flexion and extension of the neck to identify instability in the cervical spine. The images often reveal the fusion of 4-6 vertebrae into a solid bone mass.

Ultrasonography:

• heart (often Klippel-Feil syndrome is combined with patent interventricular septum, patent Botallian duct, which closes 1-2 weeks after birth);
• kidneys (sometimes with Klippel-Feil syndrome one kidney is missing).

ECG (electrocardiography) can detect signs of cardiac dysfunction.

Research of the family tree - through a detailed conversation with the patient and his relatives. Information about the presence of similar complaints in relatives, as well as the age of development of the first symptoms and the degree of relationship (close, distant) is important.

Genetic research is carried out on the patient and family members in order to search for a genetic defect that is inherited.

It is also possible to consult a neurosurgeon or medical geneticist.

Differential diagnosis is carried out with tuberculous spondylitis of the upper cervical vertebrae, bilateral and unilateral forms of muscular and other types of torticollis (especially in the absence of effect from conservative treatment).

Causes of a short neck in an adult

The reasons for the formation of a short neck in an adult can be injuries, degenerative dystrophic processes, poor posture and subluxation of the first cervical vertebra (atlas).

In adulthood, the intervertebral discs are fully formed and are strong cartilaginous structures consisting of an outer membrane (annulus fibrosus) and an internal pulposus (nucleus pulposus). They provide shock-absorbing load distribution during movements and protect the radicular nerves, extending from the spinal cord through the foraminal openings of the vertebral bodies, from compression.

At the age of 25–30 years, primary dystrophic changes in the fibrous ring begin. It is capable of receiving fluid and nutrients dissolved in it only through diffuse exchange with adjacent muscle tissues. Leading a sedentary lifestyle, prolonged static tension of the muscles of the neck and collar area (for example, when working at a computer or while watching movies) spasms of blood vessels occur. The fibrous ring of the intervertebral disc becomes dehydrated and loses its ability to straighten (restore its shape) after compression. This is the very initial stage of the development of osteochondrosis.

Then a persistent decrease in the height of the intervertebral disc is formed - this is the stage of protrusion. If the pathology continues for a long time, a disc herniation may form. It loses its shock-absorbing ability and ceases to separate adjacent vertebral bodies. The mechanism of their fusion may be triggered due to the formation of a bone callus.

The second common cause of short neck syndrome in adults is the consequences of surgery to remove an intervertebral disc affected by a herniated protrusion. During the operation, the doctor takes measures to fuse adjacent vertebral bodies. Within 2–3 years after surgery, hernial deformities damage adjacent intervertebral discs. After a series of operations, the cervical spine loses mobility and becomes short.

Other potential causes include:

• stoop and other types of poor posture;
• the habit of sitting on the gluteal muscles without emphasis on the back of the thigh, which entails inevitable dislocation of the atlas (the first cervical vertebra);
• sedentary lifestyle;
• improper organization of sleeping and working spaces;
• injuries to the neck and collar area;
• falls on the gluteal region and back.

It is necessary to exclude all possible causes before starting treatment. Therefore, an important task facing the doctor is to fully collect anamnesis and identify the etiotropic factor in the development of short neck syndrome in an adult or child.

Prices

Plagiocephaly

Therapy for positional plagiocephaly

Surgical interventions are not required; correction is carried out using conservative methods.
The main role is played by preventive measures to prevent the formation and worsening of misalignment. Parents are advised to regularly turn their baby onto his stomach, except at bedtime. Many babies with plagiocephaly do not like this position of the body, so to prevent crying and attempts to roll over, it is recommended to distract the baby’s attention with communication and bright toys. Another measure to prevent the development and progression of the deformative type of the disease is to alternately lie on one side or the other. Since children do not like to lie facing the wall, when there is resistance and turning over, it is worth turning the crib or occupying the child’s attention with bright objects. With early diagnosis, the listed measures are enough to ensure restoration of the normal shape of the head during the period of active growth of the skull.

If the method is ineffective, a corrective plastic helmet is used, which is made individually taking into account the parameters of the patient’s head. This device is a semi-closed structure that equalizes the pressure on different parts of the skull and stimulates its growth in the desired direction. Plaster casts were previously used for this purpose, but are now rarely used due to the higher level of discomfort for the child and the difficulty of care for the parents.

Treatment with a corrective plastic helmet

The greatest effectiveness is observed when treatment is carried out in the period from six months to one and a half years - at this time the brain grows sufficiently, but not too quickly, the diameter of the head increases by about half a centimeter every month, which allows you to correct the disorder that has arisen. After 1.5 years, the technique is ineffective, since head growth slows down significantly. This method is not recommended for use before 6 months, since the brain grows very quickly, wearing a helmet can cause a delay in the development of cerebral structures.

Treatment of other forms of plagiocephaly

If the severity of the compensatory type of disease is insignificant, correction is not required. As the child grows up, the hair completely hides the cosmetic defect. In severe cases, volumetric intervention is indicated - total reconstruction of the skull.

Due to significant aesthetic defects (including the facial part of the skull), functional disorders and the threat of complications, synostotic plagiocephaly is considered an indication for surgical treatment. The optimal timing of correction has not been fully determined. Some experts believe that the best period for using surgical techniques is the first three months of life. Others point to the absence of significant differences between long-term results in patients six months and one and a half years.

Achieving a satisfactory result is possible up to the age of 3 years, but the effectiveness of the method decreases as one gets older. During the operation, reconstruction of the frontal and upper orbital parts of the skull is performed. Bone flaps are formed and the upper edge of the orbit is moved. If necessary, absorbable plates or bone inserts are placed under the sunken parts. The bones are fixed with miniscrews and miniplates.

In the postoperative period, analgesics are prescribed and antibiotic therapy is carried out. If necessary, plastic helmets are additionally used to completely eliminate deformation. Non-absorbable structures are subsequently removed. Monitoring is carried out to assess the developmental characteristics and neurological status of the child, and timely detection of relapses.

Are they allowed into the army with such a diagnosis?

According to the law, young men of military age who have functional disorders of the spinal region can count on some restrictions on military service and receive special categories of fitness:

• partial unfitness for military service is established with moderately frequent pain syndromes and restriction of movement of the cervical vertebrae to fifty percent,
• in peacetime, the patient remains in reserve and cannot be called up,
• a conscript with a pathology can choose an alternative form of military service,
• a simplified form of military service (exemption from intense physical activity for medical reasons).

Important. With more serious developmental anomalies, the young man cannot be drafted into the army. The degree of suitability is determined by a military medical commission.

Reasons for the development of the syndrome

Klippel-Feil syndrome on an x-ray
A short neck in a newborn may appear under the influence of hereditary conditions or birth injuries. Another provoking factor may be a chromosomal disorder. With the syndrome, genetic abnormalities will be observed at the level of the fifth, eighth and 12th chromosomes.

Please note:

• The cause of the pathological condition is the injury received during childbirth: incorrect position of the fetus, slow passage through the birth canal;
• damage leads to degradation of the cervical parts of the spinal column and spinal cord;
• the provocateur may be Klippel-Feil syndrome, which is genetic in nature.

The latter condition in infants is associated with the absence of cervical vertebrae, a congenital decrease in their number, or subsequent fusion. In this case, the pathology may be accompanied by a disproportionate formation of the torso or internal organs.

Congenital stridor - symptoms and treatment

Stridor is a rough, variable sound caused by turbulent air flow passing through a narrowed area of ​​the upper respiratory tract [1][9].

The nature of stridor is not an indicator of the severity of the disease [7]. It becomes an alarming signal for other diseases if there are symptoms such as shortness of breath, cyanosis (blue discoloration of the skin), and deterioration in general condition.

Congenital stridor of the larynx is characterized by the absence of malformations of the respiratory tract, a predominantly benign course (in more than 70% of cases) and a temporary nature with spontaneous disappearance after 1-3 years [7]. This syndrome was described by German pediatricians in the middle of the 19th century. At the end of the 19th century, surgeons discovered that the larynx was not damaged in the syndrome [12]. Diagnosis using spontaneous breathing showed that congenital stridor is caused by laryngomalacia, an unusual congenital softness of cartilage that leads to collapse of the child’s larynx [3].

Laryngomalacia is a relatively mild congenital anomaly that is the most common cause of stridor in children immediately after birth or at 1-4 weeks of life.

There is evidence that laryngomalacia is a genetically determined disease with an autosomal dominant mode of inheritance. Its frequency in the population is more than 4%, and the proportion among all cases of congenital stridor is more than 40% [7]. The cause of laryngomalacia is the delayed development of the larynx in the prenatal period [3].

Previously, congenital stridor was reflected in the International Classification of Diseases, 10th revision (ICD 10) as an independent disease under code P 31.4. But today there is no such disease in the ICD, due to the fact that “stridor” has been recognized as a symptom and not a disease since 2014. For this reason, confusion is possible, because in medical textbooks of the Soviet period the term “congenital stridor” was used only to refer to “laryngomalacia.” The problem is that congenital laryngeal stridor is a sign not only of laryngomalacia, but also of a number of other, mainly neurological disorders [13]. And yet, at present, most English-language literary sources equate the terms “congenital stridor” and “laryngomalacia” [14][15][16].

Theories of laryngomalacia:

• anatomical - the disease occurs due to changes in the size of the structures of the larynx;
• cartilaginous - the cause of the disease is changes in the internal structure of the cartilage of the larynx;
• neurological - disorders of the neuromuscular coordination of the larynx lead to pathology [1].

A large role in the development of the anomaly is given to provoking factors: toxoplasmosis and viral diseases of the mother during pregnancy [5].

Treatment options

The Shants collar supports the neck in the correct position and stretches the spine.
During the recovery process for doll necks in infants, various techniques are used, from the Shants collar to massage and physiotherapeutic procedures. Each of the methods for correcting deformation should be considered separately.

Shants collar

It is a special orthopedic splint. The main task is to reduce the load on the cervical spine. An additional result is achieved by fixing it in the correct position from an anatomical point of view.

The Shants collar is selected individually. In this case, it is necessary to take into account the weight category and body volume of the baby. The tire is made to order or can be purchased at a specialty store.

Physiotherapeutic procedures

Electrophoresis on the neck area of ​​a child
Physiotherapeutic measures guarantee the release of tension in the muscle area, as well as enhancing the results of wearing a Shants collar. Without them, recovery in childhood will be incomplete.

Efficiency:

• manages to relieve tension in the neck muscles;
• blood circulation improves;
• the supply of oxygen to the brain increases.

Electrophoresis is indicated for short neck syndrome in infants. Special electrodes in a cover are applied to the problem area. They are impregnated with medicinal compounds and provide current. The procedure is painless.

Drug ions penetrate the skin. They also affect all problem cells of the body. This ensures the fastest possible therapeutic effect. Physiotherapy sessions are carried out over several weeks, a maximum of a month. After this, a break and additional treatment are recommended.